Anesthetic Management of Children with Congenital Disorders of Glycosylation
An 11-yo-female with congenital disorder of glycosylation type 2, underwent gastroesophageal disconnect with roux-en-Y for refractory GERD by open laparotomy. Impaired glycosylation of lipids and proteins results in widespread neurologic, hepatic and muscular dysfunction. Our patient had severe intractable seizures, neurologic impairment, hypotonia, poor feeding and GERD. Others may have severe hepatic impairment and coagulopathy. She received sevoflurane, rocuronium, small doses of ketamine and ketorolac. Opioids were avoided, bilateral rectus sheath blocks performed and patient awakened pain free. No opioids were needed in PACU. Patient recovered uneventfully. We will discuss the genetics, pathophysiology, multi-organ dysfunction and anesthetic implications of CDG.
Markatos S, Behbahani-Nejad N, Banoub M. Anesthetic management of children with congenital disorders of glycosylation. Paper presented at: The American Society of Anesthesiology Annual Meeting; 2021 Oct 9-12; San Diego, CA. Available at: https://www.abstractsonline.com/pp8/#!/9323/presentation/5339