Abstract
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.
Highlights
IntroductionSimpson-Golabi-Behmel syndrome (SGBS), first described in 1975 by Simpson et al, is an X-linked recessive condition associated with prenatal and postnatal overgrowth, macrocephaly, congenital diaphragmatic hernia, a characteristic facial appearance (large protruding jaw, broad nasal bridge, upturned nasal tip, hypertelorism, macrocephaly, macrostomia), macroglossia, supernumerary nipples, palatal abnormalities, congenital heart defects and generalized hypotonia [1] [2]
Simpson-Golabi-Behmel syndrome (SGBS), first described in 1975 by Simpson et al, is an X-linked recessive condition associated with prenatal and postnatal overgrowth, macrocephaly, congenital diaphragmatic hernia, a characteristic facial appearance, macroglossia, supernumerary nipples, palatal abnormalities, congenital heart defects and generalized hypotonia [1] [2]
We present the preoperative evaluation and intraoperative management of a 1.5-year-old child with SGBS who underwent plastic surgery for cleft palate
Summary
Simpson-Golabi-Behmel syndrome (SGBS), first described in 1975 by Simpson et al, is an X-linked recessive condition associated with prenatal and postnatal overgrowth, macrocephaly, congenital diaphragmatic hernia, a characteristic facial appearance (large protruding jaw, broad nasal bridge, upturned nasal tip, hypertelorism, macrocephaly, macrostomia), macroglossia, supernumerary nipples, palatal abnormalities, congenital heart defects and generalized hypotonia [1] [2]. Normal intelligence has been described, mild to severe mental retardation is common. This syndrome has variable expressivity, ranging from mild forms in carrier females to lethal infantile forms. Half of affected males die in the neonatal period These patients are at an increased risk of tumor development in early childhood, Wilms tumor, hepatoblastoma, adrenal neuroblastoma, gonadoblastoma and hepatocellular carcinoma, for which they require regular sonographic surveillance [3]. Abnormal airway anatomy may complicate perioperative management in this syndrome, there is only one previously published case report of the anesthetic management of SGBS in a 4.5-year-old child [4]. We present the preoperative evaluation and intraoperative management of a 1.5-year-old child with SGBS who underwent plastic surgery for cleft palate
Sign-in/Register to view highlights & summary 
Published Version (
Free)
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call