812 - A Hidden Cause of Vision Loss: Undetected Blindness Secondary to Chronic Hydrocephalus

A 5-year-old female with cerebral palsy secondary to 26-week prematurity, epilepsy, retinopathy of prematurity, interventricular hemorrhage, and posthemorrhagic neonatal hydrocephalus s/p ventricular-subgaleal shunt placement, presented to an outpatient neurology visit with lethargy, inability to sit upright, and new onset horizontal nystagmus. Her mother endorsed a 1-week history of generalized fatigue and behaviors markedly different from baseline. Of note, it had been 3 years since her last brain MRI. Given her symptoms, she was referred to the ED. Imaging revealed fourth ventricular outlet obstruction requiring prompt neurosurgical intervention with endoscopic third ventriculostomy.

Two days postoperatively, she was discovered to have previously undetected visual deficits. Prior ophthalmic examination in 2019 due to her underlying ROP had been unremarkable. On examination, she was unable to visually locate individuals in the room and had absent tracking to light. Ophthalmoscopy revealed no light perception and loss of vision with optic disc pallor bilaterally.

Discussions:

Hydrocephalus occurs when excess cerebrospinal fluid enlarges the ventricles, increasing intracranial pressure and potentially injuring the optic nerve. If unaddressed, this can lead to atrophy and blindness. This patient likely had undetected visual changes over time in the setting of chronic hydrocephalus. These changes were undetected by family and schoolteachers, possibly due to compensatory adjustments or spared cognitive visual pathways. Cortical blindness from hydrocephalus is most commonly reported in infants, with fewer cases described in preadolescents.

Conclusions:

This case highlights the importance of close follow-up and regular ophthalmologic evaluation for patients with or at risk for hydrocephalus. There is a need to train families and teachers to recognize symptoms of visual impairment. The physiatrist’s role in such cases includes facilitating care and monitoring for disease progression/complications. In this case, had the patient’s visual impairment been recognized earlier, she may have been spared from legal blindness and its functional barriers.