Comparison of dietary therapy and portacaval shunt in the management of a patient with type Ib glycogen storage disease.

A patient with glycogen storage disease type IB (GSD-Ib) is described. Beginning at age 34 months, the patient was managed with dietary measures, including nocturnal intragastric feedings. Therapy caused substantial biochemical and clinical improvement while the patient remained hospitalized; chronic noncompliance at home, however, resulted in profound growth failure and biochemical abnormalities. Due to a previous case report describing clinical, biochemical, and hematological improvement following a portacaval shunt in a similar patient, portacaval shunt was performed in our patient at age 13 years. During the 12 months following surgery, there was no improvement of our patient's growth or of biochemical or hematological abnormalities. Due to the lack of improvement following surgery, 24-hour constant infusion intragastric feedings were instituted and have been associated with substantial benefit.