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Living Donor Liver Transplantation for Unresectable Liver Adenomatosis Associated with Congenital Absence of Portal Vein: A Case Report and Literature Review.

Brasoveanu V, Ionescu MI, Grigorie R, Mihaila M, Bacalbasa N, Dumitru R, Herlea V, Iorgescu A, Tomescu D, Popescu I - Am J Case Rep (2015)

Bottom Line: Abernethy malformation (AM), or congenital absence of portal vein (CAPV), is a very rare disease which tends to be associated with the development of benign or malignant tumors, usually in children or young adults.The patient presented mild liver dysfunction and was largely asymptomatic.Postoperatively, the patient attained optimal liver function and at 9-month follow-up has returned to normal life.

View Article: PubMed Central - PubMed

Affiliation: "Dan Setlacec" Center of General Surgery and Liver Transplantation, Fundeni Clinical Institute, Bucharest, Romania.

ABSTRACT

Background: Abernethy malformation (AM), or congenital absence of portal vein (CAPV), is a very rare disease which tends to be associated with the development of benign or malignant tumors, usually in children or young adults.

Case report: We report the case of a 21-year-old woman diagnosed with type Ib AM (portal vein draining directly into the inferior vena cava) and unresectable liver adenomatosis. The patient presented mild liver dysfunction and was largely asymptomatic. Living donor liver transplantation was performed using a left hemiliver graft from her mother. Postoperatively, the patient attained optimal liver function and at 9-month follow-up has returned to normal life.

Conclusions: We consider that living donor liver transplantation is the best therapeutic solution for AM associated with unresectable liver adenomatosis, especially because compared to receiving a whole liver graft, the waiting time on the liver transplantation list is much shorter.

No MeSH data available.


Related in: MedlinePlus

Hematoxylin-eosin stain ×100 – portal tract of the explanted liver (arrow). The portal tract lacks portal venules; arteries present a hypertrophic wall (A); biliary ducts have normal aspect (B).
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f6-amjcaserep-16-637: Hematoxylin-eosin stain ×100 – portal tract of the explanted liver (arrow). The portal tract lacks portal venules; arteries present a hypertrophic wall (A); biliary ducts have normal aspect (B).

Mentions: The total time during which the portal vein was clamped was only 15 min, with no intestinal edema. The other anastomoses were as follows: common trunk of donor middle hepatic vein and left hepatic vein to the recipient common trunk of the middle and left hepatic veins; donor left hepatic artery (replaced from the left gastric artery) to recipient hepatic artery proper; donor left biliary duct to recipient main biliary duct. Even though the preoperative CT scan showed hypertrophy of the recipient hepatic artery, there was no mismatch between the anastomotic partners. The histopathological examination of the explanted liver showed an aspect of liver adenomatosis (Figure 6).


Living Donor Liver Transplantation for Unresectable Liver Adenomatosis Associated with Congenital Absence of Portal Vein: A Case Report and Literature Review.

Brasoveanu V, Ionescu MI, Grigorie R, Mihaila M, Bacalbasa N, Dumitru R, Herlea V, Iorgescu A, Tomescu D, Popescu I - Am J Case Rep (2015)

Hematoxylin-eosin stain ×100 – portal tract of the explanted liver (arrow). The portal tract lacks portal venules; arteries present a hypertrophic wall (A); biliary ducts have normal aspect (B).
© Copyright Policy
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC4581685&req=5

f6-amjcaserep-16-637: Hematoxylin-eosin stain ×100 – portal tract of the explanted liver (arrow). The portal tract lacks portal venules; arteries present a hypertrophic wall (A); biliary ducts have normal aspect (B).
Mentions: The total time during which the portal vein was clamped was only 15 min, with no intestinal edema. The other anastomoses were as follows: common trunk of donor middle hepatic vein and left hepatic vein to the recipient common trunk of the middle and left hepatic veins; donor left hepatic artery (replaced from the left gastric artery) to recipient hepatic artery proper; donor left biliary duct to recipient main biliary duct. Even though the preoperative CT scan showed hypertrophy of the recipient hepatic artery, there was no mismatch between the anastomotic partners. The histopathological examination of the explanted liver showed an aspect of liver adenomatosis (Figure 6).

Bottom Line: Abernethy malformation (AM), or congenital absence of portal vein (CAPV), is a very rare disease which tends to be associated with the development of benign or malignant tumors, usually in children or young adults.The patient presented mild liver dysfunction and was largely asymptomatic.Postoperatively, the patient attained optimal liver function and at 9-month follow-up has returned to normal life.

View Article: PubMed Central - PubMed

Affiliation: "Dan Setlacec" Center of General Surgery and Liver Transplantation, Fundeni Clinical Institute, Bucharest, Romania.

ABSTRACT

Background: Abernethy malformation (AM), or congenital absence of portal vein (CAPV), is a very rare disease which tends to be associated with the development of benign or malignant tumors, usually in children or young adults.

Case report: We report the case of a 21-year-old woman diagnosed with type Ib AM (portal vein draining directly into the inferior vena cava) and unresectable liver adenomatosis. The patient presented mild liver dysfunction and was largely asymptomatic. Living donor liver transplantation was performed using a left hemiliver graft from her mother. Postoperatively, the patient attained optimal liver function and at 9-month follow-up has returned to normal life.

Conclusions: We consider that living donor liver transplantation is the best therapeutic solution for AM associated with unresectable liver adenomatosis, especially because compared to receiving a whole liver graft, the waiting time on the liver transplantation list is much shorter.

No MeSH data available.


Related in: MedlinePlus