ANESTHETIC MANAGEMENT OF A PATIENT WITH GLYCOGEN-STORAGE-DISEASE TYPE1B

Purpose: A rare case of a ten-year old patient with type Ib glycogen s torage disease (GSD) scheduled for extracorporeal shockwave lithotrips y (ESWL), is described. Clinical features: Patients with type Ib GSD m anifest a range of clinical symptoms, including mental retardation, he patosplenomegaly, renal enlargement stomatitis, hypoglycaemic convulsi ons, bleeding diathesis, lactic acidosis and leukopaenia, thus creatin g a challenge for the anaesthetist. Following preanaesthetic administr ation of glucose-containing fluids, general anaesthesia was induced an d the patient was mechanically ventilated. Except for mild hypoglycaem ia after induction of anaesthesia, and moderate intraoperative metabol ic acidosis which was attributed to the underlying disorder, anaesthes ia was uneventful. No postoperative complications occurred and the pat ient was discharged home three days after lithotripsy. Clinical featur es of this rare inborn error of metabolism are reviewed and the approa ch for the anaesthetic management is discussed. Conclusions: A skillfu l perioperative management of patients with type Ib GSD can be achieve d by cautious attention to the metabolic and homeostatic derangements that occur with the disease.