Ann Liver Transplant.  2023 May;3(1):35-43. 10.52604/alt.23.0004.

Living donor liver transplantation with graft dextro-rotation and vein interposition in a pediatric patient with congenital absence of the portal vein

• Namgoong JM
• Hwang S
• Park GC
• Kim SH
• Kim KM
• Oh SH

• KMID: 2560920
• DOI: http://doi.org/10.52604/alt.23.0004

Abstract

Congenital absence of the portal vein (CAPV) is a rare venous malformation in which the mesenteric venous blood drains directly into the systemic circulation. We report a case of pediatric living donor liver transplantation (LDLT) for CAPV with a large portocaval shunt (PCS). A 10-year-old boy was diagnosed with CAPV at the age of 9 years. Multiple liver nodules and hepatopulmonary syndrome were progressed, therefore, we decided to perform LDLT. The graft was a left liver graft from the 45-year-old father of the patient. The recipient hepatectomy was performed according to the standard procedures of pediatric LDLT with isolation of a PCS vein. Unification venoplasty for graft outflow vein orifice was performed because of segment VIII vein at the donor liver. Iliac vein interposition was performed for portal vein extension. Graft dextro-rotation and portal vein interposition were used for anatomy-compliant vascular reconstruction. The patient recovered from the LDLT operation. The reconstructed graft hepatic vein and portal vein were maintained well without hemodynamic abnormality. This patient has been doing well for 2 months after the LDLT. In conclusion, since CAPV patients show various types of PCS, individualized portal vein reconstruction should be performed after thorough anatomical assessment before and during the liver transplantation operation.

Keyword

Portal vein agenesis; Portocaval shunt; Interposition graft; Iliac vein homograft; Dextro-rotation

Figure

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  Figure 1 Preoperative and postoperative computed tomography findings of the donor. (A) A fissural vein was identified at the medial section (arrow). (B) A large segment VIII vein was identified (arrow). (C) Parenchymal transection plane was designed to preserved the segment VIII vein (dotted line). (D) Postoperative computed tomography taken at 7 days after surgery showed no significant hepatic venous congestion at the remnant right liver.

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  Figure 2 Pretransplant computed tomography findings of the recipient. (A) Multiple liver nodules were identified. (B) Hepatic arteries were enlarged without anatomical variation. (C) Congenital absence of the portal vein was identified with development of a large portocaval shunt in to the suprahepatic inferior vena cava (arrow).

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  Figure 3 Intraoperative photographs showing perihilar anatomy and portocaval shunt. (A) Portal vein was absent at the hepatic hilum. (B–D) A large portocaval shunt vein was isolated along the caudate process area (arrows).

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  Figure 4 Intraoperative photographs of bench work for a left liver graft. (A) A small-sized left hepatic vein orifice was identified (arrow). (B) Transected middle hepatic vein trunk was exposed at the liver cut surface (arrow). (C–D) An iliac vein segment was anastomosed with the middle hepatic vein trunk. (E) The inferior vena cava side of the conduit was anastomosed with the left hepatic vein stump. (F–H) An incision was made at the left lateral wall of the graft left hepatic vein, and patch venoplasty was performed to make a wide single outflow vein orifice.

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  Figure 5 Intraoperative photographs of bench work for portal vein interposition. (A, B) An iliac vein segment was anastomosed with the graft portal vein. (C, D) A sufficient growth factor was provided during reconstruction.

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  Figure 6 Intraoperative photographs of the hepatic vein and portal vein reconstruction. (A) The graft hepatic vein was enlarged through unification venoplasty. (B) The isolated portocaval shunt vein was transected. (C–E) The liver graft was placed into the right subphrenic fossa, thus inducing dextrorotation and hepatic vein reconstruction was performed at that position. (F–H) The interposed iliac vein conduit at the graft portal vein was obliquely resected to match the length and axis of the excised portocaval shunt vein. A 1-cm-long wedge-shaped iliac vein segment (arrows) was inserted between the graft portal vein and the portocaval shunt vein stumps to prevent redundancy.

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  Figure 7 Gross photograph of the explant liver showing multiple liver nodules.

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  Figure 8 Posttransplant computed tomography scan taken at 4 days after transplantation. (A, B) Uneventful reconstruction of the graft hepatic vein was identified. (C, D) Portal vein anastomosis showed a slight anastomotic stenosis (arrows), with unusual location of the inflow portocaval shunt vein.

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  Figure 9 Posttransplant computed tomography scan taken at 14 days after transplantation. (A) Three-dimensional reconstruction of the portal vein shows stream-lined configuration (arrow). (B–F) The running course of the reconstructed portal vein shows the unusual location behind the liver graft (arrows).

Reference

1. Sanada Y, Mizuta K, Kawano Y, Egami S, Hayashida M, Wakiya T, et al. 2009; Living donor liver transplantation for congenital absence of the portal vein. Transplant Proc. 41:4214–4219. DOI: 10.1016/j.transproceed.2009.08.080. PMID: 20005372.

2. Shinkai M, Ohhama Y, Nishi T, Yamamoto H, Fujita S, Take H, et al. 2001; Congenital absence of the portal vein and role of liver transplantation in children. J Pediatr Surg. 36:1026–1031. DOI: 10.1053/jpsu.2001.24731. PMID: 11431769.

3. Wojcicki M, Haagsma EB, Gouw AS, Slooff MJ, Porte RJ. 2004; Orthotopic liver transplantation for portosystemic encephalopathy in an adult with congenital absence of the portal vein. Liver Transpl. 10:1203–1207. Erratum. DOI: 10.1002/lt.20170. PMID: 15350015.

4. Ikeda S, Sera Y, Ohshiro H, Uchino S, Uchino T, Endo F. 1999; Surgical indications for patients with hyperammonemia. J Pediatr Surg. 34:1012–1015. DOI: 10.1016/S0022-3468(99)90780-7. PMID: 10392925.

5. Sumida W, Kaneko K, Ogura Y, Tainaka T, Ono Y, Seo T, et al. 2006; Living donor liver transplantation for congenital absence of the portal vein in a child with cardiac failure. J Pediatr Surg. 41:e9–e12. DOI: 10.1016/j.jpedsurg.2006.07.014. PMID: 17101345.

6. Watanabe A. 2000; Portal-systemic encephalopathy in non-cirrhotic patients: classification of clinical types, diagnosis and treatment. J Gastroenterol Hepatol. 15:969–979. DOI: 10.1046/j.1440-1746.2000.02283.x. PMID: 11059925.

7. Morgan G, Superina R. 1994; Congenital absence of the portal vein: two cases and a proposed classification system for portasystemic vascular anomalies. J Pediatr Surg. 29:1239–1241. DOI: 10.1016/0022-3468(94)90812-5. PMID: 7807356.

8. Emre S, Arnon R, Cohen E, Morotti RA, Vaysman D, Shneider BL. 2007; Resolution of hepatopulmonary syndrome after auxiliary partial orthotopic liver transplantation in Abernethy malformation. A case report. Liver Transpl. 13:1662–1668. DOI: 10.1002/lt.21349. PMID: 18044784.

9. Soejima Y, Taguchi T, Ogita K, Taketomi A, Yoshizumi T, Uchiyama H, et al. 2006; Auxiliary partial orthotopic living donor liver transplantation for a child with congenital absence of the portal vein. Liver Transpl. 12:845–849. DOI: 10.1002/lt.20692. PMID: 16628685.

10. Tanaka Y, Takayanagi M, Shiratori Y, Imai Y, Obi S, Tateishi R, et al. 2003; Congenital absence of portal vein with multiple hyperplastic nodular lesions in the liver. J Gastroenterol. 38:288–294. DOI: 10.1007/s005350300050. PMID: 12673454.

11. Namgoong JM, Hwang S, Kim DY, Ha TY, Song GW, Jung DH, et al. 2021; Pediatric liver transplantation using a hepatitis B surface antigen-positive donor liver graft for congenital absence of the portal vein. Korean J Transplant. 35:59–65. DOI: 10.4285/kjt.20.0038. PMID: 35769617. PMCID: PMC9235344.

12. Namgoong JM, Hwang S, Park GC, Ha S, Kim KM, Oh SH. 2022; Living donor liver transplantation with proximal splenic vein ligation in a pediatric patient with congenital absence of the portal vein. Ann Liver Transplant. 2:69–77. DOI: 10.52604/alt.22.0015.

13. Namgoong JM, Hwang S, Park GC, Kwon H, Kim KM, Oh SH. 2021; Living donor liver transplantation in a pediatric patient with congenital absence of the portal vein. Ann Hepatobiliary Pancreat Surg. 25:401–407. DOI: 10.14701/ahbps.2021.25.3.401. PMID: 34402443. PMCID: PMC8382859.

14. Woodle ES, Thistlethwaite JR, Emond JC, Whitington PF, Vogelbach P, Yousefzadeh DK, et al. 1990; Successful hepatic transplantation in congenital absence of recipient portal vein. Surgery. 107:475–479.

15. Ohnishi Y, Ueda M, Doi H, Kasahara M, Haga H, Kamei H, et al. 2005; Successful liver transplantation for congenital absence of the portal vein complicated by intrapulmonary shunt and brain abscess. J Pediatr Surg. 40:e1–e3. DOI: 10.1016/j.jpedsurg.2005.02.011. PMID: 15937799.

16. Charre L, Roggen F, Lemaire J, Mathijs J, Goffette P, Danse E, et al. 2004; Hematochezia and congenital extrahepatic portocaval shunt with absent portal vein: successful treatment by liver transplantation. Transplantation. 78:1404–1406. DOI: 10.1097/01.TP.0000137931.51504.F7. PMID: 15548983.

17. Namgoong JM, Hwang S, Ko GY, Kwon H, Ha S, Oh SH, et al. 2022; Usability of intraoperative cine-portogram during liver transplantation in young pediatric patients with biliary atresia. Pediatr Transplant. 26:e14207. DOI: 10.1111/petr.14207. PMID: 34888999.

18. Namgoong JM, Hwang S, Park GC, Kim KM, Oh SH, Kwon H, et al. 2021; Dextroplantation of a reduced left lateral section graft in an infant undergoing living donor liver transplantation. Ann Hepatobiliary Pancreat Surg. 25:414–418. DOI: 10.14701/ahbps.2021.25.3.414. PMID: 34402445. PMCID: PMC8382867.