Orthognathic Surgery in Ehlers-Danlos Syndrome: Review of the Literature and Case Report

Abstract

EDS (Ehlers-Danlos Syndrome) is a heterogenous group of inheritable connective tissue disorders that commonly precludes patients from being elective surgical candidates. Patients with EDS are at a higher risk of increased bleeding, delayed wound healing, and temporomandibular joint pain refractory to treatment. Historically, patients with EDS and TMJ disorders are considered inappropriate surgical candidates due to a higher risk of delayed wound healing, increased risk for uncontrolled post-surgical bleeding, and unsubstantiated outcomes in regards to elective orthognathic surgery. A review of the literature demonstrates a paucity of data accounting the use of orthognathic surgery and maxillary-mandibular advancement in patients with EDS. The present study reports on the use of orthognathic double jaw surgery in a patient with a known diagnosis of hypermobile EDS, history of TMJ subluxation and pain. This case describes a 47-year-old woman with a history of hypermobile EDS who presented with Angle Class II malocclusion, Class II skeletal pattern, and clockwise rotation of the occlusal plane associated with pain at the bilateral TMJs. She underwent maxillary-mandibular advancement with counterclockwise rotation of the occlusal plane and genioplasty. The surgery was without complications, and at 22 months follow up, the patient healed uneventfully with improvement of pain and range of motion. This case report demonstrates that with diligent patient selection, orthognathic surgery in patients with EDS can be safe and effective and should not be an absolute contraindication.