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A 31-year-old man with vitritis, chorioretinitis, and hydrocephalus
Digital Journal of Ophthalmology 2012
Volume 18, Number 2
May 14, 2012
DOI: 10.5693/djo.03.2012.03.001
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Andrew Schneier, MD | Massachusetts Eye and Ear Infirmary, Boston, Massaachusetts
Diagnosis and Discussion
We felt that the clinical picture was consistent with reactivation toxoplasmosis in the right eye. However, the appearance of disc edema in the left eye merited emergent neuroimaging and further workup. Marked hydrocephalus was discovered and intervention was successful. The coexistence of obstructive hydrocephalus with attendant papilledema and characteristic chorioretinal lesions in our patient from Brazil raises the question of a hitherto undiagnosed congenital or acquired cerebral toxoplasmosis infection.

Classically, congenital toxoplasmosis is characterized by the triad of hydrocephalus, chorioretinal lesions, and cerebral calcifications.(1) Chorioretinal involvement is present up to 85% of infected subjects before adulthood.(2) In two large, retrospective, population-based studies, 28% to 50% of children diagnosed with congenital toxoplasmosis were found to have hydrocephalus.(3)

Cerebral toxoplasmosis is considered to be the most common opportunistic infection affecting the central nervous system in patients with HIV/AIDS.(4) However, hydrocephalus in the setting of cerebral toxoplasmosis without an associated mass lesion is an extremely rare; it has been described in fewer than 5 case reports.(5)

Though we cannot directly determine the chronicity of our patient’s conditions, it is possible that his hydrocephalus was longstanding based on the relatively normal intracranial pressure (16 cm fluid height) obtained upon fenestration of the 3rd ventricle by the neurosurgical team—a so-called “compensated” hydrocephalus. His discs, however, lacked the hallmarks of swelling secondary to chronic ICP elevation, including gliosis of the nerve fiber layer and optociliary shunt vessels. The symptoms of visual decline and headaches were also newly realized by the patient, suggesting at least an acute-on-chronic course.

Our patient initially had a good outcome with a shunting procedure, including resolution of his headache and subjective improvement in his vision. Unfortunately, serologic testing for toxoplasmosis was not obtained prior to his discharge, and he did not return to our service for follow-up. His particular presentation was suggestive of a unifying diagnosis: the cerebral and retinal stigmata of congenital toxoplasmosis. However, it is impossible to confirm this diagnosis without serologic evidence and further surveillance of the course of the disease.