Purpose: 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 centre in India. Methods: 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 BCVA of 20/200, persistent or recurrent exudative retinal detachment, persistent tumor, or disease recurrence. Results: A total of 29 patients (32 eyes) with SWS/PPV and associated DCH were included. The mean age at presentation was 25.3 ± 11.6 years, with slight female predominance (59%). 90% patients were SWS while only 10% were PPV. Exudative retinal detachment and glaucoma were observed in 50% and 41% of eyes, respectively. The mean tumor diameter and thickness at presentation were 8.31 and 4.16 mm, respectively. In selected cases, treatment included plaque brachytherapy, trabeculectomy, external beam radiotherapy, and intravitreal anti-VEGF. Complete tumor regression was achieved in 22% of eyes. Visual acuity improved significantly from a mean BCVA of 20/1200 to 20/160 at the final follow-up. Poor outcome was noted in 69% of eyes. On multivariate analysis, persistent tumor (p= 0.008) and tumor recurrence (p= 0.03) were independent predictors of poor outcome. Conclusion: 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.
Prabha et al. (Mon,) studied this question.