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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

Intraoperative aspect: VP – portosystemic shunt (common trunk of SMV and splenic vein); VC – inferior vena cava.
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f5-amjcaserep-16-637: Intraoperative aspect: VP – portosystemic shunt (common trunk of SMV and splenic vein); VC – inferior vena cava.

Mentions: Intraoperative examination confirmed the absence of a portal vein in the hepatoduodenal ligament, as well as the presence of a type Ib portosystemic shunt (Figure 5). There were pre-operative concerns that the portal conduit would be too short and that, in turn, would necessitate interposition of a vascular graft or a synthetic one. However, careful dissection of the portal conduit and its detachment from the IVC with a small (2 mm) circular cuff allowed the surgical team to perform the anastomosis between the splenomesenteric trunk and the donor left portal vein in an end-to-end manner.


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)

Intraoperative aspect: VP – portosystemic shunt (common trunk of SMV and splenic vein); VC – inferior vena cava.
© Copyright Policy
Related In: Results  -  Collection

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

f5-amjcaserep-16-637: Intraoperative aspect: VP – portosystemic shunt (common trunk of SMV and splenic vein); VC – inferior vena cava.
Mentions: Intraoperative examination confirmed the absence of a portal vein in the hepatoduodenal ligament, as well as the presence of a type Ib portosystemic shunt (Figure 5). There were pre-operative concerns that the portal conduit would be too short and that, in turn, would necessitate interposition of a vascular graft or a synthetic one. However, careful dissection of the portal conduit and its detachment from the IVC with a small (2 mm) circular cuff allowed the surgical team to perform the anastomosis between the splenomesenteric trunk and the donor left portal vein in an end-to-end manner.

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