Ophthalmology – Retinal Hemorrhages

Basics

Description

Retinal hemorrhages (RH) are collections of blood within, beneath, or in front of the neurosensory retina.

Their appearance depends strongly on the retinal layer involved.

They may be:

• Superficial intraretinal
• Deep intraretinal
• Preretinal/subhyaloid
• Sub–internal limiting membrane (sub-ILM)
• Subretinal
• Occasionally associated with vitreous hemorrhage

Retinal hemorrhage is a:

Clinical sign rather than a diagnosis

and its significance depends on:

• Age
• Distribution
• Number
• Retinal layer
• Laterality
• Associated ocular findings
• Systemic context

Major Clinical Principle

The morphology and distribution of hemorrhage provide important diagnostic clues.

For example:

• Flame-shaped hemorrhages → superficial nerve fiber layer
• Dot-blot hemorrhages → deeper retinal layers
• Boat-shaped/preretinal hemorrhage → blood between posterior hyaloid or ILM and retina
• Subretinal hemorrhage → blood beneath neurosensory retina

Retinal Anatomy and Hemorrhage Shape

The retinal architecture determines the appearance of blood.

Superficial Retina

Blood spreads along retinal nerve fibers, producing:

Flame-shaped or splinter hemorrhages

Deep Retina

Blood is confined by vertically oriented retinal structures, producing:

Dot or blot hemorrhages

Preretinal Space

Blood may form a:

Boat-shaped or horizontally layered hemorrhage

because it settles under gravity.

Flame-Shaped Hemorrhages

Flame hemorrhages occur in the:

Retinal nerve fiber layer

Common associations include:

• Hypertensive retinopathy
• Retinal vein occlusion
• Papilledema
• Anemia
• Leukemia
• Abusive head trauma

Dot-Blot Hemorrhages

Dot-blot hemorrhages arise in deeper retinal layers.

Common associations include:

• Diabetic retinopathy
• Retinal vein occlusion
• Blood dyscrasias
• Severe retinal ischemia

Preretinal / Subhyaloid Hemorrhage

Blood accumulates between the:

• Posterior hyaloid and retina

or beneath the ILM.

It may appear:

• Round
• Dome-shaped
• Boat-shaped

Common causes include:

• Valsalva retinopathy
• Proliferative diabetic retinopathy
• Retinal neovascularization
• Trauma
• Terson syndrome

Sub-ILM Hemorrhage

Sub-ILM hemorrhage lies between:

• Internal limiting membrane
• Retinal nerve fiber layer

It can appear sharply demarcated and may mimic subhyaloid hemorrhage.

Large premacular collections can cause:

Marked central visual loss

Subretinal Hemorrhage

Subretinal blood lies beneath the:

Neurosensory retina

Common causes include:

• Neovascular age-related macular degeneration
• Myopic CNV
• Trauma
• Polypoidal choroidal vasculopathy
• Retinal macroaneurysm
• Severe choroidal vascular disease

White-Centered Retinal Hemorrhages

White-centered hemorrhages are traditionally called:

Roth spots

The white center may represent:

• Fibrin
• Platelet aggregates
• Ischemic retinal tissue
• Leukemic or inflammatory material

They are:

Nonspecific

and can occur with:

• Infective endocarditis
• Leukemia
• Severe anemia
• Sepsis
• Diabetes
• Hypertension
• Other systemic illness

They are not pathognomonic of endocarditis.

Epidemiology

The frequency of retinal hemorrhage depends entirely on the population and underlying disease.

Important contexts include:

• Newborn retinal hemorrhage
• Retinal vascular disease
• Trauma
• Hematologic disease
• Intracranial disease
• Abusive head trauma

Newborn Retinal Hemorrhage

Retinal hemorrhage is relatively common after delivery, especially following:

• Vaginal delivery
• Vacuum-assisted delivery
• Forceps delivery

It is less common after:

• Cesarean delivery

Most neonatal hemorrhages:

Resolve spontaneously over days to weeks

without visual consequence.

Pediatric Importance

In infants and young children, retinal hemorrhage may result from:

• Birth-related injury
• Accidental trauma
• Abusive head trauma
• Coagulopathy
• Leukemia
• Severe systemic illness
• Intracranial disease

The pattern must always be interpreted together with:

• History
• Neurologic findings
• Systemic evaluation
• Neuroimaging

Abusive Head Trauma

Abusive head trauma (AHT) is an important cause of retinal hemorrhage in infants and young children.

Retinal findings that are especially concerning include:

• Numerous hemorrhages
• Bilateral involvement
• Multilayer hemorrhages
• Extension from posterior pole to peripheral retina
• Retinoschisis
• Perimacular retinal folds

However:

No single retinal finding is independently diagnostic of abuse.

The diagnosis requires integration of:

• Ophthalmic findings
• History
• Neuroimaging
• Skeletal findings
• Laboratory evaluation
• Multidisciplinary child-protection assessment

Important Modern Terminology

The preferred term is:

Abusive head trauma

rather than “shaken baby syndrome,” because injury may involve:

• Acceleration-deceleration
• Rotation
• Impact
• Combinations of mechanisms

Mechanisms in AHT

Proposed mechanisms include:

• Vitreoretinal traction from repetitive acceleration-deceleration
• Increased intracranial and venous pressure
• Hypoxic-ischemic injury
• Vascular dysregulation

The exact contribution of each mechanism varies.

Retinoschisis

Traumatic retinoschisis in AHT typically involves:

• Macula
• Perimacular retina

and may contain:

• Sub-ILM blood
• Intraretinal blood

Associated perimacular folds are highly concerning in the appropriate clinical context.

CPR and Retinal Hemorrhage

Cardiopulmonary resuscitation alone generally causes:

• No retinal hemorrhage
• Or only limited posterior hemorrhage

Extensive multilayer hemorrhages extending to the periphery are:

Not typically explained by uncomplicated CPR alone.

Birth-Related Retinal Hemorrhage

Neonatal birth hemorrhages are usually:

• Intraretinal
• Posterior pole predominant
• Self-resolving

Most resolve within:

• Several days to a few weeks

Some deeper hemorrhages can persist longer.

Risk Factors and Causes

Trauma

• Abusive head trauma
• Accidental head trauma
• Direct ocular trauma
• Birth trauma

Retinal Vascular Disease

• Diabetic retinopathy
• Hypertensive retinopathy
• Retinal vein occlusion
• Retinal artery macroaneurysm
• Ocular ischemic syndrome

Hematologic Disease

• Leukemia
• Thrombocytopenia
• Severe anemia
• Coagulopathy
• Disseminated intravascular coagulation
• Hemophilia
• Vitamin K deficiency
• Sickle cell disease

Infection

Possible causes include:

• Infective endocarditis
• Sepsis
• CMV retinitis
• Toxoplasmosis
• Malaria

Intracranial Disease

Retinal hemorrhages may occur with:

• Papilledema
• Terson syndrome
• Intracranial hemorrhage
• Ruptured aneurysm
• Severe intracranial hypertension

Terson Syndrome

Terson syndrome refers to intraocular hemorrhage associated with:

• Subarachnoid hemorrhage
• Intracranial hemorrhage
• Severe acute intracranial pressure elevation

Hemorrhage may be:

• Vitreous
• Preretinal
• Intraretinal

Hypertension

Severe hypertension may produce:

• Flame hemorrhages
• Cotton-wool spots
• Hard exudates
• Optic disc edema in malignant hypertension

In children, significant hypertensive retinopathy should prompt evaluation for:

• Renal disease
• Endocrine disease
• Other secondary hypertension causes

Diabetes

Diabetic retinopathy causes:

• Microaneurysms
• Dot-blot hemorrhages
• Venous changes
• Exudates
• Neovascularization in advanced disease

Diabetic retinal hemorrhage is uncommon in very young children because retinopathy generally requires:

Years of hyperglycemic exposure.

Leukemia

Leukemia may produce:

• Flame hemorrhages
• Dot-blot hemorrhages
• White-centered hemorrhages
• Cotton-wool spots
• Venous tortuosity

Mechanisms include:

• Anemia
• Thrombocytopenia
• Hyperviscosity
• Direct infiltration

Sickle Cell Disease

Sickle retinopathy can produce:

• Peripheral hemorrhage
• Salmon-patch hemorrhage
• Neovascularization
• Vitreous hemorrhage

especially in proliferative disease.

Papilledema

Severe papilledema may produce:

• Peripapillary flame hemorrhages
• Splinter hemorrhages
• Cotton-wool spots

The optic disc edema itself is usually the dominant finding.

Valsalva Retinopathy

A sudden rise in intrathoracic or intra-abdominal pressure can rupture superficial retinal capillaries.

Triggers include:

• Heavy lifting
• Vomiting
• Coughing
• Labor
• Straining

Typical finding:

Premacular preretinal/sub-ILM hemorrhage

with sudden painless central visual loss.

Retinal Vein Occlusion

Central Retinal Vein Occlusion

May show:

• Diffuse retinal hemorrhages
• Venous dilation and tortuosity
• Cotton-wool spots
• Disc edema

Branch Retinal Vein Occlusion

Produces:

• Sectoral hemorrhages
• Corresponding venous congestion

Retinal Macroaneurysm

Retinal arterial macroaneurysm can cause hemorrhage at multiple levels:

• Preretinal
• Intraretinal
• Subretinal

This “multilevel” hemorrhage pattern is especially characteristic.

History

Important questions include:

• Sudden or gradual visual loss?
• Floaters?
• Trauma?
• Recent birth?
• Head injury?
• Unexplained bruising?
• Bleeding tendency?
• Fever or infection?
• Diabetes?
• Hypertension?
• Hematologic disease?
• Anticoagulant use?

In pediatric cases, history should be documented:

Precisely and contemporaneously.

Examination

Perform a complete ocular examination including:

• Visual acuity when age appropriate
• Pupils
• Anterior segment
• IOP when appropriate
• Dilated fundus examination
• Indirect ophthalmoscopy

Describing Retinal Hemorrhages

Document:

• Number
• Size
• Shape
• Retinal layer
• Laterality
• Distribution
• Posterior vs peripheral location
• Macular involvement
• Associated retinoschisis
• Associated retinal folds

Documentation in Suspected AHT

High-quality documentation is especially important.

Whenever feasible obtain:

• Wide-field retinal photography
• Detailed drawings
• Written description

Photography is valuable for:

• Multidisciplinary review
• Monitoring resolution
• Medico-legal documentation

but does not replace a complete examination.

Indirect Ophthalmoscopy

Dilated indirect ophthalmoscopy is essential for assessing:

• Peripheral retinal extent
• Hemorrhage number
• Retinoschisis
• Retinal tears
• Retinal detachment

This is particularly important in suspected AHT because peripheral hemorrhages may be missed on limited posterior examination.

OCT

OCT is useful for:

• Macular hemorrhage
• Sub-ILM hemorrhage
• Retinoschisis
• Retinal layer localization
• Macular structural damage

Handheld OCT may be useful in infants when available.

Fundus Photography

Wide-field imaging can document:

• Extent
• Distribution
• Evolution over time

RetCam-type systems are often used in infants and young children.

B-Scan Ultrasonography

Useful when media opacity prevents retinal visualization due to:

• Dense vitreous hemorrhage
• Cataract
• Severe anterior segment opacity

It can assess for:

• Retinal detachment
• Posterior segment mass
• Vitreous hemorrhage

Laboratory Evaluation

Testing should be:

Directed by the clinical context

rather than automatically ordering every possible test.

Common initial studies when systemic bleeding disorder is possible include:

• CBC with platelet count
• PT/INR
• aPTT

Additional Hematologic Testing

When indicated, consider:

• Fibrinogen
• D-dimer
• von Willebrand testing
• Specific clotting factors
• Platelet function studies

usually in consultation with hematology.

Infectious Evaluation

If infection is suspected, investigations depend on the clinical setting and may include:

• Blood cultures
• Inflammatory markers
• Targeted serologies/PCR

Child Protection Evaluation

When AHT is a concern, evaluation may include:

• Pediatric assessment
• Neuroimaging
• Skeletal survey
• Laboratory testing for bleeding disorders
• Social/child-protection team consultation

The exact investigation follows:

Local child-protection protocols.

Important Modern Correction – Metabolic Testing

Disorders such as:

Glutaric aciduria type 1

have historically been discussed as mimics of AHT.

They should not be reflexively tested in every child with retinal hemorrhage.

Metabolic testing is best guided by:

• Clinical phenotype
• Neurologic findings
• Neuroimaging
• Genetics/metabolic consultation

Differential Diagnosis

Important causes include:

• Abusive head trauma
• Accidental trauma
• Birth-related retinal hemorrhage
• Coagulopathy
• Leukemia
• Severe anemia
• Retinal vein occlusion
• Hypertensive retinopathy
• Diabetic retinopathy
• Papilledema
• Terson syndrome
• Valsalva retinopathy
• Retinal macroaneurysm
• Infective endocarditis
• Retinal vasculitis

Treatment Principles

There is no treatment directed simply at the presence of blood.

Management focuses on:

1. Treating the underlying cause
2. Protecting vision
3. Preventing complications

Observation

Many retinal hemorrhages resolve spontaneously.

Observation is appropriate when:

• Underlying cause is controlled
• Hemorrhage is not vision-threatening
• No retinal detachment or neovascular complication exists

Hematologic Disease

Treat the systemic disorder appropriately.

Management may include:

• Platelet transfusion
• Packed red blood cells
• Fresh frozen plasma
• Vitamin K
• Specific factor replacement

depending on the underlying condition.

Retinal Vascular Disease

Management depends on cause.

Examples:

• Diabetic retinopathy → anti-VEGF/laser according to stage
• Retinal vein occlusion → anti-VEGF for macular edema ± other treatment
• Proliferative disease → PRP
• Retinal macroaneurysm → observation, laser, or anti-VEGF in selected cases

Valsalva Hemorrhage

Most premacular hemorrhages can be:

Observed

because spontaneous clearing is common.

Large dense premacular hemorrhages may occasionally be treated with:

• Nd:YAG membranotomy in selected appropriate cases
• Vitrectomy if nonclearing or complicated

Vitreous Hemorrhage

Pars plana vitrectomy may be indicated for:

• Nonclearing vitreous hemorrhage
• Retinal detachment
• Traction
• Need to visualize/treat underlying retinal disease

Pediatric Vitreous Hemorrhage

The threshold for intervention may be lower in young children because prolonged visual deprivation can cause:

Amblyopia

especially during critical periods of visual development.

Amblyopia Management

If one eye has prolonged visual deprivation:

• Refractive correction
• Occlusion therapy
• Other amblyopia treatment

may be required once the ocular media are sufficiently clear.

Retinoschisis in AHT

Surgery for traumatic macular retinoschisis is:

Rare and individualized

because intervention itself can damage fragile retinal tissue.

Most management focuses on:

• Systemic stabilization
• Documentation
• Observation of ocular findings

unless a specific surgical indication develops.

Follow-Up

Follow-up depends on:

• Cause
• Hemorrhage severity
• Macular involvement
• Vitreous involvement
• Retinal detachment risk
• Age of patient

Neonatal Hemorrhage

Most uncomplicated birth-related hemorrhages require:

• Observation

Follow-up is particularly appropriate when:

• Macula is involved
• Hemorrhage is dense
• Vitreous hemorrhage is present
• Resolution is uncertain

Pediatric AHT

Serial documentation may help assess:

• Hemorrhage resolution
• Retinoschisis
• Optic nerve injury
• Retinal scar formation
• Visual potential

Prognosis

Prognosis depends primarily on:

The underlying disease rather than the hemorrhage itself.

Small intraretinal hemorrhages often resolve without permanent visual loss.

Poor Prognostic Features

Visual prognosis is worse with:

• Dense premacular hemorrhage
• Vitreous hemorrhage
• Macular retinoschisis
• Retinal detachment
• Optic nerve injury
• Severe retinal ischemia
• Associated cortical visual impairment

Abusive Head Trauma Prognosis

Visual impairment after severe AHT may result from:

• Retinal injury
• Optic atrophy
• Retinal folds/scarring
• Amblyopia
• Cortical/cerebral visual impairment

Neurologic injury may be more important than retinal damage in determining final visual function.

Complications

Possible complications include:

• Vitreous hemorrhage
• Retinal detachment
• Retinoschisis
• Macular scar
• Epiretinal membrane
• Optic atrophy
• Amblyopia
• Strabismus
• Permanent visual loss

Ophthalmology Pearls

• Retinal hemorrhage is a sign, not a diagnosis; interpretation depends on morphology, layer, distribution, age, and systemic context.
• Flame hemorrhages arise in the nerve fiber layer, whereas dot-blot hemorrhages arise in deeper retinal layers.
• Preretinal/sub-ILM hemorrhage may appear boat-shaped and can cause severe central visual loss when premacular.
• White-centered hemorrhages (Roth spots) are nonspecific and are not pathognomonic of infective endocarditis.
• In infants and young children, retinal hemorrhage requires careful consideration of birth trauma, accidental trauma, systemic disease, coagulopathy, and abusive head trauma.
• Retinal findings particularly concerning for AHT include numerous bilateral multilayer hemorrhages extending to the retinal periphery, macular retinoschisis, and perimacular folds, but no single ocular finding proves abuse by itself.
• Suspected AHT requires a multidisciplinary child-protection evaluation, not interpretation of retinal findings in isolation.
• Wide-field photography plus detailed written documentation is highly valuable in suspected pediatric trauma.
• CPR alone generally does not explain extensive multilayer peripheral retinal hemorrhages.
• Birth-related retinal hemorrhages usually resolve spontaneously within days to weeks.
• Dense vitreous hemorrhage in a young child can produce deprivation amblyopia, so prolonged nonclearing hemorrhage may justify earlier vitrectomy.
• In older patients, common etiologies include diabetic retinopathy, retinal vein occlusion, hypertension, retinal macroaneurysm, Valsalva retinopathy, and hematologic disease.
• OCT is particularly useful for localizing premacular hemorrhage, sub-ILM blood, retinoschisis, and macular structural injury.
• Treatment is directed at the underlying cause; most uncomplicated intraretinal hemorrhages themselves require observation rather than direct therapy.
• The visual prognosis depends much more on associated macular, optic nerve, retinal ischemic, or cerebral injury than on the mere presence of hemorrhage.