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Morbidity associated with increased intracranial pressure in Apert and Pfeiffer syndromes: the need for long-term
A K Gosain1, J G McCarthy, J H Wisoff
1Variety Center for Craniofacial Rehabilitation Institute of Reconstructive Plastic Surgery, NYU Medical Center, New York, USA.
Insights
Patients with craniosynostosis syndromes face risks from elevated intracranial pressure throughout life. Regular monitoring is crucial to prevent serious complications like brainstem herniation and vision loss.
Area of Science:
- Neurosurgery
- Pediatric Surgery
- Clinical Genetics
Background:
- Craniosynostosis syndromes are associated with increased intracranial pressure.
- The clinical significance and management of elevated intracranial pressure in these patients are not well-defined.
Observation:
- A review of 106 patients (67 Apert, 39 Pfeiffer) identified morbidity directly related to elevated intracranial pressure.
- Morbidity occurred in 4.7% of patients, including two deaths and three other severe complications.
Findings:
- Complications included brainstem herniation post-Le Fort III advancement and shunt removal during syndactyly release.
- Other complications involved papilledema and venous hypertension leading to delayed surgery.
Implications:
- Patients with syndromal craniosynostoses are at lifelong risk for elevated intracranial pressure complications.
- Regular, long-term clinical evaluation is recommended to mitigate morbidity from unrecognized intracranial pressure elevations.
- Proposed clinical guidelines aim to improve patient outcomes through consistent monitoring.
Abstract:
Although the incidence of elevated intracranial pressure is increased in patients with the craniosynostosis syndromes, the significance of these findings and their impact on clinical practice remain unclear. The morbidity related to elevated intracranial pressure in patients with Apert and Pfeiffer syndromes was reviewed. Sixty-seven patients with Apert syndrome and 39 patients with Pfeiffer syndrome were identified. The morbidity in 5 of the 106 patients (4.7 percent) was felt to be directly related to persistently elevated intracranial pressure. These included 2 deaths, 1 due to brainstem herniation after an elective subcranial Le Fort III advancement and 1 following removal of a lumboperitoneal shunt during the surgical release of syndactyly. The remaining 3 complications included bilateral papilledema detected 3.5 years after cranial vault remodeling in 1 patient and venous hypertension with excessive bleeding detected during elevation of the bicoronal flap in 2 patients, resulting in delay of a planned Le Fort III advancement. Patients with the syndromal craniosynostoses are at risk for complications resulting from elevated intracranial pressure from infancy through adulthood despite surgical attempts to increase cranial capacity in infancy. Clinical guidelines are proposed by which these patients should be evaluated beyond infancy in a regular fashion so as to reduce morbidity from unrecognized elevations in intracranial pressure.