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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

CT arterial phase. Several other smaller hypervascular masses are seen in the right hemiliver (arrowheads).
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f2-amjcaserep-16-637: CT arterial phase. Several other smaller hypervascular masses are seen in the right hemiliver (arrowheads).

Mentions: The 21-year-old patient was diagnosed with a large unresectable tumor in the right hemiliver (110/100 mm, S5–8) and a type Ib Abernethy malformation during a bioptic laparotomy in 2011. Immunohistochemical study showed liver adenoma. The patient had no signs of portal hypertension and throughout the pretransplant period showed no encephalopathy or hyperammonemia. No hepatopulmonary syndrome or congenital cardiac disease was diagnosed in the patient; echocardiogram performed with agitated saline as contrast confirmed intra-pulmonary shunting. Moreover, pretransplant CT showed no additional abdominal congenital anomalies. She was placed on the DDLT waiting list, maintaining satisfactory liver function throughout the waiting period, while the right hemiliver mass remained stable in dimensions. A second large liver mass (68/66 mm, S2–3) was diagnosed by computed tomography in the left hemiliver, as well as 9 smaller ones in both hemilivers (S4: 20/21 mm; S5–S6: 32/20 mm, 14/14 mm, 16/6 mm, 7/6 mm, 9/8 mm, 8/8 mm, 7/7 mm, 19/16 mm) (Figures 1–4). Therefore, a decision was made to transplant the patient with a graft from a living related donor.


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)

CT arterial phase. Several other smaller hypervascular masses are seen in the right hemiliver (arrowheads).
© Copyright Policy
Related In: Results  -  Collection

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

f2-amjcaserep-16-637: CT arterial phase. Several other smaller hypervascular masses are seen in the right hemiliver (arrowheads).
Mentions: The 21-year-old patient was diagnosed with a large unresectable tumor in the right hemiliver (110/100 mm, S5–8) and a type Ib Abernethy malformation during a bioptic laparotomy in 2011. Immunohistochemical study showed liver adenoma. The patient had no signs of portal hypertension and throughout the pretransplant period showed no encephalopathy or hyperammonemia. No hepatopulmonary syndrome or congenital cardiac disease was diagnosed in the patient; echocardiogram performed with agitated saline as contrast confirmed intra-pulmonary shunting. Moreover, pretransplant CT showed no additional abdominal congenital anomalies. She was placed on the DDLT waiting list, maintaining satisfactory liver function throughout the waiting period, while the right hemiliver mass remained stable in dimensions. A second large liver mass (68/66 mm, S2–3) was diagnosed by computed tomography in the left hemiliver, as well as 9 smaller ones in both hemilivers (S4: 20/21 mm; S5–S6: 32/20 mm, 14/14 mm, 16/6 mm, 7/6 mm, 9/8 mm, 8/8 mm, 7/7 mm, 19/16 mm) (Figures 1–4). Therefore, a decision was made to transplant the patient with a graft from a living related donor.

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