Anesthesia Management in an 11-Month Old Infant with Pompe Disease


Department of Anesthesiology, Labbafi-Nejad Hospital, Shahid Beheshti University, M.C, TEHRAN-IRAN.


Pompe disease is a glycogen storage disease (GSD) type II. Infantile-onset Pompe disease is fatal presenting with cardiac and skeletal myopathies and has an autosomal recessive pattern of inheritance with the prevalence rate of 1 in 40,000 live births (1). Its common symptoms include cardiomegaly, hypotonia, failure to thrive (FTT) and hepatomegaly (1). The patient was a 4 kg, 11-month-old infant with the history of jaundice and recurrent seizures under treatment with phenytoin (15 mg/day) and phenobarbital (15 mg/day). He was hypotonic, cachectic and pale (Hb=9.5) when presented to the anesthesia clinic of Labbafi Nejad Hospital for bilateral lensectomy. Induction and maintenance of anesthesia were carried out via the inhalation anesthesia method (N2O/O2 and sevoflurane). Laryngeal mask airway (LMA) was placed when achieving the appropriate depth of anesthesia. Bilateral lensectomy took 2 hours. After completion of the operation, the patient regained consciousness. His vital signs were stable and he was transferred to the recovery room and then to the ward. He was discharged from the hospital the day after the operation with no complications. (Tanaffos 2009; 8(1): 79-83)