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Table 1 Diagnostic criteria of Gorlin–Goltz syndrome

From: Multiple odontogenic keratocysts in Ehlers–Danlos syndrome: a rare case report

 

Major criteria

Minor criteria

1

Presence of more than two basal cell carcinomas (BCC) or a history of one BCC below the age of 20 years

Macrocephaly

2

OKC in the jaw (confirmed histologically)

Congenital anomalies—cleft lip-palate, coarse face, hypertelorism, frontal bossing

3

Three or more palmoplantar pits

Skeletal anomalies—Sprengel deformity, pectus deformity, syndactyly

4

Falx cerebri calcification

Radiologic anomalies—sella turcica bridging, vertebral anomalies including hemivertebra and combined vertebral corpi, flame-like lucency on hand and foot X-rays

5

Bifid or combined costae

Medulloblastoma

6

Presence of a diagnosis of Gorlin–Goltz syndrome in a first-degree relative

Ovarian fibroma