線状皮脂腺母斑症候群の眼所見

Ocular involvement associated with linear nevus sebaceous syndrome

　線状皮脂腺母斑症候群の2症例に眼科的検索を行い，広範な母斑を伴う1例の同側眼底に，乳頭上鼻側方向に脈絡膜の低色素を主病変とし，同部の脈絡膜，強膜の低形成が混在するという形成異常，ならびに乳頭の生理的陥凹の拡大を認め考察を加えた．

We observed 2 cases of linear nevus sebaceous syndrome with the triad of midfacial nevus sebaceus, seizures, and mental retardation.

The first case, a 11-year-old male, presented with nevus sebaceus of Jadassohn in the left facial hemi-sphere, focal EEG abnormality in the left occipital cortex and marked dilatation of the left temporal subarachnoideal space. The left fundus was affected with marked disc cupping, sectorial hypopigmen-tation of the choroid extending upper-nasal from the disc, and scattered patches of hypoplasia of the choroid and the sclera.

The second case was a 10-year-old male with the above triad associated with hemangioma in theforearm and normal ocular findings.

The observed wide spectrum of disorders involv-ing the brain, skin and the eye seemed to represent the multiple ectodermal and mesodermal malfor-mation in a rare congenital neurocutaneous syndrome.