Temporary Exudative Retinal Detachment following Photodynamic Therapy in a Patient with Retinal Capillary Hemangioblastoma.

A 21-year-old male with von Hippel-Lindau syndrome was treated for a retinal hemangioblastoma with photodynamic therapy (PDT). We employed the standard PDT protocol with doubled treatment duration. Seven days after the procedure, there were some perilesional intra- and subretinal hemorrhages and significant exudative retinal detachment extending inferiorly. Four weeks later, the hemorrhages had mostly cleared, and diminished subretinal fluid and partial regression of the hemangioblastoma were noted. PDT might induce temporary subretinal fluid accumulation in eyes with retinal hemangiomas.

INTRODUCTION

Retinal capillary hemangioblastoma may occur sporadically either as an isolated lesion or as the first manifestation of von Hippel-Lindau (VHL) syndrome.1 The natural course of retinal capillary hemangiomas can show progression, stability, or spontaneous regression.2 However, in most eyes, varying amounts of intra- and subretinal hard exudates, epiretinal membrane and tractional retinal detachment, and secondary glaucoma may ensue during the course of the disease. Photodynamic therapy (PDT) has been performed in these cases with moderate success.

CASE REPORT

A 21-year-old otherwise healthy man presented with visual loss in the left eye of 4 months’ duration. His best corrected visual acuity was 20/20 in the right eye and 20/100 in the left eye. Slit-lamp examination and intraocular pressure were normal in both eyes. On funduscopy, there was a retinal capillary hemangioblastoma, approximately two disc diameters in size, in the inferior temporal quadrant, with prominent feeder vessels and submacular exudation in left eye [Figure 1]. Fluorescein angiography showed the classic signs of hemangioma. The patient had no family history of VHL syndrome. A systemic examination for VHL syndrome was negative but genetic testing for VHL syndrome turned out to be positive. We elected to proceed with PDT as the initial treatment. Intravenous verteporfin (6 mg/m2 ) (Visudyne®; Novartis AG, Basel, Switzerland) was administered over a 10-minute period. Five minutes after the completion of infusion, laser was delivered over the tumor at an intensity of 600 mW/cm2 using a Reichel-Mainster 2 lens (Ocular Instruments, Bellevue, WA, USA) and 6000 μm spot size. Treatment duration was 166 seconds. The feeder vessel was intentionally not treated. At the first follow-up visit, 7 days after the procedure, there were some perilesional intra- and subretinal hemorrhages and significant exudative retinal detachment extending inferiorly [Figure 2]. After 4 weeks, the hemorrhages had partially cleared and the subretinal fluid had diminished substantially [Figure 3]. The hemangioblastoma had slightly decreased in size, with some fibrosis.

Figure 1

Composite color picture of fundus shows a two-disc-diameter retinal angioma with feeder and draining vessels and macular exudation

Figure 2

Composite color picture of fundus taken a week after photodynamic therapy shows the perilesional intra- and sub-retinal hemorrhage as well as the exudative retinal detachment extending inferiorly

Figure 3

Color picture of fundus taken 4 weeks after photodynamic therapy shows fewer hemorrhages, diminished subretinal fl uid, and partially regressed hemangioblastoma

DISCUSSION

PDT is still a valid treatment option for retinal hemangiomas.3–7 While some authors prefer the standard protocol used in wet age-related macular degeneration,5–8 others use modified PDT protocols such as halving the infusion time to 5 minutes3 and/or prolonging the exposure34 to 166 seconds. PDT is either applied over the tumor and/or the feeding and draining vessels.3

Several complications following PDT have been reported previously in eyes with retinal angiomatosis. For example, increased traction may occur after PDT and there may even be tractional retinal detachment, requiring vitreoretinal surgery.3 However, an increase in traction may also occur due to the normal progression of the disease. On the other hand, acute exudative detachment following PDT is rarely reported in eyes with retinal angiomatosis. Wong et al.5 treated a solitary retinal hemangioblastoma with standard age-related macular degeneration PDT protocol and reported that a week later there was increased macular edema, subretinal hemorrhage, and exudative retinal detachment surrounding the hemangioma. The subretinal fluid completely resolved after 5 weeks. The authors proposed that exudative detachment might be the result of increased leakage from the tumor or breakdown of the vascular barrier due to PDT-induced shutdown of the choriocapillaris and retinal capillaries. Szabo et al.8 described a patient with a large retinal angioma that was treated with standard PDT protocol. A day after PDT treatment, subretinal fluid had accumulated under the macula, but this subsided spontaneously within 12 days.

The standard PDT regimen is often somewhat arbitrarily modified in cases with various vascular tumors. We have preferred a prolonged exposure time of 166 seconds and have obtained successful outcomes in optic disc hemangioma, secondary vasoproliferative tumor, and vascularized granuloma.9–11 However, prolonged exposure time might have been the cause of the exaggerated response of subretinal exudation in the present case.

In summary, one must be cognizant of the fact that the temporary exudative retinal detachment seen following PDT in eyes with retinal angiomatosis can occur without adverse long-term sequelae.