Oral Propranolol for Exudative Retinal Detachment in Diffuse Choroidal Hemangioma

The main abnormality of the uveal tract in patients with Sturge-Weber syndrome is diffuse choroidal hemangioma (DCH). Diffuse choroidal hemangioma can lead to a total retinal detachment and secondary neovascular glaucoma. Radiotherapy and photodynamic therapy are currently the preferred methods of treatment. Low-dose lens-sparing radiotherapy or proton beam irradiation can induce tumor regression and resolution of subretinal fluid.¹ Short-term treatment success using photodynamic therapy has also been reported.²

The use of oral propranolol hydrochloride has been described for infantile orbital³ and periorbital hemangioma. Recently, Léauté-Labrèze et al³ observed that systemic propranolol could inhibit the growth of infantile hemangioma (IH) lesions in children. The response of IH to propranolol catapulted the use of this treatment to first-line status among physicians managing this disease.^3-6

We report the use of oral propranolol for exudative retinal detachment in DCH associated with Sturge-Weber syndrome.

A 58-year-old Hispanic woman had decreased visual acuity in the left eye for 3 years and intermittent episodes of pain and red eye. On examination, her best-corrected visual acuity was 20/20 OD and light perception OS. She had diffuse nevus flammeus involving the left upper eyelid, cheek, and nose, suggestive of Sturge-Weber syndrome. The anterior segment of the left eye demonstrated dilated and tortuous conjunctival vessels, clear cornea, shallow anterior chamber, and normal iris. The intraocular pressure was 27 mm Hg OS, and fundus examination showed exudative retinal detachment in the left eye (Figure 1A) with shifting fluid. Fluorescein angiography (Figure 1B) demonstrated early diffuse hyperfluorescence and late hypofluorescence, and optical coherence tomography (Figure 1C) confirmed the presence of subretinal fluid. B-scan ultrasonography confirmed diffuse thickening of the choroid and an exudative retinal detachment with A-scan high internal reflectivity of the choroidal mass (Figure 1D), corresponding with the diagnosis of a DCH.

An Nd:YAG-laser iridotomy was performed in the left eye, and treatment with oral propranolol hydrochloride (80 mg orally twice a day) was started. At 18 days after the initiation of propranolol treatment, the dosage was reduced to 40 mg twice a day because of secondary effects described by the patient as dizziness and weakness. Six weeks after the initiation of propranolol treatment, best-corrected visual acuity was hand movements OS, intraocular pressure was 21 mm Hg OS, and the retina was attached, with complete reabsorption of subretinal fluid (Figure 2A and B) confirmed by optical coherence tomography (Figure 2C) and ultrasonography (Figure 2D).

Propranolol, a nonselective β-blocker, was serendipitously discovered to induce accelerated involution of a proliferating IH.³ The mechanism by which propranolol causes this dramatic effect is unclear. However, propranolol interferes with endothelial cells, vascular tone, angiogenesis, and apoptosis.⁵

Léauté-Labrèze and colleagues suggested a propranolol hydrochloride dosage of 2 mg/kg/d in children with IH. We treated our patient with a dosage usually used in adults for arterial hypertension. However, we reduced the dosage by half at day 18 because of secondary effects. Nevertheless, this lower dosage was still effective to allow for resolution of the exudative retinal detachment associated with DCH. The optimal dosage in adults for this indication is still unknown. It is possible that lower dosages may be effective and without adverse effects. In addition, the duration of treatment to induce retinal reattachment is currently unknown. However, patients with IH have been treated for several months.

Hemangiomas consist histologically of cavernous and capillary vascular networks. The mechanism by which oral propranolol aids in the resolution of exudative retinal detachment in DCH associated with Sturge-Weber syndrome is unknown. It is possible that, similar to IH, there is vasoconstriction of the DCH due to decreased release of nitric oxide, blocking of proangiogenic signals including vascular endothelial growth factor and basic fibroblast growth factor, and apoptosis in proliferating endothelial cells with vascular tumor regression.⁵

To our knowledge, the benefits of propranolol therapy have not been reported in adult hemangioma or for DCH. This is the first reported case of propranolol treatment in an adult with exudative retinal detachment in DCH associated with Sturge-Weber syndrome.

Correspondence: Dr Arevalo, Vitreoretinal Division, King Khaled Eye Specialist Hospital, Al-Oruba Street, PO Box 7191, Riyadh 11462, Saudi Arabia (arevalojf@jhmi.edu).

Funding/Support: This work was supported in part by the Arevalo-Coutinho Foundation for Research in Ophthalmology, Caracas, Venezuela.

Previous Presentation: This paper was presented at the 34th Annual Meeting of the Macula Society; March 9, 2011; Boca Raton, Florida.