Congenital portosystemic shunts: surgical treatment experience
- Authors: Stepanov A.E.1, Sukhov M.N.1, Vasilyev K.G.2, Polyaev Y.A.1, Garbuzov R.V.1, Golenishchev A.I.1, Ashmanov K.Y.1, Lyvina I.P.1, Demushkina A.A.1, Tereshina A.A.2
-
Affiliations:
- Russian Children’s Clinical Hospital of Pirogov Russian National Research Medical University
- Pirogov Russian National Research Medical University
- Issue: Vol 12, No 4 (2022)
- Pages: 473-487
- Section: Case reports
- URL: https://journals.rcsi.science/2219-4061/article/view/233295
- DOI: https://doi.org/10.17816/psaic1295
- ID: 233295
Cite item
Abstract
Congenital porto-caval shunts are rare and may have a different morphological structure (intra- and extrahepatic shunts, with or without portal blood flow). The main method of treating patients with this pathology is endovascular shunt occlusion. However, in some cases, this method is ineffective.
The article contains a description of six clinical examples of surgical treatment of congenital porto-systemic shunts in children. In the diagnosis of congenital portosystemic shunts, the leading role belongs to Doppler ultrasound, multislice computed tomography, and angiography. The indication for surgical treatment was the anatomical features of the shunt, which makes endovascular occlusion technically impossible. In one observation a wide Arantian duct was diagnosed, its open ligation was performed. In another case, the portal vein emptied directly into an aneurysmal dilatation, performed reconstructive plastic surgery on the vessels of the portal vein. In the next observation, a pronounced retrograde blood flow was determined along the dilated inferior mesenteric vein, blood was discharged through the sacral plexus into the internal iliac vein. The left internal iliac vein was isolated and ligated, the dysplastic inferior mesenteric vein was ligated and partially removed. In 2 patients, the portal vein flowed directly into the inferior vena cava in the area of aneurysmal expansion; an operation was performed — open ligation of the shunt. In one observation, a deep hypoplasia of the intrahepatic branches of the portal vein was diagnosed, and therefore the restoration of portal blood flow after the closure of the shunt is impossible. The child was sent to decide on a liver transplant.
Each case of congenital porto-caval shunts is unique. The surgeon determines the tactics directly during the operation, depending on the morphological structure of the organs, since the preoperative examination does not always give an unambiguous idea.
Full Text
##article.viewOnOriginalSite##About the authors
Alexey E. Stepanov
Russian Children’s Clinical Hospital of Pirogov Russian National Research Medical University
Email: stepanov_alexey63@mail.ru
ORCID iD: 0000-0002-6181-7036
Cand. Sci. (Med.), Doctor of the Department of Reconstructive and reconstructive Surgery of the abdominal organs
Russian Federation, MoscowMaksim N. Sukhov
Russian Children’s Clinical Hospital of Pirogov Russian National Research Medical University
Email: sukhov79mn@mail.ru
ORCID iD: 0000-0001-6972-9017
SPIN-code: 2363-1150
Dr. Sci. (Med.), Deputy Chief Physician for Surgery
Russian Federation, MoscowKirill G. Vasilyev
Pirogov Russian National Research Medical University
Email: kiravasilyev@yandex.ru
ORCID iD: 0000-0001-5106-1215
Sci. (Med.), Associate Professor of the Department of Pediatric Surgery of the Faculty of Pediatrics
Russian Federation, MoscowYuri A. Polyaev
Russian Children’s Clinical Hospital of Pirogov Russian National Research Medical University
Email: polyaev@inbox.ru
ORCID iD: 0000-0002-9554-6414
SPIN-code: 7587-9843
Dr. Sci. (Med.), Professor, Head of the Department of Endovascular Surgery
Russian Federation, MoscowRoman V. Garbuzov
Russian Children’s Clinical Hospital of Pirogov Russian National Research Medical University
Email: 9369025@mail.ru
ORCID iD: 0000-0002-5287-7889
SPIN-code: 7590-2400
Dr. Sci. (Med.), Doctor of the Department of Endovascular Surgery
Russian Federation, MoscowAnton I. Golenishchev
Russian Children’s Clinical Hospital of Pirogov Russian National Research Medical University
Email: a331821@ya.ru
ORCID iD: 0000-0003-0278-8551
Doctor of the Department of Endovascular surgery
Russian Federation, MoscowKonstantin Yu. Ashmanov
Russian Children’s Clinical Hospital of Pirogov Russian National Research Medical University
Email: ashmanov1964@icloud.com
ORCID iD: 0000-0002-5106-8852
Doctor of the Department of reconstructive Surgery of the abdominal organs
Russian Federation, MoscowIrma P. Lyvina
Russian Children’s Clinical Hospital of Pirogov Russian National Research Medical University
Email: irma_irma@mail.ru
ORCID iD: 0000-0002-8404-3715
Doctor of the microvascular surgery Department No. 2
Russian Federation, MoscowAlice A. Demushkina
Russian Children’s Clinical Hospital of Pirogov Russian National Research Medical University
Email: demushkina.alya@bk.ru
ORCID iD: 0000-0003-1502-8121
Cand. Sci. (Med.), Doctor of the Department of Radiology
Russian Federation, MoscowAnastasia A. Tereshina
Pirogov Russian National Research Medical University
Author for correspondence.
Email: dr.tereshina@mail.ru
ORCID iD: 0000-0001-7224-5777
SPIN-code: 3502-5812
Student of the Pediatric Faculty
Russian Federation, MoscowReferences
- Papamichail M, Pizanias M, Heaton N. Congenital portosystemic venous shunt. European Journal of Pediatrics. 2017;177(3):285–294. doi: 10.1007/s00431-017-3058-x
- Azad S, Arya A, Sitaraman R, Garg A. Abernethy malformation: Our experience from a tertiary cardiac care center and review of literature. Ann Pediatr Cardiol. 2019;12(3):240–247. doi: 10.4103/apc.APC_185_18
- Razumovsky AYu, Galibin IE, Feoktistova EV, et al. Endovascular transjugular closure of arantsieva flow via vascular occluder. Russian Bulletin of Pediatric Surgery, Anesthesiology and Resuscitation. 2016;6(4):78–81. (In Russ.)
- Lin Y, Li X, Li S, et al. Treatment option for abernethy malformation — two cases report and review of the literature. Front Pediatr. 2020;8:497447. doi: 10.3389/fped.2020.497447
- Brunt EM. Grading and staging the histopathological lesions of chronic hepatitis: the Knodell histology activity index and beyond. Hepatology. 2000;31(1):241–246. doi: 10.1002/hep.510310136
- Abernethy J. Account of two instances of uncommon formation in the viscera of the human body: From the Philosophical Transactions of the Royal Society of London. Med Facts Obs. 1797;7:100–108.
- Kanazawa H, Nosaka S, Miyazaki O, et al. The classification based on intrahepatic portal system for congenital portosystemic shunts. J Pediatr Surg. 2015;50(4):688–695. doi: 10.1016/j.jpedsurg.2015.01.009
- Morgan G, Superina R. Congenital absence of the portal vein: two cases and a proposed classification system for portasystemic vascular anomalies. J Pediatr Surg. 1994;29(9):1239–1241. doi: 10.1016/0022-3468(94)90812-5
- Stringer MD. The clinical anatomy of congenital portosystemic venous shunts. Clin Anat. 2008;21(2):147–157. doi: 10.1002/ca.20574
- Lautz TB, Tantemsapya N, Rowell E, Superina RA. Management and classification of type II congenital portosystemic shunts. J Pediatr Surg. 2011;46(2):308–314. doi: 10.1016/j.jpedsurg.2010.11.009
- Blanc T, Guerin F, Franchi-Abella S, et al. Congenital portosystemic surgical strategy. Ann Surg. 2014;260(1):188–198. doi: 10.1097/SLA.0000000000000266
- Bernard O, Franchi-Abella S, Branchereau S, et al. Congenital portosystemic shunts in children: recognition, evaluation, and management. Semin Liver Dis. 2012;32(4):273–287. doi: 10.1055/s-0032-1329896
- Sokollik C, Bandsma RHJ, Gana JC, et al. Congenital portosystemic shunt: characterization of a multisystem disease. J Pediatr Gastroenterol Nutr. 2013;56(6):675–681. doi: 10.1097/MPG.0b013e31828b3750
- Matsuura T, Takahashi Y, Yanagi Y, et al. Surgical strategy according to the anatomical types of congenital portosystemic shunts in children. J Pediatr Surg. 2016;51(12):2099–2104. doi: 10.1016/j.jpedsurg.2016.09.046
- Garbuzov RV, Polyaev YuA, Stepanov AE, Mylnikov AA. Abernathy malformations in children. Experience in endovascular and surgical treatment. Russian Journal of Pediatric Surgery. 2020;24(2):71–77. (In Russ.) doi: 10.18821/1560-9510-2020-24-2-71-77
- Sanada Y, Urahashi T, Ihara Y, et al. The role of operative intervention in management of congenital extrahepatic portosystemic shunt. Surgery. 2012;151(3):404–411. doi: 10.1016/j.surg.2011.07.035