syndromic craniosynostosis
Pronunciation: kray-nee-oh-sin-oh-STOH-sis
Premature skull suture fusion occurring as part of genetic syndromes, requiring multidisciplinary developmental management.
Full Definition
Early closure of skull sutures associated with genetic syndromes such as Apert, Crouzon, or Pfeiffer syndrome, distinguished from isolated craniosynostosis by additional systemic features. These conditions often involve multiple sutures and are associated with increased intracranial pressure, developmental delays, and complex medical needs. Management requires coordinated care from neurosurgery, plastic surgery, ophthalmology, and developmental specialists. Cognitive outcomes vary significantly based on syndrome type and surgical timing.
Usage
Usage note: Always specify the associated syndrome when known
In Context
- "The patient has syndromic craniosynostosis associated with Apert syndrome." — Craniofacial clinic note
- "Early intervention is crucial for children with syndromic craniosynostosis to optimize development." — Multidisciplinary care plan