|Year : 1991 | Volume
| Issue : 2 | Page : 82-83
Iris neovascularization in Sturge-Weber syndrome
Lalit Verma, Atul Kumar, SP Garg, PK Khosla
Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All Institute of Medical Sciences, New Delhi-110 029, India
S P Garg
Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All Institute of Medical Sciences, New Delhi-110 029
Source of Support: None, Conflict of Interest: None
An unusual case of Sturge-Weber syndrome with raised intraocular pressure is presented. The slit lamp examination and anterior segment fluorescein angiography were consistent with iris neovascularization. Cause and effect relationship of iris neovascularization with glaucoma in this syndrome is discussed.
Keywords: Sturge-Weber Syndrome, Iris Neovascularization, Glaucoma.
|How to cite this article:|
Verma L, Kumar A, Garg S P, Khosla P K. Iris neovascularization in Sturge-Weber syndrome. Indian J Ophthalmol 1991;39:82-3
| Introduction|| |
Sturge-Weber syndrome is a phakomatosis involving mainly the cerebellar vessels and facial vessels .
Two ocular features of the syndrome are glaucoma and choroidal hemangioma. Other less common ocular features are heterochromia of the iris, lens dislocation, conjunctival angiomas and retinal vascular tortuosity .
Neovascularization of the iris in Sturge-Weber syndrome, even in cases with advanced glaucoma, is rare and has not received much attention. We report a case of Sturge-Weber syndrome associated with iris neovascularization.
| Case report|| |
A 14- years old male came to us with painful diminution of vision and redness of the right eye of two months duration. There was no record of any similar episode in the past, nor was there any history of headache, convulsions or any neurological deficit. Family history was insignificant. The patient was of normal intellect. Systemic examination including detailed neurological checkup did not reveal any abnormality.
There was evidence of facial hypertrophy on the right side along with a reddish discolouration, suggestive of cutaneous angiomatosis [Figure - 1]
Ocular examination revealed visual acuity of 20/200 OD and 20/20 OS. Left eye examination was within normal limits. The bulbar conjunctiva of the right eye showed a capillary hemangioma near the limbus in its infero-nasal area. There was associated ciliary congestion in the same eve. The cornea was transparent and anterior chamber depth within normal limits. Slit lamp examination of the iris revealed a network of peripupillary new vessels. The pupil size was 6 mm, dilated and fixed. Lens was clear and in its normal position. Fundus examination revealed an elevated mass just temporal to the disc. Applanation intraocular pressure measured 50 mm Hg OD and 16 mm Hg OS. Gonioscopy showed an open angle in both eyes with no neovascularization or abnormalities in the angle. The coefficient of outflow (C-Value) was calculated to be 0.21 ul/min/mm Hg.
Routine investigations were within normal limits. X-ray skull did not reveal any 'fication and CT scan was normal. B-Scan echography (Sonometrics Inc.) of the right eye picked up a raised mass (1.5 - 2.0 mm elevation) with high internal reflectivity and good sound transmission, suggestive of a choroidal hemangioma [Figure - 2]. Anterior segment fluorescein angiography revealed leaking rubeotic vessels along the pupillary margin and iris surface [Figure - 3]. There was also dye leakage over the conjunctiva corresponding to the region of conjunctival hemangioma [Figure - 3]
| Discussion|| |
The typical finding of ipsilateral nevus flammeus of the face [Figure - 1], glaucoma and choroidal hemangioma [Figure - 2] in our case established the diagnosis of SturgeWeber syndrome.
Glaucoma is a common ocular feature of Sturge-Weber syndrome. It usually occurs when facial angioma involves the lids sub . The pathogenesis of glaucoma remains a subject for debate . Various theories' which have been put forward to explain the occurrence of glaucoma in this syndrome include (i) Congenital malformations of the anterior chamber angle (ii) prominent trabecular vessels (iii) raised episcleral venous pressure and (iv) hypersecretion of aqueous.
Although abnormally dilated vessels on the episclera and iris have been described , occurrence of iris neovascularization in Sturge-Weber syndrome has not been described. The cause of iris hyperfluorescence in our patient on angiography could be (i) hemangioma of iris (ii) rubeosis irides. Hemangiomas of the iris or ciliary body, or both, are extremely rare. Ferry, while reviewing the literature on hemangiomas of iris, reported that the diagnosis of hemangioma of the iris is difficult and suggested, when diagnosing any vascular lesion of the iris conditions such as xanthogranuloma, malignant melanoma and rubeosis irides should definitely be ruled out . The finding of typical red coloured network of peripupillary vessels along with progressive hyperfluorescence (leak) on anterior segment angiography [Figure - 3] supported the presence of rubeosis irides, in our patient.
Association of rubeosis with end-stage glaucoma and chronic retinal detachment is well known. The cause(s) of rubeosis irides in Sturge-Weber syndrome, however. is not clear. The possibilities which could be considered in such cases include (i) end-stage glaucoma (ii) old retinal detachment (iii) primarily a part of the disease.
| References|| |
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Duke-Elder S.. System of Ophthalmology. Vol Ill. Part II p. 1124. Henry Kimpton. London, 1964.
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[Figure - 1], [Figure - 2], [Figure - 3]