For infants and babies who can’t speak for themselves, a diagnosis of shaken baby syndrome is a matter of life or death. This was so with Baby X, a 4-month old boy with a gestational age of 35 weeks, who presented with fussiness and intermittent shaking. He had a seizure in the ED, followed by status epilecticus. He also had a fever and elevated white blood count. Baby X underwent a full battery of tests. Afterwards, an ophthalmology consult was called in to evaluate the child’s retina. Here is a case study from Vishal Parikh, MD, a resident at Cleveland Clinic’s Cole Eye Institute.
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The purported cause
Baby X lived with his mother, her husband and a 17-month-old brother. The husband was not the father of either child. According to the baby’s mother, the 17-month-old tipped over the bassinet with Baby X in it. He cried a lot, but they thought “he was scared.” No marks or redness were evident after the incident.
The patient and test findings
At birth, Baby X spent 10 days in the NICU for respiratory distress. His past ocular and medical histories were unremarkable. Sedation on phenobarbital precluded assessment of visual acuity. Digital palpation found normal intraocular pressure. Pupils were pinpoint, but equal and reactive. Alignment was bilateral exotropia.
External examination of the anterior segments of Baby X’s eyes found normal orbits. The slit lamp exam showed normal lids, lashes, and lacrimal glands and drainage; no subconjunctival hemorrhage, clear corneas; and clear lenses. The anterior chamber was deep and without hyphema or hypopyon.
Through funduscopic examination of the retina, however, evidence of hemorrhage in all layers of the retina was found in both eyes.
Lab tests found bloody, xanthochromic cerebral spinal fluid. A CT brain scan showed multifocal areas of hypoattenuation concerning for acute ischemia or edema. The brain MRI found catastrophic insult involving the cerebral hemispheres and bilateral subdural hematomas.
Fundus photo of Baby X’s retina showing retinal hemorrhage.
Differential diagnoses in a child like Baby X may include birth hemorrhages, accidental trauma, Purtscher and Purtscher’s-like retinopathy, meningitis, coagulopathies, leukemia/leukostasis, blood dyscrasias or Terson syndrome.
However, the aforementioned etiologies are significantly less likely to cause retinal hemorrhages compared to Shaken baby syndrome, which is always first on the differential for retinal hemorrhages in an infant. After review of the history and exam, the case was considered most consistent with non-accidental trauma or shaken baby syndrome.
In shaken baby syndrome, trauma to peripheral blood vessels causes significant ischemia that leads to retinopathy of prematurity-like traction and exudative retinal detachment. Examination under anesthesia/flip angle and laser-indirect ophthalmoscope are essential to prevent complex retinal detachment by using thermal ablation. A visual-evoked potential test three months later helps determine visual prognosis.
The pediatric care team called in the Department of Child and Family Services. When confronted with ophthalmic findings and the possibility of blindness, the mother’s husband confessed to causing the injuries. Baby X was weaned off sedation, but required nasogastric feeding. He was discharged with his mother to the maternal grandfather’s house for home care.