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

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  • 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 ARTICLES
  • URL: https://rps-journal.ru/jour/article/view/247

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.

About the authors

A. I. Chubarova

Russian National Research Medical University named after N.I. Pirogov; Filatov Moscow Pediatric Clinical Hospital No. 13

Author for correspondence.
Email: noemail@neicon.ru

Russian Federation

E. A. Kostomarova

Russian National Research Medical University named after N.I. Pirogov; Filatov Moscow Pediatric Clinical Hospital No. 13

Email: eleni@abloy.ru

Russian Federation

O. G. Mokrushina

Russian National Research Medical University named after N.I. Pirogov; Filatov Moscow Pediatric Clinical Hospital No. 13

Email: mokrushina@ya.ru

Russian Federation

V. S. Shumikhin

Russian National Research Medical University named after N.I. Pirogov; Filatov Moscow Pediatric Clinical Hospital No. 13

Email: vashou@gmail.com

Russian Federation

S. V. Cherkasova

Russian National Research Medical University named after N.I. Pirogov; Filatov Moscow Pediatric Clinical Hospital No. 13

Email: cherkasovasv@mail.ru

Russian Federation

N. F. Tshapov

Russian National Research Medical University named after N.I. Pirogov; Filatov Moscow Pediatric Clinical Hospital No. 13

Email: n.f.shchapov@gmail.com

Russian Federation

I. B. Dmitrieva

Filatov Moscow Pediatric Clinical Hospital No. 13

Email: contractura@mail.ru

Russian Federation

E. V. Selivanova

Filatov Moscow Pediatric Clinical Hospital No. 13

Email: Lenafast@mail.ru

Russian Federation

L. V. Petrova

Russian National Research Medical University named after N.I. Pirogov; Filatov Moscow Pediatric Clinical Hospital No. 13

Email: celine1988@mail.ru

Russian Federation

S. K. Emirbekova

Filatov Moscow Pediatric Clinical Hospital No. 13

Email: imfo@neosurg.ru

Russian Federation

O. V. Koshko

Filatov Moscow Pediatric Clinical Hospital No. 13

Email: imfo@neosurg.ru

Russian Federation

N. S. Korchagina

Filatov Moscow Pediatric Clinical Hospital No. 13

Email: nskorchagina@gmail.com

Russian Federation

References

  1. 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.
  2. Moore S.W. Total colonic aganglionosis and Hirschsprung’s disease: a review // Pediatr. Surg. Int. 2015. Jan. Vol. 31. No. 1. P. 1-9.
  3. 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.
  4. 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.
  5. 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.
  6. Hoishneider and Puri P. (eds.). Hirshprung’s Desease and Allied Disorders // Springer-Verlad Berlin Heidelberg. 2008.
  7. Исаков Ю.Ф., Володин Н.Н., Гераськин А.В. Неонатальная хирургия. М.: Издательство «Династия», 2011. С. 532-547.
  8. 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.
  9. 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.
  10. 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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