Near-total pancreatectomy for persistent hyperinsulinemic hypoglycemia of infancy (nesidioblastosis)

Abstract

Background/purpose Persistent hyperinsulinemic hypoglycemia of infancy (PHHI) is the most common cause of persistent hypoglycemia in infancy with consequences such as seizures and brain damage. Treatment that prevents the occurrence of these sequele is essential. When medical therapy fails or complications are anticipated, early pancreatectomy is recommended to maintain euglycemia. The aim of this study was short-term evaluation of near-total pancreatectomy for PHHI after failure of medical treatment in patients in Mansoura, Egypt. Patients and methods During May 2002 to May 2010, 33 patients suffering from persistent hyperinsulinemic hypoglycemia were admitted to Mansoura university child hospital. Twenty patients responded to medical treatment and 13 patients (eight girls and five boys) were treated by near-total (90–95%) pancreatectomy after failure of medical treatment. Their ages at the time of surgery ranged from 20 days to 27 months. Only patients who were surgically managed were included in this study. All of them suffered from symptoms of PHHI: thermoregulatory problems in five cases, tremors in five cases, seizures in three, irritability in five, respiratory distress in eight, apnea in four, hypotonia in four, lethargy in five, and feeding difficulty in four cases. All cases were diagnosed following strict criteria that included bouts of hypoglycemia without acidosis coinciding with increased insulin level. Follow-up ranged from 3 months to 8 years. Results Primary success occurred in two cases (15%); four more cases (31%) developed temporary diabetes mellitus, and one case (8%) had a temporary pancreatic fistula. Eventually, total cure occurred in seven cases (54%); six cases (46%) developed a variety of complications; and persistent hypoglycemia was seen in two. Persistent diabetes mellitus was observed in three patients, developmental delay in one, and persistent neurological deficit in two patients; there was one case of mortality. Conclusion Near-total (90–95%) pancreatectomy is a suitable procedure for treatment of nesidioblastosis not responding to medical treatment and should be performed as early as possible. Keywords : Mansoura, near-total pancreatectomy, persistent hyperinsulinemic hypoglycemia