Детская хирургия. Журнал им. Ю.Ф. Исакова. 2023; 27: 213-220
Мезопортальное шунтирование с врождённым портокавальным шунтом у ребёнка в возрасте 1 года и 3 месяцев
https://doi.org/10.55308/1560-9510-2023-27-3-213-220Аннотация
Введение. Мальформация Абернети (CEPS) – это редкое состояние, при котором портомезентериальная кровь минует печень через частичное или полное шунтирование в нижнюю полую вену. Данная патология крайне редко встречается у детей. Клинически может протекать практически бессимптомно или под маской других заболеваний. Проявляется признаками печеночной энцефалопатии, легочной гипертензии или гепатопульмонального синдрома. Мезопортальное шунтирование – это вариант коррекции портокавальных шунтов с возобновлением физиологичного кровотока в печени.
На базе ГБУЗ г. Москвы «Детская городская клиническая больница им. Н.Ф. Филатова ДЗМ» с 2016 г. действует центр лечения аномалий развития заболеваний гепато-панкреато-билиарной системы у детей. К настоящему времени в центре выполнено более 1500 оперативных вмешательств на органах билио-панкреато-дуоденальной зоны. Всего за период с 2016 по 2022 гг. 13 детям с врождёнными портокавальными шунтами выполнено перекрытие эндоваскулярным окклюдером, хирургическая перевязка выполнена 6 детям.
Описание клинического наблюдения. В данной статье описывается клинический случай ребёнка с врождённым портокавальным шунтом. В период новорождённости, при проведении комплексного инструментального исследования отмечалось полное отсутствие портального кровотока в печени. Селезёночная вена и верхняя брыжеечная вена на уровне ворот печени единым стволом впадали в нижнюю полую вену. Считается, что при таком типе порока единственным эффективным методом лечения является трансплантация печени. Несмотря на неоднозначную анатомию сосудов печени, пациенту было выполнено мезопортальное шунтирование, восстановившее архитектонику печени ребёнка и вернув его к абсолютно нормальной жизни без отсутствия жизнеугрожающих факторов.
Заключение. Данный клинический случай демонстрирует единственный описанный вариант полного хирургического излечения сложного порока сосудов печени у ребёнка.
Список литературы
1. Anna Baiges, Fanny Turon, Macarena Simón-Talero, Stephanie Tasayco, Javier Bueno, Kamal Zekrini, Aurélie Plessier, Stéphanie FranchiAbella. Congenital Extrahepatic Portosystemic Shunts (Abernethy Malformation): An International Observational Study. Hepatology. 2020 Feb; 71(2): 658–69.
2. Zhang B., Wu K.T., Li L., Lai H.Y. Catheter embolization of type II congenital extrahepatic portosystemic shunt with hematochezia: a case series and review of the literature. Cardiovasc Intervent Radiol. 2018; 41: 1121–7.
3. Jin-Shan Zhang, Long Li. Surgical ligation of a portosystemic shunt for the treatment of type II Abernethy malformation in 12 children. J Vasc Surg Venous Lymphat Disord. 2021 Mar; 9(2): 444–51.
4. Jerneja Peček, Petja Fister, Matjaž Homan. Abernethy syndrome in Slovenian children: Five case reports and review of literature. World J Gastroenterol. 2020 Oct 7; 26(37): 5731–44.
5. Frank DiPaola, Andrew T. Trout, Ashley E. Walther, Anita Gupta, Rachel Sheridan, Kathleen M. Campbell, Greg Tiao. Congenital Portosystemic Shunts in Children: Associations, Complications, and Outcomes. Dig Dis Sci. 2020 Apr; 65(4): 1239–51.
6. Parveen Kumar, Mona Bhatia,Amit Garg,Shashank Jain, Khemendra Kumar. Abernethy malformation: A comprehensive review. Diagn Interv Radiol. 2022; 28: 21–8.
7. Eun Sil Kim, Ki Wuk Lee. The Characteristics and Outcomes of Abernethy Syndrome in Korean Children: A Single Center Study. Pediatr Gastroenterol Hepatol Nutr. 2019 Jan; 22(1): 80–5.
8. Gong Y., Zhu H., Chen J., Chen Q., Ji M., Pa M., et al. Congenital portosystemic shunts with and without gastrointestinal bleeding – case series. Pediatr Radiol. 2015; 45: 1964–71.
9. Lee H.J., Lee J.H., Huh J., Kang I.S., Lee H.J., Suh Y.L., et al. A case of congenital extra hepatic portocaval shunt(abernethy malformation type 2) with a very largeliver mass and an atrial septal defect. Korean J Pediatr Gastroenterol Nutr. 2008; 11: 56–9.
10. Bernard O., Franchi-Abella S., Branchereau S., Pariente D., Gauthier F., Jacquemin E. Congenital portosystemic shunts in children: recognition, evaluation, and management. Semin Liver Dis. 2012; 32: 273–87.
11. Jain V., Sangdup T., Agarwala S., Bishoi A.K., Chauhan S., Dhua A., Jana M., Kandasamy D., Malik R., Kothari S.S., Patcharu R., Varshney A., Bhatnagar V. Abernethy malformation type 2: varied presentation, management and outcome. J Pediatr Surg. 2019; 54: 760–5.
12. Takama Y., Nakamura T., Santo K., Yoneda A. Liver resection for a congenital intrahepatic portosystemic shunt in a child with hyperammonemia and hypermanganesemia: a case report. Surg Case Rep. 2020; 6: 73.
13. Xiang W., Wang H., Si Z.Z., Chen G.S., Wang G.W., Li T. Type I congenital extrahepatic portosystemic shunt treated by orthotopic liver transplantation: A case report. World J Clin Cases. 2019; 7: 903–7.
14. Howard E.R., Davenport M. Congenital extrahepatic portocaval shunts–the Abernethy malformation. J Pediatr Surg. 1997; 32: 494–7.
15. Knirsch W., Benz D.C., Bühr P., Quandt D., Weber R., Kellenberger C., Braegger C.P., Kretschmar O. Catheter interventional treatment of congenital portosystemic venous shunts in childhood. Catheter Cardiovasc Interv. 2016; 87: 1281–92.
16. Elias N., Scirica C.V., Hertl M. Liver transplantation for the Abernathy malformation. N Engl J Med. 2008; 358.
17. Kanazawa H, Nosaka S, Miyazaki O, et al. The classification based on intrahepatic portal sys- tem for congenital portosystemic shunts. J Pediatr Surg. 2015; 50(4): 688-95.
18. Vishesh Jain, Tsering Sangdup, Sandeep Agarwala, Akshay Kumar Bishoi, Sandeep Chauhan. Abernethy malformation type 2: varied presentation, management and outcome. Journal of Pediatric Surgery. 2019; 54: 760–5.
19. Franchi-Abella S., Gonzales E., Ackermann O., Branchereau S., Pariente D., Guérin F. Congenital portosystemic shunts: diagnosis and treatment. Abdom. Radiol. 2018; 43: 2023–36.
20. Murray C.P., Yoo S.J., Babyn P.S. Congenital extrahepatic portosystemic shunts. Pediatr Radiol. 2003; 33(9): 614–20.
Russian Journal of Pediatric Surgery. 2023; 27: 213-220
Mesoportal bypass surgery with congenital portocaval shunt in a child aged 1 year and 3 months
https://doi.org/10.55308/1560-9510-2023-27-3-213-220Abstract
Introduction. Abernethy malformation (CEPS) is a rare condition in which portomesenteric blood bypasses the liver through a partial or complete shunt into the inferior vena cava. This pathology is extremely rare in children. Clinically, it can occur almost asymptomatically or under the guise of other diseases. It is manifested by signs of hepatic encephalopathy, pulmonary hypertension or hepatopulmonary syndrome. Mesoportal bypass surgery is an option for correcting portocaval shunts with the restoration of physiological blood flow in the liver.
Since 2016, a center for treating developmental anomalies in the hepato-pancreato-biliary system in children has been operating in Filatov Municipal Children's Hospital (Moscow). To date, the center has performed more than 1,500 surgical interventions on the organs of biliopancreato-duodenal zone. For the period from 2016 to 2022, an endovascular occluder was used in 13 children with congenital portocaval shunts; surgical dressing – in 6 children.
Description of clinical observation. This article describes a clinical case of a child with a congenital portocaval shunt. A comprehensive instrumental examination revealed a complete absence of portal blood flow in the liver of a newborn baby . The spleen vein and superior mesenteric vein flowed into the inferior vena cava as a single trunk. It is believed that liver transplantation is the only effective treatment for this type of pathology. Despite the ambiguous anatomy of liver vessels, the child was operated on for the mesoportal bypass. The architectonics of child's liver was restored, and he returned to an absolutely normal life without any life-threatening factors.
Conclusion. This clinical case demonstrates the only described case of complete surgical cure of a complex liver vascular defect.
References
1. Anna Baiges, Fanny Turon, Macarena Simón-Talero, Stephanie Tasayco, Javier Bueno, Kamal Zekrini, Aurélie Plessier, Stéphanie FranchiAbella. Congenital Extrahepatic Portosystemic Shunts (Abernethy Malformation): An International Observational Study. Hepatology. 2020 Feb; 71(2): 658–69.
2. Zhang B., Wu K.T., Li L., Lai H.Y. Catheter embolization of type II congenital extrahepatic portosystemic shunt with hematochezia: a case series and review of the literature. Cardiovasc Intervent Radiol. 2018; 41: 1121–7.
3. Jin-Shan Zhang, Long Li. Surgical ligation of a portosystemic shunt for the treatment of type II Abernethy malformation in 12 children. J Vasc Surg Venous Lymphat Disord. 2021 Mar; 9(2): 444–51.
4. Jerneja Peček, Petja Fister, Matjaž Homan. Abernethy syndrome in Slovenian children: Five case reports and review of literature. World J Gastroenterol. 2020 Oct 7; 26(37): 5731–44.
5. Frank DiPaola, Andrew T. Trout, Ashley E. Walther, Anita Gupta, Rachel Sheridan, Kathleen M. Campbell, Greg Tiao. Congenital Portosystemic Shunts in Children: Associations, Complications, and Outcomes. Dig Dis Sci. 2020 Apr; 65(4): 1239–51.
6. Parveen Kumar, Mona Bhatia,Amit Garg,Shashank Jain, Khemendra Kumar. Abernethy malformation: A comprehensive review. Diagn Interv Radiol. 2022; 28: 21–8.
7. Eun Sil Kim, Ki Wuk Lee. The Characteristics and Outcomes of Abernethy Syndrome in Korean Children: A Single Center Study. Pediatr Gastroenterol Hepatol Nutr. 2019 Jan; 22(1): 80–5.
8. Gong Y., Zhu H., Chen J., Chen Q., Ji M., Pa M., et al. Congenital portosystemic shunts with and without gastrointestinal bleeding – case series. Pediatr Radiol. 2015; 45: 1964–71.
9. Lee H.J., Lee J.H., Huh J., Kang I.S., Lee H.J., Suh Y.L., et al. A case of congenital extra hepatic portocaval shunt(abernethy malformation type 2) with a very largeliver mass and an atrial septal defect. Korean J Pediatr Gastroenterol Nutr. 2008; 11: 56–9.
10. Bernard O., Franchi-Abella S., Branchereau S., Pariente D., Gauthier F., Jacquemin E. Congenital portosystemic shunts in children: recognition, evaluation, and management. Semin Liver Dis. 2012; 32: 273–87.
11. Jain V., Sangdup T., Agarwala S., Bishoi A.K., Chauhan S., Dhua A., Jana M., Kandasamy D., Malik R., Kothari S.S., Patcharu R., Varshney A., Bhatnagar V. Abernethy malformation type 2: varied presentation, management and outcome. J Pediatr Surg. 2019; 54: 760–5.
12. Takama Y., Nakamura T., Santo K., Yoneda A. Liver resection for a congenital intrahepatic portosystemic shunt in a child with hyperammonemia and hypermanganesemia: a case report. Surg Case Rep. 2020; 6: 73.
13. Xiang W., Wang H., Si Z.Z., Chen G.S., Wang G.W., Li T. Type I congenital extrahepatic portosystemic shunt treated by orthotopic liver transplantation: A case report. World J Clin Cases. 2019; 7: 903–7.
14. Howard E.R., Davenport M. Congenital extrahepatic portocaval shunts–the Abernethy malformation. J Pediatr Surg. 1997; 32: 494–7.
15. Knirsch W., Benz D.C., Bühr P., Quandt D., Weber R., Kellenberger C., Braegger C.P., Kretschmar O. Catheter interventional treatment of congenital portosystemic venous shunts in childhood. Catheter Cardiovasc Interv. 2016; 87: 1281–92.
16. Elias N., Scirica C.V., Hertl M. Liver transplantation for the Abernathy malformation. N Engl J Med. 2008; 358.
17. Kanazawa H, Nosaka S, Miyazaki O, et al. The classification based on intrahepatic portal sys- tem for congenital portosystemic shunts. J Pediatr Surg. 2015; 50(4): 688-95.
18. Vishesh Jain, Tsering Sangdup, Sandeep Agarwala, Akshay Kumar Bishoi, Sandeep Chauhan. Abernethy malformation type 2: varied presentation, management and outcome. Journal of Pediatric Surgery. 2019; 54: 760–5.
19. Franchi-Abella S., Gonzales E., Ackermann O., Branchereau S., Pariente D., Guérin F. Congenital portosystemic shunts: diagnosis and treatment. Abdom. Radiol. 2018; 43: 2023–36.
20. Murray C.P., Yoo S.J., Babyn P.S. Congenital extrahepatic portosystemic shunts. Pediatr Radiol. 2003; 33(9): 614–20.
