Cranial Maxillofac Trauma Reconstruction 2018; 11(02): 124-130
DOI: 10.1055/s-0037-1606248
Case Report
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Maxillomandibular Syngnathia: 3D Planning and Review of the Literature

Josep Rubio-Palau
Division of Maxillofacial Surgery, Department of Pediatric Surgery, Hospital Sant Joan de Deu, Esplugues de Llobregat, Barcelona, Spain
Department of Oral Medicine and Oral Public Health, Universitat Internacional de Catalunya, Barcelona, Catalunya, Spain
,
Alejandra Prieto-Gundin
Department of Pediatric Anesthesiology, Hospital Sant Joan de Deu, Esplugues de Llobregat, Barcelona, Spain
,
Leydi Mar de Abreu Graterol
Department of Pediatric Anesthesiology, Hospital Sant Joan de Deu, Esplugues de Llobregat, Barcelona, Spain
,
Herman Vercruysse Jr.
Department of Craniofacial Surgery, Great Ormond Street Hospital for Children, NHS Trust, London, United Kingdom
› Author Affiliations
Further Information

Publication History

11 January 2017

29 April 2017

Publication Date:
25 August 2017 (eFirst)

Abstract

Congenital maxillomandibular fusion or syngnathia is a rare craniofacial disorder with only 26 cases reported in the literature. We present a case of a congenital complex zygomatico-mandibular syngnathia associated with a palatal cleft, posterior maxilla and turbinate agenesia, mild hemifacial microsomia, and a disordered dental eruption. The patient has the highest age (15 years) at diagnosis described in the literature. 3D planning of the surgery was performed to study the patient's anatomy and design the necessary osteotomies to separate the jaws. En bloc removal of the fused fragment with bilateral coronoidectomy and aggressive long-term physiotherapy for 3 months led to a stable increase in mouth opening from 0 to 21 mm inter-incisor distance. The patient reported an improvement in speech, was able to eat without restriction regarding food consistency, and could maintain a good oral hygiene.