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Orthopaedic Surgery - Clavicle Fractures
Basics
Clavicle fractures are among the most common upper-extremity fractures encountered in the emergency department.
They occur most frequently in males younger than 20 years, while among older adults, particularly those over 65 years, women account for a greater proportion of cases.
Clavicle fractures are classified according to their location, displacement, comminution, and possible intra-articular extension.
Classification
Several classification systems are available, including the Neer, Edinburgh, and Müller AO systems.
From an anatomic perspective, fractures are divided into middle-third, lateral-third, and medial-third injuries.
Middle-third fractures are by far the most common, followed by lateral and then medial clavicle fractures.
Open Fractures
Open clavicle fractures are uncommon.
When they occur, they are often associated with substantial trauma involving the head, thorax, or major vessels.
Clavicle fractures are also frequently encountered in patients with multiple traumatic injuries.
Fracture Displacement
The characteristic displacement of a midshaft clavicle fracture is produced by opposing muscular and gravitational forces.
The sternocleidomastoid muscle pulls the medial fragment superiorly, while the pectoralis major and the weight of the arm tend to pull the lateral fragment inferiorly and medially.
These forces may create visible shortening or deformity.
Neurovascular Anatomy
The clavicle lies directly over the subclavian vein and is close to the subclavian artery and brachial plexus.
Although injury to these structures is uncommon, neurovascular damage may occur at the time of trauma or during operative fixation.
A careful neurovascular examination is therefore essential.
Lateral Clavicle Fractures
The displacement pattern of lateral clavicle fractures depends strongly on the integrity of the coracoclavicular ligaments, acromioclavicular joint, and surrounding ligamentous structures.
Disruption of these stabilizers may make the fracture unstable and increase the risk of nonunion.
General Prevention
Fall prevention is particularly important in patients with impaired balance or a history of recurrent falls.
Assistive devices, home-safety assessment, and nursing or rehabilitation evaluation may help reduce future injury.
In older adults sustaining a clavicle fracture after a low-energy fall, evaluation and treatment of possible osteoporosis may reduce the risk of subsequent fragility fractures.
Epidemiology
Clavicle fractures occur more often in men than women among patients younger than 65 years, with a reported male-to-female ratio of approximately 2.2:1.
The mean age at the time of fracture has been reported to be approximately 48 years.
Fractures occur more frequently during weekends and summer months, probably reflecting increased recreational and sporting activity.
Incidence
Reported incidence varies between populations.
One epidemiologic study found approximately 5.8 clavicle fractures per 10,000 people, while a recent U.S. study reported approximately 24.4 fractures per 100,000 person-years.
The highest incidence occurs between approximately 10 and 19 years of age.
Risk Factors
Risk factors include male sex, young age, recurrent falls, osteoporosis, participation in sports, bicycle accidents, motor vehicle collisions, and other forms of trauma.
Sports account for a substantial proportion of emergency-department presentations involving clavicle fractures.
Genetics
Genetic disorders that weaken bone may increase susceptibility to clavicle fracture.
Examples include Ehlers-Danlos syndrome and fibrous dysplasia, although most clavicle fractures occur without an underlying hereditary disorder.
Etiology
The most common mechanism is a fall, frequently directly onto the shoulder.
Other mechanisms include bicycle and motorcycle accidents, motor vehicle collisions, and contact-sport injuries.
Sports-Related Injuries
Among sporting activities, bicycling is particularly commonly associated with clavicle fracture.
Football and soccer are other frequent causes.
Age and Injury Mechanism
Men are more likely to sustain clavicle fractures through higher-energy mechanisms, whereas women, particularly older women, more often sustain fractures after falls or other relatively low-energy trauma.
Associated Conditions
Associated injuries are common, particularly after high-energy trauma.
Important associated conditions include rib fractures, pneumothorax, cervical spine injury, head injury, scapular fracture, vascular injury, and nerve injury.
Polytrauma
Patients with a clavicle fracture after major trauma require a complete trauma assessment.
Concurrent fractures may involve the spinal column, scapula, cranium, forearm, proximal humerus, or other parts of the shoulder girdle.
Spinal column fractures are among the important associated skeletal injuries.
Scapulothoracic Dissociation
A widely displaced clavicle fracture in a high-energy injury should raise concern for scapulothoracic dissociation.
This severe shoulder-girdle injury may be associated with major vascular and brachial plexus injury.
Diagnosis
Signs and Symptoms
Typical findings include pain with movement of the affected upper extremity, visible or palpable deformity over the clavicle, swelling, bruising, and ecchymosis.
Patients often support the injured arm with the opposite hand.
Skin Tenting
The displaced fracture fragment may push against the overlying skin, producing skin tenting.
Marked tenting or blanching indicates threatened skin integrity and may represent an indication for urgent operative treatment.
Physical Examination
The clavicular region and the entire upper extremity should be inspected carefully.
The examiner should look for open wounds, abrasions, skin tenting, expanding hematoma, abnormal alignment, or evidence of additional injury.
Neurovascular Examination
A careful neurovascular examination is essential.
Motor function and sensation throughout the upper extremity should be evaluated and compared with the opposite side.
Vascular Examination
Although vascular injury is rare, the examiner should confirm symmetric distal pulses and brisk capillary refill.
An expanding hematoma, diminished pulse, cool extremity, or other evidence of vascular compromise requires urgent assessment.
Examination for Associated Injuries
The remainder of the injured extremity should be palpated and the major joints examined.
Additional fractures, shoulder dislocation, or other injuries may coexist and should not be overlooked.
Trauma Examination
Patients injured through high-energy mechanisms require a complete trauma examination to identify associated thoracic, spinal, abdominal, cranial, or extremity injuries.
Elderly Patients After Falls
Older adults who sustain a clavicle fracture after a fall should also be evaluated for head and cervical spine injury.
This is particularly important in patients with recurrent falls or those taking anticoagulant medication.
Imaging
Dedicated Clavicle Radiographs
Standard evaluation includes dedicated radiographs of the clavicle.
An anteroposterior view and approximately 20° cephalad view are commonly obtained.
Chest Radiograph
An AP chest radiograph can help assess fracture displacement and compare the injured clavicle with the opposite side.
It may also reveal associated injuries such as rib fractures or pneumothorax.
Shoulder Radiographs
Dedicated shoulder radiographs can help exclude associated injuries involving the scapula, acromioclavicular joint, or proximal humerus.
CT
CT is not routinely required for uncomplicated midshaft fractures.
It may be helpful for medial clavicle fractures, complex fracture patterns, or when the relationship to adjacent thoracic structures is unclear.
Vascular Imaging
If vascular injury is suspected, angiographic imaging may be required to evaluate the subclavian or other nearby vessels.
Additional Imaging
Additional radiographs or advanced imaging should be obtained according to the mechanism of injury and clinical suspicion for associated trauma.
Pathologic Fracture Evaluation
Pathologic testing is not routinely necessary.
A pathologic fracture should be considered when the injury occurs after an unusually minor mechanism, when there is a personal or strong family history of malignancy, or when radiographs demonstrate a suspicious underlying bony lesion.
Differential Diagnosis
The differential diagnosis includes acromioclavicular joint separation, rib fracture, scapular fracture, shoulder dislocation, sternoclavicular joint dislocation, and proximal humerus fracture.
Treatment
General Principles
Treatment depends on both patient factors and fracture characteristics.
Most clavicle fractures can be managed nonoperatively, although operative fixation has become more common in selected displaced fractures.
Nondisplaced Fractures
Nondisplaced or minimally displaced fractures usually heal successfully with nonoperative care.
A sling, pain control, and gradual restoration of shoulder motion are typically sufficient.
Medial and Lateral Fractures
Many medial and lateral clavicle fractures can also be managed without surgery when alignment and stability are acceptable.
However, unstable lateral fractures require closer assessment because the risk of nonunion is higher.
Children Versus Adults
Children have greater remodeling potential and a higher likelihood of healing displaced fractures than adults.
Consequently, substantial displacement that might prompt surgery in an adult may still be treated successfully without surgery in a younger child.
Monitoring Displaced Fractures
Displaced fractures treated nonoperatively should undergo repeat radiographs during the first several weeks to ensure that alignment has not worsened.
Sleeping Position
During the painful acute phase, patients may find it more comfortable to sleep in a chair or recliner rather than lying flat.
Activity
Sling Immobilization
A sling is commonly used for approximately 2–4 weeks.
During the early period, the involved upper extremity is generally kept non-weight-bearing.
Early Joint Motion
The sling should be removed several times each day for gentle elbow, wrist, and hand range-of-motion exercises.
This helps prevent stiffness in uninvolved joints.
Shoulder Motion
Gentle shoulder range of motion can usually begin after approximately 2–4 weeks, depending on pain, fracture stability, and clinical progress.
More strenuous motion and strengthening are delayed until healing has progressed.
Figure-of-Eight Brace
A figure-of-eight clavicle brace may be used, but it has not shown a clear advantage over a simple sling.
It may also produce greater discomfort during the early treatment period.
Axillary Care
When using a sling, the axillary fold should be kept clean and dry to prevent irritation, moisture accumulation, and skin breakdown.
Preoperative Activity
Patients awaiting surgery should generally remain non-weight-bearing in a sling.
After fixation, activity restrictions depend on the surgical construct, implant, and surgeon’s postoperative protocol.
Physical Therapy
Nonoperative Rehabilitation
Formal physical therapy is not necessary for every patient.
For nonoperatively treated fractures, gentle motion can begin after approximately 2–4 weeks.
Early Exercises
Rehabilitation may begin with pendulum and pulley exercises.
These are gradually advanced toward full shoulder range of motion as pain decreases and healing progresses.
Postoperative Therapy
The timing of physical therapy after surgery varies according to fixation stability, patient factors, and surgeon preference.
Stable fixation often allows earlier controlled motion.
Medication
First-Line Treatment
Pain management may include ice, acetaminophen, and NSAIDs when appropriate.
Some surgeons limit NSAID use because of theoretical concerns regarding bone healing, although practice varies.
Additional Analgesics
Tramadol may be considered for selected patients.
Short courses of opioid analgesics are sometimes required during the acute painful period.
Opioid Considerations
Before prescribing opioids, factors such as patient age, comorbidities, concurrent medications, fall risk, and overall health should be considered.
Surgery
Indications
Operative treatment may be considered for an open fracture, threatened skin, floating shoulder, severe displacement, significant shortening, or other unstable fracture patterns.
Shortening greater than approximately 2 cm may contribute to the decision for surgery in selected displaced midshaft fractures.
Floating Shoulder
A floating shoulder refers to major disruption of the shoulder suspensory complex, often involving an ipsilateral scapular neck fracture together with clavicular injury.
This pattern may warrant operative stabilization depending on displacement and overall injury severity.
Open Reduction and Internal Fixation
Surgery commonly involves open reduction and internal fixation (ORIF) with plates, screws, or other internal fixation devices.
The goals are restoration of length and alignment, stable fixation, and facilitation of earlier functional rehabilitation.
Follow-Up
Prognosis
Most clavicle fractures heal successfully.
Outcome depends on fracture location, displacement, age, soft-tissue condition, and whether the fracture is treated surgically or nonoperatively.
Operative Treatment of Displaced Midshaft Fractures
Surgical fixation of appropriately selected displaced midshaft fractures can reduce the rates of nonunion and symptomatic malunion compared with nonoperative treatment.
It may also permit a quicker early return to work and improved short-term functional recovery.
Hardware Removal
One disadvantage of operative fixation is the relatively high rate of subsequent procedures.
Many patients request hardware removal because the clavicle is subcutaneous and plates can be prominent or irritating.
Nonunion
Some displaced midshaft fractures treated nonoperatively progress to nonunion.
Not all nonunions are sufficiently symptomatic to require further treatment.
Lateral Clavicle Nonunion
Nonunion is relatively common after certain lateral clavicle fractures.
However, some radiographic nonunions remain minimally symptomatic and do not require surgery.
Complications
Infection
Surgical fixation carries a risk of wound infection and deep implant-related infection.
Hardware Failure
Internal fixation devices may loosen, break, or fail, especially if union is delayed or excessive loading occurs too early.
Nonunion
Failure of the fracture to unite is more likely with marked displacement, shortening, comminution, smoking, and certain unstable lateral fracture patterns.
Symptomatic Malunion
A clavicle fracture that heals with substantial shortening or deformity may produce pain, weakness, altered shoulder mechanics, or cosmetic dissatisfaction.
Symptomatic malunion is more commonly encountered after markedly displaced fractures treated nonoperatively.
Shoulder Stiffness
Prolonged immobilization or associated shoulder injury may result in post-traumatic stiffness.
Early controlled motion after an appropriate period of fracture protection can reduce this risk.
Supraclavicular Nerve Symptoms
Numbness over the clavicle or upper chest may occur because the cutaneous supraclavicular nerve branches can be stretched or injured during the initial trauma.
They may also be cut or stretched during surgical exposure.
Neurovascular Injury
Although rare, injury to the brachial plexus or subclavian vessels can occur because of their close relationship to the clavicle.
These complications can occur from the initial fracture or during operative treatment.
Patient Monitoring
Early Radiographic Monitoring
Radiographs should be obtained every few weeks during the early post-injury or postoperative period to confirm maintenance of alignment.
This is especially important for displaced fractures treated without surgery.
Healing Surveillance
After the acute period, imaging may be repeated approximately every 4–6 weeks until satisfactory clinical and radiographic union is demonstrated.
Clinical Monitoring
Follow-up should assess pain, skin condition, neurovascular status, shoulder range of motion, tenderness at the fracture site, and functional recovery.
Increasing pain, new neurologic symptoms, skin compromise, or progressive deformity should prompt earlier reassessment.
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Orthopaedic Surgery - Chordoma
Basics
Chordoma is a low-grade malignant bone tumor arising from remnants of the embryologic notochord.
It accounts for approximately 1–4% of primary malignant bone tumors and is one of the most important primary malignant tumors involving the axial skeleton, particularly the spine and sacrum.
Anatomic Distribution
Chordomas most commonly arise in the sacrococcygeal region, which accounts for approximately 55% of cases.
Around 30% occur in the skull base, particularly the sphenoid or clival region, while approximately 15% develop within the cervical, thoracic, or lumbar spine.
Delayed Diagnosis
Diagnosis is frequently delayed because symptoms are often nonspecific.
Sacral chordoma may present simply as chronic low back, pelvic, or sacral pain and may therefore be mistaken for much more common degenerative musculoskeletal disorders.
Dedifferentiation
Although conventional chordoma is generally considered a low-grade malignancy, a small proportion of tumors, historically reported at less than 5%, may undergo dedifferentiation into a high-grade spindle-cell sarcoma.
Dedifferentiated chordoma behaves much more aggressively than the conventional form.
General Precautions
One of the most important diagnostic pitfalls is failure to consider sacrococcygeal chordoma in a patient with persistent unexplained low back or sacral pain.
Chronic symptoms that are progressive, atypical, or associated with neurologic, bowel, or bladder dysfunction warrant further investigation.
Epidemiology
Chordoma is extremely rare.
The reported annual incidence is approximately 0.08 cases per 100,000 people.
It most commonly presents in adulthood, with a peak incidence around the fourth to fifth decades of life, although it may occur outside this age range.
Risk Factors
No established environmental or lifestyle risk factors have been identified.
Most cases occur sporadically.
Genetics
No common hereditary predisposition is recognized in the majority of patients.
The disease is generally not associated with a typical familial pattern.
Etiology
Chordoma develops from persistent notochordal remnants within the axial skeleton.
The notochord normally contributes to embryologic development of the spine and largely disappears before birth, but residual cells may persist and later undergo malignant transformation.
Tumor Growth
Chordomas often extend beyond the confines of bone.
Sacral tumors, in particular, may produce a large anterior soft-tissue mass extending into the pelvis.
Because the tumor may become very large before producing obvious symptoms, substantial local destruction can be present by the time of diagnosis.
Associated Conditions
No specific associated systemic disorders are typically present.
Diagnosis
Signs and Symptoms
Symptoms depend on tumor location but are usually slowly progressive and nonspecific.
Sacral lesions commonly produce low back pain, pelvic pain, sacral discomfort, or anal pain.
Difficulty Sitting
Patients with sacrococcygeal tumors may find prolonged sitting increasingly uncomfortable.
Pain may arise from direct pressure on the tumor or involvement of adjacent pelvic structures.
Bowel and Bladder Symptoms
Constipation, urinary disturbance, or other pelvic-organ symptoms may develop as the tumor enlarges.
These findings can result from compression or invasion of adjacent pelvic structures or sacral nerve roots.
Radiculopathy
Tumor extension around neural structures may compress or destroy adjacent nerve roots.
This can produce radicular pain, sensory loss, or motor dysfunction corresponding to the level of involvement.
In sacral chordoma, the S1, S2, and S3 roots are commonly at risk.
Physical Examination
Physical findings are often limited despite substantial tumor size.
There may be little to suggest an underlying bone malignancy on routine examination.
Rectal Examination
Large sacral chordomas frequently extend anteriorly into the pelvis.
A mass may be palpable during rectal examination in approximately half of patients with sufficiently advanced sacral disease.
Imaging
Plain Radiographs
Plain radiographs may appear normal or show only subtle abnormalities.
Findings can include bone destruction, cortical expansion, or a lytic sacral lesion.
Limitations of Radiography
Sacral tumors are particularly easy to miss on plain radiographs because bowel gas and fecal material may obscure the lesion.
A normal radiograph therefore does not exclude chordoma when clinical suspicion remains high.
CT
CT is highly sensitive for demonstrating the bony component of chordoma.
Typical findings include midline osseous destruction and an anterior soft-tissue mass.
Calcification
Scattered internal calcifications may be visible on CT.
These mineralized areas can help characterize the lesion but are not specific to chordoma.
MRI
MRI is the preferred modality for defining the full extent of the tumor and its relationship to surrounding structures.
Chordomas are typically low signal intensity on T1-weighted images and high signal intensity on T2-weighted images.
Contrast-Enhanced MRI
Contrast-enhanced MRI helps delineate the dimensions of the mass and its relationship to the spinal canal, nerve roots, pelvic organs, vessels, and surrounding soft tissues.
This information is essential for operative planning.
Imaging the Entire Sacrum
Sacrococcygeal lesions may lie at the extreme inferior edge of lumbar or pelvic MRI studies.
The tumor can therefore be overlooked if the imaging field does not extend far enough inferiorly.
When sacral chordoma is suspected, imaging should include the entire sacrum and coccyx down to the coccygeal tip.
Nuclear Medicine
Conventional technetium bone scans and PET have historically been less useful than CT and MRI for primary characterization of chordoma.
Cross-sectional imaging remains central to diagnosis, staging, and surveillance.
Differential Diagnosis
Destructive Spinal Lesions
Important alternative diagnoses include metastatic bone disease, multiple myeloma, and lymphoma, all of which can produce destructive lesions of the spine or sacrum.
Sacral Tumors
Chondrosarcoma and giant cell tumor may also arise in the sacrum.
Both can produce bone destruction, soft-tissue extension, and in some cases mineralization, making differentiation from chordoma necessary.
Chondrosarcoma
Chondrosarcoma may resemble chordoma radiographically, particularly when it occurs in the pelvis or sacrum.
Tumor location, pattern of calcification, MRI appearance, and histology help distinguish the two lesions.
Treatment
General Principles
The primary treatment for chordoma is complete surgical removal whenever technically feasible.
Because local recurrence strongly influences survival, achieving adequate margins is critical.
Wide En Bloc Resection
The preferred operative strategy is wide en bloc resection with negative surgical margins.
The tumor is removed as a single specimen together with a surrounding margin of uninvolved tissue when anatomy permits.
Importance of Surgical Margins
Incomplete excision or tumor contamination during surgery substantially increases the risk of local recurrence.
Unfortunately, chordomas often lie immediately adjacent to major nerves, blood vessels, bowel, bladder, or spinal structures, making wide margins difficult to achieve.
Chemotherapy
Conventional chemotherapy has little established role in the routine treatment of classic chordoma.
The relatively indolent biology of the tumor and limited chemosensitivity make surgery and local control the major therapeutic priorities.
Radiotherapy
High-dose radiation, particularly proton-beam or other conformal particle-based radiotherapy, may be considered in selected patients.
It can be used when surgical margins are limited, the tumor is unresectable, or as part of a combined treatment strategy.
Follow-Up
Prognosis
Chordoma has a prolonged but potentially aggressive natural history.
Historical series report a median survival of approximately 6.3 years.
Five-year survival has been reported in the range of approximately 50–67%, while 10-year survival has ranged from roughly 28–46%.
Metastatic Risk
The risk of distant metastasis has historically been estimated at approximately 10–40%.
Common metastatic sites include the lungs, bone, liver, and lymph nodes.
Importance of Local Recurrence
Chordoma is unusual among low-grade tumors because local recurrence can itself be a major cause of mortality.
Repeated local growth can progressively involve vital neural, vascular, gastrointestinal, and genitourinary structures.
Relationship Between Recurrence and Survival
Local recurrence is associated with poorer survival.
The best chance for durable control therefore comes from achieving adequate surgical margins during the initial operation whenever possible.
Complications
Wound-Healing Problems
Wound complications are common after major sacral resections.
Historical series have reported wound-healing problems in as many as 45% of patients.
Large incisions, dead space, radiation, extensive soft-tissue dissection, and contamination risk may all contribute.
Injury to Pelvic Structures
Sacral chordoma surgery may place important anterior pelvic structures at risk.
Potential injuries include damage to the iliac vessels, rectum, bladder, ureters, and other pelvic organs.
Sacral Nerve-Root Sacrifice
The functional consequences of surgery depend heavily on which sacral nerve roots must be sacrificed to achieve tumor clearance.
Higher-level bilateral sacrifice produces more severe neurologic deficits.
Bilateral S1 Sacrifice
Sacrifice of both S1 nerve roots, as may occur with total sacrectomy, can result in major lower-extremity motor and sensory deficits together with loss of bowel and bladder function.
Bilateral S2 Sacrifice
Historical data indicate that bilateral S2 root sacrifice is associated with an extremely high likelihood of bowel and bladder dysfunction.
Some patients may require permanent diversion procedures.
Bilateral S3 Sacrifice
Bilateral S3 sacrifice carries a substantial risk of bowel and bladder dysfunction.
Historical series have reported bowel dysfunction in approximately 60% and bladder dysfunction in approximately 75% of such patients.
Bilateral S4 Sacrifice
Sacrifice limited to both S4 roots is associated with a lower risk of bowel dysfunction, although bladder disturbance may still occur.
Historical series reported minimal bowel dysfunction but bladder dysfunction in approximately 31% of patients.
Sexual Dysfunction
Loss of sacral nerve function may also produce sexual dysfunction, depending on the level and extent of nerve-root sacrifice.
Spinopelvic Instability
Major sacral resections can disrupt continuity between the spine and pelvis.
This may produce spinopelvic instability or discontinuity and can require complex reconstruction.
Sacral Insufficiency Fracture
Remaining sacral or pelvic bone may be vulnerable to insufficiency fracture following extensive resection.
Mechanical reconstruction may be required when stability is compromised.
Patient Monitoring
Long-Term Surveillance
Patients require lifelong surveillance because chordoma can recur many years after initial treatment.
Follow-up commonly includes CT or MRI of the operative region once or twice each year, with intervals individualized according to recurrence risk and prior treatment.
Surveillance for Metastases
Imaging should also assess for distant metastatic disease, particularly involving the lungs.
Long-term chest surveillance is therefore commonly incorporated into follow-up.
Clinical Monitoring
Follow-up should assess for new pain, neurologic symptoms, bowel or bladder dysfunction, changes in sitting tolerance, and evidence of wound or reconstructive complications.
Any new or progressive symptom should prompt timely imaging because recurrent disease may initially be subtle.
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Orthopaedic Surgery - Chondrosarcoma
Basics
Chondrosarcoma is a primary malignant bone tumor composed of neoplastic cartilage-forming cells within a cartilaginous matrix.
It most commonly involves the pelvis, proximal femur, region around the knee, and spine, although other skeletal sites may also be affected.
Its biologic behavior varies widely depending on histologic subtype and grade.
Tumor Grade and Metastatic Risk
Low-grade, or grade 1, chondrosarcoma has a relatively low metastatic potential, generally less than approximately 5%.
Intermediate-grade, or grade 2, disease carries a higher metastatic risk, commonly around 20–30%.
High-grade or dedifferentiated tumors have a much more aggressive course, with metastatic rates that may approach or exceed 70%.
Mesenchymal chondrosarcoma is also highly aggressive, with a metastatic risk greater than 50%.
Epidemiology
Chondrosarcoma accounts for approximately 20% of primary malignant bone tumors and occurs about half as frequently as osteosarcoma.
It is predominantly a disease of older adults, most often presenting in the sixth through eighth decades of life.
Risk Factors
Important predisposing conditions include hereditary multiple exostoses, Ollier disease, and Maffucci syndrome.
These disorders increase the likelihood of malignant transformation in pre-existing cartilaginous lesions.
Genetics
The genetic basis of chondrosarcoma is heterogeneous and incompletely understood.
Abnormalities involving several chromosomes have been described, including chromosomes 2–11, 14, 15, and 21.
EXT Mutations
Patients with hereditary multiple exostoses caused by an EXT1 mutation appear to have a greater risk of malignant transformation than patients with EXT2 or EXT3 abnormalities.
Myxoid Chondrosarcoma Genetics
Myxoid variants have been associated with a characteristic chromosomal translocation involving chromosomes 9 and 22.
Etiology
Most chondrosarcomas arise de novo, meaning they develop as primary malignant tumors without a known pre-existing lesion.
Others are secondary tumors that develop through malignant transformation of a previously benign cartilage lesion.
Secondary Chondrosarcoma
Secondary chondrosarcoma may arise from a pre-existing osteochondroma or enchondroma.
This is particularly important in patients with hereditary multiple exostoses, Ollier disease, or Maffucci syndrome.
When malignant transformation occurs in these settings, the resulting tumor is often initially low grade.
Dedifferentiated Chondrosarcoma
Dedifferentiated chondrosarcoma accounts for approximately 10% of cases.
It contains a conventional cartilaginous tumor adjacent to a highly malignant noncartilaginous component.
This is one of the most aggressive forms of chondrosarcoma and carries a poor prognosis.
Associated Conditions
Chondrosarcoma is associated particularly with disorders that produce multiple exostoses or enchondromatosis.
Recognition of these underlying conditions is important because changes in pain, lesion size, or imaging appearance may indicate malignant transformation.
Diagnosis
Signs and Symptoms
The typical presentation is deep, gradually progressive pain.
Symptoms may be present for months or even years because many chondrosarcomas grow relatively slowly.
Pain Characteristics
Pain may be worse at night and may persist despite rest.
Unlike many benign musculoskeletal conditions, the discomfort often becomes progressively more constant.
Analgesics or NSAIDs may provide partial relief but usually do not eliminate the symptoms.
Soft-Tissue Mass
With longstanding disease or cortical breakthrough, a palpable soft-tissue mass may develop.
This suggests extension of the tumor beyond the confines of the bone.
History
A high index of suspicion is appropriate when an adult with a known cartilaginous lesion develops new pain, night pain, progressive enlargement, or pain that is no longer relieved by rest.
These features are particularly concerning for malignant transformation.
Physical Examination
The examination is generally nonspecific.
Patients may have tenderness with deep palpation or, in more advanced disease, a palpable soft-tissue mass.
Joint motion may be limited if the tumor is large or located near an articulation.
Laboratory Tests
Routine serum tests are generally not diagnostic.
There are no specific blood markers that reliably establish the diagnosis of chondrosarcoma.
Imaging
Plain Radiographs
Standard AP and lateral radiographs are often highly suggestive of the diagnosis.
The tumor usually appears as an intramedullary lesion containing stippled, ring-like, or arc-shaped calcifications within a cartilaginous matrix.
Cortical Changes
More aggressive lesions may produce substantial cortical abnormalities.
These can include cortical erosion, thickening, expansion, endosteal scalloping, and frank bone destruction.
Chest Imaging
Because chondrosarcoma can metastasize, particularly to the lungs, staging commonly includes chest imaging.
Chest radiographs or, more commonly, CT of the chest may be obtained to evaluate for pulmonary metastases.
MRI
MRI of the affected region is useful for defining the intramedullary extent, cortical involvement, soft-tissue extension, neurovascular relationships, and relationship to nearby joints.
It is also valuable for planning the biopsy approach and surgical resection margins.
Biopsy Planning
The biopsy should be planned carefully because the biopsy tract must usually be removed during definitive tumor resection.
Whenever possible, biopsy planning should be coordinated with the surgeon who will perform the definitive oncologic procedure.
Pathological Findings
Histologic distinction between a benign enchondroma and a well-differentiated low-grade chondrosarcoma can be difficult.
For this reason, pathologic interpretation must be correlated closely with the clinical and radiographic findings.
Trabecular Permeation
A characteristic malignant feature is permeation of pre-existing trabecular bone by the cartilaginous tumor.
This supports an infiltrative rather than expansile benign growth pattern.
Chondroid Matrix
The tumor contains malignant cartilage-producing cells within a chondroid matrix.
Lobulated growth is common.
Cellular Features
Histologic findings may include binucleated chondrocytes, increased cellularity, nuclear atypia, and progressive anaplasia with increasing tumor grade.
Histologic Grading
Conventional chondrosarcomas are usually graded from 1 through 3 according to cellularity, nuclear atypia, mitotic activity, and degree of anaplasia.
Higher grade correlates with a greater risk of metastasis and poorer survival.
Differential Diagnosis
The principal differential diagnoses include enchondroma and bone infarction.
Distinguishing a low-grade chondrosarcoma from an enchondroma can be particularly difficult and often requires integration of symptoms, imaging, and histology.
Treatment
General Measures
Surgery is the mainstay of treatment for conventional chondrosarcoma.
The goal is complete tumor removal with an adequate oncologic margin, thereby minimizing the risk of local recurrence.
Role of Chemotherapy and Radiotherapy
Conventional chondrosarcoma is relatively resistant to both chemotherapy and radiotherapy.
These modalities therefore have a limited role in routine treatment, although selected aggressive subtypes or metastatic disease may be managed differently.
Wide Resection
A wide resection removes the entire tumor together with a surrounding cuff of normal tissue.
This is the standard surgical principle for most intermediate- and high-grade lesions.
Low-Grade Disease
Low-grade lesions may be managed with appropriately selected surgical excision and close radiographic surveillance.
Follow-up commonly includes serial imaging at approximately 6-month intervals during the early surveillance period.
Intermediate- and High-Grade Disease
Patients with grade 2 or 3 chondrosarcoma require more intensive metastatic surveillance.
CT imaging of the chest is commonly performed at regular intervals because the lungs are the most frequent site of metastasis.
Long-Term Surveillance
After approximately 5 years without evidence of disease, follow-up intervals may be increased.
However, long-term surveillance remains important because some chondrosarcomas can recur late.
Physical Therapy
Physical therapy is used after surgery to restore range of motion, strength, gait, and functional independence.
The specific program depends on tumor location and the type of reconstruction performed.
Medication
There is no established medication that reliably treats localized conventional chondrosarcoma.
Patients who develop metastatic disease may receive systemic therapy, but conventional chemotherapy has historically shown limited and inconsistent benefit.
Surgery
Wide resection requires removal of all involved bone and soft tissue while maintaining an adequate margin around the tumor.
The resulting defect may require substantial reconstruction.
Reconstruction
Limb reconstruction can be performed using an allograft, custom prosthesis, modular tumor prosthesis, or other reconstructive technique depending on the site and extent of resection.
Soft-Tissue Reconstruction
Muscle flaps or other reconstructive soft-tissue procedures may be required to cover large defects and protect implants or exposed structures.
Follow-Up
Close postoperative surveillance is required to detect local recurrence, metastasis, or failure of reconstruction.
The intensity of follow-up depends on tumor grade and time since treatment.
Prognosis
Prognosis is strongly determined by histologic grade and subtype.
Low-grade conventional chondrosarcoma generally has an excellent prognosis.
Grade 1 Prognosis
Grade 1 tumors have a metastatic risk of less than approximately 5% and usually have very favorable long-term outcomes when adequately resected.
Grade 2 Prognosis
Grade 2 tumors have a higher metastatic potential, generally below approximately 30%, but overall prognosis remains substantially better than for high-grade disease.
High-Grade and Dedifferentiated Disease
Grades 3 and dedifferentiated tumors carry a poor prognosis because of their high metastatic potential.
Metastatic rates may exceed 70%.
Dedifferentiated Chondrosarcoma Prognosis
Dedifferentiated chondrosarcoma is among the most aggressive subtypes.
Historical series have reported very low long-term survival, with rapid progression once metastatic disease develops.
Mesenchymal Chondrosarcoma
Mesenchymal chondrosarcoma also carries a relatively poor prognosis because of its aggressive behavior and high metastatic potential.
Complications
Important complications include local recurrence, metastatic disease, and failure of reconstruction.
The risk of recurrence rises when surgical margins are inadequate or the tumor is high grade.
Local Recurrence
Local recurrence may result from incomplete resection or microscopic residual disease.
Recurrent tumors can be more difficult to treat and may require additional wide resection or amputation in selected cases.
Metastases
Metastatic spread occurs mainly in intermediate- and high-grade tumors.
The lungs are the most common site, although other organs and bones may also be involved.
Reconstruction Failure
Large oncologic reconstructions can fail because of infection, mechanical loosening, fracture, nonunion, implant failure, or soft-tissue complications.
These problems may require revision surgery.
Patient Monitoring
Patients should initially be reviewed frequently, often at approximately 1–3-month intervals during rehabilitation, depending on the extent of surgery and tumor grade.
Subsequent surveillance should monitor the surgical site, reconstruction, local recurrence, and pulmonary metastases, with imaging intervals adjusted according to oncologic risk.
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Orthopaedic Surgery - Chondroblastoma
Basics
Chondroblastoma is a benign bone tumor of cartilaginous origin that characteristically arises in the epiphysis of skeletally immature patients.
It most often develops in the epiphyses of long bones, particularly around major joints.
The proximal humerus is the most commonly involved site, followed by the proximal tibia and femur.
Synonyms
Chondroblastoma of the proximal humeral epiphysis has historically been referred to as a Codman tumor.
Another older term is epiphyseal chondromatous giant cell tumor.
Epidemiology
Chondroblastoma has a mild male predominance, with males affected approximately twice as often as females.
It occurs mainly in adolescents and young adults whose physes are still open or have only recently closed.
Incidence
Chondroblastoma is uncommon.
In large tumor series, it has accounted for approximately 1% of all skeletal neoplasms.
Risk Factors
No specific environmental, developmental, or lifestyle risk factors have been identified.
Genetics
No well-established hereditary predisposition has traditionally been recognized for chondroblastoma.
The tumor is generally considered sporadic.
Etiology
The precise cause remains uncertain.
The neoplastic cells are believed to arise from cartilage-producing precursor cells, or chondroblasts.
Some pathologic similarities exist between chondroblastoma and chondromyxoid fibroma, although they are distinct tumors.
Associated Conditions
Chondroblastomas may contain areas resembling an aneurysmal bone cyst (ABC).
These secondary aneurysmal changes can influence the radiographic appearance and may be associated with a greater risk of recurrence.
Diagnosis
Signs and Symptoms
The most common complaint is persistent mild to moderate pain near the involved joint.
Symptoms often develop gradually and may persist for months or even years before diagnosis.
Joint Stiffness
Because the tumor develops close to an articular surface, patients frequently experience stiffness and loss of motion in the adjacent joint.
Joint Effusion
An effusion may develop in the nearby joint.
This can make the presentation resemble an intra-articular or inflammatory disorder rather than a primary bone lesion.
Local Swelling
Visible or palpable swelling is uncommon.
The tumor usually remains contained within the bone unless it becomes unusually large or extends beyond the cortex.
Physical Examination
Examination may demonstrate a joint effusion and reduced range of motion.
A palpable soft-tissue mass is unusual.
Direct joint-line tenderness is also generally not a prominent feature.
Laboratory Tests
Routine laboratory investigations are usually normal.
Blood tests generally do not assist in establishing the diagnosis.
The erythrocyte sedimentation rate is typically normal, helping distinguish the lesion from some infectious or inflammatory conditions.
Imaging
Plain Radiographs
The classic radiographic appearance is a well-defined lytic lesion within the epiphysis.
The lesion usually has a thin surrounding rim of sclerosis.
Sclerotic Rim
The presence of a narrow sclerotic border is consistent with the relatively slow-growing and benign nature of the tumor.
The lesion may nevertheless enlarge sufficiently to expand or deform the surrounding bone.
Calcification
Small punctate calcifications may occasionally be visible within the lesion.
These reflect mineralization of the cartilaginous matrix.
Role of Radiographs
In a typical young patient with an epiphyseal lesion and characteristic symptoms, the combination of clinical history and plain radiographs may strongly suggest the diagnosis.
Definitive diagnosis, however, depends on histologic evaluation.
MRI
MRI is useful when plain radiographs are not definitive or when the full extent of the lesion must be determined.
The lesion generally has a well-demarcated margin on MRI.
Peritumoral Edema
Surrounding bone marrow and soft-tissue edema are commonly seen on MRI.
This edema can sometimes appear disproportionately extensive compared with the relatively small size of the benign tumor.
Pathological Findings
Histologic confirmation requires identification of characteristic chondroblasts.
These are small round or polygonal cells containing round or oval nuclei.
Chondroblast Appearance
Chondroblasts are often described as relatively plump cells, sometimes likened to the appearance of fried eggs on microscopy.
Chicken-Wire Calcification
One of the classic histologic features is fine calcification extending in a lattice-like pattern around individual chondroblasts.
This is referred to as “chicken-wire” calcification and is strongly associated with chondroblastoma.
Giant Cells
Multinucleated giant cells are commonly scattered throughout the tumor.
Their presence can create histologic resemblance to a giant cell tumor, particularly if the epiphyseal location is not considered.
Aneurysmal Bone Cyst Change
Secondary areas of aneurysmal bone cyst formation may also be present.
These regions contain blood-filled spaces and may contribute to expansion of the lesion.
Differential Diagnosis
Important differential diagnoses include enchondroma, giant cell tumor, osteomyelitis, and fibrous dysplasia.
Age, skeletal maturity, epiphyseal location, radiographic appearance, and histology help distinguish these conditions.
Giant Cell Tumor
Giant cell tumor is an especially important differential diagnosis because both lesions can involve the epiphysis and contain numerous giant cells.
Giant cell tumor typically occurs in skeletally mature patients, whereas chondroblastoma more commonly arises before or around skeletal maturity.
Osteomyelitis
Subacute osteomyelitis can occasionally mimic a well-defined lytic lesion.
Clinical evidence of infection, inflammatory markers, MRI findings, and tissue sampling may help differentiate infection from tumor.
Treatment
General Measures
Operative treatment is generally recommended because continued tumor growth can progressively damage the epiphysis and adjacent articular surface.
The aim is complete local removal while preserving the nearby joint whenever possible.
Surgical Challenges
Treatment may be technically difficult because chondroblastomas often lie immediately beneath the articular surface.
The surgeon must remove the tumor while minimizing damage to the joint cartilage, physis, and surrounding subchondral bone.
Bone Grafting
After removal of the lesion, the residual bone defect is commonly filled with bone graft or another suitable bone-defect substitute.
This provides structural support and promotes healing.
Activity
Pathologic fracture is not usually a major concern with typical chondroblastoma.
Therefore, strict activity restrictions are generally unnecessary unless symptoms, lesion size, postoperative status, or individual anatomy warrant protection.
Physical Therapy
Physical therapy may be useful after surgery to restore joint range of motion, strength, and function.
This is particularly important when preoperative stiffness or postoperative immobilization has limited movement.
Surgery
Because chondroblastoma is a benign tumor, local surgical treatment is usually adequate.
The standard procedure consists of thorough intralesional curettage followed by filling of the resulting defect.
Curettage
The tumor is removed by carefully curetting the lesion from the surrounding bone.
Meticulous removal is important because residual tumor increases the likelihood of recurrence.
Joint Preservation
When the lesion lies directly beneath the articular surface, surgery should preserve as much subchondral bone and cartilage as possible.
Damage to the joint surface may contribute to later stiffness or degenerative change.
Follow-Up
Long-term surveillance is important because recurrence is not uncommon.
Clinical evaluation should assess for recurrent pain, loss of motion, or new joint symptoms.
Prognosis
Overall prognosis is favorable because chondroblastoma is benign.
However, local recurrence remains an important concern.
Recurrence
The recurrence rate for conventional chondroblastoma has been reported at approximately 20% within 3 years.
Recurrence is more likely when the lesion contains substantial secondary aneurysmal bone cyst change.
Complications
The principal complications are local recurrence and joint stiffness.
Joint stiffness may result from the tumor itself, prolonged symptoms, surgical exposure, or postoperative scar formation.
Patient Monitoring
Because recurrence most often develops during the first several years after treatment, serial imaging is recommended.
Radiographs are commonly repeated approximately every 6–12 months for the first 2 years after excision.
Persistent or recurrent pain should prompt earlier reassessment and additional imaging.
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Orthopaedic Surgery - Charcot–Marie–Tooth Disease (Hereditary Sensorimotor Neuropathy)
Basics
Charcot–Marie–Tooth disease (CMT) is the most common inherited motor and sensory peripheral neuropathy.
It represents the final common clinical manifestation of numerous genetic abnormalities, involving more than 80 recognized genes, that impair normal peripheral nerve signaling.
The disorder typically progresses in a distal-to-proximal pattern. Distal lower-extremity muscle wasting and weakness develop first, often resulting in cavovarus foot deformity. In some patients, weakness later involves the upper extremities.
Classification
Several major clinical and genetic forms of CMT are recognized.
The five commonly described groups are Type I, Type II, Type III, Type IV, and X-linked CMT.
Type I CMT
Type I is the hypertrophic demyelinating form and accounts for approximately half of affected patients.
The peripheral nerves become thickened because of abnormal myelin formation and repeated demyelination.
Loss of normal myelin causes marked slowing of peripheral nerve conduction.
Type II CMT
Type II is predominantly an axonal form of the disease.
Axonal degeneration produces weakness and sensory abnormalities, while the myelin sheath is relatively preserved.
Nerve conduction velocities are therefore usually only mildly reduced compared with the marked slowing seen in demyelinating disease.
Reflexes may remain relatively preserved.
Type III CMT
Type III, traditionally called Dejerine–Sottas disease, is characterized by marked segmental demyelination.
It is generally a more severe neuropathy and may present earlier than the common forms of CMT.
Type IV CMT
Type IV consists of forms inherited in an autosomal recessive pattern.
The severity and clinical manifestations vary according to the specific genetic abnormality.
X-Linked CMT
CMT-X is inherited through the X chromosome and accounts for approximately 10% of cases.
The severity of disease may differ according to the specific mutation and sex of the affected patient.
Genetics
CMT is genetically heterogeneous.
A particularly important abnormality in common demyelinating forms involves the gene encoding peripheral myelin protein 22-kDa (PMP22).
Other CMT subtypes result from mutations affecting peripheral nerve myelin, axonal structure, intracellular transport, or other components of nerve function.
Inheritance Patterns
Autosomal dominant inheritance with variable penetrance occurs in many forms of Types I, II, and III.
Type IV follows an autosomal recessive pattern.
CMT-X is inherited through the X chromosome.
A detailed family history is therefore an important part of the diagnostic evaluation.
Pathophysiology
Muscle weakness generally progresses from the distal extremities proximally.
In the lower leg, the tibialis anterior and peroneus brevis are commonly affected early.
Selective weakness of these muscles produces characteristic imbalance around the foot and ankle and contributes to development of cavovarus deformity.
Development of Cavus
Weakness of the tibialis anterior allows the peroneus longus to act relatively unopposed.
The peroneus longus plantarflexes the first ray, producing a plantarflexed first metatarsal and contributing to elevation of the medial longitudinal arch.
Development of Hindfoot Varus
Weakness of the peroneus brevis allows the posterior tibialis to dominate.
The resulting inversion force drives the hindfoot into varus.
Toe Clawing
Weakness of the intrinsic muscles and lumbricals allows the long flexor and extensor tendons to overpower the intrinsic musculature.
This imbalance leads to the characteristic clawing of the toes.
Dynamic Hindfoot Inversion
Plantarflexion of the first metatarsal can dynamically force the hindfoot into inversion during weight-bearing.
This mechanism is responsible for a forefoot-driven cavovarus deformity in many patients.
Progressive Rigidity
As the deformity persists, soft-tissue contracture and bony remodeling progressively reduce foot flexibility.
The foot becomes increasingly rigid, with impaired shock absorption and abnormal loading through the hindfoot and midfoot.
Arthritis
Longstanding malalignment and abnormal joint loading may eventually produce degenerative arthritis.
The subtalar, midfoot, and ankle joints may become painful and stiff.
Ankle Instability
Persistent hindfoot varus repeatedly stresses the lateral ankle ligaments.
Over time, ligament attenuation may lead to recurrent ankle sprains and chronic lateral ankle instability.
Associated Conditions
CMT may be associated with musculoskeletal abnormalities outside the foot.
Important associated conditions include scoliosis and developmental dysplasia of the hip.
Scoliosis
Approximately 30% of patients may develop scoliosis.
A proportion of these patients have left thoracic curves, and significant thoracic kyphosis may also be present.
Hip Dysplasia
Developmental dysplasia of the hip occurs in approximately 6–8% of patients.
Hip motion, particularly abduction, should therefore be examined routinely.
Diagnosis
Signs and Symptoms
Patients commonly become symptomatic between approximately 10 and 20 years of age.
Typical presentations include a high medial arch, reduced endurance, decreased coordination, recurrent ankle instability, or a characteristic steppage gait.
Footwear Problems
Progressive cavovarus alignment produces uneven plantar loading.
Shoes may wear out rapidly and asymmetrically, especially along the lateral border.
Muscle Weakness Pattern
Weakness generally appears first in the ankle evertors and dorsiflexors.
The plantarflexors and invertors are typically affected later.
This imbalance contributes substantially to cavus and hindfoot varus.
Sensory Changes
Sensation and proprioception may be diminished.
Reduced proprioceptive input can worsen balance, gait control, and ankle instability.
Steppage Gait
Foot-drop weakness can produce a steppage gait.
The patient excessively flexes the hip and knee during the swing phase so that the toes clear the floor.
Circumduction Gait
Some patients compensate by swinging the affected limb outward in a circumduction pattern during the swing phase.
History
A detailed family history should identify relatives with high arches, claw toes, similar gait abnormalities, distal weakness, or a known diagnosis of CMT.
The rate of progression of weakness and deformity should also be documented.
Ankle Instability History
Patients should be questioned about repeated ankle sprains, giving-way episodes, or feelings of instability.
These symptoms may indicate chronic lateral ligament insufficiency caused by cavovarus alignment.
Pain History
Pain should be localized carefully.
Common symptomatic areas include the lateral ankle, plantar forefoot, midfoot, and sites of callus formation.
Physical Examination
The examination should assess muscle bulk, strength, sensation, foot alignment, deformity flexibility, gait, hip motion, spinal alignment, and upper-extremity involvement.
Calf Atrophy
Distal lower-extremity muscle wasting commonly produces visible calf atrophy.
Muscle Strength
The strength of all major foot and ankle muscle groups should be recorded and followed over time.
Particular attention should be given to dorsiflexion, eversion, inversion, and plantarflexion.
Sensory Examination
Sensation should be assessed, including light touch, proprioception, and protective sensation.
Reduced protective sensation increases the risk of pressure injury from abnormal foot loading.
Hindfoot Varus
The patient should be examined standing and at rest for the presence of hindfoot varus.
The severity of hindfoot varus is closely related to functional impairment, ankle instability, and lateral overload.
Coleman Block Test
The Coleman block test helps distinguish flexible from fixed hindfoot varus.
The patient stands with the heel and lateral border of the foot supported while the first ray hangs freely beyond the edge of the block.
If the heel corrects toward neutral, the hindfoot remains flexible and the varus is largely driven by the plantarflexed first ray.
Passive Hindfoot Correction
Manual correction of hindfoot varus should also be assessed.
A rigid deformity is more likely to require bony correction rather than soft-tissue procedures alone.
Lateral Ankle Ligaments
The lateral ankle ligaments should be examined for laxity and mechanical instability.
Chronic varus positioning can progressively stretch these structures.
First Metatarsal Position
The relative position of the first metatarsal should be compared with the lesser metatarsals.
A plantarflexed first ray is characteristic of CMT-associated cavus.
Toe Clawing
The presence and severity of clawing should be documented.
The examiner should determine whether the toe deformities are flexible or fixed.
Gait Examination
Gait should be observed for foot drop, steppage, circumduction, ankle instability, and abnormal push-off.
Functional gait findings help determine the clinical severity of disease.
Hip Examination
Hip abduction should be measured.
Restricted abduction may suggest associated hip dysplasia and should prompt further imaging.
Spine Examination
The spine should be evaluated for scoliosis using a forward-bend test and observation of shoulder and trunk symmetry.
Upper-Extremity Examination
The hands should be inspected for wasting of the ulnar-innervated intrinsic muscles, including the interossei and abductors.
Upper-extremity weakness tends to develop later than lower-extremity involvement.
Electrodiagnostic Studies
Electromyography and nerve conduction studies are commonly used to confirm the diagnosis and characterize the neuropathy.
They help distinguish predominantly demyelinating disease from axonal forms.
Electromyography
EMG may demonstrate increased motor unit duration and reduced amplitude, reflecting chronic denervation and reinnervation.
Nerve Conduction Studies
Motor and sensory nerve conduction velocities are reduced to varying degrees.
Marked slowing is especially characteristic of demyelinating forms such as CMT Type I.
Hand Function Testing
Grip strength and manual dexterity may be monitored over time.
The 9-hole peg test can be used to quantify changes in fine motor function.
Biopsy
Muscle and nerve biopsy are rarely required when the history, examination, electrodiagnostic findings, and genetic testing are characteristic.
Muscle Biopsy Findings
Muscle biopsy may demonstrate diffuse atrophy with replacement of normal muscle fibers by fibrous and adipose tissue.
Nerve Biopsy Findings
Nerve biopsy may show loss of myelinated fibers and increased fibrous tissue within the endoneurium and perineurium.
Because genetic and electrodiagnostic testing are less invasive, biopsy is generally unnecessary.
Genetic Testing
DNA testing can be performed from peripheral blood.
It may confirm the diagnosis, identify a specific subtype, allow testing of family members, and assist with genetic counseling.
Imaging
Standing Foot and Ankle Radiographs
Weight-bearing radiographs should be obtained to evaluate cavus alignment, hindfoot varus, first-ray plantarflexion, joint congruity, and degenerative arthritis.
They are particularly important for surgical planning.
Spine Radiographs
In an index patient without a known family history, spinal radiographs may be obtained to exclude other structural causes of cavus deformity.
They are also appropriate when scoliosis is identified clinically.
Pelvic Radiographs
Pelvic radiographs should be considered when hip abduction is limited or dysplasia is suspected.
Early identification of hip dysplasia allows more effective treatment.
MRI of the Spine
Spinal MRI may be appropriate when the diagnosis is uncertain or there is concern for spinal pathology.
It can demonstrate abnormalities of the spinal cord and help exclude other neurologic causes of cavus deformity.
Differential Diagnosis
Important differential diagnoses include tethered spinal cord, myelomeningocele, lipomeningocele, peroneal nerve palsy, early Duchenne muscular dystrophy, and other hereditary motor and sensory neuropathies.
An atypical or unilateral deformity should prompt particularly careful investigation for a focal neurologic cause.
Treatment
General Principles
Treatment aims to maintain mobility, preserve flexibility, improve alignment, reduce pain, support weak muscles, and prevent progression of secondary deformity.
Orthopaedic treatment cannot reverse the underlying genetic neuropathy.
Routine Surveillance
Patients should be followed regularly for progression of muscle weakness, foot deformity, ankle instability, sensory loss, and functional limitation.
Earlier treatment may prevent a flexible deformity from becoming rigid.
Stretching
Regular stretching of the Achilles tendon and plantar fascia may help preserve foot and ankle flexibility.
Stretching is most useful before fixed contracture has developed.
Orthotic Insoles
Custom orthoses can redistribute plantar pressure and improve alignment.
A lateral heel build-up or wedge may help reduce flexible hindfoot varus.
Forefoot Padding
Accommodative padding beneath the forefoot may help relieve metatarsalgia and pressure associated with claw toes.
Ankle Bracing
An ankle brace may provide support in patients with symptomatic instability or recurrent ankle sprains.
Ankle-Foot Orthosis
A custom ankle-foot orthosis (AFO) may be necessary for substantial foot drop caused by dorsiflexor weakness.
It improves toe clearance during swing and may reduce falls.
Footwear
Comfortable footwear with a wide toe box, adequate depth, and cushioned heel is recommended.
Shoes should accommodate claw toes, high arches, and pressure-sensitive areas.
Physical Therapy
Physical therapy may include Achilles and plantar fascia stretching, strengthening exercises, gait work, proprioceptive training, and balance exercises.
These measures can improve function and reduce instability but do not reverse the neuropathy itself.
Surgery
General Surgical Principles
Surgical treatment should be individualized according to the flexibility and location of the deformity.
As a general principle, flexible deformities are managed with soft-tissue releases, rigid deformities require osteotomies, and tendon transfers are used to rebalance abnormal muscle forces.
The goal is to obtain a stable, plantigrade, pain-free, and braceable foot.
Claw-Toe Correction
Claw toes may be treated with flexor-to-extensor tendon transfer, MTP joint release, or PIP joint fusion, depending on whether the deformity remains flexible.
Jones Procedure
Severe hallux clawing may be treated with hallux interphalangeal arthrodesis combined with transfer of the extensor hallucis longus tendon to the first metatarsal.
This combination is commonly known as a Jones procedure.
Plantar Release
Soft-tissue release may involve the plantar fascia, abductor hallucis, and toe flexors.
This helps reduce the cavus deformity and increase flexibility.
Plantar-Medial Release
More extensive deformity may require a plantar-medial release involving structures such as the posterior tibialis, long toe flexors, and talonavicular capsule.
Posterior Tibialis Transfer
The posterior tibialis tendon may be transferred partially or completely toward the anterior aspect of the foot.
This can improve dorsiflexion and eversion while reducing the deforming inversion force.
Peroneus Longus to Brevis Transfer
Transfer or tenodesis of the peroneus longus to the peroneus brevis reduces plantarflexion of the first ray and increases eversion strength.
This is particularly useful in flexible forefoot-driven cavovarus deformity.
Calcaneal Osteotomy
A lateral closing-wedge calcaneal osteotomy may be required when hindfoot varus is rigid.
The procedure repositions the heel toward neutral alignment.
Midfoot and Metatarsal Osteotomies
Rigid cavus involving the forefoot or midfoot may require midfoot or metatarsal closing-wedge osteotomies.
These procedures reduce the high arch and improve plantar pressure distribution.
Triple Arthrodesis
Triple arthrodesis is generally reserved for severe rigid deformity or painful hindfoot arthritis.
Fusion sacrifices motion but can provide a stable and better-aligned foot.
Ankle Ligament Reconstruction
Chronic lateral ankle instability may require ligament reconstruction.
The underlying cavovarus deformity should also be corrected because isolated ligament repair may fail if varus alignment persists.
Follow-Up
Specialist Referral
Neurologic consultation is appropriate for electrodiagnostic evaluation, genetic testing, and counseling.
Management often benefits from collaboration among neurology, orthopaedics, physical therapy, orthotics, and genetics specialists.
Prognosis
Even after appropriate surgery, the foot generally cannot be restored completely to normal because the underlying muscle weakness continues.
Progressive neuropathy may lead to further weakness or recurrence of deformity.
Life Expectancy
Typical Charcot–Marie–Tooth disease does not shorten life expectancy.
Most morbidity relates to progressive weakness, deformity, gait difficulty, and loss of distal function.
Recurrence
Deformity can recur after surgery, especially when soft-tissue procedures alone are used in a foot that already has fixed bony malalignment.
Successful reconstruction requires correction of both structural deformity and muscle imbalance.
Adjacent-Joint Degeneration
Fusion procedures, particularly triple arthrodesis, may transfer mechanical stress to neighboring joints.
This can contribute to later degeneration of the ankle or midfoot.
Hip Dysplasia
Failure to recognize associated hip dysplasia may make later treatment more difficult and less successful.
Routine hip examination is therefore important throughout growth.
Patient Monitoring
Patients should generally be reviewed yearly for ambulatory function, muscle strength, foot alignment, ankle stability, sensory changes, hand function, and spinal deformity.
Earlier reassessment is appropriate if there is rapid deterioration, new weakness, recurrent falls, worsening pain, or progression of deformity.
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Orthopaedic Surgery - Cervical Spine Anatomy and Examination
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Basics
The cervical spine consists of seven cervical vertebrae, C1 through C7, which support the head, protect the spinal cord, permit a wide range of motion, and provide passage for important neurovascular structures.
For practical purposes, the cervical spine can be divided into the upper cervical spine, consisting of C1 and C2, and the lower cervical spine, consisting of C3 through C7.
The upper and lower regions differ substantially in both morphology and function.
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Osteology
Typical cervical vertebrae contain a vertebral body, paired pedicles, paired laminae, transverse processes, articular structures, and a spinous process.
The bony architecture surrounds and protects the cervical spinal cord while contributing to stability and motion.
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Transverse Foramina
All cervical vertebrae contain transverse foramina within their transverse processes.
The vertebral arteries usually pass through the transverse foramina from C6 to C1, rather than through C7.
Protection of these vessels is one of the important functions of intact cervical vertebral anatomy.
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Upper Cervical Spine
The atlas (C1) and axis (C2) are considered atypical cervical vertebrae because their anatomy differs considerably from that of C3–C7.
Their specialized architecture allows extensive movement between the skull and upper cervical spine.
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Atlas
The atlas has no true vertebral body and no conventional spinous process.
It consists largely of anterior and posterior arches connected by lateral masses.
C1 articulates superiorly with the occipital condyles and inferiorly with the axis.
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Atlantoaxial Joint
The articulation between C1 and C2 forms the atlantoaxial joint.
Approximately 50% of cervical rotation occurs at this level.
The unique articulation between the atlas and odontoid process permits substantial axial rotation of the head.
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Axis
The axis (C2) contains a vertebral body and the characteristic odontoid process, or dens, which projects superiorly and acts as a pivot for rotation of C1.
The dens is clinically important because odontoid fractures are common upper cervical injuries.
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Odontoid Blood Supply
The odontoid process contains a relative vascular watershed region between its apex and base.
This limited blood supply can influence healing of certain odontoid fractures and contributes to the risk of nonunion in selected fracture patterns.
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Atlanto-Occipital Joint
A substantial proportion of upper cervical flexion and extension occurs at the atlanto-occipital articulation between the occiput and C1.
This joint is particularly important for nodding movements of the head.
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Cervical Lordosis
The normal sagittal alignment of the cervical spine is lordotic.
Loss or reversal of cervical lordosis may be associated with muscle spasm, degeneration, deformity, or traumatic injury, although alignment must always be interpreted in clinical context.
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Protective Function
Normal cervical vertebral anatomy protects both the spinal cord and vertebral arteries.
Trauma, instability, stenosis, or deformity can therefore have serious neurologic or vascular consequences.
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Neuroanatomy
There are eight cervical nerve roots despite only seven cervical vertebrae.
The cervical nerve roots generally exit above their correspondingly numbered vertebrae.
For example, the C5 nerve root exits between C4 and C5.
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C8 Nerve Root
The C8 nerve root exits between C7 and T1.
Below this level, thoracic and lumbar nerve roots exit below the correspondingly numbered vertebra.
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Orientation of Cervical Nerve Roots
Cervical nerve roots travel in a relatively horizontal direction as they leave the spinal canal.
Because of this arrangement, both central/paracentral and foraminal disc pathology may affect the same exiting cervical nerve root depending on the level and location of compression.
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Cervical Spine Examination
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Inspection
The examination should begin with visual inspection.
Adequate exposure is important so that the neck, shoulders, upper thorax, and surrounding soft tissues can be assessed systematically.
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Alignment
The examiner should assess gross cervical alignment and head position.
Abnormal posture, torticollis, deformity, or asymmetry may indicate muscle spasm, structural deformity, or neurologic disease.
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Skin and Soft Tissues
The skin should be inspected for wounds, scars, bruising, swelling, masses, or other soft-tissue abnormalities.
Muscle asymmetry, atrophy, or hypertrophy should also be noted.
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Anterior Bony Palpation
Palpation of the anterior neck should identify tenderness, crepitus, masses, asymmetry, or malalignment.
Several palpable landmarks can help estimate cervical spinal levels.
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Hyoid Bone
The hyoid bone approximately corresponds to the level of the C3 vertebral body.
It can serve as a useful surface landmark during examination.
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Thyroid Cartilage
The superior portion of the thyroid cartilage approximately corresponds to the C4 vertebral body.
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Cricoid Cartilage
The cricoid cartilage is located approximately at the C6 vertebral level.
It may be easier to palpate during swallowing because movement of the laryngeal structures makes the anatomy more distinct.
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Carotid Tubercle
The anterior tubercle of the C6 transverse process is known as the carotid tubercle.
The right and left carotid tubercles should not be compressed simultaneously because doing so could reduce blood flow through both carotid arteries.
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Tracheal Examination
The trachea should be palpated to confirm that it remains in the midline.
Deviation may indicate a mass, swelling, mediastinal process, or other abnormality.
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Posterior Bony Palpation
Posterior examination begins at the occiput and proceeds inferiorly along the cervical spinous processes.
Tenderness, step-off, displacement, or asymmetry should be noted.
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Inion
The inion is the most prominent palpable point of the lower occiput.
It serves as an important posterior surface landmark.
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Spinous Processes
The spinous processes should generally form a straight midline sequence.
C7 and T1 are usually the most prominent and easiest to palpate.
Deviation from normal alignment can suggest rotational injury, including unilateral facet dislocation.
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Bifid Spinous Processes
The spinous processes of approximately C3–C5 may be bifid.
This is a normal anatomical variation and should not be mistaken for pathology.
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Facet Joints
The cervical facet joints lie approximately 2.5 cm lateral to the spinous processes.
Degenerative disease frequently affects the lower cervical facets, particularly around C5–C6.
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Anterior Soft-Tissue Palpation
Anterior soft-tissue examination should include the sternocleidomastoid muscles, parotid glands, cervical lymph nodes, thyroid gland, carotid pulses, and supraclavicular fossae.
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Thyroid Examination
The thyroid gland should normally feel relatively smooth and symmetric.
Enlargement, nodularity, or asymmetry should be documented.
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Supraclavicular Fossa
The supraclavicular area should be palpated for abnormal masses or bony prominences.
A prominent structure may represent a cervical rib.
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Posterior Soft-Tissue Palpation
Posterior soft-tissue examination includes the trapezius muscles, greater occipital nerves, and ligamentum nuchae.
The trapezius should be assessed for tenderness, muscle asymmetry, or abnormal masses.
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Lymph Nodes
Palpable lymph nodes in the posterior neck should be considered abnormal if enlarged or otherwise clinically suspicious.
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Greater Occipital Nerves
The greater occipital nerves can become tender or prominent after trauma, including whiplash-type injuries.
Irritation may contribute to occipital pain or headache.
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Ligamentum Nuchae
The ligamentum nuchae extends from the inion to the C7 spinous process.
It can be palpated in the posterior midline.
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Neurologic Examination
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General Principles
Neurologic evaluation of the cervical spine includes sensory, motor, and reflex testing.
Findings should be carefully documented to identify nerve-root compression, spinal cord dysfunction, or peripheral neuropathy.
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Sensory Examination
Patients should be asked to identify altered sensation as specifically as possible.
The most commonly assessed sensory modalities are light touch and pinprick.
Comparison between sides is useful.
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Upper Cervical Dermatomes
Sensation from C2 through C4 generally progresses from the posterior scalp and neck toward the anterior neck and shoulder region.
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C5 Dermatome
The C5 dermatome is represented mainly over the lateral shoulder and upper arm.
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C6 Dermatome
The C6 dermatome extends along the lateral forearm toward the radial side of the hand, particularly the thumb and adjacent radial digits.
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C7 Dermatome
The C7 dermatome is commonly tested at the middle finger.
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C8 Dermatome
The C8 dermatome involves the ulnar side of the hand, particularly the ring and little fingers.
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T1 Dermatome
The T1 dermatome lies primarily along the medial forearm.
Dermatomal patterns overlap, so sensory findings should always be interpreted together with motor and reflex abnormalities.
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Motor Examination
Motor testing should evaluate major muscle groups corresponding to cervical nerve roots.
Strength should be graded consistently to allow changes over time to be recognized.
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Muscle Strength Grading
Grade 0: No visible or palpable muscle contraction.
Grade I: Muscle contraction is visible or palpable, but no joint movement occurs.
Grade II: Full range of motion is possible with gravity eliminated.
Grade III: Full range of motion is possible against gravity.
Grade IV: Full range of motion is possible against gravity and some resistance.
Grade V: Normal strength against full resistance.
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C3–C5 Motor Function
The levator scapulae can be assessed with resisted scapular elevation and receives contributions predominantly from C3 and C4, with possible C5 involvement.
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C5 Motor Function
The deltoid is tested with shoulder abduction and primarily reflects C5 motor function.
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C6 Motor Function
C6 function can be evaluated with elbow flexion through the biceps and wrist extension.
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C7 Motor Function
C7 is assessed through triceps-mediated elbow extension, wrist flexion, and finger extension.
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C8 Motor Function
C8 contributes substantially to finger flexion and thumb adduction.
Testing grip and intrinsic hand function can therefore help assess lower cervical nerve-root function.
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Deep Tendon Reflexes
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Reflex Examination
Deep tendon reflex testing helps distinguish nerve-root compression from spinal cord disease.
Diminished reflexes may indicate radiculopathy, whereas hyperreflexia can suggest an upper motor neuron lesion such as cervical myelopathy.
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Biceps Reflex
The biceps reflex primarily evaluates the C5 nerve root, with some contribution from C6.
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Brachioradialis Reflex
The brachioradialis reflex primarily evaluates C6.
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Triceps Reflex
The triceps reflex primarily evaluates C7.
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Range of Motion
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General Principles
Active cervical range of motion should be assessed in flexion, extension, rotation, and lateral bending.
Pain, limitation, asymmetry, and reproduction of radicular symptoms should be noted.
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Flexion and Extension
A large proportion of flexion-extension occurs in the upper cervical spine, while the remainder is distributed across the subaxial cervical levels.
The C5–C6 segment often demonstrates particularly substantial motion.
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Muscles Used in Flexion and Extension
Cervical flexion assesses muscles including the sternocleidomastoid and deeper cervical flexors.
Extension involves the paraspinal extensors and trapezius.
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Rotation
Approximately half of cervical rotation occurs at C1–C2, with the remainder distributed through the lower cervical spine.
The patient should normally be able to rotate the chin approximately 60–80° to either side.
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Rotational Muscles
The sternocleidomastoid is an important cervical rotator, although normal rotation is produced by coordinated activity of several muscle groups.
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Lateral Bending
Lateral bending is distributed throughout the cervical spine and usually occurs in combination with some rotation.
The patient is asked to bring the ear toward the ipsilateral shoulder without elevating the shoulder.
Normal lateral bending is approximately 45°.
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Muscles Used in Lateral Bending
The scalene muscles contribute substantially to cervical lateral flexion.
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Provocative Tests
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Modified Spurling Maneuver
The modified Spurling test evaluates cervical nerve-root irritation.
The neck is extended and rotated toward the symptomatic side, followed by gentle axial loading.
A positive test reproduces radicular pain or paresthesias in the ipsilateral upper extremity.
The test is relatively specific for cervical radiculopathy but has limited sensitivity.
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Shoulder Abduction Test
The patient actively or passively raises the symptomatic arm over the head.
Relief of radicular symptoms with ipsilateral shoulder abduction constitutes a positive test and may suggest cervical nerve-root compression.
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Cervical Distraction Test
The examiner applies gentle longitudinal traction to the head, typically with the neck in slight flexion or neutral alignment.
Improvement in radicular symptoms during distraction supports the possibility of cervical nerve-root compression.
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Lhermitte Sign
The Lhermitte maneuver is performed by flexing the cervical spine anteriorly.
An electric or shooting sensation traveling down the arms, trunk, or legs suggests irritation or dysfunction of the cervical spinal cord.
It may occur with cervical cord compression as well as other disorders affecting the dorsal columns.
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Hoffmann Sign
To test the Hoffmann reflex, the patient’s hand is supported and relaxed.
The examiner grasps the middle finger and sharply flicks the distal phalanx or nail.
A positive response consists of reflex flexion or adduction of the thumb and index finger.
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Significance of Hoffmann Sign
A positive Hoffmann sign can suggest upper motor neuron or corticospinal tract dysfunction, including cervical myelopathy.
It should not be interpreted in isolation and must be correlated with the remainder of the neurologic examination.
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Romberg Test
The Romberg test evaluates balance and proprioception.
The patient stands with the arms extended and eyes initially open, then closed.
Loss of balance when the eyes are closed suggests impaired proprioceptive function.
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Dynamic Balance Assessment
Observation of abnormal arm drift, progressive elevation of the arms, or instability during static or dynamic balance testing may provide additional evidence of neurologic dysfunction.
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Gait Assessment
Gait examination is an essential part of cervical neurologic evaluation.
It can provide information regarding balance, coordination, lower-extremity function, posture, spasticity, and possible spinal cord dysfunction.
Patients with cervical myelopathy may exhibit a stiff, broad-based, unsteady, or spastic gait.
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Imaging
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Plain Radiographs
Standard cervical spine radiography commonly includes anteroposterior and lateral views.
When possible, imaging is obtained with the patient upright so that physiologic alignment can be assessed.
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Trauma Radiographs
In trauma patients, an initial cross-table lateral radiograph may be used when appropriate, but the entire cervical spine must be adequately visualized for the image to be diagnostically useful.
Modern trauma evaluation frequently relies heavily on CT when significant cervical injury is suspected.
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Oblique Views
Oblique radiographs can help assess the neural foramina, facet alignment, subluxation, and facet dislocation.
They are particularly helpful in selected cases when foraminal anatomy requires further evaluation.
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Open-Mouth Odontoid View
The open-mouth view is used to assess the odontoid process, atlantoaxial joints, and alignment of the lateral masses of C1 relative to C2.
Asymmetry may indicate fracture, displacement, or rotational injury.
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Pediatric Radiographs
In young children, normal ossification centers and developmental variants may resemble fractures.
Knowledge of age-related cervical spine anatomy is therefore essential to avoid misinterpreting normal findings as traumatic injury.
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MRI
MRI is the preferred modality for evaluating soft-tissue structures of the cervical spine.
It provides detailed visualization of the intervertebral discs, spinal cord, nerve roots, ligaments, joint capsules, and other neural and soft-tissue structures.
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Clinical Role of MRI
MRI findings should be correlated with the patient’s symptoms and physical examination because asymptomatic degenerative abnormalities are common.
It is particularly valuable when evaluating disc herniation, spinal stenosis, ligamentous injury, spinal cord compression, and myelopathy.
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CT
CT provides excellent definition of cervical osseous anatomy.
It can rapidly and accurately identify fractures, facet injuries, displacement, and complex bony abnormalities.
For suspected cervical spine trauma, CT is particularly useful for fracture characterization and surgical planning.
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Integration of Examination and Imaging
Cervical spine assessment requires correlation of history, physical examination, neurologic findings, provocative testing, and imaging.
No single examination maneuver or imaging abnormality should be interpreted in isolation.
A systematic approach is particularly important when distinguishing cervical radiculopathy, myelopathy, structural injury, and peripheral nerve disease.
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Orthopaedic Surgery - Cervical Disc Herniation
Basics
Cervical disc herniation occurs when intervertebral disc material displaces posteriorly into the spinal canal or neural foramen, resulting in compression of a cervical nerve root, the spinal cord, or both.
Mechanical compression is accompanied by an inflammatory response and may produce neck pain, cervical radiculopathy, cervical myelopathy, or a combination of these findings.
Classification
Cervical disc herniations may be classified according to clinical onset, anatomic location, or the morphology of the displaced disc material.
Clinically, the condition may present acutely or develop gradually.
Anatomically, the herniation may be central, paracentral, foraminal, or lateral depending on its location within the spinal canal.
Classification by Disc Morphology
Disc displacement can also be described as a bulge, protrusion, extrusion, or sequestration.
A protrusion remains broadly connected with the parent disc, whereas an extrusion represents greater displacement of disc material through the annulus.
A sequestrated fragment has completely separated from the parent disc.
Classification of Myelopathy
Several classification systems are available for cervical myelopathy.
These systems generally assess factors such as gait, pain, upper- and lower-extremity weakness, upper and lower motor neuron findings, bladder function, and fine motor ability.
The purpose is to quantify severity and monitor neurologic progression or response to treatment.
Epidemiology
Cervical disc herniation occurs most commonly in adults older than 30 years, with an average age near 50 years.
Cervical radiculopathy is considerably more common than cervical myelopathy, and radiculopathy only rarely progresses to spinal cord dysfunction.
Incidence
The reported annual age-adjusted incidence of cervical radiculopathy is approximately 107.3 per 100,000 men and 64.5 per 100,000 women.
The incidence is particularly high in adults aged 50–54 years, reaching approximately 203 per 100,000 people per year.
Prevalence of Neck Pain
Neck pain is extremely common.
As many as two-thirds of adults experience at least one significant episode of neck pain during their lifetime.
Not all neck pain, however, is caused by cervical disc herniation.
Risk Factors
Risk factors include repetitive lifting, cigarette smoking, and prolonged or repetitive overhead work.
These activities may increase mechanical stress on the cervical spine or accelerate degenerative disc changes.
Pathophysiology
The mechanical component of cervical nerve compression is well established.
However, symptoms are not produced by compression alone.
Mechanical, ischemic, and inflammatory mechanisms all contribute to nerve-root and spinal-cord dysfunction.
Inflammatory Mediators
Chemical mediators released around the compressed nerve root can contribute to pain and irritation.
These include substance P, interleukin-1, interleukin-6, bradykinin, tumor necrosis factor-alpha, and prostaglandins.
This inflammatory response helps explain why symptoms may be severe even when radiographic compression appears relatively modest.
Etiology
Cervical disc herniations may occur following trauma or may develop without a specific traumatic event as part of degenerative disc disease.
Age-related degeneration weakens the annulus fibrosus and allows nucleus pulposus material to migrate posteriorly.
Associated Conditions
Cervical disc herniation may coexist with congenital cervical spinal stenosis, ossification of the posterior longitudinal ligament, and cervical spondylosis.
These abnormalities reduce the available space for the spinal cord and nerve roots and may increase the clinical significance of even relatively small disc protrusions.
Diagnosis
Signs and Symptoms
Symptoms may begin suddenly or develop gradually.
The clinical spectrum includes neck pain, occipital pain, shoulder-girdle discomfort, arm pain, paresthesias, sensory loss, and weakness.
Symptoms are often aggravated by particular positions or movements of the neck.
Cervical Radiculopathy
Compression of an individual cervical nerve root may produce a characteristic combination of motor weakness, sensory disturbance, pain, and reflex changes.
Although classic dermatomal and myotomal patterns are useful, actual symptoms may overlap and do not always follow a perfectly defined distribution.
Spurling Test
The Spurling maneuver can reproduce radicular symptoms.
The patient’s neck is extended, rotated, and laterally bent toward the symptomatic side, followed by gentle axial compression.
Reproduction of radiating pain or paresthesias into the ipsilateral arm supports cervical nerve-root irritation.
Cervical Myelopathy
Cervical myelopathy usually develops insidiously and may follow a gradual, stepwise pattern of neurologic decline.
Only a small proportion of patients experience rapid deterioration.
Because spinal cord dysfunction can become irreversible, early recognition is important.
Symptoms of Myelopathy
Typical symptoms include progressive gait disturbance, imbalance, falls, deterioration in hand dexterity, generalized weakness, and difficulty with fine motor tasks.
Patients may describe their legs as stiff or “jumpy” and may notice increasing difficulty with buttons, handwriting, or handling small objects.
Advanced Myelopathic Symptoms
More advanced spinal cord dysfunction can produce bowel, bladder, or sexual dysfunction.
These symptoms warrant prompt neurologic and surgical evaluation.
History
Patients should be questioned about the onset, duration, distribution, and progression of pain, paresthesias, numbness, and weakness.
The clinician should also specifically ask about balance problems, falls, gait changes, loss of hand dexterity, and bowel or bladder symptoms.
Sensory Symptoms
Sensory abnormalities do not always follow a precise dermatome.
Overlap between adjacent cervical nerve roots is common, and symptoms may be modified by coexisting peripheral nerve compression.
Physical Examination
The cervical spine should be assessed for range of motion, tenderness, and reproduction of symptoms with movement.
A complete neurologic examination of the upper and lower extremities should include motor testing, sensory testing, reflexes, gait assessment, and examination for long-tract signs.
Motor Examination
Muscle strength should be tested systematically to identify weakness corresponding to a particular cervical nerve root.
Weakness may involve the shoulder, elbow, wrist, or hand depending on the level of compression.
Reflex Examination
Important reflexes include:
C5 – biceps reflex
C6 – brachioradialis reflex
C7 – triceps reflex
Asymmetry or reduction may support a cervical radiculopathy, whereas generalized hyperreflexia can suggest myelopathy.
Babinski Sign
The Babinski response is tested by applying a noxious stimulus along the plantar aspect of the foot.
A positive test consists of extension of the great toe, often accompanied by fanning of the lesser toes.
This suggests an upper motor neuron abnormality and may occur in cervical myelopathy.
Hoffmann Reflex
The Hoffmann reflex is elicited by flicking or pinching the distal phalanx or nail of the middle finger.
A positive response causes reflex flexion or adduction of the thumb and index finger.
When present in the appropriate clinical context, it may indicate cervical spinal cord dysfunction.
Finger Escape Sign
The finger escape sign is associated with cervical myelopathy.
The small finger gradually abducts away from the other digits when the patient attempts to keep the fingers fully extended and adducted.
This reflects weakness or dysfunction of the intrinsic hand muscles.
Electrodiagnostic Testing
Electromyography and nerve conduction studies can provide objective evidence of neurologic dysfunction.
They are particularly useful when there is concern for coexisting peripheral neuropathy, peripheral nerve entrapment, or inconsistency between the history, physical examination, and imaging findings.
Imaging
Plain Radiographs
Conventional cervical spine radiographs can demonstrate alignment, disc-space narrowing, osteophytes, and other degenerative changes.
Oblique views may help visualize the neural foramina.
Flexion-extension radiographs can be used when instability is suspected.
Limitations of Radiographs
Degenerative changes are common in asymptomatic adults, particularly after the age of 40.
Therefore, plain radiographs should be interpreted in conjunction with the clinical picture rather than assumed to identify the source of symptoms.
They are particularly useful after trauma or when symptoms have failed to improve with conservative care.
MRI
MRI is the preferred noninvasive imaging study for most patients who require advanced evaluation.
It provides excellent visualization of intervertebral discs, spinal cord, nerve roots, ligaments, and other soft tissues without ionizing radiation.
Indications for MRI
MRI is particularly useful in patients with persistent radicular symptoms, progressive neurologic deficit, suspected myelopathy, or failure of conservative treatment.
The imaging findings should correlate with the patient’s symptoms and physical examination.
CT Myelography
CT myelography provides detailed assessment of compression from both bone and soft tissue.
It can be useful when MRI cannot be performed or when metallic hardware significantly limits MRI quality.
Because CT myelography is invasive, it is generally reserved for selected situations.
Selective Diagnostic Injections
Selective cervical nerve-root injections may occasionally be used when multiple potential sites of compression are present and the symptomatic level is unclear.
Temporary relief following injection can help identify the primary pain generator.
Pathologic Findings
The fundamental abnormality is herniation of nucleus pulposus material through or beyond the annulus fibrosus.
Compression of a nerve root produces radiculopathy, whereas compression of the spinal cord may cause myelopathy.
Differential Diagnosis
Cervical disc herniation can mimic numerous shoulder, peripheral nerve, neurologic, infectious, or neoplastic disorders.
A careful examination is therefore necessary before attributing upper-extremity symptoms solely to the cervical spine.
Shoulder and Upper-Extremity Disorders
Intrinsic disorders of the shoulder, elbow, or wrist can produce similar symptoms.
These include degenerative arthritis, shoulder impingement, rotator cuff disease, and joint instability.
Peripheral Nerve Entrapment
Peripheral nerve compression should also be considered.
Important examples include carpal tunnel syndrome, cubital tunnel syndrome, Guyon canal syndrome, and thoracic outlet syndrome.
Neurologic Disorders
Neurologic conditions that may mimic cervical radiculopathy or myelopathy include brachial plexopathy, multiple sclerosis, amyotrophic lateral sclerosis, and tumors of the brain or spinal cord.
Infection and Malignancy
Serious alternative diagnoses include infectious discitis, vertebral osteomyelitis, and metastatic malignancy.
Systemic symptoms, severe unremitting pain, fever, weight loss, or a history of cancer should prompt further investigation.
Treatment
General Measures
Most patients with cervical radiculopathy can initially be treated nonoperatively.
Management may include activity modification, analgesic medication, anti-inflammatory therapy, short-term muscle relaxants, selective use of a soft cervical collar, and physical therapy.
Natural History
Cervical radiculopathy is frequently self-limiting.
Approximately 75% of patients improve spontaneously with nonsurgical treatment.
For this reason, conservative management is generally the initial treatment of choice when no progressive neurologic deficit or myelopathy is present.
Activity Modification
Relative rest and avoidance of activities that clearly worsen symptoms can be helpful during the acute phase.
Prolonged inactivity should be avoided because it may contribute to deconditioning and stiffness.
Soft Cervical Collar
A soft cervical collar may provide short-term symptomatic relief in acute cases by limiting painful motion.
Prolonged use is generally discouraged because it can lead to muscle weakness and dependence.
Physical Therapy
Physical therapy can address pain, mobility, strength, and return to function.
Treatment may initially include passive modalities, followed by stretching, postural work, strengthening, and progressive activity.
Cervical Traction
Cervical traction may reduce radicular symptoms in selected patients.
It can be performed under supervision in therapy or with an appropriate home device.
Its effectiveness varies among individuals.
Medication
Long-term maintenance opioid therapy has no established role in routine management of cervical disc herniation.
Medication should be directed toward short-term symptom control while the underlying condition is treated conservatively or surgically as appropriate.
First-Line Medication
Common first-line options include NSAIDs, acetaminophen, and selected anti-inflammatory agents when there are no contraindications.
Gastrointestinal, renal, cardiovascular, and other patient-specific risks should be considered.
Second-Line Treatment
Selected patients may be considered for cervical epidural corticosteroid injection.
Such injections may reduce radicular inflammation and pain, although they do not correct the structural disc herniation itself.
Indications for Surgical Referral
Surgical evaluation should be considered when symptoms fail to improve after approximately 6 weeks of appropriate conservative treatment, especially when pain remains disabling.
Earlier referral is indicated for progressive neurologic deficit, severe motor weakness, or signs of cervical myelopathy.
Surgery
Operative options include anterior cervical discectomy and fusion, posterior cervical foraminotomy, laminoplasty, and cervical disc arthroplasty.
The choice depends on the location of compression, cervical alignment, number of affected levels, patient age, and associated degenerative changes.
Anterior Cervical Discectomy and Fusion
Anterior cervical discectomy and fusion (ACDF) is a commonly used procedure for cervical radiculopathy.
It is particularly appropriate when the disc herniation is central or when there is associated kyphosis, axial neck pain, or anterior spinal cord compression.
Principles of ACDF
The affected disc is removed through an anterior approach.
The compressed nerve root or spinal cord is decompressed, after which the disc space is reconstructed and fused.
Instrumentation may be added depending on the level and pathology.
Posterior Cervical Foraminotomy
A posterior laminoforaminotomy or foraminotomy can be used for lateral or foraminal soft-disc herniations, particularly when arm pain predominates.
This approach can decompress the affected nerve root while preserving the intervertebral disc and motion segment in selected patients.
Laminoplasty
Laminoplasty is a motion-preserving posterior decompression procedure primarily used for multilevel cervical spinal cord compression in a lordotic cervical spine.
It may be an alternative to multilevel laminectomy and fusion or extensive anterior decompression in appropriately selected patients.
Cervical Disc Arthroplasty
Cervical total disc arthroplasty replaces the diseased disc with an artificial disc while preserving segmental motion.
For selected patients with single-level degenerative cervical radiculopathy, short- and intermediate-term outcomes can be comparable to those of ACDF.
Surgical Treatment of Myelopathy
The operative approach for cervical myelopathy depends on cervical alignment, number of involved levels, location of compression, previous surgery, and relevant medical conditions.
Anterior, posterior, or combined approaches may be required.
Follow-Up
Patients treated conservatively should be reassessed for improvement or progression of pain, weakness, numbness, gait disturbance, and fine-motor dysfunction.
Development of new myelopathic signs requires prompt reassessment.
Referral for Possible Shoulder Pathology
Shoulder disorders can closely mimic cervical radiculopathy.
When examination suggests intrinsic shoulder disease, referral to an orthopaedic or shoulder specialist may be appropriate.
Prognosis
The overall prognosis for cervical radiculopathy is generally favorable.
Population-based studies have shown that approximately 90% of patients can achieve satisfactory outcomes with either nonoperative or operative treatment.
Prognosis of Cervical Myelopathy
Surgery for cervical myelopathy frequently produces meaningful neurologic improvement or stabilization.
However, the degree of recovery depends heavily on the severity and duration of spinal cord compression.
Importance of Early Recognition
Early diagnosis and treatment of cervical myelopathy are important because prolonged spinal cord compression can produce irreversible neurologic injury.
Patients with moderate or severe myelopathy may continue to have residual neurologic deficits even after successful decompression.
Complications of Surgery
Potential complications include infection, persistent neurologic deficit, new neurologic deficit, worsening weakness, pseudarthrosis, adjacent-segment degeneration, dysphagia, and recurrent laryngeal nerve injury.
The complication profile varies with the surgical approach.
Surgical Infection
Posterior cervical procedures generally have a higher wound-infection risk than anterior approaches.
Careful soft-tissue handling and postoperative wound monitoring are therefore important.
C5 Palsy
A new postoperative C5 nerve-root palsy can occur after either anterior or posterior cervical decompression.
It typically produces deltoid and sometimes biceps weakness.
Many patients experience substantial functional recovery over time.
Dysphagia
Difficulty swallowing is common after anterior cervical surgery.
It usually improves gradually and often resolves within several months, although persistent symptoms may occasionally occur.
Pseudarthrosis
Failure of fusion, or pseudarthrosis, can cause persistent neck pain and may occasionally require revision surgery.
The risk is influenced by smoking, multilevel surgery, bone quality, and other patient factors.
Adjacent-Segment Degeneration
Degenerative changes may develop at levels adjacent to a cervical fusion over time.
Some degeneration reflects the natural history of cervical spondylosis, while altered biomechanics following fusion may also contribute.
Plate-Related Adjacent-Level Degeneration
Anterior plates positioned close to an adjacent disc space may contribute to accelerated degeneration or ossification at that level.
Appropriate implant positioning may reduce this risk.
Hoarseness
Hoarseness after anterior cervical surgery may result from injury or irritation of the recurrent laryngeal nerve.
Most cases improve, but persistent vocal changes can occasionally occur.
Patient Monitoring
Patients should be monitored for progressive weakness, worsening sensory loss, gait deterioration, loss of fine-motor control, bowel or bladder dysfunction, and other signs of spinal cord involvement.
Any progression of myelopathic symptoms should prompt urgent specialist evaluation.
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Orthopaedic Surgery - Cerebral Palsy
Basics
Cerebral palsy is an umbrella term describing a broad group of permanent disorders of movement and posture caused by a static injury or abnormality of the developing central nervous system.
Although the underlying neurologic lesion itself is nonprogressive, its clinical consequences can change considerably as the child grows.
Muscle imbalance, contracture, skeletal deformity, altered gait, and functional limitations may therefore become more or less apparent over time even though the cerebral injury remains unchanged.
Classification
Cerebral palsy can be classified according to both the anatomic distribution of involvement and the physiologic pattern of motor abnormality.
A complete description should ideally include both components.
Anatomic Classification
Monoplegia affects one limb.
Hemiplegia involves the arm and leg on the same side of the body.
Diplegia predominantly affects both lower extremities.
Triplegia involves three extremities.
Quadriplegia involves all four extremities, with relatively preserved head control.
The term totally involved cerebral palsy is used for extensive involvement of all extremities accompanied by poor head and trunk control.
Physiologic Classification
The major physiologic patterns include spastic, athetoid, dystonic, and mixed cerebral palsy.
Spasticity is the most common motor abnormality.
Some patients demonstrate more than one movement pattern and are therefore classified as having mixed cerebral palsy.
Gross Motor Function Classification
The Gross Motor Function Classification System (GMFCS) is frequently used to describe functional mobility.
It assists with prognosis, communication among clinicians, and decision-making regarding operative and nonoperative treatment.
Functional classification is particularly useful because children with the same anatomic distribution of cerebral palsy may have very different levels of independence.
Epidemiology
Cerebral palsy affects approximately 2 per 1,000 live births.
Despite advances in obstetric and neonatal care, the overall prevalence has not shown a major decline.
Risk Factors
Important risk factors include prematurity, difficult delivery, multiple gestation, and postnatal injury to the central nervous system.
Several prenatal, perinatal, and postnatal events can ultimately produce the static cerebral lesion responsible for cerebral palsy.
Pathophysiology
Cerebral palsy produces predominantly upper motor neuron findings.
Peripheral nerves are generally anatomically normal.
The exact regions of the brain affected depend on the underlying cause and timing of the insult.
Muscle Changes
Although the primary lesion is neurologic, muscles in affected limbs can undergo secondary structural changes.
These include varying degrees of fibrosis, reduced muscle growth, shortening, and loss of normal excursion.
Such changes contribute to fixed contractures and progressive orthopaedic deformity.
Muscle biopsy is not routinely required.
Etiology
Potential causes include prenatal brain dysplasia, maternal infection, fetal hypoxia, vascular events, encephalitis, meningitis, trauma, and kernicterus.
In many patients, the precise causal event cannot be identified.
Associated Conditions
Cerebral palsy may coexist with a number of neurologic, developmental, and systemic conditions.
These include learning difficulties, behavioral disorders, abnormalities of sensory processing, visual impairment, hearing loss, seizures, and osteoporosis.
Respiratory and Gastrointestinal Disease
Children with severe cerebral palsy may also develop chronic respiratory and gastrointestinal problems.
Swallowing dysfunction, aspiration, gastroesophageal reflux, impaired cough, reduced mobility, and poor nutrition can contribute to these complications.
Diagnosis
Signs and Symptoms
One common early pattern is initial hypotonia followed later by increasing spasticity.
Developmental motor milestones may be delayed, and abnormal gait may become evident as walking begins.
Asymmetry of posture, limb use, strength, or gait may also be present.
Reflex Abnormalities
Deep tendon reflexes are often increased after the first year of life.
Clonus may develop in involved muscle groups.
Persistent primitive reflexes beyond the expected developmental period may also be observed.
Contractures
As children grow, contractures commonly develop in muscle groups exposed to persistent spasticity and reduced excursion.
The Achilles tendon, hamstrings, and hip adductors are especially prone to shortening.
Physical Examination
The examination should assess the child globally rather than focusing on a single joint or extremity.
Neurologic findings, muscle tone, mobility, joint motion, skeletal alignment, function, and independence in activities of daily living should all be considered.
Upper-Extremity Examination
The upper extremities should be examined for deformity, motor function, sensation, selective control, and practical use of the hands.
The ability to integrate sensation with motor function is especially important when considering reconstructive procedures.
Spine Examination
The spine should be examined for scoliosis, pelvic obliquity, and signs of spinal dysraphism.
Progressive spinal deformity is especially common in children with severe motor impairment.
Limb-Length Assessment
Limb lengths should be measured and compared.
Asymmetric growth may occur, especially in hemiplegic cerebral palsy.
Leg-length discrepancy can influence gait and pelvic alignment.
Joint Contractures
Range of motion should be measured systematically at all major joints.
Important measurements include ankle dorsiflexion, popliteal angle, and hip abduction.
These measurements help distinguish dynamic spasticity from established contracture.
Muscle Excursion
Muscle length and excursion should be documented.
Particular attention should be paid to the gastrocnemius-soleus complex, hamstrings, hip adductors, hip flexors, and rectus femoris.
Loss of excursion may indicate developing fixed deformity.
Dynamic Versus Fixed Deformity
The examiner should distinguish between dynamic spasticity, fixed soft-tissue contracture, bony torsional deformity, and joint instability.
This distinction is essential because each problem requires a different treatment strategy.
Gait Analysis
Instrumented and video gait analysis can provide detailed information about abnormal walking patterns.
It is particularly useful in ambulatory children with complex gait abnormalities.
Role of Instrumented Gait Analysis
Three-dimensional gait analysis may assist with planning single-event multilevel surgery (SEMLS).
It can identify the contribution of individual joints and muscle groups to the gait abnormality and can also help assess postoperative outcomes.
Imaging
Brain MRI
Most children clinically suspected of having cerebral palsy demonstrate abnormalities on brain imaging.
MRI is useful for identifying evidence of prior cerebral injury, malformation, hypoxic-ischemic damage, or other structural abnormalities.
However, a minority of children who meet clinical criteria for cerebral palsy may have a normal MRI.
Cervical Spine Imaging
Cervical spine radiographs may be indicated in selected patients with severe athetoid or dystonic movements.
Repeated involuntary neck motion can predispose some patients to cervical degeneration or instability.
Hip Radiographs
Routine surveillance radiographs of the hips are important in children with severe diplegia, quadriplegia, or total-body involvement.
These patients are at increased risk of progressive hip subluxation and dislocation.
Scoliosis Imaging
When clinical spinal deformity is present, standing or sitting scoliosis radiographs should be obtained as appropriate.
Children with severe motor impairment are particularly susceptible to progressive spinal curvature.
Differential Diagnosis
Conditions that may mimic cerebral palsy include brain or upper spinal cord tumors, upper cervical instability, neurodegenerative disorders, muscular dystrophies, metabolic disease, familial spastic paraparesis, early myopathy or neuropathy, and Rett syndrome.
Unlike cerebral palsy, many of these disorders are progressive.
Treatment
General Principles
Treatment does not correct the original cerebral lesion.
Management is therefore directed toward maximizing function, encouraging development, reducing spasticity, preventing contracture, and correcting secondary musculoskeletal deformity.
Early Stimulation
In infancy, stimulation and encouragement of movement are among the most important interventions.
Children should be given opportunities to develop motor skills, postural control, reaching, sitting, standing, and mobility according to their abilities.
Stretching
Muscle groups at risk of contracture should be stretched regularly.
The hamstrings and gastrocnemius-soleus complex commonly require particular attention.
Stretching is most useful for preserving existing range of motion rather than reversing a mature fixed contracture.
Physical Therapy
Physical therapy is most effective when directed toward specific and achievable short-term goals.
It can address gait training, mobility, stretching, strengthening, positioning, transfers, and use of adaptive equipment.
Therapists also help monitor changes in function over time.
Orthotics and Bracing
Orthoses may improve positioning, maintain range of motion, support standing or walking, and reduce deforming forces.
Ankle-foot orthoses are frequently used to improve foot position and assist gait.
Bracing should be individualized to functional goals.
Botulinum Toxin
Botulinum toxin injections can temporarily reduce focal spasticity.
They are particularly useful in younger children who are not yet appropriate candidates for definitive orthopaedic surgery.
Botulinum toxin may facilitate therapy, improve gait or positioning, and delay development of fixed contracture.
Intrathecal Baclofen
Intrathecal baclofen can reduce severe generalized spasticity by delivering medication directly into the spinal fluid.
It is used particularly in patients with widespread tone abnormalities, often in those who are nonambulatory.
Diazepam
Diazepam may reduce muscle spasm in selected circumstances but generally has limited long-term benefit.
It may occasionally be used around the time of surgery when temporary control of postoperative spasm is needed.
Multidisciplinary Care
Optimal management requires a multidisciplinary approach.
The treatment team may include physical therapists, occupational therapists, speech and language therapists, orthotists, dietitians, social workers, pediatricians, neurologists, rehabilitation specialists, and orthopaedic surgeons.
Occupational Therapy
Occupational therapy focuses on upper-extremity function, self-care, positioning, adaptive equipment, and activities of daily living.
Therapists may also assist with seating systems, communication strategies, and environmental adaptations.
Speech and Feeding Therapy
Speech and language specialists may address communication difficulties and swallowing dysfunction.
Feeding assessment is particularly important in children with severe cerebral palsy who are at risk of aspiration or inadequate nutrition.
Surgery
Surgical management is directed toward secondary effects of cerebral palsy rather than the original brain injury.
Procedures may include soft-tissue releases, tendon lengthening, selective dorsal rhizotomy, intrathecal baclofen pump placement, osteotomy, hip reconstruction, and scoliosis surgery.
Single-Event Multilevel Surgery
Single-event multilevel surgery (SEMLS) involves correcting several lower-extremity deformities during one operative episode.
Soft-tissue and bony procedures are combined according to the patient’s gait abnormalities.
The goal is to reduce the number of separate operations and consolidate rehabilitation into a single major recovery period.
Selective Dorsal Rhizotomy
Selective dorsal rhizotomy (SDR) is a neurosurgical procedure designed to reduce spasticity at the spinal cord level.
Individual dorsal sensory rootlets are electrically tested, and those producing the most abnormal spastic responses are selectively divided.
Candidates for Selective Dorsal Rhizotomy
SDR is most commonly considered in younger ambulatory children with spastic diplegia, particularly those under approximately 10 years of age who have good underlying strength and selective motor control.
Only a small proportion of children improve by an entire GMFCS level after the procedure.
The primary goal is reduction of spasticity rather than transformation of baseline neurologic function.
Intrathecal Baclofen Pump
An intrathecal baclofen pump provides continuous medication to the spinal canal.
It is particularly useful for severe generalized spasticity or dystonia and is used more commonly in nonambulatory patients.
The pump requires long-term surveillance and periodic refilling.
Muscle Lengthening
Lengthening of contracted muscles can reduce abnormal triggering and allow improved joint positioning during gait.
Commonly treated structures include the Achilles tendon, hamstrings, hip adductors, and occasionally the rectus femoris.
Overlengthening should be avoided because it may create weakness.
Foot Deformities
Significant foot deformity should generally be corrected when it interferes with standing, shoe wear, bracing, or walking.
Even children with limited ambulatory potential may benefit from a plantigrade, braceable, and pain-free foot.
Upper-Extremity Surgery
Surgery for upper-extremity muscle imbalance is undertaken selectively.
Outcomes may be less predictable when sensory integration and selective motor control are poor.
For this reason, hand surgery is less commonly beneficial than lower-extremity reconstruction in severely involved patients.
Hip Subluxation
Progressive hip displacement should ideally be treated before degenerative changes and severe pain develop.
Hip surveillance is therefore a critical component of long-term management.
Adductor Lengthening
Early hip migration caused partly by adductor contracture may be treated with adductor muscle lengthening in selected children.
The goal is to reduce deforming forces and maintain hip containment.
Femoral and Pelvic Osteotomy
More advanced hip subluxation may require a femoral osteotomy, often combined with a pelvic osteotomy.
These procedures restore alignment and improve containment of the femoral head within the acetabulum.
Salvage Hip Procedures
When severe chronic hip dislocation has already produced major pain and joint destruction, reconstructive surgery may no longer be possible.
Rarely, salvage procedures such as femoral head resection may be considered to relieve pain and improve sitting or hygiene.
Scoliosis
Progressive scoliosis can interfere with sitting balance, comfort, pulmonary function, skin care, and mobility.
Treatment depends on curve severity, growth, functional status, and symptoms.
Bracing for Scoliosis
A spinal brace may improve sitting comfort and support in selected patients.
However, bracing generally does not reliably prevent progression of neuromuscular scoliosis.
Scoliosis Surgery
Children with severe progressive curves causing poor sitting balance, pain, or functional difficulty may benefit from spinal correction and fusion.
The aim is to create a stable, balanced trunk and improve comfort and care.
Follow-Up
Children with cerebral palsy should generally be reviewed by an orthopaedic team at least once each year during growth.
Those with more severe involvement or known progressive deformity may require more frequent assessment.
Importance of Surveillance
Regular monitoring allows early detection of hip dysplasia, contracture, scoliosis, foot deformity, and declining function.
Treatment is often more effective when abnormalities are identified before they become fixed or painful.
Prognosis
Prognosis depends primarily on the severity and distribution of cerebral palsy and associated medical conditions.
Patients with extensive total-body involvement may have reduced life expectancy because of respiratory, nutritional, and other systemic complications.
Individuals with less severe forms may have a life expectancy close to that of the general population.
Walking Ability
Many ambulatory children experience some decline in walking efficiency near skeletal maturity.
Walking with cerebral palsy often requires substantially more energy than normal gait.
As body size and weight increase, this energy demand can make community ambulation more difficult.
Complications
Potential complications include progressive joint contracture, hip displacement, scoliosis, foot deformity, reduced mobility, osteoporosis, fractures, obesity, respiratory disease, and gastrointestinal dysfunction.
The pattern varies considerably according to GMFCS level and overall severity.
Weight Gain
Excessive weight gain can further impair mobility and increase the energy cost of walking or transfers.
At the same time, some severely affected patients may have poor nutritional intake.
Nutrition therefore requires careful individualized monitoring.
Fractures and Osteoporosis
Nonambulatory patients are at increased risk of low bone mineral density and fractures.
Reduced weight-bearing, nutritional deficiencies, anticonvulsant use, and limited mobility may all contribute.
Respiratory Complications
Children with severe total-body involvement are more prone to chronic respiratory problems.
Aspiration, weak cough, recurrent infection, scoliosis, and reduced mobility can impair pulmonary function.
Gastrointestinal Complications
Gastroesophageal reflux, constipation, swallowing dysfunction, and feeding difficulties are common in more severely affected children.
These problems can contribute to poor growth and recurrent respiratory illness.
Patient Monitoring
Children should be assessed periodically for functional mobility, gait, joint range of motion, muscle tone, hip stability, spinal alignment, and independence in activities of daily living.
At minimum, yearly review during growth is appropriate, with closer surveillance when progressive deformity or functional decline is present.
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Orthopaedic Surgery - Cavus Foot
Basics
Cavus foot is characterized by an abnormally elevated medial longitudinal arch, often accompanied by deformity of the hindfoot, forefoot, or both.
The deformity may be flexible in its early stages but can gradually become rigid as secondary soft-tissue contractures and bony changes develop.
Epidemiology
Cavus feet are relatively common and may occur in otherwise healthy individuals or as a manifestation of an underlying neurologic or musculoskeletal disorder.
Approximately 10% of the healthy population may demonstrate some degree of cavus foot.
A substantial proportion of clinically significant cases, historically estimated at approximately 80%, are associated with an underlying neurologic disorder.
Risk Factors
Important risk factors include neuromuscular disease, previous trauma, connective tissue disorders, and congenital deformities.
Any condition that creates chronic imbalance between the muscles acting across the foot and ankle can contribute to development of a cavus deformity.
Genetics
Several disorders responsible for cavus foot are hereditary.
The most common inherited cause is Charcot-Marie-Tooth disease, also known as hereditary motor and sensory neuropathy.
The classic form of Charcot-Marie-Tooth disease is often inherited in an autosomal dominant pattern.
Pathophysiology
Neuromuscular disease is the most common underlying cause of clinically significant cavus deformity.
Abnormal muscle strength and imbalance can produce progressive muscle atrophy, fibrosis, deformity, and joint contracture.
The deformity often begins as a flexible abnormality during childhood or adolescence and gradually becomes more rigid with growth and progression of the underlying disease.
Muscle Imbalance
Different patterns of muscle imbalance can produce different forms of cavus foot.
Excessive pull from the peroneus longus or posterior tibialis may contribute to abnormal plantarflexion or inversion forces.
Weakness of the intrinsic foot muscles can allow the long flexor tendons to dominate, resulting in clawing of the toes and progressive forefoot deformity.
Connective Tissue Disorders
Connective tissue abnormalities may also contribute to cavus foot.
Conditions such as Marfan syndrome can alter the structural support and mechanical balance of the foot.
Associated Conditions
The underlying cause should be investigated because cavus foot may be the first indication of a neurologic disorder.
The differential diagnosis differs depending on whether the deformity is bilateral or unilateral.
Bilateral Cavus Foot
Bilateral cavus deformity is commonly associated with generalized or hereditary neurologic disease.
Important causes include Charcot-Marie-Tooth disease, Friedreich ataxia, syringomyelia, myelomeningocele, muscular dystrophy, spinal cord tumor, and polyneuritis.
Unilateral Cavus Foot
A unilateral cavus deformity should raise concern for a focal neurologic or traumatic cause.
Potential etiologies include previous poliomyelitis, prior compartment syndrome of the leg or foot, traumatic peripheral nerve injury, spinal nerve root injury, severe burns, and spinal cord tumors.
A new unilateral deformity warrants particularly careful neurologic investigation.
Diagnosis
Signs and Symptoms
The characteristic finding is a high medial longitudinal arch.
Patients may also develop calluses beneath the forefoot or heel because of abnormal pressure distribution.
Some individuals remain asymptomatic, while others have pain, difficulty fitting shoes, instability, or progressive deformity.
Hindfoot Varus
The heel may be positioned in varus, causing the patient to bear excessive weight along the lateral border of the foot.
Marked hindfoot varus can predispose to recurrent lateral ankle sprains and chronic ankle instability.
Plantarflexed First Ray
A relatively plantarflexed first metatarsal or first ray is common.
This can drive the hindfoot into varus during weight-bearing and is important when distinguishing a flexible from a fixed hindfoot deformity.
Metatarsalgia
Patients may develop metatarsalgia, particularly beneath the metatarsal heads.
The elevated arch and altered forefoot loading produce excessive plantar pressure and can lead to painful callus formation.
Shoe-Wear Difficulty
Rigid or severe cavus deformity may make conventional footwear uncomfortable.
Prominent metatarsal heads, claw toes, a high instep, or lateral foot overload can create pressure points and skin irritation.
Ankle Instability
Severe cavovarus alignment increases the tendency for the ankle to invert.
Patients may therefore experience recurrent ankle sprains or chronic lateral ankle instability.
History
A complete birth, developmental, neurologic, and medical history is essential when evaluating cavus foot.
The clinician should determine when the deformity was first noticed, whether it is progressing, and whether symptoms such as weakness, numbness, gait disturbance, or balance difficulty are present.
Family History
A family history of cavus foot, neuropathy, gait abnormalities, or muscle weakness should be explored.
A positive family history may suggest an inherited disorder such as Charcot-Marie-Tooth disease.
Tethered Cord Consideration
A sudden increase in cavus deformity in a child with myelomeningocele or lipomeningocele may indicate tethering of the spinal cord.
Progressive deformity in this setting warrants neurologic and spinal evaluation.
Physical Examination
The examination should assess the entire lower extremity rather than the foot alone.
Motor strength of all muscles crossing the ankle should be measured bilaterally.
The pattern of weakness may provide clues regarding the underlying neurologic disorder.
Toe Deformities
Associated claw-toe deformities should be identified.
These often result from intrinsic muscle weakness and relative overpull of the long toe flexors and extensors.
Plantar Fascia
The plantar fascia should be assessed for tightness or contracture.
A shortened plantar fascia can help maintain the elevated arch and may contribute to rigidity of the deformity.
Neurologic Examination
Reflexes and sensation to light touch should be evaluated carefully.
Reduced reflexes, sensory loss, or distal weakness may indicate peripheral neuropathy.
Upper-Extremity Examination
The upper extremities should also be examined, particularly for intrinsic hand muscle wasting.
This can provide additional evidence of a generalized hereditary neuropathy such as Charcot-Marie-Tooth disease.
Spine Examination
The spine should be inspected for scoliosis, dimples, skin markings, hairy patches, or other abnormalities that may indicate occult spinal dysraphism or another spinal disorder.
Coleman Block Test
The Coleman block test helps determine whether hindfoot varus is flexible or rigid.
The patient stands with the heel and lateral border of the foot supported on a block while the first metatarsal and great toe hang freely off the edge.
Principle of the Coleman Block Test
Allowing the first ray to drop freely removes the effect of a plantarflexed first metatarsal on hindfoot alignment.
If the hindfoot varus corrects toward neutral, the deformity is considered flexible and forefoot-driven.
Rigid Hindfoot Varus
If the hindfoot remains in varus despite unloading the first ray, the deformity is considered rigid.
A rigid deformity is more likely to require a bony corrective procedure rather than soft-tissue treatment alone.
Electrodiagnostic Studies
Electromyography and nerve conduction studies may assist in diagnosing Charcot-Marie-Tooth disease, hereditary motor and sensory neuropathies, and lumbar radiculopathy.
These tests can characterize the distribution and severity of nerve dysfunction.
Imaging
Weight-Bearing Radiographs
Standing radiographs of the foot should be obtained to evaluate alignment and the severity of deformity.
Two useful measurements are the calcaneal pitch angle and the Meary angle.
Calcaneal Pitch
The calcaneal pitch is the angle between the inferior surface of the calcaneus and the floor.
An increased calcaneal pitch supports the presence of a high-arched cavus foot.
Meary Angle
The Meary angle is formed by the longitudinal axes of the talus and first metatarsal.
Abnormal angulation reflects disruption of normal longitudinal alignment and helps characterize the location and severity of the cavus deformity.
Spinal Imaging
When the cause of cavus foot is uncertain, radiographs or MRI of the spine may be indicated.
These studies can identify occult spinal cord abnormalities, tumors, tethering, or other neurologic causes.
Differential Diagnosis
Muscle wasting can sometimes make the longitudinal arch appear unusually high even when a true structural cavus deformity is not present.
The clinician should distinguish apparent high arch from fixed bony or soft-tissue deformity.
Treatment
General Measures
No treatment is required when the patient is asymptomatic, functionally normal, and has healthy skin without pressure lesions.
Management is directed toward symptoms, progression, instability, and the underlying cause.
Insoles and Metatarsal Support
A padded insole with an arch support or metatarsal bar may reduce plantar pressure and relieve metatarsalgia.
The orthosis should redistribute pressure rather than excessively increase an already high arch.
Lateral Heel Wedge
A lateral heel wedge may help correct a flexible hindfoot varus deformity by encouraging the heel toward a more neutral position.
It is most effective when the hindfoot remains passively correctable.
Bracing
A brace may be useful when significant muscle weakness produces instability.
Bracing can also help patients who have both cavus deformity and chronic ankle instability.
Stretching
Regular stretching may help maintain flexibility and slow the development of fixed contractures.
Particular attention may be given to the plantar fascia and other tight muscle-tendon units.
Orthoses
Custom-made foot orthoses can reduce pain in patients with cavus feet from a variety of causes.
They are particularly useful for redistributing abnormal plantar pressures and improving shoe tolerance.
Cavovarus Deformity
Cavovarus feet are often less well tolerated than isolated mild cavus feet.
Progressive deformity may lead to shoe-wear difficulty, recurrent ankle instability, painful calluses, metatarsalgia, and eventually arthritis.
Physical Therapy
Physical therapy may include stretching of the plantar fascia and other contracted muscles or tendons.
The goal is to preserve flexibility and slow progression toward a rigid deformity.
Strengthening and Proprioception
When ankle instability is present, strengthening, proprioceptive training, and closed-chain balance exercises may be helpful.
These exercises improve dynamic control of the ankle but do not correct a fixed structural cavovarus deformity.
Indications for Surgery
Surgery is usually considered for progressive deformity, persistent pain despite conservative treatment, chronic ankle instability, major shoe-wear difficulty, or rigid deformity associated with arthritis.
The procedure must be tailored to the specific components of the deformity.
Surgical Planning
Careful examination is required to determine which portions of the deformity are flexible and which are fixed.
Surgical treatment is usually stepwise and individualized, with correction of muscle imbalance, soft-tissue contracture, bony malalignment, and instability as needed.
Flexible Deformity
Flexible deformities can often be treated primarily with soft-tissue rebalancing procedures.
These may include tendon transfer, tendon lengthening, or plantar fascia release.
Rigid Deformity
Rigid deformities generally require osteotomy or arthrodesis because soft-tissue procedures alone cannot adequately correct fixed bony alignment.
The goal is to restore a plantigrade, stable, and pain-free foot.
Plantar Fasciotomy
A plantar fasciotomy may be used to release a contracted plantar fascia and help lower the excessively high arch.
It is often performed as one component of a larger reconstruction.
Midfoot and Metatarsal Osteotomies
Rigid cavus deformity may require midfoot or metatarsal osteotomies.
These procedures correct fixed bony angulation and redistribute plantar loading.
Calcaneal Osteotomy
When hindfoot varus is rigid, a lateral closing-wedge calcaneal osteotomy may be performed.
This repositions the heel into a more neutral alignment and reduces excessive loading along the lateral border of the foot.
Tendon Lengthening
Contracted muscle-tendon units may require surgical lengthening.
This decreases deforming forces and improves balance around the foot and ankle.
Tendon Transfers
Tendon transfers are commonly used when muscle imbalance is a major component of the deformity.
A functioning tendon is redirected to compensate for a weak muscle group and restore more balanced forces across the foot.
Chronic Ankle Instability
When chronic ligamentous instability accompanies cavovarus deformity, lateral ligament reconstruction may be required.
Correction of the cavovarus alignment is usually important as well, because ligament reconstruction alone may fail if the underlying varus deformity remains.
Tenodesis procedures may sometimes be added for additional stabilization.
Complex or Previously Operated Feet
Stiff or multiply operated cavus feet may require more extensive reconstruction.
Procedures such as navicular excision or cuboid closing-wedge osteotomy may be used in selected complex cases.
Triple Arthrodesis
Triple arthrodesis is generally reserved as a salvage procedure.
It may be indicated when previous reconstruction has failed or when the patient has a rigid deformity with painful arthritis involving multiple hindfoot joints.
Follow-Up
Patients with cavus foot should be followed periodically by an orthopaedic specialist, particularly when the deformity is progressive or associated with muscle weakness.
Monitoring should assess alignment, muscle strength, sensory status, skin integrity, ankle stability, and progression of contractures.
Timing of Reconstruction
Progressive deformity may be easier to correct before it becomes completely rigid.
Early recognition and reconstruction in selected patients may avoid the need for later fusion procedures.
Indications for Referral
All patients with symptomatic cavus feet should be considered for orthopaedic evaluation.
Referral is especially important when the deformity is unilateral, progressive, associated with neurologic signs, or causing recurrent instability or skin problems.
Prognosis
Cavus feet caused by an underlying neuropathy often progress gradually over time.
The rate and severity of progression depend on the underlying neurologic disease and the degree of muscle imbalance.
Neuropathic Ulceration
Patients with diabetes or Charcot-Marie-Tooth disease may lose protective sensation.
Abnormal pressure beneath prominent parts of the foot can then produce neuropathic ulceration.
Repeated ulceration may progress to infection and osteomyelitis.
Risk of Amputation
Severe neuropathic ulceration complicated by deep infection or osteomyelitis can eventually result in amputation.
For this reason, appropriate footwear, orthoses, routine skin inspection, and pressure reduction are especially important in patients with sensory loss.
Footwear in Athletes
For athletes with cavus feet, neutral cushioned running shoes may help reduce plantar pressures and lower the risk of repetitive pressure-related injury.
Footwear should be selected according to comfort, alignment, and the individual’s activity demands.
Patient Monitoring
Patients should be monitored for progressive arch elevation, increasing hindfoot varus, muscle weakness, sensory loss, recurrent ankle sprains, painful calluses, and skin ulceration.
Any rapid change in deformity or neurologic function should prompt investigation for an underlying or worsening neurologic disorder.
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Orthopaedic Surgery - Carpal Tunnel Syndrome
Basics
Carpal tunnel syndrome (CTS) is a compressive neuropathy of the median nerve as it passes through the carpal tunnel at the wrist.
The carpal tunnel is a relatively rigid anatomical space. Its floor is formed by the volar radiocarpal and intercarpal ligaments, while the transverse carpal ligament forms the roof.
Within the tunnel pass nine flexor tendons—the four flexor digitorum profundus tendons, four flexor digitorum superficialis tendons, and the flexor pollicis longus tendon—together with the median nerve.
Because there is little room for expansion, any increase in pressure within the tunnel may compress the median nerve.
Functional Effects
Compression of the median nerve may cause numbness, paresthesias, pain, weakness, and impaired fine motor function.
Sensory symptoms usually involve the palmar surface of the thumb, index finger, middle finger, and radial half of the ring finger.
With more advanced compression, weakness or atrophy of the thenar muscles may develop, particularly affecting thumb palmar abduction.
Pregnancy Considerations
Carpal tunnel syndrome occurs more frequently during pregnancy than in the general population.
Symptoms commonly improve or resolve after delivery as pregnancy-related fluid retention decreases.
Initial treatment generally consists of a nighttime wrist splint maintaining the wrist in neutral.
A corticosteroid injection may be considered for persistent symptoms when appropriate, with medication safety reviewed in conjunction with the patient’s obstetric care.
Surgery During Pregnancy
Because pregnancy-related CTS frequently resolves postpartum, surgery is generally deferred when possible.
However, severe cases involving persistent numbness, progressive weakness, or major functional impairment may occasionally require surgical decompression during pregnancy.
When necessary, carpal tunnel release can be performed using local anesthesia.
Epidemiology
Carpal tunnel syndrome affects approximately 4–5% of the population.
It occurs most frequently between approximately 40 and 60 years of age and is more common in women than men.
CTS is the most frequently diagnosed compressive neuropathy of the upper extremity.
Risk Factors
Recognized risk factors include female sex, diabetes mellitus, hypothyroidism and other endocrine abnormalities, smoking, elevated body mass index, pregnancy, perimenopausal status, rheumatoid arthritis, and previous hand or wrist tendinopathy.
Forceful repetitive hand use may also contribute.
The relationship between CTS and routine computer use remains controversial.
Occupational Factors
Work involving forceful gripping, sustained wrist positioning, repetitive hand exertion, or vibration may increase symptoms in susceptible individuals.
In occupational cases, modification of the provoking activity can be an important part of treatment.
Genetics
No specific genetic factor has been definitively established as a direct cause of carpal tunnel syndrome.
However, inherited differences in anatomy, body habitus, and systemic disease susceptibility may indirectly influence risk.
Pathophysiology
CTS develops when increased pressure within the carpal tunnel compromises the median nerve.
Compression and traction can impair the nerve’s microcirculation, producing edema and reduced oxygen delivery.
Repeated or prolonged compression may subsequently produce recurrent demyelination and slower nerve conduction.
Nerve Conduction Changes
As compression becomes more severe, electrical impulses travel more slowly through the median nerve at the wrist.
Prolonged compression may eventually cause axonal injury, resulting in persistent numbness, weakness, and thenar muscle atrophy.
Etiology
Any process that reduces the available space within the carpal tunnel or increases its contents can compress the median nerve.
In many patients, no single structural cause is identified.
Common Causes
Potential causes include flexor tenosynovitis, previous trauma involving the carpal bones, ganglion cysts, fibromas, lipomas, rheumatoid cysts, gout, and diabetic neuropathy.
Inflammatory swelling of the tendon sheaths may be particularly important because all nine flexor tendons pass through the same confined space.
Diagnosis
Carpal tunnel syndrome can often be diagnosed accurately through a careful history and physical examination.
Evaluation should include assessment for sensory disturbance, thenar weakness or atrophy, and reproduction of symptoms with provocative maneuvers.
Electrodiagnostic testing can support the diagnosis and determine severity but is not mandatory in every straightforward case.
Signs and Symptoms
Typical symptoms include numbness and tingling in the median nerve distribution, hand weakness or clumsiness, and pain involving the hand, wrist, or distal forearm.
Symptoms are frequently worse at night.
Many patients wake from sleep because of painful numbness or tingling and may shake the hand to obtain relief.
Sensory Distribution
Paresthesias typically involve the thumb, index finger, middle finger, and radial half of the ring finger.
The little finger is usually spared.
Symptoms may occasionally radiate proximally into the wrist or forearm.
Motor Symptoms
Patients may notice difficulty with tasks requiring fine motor control, such as buttoning clothing, handling coins, writing, or gripping small objects.
More advanced disease may cause weakness of thumb palmar abduction due to involvement of the abductor pollicis brevis.
Nocturnal Symptoms
Nighttime symptoms are particularly characteristic.
Wrist flexion during sleep may increase carpal tunnel pressure and aggravate median nerve compression.
This explains why neutral-position nighttime splinting can be effective.
Tinel Sign
The Tinel sign is elicited by gently tapping over the median nerve at the carpal tunnel while the wrist is held in a neutral position.
A positive test reproduces tingling or electric sensations in the median nerve distribution of the hand.
Phalen Test
The Phalen test is performed by placing the wrist in full flexion, traditionally around 90°, and maintaining the position.
Development of paresthesias in the median nerve distribution within approximately 30–60 seconds supports the diagnosis.
Flexion-Compression Test
For the flexion-compression test, the elbow is extended and the forearm is supinated.
The wrist is flexed to approximately 60°, and direct pressure is applied over the median nerve at the carpal tunnel.
Reproduction of median-distribution paresthesias within about 30 seconds is considered a positive finding.
Physical Examination
The hand should be inspected for thenar muscle wasting.
Strength of thumb palmar abduction should be assessed carefully because weakness may indicate advanced median nerve compression.
Thenar Atrophy
Visible flattening or wasting of the thenar eminence suggests chronic or severe CTS.
Once substantial muscle atrophy has developed, full motor recovery after decompression may be incomplete.
Sensory Examination
Sensation should be tested over the thumb and fingers.
Two-point discrimination can be measured on the radial and ulnar sides of each fingertip.
Normal discrimination is generally less than approximately 5–6 mm, or should be symmetric with the opposite hand when symptoms are unilateral.
Examination for Other Neuropathies
The entire upper extremity should be examined for alternative or additional sites of nerve compression.
Conditions such as cervical radiculopathy, proximal median nerve entrapment, and cubital tunnel syndrome may mimic or coexist with CTS.
Laboratory Tests
Laboratory investigations are not required in every patient but may be useful when a systemic cause is suspected.
Tests may include erythrocyte sedimentation rate, serum glucose, serum uric acid, and thyroid function studies.
These investigations may identify inflammatory, metabolic, endocrine, or crystal-related conditions contributing to nerve compression.
Electrodiagnostic Studies
Electromyography and nerve conduction studies can confirm median nerve compression at the wrist and help determine its severity.
They can also identify additional abnormalities elsewhere along the nerve pathway.
Double-Crush Syndrome
Electrodiagnostic testing may help detect a double-crush syndrome, in which median nerve dysfunction at the wrist coexists with a more proximal lesion, such as cervical radiculopathy or proximal median nerve compression.
This possibility is particularly important when symptoms persist despite apparently adequate carpal tunnel treatment.
Imaging
Plain Radiographs
Routine radiographs are not necessary for uncomplicated CTS.
Wrist radiographs may be useful in patients with a history of trauma, deformity, or longstanding inflammatory arthritis.
They can demonstrate fractures, malalignment, arthritis, or other structural abnormalities affecting the carpal tunnel.
Cervical Spine Imaging
Cervical spine radiographs may be useful when a proximal neurologic cause is suspected.
They can also demonstrate abnormalities such as a cervical rib when thoracic outlet syndrome is being considered, although thoracic outlet symptoms more often resemble ulnar-sided neuropathy.
Differential Diagnosis
Important differential diagnoses include thoracic outlet syndrome, cervical nerve root compression from degenerative disc disease or tumor, proximal median nerve entrapment, and cubital tunnel syndrome.
Clinical distribution of symptoms and electrodiagnostic testing can help distinguish these disorders.
Treatment
General Measures
Initial management is usually nonoperative, particularly in mild or intermittent disease.
The principal conservative measures are nighttime wrist splinting, activity modification, and corticosteroid injection in selected patients.
Nighttime Splinting
A wrist splint should hold the wrist in a neutral position during sleep.
Excessive flexion or extension increases carpal tunnel pressure and can worsen symptoms.
Night splinting is particularly useful for patients with nocturnal paresthesias.
Duration of Splinting
To determine whether conservative treatment is effective, nighttime splinting should generally be used consistently for at least 1 month.
Patients who improve while wearing the splint but experience rapid symptom recurrence after discontinuation may ultimately require surgical treatment.
Activity Modification
When symptoms are associated with work or repetitive activity, modification of the provoking tasks is recommended.
Reducing forceful gripping, repetitive wrist motion, prolonged wrist flexion or extension, and other aggravating activities may reduce symptoms.
Corticosteroid Injection
A corticosteroid injection into the carpal tunnel may provide temporary or sometimes prolonged symptom relief.
Injection should be performed by an experienced clinician because inadvertent injection into or injury of the median nerve can cause significant neurologic damage.
Indications for Corticosteroid Injection
Injection is particularly useful when compression is expected to be temporary, such as during pregnancy, or when modification of the precipitating activity is feasible.
It may also serve as a therapeutic trial in mild to moderate CTS.
Physical Therapy
Formal physical therapy is not routinely required for uncomplicated carpal tunnel syndrome.
Therapy may be useful for workplace modification, ergonomic assessment, or selected hand and nerve-gliding programs, although these are generally adjunctive rather than primary treatment.
Medication
No oral medication has been shown to specifically reverse median nerve compression within the carpal tunnel.
Analgesics may relieve pain temporarily but do not correct the underlying mechanical compression.
Indications for Surgery
Carpal tunnel release is considered when conservative treatment has failed or when there is evidence of constant numbness, motor weakness, thenar atrophy, or significant electrodiagnostic abnormality.
Progressive neurologic deficit is a particularly strong indication for decompression.
Surgical Principle
The goal of surgery is to decompress the median nerve by dividing the transverse carpal ligament.
This increases the volume of the carpal tunnel and reduces pressure on the nerve.
Open Carpal Tunnel Release
Open release is performed through a longitudinal palmar incision.
The incision is typically positioned in line with the ulnar border of the ring finger axis and provides direct visualization of the transverse carpal ligament and median nerve.
The ligament is divided completely to decompress the nerve.
Mini-Open Release
A mini-open technique uses a smaller incision while maintaining direct visualization of the ligament.
Its aim is to reduce soft-tissue disruption while achieving complete decompression.
Endoscopic Carpal Tunnel Release
Endoscopic release divides the transverse carpal ligament through one or more small portals using an endoscopic system.
Long-term results are generally similar to those of open release.
Some patients may return to work more quickly and experience less early scar discomfort.
Open Versus Endoscopic Release
At approximately 6 months and beyond, open and endoscopic procedures generally provide comparable symptom relief.
Endoscopic release may result in faster early functional recovery and less scar tenderness in some patients.
However, the risk of nerve injury may be slightly higher with endoscopic techniques.
Follow-Up
Patients treated conservatively should be reassessed for persistence or progression of numbness, nocturnal symptoms, weakness, and functional impairment.
Failure of a reasonable period of nonoperative management may warrant surgical referral.
Prognosis With Nonoperative Treatment
Many patients with mild or occupationally related CTS improve with a combination of night splinting, corticosteroid injection, and modification of aggravating activities.
If the provoking work activity continues unchanged, conservative measures may provide only temporary relief.
Prognosis After Surgery
Pain often improves very quickly after carpal tunnel release.
Numbness usually recovers more gradually over the following weeks to months because the median nerve requires time to recover from chronic compression.
Severe Compression
Patients with longstanding or severe compression may not regain completely normal sensation or motor strength even after technically successful decompression.
Permanent nerve injury may already have occurred before surgery.
Recovery of Strength
Grip and thenar strength may take 6 months or longer to recover maximally after release.
The speed and completeness of recovery depend partly on the duration and severity of preoperative nerve compression.
Complications
Possible complications include iatrogenic injury to the median nerve or its branches, painful scar formation, pillar pain, incomplete release, infection, and persistent or recurrent symptoms.
Nerve injury can occur with either open or endoscopic surgery but may be slightly more frequent with endoscopic techniques.
Scar Sensitivity
Tenderness or hypersensitivity around the surgical incision can occur, particularly after open release.
This usually improves gradually over several months but may persist in a minority of patients.
Pillar Pain
Pillar pain refers to discomfort on either side of the carpal tunnel after release, often around the thenar or hypothenar regions.
It commonly improves with time but can temporarily interfere with gripping or weight-bearing through the palm.
Persistent Symptoms After Surgery
Persistent symptoms after decompression may result from incomplete division of the transverse carpal ligament, an incorrect initial diagnosis, severe irreversible nerve damage, or an untreated proximal compression site.
A double-crush syndrome or generalized peripheral neuropathy should also be considered.
Patient Monitoring
Patients receiving conservative treatment should be monitored for progression from intermittent symptoms to constant numbness or motor weakness.
Development of thenar atrophy or persistent sensory loss should prompt surgical consideration.
Postoperative Monitoring
After surgery, patients are typically reviewed for wound assessment and suture removal.
Activity can then be increased progressively if wound healing is satisfactory.
Neurologic recovery may continue for several months after the incision itself has healed.
Patient Teaching
Patients should avoid prolonged or repetitive wrist positions that aggravate symptoms.
Workstation or occupational modifications may be useful when symptoms are related to repetitive or forceful hand activity.
Prevention
Reducing prolonged wrist flexion or extension and minimizing repetitive forceful hand use may help decrease symptoms in susceptible individuals.
Maintaining appropriate ergonomic wrist positioning may also be beneficial.
Frequently Asked Question: Should Pregnancy-Related CTS Be Treated Surgically?
CTS arising during pregnancy often improves after delivery.
Initial treatment should therefore consist of nighttime neutral wrist splinting, with corticosteroid injection considered when appropriate.
Surgery is generally deferred until after delivery so that spontaneous resolution can be assessed, although severe cases can be treated surgically under local anesthesia when necessary.
Frequently Asked Question: Can CTS Recur After Surgical Release?
True recurrence after an adequately performed release is uncommon.
Persistent or recurrent symptoms should prompt evaluation for incomplete ligament release, an alternative diagnosis, double-crush syndrome, or an underlying peripheral neuropathy.
Frequently Asked Question: What Are the Typical Symptoms?
Typical symptoms are pain, numbness, and tingling, especially at night.
The sensory symptoms usually involve the thumb, index finger, middle finger, and radial half of the ring finger.
Patients may also experience difficulty with fine motor tasks or hand clumsiness.
When motor weakness develops, the abductor pollicis brevis is commonly affected.
