Ann Pediatr Endocrinol Metab.  2017 Dec;22(4):272-275. 10.6065/apem.2017.22.4.272.

Congenital hyperinsulinism: diagnostic and management challenges in a developing country – case report

  • 1Angels Speciality Clinic, Chennai, India.
  • 2Department of Paediatric Surgery, Sri Ramachandra University, Chennai, India.
  • 3Anderson Diagnostics and Labs, Chennai, India.
  • 4Department of Pathology, Sri Ramachandra University, Chennai, India.
  • 5Department of Paediatric Endocrinology, Alder Hey Children’s Hospital, Liverpool, UK.


Management of congenital hyperinsulinemia of infancy (CHI) is challenging. A 4-month-old female infant with persistent hypoglycemia and elevated insulin levels was diagnosed with CHI. Gallium-68 DOTANOC positron emission tomography/computed tomography (PET/CT) scan (⁶⁸Ga-labeled [1,4,7,10-tetraazacyclododecane-N,N',N'',N'''-tetraacetic acid]-1-NaI3-octreotide) demonstrated focal disease in the body of the pancreas. Genetic studies indicated paternal inheritance, making focal disease likely. She was started on diazoxide therapy with partial improvement in blood glucose levels. Due to a suboptimal response to diazoxide and the likelihood of focal disease amenable to surgery, a laparoscopic subtotal pancreatectomy with preservation of the head of the pancreas was performed. The biopsy demonstrated diffuse hyperplastic pancreatic islet cells on immunohistochemistry, indicative of diffuse rather than focal disease. Paternal inheritance is a recognized indicator of focal disease. Gallium-68 DOTANOC PET/CT scan is the only available imaging modality in South India as ¹â¸F-L-dihydroxyphenylalanine (DOPA) PET/CT scan is not available at present. A laparoscopic approach reduces the postoperative recovery time and morbidity in such patients. The absence of ¹â¸F-L-DOPA PET/CT scan and the limited supply of diazoxide makes the management of this complex condition more challenging in developing countries.


Congenital hyperinsulinism; Hypoglycemia; Diazoxide

MeSH Terms

Blood Glucose
Congenital Hyperinsulinism*
Developing Countries*
Islets of Langerhans
Positron-Emission Tomography and Computed Tomography
Blood Glucose


  • Fig. 1. Gallium positron emission tomography scan demonstrated increased uptake of 68Ga DOTANOC in the body of the pancreas (yellow arrow).

  • Fig. 2. Resected specimen of subtotal pancreatectomy demonstrating body and tail of pancreas.

  • Fig. 3. Histopathology section in high power view focusing the pale acini (H&E, ×200), demonstrates diffuse uptake of hematoxylin throughout acini by the beta cells suggestive of diffuse disease characterised by hypertrophied beta cells with abnormal large nucleus.



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