Orthognathic surgery in Melnick-Needles-Syndrome. Case report and review of the literature.

Jung S, Wermker K, Joos U, Kleinheinz J

Abstract

Melnick-Needles Syndrome (MNS) is a congenital syndrome associated with severe architectural disorder of the skeletal system that can cause significant effects on the craniofacial skeleton including poor aesthetics, impaired speech and masticatory problems. The authors report a case of a female patient who experienced masticatory constraint, hindered speech and functional problems related to a severe bite dysfunction due to the mandibular hypoplasia and the underlying bony architectural disorder associated with MNS. The patient underwent bilateral sagittal split osteotomy (BSSO) to correct her skeletal malocclusion and improve the characteristic aesthetics and speech. The inherent bony abnormalities with their altered collagen structure presented unique challenges to orthognathic surgery in this patient with an unpredictable bone healing process. In MNS patients a fragile trabecular bone structure and an increased bleeding tendency is thought to delay or forestall wound healing. The patient was treated successfully with BSSO and monocortical plate fixation following a well-established algorithm from orthodontic preparation to surgical protocol. A very satisfying outcome has been achieved, concerning functional rehabilitation and aesthetic improvement. A review of the literature revealed that little is known about bone regeneration and fracture healing in cases with MNS.