Unilateral External Oculomotor Nerve Palsy and Nevus Sebaceous of Jadassohn

Sebaceous lesions and their associated syndromes: Part II

“…Reported eye findings include: strabismus, ipsilateral hypoplasia of the optic radiation with hemimegalencephaly, colobomata, cataracts (usually unilateral), corneal vascularization, ocular hemangiomas, downward slanting of the palpebral fissures, ptosis, ectropion, hamartomas on the eyelid, epibulbar tumors, corneal vascularization, choristomas, scleral fibromas, nonspecific cortical cataracts, peripapillary choroidal atrophy, exudative retinal detachment, generalized retinal degeneration, unilateral proptosis, microphthalmos, and cortical blindness. 3,9,20,22,[25][26][27][28][29][30][31][32][33][34][35][36][37][38][39][40][41][42][43][44] LNSS is one of the most common subtypes of ENS. 3 However, very few nevi are exclusively of one type.…”

“…These include patent ductus arteriosus, patent foramen ovale, ventricular septal defect, coarctation of the aorta, hypoplasia of the aorta, atrial flutter/fibrillation, atrial premature systoles, horseshoe kidney, duplicated urinary collection system, undescended testes, enlarged clitoris, hepatosplenomegaly, and failure to thrive. 17,20,26,32,[36][37][38]42,43 Because of the high incidence of findings other than the classic triad of linear NSJ, seizures, and mental retardation, the criteria to define this syndrome has been expanded. 15 In cases where LNSS is suspected, it is wise to search for other disorders aside from neurologic disorders.…”

Neurological involvement in the epidermal naevus syndrome.

“…Ocular complications were found in a third of Solomon and Esterly's series. Complications described include extension of the naevus into the conjunctiva, subconjunctival lipodermoids, corneal opacities and pannus formation, coloboma of the lid, iris, choroid or retina; antimongoloid lid fissures; ectopic lacrimal glands; generalised retinal degeneration; nystagmus; isolated third nerve palsies; esotropia and teratomas of the orbit (Haslam and Wirtschafter, 1972). Cases 1 and 2 described by Holden and Dekaban (1972) both had haemangiomas involving the sclera as our patient probably did.…”

The Epidermal Nevus Syndrome: Case Report and Review

“…The epidermal nevus syndrome can arise in strikingly unusual forms, many variations of which have beeti previously described (4)(5)(6)(7)(8)(9)(10)(11)(12). Commonly associated generalized abnormalities include skeletal, neurologic, and vascular defects that usually are present at birth or shortly thereafter.…”