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Chronic tonsillar herniation and Crouzon's syndrome
P M Francis1, S Beals, H L Rekate
1Division of Neurological Surgery, Barrow Neurological Institute, Phoenix, Ariz 85013.
Pediatric Neurosurgery
|January 1, 1992
Summary
Craniofacial syndrome patients may develop hydrocephalus post-surgery. Shunted patients showed chronic tonsillar herniation, linked to jugular foramen stenosis and venous pressure, suggesting a persistent issue after treatment.
Area of Science:
- Neurosurgery
- Pediatric Plastic Surgery
- Neurology
Background:
- Craniofacial syndromes like Crouzon's syndrome often necessitate surgical intervention.
- Post-operative hydrocephalus is a known complication in patients with craniofacial syndromes.
Observation:
- Ten patients with Crouzon's syndrome were analyzed.
- Five patients required shunting for hydrocephalus after cranial remodeling.
- Chronic tonsillar herniation was observed in all 5 shunted patients via MRI.
Findings:
- No shunted patients were free from chronic tonsillar herniation.
- Patients who did not require shunting did not exhibit tonsillar herniation.
- Jugular foramen stenosis appears to cause increased cerebral venous pressure, leading to CSF absorption defects and hydrocephalus.
Implications:
- The persistent increased venous pressure post-hydrocephalus treatment may drive chronic tonsillar herniation.
- This finding highlights a potential long-term complication following surgical management of craniofacial syndromes.
- Further research into managing jugular foramen stenosis and its impact on venous pressure is warranted.