Congenital extrahepatic portosystemic shunt

What is the cause of congenital extrahepatic portosystemic shunts (CEPS)?

The cause of congenital extrahepatic portosystemic shunts (CEPS) is unknown. Sometimes CEPS occurs along with other conditions such as polysplenia or congenital heart defects.

Last updated on 05-01-20

How might congenital extrahepatic portosystemic shunt be diagnosed?

Congenital extrahepatic portosystemic shunt (CEPS) is often diagnosed during an ultrasound examination of an infant or child who shows signs of liver disease. In some cases, CEPS may be diagnosed by chance during an ultrasound done for other reasons. A magnetic resonance angiography (MRA) may help to clarify a diagnosis of CEPS by allowing a physician to see the blood vessels of the liver. A contrast CT scan with 3D reconstruction can also be helpful.

Last updated on 05-01-20

How is congenital extrahepatic portosystemic shunt inherited?

Congenital extrahepatic portosystemic shunt (CEPS) is not known to be inherited in families.

Last updated on 05-01-20

What is the long-term outlook for people with congenital extrahepatic portosystemic shunts (CEPS)?

Congenital extrahepatic portosystemic shunts (CEPS) are very rare and the long-term outlook is unclear. The severity of the symptoms and the presence of other health conditions influence the long-term outlook for this condition.

Last updated on 05-01-20

How many people have congenital extrahepatic portosystemic shunt (CEPS)?

Congenital extrahepatic portosystemic shunts (CEPS) are quite rare. As of 2019, there were only about 300 reported cases in the medical literature; most of them were females under the age of 18.

Last updated on 05-01-20

How might congenital extrahepatic portosystemic shunt (CEPS) be treated?

Because congenital extrahepatic portosystemic shunts (CEPS) are rare, there are no guidelines for standard treatment of this condition. Treatment is determined on an individual basis and depends on the type of CEPS. In type I CEPS, liver transplantation is thought to be the only treatment. Type II CEPS can be treated with surgery.

Last updated on 05-01-20

Gupta P, Sinha A, Sodhi KS, Lal A, Debi U, Thapa BR, Khandelwal N. Congenital extrahepatic portosystemic shunts: Spectrum of findings on ultrasound, computed tomography, and magnetic resonance imaging Rad Res Pract. Dec 2015; 2015. Reference Link Xie L, Li Y, Jiang X, Zhao J, Xiao T. A 10-year-old boy with dyspnea and hypoxia: Abernathy malformation masquerading as pulmonary arteriovenous fistula BMC Pediatr. Feb 11, 2019; 19(1). 55-60. Reference Link

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