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Orthopaedic Surgery - Elbow Arthritis
Basics
The elbow may be affected by both inflammatory and noninflammatory arthropathies.
Regardless of the underlying cause, elbow arthritis commonly produces pain, weakness, stiffness, and progressive loss of motion.
The clinical pattern varies depending on whether the arthritis is primary degenerative, post-traumatic, inflammatory, infectious, or crystalline.
Epidemiology
Elbow arthritis is relatively uncommon compared with arthritis of weight-bearing joints.
Primary osteoarthritis accounts for only approximately 1–2% of elbow arthritis, while most remaining cases are related to inflammatory disease or previous trauma.
The condition can occur at any age, depending on the cause, and males and females are affected overall at roughly similar rates.
Total Elbow Arthroplasty and Age
Because total elbow arthroplasty carries lifelong restrictions on repetitive loading and heavy lifting, it is generally favored for older, lower-demand patients, commonly those over approximately 60 years of age.
Joint-preserving procedures are preferred whenever practical in younger and more active individuals.
Risk Factors
Important risk factors include rheumatoid arthritis, previous septic arthritis, crystalline arthropathy, hemophilia, and prior elbow trauma.
Repeated heavy use of the elbow may also contribute to primary osteoarthritis.
Etiology
The major causes of elbow arthritis include inflammatory arthropathy and trauma.
Primary osteoarthritis occurs less commonly and is typically associated with long-term repetitive loading.
Clinical Presentation
General Symptoms
Elbow arthritis usually presents with a combination of pain and restriction of motion.
The location and timing of pain during the arc of movement can provide clues regarding the stage and cause of disease.
Primary Osteoarthritis
Primary elbow osteoarthritis is most commonly seen in individuals with a history of heavy repetitive upper-extremity use, such as manual laborers, weight lifters, and throwing athletes.
Early Disease
During the early stage, pain is commonly produced at the terminal limits of motion, especially terminal extension.
This usually results from impingement between osteophytes around the olecranon, coronoid process, and their corresponding fossae.
Motion may become restricted because of these mechanical blocks.
Advanced Disease
As degeneration progresses, pain may extend throughout the entire arc of elbow motion.
Pain that is no longer limited to terminal flexion or extension suggests more advanced articular involvement.
Post-Traumatic Arthritis
Post-traumatic arthritis may develop after virtually any injury to the elbow, even when the original trauma initially appeared relatively minor.
Fractures involving the joint surface, instability, malalignment, cartilage injury, or previous dislocation may accelerate degeneration.
Sources of Pain
Pain in post-traumatic arthritis may arise from articular degeneration, osteophyte impingement, stiffness, or associated nerve entrapment.
Functional Limitations
Activities that place traction or load across the elbow, such as carrying a bag or briefcase, may become difficult and may be tolerated only briefly.
Late Changes
As disease progresses, the patient may develop substantial reduction in motion and a persistent flexion contracture.
Pain may become present during much of the available range.
Radiographic Findings
AP and lateral radiographs may demonstrate osteophyte formation, subchondral sclerosis, joint irregularity, and loose bodies.
Inflammatory Arthritis
Inflammatory arthropathies can produce a similar loss of motion but often have a different pain pattern.
Pain is frequently present throughout the entire arc of movement, rather than being confined to terminal positions.
Advanced Inflammatory Disease
With progression, patients may lose both elbow flexion-extension and forearm pronation-supination.
Joint destruction may eventually produce deformity and instability.
Signs of Inflammation
Examination may reveal joint warmth, effusion, swelling, and synovitis.
During early inflammatory disease, plain radiographs may remain relatively normal because synovial inflammation and joint fluid precede significant osseous destruction.
Physical Examination
Inspection
The elbow should be inspected for previous surgical incisions, scars, skin grafts, areas of fibrosis, deformity, swelling, and muscular atrophy.
Previous surgery or trauma may influence both diagnosis and future surgical planning.
Range of Motion
Active and passive range of motion should be measured, preferably with a goniometer.
Measurements should include flexion, extension, pronation, and supination.
Comparison with the opposite elbow is useful.
Stability
Varus, valgus, and rotational stability should be assessed.
Associated ligamentous insufficiency may contribute to pain or alter the choice of treatment.
End Point of Motion
The examiner should determine whether restriction has a firm mechanical endpoint or a softer capsular endpoint.
Crepitus, catching, or locking should also be documented because these findings may indicate osteophytes or intra-articular loose bodies.
Early Findings
Pain and loss of range of motion are often the earliest clinical findings.
Effusion
An elbow effusion is generally easiest to appreciate on the lateral side of the joint, around the soft spot bounded by the lateral epicondyle, radial head, and olecranon.
Contracture
Chronic disease may result in fixed flexion or extension contracture.
Flexion contracture is especially common.
Ulnar Neuropathy
Late-stage arthritis may produce ulnar nerve compression or irritation, particularly when osteophytes, deformity, or valgus alignment narrow the cubital tunnel.
Patients may develop numbness or paresthesias in the ring and small fingers.
Ankylosis
Advanced destructive disease can ultimately result in severe stiffness or ankylosis of the elbow.
Laboratory Evaluation
Rheumatologic Studies
A rheumatologic evaluation should be performed when an inflammatory arthropathy is suspected.
The specific laboratory studies depend on the clinical context.
Joint Aspiration
Joint aspiration is important when infection or crystalline arthropathy is being considered.
Synovial fluid can be sent for cell count, differential, Gram stain, culture, and crystal analysis as appropriate.
Suspected Septic Arthritis
When septic arthritis is a concern, inflammatory markers such as ESR and C-reactive protein should be obtained in addition to aspiration and microbiologic studies.
Crystal Analysis
Synovial fluid should be examined for crystals when gout or calcium pyrophosphate deposition disease is suspected.
Imaging
Plain Radiographs
Routine imaging consists of AP and lateral radiographs of the elbow.
Additional views may be obtained when loose bodies, specific osteophytes, or deformity require further definition.
Pathological Findings
Rheumatoid Arthritis
Rheumatoid involvement typically produces symmetric joint-space narrowing, periarticular erosions, and diffuse osteopenia.
The proliferative synovium progressively destroys cartilage and subchondral bone.
Radial Head Destruction
In advanced rheumatoid arthritis, the radial head may become substantially eroded or destroyed.
Valgus deformity and instability may subsequently develop.
Primary Osteoarthritis
Primary osteoarthritis may initially preserve much of the central ulnohumeral and radiocapitellar joint space.
Characteristic abnormalities include osteophytes around the olecranon and coronoid processes and the development of loose bodies.
Differential Diagnosis
Septic Arthritis
Septic arthritis should be excluded in patients with acute severe pain, effusion, fever, systemic symptoms, or markedly elevated inflammatory markers.
It represents an urgent diagnosis.
Elbow Instability
Ligamentous instability can produce pain, clicking, apprehension, and functional loss that may mimic arthritis.
Tendinopathy
Medial or lateral epicondylitis and other tendon disorders can cause elbow pain without significant intra-articular degeneration.
Nerve Entrapment
Ulnar, radial, or median nerve compression around the elbow can cause pain and weakness and may coexist with arthritis.
Treatment
General Principles
Initial management is generally nonoperative.
Surgery should be reserved for patients with persistent disabling pain, substantial functional limitation, or mechanical symptoms despite appropriate conservative treatment.
Activity Modification
Activities should be adjusted according to symptoms.
Patients may need to reduce repetitive lifting, heavy resistance exercises, throwing, or other movements that provoke pain.
Nonoperative Treatment
Rheumatoid Arthritis
Treatment of rheumatoid arthritis includes appropriate systemic disease-modifying antirheumatic drugs (DMARDs), activity modification, physical therapy, bracing, and supportive devices.
Control of the systemic inflammatory disease is an important part of preserving joint function.
Osteoarthritis
Nonoperative management of elbow osteoarthritis may include rest, NSAIDs when tolerated, activity modification, physical therapy, and progressive or dynamic splinting.
Corticosteroid Injection
An intra-articular corticosteroid injection may be considered for selected patients whose symptoms persist despite oral anti-inflammatory treatment.
Relief may be temporary and repeated injections should be used judiciously.
Splinting
Dynamic or progressive static splints may help improve a flexion or extension contracture.
They are most useful when a substantial portion of the motion loss remains soft-tissue rather than fixed osseous restriction.
Radiotherapy
Radiosynovectomy
Radioactive synovectomy, or radiosynoviorthesis, involves sterile intra-articular administration of a radioisotope to suppress diseased synovium.
It has been used selectively for persistent inflammatory synovitis but is not a routine treatment for degenerative elbow arthritis.
Surgery
The appropriate procedure depends on patient age, activity level, cause of arthritis, degree of joint destruction, location of disease, motion loss, instability, and condition of surrounding soft tissues.
Synovectomy
Synovectomy may be considered in rheumatoid arthritis when persistent proliferative synovitis causes pain and progressive joint damage.
Removing inflamed synovium may improve symptoms and potentially slow local destruction in selected patients.
Arthroscopic Debridement
Arthroscopic debridement is commonly used for selected patients with degenerative arthritis.
The procedure can include removal of loose bodies and impinging osteophytes, together with capsular release when needed to improve extension.
Arthroscopy After Previous Trauma
Prior trauma or surgery may distort normal anatomy and increase the risk to nearby nerves during elbow arthroscopy.
This requires careful patient selection and experienced surgical technique rather than serving as an absolute contraindication in every case.
Open Debridement Arthroplasty
Open debridement arthroplasty is especially useful in younger, active patients with symptomatic impingement and stiffness but reasonably preserved articular surfaces.
The procedure may include capsular release, osteophyte removal, and excision of loose bodies.
Interposition Arthroplasty
Interposition arthroplasty may be considered in younger patients with severe joint destruction due to inflammatory or post-traumatic arthritis when total elbow arthroplasty is undesirable.
A biological or synthetic interposition material is placed between the damaged joint surfaces.
Advantages of Interposition Arthroplasty
Unlike total elbow arthroplasty, interposition arthroplasty does not impose the same strict lifelong lifting limitations.
This can make it attractive in selected younger patients.
Limitations
The procedure is generally unsuitable for elbows with substantial fixed coronal-plane deformity, historically more than approximately 10° of fixed varus or valgus.
A major complication is postoperative instability.
Radiocapitellar Hemiarthroplasty
Radiocapitellar hemiarthroplasty may be considered when arthritis is predominantly confined to the radiocapitellar articulation, particularly after trauma or in selected cases of primary osteoarthritis.
Total Elbow Arthroplasty
Indications
Total elbow arthroplasty is considered for patients with severe pain throughout the range of motion, substantial stiffness or loss of function, advanced joint destruction, and failure of nonoperative treatment.
Patient Selection
Because the implant has limited tolerance for repetitive heavy loading, total elbow arthroplasty is best suited to older, lower-demand patients who can comply with permanent lifting restrictions.
Contraindications
Relative or absolute contraindications include poor surrounding skin or soft tissue, uncontrolled infection, major neurologic impairment affecting the limb, and inability or unwillingness to comply with postoperative activity restrictions.
Follow-Up
Rheumatoid Arthritis
Patients with rheumatoid involvement of the elbow may be followed approximately every 6–12 months, depending on symptoms and systemic disease activity.
AP and lateral radiographs can be used to monitor progressive joint destruction.
Complications
Complications of Progressive Untreated Disease
Persistent severe arthritis may lead to ankylosis, progressive deformity, and ulnar nerve palsy or neuropathy.
Total Elbow Arthroplasty Complications
Potential complications after total elbow arthroplasty include infection, ulnar nerve irritation or injury, implant wear, and aseptic loosening.
Instability
Instability may occur after procedures such as interposition arthroplasty or in advanced inflammatory disease with ligamentous destruction.
Stiffness
Residual or recurrent stiffness can occur even after debridement or capsular release, particularly in patients with extensive scarring or advanced arthritis.
Patient Monitoring
Patients should be monitored for pain, progressive loss of motion, contracture, mechanical symptoms, instability, ulnar neuropathy, and radiographic progression.
After surgery, surveillance should additionally assess wound healing, range of motion, implant integrity when applicable, and recurrence of symptoms.
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Orthopaedic Surgery - Elbow Anatomy and Examination
Basics
The elbow is a complex trochoginglymoid joint, meaning that it functions primarily as a hinge while also permitting rotational motion of the forearm.
Functionally, the elbow consists of three articulations: the humeroulnar joint, radiocapitellar joint, and proximal radioulnar joint.
Together, these joints permit flexion and extension of the elbow as well as pronation and supination of the forearm.
Osseous Anatomy
Humeroulnar Joint
The humeroulnar joint is formed by articulation of the trochlea of the distal humerus with the greater sigmoid, or trochlear, notch of the proximal ulna.
This articulation is the major contributor to elbow flexion and extension.
Coronoid and Radial Fossae
Anteriorly, the distal humerus contains the coronoid fossa and radial fossa.
During elbow flexion, these depressions accommodate the coronoid process of the ulna and radial head, respectively.
Olecranon Fossa
Posteriorly, the olecranon fossa receives the olecranon process of the ulna during elbow extension.
This relationship permits near-complete extension while maintaining joint congruity.
Ulnar Nerve Groove
A groove lies posterior to the medial epicondyle through which the ulnar nerve passes.
Because the nerve is superficial in this region, it is vulnerable to compression, irritation, trauma, and subluxation.
Radiocapitellar Joint
The radiocapitellar articulation is formed between the radial head and capitellum of the distal humerus.
The superior surface of the radial head is concave and articulates with the convex capitellum.
Radial Head
The radial head is covered with articular cartilage over most of its circumference, approximately 280°.
This allows it to articulate smoothly not only with the capitellum but also with the lesser sigmoid notch of the ulna.
Function of the Radiocapitellar Joint
The radiocapitellar joint contributes to elbow stability and allows rotation of the radius during forearm pronation and supination.
Proximal Radioulnar Joint
The proximal radioulnar joint is formed by the articulation of the radial head with the lesser sigmoid, or radial, notch of the ulna.
The radial head rotates within this articulation, producing a pivot mechanism essential for pronation and supination.
Ligaments
Ulnar Collateral Ligament
The ulnar collateral ligament (UCL) is the major medial stabilizer of the elbow.
It resists valgus stress and is especially important during overhead throwing.
The anterior bundle is the primary restraint to valgus loading through much of the functional arc.
Lateral Collateral Ligament Complex
The radial or lateral collateral ligament complex stabilizes the lateral elbow.
Its components resist varus stress and posterolateral rotatory instability.
Muscles
Biceps Brachii
The biceps brachii functions as an elbow flexor and powerful forearm supinator.
Its contribution to supination is particularly important when the elbow is flexed.
Brachialis
The brachialis is a strong elbow flexor.
Because it inserts on the ulna, its flexion function is relatively independent of forearm position.
Triceps Brachii
The triceps brachii is the primary extensor of the elbow.
Its tendon inserts on the olecranon.
Pronator Teres
The pronator teres contributes to forearm pronation and also assists with elbow flexion.
Nerves
Median Nerve
The median nerve crosses the elbow anteriorly.
It lies superficial to the brachialis muscle and generally medial to the brachial artery.
Distally, it supplies most of the flexor muscles of the forearm as well as important motor and sensory structures in the hand.
Ulnar Nerve
The ulnar nerve passes superficially behind the medial epicondyle through the cubital tunnel.
This location is a common site for ulnar nerve compression and subluxation.
The nerve ultimately supplies most of the intrinsic muscles of the hand.
Radial Nerve
The radial nerve crosses the elbow anterior to the lateral epicondylar region.
Near the elbow, it divides into the superficial radial sensory nerve and the posterior interosseous nerve.
Its motor branches supply the elbow, wrist, and finger extensor musculature.
History
A complete history should be obtained before examination.
Important information includes the patient’s activities, occupation, sport participation, hand dominance, comorbidities, previous injuries, and prior elbow surgery.
Symptoms should be characterized according to location, duration, aggravating activities, instability, neurologic symptoms, and mechanical complaints such as locking or catching.
Physical Examination
Inspection
Both upper extremities should be exposed from the shoulder girdle to the hand.
The examiner should compare the two sides from both the anterior and posterior perspectives.
The neck, shoulder, wrist, and hand should also be evaluated because pathology in these regions may refer symptoms to the elbow.
Carrying Angle
The physiologic carrying angle is a mild valgus alignment of the forearm relative to the humerus.
Typical values are approximately 11–14° of valgus in males and 13–16° in females.
Marked asymmetry may suggest previous trauma, growth disturbance, or deformity.
Visual Assessment
The examiner should look for muscular hypertrophy or atrophy, swelling, cutaneous lesions, ecchymosis, deformity, signs of acute trauma, and scars from previous surgery.
Palpation
Digital palpation should be systematic and directed toward identifying the specific anatomic structure responsible for pain.
Important areas include the medial and lateral epicondyles, radial head, olecranon, distal biceps tendon, UCL, lateral ligament complex, cubital tunnel, and joint lines.
Range of Motion
Active and passive elbow motion should be compared bilaterally.
Normal motion is approximately 0–140° of flexion, with some individuals demonstrating up to about 10° of hyperextension.
Forearm rotation is approximately 80° of supination and 80° of pronation.
Functional Range of Motion
Most activities of daily living can be performed with approximately 30–130° of elbow flexion, together with about 50° of pronation and 50° of supination.
Loss of motion outside this functional arc may therefore be tolerated better than loss within it.
Strength Testing
Isometric strength should be assessed and compared with the contralateral side.
Elbow flexion, extension, pronation, and supination should be tested individually.
Pain during resisted testing may help localize tendon pathology.
Elbow Effusion
An elbow effusion can be palpated most readily over the lateral joint in the center of the triangle formed by the lateral epicondyle, radial head, and olecranon tip.
An effusion suggests intra-articular pathology and may be accompanied by loss of terminal extension.
Lateral Epicondylitis
Lateral epicondylitis, commonly called tennis elbow, usually results from repetitive overuse of the wrist and finger extensor mechanism.
The extensor carpi radialis brevis is particularly commonly involved.
Tenderness
Maximal tenderness is typically located just anterior and distal to the lateral epicondyle, near the origin of the extensor carpi radialis brevis.
Resisted Wrist Extension Test
With the forearm pronated, the patient attempts to extend the wrist against resistance.
Reproduction of pain over the lateral epicondyle constitutes a positive test.
Chair Test
The chair test may also provoke symptoms.
The patient attempts to lift a chair with the forearm pronated and elbow extended, reproducing pain at the lateral epicondyle.
Medial Epicondylitis
Medial epicondylitis, or golfer’s elbow, affects the common flexor-pronator origin.
Pain is localized over the medial epicondyle and may be exacerbated by resisted wrist flexion or forearm pronation.
Resisted Flexion and Pronation Test
The elbow is placed in slight flexion with the forearm initially supinated.
The patient performs resisted wrist flexion and/or pronation.
Reproduction of pain at the medial epicondyle supports the diagnosis.
Olecranon Bursitis
The olecranon bursa lies subcutaneously over the posterior olecranon.
Inflammation may result from repetitive trauma, direct injury, hemorrhage, infection, or rheumatologic disease.
Examination Findings
The patient may have localized swelling over the olecranon, with or without erythema.
Tenderness varies according to the cause.
Septic bursitis is more likely to be painful, erythematous, and warm.
Elbow Instability
Ulnar Collateral Ligament Insufficiency
The UCL is the major restraint to valgus instability.
Injury is particularly common in throwing athletes because of repetitive valgus loading.
Valgus Stress Test
The elbow is flexed to approximately 30° to reduce bony stability, and a valgus force is applied.
The examiner palpates the UCL from the medial epicondyle to its insertion on the sublime tubercle of the proximal ulna.
Increased medial joint opening or absence of a firm endpoint suggests UCL insufficiency.
Additional UCL Tests
Other useful provocative maneuvers include the milking maneuver and moving valgus stress test.
These tests may reproduce medial elbow pain or demonstrate valgus instability.
Lateral Collateral Ligament Insufficiency
Lateral ligament failure may produce posterolateral rotatory instability of the elbow.
Posterolateral Rotatory Instability Test
The patient is positioned supine with the shoulder elevated overhead.
The humerus is stabilized, and the forearm is placed in full supination.
Starting with the elbow near extension, the examiner slowly flexes the elbow while applying axial loading and a slight valgus and supination force.
Positive Test
A positive test may produce a palpable clunk, posterior prominence of the radial head, and skin dimpling proximal to the radial head as the radiocapitellar and ulnohumeral articulations transiently subluxate.
Additional Instability Tests
Other maneuvers include the chair push-up test, stand-up test, and tabletop relocation test.
These reproduce symptoms by loading the elbow in positions that provoke posterolateral instability.
Valgus Extension Overload
Valgus extension overload is most commonly seen in overhead throwing athletes.
Patients may describe a painful pop or posteromedial discomfort during throwing.
Associated Abnormalities
The condition is frequently associated with UCL insufficiency, intra-articular loose bodies, and radiocapitellar cartilage injury.
Pathomechanics
Repeated throwing produces valgus stress medially, compression of the radiocapitellar joint laterally, and posteromedial impingement of the olecranon against the medial trochlea during terminal extension.
Examination
Passive forced hyperextension may reproduce posteromedial elbow pain.
Elbow Arthritis
Elbow arthritis commonly presents with loss of extension and pain at terminal extension.
Some patients also lose flexion as disease advances.
An effusion may or may not be present.
Flexion Contracture
Both active and passive extension may be restricted, resulting in a flexion contracture.
Mechanical impingement from osteophytes may contribute to terminal-motion pain.
Cubital Tunnel Syndrome
Cubital tunnel syndrome results from compression or traction of the ulnar nerve around the elbow.
Patients commonly report medial elbow discomfort together with numbness or paresthesias involving the ring and small fingers.
Symptoms are often aggravated by prolonged elbow flexion.
Tinel Sign
Percussion over the ulnar nerve posterior to the medial epicondyle may reproduce radiating paresthesias into the ulnar forearm and hand.
This represents a positive Tinel sign.
Additional Cubital Tunnel Tests
Other provocative maneuvers include the elbow flexion test, scratch collapse test, and shoulder internal rotation test.
The ulnar nerve should also be assessed dynamically for subluxation over the medial epicondyle during flexion.
Distal Biceps Tendon Rupture
A distal biceps rupture may present with acute antecubital pain, bruising, weakness, and loss of normal tendon palpability.
Supination weakness is often particularly pronounced.
Palpation
The distal biceps tendon may be absent or difficult to palpate within the antecubital fossa.
Tenderness is common in the acute setting.
Popeye Deformity
Retraction of the biceps muscle may produce a characteristic Popeye appearance.
A similar deformity can also occur with proximal biceps tendon rupture, so the location and mechanism must be considered.
Hook Test
The hook test is a useful examination for distal biceps integrity.
With the shoulder abducted and the elbow flexed approximately 90°, the patient actively supinates the forearm.
The examiner attempts to hook a finger beneath the distal biceps tendon from the lateral side.
Interpretation
In an intact tendon, the examiner can hook beneath the taut distal biceps.
Failure to palpate or hook the tendon strongly suggests a complete distal biceps rupture.
Biceps Squeeze Test
The patient’s elbow is flexed while the forearm is relaxed.
The examiner compresses the biceps muscle belly.
With an intact tendon, squeezing the muscle produces passive forearm supination.
Failure of the forearm to supinate suggests distal tendon rupture.
Passive Pronation-Supination Test
With the elbow supported at approximately 90° of flexion, the examiner palpates the biceps while passively pronating and supinating the forearm.
An intact tendon produces visible or palpable movement of the biceps muscle belly as the radius rotates.
Loss of this normal excursion may indicate tendon disruption.
General Examination Principles
Elbow pathology frequently overlaps with disorders involving the shoulder, cervical spine, forearm, wrist, and peripheral nerves.
A complete examination should therefore integrate inspection, palpation, range of motion, strength, neurologic assessment, stability testing, and condition-specific provocative maneuvers.
Comparison with the opposite elbow is particularly useful when assessing motion, carrying angle, strength, and ligamentous laxity.
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Orthopaedic Surgery - Ehlers–Danlos Syndrome
Basics
Ehlers–Danlos syndrome (EDS) refers to a heterogeneous group of inherited connective-tissue disorders caused by abnormalities affecting collagen or collagen-related proteins.
These abnormalities produce varying degrees of joint hypermobility, skin hyperextensibility and fragility, abnormal wound healing, easy bruising, and involvement of the cardiovascular, ocular, skeletal, and other organ systems.
At least 13 recognized subtypes have been described, each with a characteristic combination of clinical manifestations, inheritance patterns, and molecular abnormalities.
The age at diagnosis ranges from infancy to adulthood, depending on the severity and phenotype.
Classification
EDS encompasses several clinically and genetically distinct disorders.
Important phenotypes include classical, classical-like, cardiac-valvular, vascular, hypermobile, arthrochalasia, dermatosparaxis, kyphoscoliotic, brittle cornea, spondylodysplastic, musculocontractural, myopathic, and periodontal EDS.
Classical EDS
Classical EDS is characterized primarily by skin hyperextensibility, generalized joint hypermobility, easy bruising, and abnormal scar formation.
Subcutaneous spheroids and hernias may also occur.
Classical-Like EDS
Classical-like EDS resembles the classical form, with prominent skin and joint manifestations, but differs in its underlying genetic abnormality and certain clinical features.
Cardiac-Valvular EDS
Cardiac-valvular EDS is characterized by progressive valvular heart disease, together with connective-tissue manifestations such as skin hyperextensibility and joint abnormalities.
Chest-wall deformities such as pectus excavatum may also occur.
Vascular EDS
Vascular EDS is one of the most serious forms.
Patients are predisposed to arterial aneurysm, dissection, and rupture, sometimes at a young age.
Spontaneous rupture of hollow organs, particularly the bowel or uterus, may also occur.
Unlike some other EDS variants, generalized joint hypermobility may be less prominent.
Hypermobile EDS
Hypermobile EDS is characterized predominantly by generalized joint hypermobility, recurrent instability or dislocation, and chronic musculoskeletal pain.
Skin manifestations are usually less severe than in classical EDS.
Arthrochalasia EDS
Arthrochalasia EDS is associated with severe generalized joint hypermobility, congenital bilateral hip dislocation, muscle hypotonia, and recurrent joint instability.
Dermatosparaxis EDS
Dermatosparaxis EDS is characterized by extreme skin fragility and easy bruising.
Patients may also demonstrate short stature, redundant skin, and umbilical or other hernias.
Kyphoscoliotic EDS
Kyphoscoliotic EDS commonly presents with congenital muscular hypotonia, early-onset progressive kyphoscoliosis, generalized joint hypermobility, and recurrent subluxation or dislocation.
Reduced bone mass and osteoporosis may also be present.
Brittle Cornea Syndrome
Brittle cornea syndrome is distinguished by marked corneal thinning and fragility, which may predispose to serious ocular complications.
Musculoskeletal hypermobility may also occur.
Spondylodysplastic EDS
Spondylodysplastic EDS may present with short stature, muscle hypotonia, skeletal dysplasia, and bowing of the limbs.
Musculocontractural EDS
Musculocontractural EDS is characterized by multiple congenital contractures, distinctive craniofacial features, and characteristic abnormalities of the skin and connective tissues.
Myopathic EDS
Myopathic EDS often presents with congenital muscle hypotonia, proximal joint contractures, and hypermobility of more distal joints.
Periodontal EDS
Periodontal EDS is characterized particularly by severe early-onset periodontal disease, together with variable joint and skin manifestations.
General Prevention
EDS itself cannot be prevented, but many serious complications can be reduced through anticipatory surveillance and modification of risk.
Particular attention should be directed toward prevention of vascular rupture, excessive bleeding, recurrent joint dislocation, skin injury, and progressive spinal deformity.
Joint Protection
Patients should avoid activities that repeatedly force joints beyond their physiologic range.
Education regarding safe movement patterns, proprioception, and muscle conditioning can reduce the frequency of instability episodes.
Cardiovascular Protection
Individuals with vascular or cardiac involvement require appropriate cardiovascular surveillance.
Early recognition of aneurysm, arterial dissection, or progressive valvular disease is especially important.
Epidemiology
EDS affects both males and females.
Overall prevalence has historically been estimated at approximately 1 in 5,000 individuals, although prevalence varies considerably according to subtype and may be underestimated because milder cases are often unrecognized.
No consistent racial predilection has been established.
Risk Factors
The principal risk factor is a positive family history of EDS or characteristic manifestations, such as marked hypermobility, vascular rupture, unusual skin fragility, or recurrent joint dislocation.
However, some patients have a new pathogenic variant and therefore lack an affected relative.
Genetics
The inheritance pattern varies according to EDS subtype.
Some forms are inherited in an autosomal dominant pattern, while others are autosomal recessive.
De novo pathogenic variants can also occur, so the absence of a family history does not exclude EDS.
Molecular Abnormalities
Different subtypes result from abnormalities in various collagen molecules or proteins involved in collagen processing and extracellular matrix organization.
For example, classical EDS is commonly related to abnormalities of type V collagen, while vascular EDS results from pathogenic variants affecting type III collagen.
Other forms may involve type I collagen or other matrix-associated proteins.
Etiology
EDS results from inherited abnormalities that interfere with the normal structure, production, processing, or organization of collagen and related connective-tissue proteins.
Because collagen is present throughout the body, manifestations can involve the skin, joints, blood vessels, eyes, gastrointestinal tract, and other organs.
Diagnosis
General Principles
The diagnosis is based on the clinical phenotype, family history, and established diagnostic criteria, with molecular testing used to confirm many EDS subtypes.
Evaluation by a medical geneticist or clinician experienced in hereditary connective-tissue disorders is often appropriate.
Signs
Common findings include hyperextensible skin, easy bruising, generalized joint hypermobility, recurrent joint instability, and spinal deformity.
Some patients can place their joints into positions far beyond the normal physiologic range.
Symptoms
Patients frequently report multiple joint pains, recurrent sprains or dislocations, and diffuse or poorly localized musculoskeletal discomfort.
Chronic pain may arise from repetitive instability and soft-tissue injury.
Physical Examination
General Inspection
Height and body proportions should be documented.
The patient should be examined for skeletal disproportions, chest-wall abnormalities, limb deformity, and other features that may suggest a particular subtype.
Joint Range of Motion
Joint motion should be measured systematically.
Particular attention should be paid to hyperextension of the fingers, elbows, and knees, as well as excessive motion at other joints.
Joint Stability
The shoulders, elbows, knees, ankles, and other symptomatic joints should be assessed for instability, recurrent subluxation, or dislocation.
Skin Examination
The examiner should assess the texture, elasticity, fragility, and extensibility of the skin.
Bruising, abnormal scars, or areas of previous wound breakdown should be noted.
Ocular Examination
Visual symptoms should prompt formal ophthalmologic evaluation.
Certain EDS-related disorders may cause corneal abnormalities or other potentially serious ocular manifestations.
Spine Examination
The patient should be examined for kyphosis, scoliosis, or other spinal deformity.
A forward-bend test can help detect rotational prominence associated with scoliosis.
Diagnostic Testing
Molecular Testing
Genetic testing is available for many, although not all, EDS subtypes.
Testing should ideally be performed through an experienced genetics service capable of interpreting variants in the context of the patient’s phenotype.
Bone Quality
Patients with EDS may have impaired bone quality even when conventional bone mineral density is relatively preserved.
Measures of trabecular bone microarchitecture may therefore provide additional information about skeletal strength in selected patients.
Imaging
Cardiovascular Imaging
Patients with subtypes associated with cardiovascular abnormalities may require periodic assessment of the heart, valves, aorta, and arterial system according to their specific phenotype and genetic diagnosis.
Spine Radiographs
Plain radiographs should be obtained when examination suggests scoliosis, kyphosis, spondylolisthesis, or another structural spinal deformity.
Vertebral Fractures
Adults with EDS may demonstrate vertebral fractures even when bone mineral density is not markedly reduced.
This supports the concept that abnormalities of bone quality, rather than density alone, may influence skeletal fragility.
Pathological Findings
Microscopic examination of connective tissue may demonstrate irregular or abnormally organized collagen fibers.
Such findings reflect the underlying structural abnormality of the extracellular matrix.
Cardiovascular Pathology
In severe vascular forms, arterial tissue may be unusually fragile and susceptible to aneurysm, dissection, or rupture.
Cardiac-valvular involvement may include myxomatous degeneration and abnormalities of the supporting chordae.
Differential Diagnosis
Marfan Syndrome
Marfan syndrome may also cause joint laxity and skeletal abnormalities.
However, it has a characteristic pattern involving long-bone overgrowth, ocular abnormalities, and cardiovascular disease, whereas EDS generally demonstrates more pronounced skin fragility and joint hypermobility.
Larsen Syndrome
Larsen syndrome may present with multiple congenital joint dislocations.
Unlike typical EDS, fixed contractures and cervical kyphosis are more characteristic.
Cutis Laxa
Cutis laxa should be considered when loose, redundant skin is the predominant manifestation.
The nature of the skin laxity and associated systemic features differ from those of EDS.
Pseudoxanthoma Elasticum
Pseudoxanthoma elasticum can also produce characteristic skin and vascular abnormalities.
Its pattern of elastic-tissue mineralization distinguishes it from EDS.
Treatment
General Principles
Management is primarily supportive and preventive because the underlying connective-tissue defect cannot currently be corrected.
Treatment should be individualized according to the patient’s EDS subtype and affected organ systems.
Specialist Care
Referral to appropriate specialists is required when cardiovascular, ophthalmic, neurologic, gastrointestinal, or other systemic complications are present.
A medical geneticist often coordinates overall care.
Joint Instability
Surgery for instability should be considered cautiously because connective-tissue laxity and impaired tissue quality can increase the likelihood of recurrent instability, fixation failure, poor wound healing, and other complications.
Operative treatment is generally reserved for severe, function-limiting instability that has failed appropriate nonoperative management.
Arthrodesis
In selected severely unstable joints, fusion may occasionally be necessary when reconstruction cannot provide reliable stability.
Activity Modification
High-impact and collision activities should generally be minimized in patients with significant instability or vascular fragility.
Exercise is still encouraged, but activities should emphasize controlled, low-impact conditioning rather than extreme stretching or high-force loading.
Protective Measures
Protective padding and bandages may reduce bruising and hematoma formation during routine activities.
Skin trauma should be minimized whenever possible.
Medications Affecting Bleeding
Medications that impair platelet function or increase bleeding risk should be used cautiously, particularly in patients with substantial bruising or vascular fragility.
The risks and benefits of NSAIDs, aspirin, or anticoagulants should be considered individually rather than used indiscriminately.
Physical Therapy
Muscle Conditioning
Strengthening the muscles surrounding unstable joints can improve dynamic stability and may reduce pain and recurrent subluxation.
Exercise programs should prioritize controlled strengthening, proprioception, balance, and joint protection.
Prevention of Dislocation
Therapists can teach patients how to avoid provocative joint positions and how to modify daily activities to reduce repetitive instability.
Flexibility
Aggressive stretching is usually unnecessary in a disorder characterized by excessive joint mobility.
Therapy should focus more on control and stability than on achieving additional range of motion.
Surgery
General Considerations
EDS can produce segmental instability, scoliosis, kyphosis, and other structural deformities that occasionally require operative management.
Surgery is technically challenging because tissues may be fragile and healing may be less predictable.
Scoliosis
Spinal fusion may be considered for severe, progressive scoliosis, historically around curves exceeding approximately 45°, particularly when progression is likely and the patient’s overall medical status permits surgery.
The precise indication depends on curve type, progression, skeletal maturity, symptoms, and EDS subtype.
Surgical Risks
Operative planning should account for increased risks of bleeding, vascular injury, wound complications, implant failure, and recurrent deformity, especially in vascular and kyphoscoliotic forms.
Activity
Regular physical activity is usually beneficial when adapted to the individual’s phenotype.
Low-impact activities, controlled strengthening, and aerobic conditioning are generally preferable to contact sports, high-impact exercise, or activities requiring extreme joint motion.
Follow-Up
Referral
A medical geneticist is often the most appropriate specialist to coordinate routine long-term care, particularly when the subtype has not yet been clearly established.
Other specialists should be involved according to the affected organ systems.
Prognosis
Prognosis varies greatly among EDS subtypes.
Many individuals have a near-normal lifespan but experience chronic musculoskeletal symptoms, while certain forms—particularly vascular EDS—carry substantial risk of life-threatening complications.
Complications
Cardiovascular Events
Arterial aneurysm, dissection, or rupture can result in sudden life-threatening hemorrhage in vascular forms of EDS.
Cardiac-valvular disease may also progress in selected subtypes.
Joint Degeneration
Repeated instability and abnormal joint loading can lead to premature osteoarthritis and chronic musculoskeletal pain.
Recurrent Dislocations
Joint hypermobility may cause recurrent subluxations or dislocations involving the shoulders, patellae, hips, fingers, and other joints.
Spinal Deformity
Progressive scoliosis or kyphosis can occur, particularly in kyphoscoliotic forms.
Severe deformity may interfere with sitting balance, pulmonary function, or mobility.
Visual Complications
Certain EDS-related disorders may cause significant ocular abnormalities, including corneal fragility and visual impairment.
Skin and Wound Problems
Patients may experience easy bruising, skin splitting, delayed wound healing, widened scars, and hematoma formation.
These problems are particularly relevant when surgical treatment is planned.
Patient Monitoring
Long-term follow-up should be individualized according to the EDS subtype.
Monitoring may include joint stability, chronic pain, spinal alignment, skin and wound problems, cardiovascular status, ocular symptoms, bone health, and functional limitations.
Patients with potentially vascular forms require especially careful surveillance and counseling regarding symptoms that may indicate an acute arterial or visceral emergency.
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Orthopaedic Surgery - Dupuytren Contracture
Basics
Dupuytren contracture is a fibroproliferative disorder of the palmar fascia characterized by the development of nodules and longitudinal cords that progressively shorten and produce flexion contractures of the fingers.
The metacarpophalangeal (MCP) and proximal interphalangeal (PIP) joints are most commonly affected, particularly in the ring and small fingers.
The condition is also known as Dupuytren disease.
Typical Age and Sex
Dupuytren disease most commonly develops in men during the fifth through seventh decades of life.
Men are affected considerably more often than women, with reported male-to-female ratios ranging from approximately 2:1 to 10:1.
Women generally develop the disease later and tend to have less severe involvement.
Early-Onset Disease
Patients who develop Dupuytren disease at a younger age often experience a more aggressive clinical course, faster progression, and higher recurrence rates following treatment.
A particularly aggressive form occurring in younger patients is referred to as Dupuytren diathesis.
Epidemiology
The prevalence in the United States has historically been estimated at approximately 2–3% of the general population.
Hand dominance does not appear to influence which hand becomes affected.
Associated Medical Conditions
The disease is more frequent or severe in patients with diabetes mellitus.
Associations have also been reported with epilepsy, chronic obstructive pulmonary disease, HIV infection, and use of certain antiseizure medications.
Links with alcohol consumption and tobacco exposure have been reported, although the strength and causal significance of these associations have been debated.
Risk Factors
Important risk factors include increasing age, male sex, Caucasian ancestry, Northern European heritage, and a positive family history.
Genetics
Dupuytren disease has a strong hereditary component.
It has traditionally been described as having an autosomal dominant pattern with variable penetrance, although the genetic basis is complex and likely involves multiple susceptibility genes.
A documented family history is present in only a minority of affected patients.
Etiology
The exact cause remains incompletely understood.
Abnormal regulation of connective-tissue formation appears to produce proliferation of fibroblasts and myofibroblasts with excessive deposition and contraction of collagen within the palmar fascia.
Associated Conditions
Diabetes Mellitus
Patients with diabetes may develop Dupuytren disease more frequently and may have more extensive involvement.
Epilepsy
An association with epilepsy has historically been reported, particularly in patients receiving long-term anticonvulsant therapy.
Alcohol Use
Alcohol misuse has been associated with Dupuytren disease in some studies, although whether alcohol itself is directly causal remains uncertain.
Chronic Pulmonary Disease and HIV
Increased prevalence has also been described in patients with chronic obstructive pulmonary disease and HIV infection.
Diagnosis
Early Disease
The disease usually begins with one or more firm nodules within the palmar fascia.
These nodules may be painless or mildly tender.
Skin Changes
Skin dimpling, puckering, or adherence may develop over or around the palmar nodules as the underlying fascia contracts.
Bilateral Disease
Approximately 45% of patients may have involvement of both hands.
However, disease severity is often asymmetric.
Progression
As Dupuytren disease progresses, the abnormal fascia extends into the fingers and forms palpable cords.
Flexion contracture typically develops first at the MCP joint, followed by involvement of the PIP joint.
Web-Space Contracture
Contracture may also involve the web spaces, limiting finger abduction and interfering with hand opening.
Knuckle Pads
Approximately 20% of patients may develop firm fibrous nodules over the dorsal aspect of the PIP joints.
These are known as Garrod pads or Garrod disease.
They are often asymptomatic but may become painful when prominent or repeatedly traumatized.
Dupuytren Diathesis
Patients with a strong Dupuytren diathesis may have fibromatosis at other sites in addition to the hand.
These manifestations include plantar fibromatosis and penile fibromatosis.
Ledderhose Disease
Plantar fibromatosis involving the plantar fascia is known as Ledderhose disease.
Peyronie Disease
Fibromatosis involving the penis is known as Peyronie disease.
The coexistence of these disorders may indicate a more aggressive fibromatosis tendency.
Physical Examination
Palmar Nodules
The palm should be inspected and palpated for firm nodules.
They may be tender during the early proliferative phase but often become painless later.
Palmar Cords
Longitudinal cords may extend from the palm into one or more fingers.
These cords become increasingly prominent as the disease progresses and are responsible for the development of joint contractures.
MCP Contracture
The MCP joint is the most commonly affected joint.
Progressive shortening of the palmar fascia prevents full finger extension.
PIP Contracture
PIP involvement is particularly important because established PIP contractures are generally more difficult to correct and more likely to recur.
Hueston Table-Top Test
The Hueston table-top test is used to assess functional contracture.
The patient attempts to place the palm and all fingers flat against a table.
The test is positive when the hand cannot be placed completely flat because one or more contracted fingers remain elevated.
Pathological Findings
Myofibroblasts
The characteristic proliferating cell is the myofibroblast.
These cells have contractile properties and are believed to play a major role in progressive shortening of the diseased fascia.
Collagen Abnormality
Dupuytren tissue demonstrates an increased proportion of type III collagen relative to type I collagen compared with normal palmar fascia.
The altered collagen organization contributes to formation of thickened cords.
Differential Diagnosis
Arthritis
Degenerative or inflammatory arthritis can produce finger stiffness and contracture but does not typically create characteristic palmar nodules and fascial cords.
Post-Traumatic Contracture
Previous fractures, tendon injuries, or prolonged immobilization may produce fixed finger contractures.
A history of trauma and absence of Dupuytren cords help distinguish these conditions.
Burn Contracture
Deep burns involving the palm may lead to scar contracture and limited extension.
The scar pattern and history generally establish the diagnosis.
Ulnar Nerve Palsy
Ulnar neuropathy can cause clawing of the ring and small fingers.
Unlike Dupuytren disease, clawing results from intrinsic muscle weakness rather than fascial shortening and is accompanied by neurologic findings.
Treatment
General Principles
Treatment depends primarily on the degree of contracture, rate of progression, symptoms, and functional impairment.
The presence of nodules alone does not necessarily require intervention.
Observation
Patients with painless nodules and no significant contracture may be observed.
Slowly progressive disease that does not interfere with function can also be followed with serial examinations.
Monitoring Progression
The degree of MCP and PIP contracture should be documented over time.
Changes in the table-top test, finger extension, and functional use of the hand help determine whether treatment is becoming necessary.
Hand Therapy
Role of Therapy
Hand therapy is used primarily as an adjunct after procedural or surgical treatment rather than as a means of reversing established fascial disease.
Goals
Therapy aims to maintain the extension obtained during treatment, restore flexion, minimize edema and scar formation, and recover functional hand use.
Splinting
A comfortable and appropriately fitted extension splint may be used after intervention.
The duration of splinting varies depending on disease severity, procedure, and postoperative motion.
Independent Exercises
Patients should be instructed in regular active and passive range-of-motion exercises.
Independent home exercises are an important part of recovery.
Medication and Nonoperative Procedures
Vitamin E and Splinting Alone
Vitamin E and long-term splinting alone have not been shown to reverse established Dupuytren contracture.
Corticosteroid Injection
Corticosteroid injection into painful early nodules has been used to reduce tenderness and possibly soften or temporarily suppress nodule progression.
It may also be used selectively for symptomatic knuckle pads.
Collagenase Injection
Collagenase clostridium histolyticum has been used as a minimally invasive treatment for palpable Dupuytren cords.
The enzyme is injected directly into the abnormal cord to weaken its collagen structure.
Manipulation After Collagenase
Approximately 24–48 hours after injection, the finger is manipulated under local anesthesia to rupture the weakened cord and improve extension.
Response by Joint
Collagenase treatment has generally been more predictable for MCP contractures than for PIP contractures.
PIP disease is more difficult to correct because of secondary joint and soft-tissue changes.
Surgery
Indications
Surgery is not indicated for stable, painless palmar nodules without meaningful contracture.
Knuckle pads rarely require operative treatment.
PIP Involvement
PIP contracture deserves particular attention because it can become increasingly resistant to correction.
Progressive contracture, functional loss, or clinically important inability to extend the finger may justify procedural or surgical intervention.
Operative Options
Procedures used to treat Dupuytren contracture include percutaneous needle aponeurotomy, open fasciotomy, limited or selective fasciectomy, more extensive fasciectomy, dermofasciectomy with skin grafting, and, rarely, amputation.
The choice depends on disease severity, recurrence, anatomy, and patient factors.
Percutaneous Needle Aponeurotomy
Needle aponeurotomy divides the pathological cord percutaneously.
It offers rapid recovery and minimal surgical trauma but generally has a higher recurrence rate than more extensive fasciectomy.
Open Fasciotomy
Open fasciotomy releases the cord through an incision without removing large amounts of diseased fascia.
Limited Fasciectomy
Limited or selective fasciectomy removes the pathological fascial cords responsible for the contracture while preserving uninvolved tissue.
This is one of the most commonly used operative approaches.
Extensive Fasciectomy
More extensive fasciectomy may be considered for severe or recurrent disease but carries increased risks of wound complications, nerve injury, stiffness, and vascular compromise.
Dermofasciectomy
In aggressive or recurrent disease, involved skin and underlying diseased fascia may be removed together.
A skin graft is then used to cover the defect.
Amputation
Amputation is rarely required and is reserved for severe, recurrent, painful, or nonfunctional digits in which repeated reconstructive procedures are unlikely to provide useful function.
Factors Affecting Procedure Selection
Treatment planning should consider the degree and location of contracture, patient age, occupation, general health, condition of the palmar skin, presence of arthritis, previous procedures, and likelihood of recurrence.
Postoperative Splinting
The frequency and duration of postoperative splinting vary according to the procedure and severity of disease.
Historically, many patients have used extension splints for several months, including nighttime splinting during later recovery.
Return to Activity
Return to normal use depends on the treatment performed and wound healing.
After open surgery, substantial recovery of hand function is often expected within approximately 2–3 months, although more extensive disease may require longer rehabilitation.
Follow-Up
Prognosis
Approximately 80% of patients undergoing primary surgery may regain near-full flexion and extension, particularly when severe fixed PIP deformity is absent.
Aggressive Disease
Young male patients with a strong family history and other features of Dupuytren diathesis are more likely to experience rapid progression and recurrent contracture.
Associated Conditions and Severity
Patients with diabetes, epilepsy, or heavy alcohol exposure have historically been reported to develop more severe disease.
Recurrence
Recurrence rates vary widely according to disease biology, procedure, duration of follow-up, and definition of recurrence.
Published long-term recurrence rates have ranged from approximately 26–80%.
Need for Repeat Procedures
Many patients with recurrent disease do not require another operation.
Repeated procedures are most often necessary in younger patients with an aggressive Dupuytren diathesis.
Complications
Joint Stiffness
Finger stiffness is an important postoperative complication.
Early controlled motion, hand therapy, and patient education can reduce this risk.
Nerve Injury
Digital nerve injury may occur during surgery because the neurovascular bundles can be displaced or wrapped by diseased cords.
Historical rates of nerve injury have been approximately 1–3%, with greater risk during revision surgery.
Recurrence
Recurrence remains the major long-term problem.
Substantial recurrent disease may develop within 5–10 years, particularly in patients with aggressive disease.
Wound Problems
Skin necrosis, delayed wound healing, hematoma, infection, and scar sensitivity may occur following open fasciectomy, especially in severe or recurrent disease.
Vascular Injury
Digital vessels may be injured during difficult dissection, particularly when longstanding contracture has distorted normal anatomy.
Complex Regional Pain Syndrome
A small proportion of patients may develop disproportionate pain, swelling, stiffness, and autonomic changes consistent with complex regional pain syndrome.
Patient Monitoring
Patients undergoing surgery should initially be followed closely, often weekly during the first postoperative month, to monitor wound healing and identify stiffness, infection, or neurovascular problems.
After healing is established, follow-up may be performed as clinically needed.
Long-term reassessment should document finger extension, MCP and PIP contracture, recurrence of cords, hand function, and the need for additional treatment.
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Orthopaedic Surgery - Discoid Meniscus
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Basics
A discoid meniscus is an abnormally broad, thickened, disc- or pancake-shaped meniscus rather than the normal semilunar structure.
It most commonly involves the lateral meniscus, while medial discoid menisci are much less common.
The condition may produce popping, snapping, locking, or pain during childhood or early adulthood, although many individuals remain asymptomatic.
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Laterality
Approximately 75% of cases are unilateral, while around 25% are bilateral.
Because bilateral involvement is possible, the contralateral knee may also require evaluation if symptoms develop.
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Watanabe Classification
Discoid lateral menisci are traditionally divided into three types according to the Watanabe classification.
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Type 1: Complete
A complete discoid meniscus covers essentially the entire lateral tibial plateau.
The meniscus is markedly wider than normal and forms a nearly complete disc over the lateral compartment.
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Type 2: Incomplete
An incomplete discoid meniscus is larger and broader than a normal meniscus but does not completely cover the lateral tibial plateau.
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Type 3: Wrisberg Variant
The Wrisberg-type discoid meniscus has a thickened posterior horn and lacks the normal posterior meniscotibial attachment to the tibia.
Because of this absent attachment, the meniscus is excessively mobile and unstable.
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Epidemiology
The reported incidence is approximately 3–5% in the United States, although prevalence is considerably higher in some Asian populations and has been reported at more than 20% in Japan.
The true incidence is probably higher than recognized because many affected individuals never develop symptoms.
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Etiology
A discoid meniscus is considered a developmental or anatomic abnormality of the meniscus.
Genetic or familial influences may also contribute, although the exact mechanism remains uncertain.
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Diagnosis
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Clinical Presentation
Presentation varies considerably depending on the type of discoid meniscus, its stability, and whether a tear is present.
Some patients are entirely asymptomatic and the abnormality is discovered incidentally.
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Snapping Knee Syndrome
The classic presentation is the so-called snapping knee syndrome, characterized by a palpable or audible snap or clunk near the terminal limits of knee flexion or extension.
This is particularly associated with the unstable Wrisberg variant.
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Snapping in Other Variants
Snapping may also occur in an otherwise stable discoid meniscus if it becomes torn or develops secondary instability.
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Pain and Mechanical Symptoms
Symptomatic patients may report pain, clicking, popping, swelling, locking, or intermittent inability to fully move the knee.
Mechanical symptoms are especially suggestive of an associated tear or unstable meniscal tissue.
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Onset
Symptoms commonly begin gradually without a specific injury.
When symptoms appear suddenly after trauma, an acute tear of the discoid meniscus should be suspected.
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Physical Examination
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Effusion
A knee joint effusion may be present, particularly when the discoid meniscus has torn.
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Anterolateral Bulge
With the knee in full flexion, a visible or palpable anterolateral bulge may occasionally be present because of the increased meniscal tissue.
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Joint-Line Tenderness
Tenderness may be present along the lateral joint line.
It is often mild in an intact discoid meniscus but may become more pronounced when a tear is present.
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Mechanical Click
A pop or click may be appreciated during meniscal provocative testing, particularly the McMurray maneuver.
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Loss of Extension
Some patients have a mechanical block to full knee extension.
Others may show apprehension or discomfort as the knee approaches complete extension.
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McMurray Test
The McMurray test is performed by taking the knee into maximal flexion, applying rotation, and progressively extending the knee.
A positive test consists of pain, clicking, or popping along the lateral joint line.
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Imaging
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Plain Radiographs
Radiographs are not diagnostic in every case, but AP, lateral, tunnel, and skyline views may reveal secondary skeletal features associated with a discoid lateral meniscus.
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Radiographic Findings
Possible findings include widening of the lateral joint space, lateral joint-line lipping, cupping of the lateral tibial plateau, flattening or squaring of the lateral femoral condyle, hypoplasia of the tibial eminence, and elevation of the fibular head.
These findings may suggest the diagnosis but are not always present.
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MRI
MRI is the preferred imaging study for confirming the morphology of the meniscus and identifying associated tears or instability.
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Sagittal MRI Findings
A discoid meniscus is suggested when continuity between the anterior and posterior horns is visible on three or more consecutive 5-mm sagittal images.
This reflects the abnormal width of the meniscus.
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Coronal MRI Findings
On coronal imaging, the meniscus may demonstrate a broad block-like or bow-tie appearance, with increased meniscal width across the lateral compartment.
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Pathological Findings
The affected meniscus is disc-shaped rather than semilunar.
Histologically, discoid menisci demonstrate reduced collagen content and abnormal orientation of collagen fibers, which may contribute to their increased susceptibility to tearing.
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Stable Variant
A stable discoid meniscus retains normal peripheral and tibial ligamentous attachments.
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Unstable Variant
The unstable variant lacks normal posterolateral meniscotibial attachments, producing excessive mobility of the posterior meniscus.
This is characteristic of the Wrisberg type.
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Differential Diagnosis
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Acute Meniscal Tear
A normal-shaped meniscus with an acute tear can produce similar pain, clicking, locking, and joint-line tenderness.
MRI helps distinguish the underlying anatomy.
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Meniscal Cyst
A meniscal cyst may cause joint-line swelling and pain and is often associated with a meniscal tear.
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Osteochondritis Dissecans
Osteochondritis dissecans may cause knee pain, swelling, catching, or locking and should be considered in younger patients with mechanical symptoms.
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Osteochondral Fracture
An osteochondral fracture can produce acute pain and mechanical blockage, especially after trauma.
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Physeal Fracture
In skeletally immature patients, physeal injury may mimic internal derangement of the knee.
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Tibial Eminence Fracture
A fracture of the tibial eminence can produce pain, swelling, and extension loss and may resemble an ACL-associated injury.
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Anterior Cruciate Ligament Tear
ACL injury may produce swelling, instability, and mechanical symptoms and should be considered particularly after acute trauma.
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Treatment
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General Principles
Many discoid menisci are asymptomatic and require no treatment.
Observation is appropriate when the patient has no pain, locking, swelling, or functional impairment.
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Symptomatic Disease
Patients with persistent pain, locking, swelling, popping, or instability may require surgical treatment.
The operative strategy depends on whether the meniscus is torn and whether it is mechanically stable.
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Nonoperative Treatment
Nonoperative management has a limited role in a persistently symptomatic discoid meniscus.
A short period of immobilization or activity modification may be attempted in patients with acute-onset symptoms, particularly when the diagnosis remains uncertain.
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Return to Activity
Patients may gradually resume normal activities once symptoms have resolved and full motion and function have returned.
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Surgery
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General Surgical Principle
Modern treatment emphasizes preservation of as much functional meniscal tissue as possible.
Total meniscectomy should be avoided whenever feasible because removal of the entire meniscus substantially increases the risk of later osteoarthritis.
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Stable Discoid Meniscus
For a symptomatic stable discoid meniscus, the preferred procedure is arthroscopic partial meniscectomy with saucerization.
Excess central meniscal tissue is removed to recreate a more normal semilunar shape while preserving a stable peripheral rim.
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Meniscal Repair
If a meniscal tear or unstable peripheral attachment is present, repair may be performed at the same time as saucerization.
Preservation of the peripheral meniscus helps maintain normal load transmission across the knee.
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Importance of Meniscal Preservation
Maintaining a functional meniscus allows continued shock absorption, load distribution, and joint stabilization.
This may decrease the risk or delay the development of degenerative osteoarthritis compared with complete meniscectomy.
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Unstable Discoid Meniscus
An unstable meniscus is generally treated with arthroscopic saucerization and stabilization of the remaining peripheral meniscus.
If the posterior attachment is deficient, it may be reattached to create a stable and functional meniscal rim.
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Complete Meniscectomy
Complete meniscectomy is reserved for situations in which the meniscus is not salvageable.
Possible indications include extensive degenerative change, a massive irreparable tear, or severely abnormal meniscal tissue that cannot be reconstructed.
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Follow-Up
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Prognosis
Patients with a stable discoid meniscus generally have good outcomes following appropriately performed saucerization.
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Effect of Age
Younger patients often have better postoperative results than older patients, particularly when the meniscal tissue can be preserved before substantial degenerative change develops.
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Extent of Meniscal Resection
Removal of a larger amount of meniscal tissue is associated with a greater subsequent risk of osteoarthritis.
For this reason, preservation of a stable peripheral rim is preferred whenever technically possible.
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Partial Versus Total Meniscectomy
Partial meniscectomy and saucerization generally produce better clinical and radiographic outcomes than total meniscectomy at both short- and long-term follow-up.
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Complications
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Osteoarthritis
The major long-term complication after excessive meniscal resection is degenerative osteoarthritis of the involved compartment.
This risk is particularly high after complete meniscectomy.
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Recurrent Meniscal Tear
Even after saucerization, the remaining meniscus retains abnormal tissue architecture.
Therefore, the residual discoid meniscus remains more susceptible to future tearing than a normal meniscus.
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Persistent or Recurrent Symptoms
Pain, popping, or locking may recur if residual unstable tissue or a new tear develops.
Repeat arthroscopic evaluation may occasionally be required.
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Patient Monitoring
Patients should be followed until pain, swelling, locking, and mechanical symptoms have resolved and knee motion has returned satisfactorily.
After surgery, follow-up should also assess meniscal stability, range of motion, return to activity, recurrent tearing, and the development of degenerative changes.
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Orthopaedic Surgery - Discitis
Basics
Discitis is an infection involving the intervertebral disc space and adjacent vertebral endplates.
The infection may occur through hematogenous spread or after direct inoculation during a spinal procedure.
Although the lower lumbar spine is affected most often, discitis can involve any intervertebral level.
Classification
Discitis may be classified as spontaneous or hematogenous, in which infection reaches the disc through the bloodstream, or iatrogenic, occurring after procedures such as discectomy or discography.
The infecting process may be pyogenic, granulomatous, or, rarely, parasitic.
Pyogenic Infection
Pyogenic discitis is most commonly caused by Staphylococcus aureus.
Other organisms become more important in immunocompromised patients, intravenous drug users, and patients exposed to healthcare-associated infections.
Granulomatous Infection
Granulomatous disc infection may occur with tuberculosis and other chronic infectious processes.
These infections often have a slower course and may cause more extensive adjacent vertebral destruction.
Epidemiology
Hematogenous discitis is uncommon.
The mean age of spontaneous childhood discitis has historically been reported at approximately 7 years, although the condition can occur at any age.
Incidence
The reported incidence of discitis and related spinal infection is approximately 4–24 cases per million people per year.
Risk Factors
Important risk factors include diabetes mellitus, alcohol misuse, organ transplantation, immunosuppression, intravenous drug use, and procedures that enter or approach the disc space.
Examples of iatrogenic risk include discography, discectomy, and spinal procedures.
Etiology
The causative organism varies according to age and host factors.
Staphylococcal species are the most common pathogens in routine pyogenic infection.
In compromised hosts and intravenous drug users, gram-negative aerobic organisms and fungal pathogens such as Candida become more important.
Kingella kingae
In young children, particularly between approximately 6 months and 4 years of age, Kingella kingae is an important cause of osteoarticular infection and may be responsible for discitis.
Pediatric Vascularity
Young children are particularly susceptible because the disc and adjacent endplate region have a richer blood supply than in adults.
Until approximately 8 years of age, vessels may cross the cartilaginous endplate from the adjacent vertebral body, facilitating hematogenous spread into the disc region.
Associated Conditions
Discitis may coexist with or progress into vertebral osteomyelitis.
In advanced disease, the distinction between disc infection and adjacent vertebral infection may become less clear because both structures can be involved.
Diagnosis
General Principles
The diagnosis is established through a combination of clinical findings, laboratory studies, and imaging.
No single test is sufficient in every case.
Signs and Symptoms
The most common symptom is back pain, usually insidious in onset and progressively worsening.
Children may present less specifically, with abdominal pain, reduced appetite, irritability, malaise, or refusal to walk.
Back Stiffness
Spinal stiffness is common.
Patients may avoid flexing the spine because movement increases pain.
Refusal to Walk
Young children may refuse to stand, walk, or sit normally even when they cannot clearly localize their pain.
This can be an important presenting feature.
Pain With Spinal Percussion
Tenderness or pain with percussion over the affected spinal level may be present.
Loss of Lordosis
Lumbar discitis may cause flattening of the normal lumbar lordosis because of protective muscle spasm.
Fever
Fever is often low grade and may be absent.
The lack of fever does not exclude discitis.
Physical Examination
Lumbar Alignment
The examiner should determine whether normal lumbar lordosis is maintained or reduced.
Loss of lordosis may reflect pain and paraspinal muscle spasm.
Forward Flexion
Patients may have pain or refuse to bend forward.
This is often one of the most useful functional signs during examination.
Paraspinal Percussion
Gentle percussion over the spine may reproduce focal pain.
Abdominal Examination
Lumbar discitis can occasionally present with abdominal pain.
The abdomen should therefore be palpated, particularly in children with poorly localized symptoms.
Neurologic Examination
The neurologic examination is usually normal.
Neurologic deficits are more concerning for advanced infection, epidural involvement, abscess, or neural compression.
Laboratory Tests
White Blood Cell Count
The white blood cell count may be mildly elevated but can remain normal.
A normal count therefore does not exclude infection.
ESR and C-Reactive Protein
The erythrocyte sedimentation rate and C-reactive protein are commonly elevated, but the rise may be modest.
Either marker can occasionally be normal, particularly early in the disease course.
Blood Cultures
Blood cultures should be obtained when discitis is suspected.
However, they are positive in fewer than approximately 30% of cases in some series.
Biopsy
Direct biopsy with microbiologic analysis provides the most definitive method of identifying the causative organism.
Biopsy is particularly useful in immunocompromised patients, atypical infections, or cases that fail to respond to empiric treatment.
Imaging
Plain Radiographs
Plain radiographs are often normal early in the disease.
Abnormalities may not become visible until several weeks after symptoms begin.
Radiographic Findings
Later findings include disc-space narrowing, irregularity of the vertebral endplates, and mild adjacent osseous destruction.
MRI
MRI is the preferred imaging modality for suspected discitis because it can demonstrate infection before changes become visible on radiographs.
It provides excellent anatomic detail of the disc, endplates, vertebral marrow, epidural space, and surrounding soft tissues.
Bone Scan
Bone scintigraphy can be used when MRI is unavailable or contraindicated.
However, degenerative changes may cause false-positive findings, and MRI generally provides superior anatomic information.
FDG-PET
FDG-PET may help distinguish spinal infection from degenerative abnormalities in selected cases.
Its use is generally reserved for situations in which conventional imaging is inconclusive.
Pathological Findings
Discitis produces chronic inflammatory change with destruction of the intervertebral disc and adjacent endplates.
As infection progresses, the distinction between discitis and vertebral osteomyelitis may become less pronounced.
Differential Diagnosis
Tuberculous Spondylodiscitis
Tuberculosis should be considered, particularly when there is extensive vertebral body destruction, paraspinal involvement, or a more indolent clinical course.
Tuberculous disease often produces greater bony destruction than routine pyogenic discitis.
Vertebral Osteomyelitis
Vertebral osteomyelitis primarily affects the bone, whereas discitis begins in the disc and adjacent endplates.
In practice, the two processes frequently overlap.
Treatment
General Principles
Treatment usually consists of appropriate antibiotic therapy for approximately 6–8 weeks.
Surgery is reserved for selected patients with neurologic compression, severe pain, major bone destruction, abscess requiring drainage, instability, or failure of medical treatment.
Rest
Relative rest may be helpful during the painful early phase.
Immobilization
A brace may be used when pain is significant or when additional support is needed.
Prolonged unnecessary immobilization should be avoided once symptoms begin to improve.
Antibiotic Therapy
Antibiotics should target the most likely organism initially and then be adjusted if culture results identify a specific pathogen.
The route of therapy depends on illness severity and patient age.
Childhood Spontaneous Discitis
In otherwise healthy children with typical spontaneous discitis, empiric treatment directed against common pathogens is often successful.
Routine biopsy or operative debridement is not always required when the clinical picture is classic and the child responds appropriately.
Intravenous Versus Oral Therapy
Severely ill patients generally require intravenous antibiotics initially.
Mildly affected patients may be treated orally in selected circumstances, with close follow-up.
Compromised Host
In immunocompromised patients or those with atypical risk factors, biopsy and drainage are more strongly indicated because unusual organisms are more likely.
Physical Therapy
Physical therapy is generally not required during the acute painful phase.
In adults with persistent stiffness after infection control has begun, therapy may help restore spinal mobility, strength, and function.
Medication
Routine Antibiotic Coverage
Historically, agents such as oxacillin, dicloxacillin, or cephalosporins have been used for susceptible staphylococcal infection.
Current empiric therapy should reflect local resistance patterns and patient-specific risk factors.
MRSA Coverage
Because methicillin-resistant Staphylococcus aureus (MRSA) is an important pathogen in both hospital and community settings, vancomycin or another appropriate anti-MRSA agent may be required, particularly in severe cases.
Broad-Spectrum Coverage
Complicated infections and infections in compromised hosts may require broader coverage against gram-negative and anaerobic organisms, depending on the clinical scenario.
Pain Control
NSAIDs or short-term mild opioid analgesics may be used for severe pain during the acute phase until the infection comes under control.
Surgery
Indications for Biopsy
Biopsy is especially appropriate in the immunocompromised patient, the patient with atypical infection, or the patient who fails to improve with empiric medical therapy.
Biopsy Approach
Biopsy can be performed through an anterolateral or posterolateral route with imaging guidance.
Percutaneous image-guided techniques are commonly used when feasible.
Drainage and Debridement
Patients who fail to improve with medical treatment or who develop abscess, neurologic compression, or substantial tissue destruction may require surgical drainage and debridement.
Anterior Approach
An anterior approach may provide direct access to the involved disc space and vertebral endplates for decompression and debridement in selected cases.
Reconstruction
Adults with extensive destruction of the disc space or vertebral endplates may require spinal reconstruction to restore stability and alignment.
Minimally Invasive Techniques
Endoscopic or minimally invasive discectomy and drainage may be used in selected adults.
These techniques are less commonly required in children.
Follow-Up
Prognosis
The overall prognosis is generally good once the infection is successfully eradicated.
Children usually recover particularly well.
Childhood Outcome
After childhood discitis, the vertebrae adjacent to the infected disc may undergo spontaneous, painless fusion.
This usually does not cause major long-term functional problems.
Adult Outcome
In adults, spontaneous fusion is less reliable.
Residual chronic back pain or stiffness may persist even after the infection has resolved.
Complications
Persistent Infection
Failure to improve clinically within approximately 1–2 weeks should raise concern for an incorrect organism, inadequate antimicrobial coverage, abscess formation, resistant infection, or the need for debridement.
Neurologic Complications
Although uncommon, advanced infection can cause epidural extension, neural compression, or neurologic deficit.
These findings require urgent evaluation.
Structural Destruction
Severe infection can produce substantial endplate and vertebral destruction, resulting in deformity or instability.
Patient Monitoring
Clinical examination is one of the most useful methods for following recovery.
The examiner should monitor pain, tenderness to percussion, spinal flexibility, gait, and general function.
Laboratory and Radiographic Follow-Up
ESR and radiographic changes often lag behind clinical improvement.
Persistent radiographic abnormalities do not necessarily indicate treatment failure if symptoms and inflammatory markers are improving.
MRI or repeat imaging should be reserved for patients whose clinical course is atypical or worsening.
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Orthopaedic Surgery - Developmental Dysplasia of the Hip
Basics
Developmental dysplasia of the hip (DDH) describes a spectrum of abnormal development and instability of the hip joint, ranging from mild acetabular dysplasia and subluxation to complete dislocation of the femoral head.
The femoral head tends to migrate superolaterally, while the acetabulum becomes progressively shallow and deformed.
Most abnormalities develop in utero, although instability may occasionally evolve during early infancy.
Clinical Presentation Across Age Groups
DDH is usually identified during routine newborn or early-childhood screening.
Occasionally, a mild subluxation or acetabular dysplasia is missed during infancy and first becomes apparent during adolescence or early adulthood because of limping, activity-related discomfort, or hip pain.
Classification by Severity
The condition may be classified according to the degree of hip instability.
A subluxable hip is normally reduced but can be partially displaced from the acetabulum with gentle provocative pressure and spontaneously returns to its reduced position.
A dislocatable hip can be completely displaced from the acetabulum during examination and subsequently reduced.
A dislocated hip remains outside the acetabulum at rest and requires a specific reduction maneuver to relocate the femoral head.
Barlow and Ortolani Findings
The Barlow maneuver assesses whether a located hip can be displaced posteriorly from the acetabulum.
A clearly dislocatable hip represents a positive Barlow test.
The Ortolani maneuver assesses whether an already dislocated hip can be reduced into the acetabulum.
A palpable reduction clunk represents a positive Ortolani sign.
Synonyms
Older terminology includes congenital dislocation of the hip, although developmental dysplasia of the hip is preferred because the disorder includes a much broader spectrum than complete congenital dislocation.
Other terms include hip dysplasia and unstable hip.
General Prevention
There is no reliable method for preventing DDH completely.
The most important strategy is early detection, because treatment becomes more difficult and the risk of complications increases as the child gets older.
Children born to parents with hip dysplasia should be examined particularly carefully.
Epidemiology
Females are affected approximately four times more often than males.
This difference is thought to relate partly to greater ligamentous laxity and hormonal influences.
Incidence
When all degrees of neonatal hip instability are included, DDH occurs in approximately 1 in 200 births.
Complete dislocation is less common, occurring in roughly 1 in 1,000 births.
Risk Factors
Important risk factors include breech presentation, first-born status, female sex, oligohydramnios, and a positive family history.
Connective-tissue disorders associated with ligamentous laxity, such as Ehlers–Danlos syndrome and Marfan syndrome, may also increase risk.
Breech Presentation
Breech positioning is one of the strongest recognized risk factors.
The position increases hamstring tension and alters the mechanical forces acting across the developing hip.
First-Born Status
First-born infants may have less available intrauterine space because of greater uterine and abdominal wall tone, increasing the effect of mechanical constraint.
Oligohydramnios
Reduced amniotic fluid limits fetal movement.
Persistent hip adduction can direct the femoral head toward the edge of the acetabulum and interfere with normal acetabular development.
Genetics
A positive family history substantially increases risk, but DDH does not follow a simple established Mendelian inheritance pattern.
The disorder is generally considered multifactorial, with both genetic susceptibility and mechanical factors contributing.
Etiology
DDH is thought to result from unfavorable mechanical forces acting on a susceptible hip during fetal development.
Persistent adduction or abnormal positioning of the femoral head reduces normal concentric contact between the femoral head and acetabulum.
Because normal acetabular development depends on the femoral head remaining well centered, prolonged displacement leads to increasing dysplasia.
Severity and Timing
The earlier abnormal mechanical forces develop during gestation, the more severe the resulting hip dysplasia may be.
Severe early displacement can result in substantial deformity of both the femoral head and acetabulum.
Ligamentous Laxity
Generalized ligamentous laxity can increase hip instability.
This may partly explain the greater frequency in females and the tendency for DDH to cluster within families.
Postnatal Factors
Postnatal positioning can also influence hip stability.
Persistent adduction, a contralateral abduction contracture, or tight swaddling with the hips extended and adducted may interfere with spontaneous stabilization.
Associated Conditions
DDH may occur with other so-called packaging disorders caused by limited intrauterine space.
These include muscular torticollis and metatarsus adductus.
Syndromic Associations
Hip dysplasia may also be seen in genetic disorders, particularly connective-tissue diseases and skeletal dysplasias.
The presence of associated congenital abnormalities should increase clinical suspicion.
Diagnosis
Signs and Symptoms in Infancy
Infants with DDH are usually asymptomatic.
The diagnosis during the first months of life depends primarily on careful physical examination and, when indicated, imaging.
Hip Instability
The most important early finding is a palpable clunk as the femoral head moves out of or back into the acetabulum during the Barlow or Ortolani maneuver.
This should be distinguished from a benign soft-tissue click.
Hip Position
The affected hip may rest in slight adduction.
A deeper proximal thigh crease can sometimes be present, although skin-crease asymmetry alone is neither sensitive nor specific.
Limited Abduction
Reduced hip abduction is an important finding.
The affected hip may abduct less than approximately 50–60°, depending on age and examination technique.
Parents may first notice this limitation while changing diapers.
Hip Click
An isolated hip click without a true clunk is usually nonspecific.
Clicks may arise from the fascia lata, synovial folds, or even structures around the knee and may occur in completely normal infants.
Loss of Instability Clunk With Age
By approximately 6 months of age, the classic Barlow and Ortolani signs may disappear because a chronic dislocation becomes more fixed.
At this stage, limited abduction becomes increasingly important diagnostically.
Walking Development
A child with DDH may begin walking at the expected age or only slightly later.
Therefore, normal timing of walking does not exclude hip dysplasia.
Findings After Walking Age
Once walking begins, asymmetry may become more obvious.
The affected thigh may appear smaller, proximal thigh creases may become more pronounced, and leg-length discrepancy may be evident.
Toe Walking
A child with unilateral dislocation may appear to toe walk on the affected side as compensation for limb shortening.
Trendelenburg Gait
Weakness of the hip abductor mechanism can produce a Trendelenburg limp.
When the child stands on the affected side, the pelvis drops on the opposite side because the displaced hip provides a poor mechanical lever arm for the abductors.
Bilateral DDH
When both hips are dislocated, limb-length asymmetry may be less obvious.
The child may instead develop a waddling gait and increased lumbar lordosis.
Pain
Pain is generally absent during childhood.
Symptoms usually appear only after secondary cartilage degeneration and osteoarthritis develop, potentially beginning in late adolescence but often much later.
Physical Examination
Hip Abduction
Hip abduction should be assessed carefully in every infant.
Asymmetric or restricted abduction is one of the most useful clinical signs of DDH across age groups.
Barlow Test
The infant should be warm, calm, and relaxed.
With one hip examined at a time, the hip is flexed and gently adducted while posteriorly directed pressure is applied through the knee or thigh.
A positive test occurs when the femoral head can be partially or completely displaced from the acetabulum.
Ortolani Test
After the Barlow maneuver, the hip is gently abducted while the examiner lifts the greater trochanter anteriorly.
A palpable clunk as the femoral head reduces into the acetabulum indicates a positive Ortolani sign.
Examination Technique
The examination should be gentle.
Forceful manipulation should be avoided because aggressive testing can cause discomfort and makes the infant less relaxed, reducing examination reliability.
Galeazzi or Allis Sign
With the infant supine, hips and knees are flexed and the feet placed symmetrically.
A difference in knee height indicates apparent femoral shortening and suggests unilateral hip dislocation.
This is known as the Galeazzi or Allis sign.
Gait Examination
Walking children should be observed for limping, Trendelenburg gait, toe walking, waddling, and excessive lumbar lordosis.
Imaging
Ultrasound
Ultrasound is the preferred imaging modality during the first several months of life, before sufficient ossification of the femoral head makes radiographs more informative.
It is particularly indicated when the physical examination is abnormal or important risk factors are present.
Timing of Ultrasound
Ultrasound is most useful during the first approximately 6 months of life.
Interpretation requires experience because neonatal hip anatomy is largely cartilaginous.
Plain Radiographs
Plain radiographs become increasingly useful after approximately 4–6 months, as the femoral head and acetabulum become more readily evaluated radiographically.
Radiographic Assessment
Radiographs should assess both the shape of the acetabulum and the position of the proximal femur relative to it.
Acetabular shallowness, lateral migration, and proximal displacement may all be present.
Shenton Line
The Shenton line is a curved line formed by the medial femoral neck and the superior pubic ramus.
In a normally located hip it forms a smooth continuous arc.
Disruption suggests displacement of the proximal femur.
Femoral Head Position
The proximal femoral epiphysis should lie medial to the outer margin of the acetabulum.
Lateral or superior displacement supports the diagnosis of subluxation or dislocation.
Arthrography
Arthrography can be used during reduction procedures to define the cartilaginous femoral head, acetabulum, and structures that may block reduction.
A small residual space between the femoral head and acetabulum following reduction suggests a better-quality reduction.
CT and MRI
CT and MRI have limited roles in routine screening and diagnosis.
They may be useful in selected cases, particularly after reduction when clarification of hip position is necessary or when complex anatomy must be assessed.
Pathological Findings
Acetabular Changes
The acetabulum becomes shallow and flattened, particularly posterosuperiorly, when the femoral head is not concentrically reduced.
Femoral Head Changes
The femoral head may become flattened anteriorly, and femoral anteversion may increase.
These abnormalities become more pronounced with longstanding displacement.
Degenerative Changes
Chronic dysplasia causes abnormal joint loading.
Cartilage degeneration and secondary osteoarthritis can develop beginning in the second decade of life or later.
Differential Diagnosis
Benign Soft-Tissue Click
A simple hip or knee click caused by fascia or synovial tissue should not be confused with a true instability clunk.
Neuromuscular Hip Dysplasia
Children with cerebral palsy or spina bifida may develop hip subluxation or dislocation because of abnormal muscle forces.
This is a different mechanism from classic developmental dysplasia.
Congenital Femoral Abnormalities
Congenital femoral shortening and coxa vara can produce limb-length discrepancy or altered gait while the femoral head remains located within the acetabulum.
Treatment
General Principles
The goal of treatment is to obtain and maintain a concentrically reduced hip early enough to permit normal development of the acetabulum and proximal femur.
Earlier treatment is generally easier, safer, and more successful.
Early Reduction
When a true dislocation is identified in early infancy, reduction should be achieved promptly, ideally within the first several weeks of life.
Delay allows soft tissues to contract and acetabular deformity to progress.
Treatment by Age
Management varies substantially according to the child’s age and the severity and stability of the hip.
Newborn Management
Mild Instability
A newborn with a mild click or transient subluxability may initially undergo serial examinations, because some unstable neonatal hips stabilize spontaneously during the first days or weeks of life.
A true dislocation or persistent instability requires treatment.
Birth to 6 Months
Pavlik Harness
Persistent instability or a dislocated hip in a young infant is commonly treated with a Pavlik harness or similar dynamic abduction brace.
The harness holds the hips flexed and abducted while allowing controlled movement.
Hip Position
Hip flexion is usually maintained beyond approximately 90°, while excessive forced abduction is avoided.
The goal is to guide the femoral head gently into the acetabulum without compromising its blood supply.
Specialist Supervision
Pavlik harness treatment should be supervised by an orthopaedic surgeon experienced in pediatric hip disorders.
Improper positioning can cause complications.
Confirmation of Reduction
The hip should become reduced within the first few weeks of harness treatment.
Reduction is confirmed using ultrasound or appropriate radiographic imaging.
If the hip remains unreduced, prolonged ineffective harness treatment should be avoided.
Duration of Bracing
The brace is typically worn full-time until the hip becomes clinically and radiographically stable.
It may then be used part-time until acetabular development has normalized sufficiently.
After 6 Months
Closed or Open Reduction
After approximately 6 months of age, treatment frequently requires closed reduction under anesthesia, with open reduction performed when a stable concentric reduction cannot be achieved.
Spica Casting
Following successful reduction, the hip is usually maintained in a hip spica cast for a period of several months.
Age 6–24 Months
Traction
Some surgeons historically use a period of skin traction before reduction to gradually stretch contracted soft tissues.
Its use varies by treatment protocol.
Reduction
Closed reduction is attempted when feasible.
If soft-tissue structures prevent stable reduction, an open procedure is required.
Post-Reduction Immobilization
After reduction, the hip may be maintained in a spica cast for approximately 3–6 months, with cast changes and imaging as required.
Older Than 24 Months
Open Reduction
Older children more commonly require open reduction because the capsule, muscles, and intra-articular structures have become contracted and dysplastic.
Femoral Osteotomy
A femoral osteotomy may be performed to shorten, derotate, or realign the proximal femur.
Shortening can decrease excessive pressure on the reduced femoral head and may reduce the risk of avascular necrosis.
Pelvic Osteotomy
An iliac or other pelvic osteotomy may be needed to improve acetabular coverage of the femoral head.
The choice depends on age and the specific acetabular deformity.
Adductor Tenotomy
An adductor tenotomy may be performed when tight adductor muscles limit the safe range of hip abduction after reduction.
The goal is to enlarge the stable zone in which the hip remains reduced without excessive pressure.
Age and Surgical Outcome
The results of surgical reduction generally become less predictable as the child gets older.
Nevertheless, reduction may still provide worthwhile functional improvement in selected children up to approximately 6–8 years of age.
Physical Therapy
Routine physical therapy is usually limited.
Young children generally regain strength and hip motion naturally after reduction and immobilization.
Therapy may be used selectively when stiffness, weakness, or gait abnormalities persist.
Follow-Up
Long-Term Surveillance
Children treated for DDH require follow-up until skeletal maturity.
Even after successful reduction, acetabular and femoral development may remain abnormal.
Residual Dysplasia
Approximately 10–25% of successfully reduced hips may fail to remodel completely.
Persistent acetabular or proximal femoral dysplasia may eventually require an osteotomy.
Prognosis
Early Treatment
When DDH is identified and treated successfully during infancy, the hip may develop nearly normally and long-term function can be excellent.
Untreated Complete Dislocation
Untreated complete dislocation results in abnormal hip mechanics and usually produces a permanent waddling or Trendelenburg gait.
Pain and degenerative change frequently develop by approximately 30–50 years of age, sometimes earlier.
Subluxated Hip
A persistently subluxated hip may develop symptomatic osteoarthritis earlier than a completely dislocated hip because the remaining articular surface is exposed to highly concentrated abnormal loading.
Complications
Femoral Nerve Palsy
Excessive hip flexion in a Pavlik harness can produce transient femoral nerve palsy.
Historically, this has been reported in approximately 2.5% of treated infants.
Redislocation
Redislocation may occur after reduction, with reported rates around 5%.
Stable maintenance of reduction and careful follow-up are therefore important.
Residual Dysplasia
Residual acetabular dysplasia may persist despite successful reduction.
Historical rates have been reported around 25%, depending on age and treatment method.
Avascular Necrosis
Avascular necrosis of the proximal femoral epiphysis is one of the most serious complications of treatment.
It may occur if blood supply to the femoral head is compromised by excessive positioning, forceful reduction, or other treatment-related factors.
Historical rates have been approximately 10%, although incidence varies substantially by technique and severity.
Consequences of Avascular Necrosis
Growth disturbance following AVN can produce femoral head deformity, shortening, altered neck-shaft relationship, joint incongruity, and later degenerative arthritis.
Once established, these changes may not be fully reversible.
Patient Monitoring
Patients require regular examination and imaging after treatment to ensure that the hip remains reduced and the acetabulum continues to develop normally.
Monitoring should include hip range of motion, limb length, gait, acetabular development, femoral head growth, and signs of avascular necrosis or recurrent instability.
Because incomplete remodeling may not become apparent until later childhood, surveillance should continue through skeletal maturity.
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Orthopaedic Surgery - Denosumab
Basics
Denosumab is a fully human IgG2 monoclonal antibody directed against receptor activator of nuclear factor-kappa B ligand (RANKL).
By binding RANKL, denosumab prevents activation of the RANK receptor on osteoclast precursors and mature osteoclasts. This inhibits osteoclast formation, activity, and survival, thereby reducing osteoclast-mediated bone resorption.
Indications
Denosumab is used in disorders associated with excessive osteoclast activity or increased bone turnover.
Important indications include metastatic bone disease, multiple myeloma, postmenopausal osteoporosis, and giant cell tumor of bone.
Giant Cell Tumor of Bone
In giant cell tumor of bone, denosumab may be considered when the lesion is unresectable, metastatic, or when tumor reduction may facilitate a less morbid operation and preservation of bone or an adjacent joint.
Its therapeutic effect is related to inhibition of the RANK/RANKL pathway that is central to formation of the osteoclast-like giant cells within these tumors.
Treatment
Dosing for Giant Cell Tumor and Malignant Bone Disease
For giant cell tumor of bone and selected oncologic indications, denosumab is commonly administered at 120 mg subcutaneously every 4 weeks.
Additional loading doses may be given on days 8 and 15 during the first month of therapy.
Osteoporosis Treatment
Denosumab is also used at a lower cumulative dose for osteoporosis.
The exact regimen depends on the indication and formulation, and treatment should follow the appropriate prescribing protocol for osteoporosis rather than the higher-dose oncologic regimen.
General Measures
Activity
Denosumab itself does not usually require restriction of routine physical activity.
Activity limitations, when present, are generally related to the underlying skeletal disease, fracture risk, or associated treatment rather than to the drug alone.
Dental Procedures
Invasive dental procedures should ideally be addressed before therapy begins whenever feasible.
Elective dental extractions and other procedures involving substantial manipulation of the jaw should be approached cautiously during treatment because of the risk of osteonecrosis of the jaw.
Mechanism of Action
RANKL is required for normal development and activation of osteoclasts.
Denosumab binds RANKL and prevents interaction with the RANK receptor, resulting in a marked decrease in osteoclast-mediated bone turnover.
Unlike bisphosphonates, denosumab does not become permanently incorporated into bone, so its pharmacologic effects diminish after treatment is discontinued.
Complications
Osteonecrosis of the Jaw
One of the most important complications is osteonecrosis of the jaw (ONJ).
The risk generally increases with greater cumulative exposure and is higher in patients receiving high-dose treatment for malignancy.
Risk Factors for Osteonecrosis of the Jaw
Factors associated with greater ONJ risk include longer treatment duration, multiple myeloma, corticosteroid use, diabetes mellitus, antiangiogenic therapy, poor oral hygiene, and invasive dental procedures such as tooth extraction.
Preventive dental care before starting treatment is therefore important.
Dental Evaluation
Patients should undergo an appropriate dental examination before denosumab is initiated, particularly when high-dose oncologic therapy is planned.
Active dental infection or necessary invasive dental treatment should be addressed when possible before exposure.
Atypical Femoral Fracture
Long-term suppression of bone turnover may rarely be associated with atypical femoral fractures.
These fractures may occur in the subtrochanteric region or femoral shaft, extending from just below the lesser trochanter toward the supracondylar region.
Prodromal Femoral Pain
Patients may experience thigh, groin, or hip pain with weight-bearing before an atypical fracture becomes complete.
New unexplained pain in these locations should prompt evaluation for a stress reaction or incomplete fracture.
Reversibility
The antiresorptive effects of denosumab are reversible after treatment is stopped.
Bone turnover can rise substantially after discontinuation, so cessation or interruption of therapy should be planned carefully according to the underlying indication and subsequent osteoporosis or oncologic management.
Hypocalcemia
Denosumab can lower serum calcium because of reduced osteoclastic release of calcium from bone.
Hypocalcemia may be particularly important in patients with renal impairment, vitamin D deficiency, or other disturbances of calcium metabolism.
Patient Monitoring
Dental Examination
A dental assessment should be performed before therapy when clinically appropriate.
During treatment, patients should maintain good oral hygiene and report persistent dental pain, exposed bone, or delayed healing following dental procedures.
Serum Calcium
Serum calcium should be checked before and during treatment.
Calcium and vitamin D supplementation should be provided when indicated to reduce the risk of treatment-associated hypocalcemia.
Symptoms of Hypocalcemia
Patients should be educated about symptoms suggesting low serum calcium.
These may include muscle spasms, twitching, numbness or tingling around the mouth or fingers, facial twitching, and seizures in severe cases.
Monitoring for Stress Fracture
Patients should report new thigh or groin pain during weight-bearing.
When symptoms are suspicious, imaging should be obtained to assess for an incomplete or atypical femoral stress fracture.
Pregnancy
Denosumab should not be used during pregnancy.
Patients with reproductive potential should receive counseling regarding pregnancy avoidance during therapy and for the appropriate period after treatment according to the prescribed regimen.
Patient Teaching
Patients should understand the important warning symptoms associated with therapy, particularly those of hypocalcemia, jaw osteonecrosis, and atypical femoral fracture.
Good dental hygiene, appropriate calcium and vitamin D intake, and prompt reporting of new skeletal pain are important components of treatment.
Frequently Asked Questions
How Long Can Denosumab Be Used?
Treatment duration depends on the underlying indication, fracture or oncologic risk, treatment response, and adverse effects.
Long-term therapy requires periodic reassessment.
Unlike some bisphosphonate regimens, denosumab should not be stopped casually or followed by an unsupervised drug holiday, because rapid rebound in bone turnover can occur after discontinuation.
Can a Patient Become Pregnant While Taking Denosumab?
No. Denosumab is not approved for use during pregnancy.
Patients with reproductive potential should avoid conception during treatment and follow the recommended post-treatment contraception interval.
What Is an Important Contraindication?
Pre-existing hypocalcemia is a major contraindication to starting denosumab.
Serum calcium abnormalities should be corrected before treatment is initiated.
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Orthopaedic Surgery - De Quervain (Radial Styloid) Tenosynovitis
Basics
De Quervain tenosynovitis, also called radial styloid tenosynovitis, is a stenosing disorder involving the first dorsal extensor compartment of the wrist.
This compartment contains the abductor pollicis longus (APL) and extensor pollicis brevis (EPB) tendons.
Patients typically complain of pain and tenderness over the radial side of the wrist, particularly near the radial styloid.
Epidemiology
De Quervain tenosynovitis can occur in either sex and at almost any age, but it is especially common in middle-aged women.
Among working-age adults, the reported incidence is approximately 1.3% in women and 0.5% in men.
Risk Factors
Important risk factors include female sex, middle age, repetitive wrist use, and repetitive thumb motion.
The condition is also frequently seen in new mothers, often because of the repetitive wrist and thumb positions used while lifting and caring for an infant.
Etiology
The disorder is generally considered more of a tendinopathy and stenosing tenosynovial condition than a classic inflammatory tendinitis.
Repeated mechanical stress causes thickening and irritation of the tendon sheath and narrowing of the fibro-osseous tunnel through which the APL and EPB tendons pass.
Repetitive Activities
Activities involving repeated thumb abduction, thumb extension, or wrist deviation may provoke symptoms.
Commonly associated activities include racquet sports, fly fishing, golf, and repetitive infant care.
In golfers, the nondominant wrist may be affected because of the mechanical stresses generated during the swing.
Associated Conditions
De Quervain tenosynovitis may occur in association with rheumatoid arthritis and other inflammatory disorders involving the tendon sheaths.
Diagnosis
Signs and Symptoms
The typical presentation is pain and tenderness along the radial aspect of the wrist, usually directly over the first dorsal compartment near the radial styloid.
The discomfort may radiate proximally into the forearm or distally toward the thumb.
Aggravating Movements
Symptoms are commonly worsened by thumb extension or abduction combined with ulnar deviation of the wrist.
Repetitive gripping, lifting, twisting, and thumb use may be particularly painful.
Physical Examination
Examination may demonstrate localized tenderness, swelling, bogginess, or crepitus over the first dorsal compartment.
The most characteristic area of tenderness lies over the radial side of the radial styloid.
Eichhoff Test
The Eichhoff test, which is frequently mislabeled as the Finkelstein test, is performed by having the patient place the thumb within the palm and close the fingers around it.
The wrist is then deviated toward the ulna.
Reproduction of sharp pain over the first dorsal compartment constitutes a positive test.
Finkelstein Maneuver
The true Finkelstein maneuver involves the examiner gently pulling the patient’s thumb distally and directing the wrist into ulnar deviation.
Pain localized over the first dorsal compartment supports the diagnosis.
WHAT Test
The wrist hyperflexion and abduction of the thumb (WHAT) test is another provocative maneuver.
The patient hyperflexes the wrist and actively abducts the thumb against the examiner’s resistance.
Reproduction of the characteristic radial wrist pain indicates a positive test.
Thumb CMC Examination
The first carpometacarpal joint should also be examined because thumb CMC osteoarthritis may coexist with or mimic de Quervain tenosynovitis.
Range of motion and a CMC grind test should be assessed.
Intersection Syndrome
Intersection syndrome is an important alternative diagnosis.
It is less common than de Quervain tenosynovitis and often produces pain and crepitus approximately 4 cm proximal to the wrist, rather than directly over the radial styloid.
Laboratory Tests
Routine laboratory testing is not required for uncomplicated de Quervain tenosynovitis.
Laboratory studies may be considered only when a systemic inflammatory or rheumatologic disorder is suspected.
Imaging
Plain Radiographs
AP and lateral wrist radiographs may be obtained when there is concern for associated osseous pathology.
Dedicated views of the first carpometacarpal joint may help identify degenerative arthritis that could account for similar symptoms.
Role of Imaging
Imaging is not usually required to establish the diagnosis when the history and examination are classic.
Its primary role is to exclude alternative or concomitant pathology.
Pathophysiology
The APL and EPB tendons pass through a relatively rigid fibro-osseous tunnel within the first dorsal compartment.
Thickening of the tendon sheath and narrowing of the tunnel impair normal tendon gliding.
This produces friction, pain, and progressive irritation with repetitive thumb or wrist movement.
Anatomic Variations
Considerable anatomic variation exists within the first dorsal compartment.
The APL may consist of multiple tendon slips, while the EPB may travel within a separate subsheath.
These variations are important because an unrecognized separate compartment can cause failure of injection or incomplete surgical release.
Differential Diagnosis
Thumb CMC Osteoarthritis
Degenerative disease of the first carpometacarpal joint can produce pain near the base of the thumb.
Pain with the CMC grind test and radiographic degenerative changes favor arthritis.
Intersection Syndrome
Intersection syndrome causes pain where the first dorsal compartment tendons cross the second dorsal compartment tendons.
The tenderness is usually several centimeters proximal to the radial styloid.
Radiocarpal Arthritis
Degenerative disease of the radiocarpal joint can cause radial-sided wrist pain and stiffness.
Radiographs and joint-specific examination help distinguish it from de Quervain disease.
Wartenberg Syndrome
Wartenberg syndrome results from entrapment or irritation of the superficial branch of the radial nerve.
It typically causes burning pain or sensory disturbance over the dorsoradial hand rather than isolated tendon pain.
Treatment
General Principles
Initial management is nonoperative.
A trial of conservative treatment for approximately 3–6 months is appropriate in most patients unless symptoms are unusually severe or persistent.
Activity Modification
Patients should avoid or reduce repetitive movements that aggravate symptoms, particularly repeated thumb abduction, extension, forceful gripping, and sustained wrist deviation.
Thumb Spica Splint
Immobilization of the wrist and thumb in a thumb spica splint may reduce tendon motion and allow symptoms to settle.
Splinting is particularly useful during activities that provoke pain.
NSAIDs
Nonsteroidal anti-inflammatory drugs may be used for symptomatic relief when medically appropriate.
They may decrease pain but do not directly correct the mechanical stenosis.
Corticosteroid Injection
Corticosteroid injection into the first dorsal compartment is highly effective in many patients.
Reported success rates may be as high as approximately 91%.
Injection Technique
Accurate placement within the tendon sheath is important.
Injection failure may occur if an unrecognized EPB subsheath or another septum prevents medication from reaching all involved tendons.
Ultrasound-Guided Injection
Ultrasound guidance can improve visualization of the tendon sheath and anatomic septations.
This may improve injection accuracy, particularly when variant anatomy is present.
Injection Complications
Potential complications include skin depigmentation and subcutaneous fat atrophy at the injection site.
Patients should be counseled about these cosmetic risks.
Physical Therapy
Splinting and activity modification are often the most useful rehabilitation measures.
Therapy may also include gentle stretching, ergonomic modification, and education regarding movements that increase friction within the first dorsal compartment.
Medication
Medical treatment primarily consists of NSAIDs and corticosteroid injection.
Repeated injections should be considered cautiously because of local tissue risks.
Surgery
Indications
Surgery is considered when significant symptoms persist or recur despite appropriate nonoperative treatment.
A previous temporary improvement after corticosteroid injection supports the first dorsal compartment as the source of pain before proceeding with surgery.
Surgical Release
The operation consists of release of the first dorsal extensor compartment.
A radial-sided incision is made over the compartment while carefully protecting the superficial radial sensory nerve branches.
Superficial Radial Nerve Protection
The dorsal sensory branches of the radial nerve frequently cross the surgical field.
They must be identified and protected because direct injury can cause persistent numbness or a painful neuroma.
Release of the Fibro-Osseous Tunnel
The constricting fibro-osseous sheath is opened to allow unrestricted gliding of the APL and EPB tendons.
All internal septa and separate tendon subsheaths must be identified and released.
Dorsal-Sided Release
The sheath is commonly opened along its dorsal margin.
This leaves a volar portion of the retinaculum intact and may help prevent postoperative volar subluxation of the tendons.
Anatomic Variations During Surgery
The surgeon should specifically search for multiple APL slips and a separate EPB compartment.
Failure to recognize these variants is one of the most common reasons for persistent symptoms after surgery.
Postoperative Immobilization
Patients are commonly placed in a thumb spica splint for a short period after surgery.
Motion is then gradually resumed according to wound healing and symptoms.
Follow-Up
Prognosis
The prognosis is generally excellent.
Most patients improve with splinting, corticosteroid injection, or surgical decompression when required.
Follow-Up Schedule
Patients may be reviewed at approximately 3-month intervals until symptoms resolve or a decision regarding operative treatment is made.
Complications
Superficial Radial Nerve Injury
The most serious operative complication is injury to the superficial radial sensory nerve.
Because the nerve lies close to the first dorsal compartment, careful surgical dissection is essential.
Sensory Loss
Nerve injury may result in a localized area of numbness or diminished sensation over the dorsoradial wrist and hand.
Painful Neuroma
More severe nerve injury may lead to formation of a painful neuroma.
This can produce significant chronic pain and may require additional surgery.
Persistent Symptoms
Persistent symptoms after surgical release are most commonly caused by an unreleased septum or an unrecognized separate EPB subsheath.
Revision decompression may be required.
Tendon Subluxation
If the compartment is released improperly, the APL and EPB tendons may subluxate volarly during wrist movement.
A dorsal-sided release technique helps reduce this risk.
Patient Monitoring
Follow-up should assess radial wrist pain, tenderness, thumb and wrist motion, response to splinting or injection, and any sensory changes in the superficial radial nerve distribution.
After surgery, monitoring should also include wound healing, tendon stability, recurrence of symptoms, and evidence of nerve irritation.
- Published on
Orthopaedic Surgery - Cubital Tunnel Syndrome
Basics
Cubital tunnel syndrome (CuTS) is an ulnar nerve entrapment neuropathy at the elbow caused by compression, traction, or both as the ulnar nerve passes through the cubital tunnel.
Patients typically experience pain, numbness, and paresthesias along the ulnar side of the forearm and hand. More advanced disease may produce weakness and wasting of muscles supplied by the ulnar nerve.
Synonym
Cubital tunnel syndrome is sometimes referred to as ulnar tunnel syndrome, although the term should be distinguished from compression of the ulnar nerve at Guyon canal in the wrist.
Epidemiology
Cubital tunnel syndrome is the second most common upper-extremity entrapment neuropathy, after carpal tunnel syndrome.
Men and women are affected at approximately similar rates, with reported incidences of about 25 per 100,000 person-years in men and 19 per 100,000 person-years in women.
Risk Factors
Important risk factors include diabetes mellitus and previous trauma around the elbow.
Degenerative changes, repetitive elbow flexion, local structural abnormalities, and prolonged external pressure may also contribute.
Etiology
Symptoms result from a combination of compression and traction on the ulnar nerve, particularly during elbow flexion.
Flexion decreases the volume of the cubital tunnel while simultaneously stretching the ulnar nerve around the medial epicondyle.
Potential Sites of Compression
The ulnar nerve may be compressed at several locations around the elbow.
These include the Arcade of Struthers, medial intermuscular septum, cubital tunnel proper, and the interval between the two heads of the flexor carpi ulnaris.
Cubital Tunnel Proper
The most common site of compression is the cubital tunnel itself, where the ulnar nerve passes behind the medial epicondyle and beneath Osborne’s ligament.
External and Structural Causes
Potential causes of compression include an enlarged medial head of the triceps, previous trauma, recurrent ulnar nerve subluxation, osteophytes from arthritis, ganglion cysts, and anomalous muscles such as an anconeus epitrochlearis.
Associated Conditions
Cubital tunnel syndrome may coexist with other compressive neuropathies, including carpal tunnel syndrome and thoracic outlet syndrome.
More than one site of nerve compression may therefore be present in the same patient.
Diagnosis
General Principles
The diagnosis is primarily clinical, based on the characteristic history and physical examination.
Nerve conduction studies can support the diagnosis, although false-negative electrodiagnostic studies may occur.
Signs and Symptoms
Patients commonly report a vague aching pain around the medial elbow that may extend into the ulnar aspect of the forearm, wrist, or hand.
Numbness and paresthesias commonly involve the ring and small fingers.
Sensory Symptoms
Altered sensation may occur in the ulnar distribution of the hand and sometimes the medial forearm.
Symptoms are often aggravated by prolonged elbow flexion, such as during sleep, driving, or telephone use.
Physical Examination
Sensory Examination
The examiner should identify sensory abnormalities in the ulnar nerve distribution but should also examine other dermatomes and peripheral nerves.
This is important for excluding cervical radiculopathy, additional peripheral nerve entrapment, or double-crush syndrome.
Dorsal Ulnar Hand Sensation
Numbness over the dorsal ulnar aspect of the hand suggests that ulnar nerve compression is proximal to Guyon canal because the dorsal sensory branch arises before the nerve enters the wrist canal.
Intrinsic Muscle Strength
Intrinsic hand strength should be assessed carefully.
Weakness may affect pinch, finger abduction and adduction, and other fine motor functions.
Froment Sign
The Froment test evaluates weakness of the adductor pollicis.
The patient grasps a piece of paper between the thumb and index finger while the examiner attempts to pull it away.
Positive Froment Sign
If the adductor pollicis is weak, the patient compensates by flexing the thumb interphalangeal joint using the flexor pollicis longus.
This compensatory thumb flexion constitutes a positive Froment sign.
Wartenberg Sign
A Wartenberg sign occurs when the small finger rests persistently in an abducted position.
This reflects weakness of the intrinsic muscles responsible for finger adduction.
Muscle Wasting
The hand should be inspected for intrinsic muscle atrophy.
Wasting is often especially visible in the first dorsal interosseous muscle and along the hypothenar region.
Tinel Sign
Tapping over the ulnar nerve at the cubital tunnel may reproduce tingling or electric sensations into the ring and small fingers.
This constitutes a positive Tinel sign at the elbow.
Elbow Flexion Test
The elbow is held in maximal flexion for approximately 1 minute, usually with the wrist maintained neutral or slightly extended.
Reproduction of ulnar-sided numbness or paresthesias supports the diagnosis.
Keeping the wrist neutral helps avoid provoking coexisting carpal tunnel syndrome.
Scratch Collapse Test
The scratch collapse test has been described as an additional provocative maneuver.
The patient resists shoulder internal rotation while the examiner lightly scratches the skin over the suspected compression site.
Transient loss of resistance has been described in association with compressive neuropathy, although this maneuver should be interpreted together with the rest of the examination.
Ulnar Nerve Subluxation
The ulnar nerve should be palpated while the elbow moves from extension into flexion.
Subluxation or dislocation of the nerve over the medial epicondyle may affect surgical planning and can favor anterior transposition in selected patients.
Imaging and Diagnostic Testing
Plain Radiographs
Elbow radiographs may be obtained when there is concern for a bony cause of compression, previous trauma, deformity, or osteoarthritis.
Radiographs are not required in every uncomplicated case.
Nerve Conduction Studies
Nerve conduction velocity studies may demonstrate slowing of ulnar nerve conduction across the elbow.
The above-elbow and below-elbow conduction velocities are compared.
Electrodiagnostic Criteria
Findings supporting cubital tunnel syndrome include a conduction velocity drop of more than approximately 10 m/s across the elbow or an absolute conduction velocity of less than approximately 50 m/s across the involved segment.
Electrodiagnostic findings should be correlated with clinical symptoms.
Pathological Findings
During surgical decompression, one or more specific areas of nerve constriction may be visible.
All potential sites of compression should be inspected to ensure complete release.
Differential Diagnosis
Cervical Radiculopathy
Compression of the C8 or T1 cervical nerve roots can produce hand numbness, weakness, and intrinsic muscle dysfunction similar to CuTS.
A cervical examination is therefore important when symptoms are atypical.
Thoracic Outlet Syndrome
Thoracic outlet syndrome may produce ulnar-sided paresthesias and upper-extremity discomfort.
The distribution and associated vascular or proximal neurologic findings can help distinguish it from isolated cubital tunnel syndrome.
Guyon Canal Syndrome
Ulnar nerve compression at Guyon canal in the wrist can produce sensory and motor abnormalities in the ulnar hand.
Preserved dorsal ulnar hand sensation favors compression at the wrist rather than the elbow.
Carpal Tunnel Syndrome
Carpal tunnel syndrome affects the median nerve rather than the ulnar nerve but may coexist with CuTS.
The sensory distribution and provocative examination findings help distinguish the two.
Neurologic Disorders
Systemic neurologic conditions such as Guillain–Barré syndrome and amyotrophic lateral sclerosis can produce weakness or sensory changes that mimic peripheral entrapment neuropathy.
Medial Epicondylitis
Medial epicondylitis causes pain over the medial elbow but does not usually produce ulnar nerve sensory loss or intrinsic muscle weakness unless associated CuTS is also present.
Ulnohumeral Osteoarthritis
Degenerative arthritis of the elbow can cause medial elbow pain and osteophyte formation.
Large osteophytes may also contribute directly to ulnar nerve compression.
Treatment
General Measures
Initial treatment is usually nonoperative in patients with mild or moderate symptoms and no progressive motor deficit.
The primary goals are to reduce nerve compression and minimize prolonged elbow flexion.
Night Splinting
A nighttime elbow splint, brace, or soft wrap can be used to prevent excessive flexion during sleep.
Keeping the elbow from flexing beyond approximately 50° may reduce nocturnal symptoms.
Activity Modification
Patients should avoid prolonged elbow flexion and repetitive activities that provoke symptoms.
Direct pressure over the cubital tunnel, such as leaning the medial elbow on a desk or armrest, should also be minimized.
Duration of Conservative Treatment
A trial of nonoperative treatment for approximately 1–3 months is reasonable in patients without severe weakness or progressive neurologic impairment.
Surgery
Indications
Surgery should be considered when symptoms persist despite appropriate conservative care, when numbness is worsening, or when weakness of ulnar-innervated muscles is present.
Progressive muscle wasting is a particularly important indication for surgical evaluation.
Surgical Options
Operative techniques include in situ decompression, anterior ulnar nerve transposition, and medial epicondylectomy.
The optimal procedure depends on nerve stability, anatomy, previous surgery, and the specific site of compression.
In Situ Decompression
Simple decompression releases constricting structures while leaving the ulnar nerve in its native position.
The procedure can be performed through an open or endoscopic approach.
Anterior Transposition
Anterior transposition relocates the ulnar nerve from behind the medial epicondyle to a position anterior to it.
The nerve may be placed subcutaneously, intramuscularly, or submuscularly.
Indications for Transposition
After decompression, the nerve is examined dynamically.
If it is unstable, subluxates, or dislocates over the medial epicondyle, anterior transposition may be performed.
Medial Epicondylectomy
Medial epicondylectomy removes part of the medial epicondyle to reduce tension and compression on the ulnar nerve.
Care must be taken to protect the medial collateral ligament of the elbow.
Medial Antebrachial Cutaneous Nerve
Branches of the medial antebrachial cutaneous nerve cross the operative field during medial elbow surgery.
These branches should be carefully identified and protected because injury can produce painful neuroma or numbness.
Follow-Up
Nonoperative Prognosis
In patients with mild cubital tunnel syndrome, nonoperative management has historically produced excellent results in approximately 58% of cases, excluding many post-traumatic neuropathies.
Surgical Prognosis
Surgical treatment produces good to excellent outcomes in approximately 70–90% of patients.
The degree of recovery depends partly on the severity and duration of preoperative nerve dysfunction.
Decompression Versus Transposition
In the absence of ulnar nerve instability or hypermobility, outcomes after simple decompression are generally comparable with those following anterior transposition.
This allows a less extensive procedure in appropriately selected patients.
Recovery of Weakness
Sensory symptoms may improve earlier than motor weakness.
When severe intrinsic muscle atrophy has been present for a prolonged period, complete strength recovery may not occur even after adequate decompression.
Complications
Postoperative Nerve Irritation
Persistent or new nerve irritation may occur after surgery.
Scar formation, incomplete decompression, nerve instability, or iatrogenic injury may contribute.
Complex Regional Pain Syndrome
Complex regional pain syndrome, historically termed reflex sympathetic dystrophy, may rarely occur after surgery and can produce disproportionate pain, stiffness, and autonomic changes.
Untreated Severe Neuropathy
Progressive untreated ulnar neuropathy can lead to intrinsic muscle atrophy, persistent sensory loss, and clawing of the ring and small fingers.
Ulnar Clawing
Loss of intrinsic muscle function allows imbalance between the extrinsic flexors and extensors.
This can produce hyperextension at the metacarpophalangeal joints and flexion at the interphalangeal joints of the ring and small fingers.
Joint Contractures
Longstanding muscle imbalance and clawing can eventually produce fixed joint contractures.
Early treatment of progressive motor dysfunction may reduce this risk.
Patient Monitoring
Follow-up examinations should document motor strength, sensory function, intrinsic muscle bulk, provocative findings, and progression or improvement of symptoms.
Patients treated surgically should also be monitored for wound problems, recurrent nerve instability, persistent compression, and recovery of hand function.
