Bariatric surgery is not contraindicated in obese patients suffering from glycogen storage disease type IXa. A case report with follow-up at three years

Abstract

INTRODUCTIONGlucose storage disease type IXa (GSD IXa) is an uncommon condition presenting with childhood onset hepatomegaly, growth retardation, and often, fasting ketosis and hypoglycemia. Despite its benign course, the lack of dietary counseling may favor uncontrolled weight gain. We investigated the efficacy of bariatric surgery in one 17 years old female suffering from GSD IXa and morbid obesity. PRESENTATION OF CASEThe diagnosis was GSD type IXa in a patient with a body mass index (BMI) of 45.5kg/m2. Onset of hypoglycemia was reported twice each month. She was treated her implanting an adjustable gastric banding through laparoscopy. Three years after surgery the patient presents a BMI of 30.1kg/m2 and an excess of weight loss (EWL) of 71.1%. Only once, following surgery, she had to deflate her band to allow a faster transit of food through her stomach, thus reaching a prompt euglycemic condition, due to an incoming hypoglycemic crisis. DISCUSSIONLaparoscopic adjustable gastric banding (LAGB) is one of the most used approaches to treat morbid obesity. It is a restrictive procedure unable to affect the absorption of any nutrient, presenting a very low intra and perioperative complication rate. In our GSD IXa patient, it offered a prompt modification of food intake restriction whenever requested, thus avoiding hypoglycemia. CONCLUSIONLAGB is effective in determining weight loss without inducing significant side effects or worsening hypoglycemia, in this morbid obese patient, suffering from GSD type IXa.