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This article reports the surgical management of a 3-month-old girl with Saethre-Chotzen syndrome, who presented with bicoronal synostosis and a large midline sinus pericranii with abnormal cerebral venous drainage via scalp veins. Raised intracranial pressure was demonstrated on monitoring, indicating the need for calvarial expansion necessitating a coronal access incision. A 2-staged delayed raising of the coronal flap was performed to reduce the potential risk of cerebral venous infarction. Monitoring for clinical sequelae and a computerised tomography venogram followed each of these procedures, demonstrating successful redirection of the venous drainage of the brain posteriorly. Finally, a successful fronto-orbital advancement and remodeling procedure was performed with no complications.

Original publication

DOI

10.1097/SCS.0000000000004786

Type

Journal

J craniofac surg

Publication Date

10/2018

Volume

29

Pages

1956 - 1959

Keywords

Acrocephalosyndactylia, Cerebral Veins, Computed Tomography Angiography, Craniosynostoses, Female, Humans, Infant, Intracranial Hypertension, Sinus Pericranii, Surgical Flaps