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
In the evaluation of the beauty and functional integrity of the lower face, the oral cavity, teeth, mandible, maxilla, and the size and position of the tongue are important. The tongue locates forward and is larger th...In the evaluation of the beauty and functional integrity of the lower face, the oral cavity, teeth, mandible, maxilla, and the size and position of the tongue are important. The tongue locates forward and is larger than normal in prognathism, in which the jaw protrudes from the skull. It is not clear whether an enlarged tongue causes the open bite, protrusion or dental arch misalignment, or is a result of them. Here, we report two patients with mandibular prognathism and relative macroglossia, which were treated by mandibular setback surgery using a bilateral sagittal split osteotomy (BSSO) and excision of the tongue. This procedure achieved aesthetic improvement of the face and dentition.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.
文摘In the evaluation of the beauty and functional integrity of the lower face, the oral cavity, teeth, mandible, maxilla, and the size and position of the tongue are important. The tongue locates forward and is larger than normal in prognathism, in which the jaw protrudes from the skull. It is not clear whether an enlarged tongue causes the open bite, protrusion or dental arch misalignment, or is a result of them. Here, we report two patients with mandibular prognathism and relative macroglossia, which were treated by mandibular setback surgery using a bilateral sagittal split osteotomy (BSSO) and excision of the tongue. This procedure achieved aesthetic improvement of the face and dentition.
