Diffuse Choroidal Hemangioma in Sturge-Weber Syndrome: Correlation between Clinical Features, Imaging, and Treatment...

The aim of this study was to describe the clinical features, multimodal imaging characteristics, treatment outcomes, and prognostic factors in patients with diffuse choroidal hemangioma (DCH) associated with Sturge-Weber syndrome (SWS)/phakomatosis pigmentovascularis (PPV) at a referral center in India.

A retrospective observational study was conducted between 2013 and 2024 on patients diagnosed with SWS/PPV and associated DCH. A poor outcome was defined as a final best-corrected visual acuity (BCVA) of 20/200, persistent or recurrent exudative retinal detachment, persistent tumor, or disease recurrence.

A total of 29 patients (32 eyes) with SWS/PPV-associated DCH were included. The mean age at presentation was 25.3 ± 11.6 years, with a female predominance (59%). Most patients had SWS (90%), while 10% had PPV. Exudative retinal detachment and glaucoma were observed in 46% and 31% of SWS eyes, and 66% and 83% of PPV eyes, respectively. The mean tumor basal diameter and thickness were 8.31 mm and 4.16 mm at presentation. Selected cases were managed with plaque brachytherapy, trabeculectomy, external beam radiotherapy, or intravitreal anti-VEGF injections. Complete tumor regression was achieved in 22% of eyes. Mean BCVA improved significantly from 20/1200 to 20/160 at final follow-up; however, a poor visual outcome occurred in 69% of eyes. On multivariate analysis, persistent tumor (p = 0.008) and tumor recurrence (p = 0.03) were independent predictors of poor outcome.

DCH in SWS is a vision-threatening condition requiring individualized multimodal treatment. Although anatomical and visual improvement is achievable, recurrence and tumor persistence are common and predict poor outcomes. Thus, long-term follow-up and early intervention are critical for optimal management.