Sir,

Central retinal vein occlusion associated with Sturge Weber syndrome is rare, to our knowledge there are only two other reported cases in the literature.1,2 We report such a case.

Case report

A 22-year-old male patient with left-sided Sturge Weber syndrome and well-controlled ipsilateral glaucoma, presented to the eye casualty department with a left central retinal vein occlusion (Figure 1). The visual acuity was 6/36 in the left eye and intraocular pressure 18 mmHg. The cornea was clear and there was no evidence of rubeosis iridis. A relative afferent pupilliary defect was noted prior to dilated fundoscopy, which revealed the typical features of a central retinal vein occlusion. Routine blood investigations, including a thrombophilia screen, were all normal, and antithrombin III and plasminogen levels were all within normal limits.

Figure 1
figure 1

Central retinal vein occlusion in the left eye.

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The patient suffering from Sturge Weber syndrome originally presented to the eye department at 9 years of age, with an elevated intraocular pressure of 34 mmHg in the left eye, the right was 20 mmHg. The visual acuity was 6/6 in each eye and the optic discs were healthy (Figure 2). The left corneal diameter was 2 mm greater than the right. A visual field test revealed a right homonymous hemianopia, which was attributed to Sturge Weber syndrome. He was known to be epileptic and controlled with sodium valproate and carbamazepine. There was no other significant medical history. A diagnosis of Sturge Weber syndrome-related glaucoma was made and treated with guttae timoptol 0.5%. Over the following 13 years, the intraocular pressure in the left eye remained well controlled.

Figure 2
figure 2

The left eye with a healthy optic disc and diffuse choroidal haemangioma.

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Three weeks after presentation with the CRVO, he developed an acute painful red eye. Examination revealed an elevated intraocular pressure of 52 mmHg and rubeosis iridis. A diagnosis of neovascular glaucoma was made and the eye was treated with guttae pred forte 1% q.d.s., guttae atropine 1% b.d. and oral acetozolamide 250 mg q.d.s. Extensive panretinal photocoagulation (PRP) was performed. Unfortunately despite maximal treatment, the IOP remained elevated, which required cyclodiode laser treatment. Over the next 4 months, two further treatments of laser PRP were needed and cyclodiode laser treatment was repeated. At last review the eye was pain free with an IOP of 30 mmHg on guttae timoptol. The vision was counting fingers.

Discussion

Central retinal vein occlusion is a condition more commonly attributed to the elderly, when it is associated with arteriosclerosis, hypertension, hyperlipidaemia, hypercoagulability and uncontrolled glaucoma.3 It is less common in patients under 50 years of age when in the vast majority of cases, there is no known association with systemic disease.4 However, in the case of bilateral and recurrent CRVO, a systemic cause should be suspected, especially a hyperviscosity or hypercoagulation state, such as abnormal platelet function or anticoagulant protein deficiencies.5,6 Local ocular conditions such as uncontrolled glaucoma and optic nerve drusen4,7 should also be excluded in unilateral cases, as these conditions have been reported in association with CRVO.

The prognosis for young patients suffering from CRVO is generally good, with most cases resolving spontaneously over the following 6 months, however the final visual outcome, is related to the amount of retinal ischaemia. Neovascular glaucoma occurs in approximately 20% of patients and is treated with panretinal photocoagulation.4

A patient suffering from Sturge Weber syndrome (encephalofacial angiomatosis), typically suffers from the quartet, of port wine staining (naevus flammeus) of the face roughly in the distribution of the first, second or third divisions of the trigeminal nerve, ipsilateral intracranial haemangioma, choroidal haemangioma, and congenital glaucoma. Incomplete forms exist.8

We know of only two previous reports of CRVO in association with Sturge Weber syndrome, one of which describes a patient who also had disc drusen in addition to Sturge Weber syndrome.1,2 Our patient had been maintained at an intraocular pressure, which, in this young patient, prevented the development of any optic nerve fibre damage. Despite this, the intraocular pressure may have been high enough to predispose to CRVO in an eye with the vascular anomalies of Sturge Weber syndrome.