{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,18]],"date-time":"2026-09-18T10:54:42Z","timestamp":1789728882903,"version":"4.0.1"},"reference-count":30,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2020,9,9]],"date-time":"2020-09-09T00:00:00Z","timestamp":1599609600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Develop Med Child Neuro"],"published-print":{"date-parts":[[2021,1]]},"abstract":"<jats:sec><jats:title>Aim<\/jats:title><jats:p>To assess the long\u2010term outcomes of our management protocol for Saethre\u2013Chotzen syndrome, which includes one\u2010stage fronto\u2010orbital advancement.<\/jats:p><\/jats:sec><jats:sec><jats:title>Method<\/jats:title><jats:p>All patients born with Saethre\u2013Chotzen 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\u2019s exact test.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Thirty\u2010two 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 our center (range: 3.6\u201313.0mo), the median (SD) age at last follow\u2010up was 13 years (5y 7mo; range: 3\u201325y). Seven patients had papilledema preoperatively, which recurred in two. Two patients had papilledema solely after first surgery. Second cranial vault expansion was indicated in 20%. Thirteen patients had an OFC deflection, indicating restricted skull growth, one patient had ventriculomegaly, and none developed hydrocephalus. Eleven patients had emissary veins, while the transverse sinus was aberrant unilaterally in 13 (hypoplastic <jats:italic>n<\/jats:italic>=10 and absent <jats:italic>n<\/jats:italic>=3). Four patients had mild tonsillar descent, one of which was a Chiari type I malformation. Four patients had obstructive sleep apnoea (two mild, one moderate, and one severe). An aberrant transverse sinus was associated with papilledema (<jats:italic>p<\/jats:italic>=0.01).<\/jats:p><\/jats:sec><jats:sec><jats:title>Interpretation<\/jats:title><jats:p>Single one\u2010stage fronto\u2010orbital advancement was sufficient to prevent intracranial hypertension for 80% of our patients with Saethre\u2013Chotzen syndrome. Follow\u2010up should focus on OFC deflection and venous anomalies.<\/jats:p><\/jats:sec>","DOI":"10.1111\/dmcn.14670","type":"journal-article","created":{"date-parts":[[2020,12,20]],"date-time":"2020-12-20T03:56:32Z","timestamp":1608436592000},"page":"104-110","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":16,"title":["Saethre\u2013Chotzen syndrome: long\u2010term outcome of a syndrome\u2010specific management protocol"],"prefix":"10.1111","volume":"63","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4586-0317","authenticated-orcid":false,"given":"Bianca K","family":"Den Ottelander","sequence":"first","affiliation":[{"name":"Department of Plastic and Reconstructive Surgery and Hand Surgery Dutch Craniofacial Center Erasmus MC \u2013 Sophia Children\u2019s Hospital University Medical Center Rotterdam Rotterdam the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marie\u2010Lise C","family":"Van 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