Distraction osteogenesis therapy in patients affected by Goldenhar syndrome: a case series

  • Francesco Grecchi | info@ariesdue.it Consultant, Maxillofacial Surgery, Galeazzi Hospital, Milano, Italy.
  • Giovanni Evangelista Mancini Consultant, Orthodontist, ANDI Foundation, Milano, Italy.
  • Raffaella Bianco Consultant, Maxillofacial Surgery, Galeazzi Hospital, Milano, Italy.
  • Ilaria Zollino Maxillofacial Surgery, University of Ferrara, Ferrara, Italy.
  • Francesco Carinci Maxillofacial Surgery, University of Ferrara, Ferrara, Italy.

Abstract

Background Hemifacial microsomia (HM) is a syndrome characterized by the presence of structural alterations of the skeletal, nervous, vascular, and muscular structures derived from the first and second branchial arch. Goldenhar syndrome (Gs) consistisof the triad of craniofacial microsomia, ocular dermoid cysts, and spinal anomalies. When the patient has hypoplasia of the mandible, orthognatic surgery or distraction osteogenesis (DO) can be used to correct the asymmetry. Mandibular DO has been applied for many years, but long-term reports showed controversial results. The aim of this paper is to describe three cases of patients affected by Gs in which DO was performed to correct the mandibular asymmetry. Case seriesThe cases reported show an increasing degree of dismorphism which required a increasing complexity of the surgical approach: a single mandibular DO in the first patient, and a mandibular DO associated with a Le Fort I osteotomy in the second one, a double mandibular DO associated with Le Fort I and surgical disjunction of the middle palatal suture in the third case. Discussion The effects of DO involve not only the skeletal segment but also all the surrounding soft tissues. DO leads to rapid and remarkable improvement in facial symmetry due to emimandible hypoplasia. When correct spatial repositioning of the maxilla cann ot be expected, mandibular DO can be carried out by associating a Le Fort I osteotomy. In this way DO minimize the need for major osteotomies and allows an earlier treatment in selected cases.

Downloads

Download data is not yet available.
Published
2011-06-30
Section
Articles
Keywords
Bone, Distraction osteogenesis, Goldenhar syndrome, Orthognathic surgery
Statistics
Abstract views: 55

PDF: 60
Share it
Received 2017-06-08
Accepted 2017-06-08
Published 2011-06-30
How to Cite
Grecchi, F., Mancini, G. E., Bianco, R., Zollino, I., & Carinci, F. (2011). Distraction osteogenesis therapy in patients affected by Goldenhar syndrome: a case series. Journal of Osseointegration, 3(2), 25-34. https://doi.org/10.23805/jo.2011.03.02.02

Most read articles by the same author(s)