Pregnancy

Atresia of the bile ducts

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Biliary atresia refers to severe congenital malformations inside the womb of the mother. Pathology is accompanied by a complete or segmental fusion of the bile ducts. The disease is detected at an early age, is rare. Only 8% of all malformations of organs account for artesia. Pathology is dangerous, therefore it is important to identify and surgically correct this deficiency. If timely assistance is not provided, the child will die in the first months of life from the development of food bleeding, liver failure, infection or intoxication of the body.

Pathology description

Atresia of the biliary tract is characterized by two conditions:

  • prenatal malformation, accompanied by a complete absence of bile ducts,
  • sluggish inflammatory process that developed during the period of prenatal development, as a result of which the lumen of the bile ducts of the extrahepatic tissues partially or completely narrows.

In both cases, there is underdevelopment or a complete absence of biliary tract, which is accompanied by dysfunction of the channels located inside or outside the liver.

Obliteration or narrowing of the bile ducts may include:

  • all departments of extrahepatic excretory canals,
  • individual sections of the ductal mesh,
  • only channels in the liver.

Atresia biliary tract in rare cases, one flows. In most cases, there are deformities of the cranial bones and the spine, pathologies of the excretory system, blood vessels and the heart against the background of pathology.

The factors provoking the pathology of bile-conductive passages have not been fully studied. Most patients with atresia have formed bile ducts, but bile cannot be removed from them due to the strong narrowing of the lumen or the progressive destruction of the ductal walls from the inside.

In the second case, the cause of atresia is obstructive cholangiopathy, when the bile ducts are affected by an infection transmitted from mother to child during gestation. Such viruses include cytomegaly, herpes, rubella, neonatal hepatitis.

Rare cases is the detection of atresia of the bile ducts caused by blockage of the channels. Such a course can be caused by congenital anomalies of development or appear in the perinatal period from the 28th week of pregnancy on the background of progressive inflammation.

The complete absence of the bile duct system is associated with a disorder in the laying of the biliary system in the first 2 months of pregnancy when the embryo is formed. With such a development of pathology, the gallbladder may form or be absent.

In 20% of cases, atresia of the bile ducts is accompanied by abnormal development of other organs and systems, such as the heart (congenital malformations), the gastrointestinal tract (incomplete bowel bend), the immune system (absence or presence of more than one spleen).

Classification

There are several parameters by which atresia is subdivided into subspecies:

  • By location:
    • extrahepatic tissues
    • inside the liver
    • mixed type.
  • According to the localization of the sites of narrowing is divided into:
    • correctable pathology, when the patency of the main hepatic or gall channel is impaired,
    • uncorrected anomaly, when all ducts are replaced by inert fibrous tissue.

The degree of blockage of the bile ducts and the location of atresia are the main parameters affecting the choice of method of operation.

The first manifestations of bile duct atresia are detected during the first 6 weeks of a child's life. If the newborn has all the anthropometric indices normal, small jaundice is attributed to the natural physiological adaptation of the small organism. Therefore, often these children are discharged from the hospital with a diagnosis of "prolonged physiological jaundice."

Over time, the yellowness of the skin increases, appears on the eyeballs. Urine becomes dark, feces discolored. The spleen with the liver gradually increases, their tissues become denser. The deterioration is noticeable from the second month of life. The child has:

  • lack of appetite
  • body weight is reduced,
  • physical development is delayed
  • there is a sharp decline in strength
  • muscular weakness develops with a decrease or cessation of motor activity.

Mechanical jaundice is aggravated and causes constant itching, which is why the child is constantly crying. On skin appear flat benign bumps of yellow color - lipid deposits. By the sixth month of the baby's life:

  • biliary cirrhosis of liver tissue develops,
  • increases pressure on the main portal vein, which connects the blood flow of the intestine and liver,
  • the abdominal organs are filled with free fluid
  • there are punctate hemorrhages on the skin, bleeding of the mucous membranes and esophagus,
  • impulse transmission from the brain and spinal cord to various organs is disturbed.

Due to the deficiency of vitamins, rickets and neuromuscular disorders appear, and vascular fragility increases, which causes seizures of blood vomiting.

Diagnostics

A highly informative method for diagnosing atresia in newborns is a percutaneous liver tissue biopsy. Additionally, differential diagnosis is carried out in order to recognize the atresia of the bile duct pathways among similar symptoms:

  • hypothyroidism
  • bile thickening syndrome
  • genetic metabolic disorders
  • medicinal lesions of the liver tissue.
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Atresia can be cured in small patients only by surgery. In this case, the operation must be carried out before the child is 3 months old. Otherwise, irreversible destruction occurs in other organs and systems of the child’s body. In such cases, the operation will be unsuccessful.

If atresia manifests as obstruction of the main bile or hepatic canal with the formation of a conical narrowing with uneven, asymmetric contours, is used:

  • surgery with the imposition of choledochoenteroanastomosis with interintestinal fistula and switching off of the afferent loop,
  • method of forming a bypass of the hepato-intestinal anastomosis between the hepaticocholedochus and jejunum with the imposition of hepaticojejunostomy.

In case of complete atresia of the bile ducts of the extrahepatic tissues, an anastomosis (a natural connection) is required between the hepatic gates and the small intestine - portoenterostomy according to Kasai. In the case of severe gastrointestinal bleeding with increased pressure on the portal vein, portocaval shunting is performed (drainage of the portal vein into the caudal vena cava).

If there is progressive liver failure with a constant enlargement of the spleen, the question of liver transplantation is being considered.

In the postoperative period, complications are possible in the form of inflammation of the bile ducts, accumulation of purulent masses due to infection of the liver tissues. In the absence of surgery, children with atresia do not live to the age of 1 year. They die from acute liver failure, heavy bleeding and intercurrent (aggravating) diseases.

With surgical intervention in the early stages of the outcome of treatment is favorable. If the operation is carried out before the expiration of 2 months of the child's life, the chance of survival of the next 10 years is 70% or more. After surgery at 2-3 months of age, the number of survivors is 27%. If surgical treatment is applied to older children, only 11% survive.

Inflammation of the bile duct reduces the chances of recovery for young patients. The method of surgical intervention developed by Kasai - portoenterostomy allows to reduce the risk of complications.

Therefore, in order to achieve a favorable outcome of atresia treatment, it is important to promptly apply with examination of the newborn if an anomaly of the bile duct is suspected. In those cases, if the illness is not subject to cure by surgery, the operation according to the method of Kasai gives a long-lasting positive effect. In case of hepatic holedochoenterostomy, a favorable effect is also possible, but it is short-lived. Due to the drainage of the bile ducts with the removal of excess pressure from the walls prevents the development of cirrhosis of the liver tissue. In this case, the normal development and growth of the child does not stop until the operation of liver transplantation.

Atresia of the bile ducts

Atresia of the bile ducts (biliary atresia) is a severe congenital malformation, characterized by partial or complete obliteration of the lumen of the bile ducts. Atresia of the bile ducts is found in pediatrics and pediatric surgery in about 1 case per 20,000-30000 births, accounting for about 8% of all malformations of the internal organs in children. Atresia of the bile ducts is an extremely dangerous condition that requires early detection and prompt surgical treatment. Without timely surgical care, children with atresia of the bile ducts die during the first months of life from esophageal bleeding, liver failure, or infectious complications.

Causes of atresia of the bile ducts

The causes and pathogenesis of atresia of the bile ducts are not completely clear. In most cases with atresia, the bile ducts are formed, but their patency is impaired due to obliteration or progressive destruction. The most common cause of obstructive cholangiopathy are intrauterine infections (herpes, rubella, cytomegaly, etc.) or neonatal hepatitis. The inflammatory process causes damage to the hepatocytes, the endothelium of the bile ducts, followed by intracellular cholestasis and fibrosis of the bile ducts. Less commonly, atresia of the bile ducts is associated with duct ischemia. In these cases, atresia of the bile ducts will not necessarily be congenital, but may develop in the perinatal period due to a progressive inflammatory process.

True atresia of the bile ducts is detected less frequently and is associated with a violation of the primordial laying of the biliary tract in the embryonic period. In this case, in case of violation of the hepatic diverticulum or sewage of the distal parts of the biliary system, atresia of the intrahepatic bile ducts develops, and if the formation of the proximal biliary system is disturbed, atresia of the extrahepatic bile ducts is formed. The gallbladder may be the only part of the external biliary tract or be absent completely.

In about 20% of children, atresia of the bile ducts is combined with other developmental abnormalities: congenital heart defects, incomplete bowing of the intestine, asplenia or polysplenia.

Symptoms of atresia of the bile ducts

Children with atresia of the bile ducts are usually born full-term with normal anthropometric parameters. On the 3-4th day of life, they develop jaundice, but unlike the transient hyperbilirubinemia of newborns, jaundice with biliary atresia lasts long and gradually increases, giving the baby's skin a saffron or greenish tint. Usually, atresia of the bile ducts is not recognized in the first days of a child’s life, and the newborn is discharged from the hospital with a diagnosis of prolonged physiological jaundice.

Acholic (bleached) stools and urine of intense dark ("beer") color are characteristic signs of atresia of the bile ducts present from the first days of a child's life. Preservation of bleached feces for 10 days indicates atresia of the bile ducts.

By the end of the first month of life, a decrease in appetite, lack of mass, weakness, skin itch, and delayed physical development. At the same time there is an increase in the liver, and then - the spleen. Hepatomegaly is a consequence of cholestatic hepatitis, against the background of which biliary cirrhosis quickly develops, ascites, and by 4-5 months - liver failure. Impaired blood supply to the liver contributes to the formation of portal hypertension and, as a result, the esophageal varicose veins, splenomegaly, and the appearance of the subcutaneous venous network on the anterior abdominal wall. In children with atresia of the bile ducts, hemorrhagic syndrome is observed: petechial or extensive hemorrhages on the skin, bleeding from the umbilical cord, esophageal or gastrointestinal bleeding.

In the absence of timely surgical treatment, children with atresia of the bile ducts usually do not live up to 1 year, dying from liver failure, bleeding, adhered pneumonia, cardiovascular failure, intercurrent infections. With incomplete atresia of the bile ducts, some children can live to 10 years.

Treatment of bile duct atresia

An important condition for the success of treatment of atresia of the bile ducts is a surgical intervention at the age of up to 2-3 months, since in older patients the operation is usually unsuccessful due to irreversible changes.

In case of obstruction of the common bile or hepatic ducts and the presence of suprastenic enlargement, choledochoenteroanastomosis (choledochojejunostomy) or hepaticojejunostomy with a loop of the jejunum is performed. Complete atresia of extrahepatic bile ducts dictates the need to perform portoenterostomy by Kasai - the imposition of an anastomosis between the gate of the liver and the small intestine. The most frequent postoperative complications are postoperative cholangitis, liver abscesses.

In severe portal hypertension and recurrent gastrointestinal bleeding, portocaval bypass can be performed. In the case of progressive liver failure and hypersplenism, the issue of liver transplantation is resolved.

Forecast atresia of the bile ducts

In general, the prognosis of bile duct atresia is very serious, since cirrhotic changes in the liver are progressive. Without surgical care, children are doomed to death within 12-18 months.

It is very important to identify the clinical manifestations of bile duct atresia (obstinate jaundice, acholic feces) in the neonatal period and to carry out early surgical treatment. In most cases, the imposition of choledocomplex anastomoses or hepatic anastomoses can prolong the life of a child for several years and delay the need for a liver transplant.

Causes:

The causes of atresia are not exactly known. It develops at an early stage of embryogenesis, no connection with drugs or vaccinations entering the mother’s body during pregnancy is seen. In the US, it occurs in 1 child of about 10-20 thousand. Girls with atresia are born more often than boys. Cases are common when atresia gives birth to only one child from several in the family (and even just one of the twins).

Surgical

When partial obstruction or fusion is performed choledochoenteroanastomosis. The purpose of the operation is to artificially create a biliary fistula between the common hepato-bile duct and the small intestine. In true atresia, when there is no duct at all, a channel is created between the liver and the small intestine, through which bile passes. Surgical treatment makes sense to spend up to two months of a baby’s life, later irreversible changes occur. The operation is contraindicated at high risk of cardiac shock.

Conservative

Principles of supportive drug therapy:

  1. Hemostatic drugs (etamzilat sodium). They stop the bleeding. Appointed after surgery as a symptomatic treatment of hemorrhage.
  2. Antibiotics and antifungal agents (cefuroxime, amikacin, fluconazole). They are prescribed for the prevention of bacterial complications and as a preparation for surgery to prevent infection.
  3. Anesthesia (Analgin, Aspirin, Indometacin). They are taken after surgery to relieve postoperative pain.
  4. Infusion therapy (Saline solution, Albumin solution 10%, Glucose 5%). It restores the volume of circulating blood, normalizes the water-salt and acid-base balance.
  5. Nutrition (Vitamin complexes, amino acid complexes). Restores nutrient deficiencies.
  6. Choleretic drugs (Ursodeoxycholic acid). Stimulates the passage of bile into the intestines.

Also, symptomatic therapy is prescribed, for example, antiviral agents, if a virus is detected, or neurological drugs, if neurological disorders are observed in the clinical picture.

Treatment is considered effective in such cases:

  • the signs of jaundice were eliminated,
  • postoperative wound has stretched, there is no infection and symptoms of suppuration,
  • laboratory tests are normalized,
  • the bile enters the small intestine and the feces turns yellow.

Prevention

The disease is congenital, so there is no specific prevention.

In pathology, the prognosis is unfavorable: cirrhosis develops rapidly, reaching a peak by 1-1.5 years of life. If by this time the disease is not cured, the child dies by the end of the second year of life. With a successful operation, life is prolonged for several years, and a liver transplant is able to delay death for 3-5 years.

Если операция прошла без осложнений, ребенка выписывают из стационара. Родителям даются рекомендации по питанию и уходу. In the future, the baby is observed by a pediatric surgeon, a gastroenterologist and a hepatologist.

Conclusion

Atresia of the biliary tract is a congenital defect in which the biliary tract is absent, underdeveloped, or there is an obstruction in them. The reason - a violation of fetal development due to infections or a violation of the formation of the bookmark. Symptoms: jaundice appears on the 3-4th day, feces become discolored. The liver and spleen are enlarged, point hemorrhages appear on the body. Without treatment, biliary cirrhosis develops by the sixth month of life. The disease is diagnosed using complaints, laboratory and instrumental methods of research. The main treatment is surgery, which is supported by medication. The prognosis is unfavorable: the child dies by the second year of life. The operation postpones death for 2-3 years.

What is atresia?

This is a condition that occurs in newborns: one case per 30 thousand births, and the disease appears more often in girls than in boys. The only effective help for such children is surgery. The procedure involves the creation of an artificial duct or a complete liver transplant. Yet after surgery, the risk of death is more than 50%.

The disease is very dangerous, so it is important to identify the pathology as early as possible and assist the child in a timely manner, otherwise children die in the first months of life. Hepatic failure, intoxication on the background of infection.

During the period of prenatal development, the fetus is progressing inflammation, the process has a long sluggish course. As a result, atresia of the biliary tract occurs, the duct is partially narrowed or completely overgrown. The function of the channels is impaired, internal and external bile ducts and hepatic ducts are exposed to inflammation.

The disease rarely occurs as an independent, comorbid pathology: deformation of the bones of the skull and spine, pathology of the organs of the excretory system, as well as vascular and cardiac.

Causes of pathology

Factors in the development of pathology are not fully understood. Most of the diseased babies have fully formed ducts. But pathologically expanding connective tissue disrupts their patency, up to the complete overgrowth of the biliary tract. Bile is not excreted due to narrowing of the lumen, or a progressive infectious process destroys the walls of blood vessels from the inside.

Destruction occurs due to infection of the fetus from a pregnant woman with such infections as rubella, herpes, cytomegalovirus infection, SARS or intrauterine hepatitis. The process leads to damage to the hepatocytes and the endothelium of the bile ducts. Subsequently, there is stagnation of bile and duct fibrosis.

Sometimes obstruction is associated with ischemia and develops in the neonatal period as a result of an inflammatory disease. In this case, the disease is not considered congenital.

The development of the main organs and systems of the fetus during pregnancy occurs in the first two months. Atresia occurs at the stage of embryogenesis. The so-called true atresia of the biliary tract appears due to the violation of intrauterine organ insertion and is characterized by the complete absence of all ducts, and sometimes the gallbladder itself.

Manifestations of the disease

Obstruction of the ducts makes itself felt by the appearance of jaundice immediately after birth. If the baby has no other abnormalities, and all indicators are normal, they mistakenly diagnose jaundice as physiological, missing the time for treatment. At atresia, this sign progresses, increases, and liver failure occurs. The urine of a newborn acquires the color of beer, and the stool becomes almost white. If during 10 days the color of feces does not change, this indicates obstruction of the bile ducts. Symptoms may increase for 1.5 months. Over time, the yellowness appears on the eyeballs, the liver and spleen noticeably increase and thicken. There are other signs:

  • appetite disappears,
  • the child is losing weight
  • the physical development of the infant lags behind
  • the baby becomes sluggish, weak,
  • decreases motor activity.

Jaundice of the skin is accompanied by severe itching, the child becomes restless. On the body appear yellowish pimples - benign fatty deposits. By six months, a child may develop cirrhosis of the liver. The vein that provides blood flow to the intestines suffers from high blood pressure.

Because of the marked irregularities in the peritoneum, fluid is collected. Hemorrhages appear not only on the mucous membrane of the esophagus, but also on the skin. The condition is aggravated by bloody vomiting, signs of rickets, and neuromuscular disorders. If measures are not taken on time, the life expectancy of such children is calculated from a period of 10 to 18 months.

The essence of the problem

Biliary atresia of the bile ducts is a congenital anatomical defect in children, the essence of which is inflammation of the bile ducts, that is, biliary obstruction, when the fluid secreted by the liver to digest fat does not reach the small intestine. Inflammation leads to fibrosis of the bile ducts and in addition to their obstruction. The consequence of this process is the cessation of outflow of bile from the liver into the intestine - cholestasis.

Bile accumulates in excess in the liver and bile ducts, which eventually leads to an increase in pressure in the biliary tract and damage. Liver cells. The incidence of congenital biliary atresia of the biliary tract is estimated by medical experts to be about 1 case per 20,000 newborns, and girls suffer from a congenital abnormality twice as often as boys.

Atresia of the biliary tract - causes

The causes of the confluence of the bile ducts are unknown. It is assumed that the disease has an autoimmune basis, that is, the cells of the immune system attack the properly formed bile ducts, and lead to their inflammation.
The disease can also be genetically determined, although this applies to a smaller number of cases. It is also known that biliary atresia is not a hereditary pathology.

Biliary atresia - symptoms

Symptoms of atresia of the bile duct appear in the first days, or weeks of the child's life, usually between the 2nd and 6th weeks. The main symptom is prolonged jaundice, that is, yellow discoloration of the skin and eye mucosa. As a rule, jaundice can occur in many newborns and usually disappears during the first two weeks of life. If this does not happen, then with high probability we can assume atresia of the bile duct in a child.

In addition, the disease is accompanied by other symptomatic signs, for example:

  • gray or light yellow clay stools in a child,
  • dark beer color urine
  • slightly enlarged liver, visually defined in the upper right abdominal square,
  • prolonged bleeding from the navel.

Also in 10–20% of cases, in children with biliary atresia, other congenital anomalies, such as heart defects, double spleen, or polycystic kidney disease, are detected.

Atresia of the biliary tract - diagnosis

If biliary atresia is suspected, an abdominal ultrasound examination is performed, due to which the doctor can evaluate the function of the liver, bile ducts and gall bladder. In addition, bile duct scintigraphy and blood tests are recommended. When bile duct joins, bilirubin, GGTP, cholesterol, alkaline phosphatase and slightly transaminase increase.

These tests confirm the diagnosis and rule out other possible causes, such as viral and bacterial infections of the liver or congenital metabolic diseases. If the study does not give a definite result, then a liver biopsy may be required, which involves taking a fragment of an organ with a special needle for examination under a microscope.

Atresia of the biliary tract - treatment

The only way to restore the flow of bile from the liver into the intestine is an operation that should be performed as soon as possible. Surgical intervention is desirable to make no later than two months of the child's life. The later the procedure is performed, the less its effectiveness. The operation is called portosystemic anastomosis or Kasai treatment, on behalf of the doctor who administered it. Surgical manipulation involves the complete removal of the fibrous extrahepatic bile ducts. After surgery, antibacterial therapy and analgesics are used.

About half of the children, especially those who were operated on for up to two months of life, successfully avoided complications such as liver failure and other aggravating pathologies. However, even after successful surgery, progressive liver damage occurs. In this case, it is necessary to transplant an organ of vital activity during the first two years of the child's life.
Take care of your children and be always healthy!

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