Simpson-Golabi-Behmel syndrome (SGBS) is an X-linked recessive condition associated with pre-natal and postnatal overgrowth with visceral and skeletal anomalies. Abnormal airway anatomy may complicate the perioperativ...Simpson-Golabi-Behmel syndrome (SGBS) is an X-linked recessive condition associated with pre-natal and postnatal overgrowth with visceral and skeletal anomalies. Abnormal airway anatomy may complicate the perioperative management of SGBS. However, there have been few reports regarding the anesthetic management of children with SGBS. We present the preoperative evalua-tion and intraoperative management of a 1.5-year-old child with SGBS undergoing plastic surgery for cleft palate. Sevoflurane-fentanyl anesthesia without a neuromuscular blocker was planned, due to the preoperative evaluation that airway management would be complicated by the macroglossia and short neck characteristic of SGBS. Fortunately, there was no difficulty in endotracheal intubation in the present patient due to careful planning of general anesthesia and meticulous airway management. Appropriate preoperative preparation for a difficult airway by the anesthe-siologist can ameliorate the difficulties inherent to the anesthetic management of patients with SGBS.展开更多
文摘Simpson-Golabi-Behmel syndrome (SGBS) is an X-linked recessive condition associated with pre-natal and postnatal overgrowth with visceral and skeletal anomalies. Abnormal airway anatomy may complicate the perioperative management of SGBS. However, there have been few reports regarding the anesthetic management of children with SGBS. We present the preoperative evalua-tion and intraoperative management of a 1.5-year-old child with SGBS undergoing plastic surgery for cleft palate. Sevoflurane-fentanyl anesthesia without a neuromuscular blocker was planned, due to the preoperative evaluation that airway management would be complicated by the macroglossia and short neck characteristic of SGBS. Fortunately, there was no difficulty in endotracheal intubation in the present patient due to careful planning of general anesthesia and meticulous airway management. Appropriate preoperative preparation for a difficult airway by the anesthe-siologist can ameliorate the difficulties inherent to the anesthetic management of patients with SGBS.
