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

作者: 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
隶属关系:
1. Russian National Research Medical University named after N.I. Pirogov
2. Filatov Moscow Pediatric Clinical Hospital No. 13
期: 卷 6, 编号 2 (2016)
页面: 26-37
栏目: Original Study Articles
##submission.dateSubmitted##: 30.01.2017
##submission.datePublished##: 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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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.

关键词

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

参考

Hukkinen M., Koivusalo A., Merras-Salmio L., Rintala R.J., Pakarinen M. P. et al. Postoperative outcome and survival in relation to small intestinal involvement of total colonic aganglionosis // Journ. of Pediatric Surgery. 2015. Nov. Vol. 50. No. 11. P. 1859-1864.
Moore S.W. Total colonic aganglionosis and Hirschsprung’s disease: a review // Pediatr. Surg. Int. 2015. Jan. Vol. 31. No. 1. P. 1-9.
Ruttenstock E., Puri P. A meta-analysis of clinical outcome in patients with total intestinal aganglionosis // Pediatr. Surg. Int. 2009. Vol. 25. P. 833-839.
Finaly R., Cohen Z., Mares A.J. Near Total Intestinal. Aganglionosis with Extreme Short-Bowel Syndrome A Difficult Surgical Dilemma // Eur. J. Pediatr. Surg., 1999. Aug. Vol. 9. No. 4. P. 253-255.
Harajai M.M., Puri B., Ram R. Total colonic aganglionosis (zuelzerwilson syndrome) // Medical Journal Armed Forces India (MJAFI). 2000. Vol. 56. No. 4. P. 357-358.
Hoishneider and Puri P. (eds.). Hirshprung’s Desease and Allied Disorders // Springer-Verlad Berlin Heidelberg. 2008.
Исаков Ю.Ф., Володин Н.Н., Гераськин А.В. Неонатальная хирургия. М.: Издательство «Династия», 2011. С. 532-547.
Кучеров Ю.И., Жиркова Ю.В., Шишкина Т.Н., Рехвиашвили М.Г. Пороки развития кишечника у недоношенных, протекающие под маской некротического энтероколита // Вопросы современной педиатрии. 2015. Том 14. № 2. С. 300-304.
Ziegler M.M., Ross A.J. and Bishop H.C. Total Intestinal Aganglionosis: A New Technique for Prolonged Survival // Journal of Pediatric Surgery. 1987. Jan. Vol. 22. No. 1. P. 82-83.
Kostomarova Е., Chubarova А., Zhikhareva N., Radchenko E. The use of skin-tunneled central venous catheters reduces the incidence of complications in children with short bowel syndrome receiving home parenteral nutrition // Clinical Nutrition. 2015. Vol. 34.

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

全文:

详细

关键词

作者简介

A. Chubarova

E. Kostomarova

O. Mokrushina

V. Shumikhin

S. Cherkasova

N. Tshapov

I. Dmitrieva

E. Selivanova

L. Petrova

S. Emirbekova

O. Koshko

N. Korchagina

参考

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