Medical Dictionary

Biliary atresia - Medical

Biliary atresia: Congenital absence or closure of the major bile ducts, the ducts that drain bile from the liver.

Biliary atresia results in a progressive inflammatory process which may lead to cirrhosis of the liver. The infant looks normal at birth but develops jaundice after the age of 2 to 3 weeks with yellowing of the eyes and skin, light-colored stools and dark urine caused by the build up of the pigment called bilirubin in the blood. The abdomen may be swollen with a firm, enlarged liver. Weight loss and irritability develop as the jaundice increases.

There is one case of biliary atresia out of every 15,000 live births. Females are affected slightly more often than males.

The preferred treatment is to attach the small intestine directly to the liver at the spot where bile is found or expected to drain. Bile flow is re-established in approximately 80% of infants who are operated before 3 months of age. Of these, about 50% will have some bile drainage and as many as 30% will have complete bile drainage. About 20% of infants will not be helped by the procedure. In these cases, the only option is a liver transplant.


Last Editorial Review: 2/19/2001

Congenital: Present at birth. A condition that is congenital is one that is present at birth. There are numerous uses of "congenital" in medicine. There are, for example, congenital abnormalities. (For more examples, see below.)

Versus "genetic": One dictionary erroneously defines "congenital" as meaning: "Occurring prior to birth, due to parent's genetic input." Congenital does not mean genetic. Something that is congenital may or may not be genetic (inherited). For example, congenital syphilis is present at birth but is not genetic.

Timing: Something that is congenital may or may not occur "prior to birth." The essential feature is that it is there at birth (if not before).

Etymology: Congenital comes from the Latin congenitus which is made up of com-, with + genitus, the past participle of gignere, to bring forth. The word "congenital" has not been used in English since its birth but first appeared in 1796. The term "congenital" is synonymous with "innate."

Examples of terms using the word "congenital:

  • Congenital achromatopsia
  • Congenital anemia and triphalangeal thumbs
  • Congenital anomaly
  • Congenital arthrogryposis
  • Congenital central hypoventilation syndrome
  • Congenital clasped thumb with mental retardation
  • Congenital deafness
  • Congenital defect
  • Congenital heart disease
  • Congenital hemolytic jaundice
  • Congenital hip dislocation
  • Congenital hypothyroidism
  • Congenital malformation
  • Congenital neutropenia
  • Congenital ptosis of the eyelids
  • Congenital torticollis

Last Editorial Review: 4/27/2011 5:27:15 PM

Liver: An organ in the upper abdomen that aids in digestion and removes waste products and worn-out cells from the blood. The liver is the largest solid organ in the body. The liver weighs about three and a half pounds (1.6 kilograms). It measures about 8 inches (20 cm) horizontally (across) and 6.5 inches (17 cm) vertically (down) and is 4.5 inches (12 cm) thick.

Liver Organ - Illustration

The liver has a multitude of important and complex functions. Some of these functions are to:

  • Manufacture (synthesize) proteins, including albumin (to help maintain the volume of blood) and blood clotting factors
  • Synthesize, store, and process (metabolize) fats, including fatty acids (used for energy) and cholesterol
  • Metabolize and store carbohydrates, which are used as the source for the sugar (glucose) in blood that red blood cells and the brain use
  • Form and secrete bile that contains bile acids to aid in the intestinal absorption (taking in) of fats and the fat-soluble vitamins A, D, E, and K.
  • Eliminate, by metabolizing and/or secreting, the potentially harmful biochemical products produced by the body, such as bilirubin from the breakdown of old red blood cells and ammonia from the breakdown of proteins
  • Detoxify, by metabolizing and/or secreting, drugs, alcohol, and environmental toxins

Last Editorial Review: 4/27/2011 5:27:15 PM

Atresia: Absence of a normal opening or failure of a structure to be tubular.

Atresia can affect many structures in the body, including:

  • Anal atresia -- congenital absence of a hole at the bottom end of the intestine. Also called imperforate anus.
  • Aortic atresia -- congenital absence of the normal valvular opening into the aorta.
  • Biliary atresia -- absence of the major bile ducts, causing jaundice.
  • Choanal atresia -- congenital failure of one or both nasal passages to open.
  • Esophageal atresia -- a birth defect in which part of esophagus is not hollow.
  • Intestinal atresia -- obliteration of the hollow of the small intestine, involving the ileum (50% of cases) or the jejunum or duodenum.
  • Laryngeal atresia -- congenital failure of the laryngeal opening to develop, resulting in partial or total obstruction at or just above or below the glottis.
  • Pulmonary atresia -- congenital absence of the pulmonary valve opening in the heart.
  • Tricuspid atresia -- congenital lack of the tricuspid valve opening.
  • Vaginal atresia -- congenital occlusion of the vagina or subsequence adhesion (sticking together) of the walls of the vagina occluding it.

Last Editorial Review: 4/27/2011 5:27:15 PM

Progressive: Increasing in scope or severity. Advancing. Going forward. In medicine, a disease that is progressive is going from bad to worse.


Last Editorial Review: 4/27/2011 5:27:15 PM

Cirrhosis: An abnormal liver condition characterized by irreversible scarring of the liver. Alcohol and viral hepatitis B and C are among the many causes of cirrhosis. Cirrhosis can cause yellowing of the skin ( jaundice), itching, and fatigue. Diagnosis of cirrhosis can be suggested by physical examination and blood tests, and can be confirmed by liver biopsy in some patients. Complications of cirrhosis include mental confusion, coma, fluid accumulation ( ascites), internal bleeding, and kidney failure. Treatment of cirrhosis is designed to limit any further damage to the liver as well as complications. Liver transplantation is becoming an important option for patients with advanced cirrhosis.


Last Editorial Review: 4/27/2011 5:27:15 PM

Date 20 - 10 - 2011