Surgical Ligation for the Treatment of an Unusual Presentation of Type II Abernethy Malformation

Zhou, Mantian; Ju-qiang, Zhang; Li, Luo; Wang, Botian; Zheng, Renjun; Li, Linpeng; Jing, Hailing; Zhang, Shengzu

doi:10.1016/j.avsg.2019.10.094

Cited by 5 publications

(4 citation statements)

References 15 publications

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“…Hepatic damage in patients with Abernethy malformations is milder than the damage in patients with cirrhosis, and the increases in bilirubin and aminopherase are often not obvious[ 14 ]. This patient’s hepatic function was normal, with no hepatic encephalopathy, which is in line with previous research results[ 5 , 15 ]. Most patients with Abernethy malformations have elevated blood ammonia, but fewer patients present with hepatic encephalopathy.…”

Section: Discussionsupporting

confidence: 92%

“…The choice of an interventional or surgical method is based on the degree of extrahepatic portosystemic shunts, the anatomical position of shunts, and the diameter of the shunts. The liver tumors, hepatopulmonary syndrome, and hyperammonemia disappear after the occlusion of the abnormal shunts[ 4 , 15 ]. Portal hypertension caused by Abernethy malformation has not been reported; thus, we first recommended liver transplantation for this patient, and the patient finally chose conservative treatment.…”

Section: Discussionmentioning

confidence: 99%

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Rare portal hypertension caused by Abernethy malformation (Type IIC): A case report

Yao,

Liu,

et al. 2023

World J Radiol

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BACKGROUND Abernethy malformation is a rare congenital vascular malformation with a portosystemic shunt that may clinically manifest as cholestasis, dyspnea, or hepatic encephalopathy, among other conditions. Early diagnosis and classification are very important to further guide treatment. Typically, patients with congenital portosystemic shunts have no characteristics of portal hypertension. Herein, we report an 18-year-old female with prominent portal hypertension that manifested mainly as rupture and bleeding of esophageal varices. Imaging showed a thin main portal vein, no portal vein branches in the liver, and bleeding of the esophageal and gastric varices caused by the collateral circulation upwards from the proximal main portal vein. Patients with Abernethy malformation type I are usually treated with liver transplantation, and patients with type II are treated with shunt occlusion, surgery, or transcatheter coiling. Our patient was treated with endoscopic surgery combined with drug therapy and had no portal hypertension and good hepatic function for 24 mo of follow-up. CASE SUMMARY This case report describes our experience in the diagnosis and treatment of an 18-year-old female with Abernethy malformation type IIC and portal hypertension. This condition was initially diagnosed as cirrhosis combined with portal hypertension. The patient was ultimately diagnosed using liver histology and subsequent imaging, and the treatment was highly effective. To publish this case report, written informed consent was obtained from the patient, including the attached imaging data. CONCLUSION Abernethy malformation type IIC may develop portal hypertension, and traditional nonselective beta-blockers combined with endoscopic treatment can achieve high efficacy.

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Section: Discussionsupporting

confidence: 92%

Section: Discussionmentioning

confidence: 99%

Rare portal hypertension caused by Abernethy malformation (Type IIC): A case report

Yao,

Liu,

et al. 2023

World J Radiol

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“…Closure of the shunt can be performed either surgically or percutaneously [13]. Zhou et al used surgical ligation for patients with Type II AM having giant shunt [14]. Our center has the facilities of interventional radiology.…”

Section: Discussionmentioning

confidence: 99%

Type II Abernethy malformation in an adult male patient—a rare and reversible cause of pulmonary hypertension: a case report with review of literature

et al. 2021

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Background Co-occurrence of Type II Abernethy malformation (AM) with Type 1 pulmonary hypertension (PH) is extremely rare. In these patients, management of AM leads to reversal of PH. Case presentation We report a case of 24-year-old male that presented with fever and dyspnea of insidious-onset. Initial X-ray chest revealed pulmonary edema, prominent pulmonary knuckle, and dilated right descending pulmonary artery. Two-dimensional echocardiography suggested raised pulmonary artery systolic pressure (145 mmHg) and pulmonary angiogram revealed findings suggestive of Type 1 PH. Treatment with Tab. Tadalafil-Bosentan (20/5 mg, thrice daily) was initiated, but no major relief was obtained. On day 10, dyspnea worsened. Routine ultrasound revealed splenomegaly. Further evaluation with Doppler of the portal vein was suggestive of portal hypertension, and arterial ammonia was found to be raised. Contrast-enhanced computed tomography of abdomen and pelvis demonstrated a single dilated (1.3 cm) and torturous venous shunt between right internal iliac vein and superior mesenteric vein. Thus, a definitive diagnosis of Type II AM was reached. The patient was stabilized and subjected to the shunt closure with ASD Cocoon stent graft and recovered well. Conclusion Our case demonstrates a rare but reversible cause of PH. Type II AM should be included in the differential diagnosis of a patient presenting with dyspnea and provisionally diagnosed as a case of PH.

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“…Long-term complications include benign hyperplasia, hemangioma, and primary liver cancer. The manifestations include a variety of cardiovascular system abnormalities [11][12][13], hepatic focal nodular hyperplasia [14], hyperbilirubinemia [15], hyperammonemia [16], mental retardation [17], and hepatic encephalopathy. For all CAPV cases, conservative treatment should be considered first, and invasive treatment, mainly portal reconstruction, can be performed when conservative treatment fail.…”

Section: Discussionmentioning

confidence: 99%

Living donor liver transplantation for congenital absence of portal vein in portal venous reconstruction with a great saphenous vein graft

Hidaka

Hamada

et al. 2020

surg case rep

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Background Congenital absence of portal vein (CAPV) is a rare structural anomaly in which the portal vein (PV) blood that normally flow into the liver directly drains into the systemic venous system through other collateral circulation. Congenital portal vein shunts (CPSs) is classified into types I and II according to the absence or presence of the intrahepatic portal vein, respectively. The CPS type I is also known as CAPV. The liver transplantation may be the only treatment option for CAPV. The key point of liver transplantation for CAPV is the reconstruction of the PV. Case presentation A 29-year-old man was diagnosed with CAPV with splenomegaly and gastroesophageal varix when being treated for pancytopenia and liver dysfunction. A living donor liver transplantation was performed for him using the right lobe which had been donated by his mother. The PV was reconstructed using his own great saphenous vein (GSV) as a graft vein. The end of the GSV graft was anastomosed to the inferior mesenteric vein while the other end was anastomosed to the vein graft of the right hepatic vein from the explanted liver. Conclusion Using the patient’s own GSV for PV reconstruction during living donor transplantation in the patient with CAPV seems to be an effective method.

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Surgical Ligation for the Treatment of an Unusual Presentation of Type II Abernethy Malformation

Cited by 5 publications

References 15 publications

Rare portal hypertension caused by Abernethy malformation (Type IIC): A case report

Rare portal hypertension caused by Abernethy malformation (Type IIC): A case report

Type II Abernethy malformation in an adult male patient—a rare and reversible cause of pulmonary hypertension: a case report with review of literature

Living donor liver transplantation for congenital absence of portal vein in portal venous reconstruction with a great saphenous vein graft

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