Browse > Article
http://dx.doi.org/10.5223/pghn.2022.25.2.147

Endovascular Treatment of Congenital Portosystemic Shunt: A Single-Center Prospective Study  

Ponce-Dorrego, Maria-Dolores (Department of Interventional Radiology, Hospital General Universitario La Paz)
Hernandez-Cabrero, Teresa (Department of Interventional Radiology, Hospital General Universitario La Paz)
Garzon-Moll, Gonzalo (Department of Interventional Radiology, Hospital General Universitario La Paz)
Publication Information
Pediatric Gastroenterology, Hepatology & Nutrition / v.25, no.2, 2022 , pp. 147-162 More about this Journal
Abstract
Purpose: To design a prospective study on endovascular closure of congenital portosystemic shunts. The primary endpoint was to assess the safety of endovascular closure. The secondary endpoint was to evaluate the clinical, analytical and imaging outcomes of treatment. Methods: Fifteen patients (age range: 2 days to 21 years; 10 male) were referred to our center due to congenital portosystemic shunts. The following data were collected prior to treatment: age, sex, medical history, clinical and analytical data, urine trimethylaminuria, abdominal-US, and body-CT. The following data were collected at the time of intervention: anatomical and hemodynamic characteristics of the shunts, device used, and closure success. The following data were collected at various post-intervention time points: during hospital stay (to confirm shunt closure and detect complications) and at one year after (for clinical, analytical, and imaging purposes). Results: The treatment was successful in 12 participants, migration of the device was observed in two, while acute splanchnic thrombosis was observed in one. Off-label devices were used in attempting to close the side-to-side shunts, and success was achieved using Amplatzer™ Ductus-Occluder and Amplatzer™ Muscular-Vascular-Septal-Defect-Occluder. The main changes were: increased prothrombin activity (p=0.043); decreased AST, ALT, GGT, and bilirubin (p=0.007, p=0.056, p=0.036, p=0.013); thrombocytopenia resolution (p=0.131); expansion of portal veins (p=0.005); normalization of Doppler portal flow (100%); regression of liver nodules (p=0.001); ammonia normalization (p=0.003); and disappearance of trimethylaminuria (p=0.285). Conclusion: Endovascular closure is effective. Our results support the indication of endovascular closure for side-to-side shunts and for cases of congenital absence of portal vein.
Keywords
Portosystemic shunt; Portal vein; Ammonia; Trimethylaminuria;
Citations & Related Records
연도 인용수 순위
  • Reference
1 Guerin F, Blanc T, Gauthier F, Abella SF, Branchereau S. Congenital portosystemic vascular malformations. Semin Pediatr Surg 2012;21:233-4.   DOI
2 Turkbey B, Karcaaltincaba M, Demir H, Akcoren Z, Yuce A, Haliloglu M. Multiple hyperplastic nodules in the liver with congenital absence of portal vein: MRI findings. Pediatr Radiol 2006;36:445-8.   DOI
3 Pupulim LF, Vullierme MP, Paradis V, Valla D, Terraz S, Vilgrain V. Congenital portosystemic shunts associated with liver tumours. Clin Radiol 2013;68:e362-9.   DOI
4 Lautz TB, Shah SA, Superina RA. Hepatoblastoma in children with congenital portosystemic shunts. J Pediatr Gastroenterol Nutr 2016;62:542-5.   DOI
5 Fu L, Wang Q, Wu J, Guo Y, Huang M, Liu T, et al. Congenital extrahepatic portosystemic shunt: an underdiagnosed but treatable cause of hepatopulmonary syndrome. Eur J Pediatr 2016;175:195-201.   DOI
6 Messenger J, Clark S, Massick S, Bechtel M. A review of trimethylaminuria: (fish odor syndrome). J Clin Aesthet Dermatol 2013;6:45-8.
7 Peker A, Ucar T, Kuloglu Z, Ceyhan K, Tutar E, Fitoz S. Congenital absence of portal vein associated with nodular regenerative hyperplasia of the liver and pulmonary hypertension. Clin Imaging 2009;33:322-5.   DOI
8 Barton JW 3rd, Keller MS. Liver transplantation for hepatoblastoma in a child with congenital absence of the portal vein. Pediatr Radiol 1989;20:113-4.   DOI
9 Kawano S, Hasegawa S, Urushihara N, Okazaki T, Yoshida A, Kusafuka J, et al. Hepatoblastoma with congenital absence of the portal vein - a case report. Eur J Pediatr Surg 2007;17:292-4.   DOI
10 Osorio MJ, Bonow A, Bond GJ, Rivera MR, Vaughan KG, Shah A, et al. Abernethy malformation complicated by hepatopulmonary syndrome and a liver mass successfully treated by liver transplantation. Pediatr Transplant 2011;15:E149-51.   DOI
11 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.   DOI
12 Iida T, Ogura Y, Doi H, Yagi S, Kanazawa H, Imai H, et al. Successful treatment of pulmonary hypertension secondary to congenital extrahepatic portocaval shunts (Abernethy type 2) by living donor liver transplantation after surgical shunt ligation. Transpl Int 2010;23:105-9.   DOI
13 Kanazawa H, Nosaka S, Miyazaki O, Sakamoto S, Fukuda A, Shigeta T, et al. The classification based on intrahepatic portal system for congenital portosystemic shunts. J Pediatr Surg 2015;50:688-95.   DOI
14 Morikawa N, Honna T, Kuroda T, Kitano Y, Fuchimoto Y, Kawashima N, et al. Resolution of hepatopulmonary syndrome after ligation of a portosystemic shunt in a pediatric patient with an Abernethy malformation. J Pediatr Surg 2008;43:e35-8.
15 Hao Y, Hong X, Zhao X. Congenital absence of the portal vein associated with focal nodular hyperplasia of the liver and congenital heart disease (Abernethy malformation): a case report and literature review. Oncol Lett 2015;9:695-700.   DOI
16 Correa C, Luengas JP, Howard SC, Veintemilla G. Hepatoblastoma and Abernethy malformation type I: case report. J Pediatr Hematol Oncol 2017;39:e79-81.   DOI
17 Fernandez MS, Gutierrez C, Vila JJ, Lopez A, Ibanez V, Sanguesa C, et al. Congenital intrahepatic portocaval shunt associated with trimethylaminuria. Pediatr Surg Int 1997;12:196-7.   DOI
18 Satoh M, Yokoya S, Hachiya Y, Hachiya M, Fujisawa T, Hoshino K, et al. Two hyperandrogenic adolescent girls with congenital portosystemic shunt. Eur J Pediatr 2001;160:307-11.   DOI
19 Ersch J, Banziger O, Braegger C, Arbenz U, Stallmach T. An infant with pulmonary hypertension due to a congenital porto-caval shunt. Eur J Pediatr 2002;161:660-2.   DOI
20 Eroglu Y, Donaldson J, Sorensen LG, Vogelzang RL, Melin-Aldana H, Andersen J, et al. Improved neurocognitive function after radiologic closure of congenital portosystemic shunts. J Pediatr Gastroenterol Nutr 2004;39:410-7.   DOI
21 Ono H, Mawatari H, Mizoguchi N, Eguchi T, Sakura N. Clinical features and outcome of eight infants with intrahepatic porto-venous shunts detected in neonatal screening for galactosaemia. Acta Paediatr 1998;87:631-4.   DOI
22 Matsuura T, Soejima Y, Taguchi T. Auxiliary partial orthotopic living donor liver transplantation with a small-for-size graft for congenital absence of the portal vein. Liver Transpl 2010;16:1437-9.   DOI
23 Bruckheimer E, Dagan T, Atar E, Schwartz M, Kachko L, Superina R, et al. Staged transcatheter treatment of portal hypoplasia and congenital portosystemic shunts in children. Cardiovasc Intervent Radiol 2013;36:1580-5.   DOI
24 Chandler TM, Heran MK, Chang SD, Parvez A, Harris AC. Multiple focal nodular hyperplasia lesions of the liver associated with congenital absence of the portal vein. Magn Reson Imaging 2011;29:881-6.   DOI
25 Rosenberg HK, Markowitz RI, Kolberg H, Park C, Hubbard A, Bellah RD. Normal splenic size in infants and children: sonographic measurements. AJR Am J Roentgenol 1991;157:119-21.   DOI
26 Yamagami T, Yoshimatsu R, Matsumoto T, Terayama K, Nishiumra A, Maeda Y, et al. Successful embolization using interlocking detachable coils for a congenital extrahepatic portosystemic venous shunt in a child. J Pediatr Surg 2007;42:1949-52.   DOI
27 Franchi-Abella S, Branchereau S, Lambert V, Fabre M, Steimberg C, Losay J, et al. Complications of congenital portosystemic shunts in children: therapeutic options and outcomes. J Pediatr Gastroenterol Nutr 2010;51:322-30.   DOI
28 Kasahara M, Nakagawa A, Sakamoto S, Tanaka H, Shigeta T, Fukuda A, et al. Living donor liver transplantation for congenital absence of the portal vein with situs inversus. Liver Transpl 2009;15:1641-3.   DOI
29 Uchida H, Sakamoto S, Shigeta T, Hamano I, Kanazawa H, Fukuda A, et al. Living donor liver transplantation with renoportal anastomosis for a patient with congenital absence of the portal vein. Case Rep Surg 2012;2012:670289.   DOI
30 Matsumoto N, Matsusaki T, Hiroi K, Kaku R, Yoshida R, Umeda Y, et al. Pediatric living donor liver transplantation for congenital absence of the portal vein with pulmonary hypertension: a case report. Transplant Proc 2020;52:630-3.   DOI
31 Bas S, Guran T, Atay Z, Haliloglu B, Abali S, Turan S, et al. Premature pubarche, hyperinsulinemia and hypothyroxinemia: novel manifestations of congenital portosystemic shunts (Abernethy malformation) in children. Horm Res Paediatr 2015;83:282-7.   DOI
32 Brasoveanu V, Ionescu MI, Grigorie R, Mihaila M, Bacalbasa N, Dumitru R, et al. Living donor liver transplantation for unresectable liver adenomatosis associated with congenital absence of portal vein: a case report and literature review. Am J Case Rep 2015;16:637-44.   DOI
33 Gulsen Z, Yigit H, Demir P. Multiple regenerative nodular hyperplasia in the left infrarenal vena cava accompanied by Abernethy malformation. Surg Radiol Anat 2016;38:373-8.   DOI
34 Grimaldi C, Monti L, Falappa P, d'Ambrosio G, Manca A, de Ville de Goyet J. Congenital intrahepatic portohepatic shunt managed by interventional radiologic occlusion: a case report and literature review. J Pediatr Surg 2012;47:e27-31.