Anesthetic Management for a Patient with Apert's Syndrome

Lim, Yong Gul; Lee, Eun Ju; Ok, Kwang Ick; Kang, Yoo Jin

Abstract

Apert's syndrome is rare autosomal dominant defect characterized by craniosynostosis, midface hypoplasia and symmetrical syndactyly. Multiple surgical procedures will be needed for the child with this syndrome. Especially surgery for craniosynostosis is most often performed between 2 and 6 months of life, a period of physiologic anemia. Perioperative complications can occur from numerous sources-massive blood loss, venous air embolism, cerebral edema, hypothermia and hazardous airway management, thus more monitoring, large bored central venous line and early transfusion are recommended. We report a case of 5 month-old female Apert patient undergoing frontal bone advancement and cranioplasty. At the end of uneventful surgery, we removed endotracheal tube in operating room and permitted early feeding because the procedure was limited above the orbital ridge. As soon as feeding the patient presented cyanosis with generalized seizure. Patient was immediately intubated, sucked out secretions and inhaled with oxygen. Blood gas showed moderate hypoxemia which was improved by oxygen therapy. Suspicious aspiration pneumonia was marked on chest film but disappered I week later. The condition was favorable thereafter and discharged without complication. Keypoints in management of Aperts syndrome are close observation, early detection and preventive treatment of possible complications in perioperative period. (Korean J Anesthesiol 1995; 29: 300~303)