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Jugular foraminal stenosis in Crouzon syndrome
D Martinez-Perez1, D L Vander Woude, P D Barnes
1Craniofacial Center, Children's Hospital, Harvard Medical School, Boston, Mass., USA.
Pediatric Neurosurgery
|November 1, 1996
Summary
A boy with Crouzon syndrome developed increased intracranial pressure and a scalp arteriovenous fistula due to jugular vein atresia. This case highlights rare venous complications in syndromic craniosynostosis.
Area of Science:
- Neurology
- Genetics
- Pediatric Surgery
Background:
- Crouzon syndrome is a craniosynostosis often associated with other congenital anomalies.
- Increased intracranial pressure (ICP) can necessitate surgical intervention.
Observation:
- A 4-year-old boy with Crouzon syndrome, acanthosis nigricans, and Chiari-I malformation presented with elevated ICP requiring posterior cranial expansion.
- Post-surgery, he developed a mastoid arteriovenous fistula.
- Cerebral angiography revealed bilateral jugular vein atresia and sinus occlusions.
Findings:
- The patient's condition was attributed to increased intracranial venous pressure from bilateral jugular foraminal stenosis, leading to hydrocephalus and scalp venous dilation.
- This venous dilation likely predisposed the scalp to traumatic arteriovenous fistula formation.
Implications:
- Jugular atresia is a basilar malformation linked to syndromic craniosynostoses and achondroplasia.
- This case demonstrates a novel association with fibroblast growth factor receptor 3 mutation and highlights potential vascular complications post-neurosurgery.