Anesthetic Management of Aglossia-Adactylia Syndrome: Report of a Case

Abstract

M Hegazy, H Farag, M Naguib, Anesthetic Management of Aglossia-Adactylia Syndrome: Report of a Case. 1988; 8(3): [221][222][223] Aglossia-adactylia syndrome is a rare congenital malformation first reported by Rosenthal in 1932. 1 The main features of the syndrome are hypoglossia, hypognathia, microstomia, syngnathia, hypertrophy of the submandibular salivary glands, total or partial absence of distal parts of the limbs, and in some cases, a man-dibulomaxillary fusion. Associated anomalies in the reported cases were transposed viscera and dextrocardia, imperforate anus, and mental retardation.2 In cases of aglossia-adactylia with man-dibulomaxillary fusion, the most serious problem is upper airway management. We describe the anesthetic management of a patient with aglossiaadactylia syndrome. Case ReportA 10-week-old boy weighing 3 kg was referred to the plastic surgery unit for separation of congenital mandibulomaxillary fusion. The mother, a 42-year-old, gravida 11, para 11, had a normal full-term delivery. She was asthmatic and took dexamethasone, 2 mg, and dexachlorpheniramine maleate, 5 mg twice daily, during the pregnancy. The father was a healthy 63-year-old man. The parents were first cousins, but without family history of congenital anomalies.The baby had micrognathia, microstomia, and complete mandibulomaxillary fusion and both lips were adherent to the alveolar margins. In addition, he had a lobster-claw deformity of both hands, absent toes, and bilateral inguinal hernias. The baby had a nasogastric tube for feeding since birth. He was nursed in the prone position with frequent pharyngeal suction through the nose to avoid choking from secretions. X-ray films disclosed a cartilagenous mandibulomaxillary fusion with an osseous part at the mandibular symphysis. Chest X-ray film and abdominal CT scan were normal.Before separation of the mandibulomaxillary fusion, the infant was premedieated with 10 mg of vitamin K 12 hours before surgery and 0.1 mg of atropine 30 minutes before surgery, both administered intramuscularly. In the operating room the nasogastric tube was removed, and nasal decongestant drops were instilled. A 5% dextrose in 0.2% saline infusion was started through a 22-gauge cannula at a rate of 20 ml/hour. The ECG and rectal temperature were monitored continuously, and blood pressure was monitored every five minutes using a neonatal electronic oscillotonometer. An awake blind nasal intubation was attempted under X-ray visualization with a No. 3.5 tube and wire stylet, but the larynx was located too far anteriorly for successful intubation. A tracheostomy was then performed under local anesthesia, and a No. 3.5 tracheostomy tube inserted. The baby was anesthetized with 70% nitrous oxide in oxygen via a Jackson Rees modification of Ayer's T-piece circuit, and ventilation was controlled with 2 mg of atracurium.The cartilagenous and bony fusion was divided surgically. A blind bilateral osteotomy was performed at the ascending mandibular rami for adequate movement of the mandible. A cleft soft pal...