816 A Case of obstructive Sleep Apnea in an Adolescent girl with crouzon syndrome

Abstract

\n \n \n Crouzon syndrome is an autosomal dominant type of craniosynostosis, first reported by French neurologist Octave Crouzon in 1912. Craniosynostosis refers to the premature closure of cranial sutures. The orofacial manifestations of this syndrome includes maxillary hypoplasia, external nasal deformity and prognathism, which can all contribute to breathing difficulties. The prevalence of Obstructive Sleep Apnea (OSA) is above 60%. The severity of obstruction can be life threatening prompting early surgical intervention ranging from tracheostomy to mid facial distraction like LeFort III osteotomy. Report of case(s): An 18-year-old girl with Crouzon syndrome, who was referred for snoring. She had a significant past surgical history of craniotomy and ventricular-peritoneal shunt at the age of 2 years, tracheostomy dependency until the age of 5 years after undergoing a LeFort III osteotomy, and adeno-tonsillectomy at 6 years. She had never had a polysomnography (PSG) before. Her in-lab PSG showed moderate to severe OSA with an Apnea-Hypopnea Index (AHI) of 28.5/hr. She had no central apneas. Obstructive events were not controlled with Continuous Positive Airway Pressure (CPAP), but did respond to Bi-level therapy. Plastic Surgery favored a repeat LeFort III osteotomy and cranioplasty instead. A post-surgical PSG showed resolution of OSA with an AHI of 2.8/hr.\n \n \n \n This case reminds us that patients with craniofacial abnormalities are at higher risk for OSA and are more difficult to treat. Our patient had significant sleep apnea despite prior surgical interventions. Patients with Crouzon syndrome should be studied for OSA, and re-assessed over time. Her repeat surgery had a positive outcome, and avoided PAP therapy. Therefore, Plastic Surgery should always be part of the therapeutic discussion.\n \n \n \n 1. Samuel N, Arvind B, Sameep K and Eugene M (2014) Revisiting Crouzon syndrome: reviewing the background and management of a multifaceted disease. 2. J Sirotnak 1, L Brodsky, M Pizzuto (1995) Airway obstruction in the Crouzon syndrome: case report and review of the literature. 3. Mitsukawa N, Satoh K, Hayashi T, Furukawa Y, Uemura T (2004) A reflectable case of obstructive sleep apnea in an infant with Crouzon syndrome.\n