Choroidal Vascular Pattern in Cases of Sturge-Weber Syndrome.

Purpose: To study the choroidal vascular pattern in patients with Sturge-Weber syndrome (SWS) using swept-source OCT (SS-OCT).

Design: Prospective comparative observational study.

Participants: All patients with SWS with no history of prior treatment for posterior segment pathology were included.

Methods: Both eyes of all patients were studied using fundus imaging, SS-OCT, fundus fluorescein angiography (FFA), and indocyanine green angiography (ICG) by 2 independent observers.

Main Outcome Measures: The FFA and ICG were screened for any vascular abnormalities. The SS-OCT was evaluated for choroidal changes.

Results: A total of 34 eyes of 17 patients with diagnosed SWS in the age group 9 to 26 years were studied. The FFA and ICG in 7 and 11 patients, respectively, showed some vascular abnormalities. SS-OCT was performed in all patients. The diffuse choroidal hemangioma (DCH) was characterized by loss of the choroidal vascular pattern, increase in the choroidal thickness and loss of visualization of the sclerochoroidal interface. Based on the FFA, ICG, and SS-OCT imaging, there were 3 patients with no DCH, 5 with bilateral DCH, and the remaining 9 patients had unilateral DCH. The detection rate was 50% clinically: 52.94% with FFA, 82.35% with ICG, and 86.36% with SS-OCT. There was substantial agreement between the 2 observers for all 3 investigations.

Conclusion: SS-OCT is a reliable noninvasive imaging modality for early diagnosis and follow-up of DCH over time.