Saethre-Chotzen syndrome: long-term outcome of a syndrome-specific management protocol.

ABSTRACT:

Aim

To assess the long-term outcomes of our management protocol for Saethre-Chotzen syndrome, which includes one-stage fronto-orbital advancement.

Method

All patients born with Saethre-Chotzen syndrome between January 1992 and March 2017 were included. Evaluated parameters included occipital frontal head circumference (OFC), fundoscopy, neuroimaging (ventricular size, tonsillar position, and the presence of collaterals/an abnormal transverse sinus), polysomnography, and ophthalmological outcomes. The relationship between papilledema and its associated risk factors was evaluated with Fisher's exact test.

Results

Thirty-two patients (21 females, 11 males) were included. Median (SD) age at first surgery was 9.6 months (3.1mo) for patients who were primarily referred to o

SUBMITTER: Den Ottelander BK 

PROVIDER: S-EPMC7754116 | biostudies-literature | 2021 Jan

REPOSITORIES: biostudies-literature