SURGICAL TREATMENT OF ATIPICAL FORMS OF CONGENITAL HYPERINSULINISM



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Abstract

Introduction. 30-60% of all patients with congenital hyperinsulinism have a delayed psychomotor development, and 15-25% of them have a severe organic brain damage, including epilepsy. The timely diagnostics and intensive care can prevent severe neurological complications. If the conservative therapy is not effective, then the surgical treatment is a need. Objective: To demonstrate features of surgical correction of atypical forms of congenital hyperinsulinism. Material and methods. 11 children with atypical forms of congenital hyperinsulinism were operated on in the department of pediatric surgery in the V.A. Almazov National Medical Center in 2017-03.2019. Results. 2 children demonstrated a complete relief of hyperinsulinism in 9 months (1.5 years) of follow-up; one patient had a significant improvement of his psycho-motor development. Conclusion. Some children with presumably focal forms of hyperinsulinism, by PET-CT findings, may have histologically atypical forms. In the atypical lesion of the pancreas, an intraoperative biopsy dramatically changes the planned volume of pancreatectomy up to almost total removal of the gland.

About the authors

A. A. Sukhotskaya

V.A. Almazov National Medical Research Centre

Author for correspondence.
Email: sukhotskaya_aa@almazovcentre.ru
ORCID iD: 0000-0002-8734-2227
Russian Federation

V. G. Bairov

V.A. Almazov National Medical Research Centre

ORCID iD: 0000-0002-8446-830X
Russian Federation

L. B. Mitrofanova

V.A. Almazov National Medical Research Centre

Russian Federation

D. V. Ryzhkova

V.A. Almazov National Medical Research Centre

Russian Federation

I. L. Nikitina

V.A. Almazov National Medical Research Centre

Russian Federation

S. A. Amidhonova

V.A. Almazov National Medical Research Centre

Russian Federation

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