syndromic craniosynostosis
Premature fusion of skull sutures occurring as part of a genetic syndrome with multiple anomalies.
Full Definition
A form of craniosynostosis that occurs in association with genetic syndromes affecting multiple organ systems, such as Crouzon, Apert, or Pfeiffer syndrome. Unlike isolated craniosynostosis, syndromic cases typically involve multiple suture fusions and are associated with additional craniofacial malformations, limb abnormalities, and potential cognitive impairment. Treatment requires multidisciplinary management addressing not only the cranial deformity but also associated anomalies.
Usage
Usage note: Distinguish from isolated/nonsyndromic craniosynostosis which occurs without associated genetic syndromes.
In Context
- "The genetic counselor confirmed syndromic craniosynostosis associated with Apert syndrome." — Diagnosis documentation
- "Management of syndromic craniosynostosis requires coordination between neurosurgery, plastic surgery, and genetics teams." — Treatment protocol