A hepatoportoenterostomy or Kasai portoenterostomy is a

surgical Surgery ''cheirourgikē'' (composed of χείρ, "hand", and ἔργον, "work"), via la, chirurgiae, meaning "hand work". is a medical specialty that uses operative manual and instrumental techniques on a person to investigate or treat a pat ...

treatment performed on infants with Type IVb choledochal cyst and

biliary atresia Biliary atresia, also known as extrahepatic ductopenia and progressive obliterative cholangiopathy, is a childhood disease of the liver in which one or more bile ducts are abnormally narrow, blocked, or absent. It can be congenital or acquired. I ...

to allow for bile drainage. In these infants, the

bile Bile (from Latin ''bilis''), or gall, is a dark-green-to-yellowish-brown fluid produced by the liver of most vertebrates that aids the digestion of lipids in the small intestine. In humans, bile is produced continuously by the liver (liver bi ...

is not able to drain normally from the small

bile duct A bile duct is any of a number of long tube-like structures that carry bile, and is present in most vertebrates. Bile is required for the digestion of food and is secreted by the liver into passages that carry bile toward the hepatic duct. ...

s within the

liver The liver is a major organ only found in vertebrates which performs many essential biological functions such as detoxification of the organism, and the synthesis of proteins and biochemicals necessary for digestion and growth. In humans, it i ...

into the larger bile ducts that connect to the

gall bladder In vertebrates, the gallbladder, also known as the cholecyst, is a small hollow organ where bile is stored and concentrated before it is released into the small intestine. In humans, the pear-shaped gallbladder lies beneath the liver, although ...

and

small intestine The small intestine or small bowel is an organ (anatomy), organ in the human gastrointestinal tract, gastrointestinal tract where most of the #Absorption, absorption of nutrients from food takes place. It lies between the stomach and large intes ...

Procedure

The surgery involves exposing the porta hepatis (the area of the liver from which bile should drain) by radical excision of all bile duct tissue up to the liver capsule and attaching a

Roux-en-Y In general surgery, a Roux-en-Y anastomosis, or Roux-en-Y, is an end-to-side surgical anastomosis of bowel used to reconstruct the gastrointestinal tract. Typically, it is between stomach and small bowel that is distal (or further down the gastr ...

loop of jejunum to the exposed liver capsule above the bifurcation of the portal vein creating a portoenterostomy. The rationale for this approach is that minute residual bile duct remnants may be present in the fibrous tissue of the porta hepatis and thus provide direct connection with the intrahepatic ductule system to allow bile drainage. This procedure was developed in 1951 by Japanese biliary and hepatic pediatric surgeon Morio Kasai (1922–2008). :de:Kasai Morio

Prognosis

Prognosis of this condition/procedure comprises the following: * If performed before 60 days of age, 80% of children achieve some

drainage * Prognosis is progressively worse the later surgery is done * Post-operatively, cholangitis and

malabsorption Malabsorption is a state arising from abnormality in absorption of food nutrients across the gastrointestinal (GI) tract. Impairment can be of single or multiple nutrients depending on the abnormality. This may lead to malnutrition and a variety ...

are common * Many children with biliary atresia will require

liver transplantation Liver transplantation or hepatic transplantation is the replacement of a Liver disease, diseased liver with the healthy liver from another person (allograft). Liver transplantation is a treatment option for Cirrhosis, end-stage liver disease and ...

despite the attempted surgical repair, although on occasion, it can be delayed until adulthood.

References

Accessory digestive gland surgery Hepatology Pediatric surgical procedures {{surgery-stub

Procedure

Prognosis

See also

References