Craniosynostosis involving the squamous temporal sutures: a rare and possibly underreported etiology for cranial vault asymmetry.
Craniosynostosis is a condition in which 1 or more cranial sutures fuse prematurely, often secondary to a fibroblast growth factor receptor (FGFR) mutation, typically involving FGFR2 or FGFR3. This mutation may occur sporadically or in the setting of a genetic syndrome and typically presents within the first few days of life or in early infancy. Most commonly, the sagittal and coronal sutures are involved, although involvement of the lambdoidal and/or metopic sutures is not uncommon. Surgical correction is undertaken both for cosmetic purposes and to relieve raised intracranial pressure, both of which can be severe, depending on the sutures involved. We report on 2 children who presented in their first year of life with synostosis involving: in one instance, a single squamous temporal suture, and in the other, both squamous temporal sutures. The initial presentation and clinical courses of these 2 patients are highly distinct from one another, although both ultimately did quite well after extensive cranial remodeling. To the best of our knowledge, only a handful of patients with squamous synostosis have been reported in the medical literature.
Ranger
Adrianna
A
Department of Neurosurgery, Children's Hospital, London Health Sciences Center, Schulich School of Medicine and Dentistry, London, Ontario, Canada. aranger@uwo.ca
Chaudhary
Navjot
N
Matic
Damir
D
D002648:Child; D002675:Child, Preschool; D003393:Cranial Sutures / Q000000981:diagnostic imaging / Q000473:pathology* / Q000601:surgery; D003398:Craniosynostoses / Q000000981:diagnostic imaging / Q000235:genetics / Q000473:pathology* / Q000601:surgery; D003399:Craniotomy; D006801:Humans; D008297:Male; D010027:Osteotomy; D010641:Phenotype; D014057:Tomography, X-Ray Computed