It is an operation that results in a hepato-porto-enterostomy. Named after the surgeon who invented it, the Kasai procedure is the first-line option for treating biliary atresia. Failure of the bile ducts, like in biliary atresia, causes bilirubin to accumulate in the blood. Usually, bilirubin is taken up by the liver to be processed and excreted into bile. These symptoms are a direct consequence of the failure of the liver to remove bilirubin, which is a substance physiologically produced by the breakdown of hemoglobin.

Other findings in these infants include light-colored stools (grey or white), poor growth and weight gain as well as very dark urine. The infant has yellow skin as well as eyes. BA has a predilection in premature babies, females, and infants of African American or Asian descent. It has been suggested that BA is not inherited from the parents, however, possible triggers include infections after birth (e.g., CMV), exposure to toxic substances, gene mutations, autoimmune dysfunction, and faulty embryogenesis. The exact cause has not yet been identified, but several factors have been taken into consideration as potential culprits. Like embryonic BA, these infants have a poor prognosis as well.īiliary Atresia - What is Biliary Atresia Play Pathophysiological Mechanisms and Clinical Presentation BA with other congenital malformations - Occurs in about 5% of affected infants and they present with malformations in organ systems such as the kidneys and may have heart defects, intestinal atresia, and imperforate anus.

It occurs in about 10% of infants affected with BA and they tend to have a poorer prognosis compared to those that have BA without any congenital malformations.

Embryonic BA - This form of BA is also called BA splenic malformation and these infants tend to have cardiac anomalies and inferior vena cava interruptions and, as implied by the name, splenic anomalies such as polysplenia (accessory spleens) or asplenia (absence of normal spleen function).

However, they do develop jaundice within their first two months and their stool becomes increasingly alcoholic.

BA without other malformations - This pattern of BA is seen in up to 85% of those affected and these infants do not present with jaundice at birth.Infants affected by the condition may be grouped into three categories:

Atresia Das Vias Biliares Pdf