Growth and development in patients operated on for islet cell dysplasia.

Neonatal hypoglycemia caused by islet cell dysplasia (ICD), sometimes called nesidioblastosis, may lead to psychomotor retardation and neurologic dysfunction in up to 50% of patients who are not given early aggressive treatment. In 1979, we adopted a more aggressive protocol for treating this condition that consists of the following steps: immediate insertion of a silastic central venous line for reliable venous access; continuous intravenous infusion of glucose and glucagon to maintain euglycemia; oral diazoxide; and near total pancreatectomy if the first steps fail to overcome the hypoglycemia or the patient cannot be weaned off intravenous therapy. Twelve consecutive patients who underwent pancreatectomy for control of hypoglycemia between 1979 and 1984 were recalled and evaluated for growth delay, neurologic dysfunction, and psychomotor retardation using the Revised Yale Developmental Schedules, the Peabody Picture Vocabulary, and the Draw a Man Test. Follow-up ages ranged from 1.2 to 6.0 years with a median of 3.6 years. No significant growth abnormalities were identified. No patient exhibited focal neurologic dysfunction, although some demonstrated soft neurologic signs, which did not appear to be related to their earlier hypoglycemia. Psychomotor function for the group as a whole was normal, with a mean developmental quotient (DQ) of 99.2. The DQ was average for five patients and above average for four; no patient had a DQ in the frankly subnormal range. Psychomotor development correlated better with the family's socioeconomic and educational status than with the neonatal hypoglycemia. These children are developmentally and neurologically normal despite severe neonatal hypoglycemia. Continued follow-up will be necessary to detect any late sequelae.(ABSTRACT TRUNCATED AT 250 WORDS)