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Case Reports and Small Case Series
October 1999

Bilateral Massive Retinal Hemorrhages in a 6-Month-Old Infant: A Diagnostic Dilemma

Arch Ophthalmol. 1999;117(10):1432-1434. doi:

Retinal hemorrhages in infants sometimes pose a diagnostic dilemma for ophthalmologists.

Report of a Case.

A 6-month-old infant was treated in the hospital for a rotavirus gastroenteritis. Two days after discharge from the hospital, he was readmitted, profoundly dehydrated and in hypovolemic shock. He had collapsed and was unresponsive. His Glasgow Coma Scale score was 4, and his pupils were fixed and dilated. He was intubated and ventilated. Serum and plasma levels were measured and revealed hypernatremic dehydration consistent with severe water loss via the gastrointestinal tract: sodium, 169 mmol/L; potassium, 7.3 mmol/L; chloride, 136 mmol/L; urea nitrogen, 25 mmol/L (70 mg/dL); and creatinine, 222 µmol/L (2.51 mg/dL).The infant was acidotic with a pH of 6.8; PO2, 10.6; PCO2, 5.2; standard bicarbonate, 6.8 mmol/L; and base excess, 23 mmol/L.

Observations from funduscopy revealed massive bilateral retinal hemorrhages radiating from the posterior pole of the eyeball (Figure 1). Findings from coagulation studies, complete blood cell and differential cell counts, and thrombophilic screens were normal. His profiles for amino acid, fatty acid, and organic acid were normal. A computed axial tomographic scan of the brain showed diffuse cerebral edema, subdural blood in the temporal fossa, and diffuse subarachnoid hemorrhage (Figure 2). The infant's clinical condition deteriorated and he died.

Figure 1.
Retinal hemorrhages radiating from the posterior pole of the eyeball in a 6-month-old infant.

Retinal hemorrhages radiating from the posterior pole of the eyeball in a 6-month-old infant.

Figure 2.
Noncontrast computed axial tomographic scans of the brain. Left, The arrow indicates subdural hemorrhage in the temporal fossa. Right, Diffuse cerebral edema and subarachnoid hemorrhage are indicated by the arrows.

Noncontrast computed axial tomographic scans of the brain. Left, The arrow indicates subdural hemorrhage in the temporal fossa. Right, Diffuse cerebral edema and subarachnoid hemorrhage are indicated by the arrows.

Findings from histopathologic examination of the eyes revealed massive retinal hemorrhages with subhyaloid and subretinal hemorrhages in both eyes (Figure 3). The brain scan revealed venous and capillary congestion with subarachnoid hemorrhage, a subdural collection, and focal intracerebral hemorrhages. There was also diffuse microvessel thrombosis in many organs, including the lungs, kidneys, and myocardium, consistent with disseminated intravascular coagulation. Nonaccidental injury (NAI) was suspected because of the findings from clinical examination; in particular, massive retinal hemorrhages in association with intracerebral hemorrhage. However, there was no evidence of trauma. A skeletal survey revealed no abnormalities. The findings from clinicalexamination were consistent with severe hypernatremic dehydration causing diffuse intracerebral hemorrhage, subarachnoid hemorrhage, retinal hemorrhages, and ultimately brain death.

Figure 3.
Findings from histopathologic examination of the retina show subretinal (single arrow), intraretinal (double arrows), and subhyaloid hemorrhage (triple arrows).

Findings from histopathologic examination of the retina show subretinal (single arrow), intraretinal (double arrows), and subhyaloid hemorrhage (triple arrows).

Comment.

Retinal hemorrhages in infancy are believed to be a cardinal sign of NAI. They may occur in up to 89% of infants with NAI.1 They may result from direct head trauma or the acceleration and deceleration forces generated by the shaking of the head. Shaken baby syndrome is a unique form of child abuse in which the only consistent external physical signs are ocular manifestations. Differential diagnoses of retinal hemorrhages include thrombocytopenias, leukemias, and infections such as infective endocarditis.

In this case profound electrolyte disturbance, namely, hypernatremic dehydration, caused intracerebral, subdural, and subarachnoid hemorrhages. Hypernatremia causes cerebral cellular dehydration and results in brain shrinkage. This leads to the rupture of bridging veins, causing subdural and intracerebral hemorrhages. Elevated intracranial pressure leads to increased retinal venous pressure and results in retinal hemorrhages.

Finberg2,3 reported 12 cases of subarachnoid or subdural hemorrhage from hypernatremic dehydration in infants, 2 of whom died and 10 who had severe residual neurological damage. Pathologic effects of hypernatremia were also reported following a nursery disaster in which an improper infant food mixturecontaining an excess of sodium chloride was administered to infants. Findings from autopsy showed subarachnoid hemorrhage, intracerebral hemorrhage, cortical venous thrombosis, and venous infarctions. Similar cases in adults have shown widespread cerebral hemorrhage.4 Infants are more susceptible to hypernatremia because of their large surface area and poor renal concentrating ability. The typical radiological findings from computed tomographic scans of infants with hypernatremia include brain parenchymal abnormalities, multifocal areas of hemorrhage, and infarction.5 Retinal hemorrhages were not documented in these cases.

In conclusion, we report a case of massive bilateral retinal hemorrhages and intracranial hemorrhages attributable to profound hypernatremic dehydration in an infant. The findings from clinical examination are similar to those seen in NAI. It is important to highlight this to avoid potential mistaken diagnoses. Unexplained retinal hemorrhages in infancy mandate a full clinical workup. The NAI remains high on our list of differential diagnoses, but other pathologic conditions can mimic NAI and have an identical clinical presentation.

References
1.
Tyagi  AKScotcher  SKozeis  NWillshaw  HE Can convulsions alone cause retinal hemorrhages in infants? Br J Ophthalmol. 1998;82659- 660Article
2.
Finberg  L Pathogenesis of lesions in the nervous system in hypernatremic states. Pediatrics. 1959;1940- 45
3.
Finberg  LHarrison  HE Hypernatremia in infants [editorial]. Pediatrics. 1955;161
4.
Davis  RLRobertson  DN Textbook of Neuropathology. 2nd ed. Baltimore, Md Williams & Wilkins1990;
5.
Han  BKLee  MYoon  HK Cranial ultrasound and CT findings in infants with hypernatremic dehydration. Pediatr Radiol. 1997;9739- 742Article
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