Comprehensive review of 18 cases with Abernethy malformation in China from 2001 to 2012
10.3969/cma.j.issn.2095-428X.2013.19.012
- VernacularTitle:2001年至2012年中国18例Abernethy畸形综合报道
- Author:
Ya-Mei WANG
1
;
Lin ZHONG
;
Yu-Hong TAO
Author Information
1. 四川大学华西第二医院儿科
- Keywords:
Abernethy malformation;
Absence of portal vein;
Portocaval shunt
- From:
Chinese Journal of Applied Clinical Pediatrics
2013;28(19):1478-1482
- CountryChina
- Language:Chinese
-
Abstract:
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.
