Congenital hyperinsulinemia of infancy (CHI) is a major cause of hypoglycemia in the neonatal period and early infancy. It has also been referred to as persistent hyperinsulinemic hypoglycemia of infancy, primary islet cell hypertrophy and nesidioblastosis. It is characterized by inappropriate secretion of insulin in relation to blood glucose levels and can result in recurrent and persistent hypoglycemia in neonates and infants.

Early recognition of CHI and maintenance of euglycemia is necessary to minimize neurologic damage which can result from recurrent and/or prolonged episodes of hypoglycemia.

The identification of focal disease by (DOPA) positron emission tomography/computed tomography (PET/CT) scan may help prompt removal of the focal lesions, thereby possibly rendering cure.

Medical options for treatment of diffuse CHI include diazoxide and octreotide therapies.

Surgery is considered as a treatment option if focal disease has been identified on imaging or in medically unresponsive diffuse disease.

Laparoscopic subtotal pancreatectomy  should be the initial surgical approach in PHHI children who fail the medical therapy. If the initial surgical resection is inadequate and the patient is still hypoglycemic, further pancreatic resection can be easily performed laparoscopically in the absence of adhesions and scarring. Three ports laparoscopy is done. Umbilicus for camera and two as working ports. The stomach is retracted up towards the anterior abdominal wall with stay sutures to expose the lesser sac. The pancreas is resected from the splenic hilum to the mesenteric vessels. The splenic vein is dissected carefully from under the surface of the pancreas using electrocautary thereby preserving the spleen. The pancreatic mobilization and dissection is carried medially to the right using monopolar. The transaction of pancreatic tissue is done using a harmonic scalpel. A  drain is left in the lesser sac.

Further Reading:

1. Sunehag A, Haymond MW. Pathogenesis, clinical features, and diagnosis of persistent hyperinsulinemic hypoglycemia of infancy [Internet]. Waltham (MA): UpToDate; 2016 [2016 Mar 29]. Available from: http://www.uptodate.com/ contents/pathogenesis-clinical-features-and-diagnosis-ofpersistent-hyperinsulinemic-hypoglycemia-of-infancy.
2. Meissner T, Wendel U, Burgard P, Schaetzle S, Mayatepek E. Long-term follow-up of 114 patients with congenital hyperinsulinism. Eur J Endocrinol 2003;149:43-51.
3. Cheri Mathews John , Prakash Agarwal, S u r i y a k u m a r Govindarajulu, Sandhya Sundaram, Senthil Senniappan. Congenital Hyperinsulinism: Diagnostic and management challenges in a developing country – case report. Ann Pediatr Endocrinol Metab 2017;22:203-206.