Phakomatosis Pigmentovascularis (Type IIb) With Bilateral Glaucoma and Urrets-Zavalia Syndrome Following MicroPulse TSCPC: A Case Report.

Abstract

Purpose: We are aimed at describing the clinical course and management of a rare case of phakomatosis pigmentovascularis (PPV Type IIb) presenting with bilateral glaucoma, complicated by bilateral Urrets-Zavalia syndrome (UZS) following MicroPulse transscleral cyclophotocoagulation (MP-TSCPC).

Observations: A 6-year-old girl with PPV Type IIb and syndromic glaucoma underwent MP-TSCPC as an initial surgical intervention for uncontrolled intraocular pressure (IOP). Postoperatively, the patient developed bilateral fixed dilated pupils consistent with UZS. Due to persistent IOP elevation and the complication of UZS, bilateral deep sclerectomy (DS) was performed, resulting in adequate IOP control. (10-mmHg OD, 17-mmHg OS) and gradual improvement in pupillary function. No further complications were observed. The case was classified under PPV Type II based on the coexistence of vascular and pigmentary anomalies. A review of the literature confirms the rarity of such a constellation of findings and the sparsity of reported bilateral UZS following MP-TSCPC.

Conclusion and importance: This case highlights that PPV Type IIb requires careful examination because of the risk of associated glaucoma. UZS should be considered as a possible complication after MP-TSCPC, especially when both eyes are treated. In complex cases with vascular involvement, DS can provide effective and safe IOP control.