Table 1.

ECCL diagnostic criteria purposed by Hunter [9] and modified by Moog [10].

System	Major criteria	Minor criteria
Skin	Proven nevus psiloliparus (NP) Possible NP and >1 of the minor criteria 2–5 >2 Minor criteria 2–5	Possible NP Patchy or streaky non-scarring alopecia without fatty nevus Subcutaneous lipoma(s) in frontotemporal region Focal skin aplasia/hypoplasia on scalp Small nodular skin tags on eyelids or between outer canthus and tragus
Ocular	Choristoma with or without associated anomalies	Corneal and other anterior chamber anomalies Ocular and eyelid coloboma Calcification of globe
Central Nervous System	Intracraniallipoma Intraspinallipoma Two of the minor criteria	Abnormal intracranial vessels, e.g., angioma, excessive vessels Arachnoid cyst or other abnormalities of meninges Complete or partial atrophy of hemisphere Porencephalic cysts(s) Asymmetrically dilated ventricles or hydrocephalous Calcification (not basal ganglia)
Other System	Jaw tumor (osteoma, odontoma, ossifying fibroma Multiple bone cyst Coarctation of aorta