Photodynamic therapy (PDT) has been used in treating peripheral retinal capillary hemangioma (RCH) with satisfactory results. We report a rare case of von Hippel-Lindau (VHL) disease with three large peripheral RCHs, treated with PDT and intravitreal bevacizumab injection (IVB), who developed persistent bullous exudative retinal detachment (RD) despite significant tumor regression. The patient is a sporadic case of VHL disease, with a de novo nonsense mutation in codon 161 with C → T transition at nucleotide position 694 of the VHL gene. Multiple RCHs were noted in both eyes. Four small RCHs were found in the left eye and were treated with laser photocoagulation. Three large RCHs in the peripheral retina of the right eye were complicated with cystoid macular edema and subretinal fluid accumulation. The RCHs were treated with PDT combined with IVB, and bullous exudative RD developed on the second day after treatment. Three months after PDT, the tumors had regressed significantly, but exudative RD persisted, despite multiple IVB and intravitreal triamcinolone acetonide injection (IVTA). External drainage with sclera buckling, IVB, and IVTA were performed, and the retina attached after surgical intervention. The application of PDT in the treatment of RCHs and its possible complications are discussed.
Retinal detachment with a break at the pars plicata associated with congenital malformation of lens–zonule–ciliary body complex is rare; most reports are of young Japanese male patients with atopic dermatitis. The present case report is the first to describe the condition in a Chinese patient with no atopic dermatitis or trauma history. A 22-year-old male presented with blurred vision in the left eye for 4 months. Fundus examination revealed shallow lower temporal retinal detachment. Further examination with scleral indentation under maximal pupil dilatation identified a break at the far periphery beyond the ora serrata and pars plana. Gonioscopy revealed a pars plicata break at the nonpigmented ciliary epithelium associated with congenital ciliary process hypoplasia and subtle lens defect at the same meridian. The retina was successfully reattached after segmental scleral buckling, cryopexy, and laser photocoagulation.
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