Neonatal Islet Cell Adenoma: Case Report and Literature Review

Islet cell adenomata are uncommon in children, although more than 50 cases have been reported.1-3 Of these, only about a dozen cases were in the newborn period.4-12 For this reason we report here another case and provide a brief review of this disorder in the neonatal period. CASE REPORT A male infant was born April 14, 1972, after an uncomplicated pregnancy and a spontaneous term delivery. Birth weight was 4.2 kg. The one- and five-minute Apgar scores were 9 and 10, respectively. Attacks of sweating and cyanosis associated with severe hypoglycemia (blood glucose <10 mg/100 ml) were first noted at 24 hours of age. These persisted despite repeated glucose administration and in the face of continuous intravenous glucose infusion (25 gm/kg/24 hr) and intravenous hydrocortisone (10 mg/kg/24 hr). After some days on oral glucose, 30 gm/kg/24 hr, the symptoms were ameliorated and the steroids were then discontinued. The child was discharged 15 days after birth on frequent oral feedings, although normoglycemia could not be maintained. Two days after discharge, seizures and lethargy prompted readmission. An intravenous glucose tolerance test and a glucagon tolerance test were performed; the results are shown in Table I. Other studies included normal blood electrolytes, calcium, and BUN. Plasma cortisol was 6µg/100 ml. During this admission, the severe hypoglycemia persisted and treatment with prednisone (2 mg/kg/day) for one week followed by a trial of diazoxide (10 mg/kg/day) for seven days produced no obvious benefit (Table I). At 6 weeks of age, the patient developed cardiac failure while still receiving 10 mg/kg/day of diazoxide and was transferred to the University of Oregon Medical School.