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European Journal of Oral and Maxillofacial Surgery 2020 December;4(3):111-6

DOI: 10.23736/S2532-3466.19.00201-7


lingua: Inglese

Management of a Goldenhar Syndrome patient with a custom-made osteosynthesis mesh

Carlo TIAN 1, Ingrid TONNI 1, Umberto ZANETTI 1, Luca VISCONTI 1, Francesco DALEFFE 1 , Andrea CASTELLANI 2

1 Dental School, University of Brescia, Brescia, Italy; 2 Department of MaxilloFacial Surgery, Civil Hospital, Brescia, Italy

Craniofacial microsomia (CFM) is the second most common congenital craniofacial malformation behind cleft lip and palate. Also called oculo-auriculo-vertebral dysplasia, this syndrome is characterized by accessory tragus, mandibular hypoplasia and epibulbar dermoid. The most commonly used classification of CFM for clinical and research purposes is the Kaban modification of the Pruzansky classification. Adults with hemifacial microsomia, mandibular hypoplasia and facial asymmetry are traditionally treated with orthognathic surgery. This treatment may sometimes be combined with TMJ and ramus reconstruction, using a wide range of various techniques. In this case report we describe the orthodontic-surgical therapy of a CFM type IIa in a non-growing patient previously underwent to palatoplasty, costochondral graft and distraction osteogenesis over mandibular malformation. At the end of the growth were surgically performed a splintless Le Fort I osteotomy, a sagittal split osteotomy on the healthy mandibular side and an inverted L ramus osteotomy with custom-made prosthetic grid for osteosynthesis on the dysmorphic side. This approach granted a good stomatognathic function together with an improved aesthetics of facial contour. Combining different methods in facing clinical problems can grant excellent results. This may include, besides orthodontic and surgical skills, team-communication and team-working abilities, some grade of mental resilience and flexibility.

KEY WORDS: Goldenhar syndrome; Orthognathic surgery; Case report

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