Sympathetic Ophthalmia in a 22-Month-Old Infant With Sturge–Weber Syndrome With Atypical Histopathological Correlation

Abstract

Purpose:

Sturge–Weber syndrome (SWS) and sympathetic ophthalmia (SO) are unusual pathologies. Their association has not been previously reported. This unusual diagnosis can be elucidated by clinical suspicion.

Methods:

Case report of a 22-month-old female with SO.

Results:

SWS with a unilateral diffuse choroidal hemangioma. The exciting eye had multiple surgeries for glaucoma that required enucleation. Clinical picture of SO was found on the sympathizing eye. Atypical histopathology (nongranulomatous choroidal infiltrate and perivascular mononuclear cells) is found in the enucleated eye.

Conclusions:

To the best of the authors’ knowledge, this is the first description of an association of SWS, diffuse choroidal hemangioma, infantile glaucoma, and SO. Many confounding factors are present (ie, multiple surgeries, risk of infectious endophthalmitis). Therefore, a high index of suspicion must be kept in mind to make the diagnosis. We aim to raise awareness of such a devastating condition in a pediatric patient with many comorbidities.