RESUMEN
The loss of the maxilla is a severe mutilation resulting from inadequate surgery of bilateral clefts of the lip and palate. It is usually associated with palatal fistulae, collapse of the maxillary segments, and limited facial growth.Functional rehabilitation can be achieved by reconstruction of the premaxilla with osteomucosal fibula grafts. Mucosal grafts are fixed to the fibula in a preliminary stage. The composite graft is transplanted to the maxilla 10 to 12 weeks later. Osteointegrated implants are placed 3 months later.The procedure was used in 7 patients, 1 holoprosencephaly and 6 with sequelae of bilateral clefts; mean age, 17.28 years, with a follow-up of 14 to 70 months.The mucosal grafts integrated successfully to the fibula in all the patients. The osteocutaneous graft achieved a solid maxillary arch in all the patients. Normal mastication was achieved with a prosthesis fixed to the osteointegrated implants. Facial proportions were greatly improved.
Asunto(s)
Labio Leporino/cirugía , Fisura del Paladar/cirugía , Peroné/trasplante , Maxilar/anomalías , Maxilar/cirugía , Mucosa Bucal/trasplante , Procedimientos de Cirugía Plástica/métodos , Adolescente , Placas Óseas , Femenino , Humanos , Masculino , Oseointegración , Reoperación , Colgajos Quirúrgicos , Resultado del TratamientoRESUMEN
Melnick-Needles syndrome is an X-inked-dominant skeletal dysplasia in which there is deficient osteoblastic activity. Patients present with craniofacial anomalies consisting of a prominent forehead, exorbitism, mandibular hypoplasia, cheek fullness, and class II malocclusion. Severe mandibular hypoplasia leads to upper airway restriction, an increased incidence of sleep apnea and pneumonias, and occasionally respiratory failure. This is a report of a patient with Melnick-Needles syndrome who presented to our unit after multiple bouts of respiratory failure and with a tracheostomy in whom mandibular distraction osteogenesis was used to retire her tracheostomy and to cure her sleep apnea. The patient underwent bilateral, external, unidirectional mandibular distraction with a vector parallel to the occlusal plane. After a latency period of 5 days, distraction was initiated at a rate of 1 mm/day for 34 days. At this point, the patient was able to breathe with the tracheostomy plugged, and her occlusion had changed from a class II to a class III relationship. She no longer snored, and pulse oximetry on room air was normal while standing or supine. Interestingly, the patient's consolidation phase was prolonged--255 days--possibly attributable to altered bony metabolism. To our knowledge, this is the first reported case of mandibular distraction osteogenesis used to cure obstructive sleep apnea and eliminate the need for tracheostomy in a patient with Melnick-Needles syndrome. In the future, prophylactic mandibular distraction may prevent the need for tracheostomy in this group of patients.