Congenital portosystemic shunts with and without gastrointestinal bleeding – case series

Gong, Yandong; Zhu, Hui; Chen, Qi; Ji, Min; Modesti, Pietro Amedeo; Zheng, Shan; Zhang, Qiao

doi:10.1007/s00247-015-3417-6

Cited by 21 publications

(14 citation statements)

References 25 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…The results of the current study show that patients with CEPS can remain asymptomatic until late in the disease and be undiagnosed until an advanced age. This is in contrast with previous case reports and series, where approximately 75% of CEPS were diagnosed during childhood . However, because most of the participating centers take care of adult patients, a bias evaluating the age of our patients at diagnosis cannot be discarded.…”

Section: Discussionmentioning

confidence: 61%

See 1 more Smart Citation

Congenital Extrahepatic Portosystemic Shunts (Abernethy Malformation): An International Observational Study

et al. 2019

View full text Add to dashboard Cite

Congenital extrahepatic portosystemic shunt (CEPS) or Abernethy malformation is a rare condition in which splanchnic venous blood bypasses the liver draining directly into systemic circulation through a congenital shunt. Patients may develop hepatic encephalopathy (HE), pulmonary hypertension (PaHT), or liver tumors, among other complications. However, the actual incidence of such complications is unknown, mainly because of the lack of a protocolized approach to these patients. This study characterizes the clinical manifestations and outcome of a large cohort of CEPS patients with the aim of proposing a guide for their management. This is an observational, multicenter, international study. Sixty‐six patients were included; median age at the end of follow‐up was 30 years. Nineteen patients (28%) presented HE. Ten‐, 20‐, and 30‐year HE incidence rates were 13%, 24%, and 28%, respectively. No clinical factors predicted HE. Twenty‐five patients had benign nodular lesions. Ten patients developed adenomas (median age, 18 years), and another 8 developed HCC (median age, 39 years). Of 10 patients with dyspnea, PaHT was diagnosed in 8 and hepatopulmonary syndrome in 2. Pulmonary complications were only screened for in 19 asymptomatic patients, and PaHT was identified in 2. Six patients underwent liver transplantation for hepatocellular carcinoma or adenoma. Shunt closure was performed in 15 patients with improvement/stability/cure of CEPS manifestations. Conclusion: CEPS patients may develop severe complications. Screening for asymptomatic complications and close surveillance is needed. Shunt closure should be considered both as a therapeutic and prophylactic approach.

show abstract

Section: Discussionmentioning

confidence: 61%

“…This is in contrast with previous case reports and series, where approximately 75% of CEPS were diagnosed during childhood. (3,16,19,25,26) However, because most of the participating centers take care of adult patients, a bias evaluating the age of our patients at diagnosis cannot be discarded.…”

Section: Discussionmentioning

confidence: 99%

Congenital Extrahepatic Portosystemic Shunts (Abernethy Malformation): An International Observational Study

et al. 2019

View full text Add to dashboard Cite

show abstract

“…Gastrointestinal bleeding as a presenting symptom has been reported in 8.1% of cases with EPSS. In the majority of these cases, the ending systemic veins of the shunt were the iliac veins, resulting in colonic and rectal varices [ 45 ]. Encephalopathy and liver tumors were uncommon in this group of patients perhaps suggesting that the shunt was only partial and provided a degree of protection.…”

Section: Clinical Picturementioning

confidence: 99%

Congenital portosystemic venous shunt

2017

View full text Add to dashboard Cite

Congenital portosystemic venous shunts are rare developmental anomalies resulting in diversion of portal flow to the systemic circulation and have been divided into extra- and intrahepatic shunts. They occur during liver and systemic venous vascular embryogenesis and are associated with other congenital abnormalities. They carry a higher risk of benign and malignant liver tumors and, if left untreated, can result in significant medical complications including systemic encephalopathy and pulmonary hypertension. Conclusion: This article reviews the various types of congenital portosystemic shunts and their anatomy, pathogenesis, symptomatology, and timing and options of treatment. What is Known: • The natural history and basic management of this rare congenital anomaly are presented. What is New: • This paper is a comprehensive review; highlights important topics in pathogenesis, clinical symptomatology, and treatment options; and proposes an algorithm in the management of congenital portosystemic shunt disease in order to provide a clear idea to a pediatrician. An effort has been made to emphasize the indications for treatment in the children population and link to the adult group by discussing the consequences of lack of treatment or delayed diagnosis.

show abstract

“…Over time, unprocessed portal metabolites result in hyperammonemia and subsequent hepatic encephalopathy . Patients with shunts that go unrecognized may be misdiagnosed with psychiatric and/or neurologic disorders .…”

Section: Introductionmentioning

confidence: 99%

“…7 Over time, unprocessed portal metabolites result in hyperammonemia and subsequent hepatic encephalopathy. 9 Patients with shunts that go unrecognized may be misdiagnosed with psychiatric and/or neurologic disorders. 5 Doppler ultrasound has been described as one of the most important diagnostic tools which help to calculate the portovenous shunt ratio by dividing the total blood flow volume in the shunt by that in the portal vein.…”

Section: Introductionmentioning

confidence: 99%

Congenital intrahepatic portosystemic venous shunt embolization: A two‐case experience

Molano

Krauthamer

Barrera

et al. 2020

Clinical Case Reports

View full text Add to dashboard Cite

show abstract

Congenital portosystemic shunts with and without gastrointestinal bleeding – case series

Abstract: Most congenital portosystemic shunt patients with gastrointestinal bleeding had a shunt that drained portal blood into the iliac vein via an inferior mesenteric vein. This type of shunt was uncommon, but the concomitant rate of gastrointestinal bleeding with this type of shunt was high.

Cited by 21 publications

References 25 publications

Congenital Extrahepatic Portosystemic Shunts (Abernethy Malformation): An International Observational Study

Congenital Extrahepatic Portosystemic Shunts (Abernethy Malformation): An International Observational Study

Congenital portosystemic venous shunt

Congenital intrahepatic portosystemic venous shunt embolization: A two‐case experience

Contact Info

Product

Resources

About