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PubMed1980

Hemifacial microsomia: priorities and sequence of comprehensive otologic management.

Hemifacial microsomia is evidenced by the clinical spectrum of microtia, mandibular deformities, and middle ear malformations. This constellation of defects suggests that the most efficacious treatment will be multidisciplinary management, which includes the otolaryngologist, audiologist, plastic surgeon, and temporal bone radiologist. In the past, microtia--the most clinically apparent malformation--usually has been studied and treated with respect to the area of special interest of the investigator, whether from an otologic, reconstructive, or radiologic perspective. Newer management, however, utilizes the combined skills of the clinical and behavioral disciplines in the treatment of the microtic auricle, associated middle ear pathology, and hearing loss. Such multidisciplined approach, commencing during the first year of life, is deemed advisable to cope with the severe unilateral or bilateral hearing loss which may lead to potentially serious sensory deprivation and impede adequate language development in these children.

Audiometry, Evoked Response↗
PubMed1980

Velopharyngeal insufficiency in the facio-auriculo-vertebral malformation complex.

Twenty-two patients with the Facio-Auriculo-Vertebral Malformation Complex (hemifacial microsomia) were examined with multi-view videofluoroscopy and nasopharyngoscopy at rest and during speech. Fifty-five per cent of the subjects had velopharyngeal insufficiency (VPI) and hypernasal speech. Only two of these 12 subjects had cleft palate. VPI was related to structural and physiological asymmetry of the pharynx. No relationship was found between the severity of facial malformation and the presence of VPI.

Adolescent↗
PubMed1981

Obstructive sleep apnea in Treacher-Collins syndrome.

Studies of the Treacher-Collins syndrome have emphasized hearing and surgical considerations. Although craniofacial anomalies have been associated with respiratory disorders in infancy, the presence of such problems in older children has not been emphasized. An eight-year-old with Treacher-Collins syndrome presented a history of recent behavioral problems at home, poor attention span and performance in school, daytime somnolence, and sleep apnea with relatively long periods of chest movement but no airflow. He also had abnormal sleep behavior consisting of rocking to and fro on his hands and knees, often to such an extent that his nose became abraded. ICU monitoring with observation and recording of sleep patterns and sounds, and fluoroscopy of his upper airway utilizing cineradiography while asleep confirmed the diagnosis of obstructive sleep apnea. The patient subsequently underwent an orthognathic surgery consisting of insertion of rib bone grafts after anterior advancement of his mandible. This procedure resulted in disappearance of the obstructive sleep apnea and associated symptoms. Because of micrognathia, patients with Treacher-Collins syndrome are at high risk for developing obstructive sleep apnea. Surgical correction of their deformities can result in improvement in cosmetic appearance as well as in resolution of the obstructive episodes with improvement in performance and behavior.

Airway Obstruction↗
PubMed1980

The use of microvascular free flaps for soft tissue augmentation of the face in children with hemifacial microsomia.

The extent of facial deformity from hemifacial microsomia varies considerably. Minor degrees of asymmetry may be barely perceptible. Severe defects involve deficiencies and asymmetry of bone and soft tissue. Hence, reconstruction of both soft and hard tissues may be necessary. The authors have utilized a free flap based on the superior gluteal vessels to replace soft tissue deficits in three children with hemifacial microsomia. The flap, composed of fascia lata, adjacent muscle and fat (two patients) and overlying skin (one patient), is harvested through an incision high on the lateral thigh in the "bathing trunk" area. The vessels, measuring 0.5--1.0 mm. were anastomosed to the facial (one patient) and superficial temporal (two patients) vessels. In one child, simultaneous mandibular restructuring was done with bone grafts. The children ranged in age from four to eight years. Follow-up is for five to seven months. Although this microvascular procedure requires a prolonged anesthetic, it permits more accurate and predictable reconstruction. Scars are placed in inconspicuous or concealed areas. Simultaneous bony reconstruction can be done. Details of the procedure and an evaluation of postoperative results are presented.

Adipose Tissue↗