Atresia of the extrahepatic bile ducts. Biliary atresia - diagnosis of the condition. Congenital biliary atresia

It is important that at the slightest suspicion of atresia you need to consult a doctor and undergo an examination. Late surgery increases the risk of death of the child; the percentage of surviving children is sharply reduced, as there is a threat of developing cirrhosis of the liver.

Biliary atresia is a complex pathological condition characterized by partial or complete fusion bile ducts. This is a fairly rare congenital malformation. Biliary atresia in children accounts for 8% of all emerging organ pathologies.

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

The disease is very dangerous, so it is important to identify the pathology as early as possible and provide assistance to the child in a timely manner, in otherwise children die in the first months of life. Developing liver failure, intoxication due to infection.

During intrauterine development inflammation progresses in the fetus, the process has a long, sluggish course. The result is atresia biliary tract, the duct partially narrows or becomes completely overgrown. The function of the channels is disrupted, the internal and external bile and hepatic ducts become inflamed.

The disease rarely occurs on its own, accompanying pathologies: deformation of the bones of the skull and spine, organ pathologies excretory system, as well as vascular and cardiac.

Causes of pathology

Factors in the development of pathology have not been fully studied. Most affected infants have fully formed ducts. But pathologically growing connective tissue disrupts their patency until the bile ducts become completely overgrown. Bile is not excreted due to narrowing of the lumen, or 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 infections such as rubella, herpes, cytomegalo viral infection, ARVI or intrauterine hepatitis. The process leads to damage to hepatocytes and endothelium bile ducts. Subsequently, stagnation of bile and fibrosis of the ducts occurs.

Sometimes obstruction is associated with ischemia and develops during the neonatal period as a consequence 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 biliary atresia appears as a result of a violation of the intrauterine anlage of organs 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. If the baby has no other abnormalities, and all indicators are normal, jaundice is mistakenly diagnosed as physiological, missing time for treatment. With atresia, this symptom progresses, increases, and liver failure occurs. The newborn's urine becomes the color of beer, and the stool becomes almost white. If the color of the stool does not change within 10 days, this indicates obstruction of the bile ducts. Symptoms may increase over 1.5 months. Over time, yellowness appears on eyeballs, the liver and spleen noticeably enlarge and thicken. Other signs appear:

• appetite disappears;
• the child loses weight;
• the physical development of the baby is delayed;
• the baby becomes lethargic and weak;
• motor activity decreases.

Jaundice skin accompanied by severe itching, the child becomes restless. Yellowish pimples appear on the body - benign body fat. By six months, a child may develop cirrhosis of the liver tissue. From high blood pressure the vein that provides blood flow to the intestines suffers.

Due to the noted disorders, fluid collects in the peritoneum. Hemorrhages appear not only on the esophageal mucosa, but also on the skin. Condition, signs of rickets, neuromuscular disorders. If measures are not taken in time, the life expectancy of such children is calculated from 10 to 18 months.

Diagnostics

In the first days of a baby’s life, it is difficult to distinguish from pathology of the bile ducts, therefore best time for diagnosis - after the first week of life and up to one and a half months.

Particular attention should be paid to the fact that jaundice does not decrease over time, but increases.

Atresia of the bile ducts has several forms: hypoplasia and atresia, disorder of branching and fusion, perforation, stenosis and cysts of the common bile duct, bile ducts. Depending on the location of the pathology, atresia of the extrahepatic ducts, disorders within the liver, and a mixed type disease are distinguished.

Based on the location of the narrowing of the ducts (hepatic, bile), correctable and uncorrectable obstruction of the main canals is distinguished. If all the ducts inside are covered with fibrous tissue, the pathology is considered uncorrectable. The complexity of the canal obstruction, the area of ​​atresia, and its characteristics directly influence the choice of surgical intervention technique.

Treatment

The disease can only be treated surgically. Surgical intervention is performed on children under two months of age. It is important to consult a doctor at the slightest suspicion of atresia and undergo an examination.

Late surgery increases the risk of death of the child; the percentage of surviving children is sharply reduced, as there is a threat of liver cirrhosis.

The operation is to create an adequate outflow for bile. The most commonly used reconstructive technique is Kasai, named after the Japanese surgeon. An anastomosis is performed connecting the gates of the liver and the small intestine. The operation provides positive effect on a long period. However, it is possible postoperative complications in the form or liver abscess due to tissue infection.

If the operation is performed in a timely manner, positive dynamics are observed, the outcome is favorable in 30% of children. When there is no effect from treatment, the intrahepatic ducts are covered by the fusion or reconstructive surgery is impossible, the need arises complete transplant liver.

Biliary atresia(biliary atresia) is a rare congenital pathology in which the bile ducts are obstructed or absent. The only treatment is surgery newborn for the purpose of artificially creating ducts ( portoenterostomy ru en, Kasai procedure) or liver transplant. But even with surgery, the probability of death is above 50%.

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Biliary atresia - causes, symptoms, diagnosis, treatment & pathology

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Atresia is the closure or absence of a cavity or passage, and biliary refers to the bile, bile duct or bladder, so biliary atresia is some kind of blockage, deformation or even complete absence bile duct. Biliary atresia occurs especially often in newborns or infants, and may mean congenital pathology, that is, what happened in intrauterine development, but in a broad sense it is its own a kind of inflammation, which occurs shortly after birth and leads to the destruction of the bile ducts. There is no specific cause of biliary atresia, but it is generally accepted that the inflammation is caused by some kind of virus or toxin. It was also assumed that this was some kind of genetic mutation, such as in the CFC1 gene, which is involved in the development of the left-right axes of the embryo. Mutations in this gene have also been associated with other congenital disorders. In any case, its processes and pathogenesis are very unclear, and most likely depend on a combination of genetic and environmental factors. Newborns with biliary atresia usually present with jaundice. Since the bile ducts are damaged or destroyed, there is obviously an obstruction in the flow of bile, right? Bile accumulates, the pressure in the ducts increases and the bile is sooner or later pushed through the tight ligaments between the epithelial cells connecting the ducts and flows into the blood. This increases the level of paired bilirubin in the blood, which leads to a yellow tint to the skin and eyes. Although it is not the most common cause of jaundice in babies, it is the most common cause of jaundice that can be treated with surgery, and early diagnosis of the disease makes surgery more effective. Regular surgical procedure for biliary atresia, the Kasai operation is called, in which the damaged bile ducts located with outside liver, and then the small intestine is cut and one end is directly attached to the liver, where the bile comes from and where the bile duct used to be. This allows bile to flow directly into the small intestine. Its other end is attached to another place in the intestine in such a way that it creates both a passage from the stomach to the small intestine and a passage for bile from the liver. Although such surgery may be effective in restoring at least some bile flow, many infants continue to experience slow progression of liver disease, and they often require a liver transplant by age two.

Classification of bile duct atresia according to Sawaguchi

Location of obstruction:

• Type I - Common bile duct
• Type II - Hepatic duct
• Type III - Intrahepatic ducts

Condition of the common bile duct:

• unchanged
• in the form of a fibrous cord
• aplasia
• other changes

Symptoms

Atresia of the extrahepatic ducts manifests itself immediately after birth with jaundice with a greenish tint, which further progresses with damage to hepatocytes and the development of liver failure. From the first days, the urine is the color of dark beer, and the stool is acholic. Without treatment, children's life expectancy is 11-16 months. Diagnosis is possible between 1 and 6 weeks after birth; in the first days of life, it cannot be distinguished from ordinary jaundice in newborns, which occurs quite often and does not indicate anything dangerous. Characteristic of atresia is the increase in jaundice. About 40% of infants develop characteristic grayish-white stools without bile (acholic).

The exact causes of atresia are unknown. It develops at an early stage of embryogenesis; no connection is seen with medications or vaccinations entering the mother’s body during pregnancy. In the United States, it occurs in 1 child out of approximately 10-20 thousand. Girls with atresia are born slightly more often than boys. It is common for only one child out of several in a family to be born with atresia (and even only one of twins).

Treatment

It is advisable to perform the operation no later than on two-month-old children, since positive results surgery can destroy developing cirrhosis of the liver, sharply reducing the survival rate. Currently, reconstructive operations (the Kasai procedure, named after the Japanese surgeon who proposed it) are widely used; if performed in a timely manner, a positive prognosis is expected in 30-40% of children. In the absence of effect or impossibility of reconstructive surgery (for example, disruption of the intrahepatic ducts), liver transplantation is indicated.

Rarely observed. On average, for every 20,000-30,000 newborns, there is one with biliary atresia or aplasia. According to Tour's observations, they are much more common. These are our observations.

The cause of atresia is most likely due to delayed recanalization. Obstruction of the lumen may be a consequence of an inflammatory process suffered during intrauterine development or mechanical compression by tumors, cysts, or gallstones. Predominantly observed are atresias of the extrahepatic biliary tract - ductus hepaticus cysticias, choledochus, separately or all at the same time. Intrahepatic bile ducts are less commonly affected (approximately 20 times less frequently). The gallbladder may also be completely absent, or it may be small and underdeveloped. Combination with other congenital defects is rare.

Clinical manifestations for these anomalies are the same. The picture comes to the fore obstructive jaundice. Children are usually born healthy. In the complete absence of the bile ducts, jaundice appears in the first hours after birth. It is characteristic of jaundice that it persists and becomes more intense, intensifying every day. With partial or complete atresia of the biliary tract, jaundice may appear after a few days. The child's skin gradually acquires a yellowish-green color. Mucous oral cavity and the sclera is also icteric. Itching and bradycardia, however, are absent. Despite jaundice, the general condition of children remains normal for a long time. They retain their appetite, suck well and gain weight normally in the first 1-2 months. Then children begin to lose weight, vomiting, malnutrition appear, and as a result of impaired assimilation of fats and lipid-soluble vitamins A, D, K, E, phenomena associated with their deficiency develop - hepatic rickets, keratomalacia, etc. The bowel movements are usually copious, light-colored and very foul-smelling. . They lack bile pigments, and the test for stercobilin is most often negative. With incomplete obstruction, the discoloration of stool becomes intermittent. Sometimes, however, the surface of the stool has a light yellow coloration, which can be misleading. On a cut, such feces are aholic. They acquire a light yellow color due to bilirubin secreted by the intestinal glands and intestinal mucosa, which enters the feces through diapedesis. Such pathological permeability of the intestinal wall exists only in the first months. Urine color ranges from dark amber to dark green. The diapers are colored yellow-brown. The test for bilirubin in the urine is positive, urobilin and urobilinogen are absent. There are almost always traces of albumin in the urine. Tears and cerebrospinal fluid intense yellow color. The content of bilirubin, mainly direct, in the blood serum is sharply increased. Content total bilirubin may reach 856 µmol/l, but is never observed kernicterus. The content of cholesterol and lipids in the blood increases. The amino acid content is increased, the ammonia content is low. Level alkaline phosphatase in blood serum is increased or increases intermittently. The galactose test and other liver tests (McLagan, Veltman, etc.) remain negative for a long time and later become positive. Due to impaired liver function, serum transaminase is gradually activated, and its content can reach 700 U. Biliary atresia is also characterized by a significant increase in the content of glutamate dehydrogenase in the blood serum (A. Dobronravov). With biliary atresia, SGOT increases slowly and remains constant at the same high level. IN peripheral blood slight anemia is noted; no changes were noted in the white blood. ESR, as a rule, is not accelerated and in severe liver damage can be 0 mm. Bleeding time and blood clotting time, prothrombin time are initially normal, later they increase. Bleeding from the stump may be observed in the first 2-3 weeks umbilical cord, melena, skin hemorrhages, most likely associated with transient hypothrombinemia. The liver is initially enlarged, its surface is smooth with a hard, sharp edge. Without treatment it gradually develops biliary cirrhosis, the liver shrinks, its surface becomes uneven, lumpy, the spleen enlarges, the abdomen swells, and ascites appears. Typically, children die by the age of 5-6 months due to symptoms of liver failure. Death may occur earlier from any intercurrent disease. Neurological phenomena are usually absent.

Diagnosis does not present any difficulty unless the possibility of biliary atresia is excluded. Early jaundice, direct bilirubinemia, hypercholesterolemia, increased content alkaline phosphatase, etc. - the main indicators that help clarify the diagnosis.

Differential diagnosis. Most often, diagnostic errors are caused by insufficient differentiation with icterus neonatorum prolongatus, which is observed mainly in premature infants and is associated with liver immaturity. If with physiological jaundice icterus disappears on average after 2 weeks, then with prolonged jaundice it can last 3-4 or more weeks. The stools, however, are not acholic, the urine is light and does not contain bilirubin.

Differential diagnosis should be made with the following diseases: 1. icterus gravis with hemolytic disease in newborns. Jaundice appears on the first day, quickly intensifies, but sometimes has an intermittent character. Skin color is yellow-red. The stools are normally colored. There is no bilirubin in the urine, urobilinogen is positive. Significant hemolysis can lead to an increase in the amount of bile pigments and to the formation of bile thrombi and bile duct obstruction. In such cases, acholic bowel movements are possible. The liver and spleen are enlarged from the very beginning. Anamnestic data on Rh incompatibility, positive test Coombs, anemia with erythroblastosis facilitates the diagnosis. 2. Sepsis. Jaundice usually appears in the second week. The child has a high fever, weakness, reluctance to suckle, and refuses to breastfeed. Petechial or other rashes often appear on the skin. The bowel movements are not aholic. Urine color is dark. There is a shift in peripheral blood leukocyte formula to the left and anemia. ESR is usually accelerated. The content of bilirubin in the blood is normal or slightly increased. The spleen is enlarged. 3. Congenital syphilis. Jaundice is mild. The color of the urine is straw-yellow, the stool is normally colored. General state slightly disturbed. A child is usually born with a stuffy nose. Cracks on lips skin changes, enlarged liver and spleen from the very beginning, positive reaction Wasserman, etc. - they speak against the presence of biliary atresia. 4. Congenital malaria. This disease is extremely rare. The child is born healthy, and 7-10 days after birth a high temperature appears; he quickly weakens, anemia develops. Jaundice is very mild. 3 peripheral blood plasmodia are detected. 5. Infectious hepatitis. The child has a high temperature, increasing weakness, and vomiting. It often turns out from the anamnesis that the mother suffered from hepatitis. Urine here is dark, and feces are light. In peripheral blood, leukopenia and mild anemia are most often observed. Liver flocculation tests are strongly positive. SGOT for hepatitis is very high at the very beginning. After trial therapy with prednisone at 2-3 mg per kg of body weight per day, jaundice decreases, the bilirubin content in the blood serum decreases, which is not observed with biliary atresia. Differential diagnosis for these two diseases is often very difficult. Differences in the clinical picture of biliary atresia and congenital hepatitis are not always reliable criteria for differential diagnosis. During laparoscopic studies, histomorphological changes in both cases are of a similar nature and are not sufficient to distinguish between these diseases. IN in doubtful cases When the level of bilirubin constantly increases and urobilinogen is not found in the urine, laparoscopy with puncture biopsy is indicated to clarify the diagnosis, but not a very traumatic laparotomy with cholangiography, which is dangerous in case of hepatitis. New reports from Lawson and Boggs show that surgical exploration does not worsen the prognosis of non-familial hepatitis, which, in their opinion, is useful for early diagnosis. 6. Galactosemia. Except early jaundice direct type, the presence of galactosuria is important for diagnosis ( positive test for sugar in the urine), the subsequent appearance of cataracts, renal dysfunction (albuminuria, aminoaciduria), enlarged liver, spleen, etc. 7. Cytomegaly. The clinical picture has much in common with the clinical picture of hemolytic disease. Jaundice appears immediately after birth, the spleen and liver are enlarged and, most importantly, there are hemorrhages in the skin. 8. Toxoplasmosis. Jaundice appears after the third day and is mild. The presence of micro- or hydrocephalus and various disorders from the side of the eyes. 9. Listeriosis. The presence of jaundice is not a mandatory symptom here. Children are born with fever, convulsions, etc. In the mother's history, there is evidence of observed last days pregnancy phenomena of pyelitis, etc. Any jaundice in a newborn, lasting more than 15 days, without a tendency to decrease, should always raise suspicion about the presence of an anomaly of the biliary tract. Early diagnosis and early surgery sometimes they can save a child.

Treatment- purely surgical. The purpose of the operation is to create an outflow of bile into digestive tract. This is possible on average in 20% of cases. The most favorable moment for surgery is the end of the first and beginning of the second month of the child’s life, i.e., irreversible changes in the liver have not yet occurred. Yu. F. Isakov and S. Ya. Doletsky also recommend taking surgical treatment between 1st and 2nd month. Later, cirrhosis of the liver develops and the likelihood of recovery is very low. Grob believes that even if cirrhosis sets in, the child should be operated on, since at this age the regenerative capabilities are very great. Only children with atresia of the extrahepatic biliary tract are subject to surgical intervention. It is very important to first rule out hepatitis, since any surgery and anesthesia for hepatitis worsen the condition of the affected liver. Surgery for hepatitis will inevitably lead to death of the child. With this in mind, a trial laparatomy should be undertaken no earlier than the 3rd month. If we are talking about hepatitis, then by this point there may be full recovery and then the operation becomes unnecessary.
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Biliary atresia, or biliary atresia, is congenital deficiency, which is expressed in blocking the bile ducts of the liver.

Bile is a liquid mixture of cholesterol, bile salts and waste products, including bilirubin, which are excreted by the liver through thousands of small bile ducts into the intestines, where bile is involved in digestive processes. Small channels lead into larger ducts, resembling a city pipeline, and end in the duodenum (part small intestine). Between the liver and duodenum, this canal has a side branch connected to the gallbladder.

Biliary atresia - description

When bile cannot be excreted because the bile ducts are blocked, it accumulates in the liver and eventually travels to the rest of the body. The main pigment in bile, a chemical called bilirubin, is a breakdown product of hemoglobin. If there is excess bilirubin in the body, jaundice develops. Biliary atresia causes brown-colored urine and dark-colored stools. Excess bile in the liver damages the cells of this organ, causing cirrhosis. Scarring of the liver can cause increased pressure in the liver portal vein, which is the main vein supplying blood from the intestines to the liver. Venous hypertension can lead to damage to the veins of the intestinal mucosa, stomach, esophagus, and bleeding of these organs. This condition requires emergency medical attention.

Biliary atresia is the most common fatal congenital liver disease in children, with girls being affected slightly more often than boys. The incidence of biliary atresia is highest in children in Asian countries.

Causes and symptoms of biliary atresia

The causes of biliary atresia are unknown. However, there are indications that viral infections or autoimmune mechanisms. About ten percent of children with biliary atresia also have birth defects blood vessels, heart, spleen or intestines.

A child with biliary atresia appears normal at birth and during the subsequent postperinatal period. After about two to three weeks, the sick child develops jaundice. His eyes and skin turn yellow, his urine becomes dark yellow or brown due to an increase in bilirubin levels, his stools become dark color. The abdomen enlarges due to the expansion of the liver, the child looks very bad in appearance. Weight loss and irritability increase as the effects of jaundice increase. Some babies may experience intense itching. Almost all children without treatment die from liver failure within two years.

When to see a doctor for biliary atresia

The doctor should examine the child if he has jaundice after two weeks or has other symptoms. characteristic symptoms biliary atresia.

You should consult a doctor if, after surgery to treat biliary atresia, the baby still has jaundice, high temperature for more than 24 hours, or changes in the color of stool or urine. Additionally, your baby may experience abnormal fluid accumulation in the abdomen after surgery, so you should consult your doctor if your baby's stomach is enlarged.

If a child has dark-colored stools, is pale, and is vomiting blood due to the development of portal hypertension, emergency medical attention is also required.

Biliary atresia - diagnosis

The persistence or development of jaundice in the second week in a newborn indicates an obstruction to the bile flow. Immediate evaluation, which includes blood tests and imaging biliary system using ultrasound, specialized x-ray techniques or radiological studies are essential to confirm the diagnosis. Other liver diseases that cause symptoms similar to those of bile duct atresia should be excluded during testing. In addition, in most cases, for staging final diagnosis A liver biopsy or surgical exploration of the infant's abdomen may be necessary.

Biliary atresia - treatment

Surgery is the only treatment for biliary atresia. The surgeon creates an adequate path for bile. Changes in the anatomy of the biliary system are different in each specific case, and require the skill and experience of the surgeon to select and perform the most effective option.

A possible complication after surgery for bile duct atresia is a bile duct infection (cholangitis) and should be treated immediately with intravenous antibiotics. If a child develops ascites (an abnormal accumulation of fluid in the abdomen), treatment consists of medications and diet changes to reduce salt and fluid intake.

Surgeries for bile duct atresia have the best results in infants under eight weeks of age. However, in many cases, liver damage may continue to occur, and without further intervention, cirrhosis and associated complications may develop. In certain situations, a liver transplant is required. Improved methods of its transplantation allow surgeons to work with children of any age, and modern medicines– overcome the problems of organ rejection.

Problems, prognosis and prevention

After surgery for bile duct atresia, the child requires a low-fat diet with additional vitamins. Postoperative breastfeeding is recommended whenever possible, as breast milk contains lipases and bile salts, which are actively involved in digestion. Infants on artificial feeding should be given special compounds containing chemical substances to improve the digestion of dietary fats. Additional calories may be needed to help your child gain weight faster.

Early diagnosis of biliary atresia has important, since few children survive past two years without treatment for the condition. If the operation is performed before two months of age, success is much more likely, while after three months, it is much less likely. Unfortunately, for many children, surgery is not a panacea, and complications of liver cirrhosis can develop gradually. Ultimately, these children require a liver transplant.

Because specific reason bile duct atresia is unknown, there is no way to prevent it congenital disease. Biliary atresia, however, is not a hereditary disease.

Denial of responsibility: The information presented in this article about assessing the risks of anesthesia is intended to inform the reader only. It is not intended to be a substitute for advice from a healthcare professional.

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Congenital biliary atresia

Biliary atresia refers to severe congenital malformations inside the womb. The pathology is accompanied by complete or segmental fusion of the bile ducts. The disease is detected in early age, is rare. Only 8% of all organ malformations occur at the artesia. The pathology is dangerous, so it is important to identify this deficiency early and surgically correct it. If timely assistance will not be 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.

The disease is a congenital defect.

Description of the pathology

Biliary atresia is characterized by two conditions:

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

In both cases, there is underdevelopment or complete absence of the 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 sections of the extrahepatic excretory canals;
• individual sections of the ductal network;
• only channels in the liver.

In rare cases, biliary atresia occurs alone. In most cases, against the background of pathology, there are deformations of the cranial bones and spine, pathologies of the excretory system, blood vessels and heart.

Causes

The factors that provoke pathology of the bile ducts have not been fully studied. Most patients with atresia have formed bile ducts, but bile cannot be removed from them due to severe narrowing of the lumen or progressive destruction of the duct 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 the mother to the child during pregnancy. Such viruses include cytomegaly, herpes, rubella, and neonatal hepatitis.

The ducts are affected by an infection that is transmitted from mother to child during pregnancy.

In rare cases, biliary atresia caused by blockage of the canals is detected. This trend may be caused congenital anomalies development or appear in the peripartum period from the 28th week of pregnancy against the background of progressive inflammation.

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

In 20% of cases, atresia of the bile ducts is accompanied by developmental anomalies of other organs and systems, for example, the heart (congenital defects), the gastrointestinal tract (incomplete bend of the intestine), immune system(absence or presence of more than one spleen).

Classification

There are several parameters according to which atresia is divided into subtypes:

• By location:
  • extrahepatic tissues;
  • inside the liver;
  • mixed type.
• Based on the location of the narrowing sites, it is divided into:
  • correctable pathology when the patency of the main hepatic or bile canal is impaired;
  • an uncorrectable 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 influencing the choice of surgical technique.

Symptoms

Often the disease is not detected, but the child is discharged from the maternity hospital with “protracted jaundice.”

The first manifestations of bile duct atresia are detected during the first 6 weeks of a child’s life. If all anthropometric indicators are normal in a newborn, slight jaundice is attributed to the natural physiological adaptation of the small organism. Therefore, such children are often discharged from the maternity hospital with a diagnosis of “protracted physiological jaundice.”

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

• no appetite;
• body weight decreases;
• physical development is delayed;
• there is a sharp loss of strength;
• develops muscle weakness with a decrease or cessation of physical activity.

Obstructive jaundice worsens and causes constant itching, because of which the child constantly cries. Flat, benign yellow bumps appear on the skin - lipid deposits. By the sixth month of the baby's life:

• biliary cirrhosis of liver tissue develops;
• the pressure on the main portal vein, which connects the bloodstream of the intestine and liver, increases;
• the abdominal organs fill with free fluid;
• pinpoint hemorrhages appear on the skin, bleeding of the mucous membranes and esophagus;
• the transmission of impulses from the brain and spinal cord to various organs is disrupted.

Due to a deficiency of vitamins, rickets and neuromuscular disorders appear, and the fragility of blood vessels increases, which causes bouts of bloody vomiting.

Diagnostics

Diagnosis may include surgical and instrumental methods.

• Examination by a pediatrician or neonatologist. The diagnosis is made based on symptoms of increasing congenital jaundice.
• Blood, urine and stool analysis. Shows high concentration bound bilirubin in urine, no detection of stercobilin in feces. A blood test diagnoses hypoalbuminemia and hypoproteinemia.
• Instrumental research methods: ultrasound.
• Surgical diagnostic methods: contrast cholangiography and cholecystitography.

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

• hypothyroidism;
• bile thickening syndrome;
• genetic metabolic disorders;
• medicinal lesions of liver tissue.

Treatment

Operation is the only thing possible treatment diseases.

Atresia in young patients can only be cured surgical method. In this case, the operation should be performed before the child is 3 months old. Otherwise, irreversible destruction occurs in other organs and systems. child's body. In such cases, the operation will be unsuccessful.

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

• surgery with the application of choledochoenteroanastomosis with the interintestinal anastomosis and turning off the afferent loop;
• a method of forming a bypass hepatic-intestinal anastomosis between the hepaticocholedochus and the jejunum with the imposition of hepaticojejunostomy.

With complete atresia of the bile ducts of extrahepatic tissues, an anastomosis (natural connection) between the hepatic gates and small intestine- portoenterostomy according to Kasai. In severe cases of gastrointestinal bleeding with increased pressure on the portal vein, a portacaval shunt is performed (drainage 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 considered.

Forecasts

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 tissue. Without surgery, children with atresia do not survive beyond 1 year of age. They die from acute liver failure heavy bleeding and intercurrent (aggravating pathology) diseases.

During surgery on early stages The treatment outcome is favorable. If the operation is performed before 2 months of the child’s life, the chance of survival for the next 10 years is 70% or higher. After the operation in 2-3 one month old the number of survivors is 27%. If surgical treatment is applied to older children, only 11% survive.

Inflammation of the bile ducts reduces the chances of recovery for young patients. The method of surgical intervention developed by Kasai - portoenterostomy - can reduce the risk of developing complications.

Therefore, to achieve a favorable outcome in the treatment of atresia, it is important to promptly examine the newborn if an abnormality of the bile ducts is suspected. In cases where the disease cannot be cured by surgery, surgery using the Kasai method gives a long-term positive effect. With hepatocholedochoenterostomy, a beneficial effect is also possible, but it is short-lived. Thanks to the drainage of the bile canals and the removal of excess pressure from the walls, the development of cirrhosis of the liver tissue is prevented. Wherein normal development and the child’s growth does not stop until the liver transplant operation.

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Biliary atresia, or Biliary atresia in children

Biliary atresia, or biliary atresia, is a congenital deficiency that results in blockage of the bile ducts of the liver.

Bile is a liquid mixture of cholesterol, bile salts and waste products, including bilirubin, that are excreted by the liver through thousands of small bile ducts into the intestines, where bile is involved in digestive processes. Small channels lead into larger ducts, resembling a city pipeline, and end in the duodenum (part of the small intestine). Between the liver and duodenum, this canal has a side branch connected to the gallbladder.

Biliary atresia - description

When bile cannot be excreted because the bile ducts are blocked, it accumulates in the liver and eventually travels to the rest of the body. The main pigment in bile, a chemical called bilirubin, is a breakdown product of hemoglobin. If there is excess bilirubin in the body, jaundice develops. Biliary atresia causes brown-colored urine and dark-colored stools. Excess bile in the liver leads to damage to the cells of this organ, causing cirrhosis. Scarring of the liver can cause increased pressure in the portal vein, which is the main vein that supplies blood from the intestines to the liver. Venous hypertension can lead to damage to the veins of the intestinal mucosa, stomach, esophagus, and bleeding of these organs. This condition requires emergency medical attention.

Biliary atresia is the most common fatal congenital liver disease in children, with girls being affected slightly more often than boys. The incidence of biliary atresia is highest in children in Asian countries.

Causes and symptoms of biliary atresia

The causes of biliary atresia are unknown. However, there are indications that viral infections or autoimmune mechanisms may be responsible for the development of biliary atresia. About ten percent of children with biliary atresia also have birth defects of the blood vessels, heart, spleen, or intestines.

A child with biliary atresia appears normal at birth and during the subsequent postperinatal period. After about two to three weeks, the sick child develops jaundice. His eyes and skin turn yellow, his urine becomes dark yellow or brown due to an increase in bilirubin levels, and his stools are dark in color. The abdomen enlarges due to the expansion of the liver, the child looks very bad in appearance. Weight loss and irritability increase as the effects of jaundice increase. Some babies may experience intense itching. Almost all children without treatment die from liver failure within two years.

When to see a doctor for biliary atresia

The doctor should examine the child if he has jaundice or other characteristic symptoms of biliary atresia after two weeks.

You should see a doctor if, after surgery to treat biliary atresia, the baby still has jaundice, a high fever for more than 24 hours, or changes in the color of stool or urine. Additionally, your baby may experience abnormal fluid accumulation in the abdomen after surgery, so you should consult your doctor if your baby's stomach is enlarged.

If a child has dark-colored stools, is pale, and is vomiting blood due to the development of portal hypertension, emergency medical attention is also required.

Biliary atresia - diagnosis

The persistence or development of jaundice in the second week in a newborn indicates an obstruction to the bile flow. Immediate evaluation, which includes blood tests and imaging of the biliary system with ultrasound, specialized x-rays or radiological studies, is essential to confirm the diagnosis. Other liver diseases that cause symptoms similar to those of bile duct atresia should be excluded during testing. In addition, in most cases, a liver biopsy or surgical exploration of the infant's abdomen may be necessary to make a definitive diagnosis.

Biliary atresia - treatment

Surgery is the only treatment for biliary atresia. The surgeon creates an adequate path for bile. Changes in the anatomy of the biliary system are different in each specific case, and require the skill and experience of the surgeon to select and perform the most effective option.

A possible complication after surgery for bile duct atresia is a bile duct infection (cholangitis) and should be treated immediately with intravenous antibiotics. If a child develops ascites (an abnormal accumulation of fluid in the abdomen), treatment consists of medications and diet changes to reduce salt and fluid intake.

Surgeries for bile duct atresia have the best results in infants under eight weeks of age. However, in many cases, liver damage may continue to occur, and without further intervention, cirrhosis and associated complications may develop. In certain situations, a liver transplant is required. Improved methods of transplantation allow surgeons to work with children of any age, and modern medicines help overcome the problems of organ rejection.

Problems, prognosis and prevention

After surgery for bile duct atresia, the child requires a low-fat diet with additional vitamins. Postoperative breastfeeding is recommended whenever possible because breast milk contains lipases and bile salts, which are actively involved in digestion. Formula-fed babies should be given special formulations containing chemicals to improve the digestion of dietary fats. Additional calories may be needed to help your child gain weight faster.

Early diagnosis of biliary atresia is important because few children survive beyond the age of two without treatment for the condition. If the operation is performed before two months of age, success is much more likely, while after three months, it is much less likely. Unfortunately, for many children, surgery is not a panacea, and complications of liver cirrhosis can develop gradually. Ultimately, these children require a liver transplant.

Since the specific cause of bile duct atresia is unknown, there is no way to prevent this congenital disease. Biliary atresia, however, is not a hereditary disease.

Disclaimer: The information presented in this article on anesthesia risk assessment is intended for the reader's information only. It is not intended to be a substitute for advice from a healthcare professional.

moskovskaya-medicina.ru

Biliary atresia in newborns

It happens that the birth of a long-awaited baby is overshadowed by the realization that the child is not entirely healthy. In 1 case out of 20-30 thousand, parents will be told that the newborn has biliary atresia. This means that if a baby has a developmental defect, there is an 8% chance that it is this particular disease.

Essence of the disease

This disease consists in the fact that the baby’s bile ducts are obstructed. This can happen inside the liver or outside it. This developmental anomaly is extremely dangerous and it is extremely important that it is diagnosed promptly. In this case, the child will be urgently operated on and will have a chance to prolong his life. There are often cases when, after these years, a liver transplant is still required, but most children do without it.

If the operation is not performed soon after birth, the baby will not survive beyond the first months of life. The cause of death is esophageal bleeding, or liver failure, or a complication after an infection. These are the types of cases that make home births, which are fashionable these days, extremely dangerous. They bring it back to the previous level natural selection, which doctors are already fighting quite successfully.

Causes of the disease

Doctors do not yet know for sure the reason why newborns are diagnosed with such a defect as biliary atresia. In rare cases, it occurs that during the embryonic period even the laying of these bile conductors did not occur. This form of the disease is called true.

Much more often, the bile ducts are still present. But for one reason or another they are impassable. This can be either progressive destruction or obliteration. The formation of bile ducts occurs in the early stages of embryonic development. This means that if future mom During this period, they will suffer a disease that is dangerous for them, and a defect may develop. Doctors' list of such diseases includes: rubella, cytomegaly, hepatitis and herpes. These diseases give rise to an inflammatory process that negatively affects hepatocytes and the endothelium of the bile ducts. And they further influence the fact that the bile ducts seem to stick together inside and do not allow bile to pass its path.

At the same time, in order to carry out early operations, doctors need to take into account that such a defect is found in about 15% of cases together with some other developmental defect. These may be congenital heart defects, incomplete intestinal rotation, asplenia or polysplenia.

Symptoms

A child with atresia is born absolutely normal, with a normal weight and at the right time. The first days everything goes exactly the same as for everyone else, but on the 3-4th day jaundice appears. It is initially confused with physiological jaundice, which is diagnosed in many infants and goes away quite quickly. Mother and baby may even be discharged from the maternity hospital without paying attention to this phenomenon. And here the attention of the mother and close relatives is already necessary in order to notice in time that the jaundice does not go away, but only intensifies. And the child soon even takes on a saffron or greenish tint.

If we add to this the fact that the newborn has almost colorless feces and urine of an intense beer shade, then this is already serious symptom and a reason for an urgent visit to the pediatrician. If the stool does not change color for 10 days, then this directly indicates that the baby has biliary atresia.

By the time you celebrate the first month of life, you can already notice the first results of the disease. The child lags behind in weight gain, does not move as actively as his peers, is reluctant to eat and experiences itchy skin. By 4-5 months you can already expect liver failure. Children with atresia have small or large hemorrhages on the skin, the so-called hemorrhagic syndrome. Blood is flowing and from the navel, and also happen internal bleeding, from the esophagus or gastrointestinal tract.

If the parents did not consult a doctor and the operation was not performed, the child most likely will not celebrate his first birthday. And the sooner help is provided, the greater the chance of survival. Every month the hope for a successful outcome decreases.

Diagnostics

The ideal option for the child and his parents is when the disease is detected in the maternity hospital or during patronage in the first weeks of life. The symptoms that appear should prompt the need for additional examination.

The biochemical blood test undergoes a change in the very first days of the baby’s life. Liver markers increase their values ​​literally before our eyes. First of all, the indicators of bilirubin, alkaline phosphatase, and gamma-glutamyl transferase are alarming. In addition, there is a drop in hemoglobin, which indicates progressive anemia, and the absence of stercobilin in the stool.

The final answer about the presence of a congenital defect can be given by ultrasound of the liver, spleen and bile ducts, as well as some other, less common research methods, such as, for example, MR cholangiography, static scintigraphy liver and some others. In order to finally make sure, a liver biopsy or even a laparoscopic diagnostic examination may be prescribed.

All these examinations are necessary in order not to confuse atresia with the already mentioned jaundice of newborns or blockage of the bile ducts with plugs. Traffic jams can form from mucus or bile.

Treatment

As has been said many times before, it is important to carry out surgical intervention at a time when the baby has not yet reached 2-3 months. Otherwise, the changes that occur in the body due to pathology become irreversible and the use of surgery simply does not make sense. After such a conditional threshold - 3 months, the liver is already in a state of cirrhosis, which significantly complicates treatment and reduces survival prognosis to almost zero.

The most commonly performed operation is named after the doctor who first proposed it, the Japanese scientist Kasai. It created a connection between the intestines and the liver. In other cases, they resort to liver transplantation, but it is advisable to perform this operation in older children. Therefore, primary operations are carried out and the condition is monitored. little patient in order to intervene in his body again, if necessary, for more radical method. Very often it is possible to postpone transplantation for several years.

Help Wanted. Biliary atresia

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medistoriya.ru

Biliary atresia, Medical site for doctors and patients

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Source: babysovet.ru

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