Email this article 
SURGICAL TREATMENT OF FOCAL FORMS OF CONGENITAL HYPERINSULINISM: IS ALL CLEAR?
- Authors: Sukhotskaya A.A.1, Bairov V.G.1, Nikitina I.L.1, Ryzhkova D.V.1, Mitrofanova L.B.1, Amidkhonova S.A.1
-
Affiliations:
- V.A. Almazov National Medical Research Centre, Institute of Perinatology and Pediatrics, Institute of Surgery for Congenital and Hereditary Pathologies, Surgical Department of Developmental Malformations
- Issue: Vol 23, No 6 (2019)
- Pages: 296-302
- Section: ORIGINAL ARTICLES
- Submitted: 05.03.2021
- Published: 05.12.2019
- URL: https://jps-nmp.ru/jour/article/view/179
- DOI: https://doi.org/10.18821/1560-9510-2019-23-6-296-302
- ID: 179
Cite item
Full Text
Abstract
Introduction. 30-60% of all patients with congenital hyperinsulinism have a delayed psychomotor development, and 15-25% of them have severe organic brain damage, including epilepsy. Timely diagnostics and intensive treatment can prevent severe neurological complications. With the ineffectiveness of conservative therapy, surgical treatment is a necessary alternative. Objective. To define indications for various options of surgical correction of focal forms of congenital hyperinsulinism. Material and Methods. In the Department of Pediatric Surgery of V.A. Almazova National Medical Research Centre, in 2017-03.2019, 14 children with focal forms of congenital hyperinsulinism were operated after analyzing their PET-CT findings; in 2 patients out of them they were doubtful. Results. 13 (93%) patients had a complete relief of hyperinsulinism and a significant improvement in their psycho-motor development. 10 (71%) of 14 children had a complete recovery; 1 (7%) - a significant positive trend. 3 (21%) patients needed insulin therapy with minimal dosages. They all were children with an atypical (mixed adenomatous-diffuse) form of hyperinsulinism. Conclusions. In case of focal lesions in the pancreas, partial pancreatectomy with maximal preservation of healthy tissue, defined by the express biopsy, and with any lesion localization is indicated. For atypical forms, the volume for pancreatectomy dissection is defined individually.
About the authors
A. A. Sukhotskaya
V.A. Almazov National Medical Research Centre, Institute of Perinatology and Pediatrics, Institute of Surgery for Congenital and Hereditary Pathologies, Surgical Department of Developmental Malformations
Author for correspondence.
Email: sukhotskaya_aa@almazovcentre.ru
ORCID iD: 0000-0002-8734-2227
Россия
V. G. Bairov
V.A. Almazov National Medical Research Centre, Institute of Perinatology and Pediatrics, Institute of Surgery for Congenital and Hereditary Pathologies, Surgical Department of Developmental Malformations
Email: noemail@neicon.ru
Россия
I. L. Nikitina
V.A. Almazov National Medical Research Centre, Institute of Perinatology and Pediatrics, Institute of Surgery for Congenital and Hereditary Pathologies, Surgical Department of Developmental Malformations
Email: noemail@neicon.ru
Россия
D. V. Ryzhkova
V.A. Almazov National Medical Research Centre, Institute of Perinatology and Pediatrics, Institute of Surgery for Congenital and Hereditary Pathologies, Surgical Department of Developmental Malformations
Email: noemail@neicon.ru
Россия
L. B. Mitrofanova
V.A. Almazov National Medical Research Centre, Institute of Perinatology and Pediatrics, Institute of Surgery for Congenital and Hereditary Pathologies, Surgical Department of Developmental Malformations
Email: noemail@neicon.ru
Россия
S. A. Amidkhonova
V.A. Almazov National Medical Research Centre, Institute of Perinatology and Pediatrics, Institute of Surgery for Congenital and Hereditary Pathologies, Surgical Department of Developmental Malformations
Email: noemail@neicon.ru
Россия
References
- McQuarrie I. Idiopathic spontaneously occurring hypoglycemia in infants; clinical significance of problem and treatment. Am J Dis Child. 1954; 87(4): 399-428.
- Bruining GJ. Recent advances in hyperinsulinism and the pathogenesis of diabetes mellitus. Curr Opin Pediatr. 1990; 2: 758-65.
- Mathew PM, Young JM, Abu-Osba YK, et al. Persistent neonatal hyperinsulinism. ClinPediatr (Phila). 1988; 27: 148-51. [PubMed]
- Roženková K, Güemes M, Shah P, Hussain K1. The Diagnosis and Management of HyperinsulinaemicHypoglycaemia. J Clin Res Pediatr Endocrinol. 2015 Jun; 7(2): 86-97. doi: 10.4274/jcrpe.1891.
- Giurgea I1,et al. Acute insulin responses to calcium and tolbutamide do not differentiate focal from diffuse congenital hyperinsulinism. J. Clin Endocrinol Metab. 2004 Feb; 89(2): 925-9.
- Ribeiro MJ1,et al. The added value of [18F]fluoro-L-DOPA PET in the diagnosis of hyperinsulinism of infancy: a retrospective study involving 49 children. Eur J Nucl Med Mol Imaging. 2007 Dec; 34(12): 2120-8. Epub 2007 Jul 28.
- Lovvorn HN 3rd1, et al. Congenital hyperinsulinism and the surgeon: lessons learned over 35 years. J. Pediatr Surg. 1999 May; 34(5): 786-92; discussion 792-3.
- Меликян М.А., и др. Врожденный гиперинсулинизм: диагностика и лечение. Педиатрия. 2011; 90(1): 59-65
- Graham EA, Hartmann AF. Subtotal resection of the pancreas for hypoglycaemia. Surg. Gynecol. Obstet. 1934; 59: 474-9.
- Gussinyer M., et al. Glucose intolerance and diabetes are observed in the long-term follow-up of nonpancreatectomized patients with persistent hyperinsulinemic hypoglycemia of infancy due to mutations in the ABCC8 gene. Diabetes Care. 2008; 31: 6: 1257-9.
- Lord K1, et al. High Risk of Diabetes and Neurobehavioral Deficits in Individuals With Surgically Treated Hyperinsulinism. J. Clin. Endocrinol. Metab. 2015 Nov; 100(11): 4133-9. doi: 10.1210/jc.2015-2539. Epub 2015 Sep 1.
- Rahier J, Wallon J, Henquin JC. Cell populations in the endocrine pancreas of human neonates and infants. Diabetologia. 1981; 20: 540-6.
- Palladino AA, Stanley CA. Nesidioblastosis no longer! It’s all about genetics. J. Clin. Endocrinol. Metab. 2011; 96: 617-9.
- Pablo Laje, et al. Pancreatic head resection and Roux-en-Y pancreaticojejunostomy for the treatment of the focal form of congenital hyperinsulinism. J. Pediatr. Surg. 2012 Jan; 47(1): 130-5.
- Kassem SA, Ariel I, Thornton PS, Scheimberg I, Glaser B. Beta-cell proliferation and apoptosis in the developing normal human pancreas and in hyperinsulinism of infancy. Diabetes. 2000; 49: 1325-33.
- Katherine Lord, et al. Clinical Presentation and Management of Children With Diffuse and Focal Hyperinsulinism: A Review of 223 Cases. J. Clin.Endocrinol.Metab. 2013 Nov; 98(11): E1786-9. Published online 2013 Sep 20. doi: 10.1210/jc.2013-2094 PMCID: MC3816257 PMID: 24057290
- Mazor-Aronovitch K1, Landau H, Gillis D. Surgical versus non-surgical treatment of congenital hyperinsulinism. Pediatr. Endocrinol. Rev. 2009 Mar; 6(3): 424-30.
- Ismail D1, Werther G. Persistent hyperinsulinaemichypoglycaemia of infancy: 15 years’ experience at the Royal Children’s Hospital (RCH), Melbourne. J. Pediatr.Endocrinol.Metab. 2005 Nov; 18(11): 1103-9.
- Meissner T1, et al. Long-term follow-up of 114 patients with congenital hyperinsulinism. Eur. J. Endocrinol. 2003 Jul; 149(1): 43-51.
Supplementary files
