Visual field loss in Sturge-Weber syndrome and phakomatosis pigmentovascularis: Implications for glaucoma assessment.
In a retrospective cohort of 86 patients with Sturge-Weber syndrome or phakomatosis pigmentovascularis, glaucoma was the predominant source of visual-field loss, while neurological and radiotherapy-associated defects could mimic glaucomatous damage.
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In a retrospective cohort of 86 patients with Sturge-Weber syndrome or phakomatosis pigmentovascularis, glaucoma was the predominant source of visual-field loss, while neurological and radiotherapy-associated defects could mimic glaucomatous damage.
Research significance
The evidence shows clinically distinguishable patterns of glaucomatous, neurological, combined, and post-radiotherapy visual-field loss; by inference, improved interpretation of serial perimetry could reduce misclassification and support better glaucoma-management decisions, but the study does not test whether this approach changes treatment or preserves vision.
Source abstract
OBJECTIVES: To evaluate the patterns of visual field (VF) defects in patients with Sturge-Weber syndrome (SWS) and phakomatosis pigmentovascularis (PPV) using standard automated perimetry. METHODS: This retrospective study analyzed the clinical and VF data of patients with SWS or PPV undergoing treatment for glaucoma and had VF test data over a 27-year period. VF defects were classified as glaucomatous, neurological, combined, or unclassifiable. Statistical analyses were performed to determine factors associated with type of field defects. RESULTS: A total of 86 patients (172 eyes) were included, with 62 (72%) having SWS and 24 (28%) PPV. Glaucoma was present in 49.2% of SWS eyes and 91.6% of PPV eyes. Reliable VF tests were more common in SWS than in PPV ( P < 0.001). Glaucomatous defects were observed in 35.5% of eyes, 7.7% had neurological defects, and 4.1% had combined defects. Advanced field loss was the most common glaucomatous defect (54%). Eyes treated with external beam radiotherapy for diffuse choroidal hemangioma developed localized field defects mimicking glaucoma and homonymous hemianopia. CONCLUSIONS: Glaucoma remains the primary cause of VF loss in SWS and PPV, with a significant proportion of patients demonstrating advanced glaucomatous damage. Accurate interpretation of VF results is crucial for differentiating glaucomatous progression from other ocular and neurological causes in these rare phakomatoses.