require treatment. The retinopathy of IP has been managed by photocoagulation or cryotherapy with varying degrees of success. Treatment is usually applied primarily to the avascular peripheral retina, similar to the management of retinopathy of prematurity.

O’Doherty M, McCreery K, Green AJ, Tuwir I, Brosnahan D. Incontinentia pigmenti—ophthalmological observation of a series of cases and review of the literature. Br J Ophthalmol. 2011;95(1):11–16.

Wyburn-Mason Syndrome

Wyburn-Mason syndrome, also known as Bonnet-Dechaume-Blanc syndrome or racemose angioma, is a nonhereditary arteriovenous malformation of the eye and brain, especially midbrain, that typically involves the ipsilateral optic disc or retina. Skin lesions are present in approximately half of patients and usually occur on the face. The complete syndrome is considerably less common than an isolated occurrence of similar ocular or intracranial disease. Seizures, mental changes, hemiparesis, and papilledema may result from the brain lesions, which are frequently a source of hemorrhage (unlike the vascular brain lesions of SWS).

Ocular manifestations are unilateral and congenital, and they may progress during childhood. The typical lesion consists of markedly dilated and tortuous vessels that shunt blood directly from arteries to veins. These vessels do not leak fluid (Fig 28-15). Vision ranges from normal to markedly reduced. Intraocular hemorrhage and secondary neovascular glaucoma are possible complications. More than half of affected eyes are blind, and an additional one-quarter have severe visual impairment. No treatment is indicated for primary lesions. Treatment may be considered for associated complications, such as scatter photocoagulation for ischemic venous occlusive disease, vitrectomy for nonclearing vitreous hemorrhage, and cyclodestructive treatment for neovascular glaucoma.