Live donor liver transplantation for a child presented with severe hepatopulmonary syndrome and nodular liver lesions due to Abernethy malformation

Yilmaz, Cahit; Önen, Zafer; Farajov, Rasim; Duygu, Onur S.; Doğanay, Latife; Yüksel, Alper; Alper, Mehmet; Zeytunlu, Murat; Arıkan, Çiğdem; Kilic, Murat

doi:10.1111/petr.12874

Cited by 6 publications

(3 citation statements)

References 16 publications

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“…Indication for liver transplant includes hepatic encephalophaty not responding to medical management, 12 , 13 liver tumor like hepatoblastoma, 5 focal nodular hyperplasia and for associated mal-formations like biliary atresia. 14 None of the patients with EHPCS type 1 in our series developed any of these compilations and are on regular medical follow up.…”

Section: Methodsmentioning

confidence: 76%

Congenital extrahepatic portocaval malformation: Rare but potentially treatable cause of pulmonary hypertension

Bobhate

Garg

Sharma

et al. 2021

Indian Heart Journal

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Objective Abernethy malformation (congenital extrahepatic portosystemic shunt) is a rare anomaly of the splanchnic venous system. Though rare, it is an important cause of pulmonary artery hypertension (PAH) which is often missed. All patients with PAH should be carefully evaluated for presence of Abernethy malformation before labelling them as Idiopathic PAH. Methods This is a retrospective analysis of prospectively collected data. We reviewed the data of all patients referred to our center for evaluation of PAH. 10 patients were diagnosed to have an extrahepatic portocaval malformation. We reviewed their presentation, diagnosis, catheterization data, intervention and their outcome along with review of literature. Results 10/104 patients with pulmonary hypertension and no intra or extracardiac shunt were found to have extrahepatic portocaval shunt (EHPCS). 3 patients had EHPCS type 1 and 7 had type 2 EHPCS. 6/7 patient with EHPCS type 2 underwent closure of the shunt. There was no procedure related complication. There was one death 3 months post procedure and one patient who was advised surgical closure was lost to follow up. Closure of the shunt resulted in normalization of the pulmonary artery pressures in 4/5 patients. Conclusion Congenital portosystemic malformations form an important and potentially treatable cause of pulmonary hypertension.

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Section: Methodsmentioning

confidence: 76%

Congenital extrahepatic portocaval malformation: Rare but potentially treatable cause of pulmonary hypertension

Bobhate

Garg

Sharma

et al. 2021

Indian Heart Journal

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“…In general, patients with hepatopulmonary syndrome showed improvement of cyanosis when the hepatic function was restored or the portosystemic shunt was abolished (7). Adult patients with small shunts are amenable to interventional procedures such as device closure ( 8), while small children are usually selected for surgical ligation of the portosystemic shunt or orthotopic liver transplantation (7,9). Only occasional cases of transcatheter closure of the shunting vessel at a pediatric age were reported (10).…”

Section: Discussionmentioning

confidence: 99%

Successful percutaneous treatment with the Konar MFTM-VSD Occluder in an infant with Abernethy syndrome—case report

Loureiro

Georgiev

Ewert

et al. 2021

Cardiovasc Diagn Ther

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Cyanosis persisting after surgical repair of complex congenital heart disease (CHD) may be related to the underlying disease. However, extracardiac causes should be also considered. We report on a patient with heterotaxy syndrome and double outlet right ventricle, in whom postoperative cyanosis was associated with an Abernethy malformation type II causing a hepatopulmonary syndrome. Despite this complex anatomy, interventional closure of the portosystemic shunt was done with a Konar MF™-VSD Occluder. The patient recovered rapidly with relief of cyanosis within one month. This case highlights the importance of a careful diagnostic assessment in patients with complex CHD, who presents cyanoses after surgical repair. In addition, it shows the feasibility and safety of a percutaneous approach with complete closure of the vascular malformation in a patient with a complex anatomy.

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“…The median age at diagnosis is 21 years, with a range of 0 to 66 years [9]. The chronicity and severity of symptoms depend on the degree of intrahepatic portal vein hypoplasia and the volume of blood moving through any shunts that are present [4,[10][11]. Patients diagnosed during childhood can have failure to thrive, psychomotor delay, cholestasis, and hypergalactosemia.…”

Section: Introductionmentioning

confidence: 99%

Liver Transplantation as a Treatment for Unresectable Hepatic Adenoma in a Patient With Abernethy Syndrome

Patel,

Thompson,

Sharshar

et al. 2024

Cureus

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Abernethy syndrome is a rare congenital anomaly characterized by an intrahepatic or extrahepatic portosystemic shunt. Most patients are asymptomatic; however, due to the alteration in, or lack of, a portovenous flow, patients with Abernethy syndrome are at high risk of developing sequelae of liver failure. Once these complications develop, the only definitive treatment is transplantation. Patients with Abernethy syndrome are also at a higher risk of developing benign and malignant liver lesions, including hepatic adenomas. Here, we describe the first case of deceased donor liver transplantation as a treatment for a patient with type 1 Abernethy syndrome complicated by large, unresectable hepatic adenoma, found to have focal hepatocellular carcinoma on pathologic examination. Our male patient was found to have elevated liver enzymes at age 33, during a routine outpatient medical appointment. Despite being asymptomatic, his history of prior liver resection prompted CT imaging, which revealed two large liver lesions concerning for hepatic adenomas. When surveillance imaging showed a significant growth of the liver lesions, biopsy was pursued, which confirmed a diagnosis of hepatic adenomas. However, given the size of these lesions, resection was not a viable option for the patient. Instead, the patient underwent liver transplantation at age 41, which he tolerated well. Our case demonstrates the utility of deceased donor liver transplantation as a treatment for patients with Abernethy syndrome complicated by unresectable adenomas.

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Live donor liver transplantation for a child presented with severe hepatopulmonary syndrome and nodular liver lesions due to Abernethy malformation

Cited by 6 publications

References 16 publications

Congenital extrahepatic portocaval malformation: Rare but potentially treatable cause of pulmonary hypertension

Congenital extrahepatic portocaval malformation: Rare but potentially treatable cause of pulmonary hypertension

Successful percutaneous treatment with the Konar MFTM-VSD Occluder in an infant with Abernethy syndrome—case report

Liver Transplantation as a Treatment for Unresectable Hepatic Adenoma in a Patient With Abernethy Syndrome

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