COMPLICATIONS OF DIAGNOSTICS AND TREATMENT OF PATIENTS WITH TOTAL AND SUBTOTAL INTESTINAL AGANGLIONOSIS

A. I. Chubarova; Чубарова Антонина Игоревна; E. A. Kostomarova; Костомарова Елена Андреевна; O. G. Mokrushina; Мокрушина Ольга Геннадьевна; V. S. Shumikhin; Шумихин Василий Сергеевич; S. V. Cherkasova; Черкасова Светлана Вячеславовна; N. F. Tshapov; Щапов Николай Федорович; I. B. Dmitrieva; Дмитриева Ирина Борисовна; E. V. Selivanova; Селиванова Елена Владимировна; L. V. Petrova; Петрова Любовь Валериановна; S. K. Emirbekova; Эмирбекова Светлана Курбановна; O. V. Koshko; Кошко Ольга Владимировна; N. S. Korchagina; Корчагина Наталья Сергеевна

doi:10.17816/psaic247

COMPLICATIONS OF DIAGNOSTICS AND TREATMENT OF PATIENTS WITH TOTAL AND SUBTOTAL INTESTINAL AGANGLIONOSIS

Authors: Chubarova A.I.¹^,2, Kostomarova E.A.¹^,2, Mokrushina O.G.¹^,2, Shumikhin V.S.¹^,2, Cherkasova S.V.¹^,2, Tshapov N.F.¹^,2, Dmitrieva I.B.², Selivanova E.V.², Petrova L.V.¹^,2, Emirbekova S.K.², Koshko O.V.², Korchagina N.S.²
Affiliations:
1. Russian National Research Medical University named after N.I. Pirogov
2. Filatov Moscow Pediatric Clinical Hospital No. 13
Issue: Vol 6, No 2 (2016)
Pages: 26-37
Section: Original Study Articles
Submitted: 30.01.2017
Published: 30.06.2016
URL: https://rps-journal.ru/jour/article/view/247
DOI: https://doi.org/10.17816/psaic247
ID: 247

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Full Text

Abstract

Total and subtotal intestinal aganglionosis (hypogangliosis) is a severe and potentially lethal condition related to neuromuscular intestinal diseases. Some authors consider it as a rare, atypical and most severe form of rectocolic aganglionosis which constitutes 1% of all cases related to the disease [6,7,8]. According to meta-analysis performed by Ruttenstock, 68 patients with total aganglionosis were found in the world literature for 2009 [8]. Different surgical methods (Martin, Kimura, Zigler procedures (extended myectomy and myotomy), bringing down the ileum with formation of ileoanal anastomosis, Bianchi’s plasty and STEP-procedure, intestinal transplantation) are proposed for treatment of total intestinal aganglionosis; however, none of them were considered as superior to the others [3,6,8]. The article describes three clinical cases of total and subtotal intestinal aganglionosis in patients of the neonatal surgery department of Filatov Moscow Pediatric Clinical Hospital No. 13 in 2015-2016. According to our experience, the tactics of sparing resection with preservation of the small intestine maximum length is being optimal. However, it doesn’t exclude the need for durative parenteral nutritional support. Using domestic parenteral nutrition in the management of this group of patients is pharmaco-economically viable.

Keywords

тотальный аганглиоз, гипоганглиоз кишечника, болезнь Гишпрунга, парентеральное питание, домашнее парентеральное питание, total intestinal aganglionosis, rectocolic aganglionosis, parenteral nutrition, domestic parenteral nutrition

References

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COMPLICATIONS OF DIAGNOSTICS AND TREATMENT OF PATIENTS WITH TOTAL AND SUBTOTAL INTESTINAL AGANGLIONOSIS

Full Text

Abstract

Keywords

About the authors

A. I. Chubarova

E. A. Kostomarova

O. G. Mokrushina

V. S. Shumikhin

S. V. Cherkasova

N. F. Tshapov

I. B. Dmitrieva

E. V. Selivanova

L. V. Petrova

S. K. Emirbekova

O. V. Koshko

N. S. Korchagina

References

Supplementary files

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