Sinus disease in Apert's syndrome: Durable quality of life improvement following surgical intervention

Abstract

Apert's syndrome is an autosomal dominant condition characterized by craniosynostosis, midface hypoplastia, and syndactyly of the hands and feet. In severe presentations, patients undergo primary.front-orbital advancement during infancy with revisions as necessary. We describe a case of surgical management and clinical outcome in a patient with chronic sinusitis complicated by Apert's syndrome and multiple previous craniofacial surgeries. The complexity of the sinonasal anatomy of this patient amplified the risk of surgical complications. However, we have demonstrated that detailed pre-operative planning and subsequent functional endoscopic sinus surgery can be accomplished safely. Additionally, post-operative SN-5 surveys demonstrate a durable quality of life benefit. Surgical intervention for sinus disease should be considered an option in the management of this patient population.