The Background: Williams syndrome is a neurodevelopmental multisystem genetic disorder characterized by dysmorphic features and a wide range of congenital cardiac, renal, musculoskeletal anomalies. Presence of cleft p...The Background: Williams syndrome is a neurodevelopmental multisystem genetic disorder characterized by dysmorphic features and a wide range of congenital cardiac, renal, musculoskeletal anomalies. Presence of cleft palate in these patients is an unusual condition. More than 22 cases of sudden cardiac death reported in literature especially during anesthetic management in the patients of Williams syndrome. So these patients present major perioperative challenges to anesthesiologist. The authors report successful anesthetic care for repair of cleft palate in presence of uncorrected patent ductus arteriosus (PDA) and mild supraventricular aortic stenosis (SVAS) in a 3.5-year-old female child diagnosed with Williams syndrome. Case report: Diagnosed case of Williams syndrome with characteristic facial dysmorphic features, SVAS and rare malformation of PDA, was scheduled for cleft palate repair. After keen preoperative assessment, general anesthesia was administered for the procedure with continuous monitoring for vital parameters. Because of dysmorphic features, difficulty was faced during intubation. Otherwise intraoperative procedure was uneventful. Postoperative analgesia was managed with intravenous paracetamol. Conclusion: Being a multisystem disorder, each patient of WS requires meticulous preoperative evaluation and high level of intraoperative and postoperative continuous monitoring regardless of any surgical procedure. Surgical correction of cleft palate in such patients demands very high anesthetic and surgical skills as both share common airway. Present case report highlights the significance of aggressive perioperative management in WS which can result in successful outcome.展开更多
文摘The Background: Williams syndrome is a neurodevelopmental multisystem genetic disorder characterized by dysmorphic features and a wide range of congenital cardiac, renal, musculoskeletal anomalies. Presence of cleft palate in these patients is an unusual condition. More than 22 cases of sudden cardiac death reported in literature especially during anesthetic management in the patients of Williams syndrome. So these patients present major perioperative challenges to anesthesiologist. The authors report successful anesthetic care for repair of cleft palate in presence of uncorrected patent ductus arteriosus (PDA) and mild supraventricular aortic stenosis (SVAS) in a 3.5-year-old female child diagnosed with Williams syndrome. Case report: Diagnosed case of Williams syndrome with characteristic facial dysmorphic features, SVAS and rare malformation of PDA, was scheduled for cleft palate repair. After keen preoperative assessment, general anesthesia was administered for the procedure with continuous monitoring for vital parameters. Because of dysmorphic features, difficulty was faced during intubation. Otherwise intraoperative procedure was uneventful. Postoperative analgesia was managed with intravenous paracetamol. Conclusion: Being a multisystem disorder, each patient of WS requires meticulous preoperative evaluation and high level of intraoperative and postoperative continuous monitoring regardless of any surgical procedure. Surgical correction of cleft palate in such patients demands very high anesthetic and surgical skills as both share common airway. Present case report highlights the significance of aggressive perioperative management in WS which can result in successful outcome.
