Gorlin-Goltz syndrome

Case contributed by Yaïr Glick
Diagnosis certain

Presentation

Motor vehicle accident.

Patient Data

Age: 60 years
Gender: Female
ct

Left parietal scalp hematoma.

Signs of known Gorlin-Goltz syndrome:

  • mild macrocephaly and frontal bossing

  • falcine, tentorial and mild petroclival ligament calcification

  • bridging of the sella turcica

  • maxillary odontogenic keratocysts, contiguous with the maxillary sinuses

  • status post removal of basal cell carcinoma from the left parietal scalp, superior to the hematoma

Pineal gland measuring 13 x 16 x 10 mm, with a large cystic component isodense to CSF and peripheral calcification - may represent a pineocytoma.

Status post bilateral maxillary antrostomy, posterior ethmoidectomy, and inferior turbinectomy. The bony walls of the maxillary sinuses are markedly thickened, as is the mucosa.

x-ray

Chest x-ray from the same day as the CT.

Chest unremarkable.

Bifid right 2nd rib - additional sign of Gorlin-Goltz syndrome.

Case Discussion

Known diagnosis of Gorlin-Goltz syndrome with relatively mild manifestations.

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