Author Affiliations: Retina and Vitreous Service, Clínica Oftalmológica Centro Caracas, Caracas, Venezuela. Dr Arevalo is now with the Retina Division, Wilmer Eye Institute, Johns Hopkins University School of Medicine, Baltimore, Maryland, and the Vitreoretinal Division, King Khaled Eye Specialist Hospital, Riyadh, Saudi Arabia.
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.
Figure 1. Initial fundus examination, fluorescein angiography, optical coherence tomography, and ultrasonography. A, Fundus examination shows inferior exudative retinal detachment with shifting fluid. B, Fluorescein angiography demonstrates early diffuse hyperfluorescence and late hypofluorescence. The late-phase fluorescein angiograph is shown. C, Optical coherence tomography demonstrates an exudative retinal detachment. S indicates superior; T, temporal; I, inferior; and N, nasal. D, B-scan ultrasonography confirms diffuse thickening of the choroid and an exudative retinal detachment with A-scan high internal reflectivity of the choroidal mass, corresponding with the diagnosis of a diffuse choroidal hemangioma.
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).
Figure 2. Fundus examination, fluorescein angiography, optical coherence tomography, and ultrasonography after treatment. A, Color photograph demonstrates that the retina is attached with a demarcation line (arrows). The inferotemporal vein is elevated and casting a shadow (arrowhead), suggesting the persistence of a small amount of subretinal fluid. B, Fluorescein angiography demonstrates mottled hyperfluorescence due to retinal pigment epithelium changes. No subretinal fluid is seen. Optical coherence tomography (C) and A- and B-scan ultrasonography (D) confirm that the retina is attached. S indicates superior; T, temporal; I, inferior; and N, nasal.
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 (firstname.lastname@example.org).
Financial Disclosure: None reported.
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.
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