چكيده لاتين :
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)
