Kathleen Romero
Alex V. Levin

BASICS

DESCRIPTION

• RH may occur on the surface or just below the retina (preretinal or subretinal), or they may be found within the retinal layers. Intraretinal hemorrhages include those in the nerve fiber layer (flame, splinter) or RH deep in retinal tissue (dot, blot).

• Intraretinal and preretinal hemorrhages may have white centers, a nonspecific finding found in virtually any cause of RH.

EPIDEMIOLOGY

Incidence

• Hemorrhage is seen in ~85% of shaken baby syndrome (SBS)/Abusive head trauma (AHT) victims.

• Hemorrhage can be seen in over 40% of children after birth in the first 24 h of life.

• Newborn RH are seen most often with vacuum-assisted delivery followed by forceps delivery, with the lowest incidence in C-section delivery.

RISK FACTORS

• Trauma: Abusive head trauma (AHT), direct orbital/ocular injury, birth process, accidental

• Infection: Bacterial endocarditis, sepsis, meningitis, CMV retinitis, toxoplasmosis, malaria

• Intracranial: Papilledema, arteriovenous malformation (AVM), ruptured aneurysm

• Systemic disease: Hemolytic uremic syndrome, vasculitis, diabetes (post pubertal only), sickle cell, hypertension (acute on chronic, older children only)

• Coagulopathies: Including thrombocytopenia, severe anemia, leukemia, factor deficiencies, protein C/S deficiency, vitamin K deficiency

• Cardiopulmonary: Carbon monoxide poisoning, hypoxia, hypotension, ECMO

• Metabolic/endocrine: Hypo/hypernatremia, galactosemia, glutaric aciduria type 1

Genetics

Genetic retinal disease may be associated with RH: Norrie disease, Coats disease, juvenile X-linked retinoschisis, von Hippel Lindau.

GENERAL PREVENTION

• Control of systemic diseases

• Child abuse prevention

PATHOPHYSIOLOGY

• AHT: Repetitive acceleration–deceleration with or without impact causing vitreo-retinal traction is a major factor. Modulating factors may include hypoxic ischemic injury, increased venous pressure, and/or disruption of autonomic supply to the retinal vessels.

• Increased intracranial pressure, intracranial hemorrhage, blunt trauma, occlusive thrombi in retinal veins, leaky vessels, and infection.

ETIOLOGY

• Hemorrhage originates from retina or choroid.

• It depends on a causative agent.

COMMONLY ASSOCIATED CONDITIONS

• AHT, accidental trauma

• Multiple systemic and retinal diseases

DIAGNOSIS

HISTORY

• Any history of trauma (trauma to orbit, head trauma), fussy irritable child, increased/abnormal bruising, bleeding

• Newborn

• Investigate for related systemic diseases

• Spotted vision, decreased vision in older verbal children

PHYSICAL EXAM

• Complete eye evaluation including visual acuity if age appropriate, visual fields, indirect ophthalmoscopy.

• Describe RH: Types, patterns, distribution, and number

• Use detailed descriptions, both words and careful drawings. Photography not required but may be useful.

• Pharmacologic dilation of pupils is essential when possible.

DIAGNOSTIC TESTS & INTERPRETATION

Lab

Initial lab tests

CBC with differential, PT, aPTT/INR

Follow-up & special considerations

• If possible, in case of trauma, consider liver function tests, amylase/lipase.

• If there is any concern about coagulopathy, consider vWF, ristocetin cofactor, platelet function, Factors VII, VIII, XII, and XVIII. D-dimer, fibrinogen.

• If there is any concern about vasculitis/hemoglobinopathy, consider ANA, ESR, ACE, serum Ca, sickle cell electrophoresis.

• If possible conduct the glutaric aciduria test for urine organic acids.

Imaging

• Retinal photography if possible

• OCT if available

• Possible role for ultrasound if view of retina obscured

• Skeletal survey and bone scan if there is any concern about abusive injury, CT/MRI head for intracranial bleed

Diagnostic Procedures/Other

Indirect ophthalmoscopy required for full retinal view.

Pathological Findings

• Traumatic retinoschisis (most often subinternal limiting membrane blood) has particular diagnostic significance in recognizing SBS/AHT.

• RH in AHT (present in two thirds of cases) are often described as diffuse, extensive, multilayered RH extending to ora serrata, although some cases may have milder RH in the posterior pole.

• Additionally, there are reports of diffuse, extensive RH with leukemia, fatal head crush injury, newborns, and fatal motor vehicle accidents.

• Hemorrhages associated with other systemic conditions and accidental head injury are usually few in number and confined to the posterior pole; subretinal hemorrhage is extremely rare is these circumstances and retinoschisis is not reported (although sub-ILM hemorrhage can be seen in leukemia).

DIFFERENTIAL DIAGNOSIS

• Inflicted AHT

• Accidental head trauma

• Neonatal birth trauma

• Hematologic malignancies

• Collagen vascular disease, vasculitis

• Coagulopathies

• Retinal hemangioma

• Prolonged cardiopulmonary resuscitation: Causing only mild RH in posterior pole

• AVM rupture: Case reports

• Galactosemia

• Glutaric aciduria type I

• Malaria

• Carbon monoxide poisoning

• Hyper/hyponatremia

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