Skip to main content
Log in

Nesidioblastosis in infants: report of two cases and review of the literature

  • Case Reports
  • Published:
Pediatric Surgery International Aims and scope Submit manuscript

Abstract

In the last 2 years, two newborns were found to have persistent hypoglycaemia due to nesidioblastosis. Both required more than 15 mg/kg/min IV glucose and had inappropriately high plasma insulin levels. Near-total pancreatectomy (NTP) with splenic conservation was curative in both, with negligible morbidity and no mortality. Prompt diagnosis and stringent control of plasma glucose with hourly monitoring in an intensive care unit, use of a central venous line, and oral diazoxide prevented subsequent neurological handicaps. A high index of suspicion for this rare disorder should be kept in mind in a chubby infant who is jittery, apathetic, and has seizures with hypoglycaemia. Medical management is required to confirm the nonketotic, hyperinsulinaemic hypoglycaemia, whereas NTP provides a long-term cure.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Similar content being viewed by others

References

  1. Avery GB (1987) Glucose homeostasis in the newborn. In: Neonatology. J B Lippincott Co, pp 694–699

  2. Aynsley-Green A, Polak JM, Block SR, Gough MH, Keeling J, Ashcraft SJH, Turner RC, Baun JD (1981) Nesidioblastosis of the pancreas: definition of the syndrome and management of the severe neonatal hyperinsulinemic hypoglycemia. Arch Dis Child 56: 496–508

    Google Scholar 

  3. Brown RE, Young RB (1970) A possible role for the pancreas in the pathogenesis of neonatal leucine sensitive hypoglycemia. Am J Dig Dis 15: 65–72

    Google Scholar 

  4. Campbell JR (1987) Editorial — Surgeon's approach to hypoglycemia in infants and children. West J Med 146: 612–613

    Google Scholar 

  5. Carcassone M, Delarue A, Le Tourneau JN (1983) Surgical treatment of organic pancreatic hypoglycemia in the pediatric age. Pediatr Surg 18: 75–79

    Google Scholar 

  6. Cox JN, Guelpa G, Terrapon M (1976) Letter Lancet 2: 739–740

    Google Scholar 

  7. Crough MH (1984) The surgical treatment of hyperinsulinism in infancy and childhood. Br J Surg 71: 75–78

    Google Scholar 

  8. Daga SR, Talwalkar VC, Mukhadan Y, Nagle SB (1986) Beta cell nesidioblastosis. Ind Pediatr 23: 213–215

    Google Scholar 

  9. Davidson PM, Young DG, Logan RW, Lockhart S, Patrick WJA (1984) Alloxan therapy for nesidioblastosis. J Pediatr 19: 87–89

    Google Scholar 

  10. Fonkalsrud EW, Trout HH, Lippe B, LaFranchi S, Dakake C (1974) Idiopathic hypoglycemia in infancy. Arch Surg 108: 801–802

    Google Scholar 

  11. Gabbay KH (1978) Case reports. N Engl J Med 299: 241–248

    Google Scholar 

  12. Grappusso PA, Deluca F, O'Shea PA, Swartz R (1985) Near total pancreatectomy for hyperinsulinism — report of transientness of diabetes mellitus. Acta Pediatr Scand 74: 311–315

    Google Scholar 

  13. Harken AH, Filler RM, Auruskin N, Grigler JF Jr (1971) The role of pancreatectomy in the treatment of unremitting hypoglycemia of infancy. J Pediatr Surg 6: 284–289

    Google Scholar 

  14. Harrison TS, Fajans SS, Floyd JC, Thompson NW, Rasbach DA, Santes RJ, Cohen C (1984) Recognition and management of hyperinsulinemic hypoglycaemia. World J Surg 8: 583–589

    Google Scholar 

  15. Kramer JL, Bell MJ, DeSchruer K, Bower RJ, Ternberg JL, White NH (1982) Clinical and histologic indications for extensive pancreatic resection in nesidioblastosis. Am J Surg 143: 116–119

    Google Scholar 

  16. Laidlaw GF (1938) Nesidioblastoma: islet cell tumor of the pancreas. Am J Pathol 14: 125–134

    Google Scholar 

  17. Langer FC, Filler RM, Wesson DE, Sherwood G, Cutz E (1984) Surgical management of persistent neonatal hypoglycemia due to islet cell dysplasia. J Pediatr Surg 19: 786–792

    Google Scholar 

  18. Martin LW, Ryckman RC, Sheldon CA (1984) Experience with 95% pancreatectomy and splenic salvage for neonatal nesidioblastosis. Ann Surg 200: 355–362

    Google Scholar 

  19. Mathew PM, Young JM, Abu-osba YK, Mulhelm BD, Hemmoudi S, Handan JA, Saadi AR (1987) Clin Paediatr 27: 148

    Google Scholar 

  20. Moazam F, Rodgers BM, Talbert JL, Rosen Bloom AL (1982) Near total pancreatectomy in persistent infantile hypoglycemia. Am J Surg 143: 116

    Google Scholar 

  21. Moosa AR, Baker L, Lowqell Jones M (1987) Hypoglycemic syndrome in infancy and childhood. A surgeons perspective. West J Med 146: 585

    Google Scholar 

  22. Polak JM, Bloom SR (1980) Somatostatin deficiency in persistent neonatal hyperinsulinaemic hypoglycaemia. In: Marks V, Andreani D (eds) Current views on hypoglycaemia and glucagon. Academic Press, London, pp 367–378

    Google Scholar 

  23. Spitz L, Buick DG, Grant OB (1986) Surgical treatment of nesidioblastosis. Pediatr Surg Int 1: 26–29

    Google Scholar 

  24. Stephens La Franchi (1987) Hypoglycemia of infancy and childhood. Pediatr Clin North Am 34: 961–982

    Google Scholar 

  25. Telander RL, Wolf SA, Simmons PS, Zimmermas D, Haymond MW (1986) Endocrine disorders of the pancreas and adrenal cortex in pediatric patients. Mayo Clin Proc 61: 459–466

    Google Scholar 

  26. Warden MJ, German JC, Buckingham BA (1988) Surgical management of hyperinsulinism in infancy due to nesidioblastosis. J Paediatr Surg 23: 462–465

    Google Scholar 

  27. Wooley M (1983) In discussion of Cambell JR, Rivers SP, Harrison MW, Cambell TJ. Am J Surg 146: 21–28

    Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Additional information

Offprint requests to: M. Roohatgi

Rights and permissions

Reprints and permissions

About this article

Cite this article

Rohatgi, M., Gupta, D.K., Menon, P.S.N. et al. Nesidioblastosis in infants: report of two cases and review of the literature. Pediatr Surg Int 6, 365–367 (1991). https://doi.org/10.1007/BF00178659

Download citation

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF00178659

Key words

Navigation