Objective To acquire more
knowledge about Abernethy malformation.
Methods Eighteen cases with Abernethy malformation published previously in
China from 2001 to 2012 were reviewed.The clinical,radiological and pathological manifestations and
treatment were summarized.Results Of 13 cases with type Ⅰ Abernethy malformation (including 6 type Ⅰ a and 7 type Ⅰ b,5
male and 8
female),11 cases occurred by the age of 18 years.Their clinical manifestations included
hematochezia in 4,
hematemesis in 4,
liver dysfunction in 5,
liver cirrhosis in 2,
hepatic encephalopathy in 2,hepatic nodule in 4,
splenomegaly in 5,
hypersplenism in 4,
portal hypertension in 3 and other associated malformations in 3.Of 5 cases with type Ⅱ Abernethy malformation,2 cases occurred by the age of 18 years.Their clinical manifestations included
liver dysfunction in 4,
hematochezia in 1,
liver cirrhosis in 1,
hepatic encephalopathy in 1,hepatic nodule with
focal nodular hyperplasia in 1.All 18
patients underwent imaging evaluation,multi-slice computed
tomography(CT)
angiography (MSCTA) in 16,including vascular ultrasound in 15,
digital subtraction angiography (DSA) in 7,
magnetic resonance angiography (MRA) in 1 and
magnetic resonance imaging (MRI) in 1.The sites of
drainage for
portal vein system in 15 cases were documented,including the
inferior vena cava (2/15 cases),internal
iliac vein (5/15 cases),left
renal vein (3/15 cases),
azygos vein (2/15 cases),
right atrium (2/15 cases) and pelvic venous plexus (1/15 case).And there was no
record of specific draining site in other 3 cases.Ballooning degeneration of
liver cells,
liver cells nodular
hyperplasia and fatty degeneration were detected in 2 cases of type Ⅰ Abernethy malformation.And
liver focal nodular hyperplasia was demonstrated in 1 case of type Ⅱ Abernethy malformation.Four cases with
hematochezia were misdiagnosed as
ulcerative colitis,
hemorrhoids or
purpura.Two cases with clitoral
hypertrophy were misdiagnosed as
genital malformations.Due to
crying,one 5-month-old baby was misdiagnosed as
sepsis.Of these 18 cases of Abernethy malformation,most of the cases received
conservative treatment.Surgical
techniques such as
splenectomy,surgical
ligation of the
veins of
sigmoid and interventional embolization to occlude the shunt were used to treat
splenomegaly,
hematochezia,
hematemesis or
hepatic encephalopathy.Conclusions Abernethy malformation mainly occurs in
children.Clinical presentation is nonspecific.
Liver dysfunction,hepatic nodule,
hematochezia,
hematemesis,plenomegaly and
hypersplenism are common manifestations.Compared with overseas
reports,hepatic nodule,
hepatic encephalopathy and concomitant
abnormalities are rare,while
hematochezia and
hematemesis are more common.The
diagnosis is made primarily according to imaging changes,and MSCTA is a useful tool to make a
diagnosis.Individualized
treatment of Abernethy malformation is determined by the type of
deformity and the conditions of the
patient.