Maxillary sinus augmentation in a patient with hereditary angioedema with normal C1 inhibitor and familial Mediterranean fever

Abstract

Hereditary angioedema (HAE) with normal C1 inhibitor (C1-INH) (HAE-nC1-INH) is a rare disease that presents with laryngeal edema due to oral surgical procedures. Familial Mediterranean fever (FMF) is a disorder commonly characterized by an autosomal recessive inflammatory process, and manifests in the oral cavity and facial structures. A 50-year-old woman with HAE-nC1-INH and FMF was referred to our department for bone augmentation in the right maxillary molar region. We performed lateral window sinus augmentation under the backup support of an anesthesiologist. A hematologist used intravenous Berinert® and oral Orladeyo® to prevent perioperative angioedema attacks. The postoperative course was uneventful. Regarding surgical intervention in patients with HAE, interdepartmental cooperation is crucial to prevent angioedema attacks and prepare for life-threatening complications. Oral hygiene and occlusion should be considered in the dental implant treatment of patients with FMF. Every oral and maxillofacial surgeon and dental practitioner should familiarize themselves with HAE and FMF.