Surgical correction of Goldenhar's syndrome.
Goldenhar's syndrome is a variant of hemifacial microsomia - one of the more common congenital syndromes of the first and second arch. The Goldenhar's type is characterized by varying degrees of underdevelopment of craniofacial structures. The major deformities involve the mandible and the ear; but they also include the orbit and vertebral column, specific defects pathognomonic of this condition. Medical management requires reconstructive surgery of which treatment must take into consideration the timing of surgery and the effects of surgery upon the growth and development of the poorly formed structures. This paper presents a discussion of the pathogenesis and the favorable and unfavorable factors affecting the selection and timing of surgery. The literature is reviewed and two additional cases are added with an evaluation of the effects of early reconstruction. A single-stage procedure, utilizing rib autograft, is described for correction of the defective hemimandible, glenoid fossa, and zygoma. The results are evaluated by means of photographs and radiographs with a follow-up period of at least one and one-half years. The data suggest a remodeling of the grafts to resemble normal structures, and a downward growth of the maxilla to fill a space created by the reconstruction of the mandible and expansion of the lower portion of the face. Multiple stages of surgery as well as unnecessary delays in performing surgical procedures are challenged by the report.
D002648:Child; D003937:Diagnosis, Differential; D005260:Female; D006053:Goldenhar Syndrome / Q000175:diagnosis / Q000209:etiology / Q000601:surgery*; D006801:Humans; D008334:Mandible / Q000601:surgery; D008342:Mandibulofacial Dysostosis / Q000175:diagnosis / Q000601:surgery*; D012272:Ribs / Q000637:transplantation; D013518:Surgery, Plastic / Q000379:methods*; D014182:Transplantation, Autologous; D015050:Zygoma / Q000601:surgery