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Redkar R, Karkera PJ, Krishnan J, et al. Subtotal Pancreatectomy for Congenital Hyperinsulinism: Our Experience and Review of Literature. Indian J Surg. 2013;77:778-82doi: 10.1007/s12262-013-0999-9.
Redkar, R., Karkera, P. J., Krishnan, J., & Hathiramani, V. (2015). Subtotal Pancreatectomy for Congenital Hyperinsulinism: Our Experience and Review of Literature. The Indian journal of surgery, 77778-82. https://doi.org/10.1007/s12262-013-0999-9
Redkar, Rajeev, et al. "Subtotal Pancreatectomy for Congenital Hyperinsulinism: Our Experience and Review of Literature." The Indian journal of surgery vol. 77 (2015): 778-82. doi: https://doi.org/10.1007/s12262-013-0999-9
Redkar R, Karkera PJ, Krishnan J, Hathiramani V. Subtotal Pancreatectomy for Congenital Hyperinsulinism: Our Experience and Review of Literature. Indian J Surg. 2015 Dec;77:778-82. doi: 10.1007/s12262-013-0999-9. Epub 2013 Oct 31. PMID: 27011456; PMCID: PMC4775638.
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Indian J Surg. 2015 Dec;77:778-82. doi: 10.1007/s12262-013-0999-9. Epub 2013 Oct 31.

Subtotal Pancreatectomy for Congenital Hyperinsulinism: Our Experience and Review of Literature.

The Indian journal of surgery

Rajeev Redkar, Parag J Karkera, Janani Krishnan, Varun Hathiramani

Affiliations

  1. Department of Pediatric Surgery, Lilavati Hospital and Research Centre, Bandra Reclaimation, Bandra (West), Mumbai, 400 016 India ; Shushrusha Citizen's Co-operative Hospital, Dadar, Mumbai, India ; Bai Jerbai Wadia Children's Hospital, Parel, Mumbai, India.
  2. Department of Pediatric Surgery, Lilavati Hospital and Research Centre, Bandra Reclaimation, Bandra (West), Mumbai, 400 016 India.

PMID: 27011456 PMCID: PMC4775638 DOI: 10.1007/s12262-013-0999-9

Abstract

Congenital hyperinsulinism (HI) is characterized by profound hypoglycemia caused by inappropriate insulin secretion. HI is a heterogeneous disorder with at least two histologic lesions and several implicated genes. If HI is caused by a focal lesion, elective surgery is the treatment of choice because it leads to complete recovery without diabetes. On the contrary, near-total pancreatectomy though recommended for diffuse HI, long-term risks of endocrine and exocrine deficiencies are present. Between the years 2006-2011, three patients of HI were referred to and operated by a single surgeon. The preoperative diagnosis was confirmed by recurrent hypoglycemia, inappropriately high insulin levels, and augmented glucose requirements. The medical records of all three patients were reviewed to study their clinical features, medical and surgical treatment, and postoperative outcome (short- and long-term). There were three patients in this series (male/female ratio, 1:2), all presenting in the neonatal age. All patients failed medical treatment, and radiological imaging did not reveal any pancreatic lesion. All patients underwent subtotal (80 %) pancreatectomy. Two patients had diffuse type of HI and one focal HI. One patient had transient hyperglycemia for 3 months, which needed insulin supplementation. No patient has developed recurrent hypoglycemia, malabsorption syndrome, or any neurological sequelae until the last follow-up. Doing subtotal or near-total pancreatectomy in diffuse type of HI still remains controversial as one has a higher risk of recurrent hypoglycemia, while the other has a higher rate of insulin dependent diabetes mellitus. Subtotal (80 %) pancreatectomy may be considered as the primary modality of surgical intervention in diffuse type of HI, especially when the diagnostic facilities are limited or diagnosis is not known after preliminary investigations. This minimizes the chances of postoperative diabetes mellitus, and redo surgery can always be considered if there is recurrent hypoglycemia.

Keywords: Congenital hyperinsulinism; Hypoglycemia; Nesidioblastosis; Subtotal pancreatectomy

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