Bilateral Sturge-Weber and Phakomatosis Pigmentovascularis with Glaucoma, an Overlap Syndrome

Abstract EN

Aim.

To report a case of bilateral Sturge-Weber and Phakomatosis pigmentovascularis with secondary glaucoma in a child.

Method.

Case report.

Results.

A 4-year-old male child was referred to us for control of intraocular pressure (IOP).

Sleeping IOP was 36 mm Hg in right eye and 28 mm Hg in the left eye.

The sclera of both the eyes showed bluish black pigmentation—melanosis bulbi.

Fundus examination of both eyes showed diffuse choroidal hemangiomas with glaucomatous cupping.

Nevus flammeus was present on both sides of face along all the 3 divisions of trigeminal nerve with overlying hypertrophy of skin and on left forearm.

Nevus fuscocaeruleus was present on upper trunk.

All skin lesions were present since birth and were stationary in nature.

CT scan of head revealed left-sided cerebral atrophy.

Intraocular pressure was controlled after treatment with topical antiglaucoma medications.

Pulsed Dye Laser has been advised by dermatologist for skin lesions.

Patient has been advised for regular follow-up.

Conclusion.

The two overlapping dermatological disorders and their association with glaucoma are a rare entity.

Management should be targeted both for dermatological and eye conditions.