Abstract
Objectives:
(1) Describe the first reported case in otolaryngology literature of a patient with Crouzon syndrome with late cerebrospinal fluid (CSF) rhinorrhea and encephalocele formation after prior Le Fort III facial advancement surgery. (2) Review the literature pertaining to the incidence and management of this complication in craniofacial dysostoses (CD). (3) Analyze issues surrounding repair of these complications, including occult elevations in intracranial pressure (ICP), the use of perioperative CSF shunts, and the importance of considering alternative repair schemes.
Methods:
Literature review of studies describing CSF rhinorrhea and encephalocele formation following facial advancement in CD, focusing on management strategies.
Results:
CSF rhinorrhea and encephalocele formation are rare complications of craniofacial advancement procedures. Occult elevations in ICP complicate the prospect of permanent surgical repair, necessitating the use of transcranial repair and CSF shunts in addition to the now more common endoscopic approach. Though no consensus exists regarding the use of perioperative CSF drains, strong associations exist between elevated ICP and failed surgical repair. Additionally, the anatomic changes in the frontal and ethmoid sinuses after facial advancement present a challenge to endoscopic management.
Conclusions:
Otolaryngologists should be aware of the possibility of occult elevations in ICP as well as sinonasal anatomic abnormalities when repairing CSF rhinorrhea in patients with CD. Clinicians should consider CSF shunt placement and carefully weigh the advantages of the open, transcranial approach versus endonasal, endoscopic techniques.
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