Portal Diversion In Glycogen-Storage Disease (Gsd) - Studies In 11 Cases

Successful treatment of GSD by portal diversion was reported with subsequent improvement in growth and return to normal dietary habits. An end-to-side portacaval shunt has been performed in 11 patients aged 7 months to 11 1/2 years (6 with type I and 5 with type III GSD). In 10 patients, patency of the anastomosis was confirmed. In 9 of them, a dramatic improvement of physical activity was observed in spite of persistent hypoglycemia and hyperlactacidemia. Liver size decreased in 7. A threefold decrease in blood cholesterol level (M 164 mg/dl vs. 415 peroperatively) was observed in 8 patients and a tenfold decrease in blood cholesterol level (M 170 mg/dl vs. 1548) in 6 out of 7 tested patients. Uricemia, increased in 4 cases, returned to normal. An increased linear growth rate (M 0.66 cm/month vs. 0.27) was noted in only 5 patients. Two type I patients died, 2 years (thrombosis of the anastomosis) and 6 months (intercurrent infection) after surgery. These results show that the most important benefits of surgery are improvement of general condition and normalization of lipids and uric acid metabolism.