Congenital anomalies of the biliary tract: causes, symptoms, diagnosis, treatment

Most congenital anomalies of the biliary tract can be associated with a violation of the initial budding from the primary anterior gut or with a violation of the reopening of the lumen of a dense gallbladder and a cholangic diverticulum.

The liver and the bile ducts are formed from the kidney-like outgrowth of the ventral wall of the primary anterior colon, located cranial to the yolk sac. Of the two dense cell shoots, the right and left lobes of the liver are formed, and the liver and general bile ducts are formed from the elongated diverticulum. The gallbladder is formed from a smaller cell cluster in the same diverticulum. Already in the early stages of intrauterine development, the bile ducts are passable, but subsequently the proliferating epithelium closes their lumen. Over time, there is a reopening of the lumen, which starts simultaneously in different parts of the dense rudiment of the gallbladder and gradually spreads to all the bile ducts. By the 5th week, the formation of the bladder, general bile duct and hepatic ducts is completed, and by the 3rd month of the intrauterine period, the fetal liver begins to secrete bile.

[1], [2], [3], [4], [5]

Classification of congenital anomalies of bile ducts

Anomalies of the outgrowth of the primary anterior bowel

• Lack of growth
• Absence of bile ducts
• The absence of the gallbladder
• Additional outgrowth or cleavage of the growth
• Additional gallbladder
• Dicotyledonary gallbladder
• Additional bile ducts
• Migrating the outgrowth to the left (normal to the right)
• The left-sided arrangement of the gallbladder

Anomalies of lumen formation from a dense gallstick

• Violation of the formation of the lumen of bile ducts
• Congenital obliteration of bile ducts
• Congenital obliteration of the cystic duct
• Choledocha cyst
• Violation of the formation of the gallbladder lumen
• Rudimentary gallbladder
• Diverticulum of the bottom of the bladder
• Serous type of "Phrygian cap"
• Gallbladder in the form of an hourglass

Preservation of bladder-hepatic duct

• Diverticulum of the body or cervix of the gallbladder

Preservation of intrahepatic gallbladder

Anomalies of the bookmark of the rudiment of the gallbladder

• Retroserous type of "Phrygian cap"

Additional folds of peritoneum

• Congenital adhesions
• Wandering gallbladder

Anomalies of the hepatic and pancreatic arteries

• Additional arteries
• Abnormal location of the hepatic artery relative to the cystic duct

These congenital anomalies usually have no clinical significance. Sometimes biliary anomalies cause stagnation of bile, inflammation and the formation of gallstones. This is important for radiologists and surgeons operating on the biliary tract or performing liver transplantation.

Anomalies of biliary tract and liver can be combined with other congenital anomalies, including heart disease, polydactyly and polycystic kidney disease. The development of biliary tract abnormalities can be associated with viral infections in the mother, for example, with rubella.

[6], [7], [8], [9]

The absence of the gallbladder

There are two types of this rare congenital anomaly.

Anomalies of type I are associated with a violation of the escape of the gallbladder and the bladder duct from the hepatic diverticulum of the anterior gut. These anomalies are often combined with other anomalies of the biliary system.

Anomalies of type II are associated with impaired lumen formation in the dense rudiment of the gallbladder. They are usually combined with atresia of extrahepatic bile ducts. The gall bladder is present, only in a rudimentary state. These anomalies are detected in infants with signs of congenital atresia of the biliary tract.

In most cases, these children have other serious congenital anomalies. Adults usually do not have other anomalies. In some cases, pain is possible in the upper right quadrant of the abdomen or jaundice. The inability to detect a gallbladder with ultrasound is sometimes regarded as a disease of the gallbladder and sends the patient to the operation. The doctor should be aware of the possibility of agenesis or ectopic localization of the gallbladder. Cholangiography is the most important for establishing the diagnosis. The non-appearance of the gall bladder during the operation can not serve as a proof of his absence. The gallbladder can be located inside the liver, hide in pronounced adhesions, atrophy due to cholecystitis.

Intraoperative cholangiography should be performed.

Double gallbladder

A double gallbladder is very rare. When embryonic development in the hepatic or general bile duct, small pockets are often formed. Sometimes they persist and form a second gallbladder, having its own cystic duct, which can pass directly through the liver tissue. If the pocket is formed from the vesicular duct, the two gallbladder have a common Y-shaped bladder duct.

A double gallbladder can be identified using various imaging techniques. In the additional organ, pathological processes often develop.

A bipartite gallbladder is an extremely rare congenital anomaly. In the embryonic period there is a doubling of the rudiment of the gallbladder, but the original compound is preserved and two separate independent bladder with a common bladder duct are formed.

The anomaly has no clinical significance.

[10], [11], [12], [13], [14], [15], [16], [17]

Additional bile ducts

Additional bile ducts are rare. The additional duct usually refers to the right parts of the liver and is connected to the common hepatic duct on the interval between its beginning and the place of the confluence of the bladder duct. However, it can be connected to the bladder duct, gall bladder or common bile duct.

The bladder-hepatic ducts are formed due to the preservation of the existing fetal junction of the gallbladder with the liver parenchyma in violation of the recanalization of the lumen of the right and left hepatic ducts. Outflow of bile is provided by the bladder duct, which flows directly into the stored hepatic or general hepatic duct or into the duodenum.

The presence of additional ducts must be taken into account in operations on the biliary tract and liver transplantation, because with accidental bandaging or crossing, strictures or fistulas may develop.

[18]

The left-sided arrangement of the gallbladder

With this rare anomaly, the gallbladder is located under the left lobe of the liver to the left of the crescent ligament. It is formed when the rudiment from the hepatic diverticula migrates to the left in the embryonic period. At the same time, an independent formation of the second gallbladder from the left hepatic duct is possible if the development or regression of a normally located gallbladder is disturbed.

With the transposition of the internal organs, the normal interposition of the gallbladder and liver located in the left side of the abdomen is preserved.

The left-sided arrangement of the gallbladder is of no clinical significance.

Sinuses of Rokytansky-Ashoff

Sinuses of Rokitansky-Ashoff are hernial protrusions of the mucous membrane of the gallbladder through the muscular layer (intramural diverticulosis), which are especially pronounced in chronic cholecystitis, when the pressure in the lumen of the bladder rises. With oral cholecystography, the sinuses of Rokitansky-Ashoff resemble a wreath around the gallbladder.

Folded gall bladder

The gallbladder, as a result of a sharp inflection in the bottom, deforms in such a way that it resembles the so-called Phrygian cap.

The Phrygian cap is a conical cap or cap with a curved or anteriorly inclined top that was worn by the ancient Phrygians; it is called the "cap of freedom" (Oxford Dictionary of English). There are two varieties of anomalies.

1. And the bend between the body and the bottom is a retrograde "Phrygian cap". Its cause is the formation of an abnormal fold of the gallbladder inside the fetal fossa.
2. The bend between the body and the funnel is a serous "Phrygian cap". Its cause is the anomalous curve of the pit itself in the early stages of development. The bending of the gallbladder is fixed by fetal ligaments or residual septa formed as a result of the delay in the formation of the lumen in the dense epithelial rudiment of the gallbladder.

Emptying of the folded gallbladder is not violated, so the anomaly is not of clinical significance. You should know about it in order to correctly interpret the data of cholecystography.

Gallbladder in the form of an hourglass. Probably, this anomaly is a kind of "Phrygian cap", presumably serous, only more pronounced. The constancy of the bottom position during contraction and the small size of the communication between the two parts of the gallbladder indicate that this is a fixed congenital anomaly.

[19], [20], [21], [22]

Diverticula of gallbladder and ducts

Diverticula of the body and neck can come from the remaining vesicoureteral ducts, which normally in the embryonic period connect the gallbladder with the liver.

Diverticula of the bottom are formed with incomplete re-formation of the lumen in the dense epithelial rudiment of the gallbladder. When pulling an incomplete septum in the region of the bottom of the gallbladder, a small cavity is formed.

These diverticula are rare and do not have clinical significance. Congenital diverticula should be distinguished from pseudodiverticles, which develop in diseases of the gallbladder as a result of its partial perforation. Pseudodiverticul in this case usually contains a large gallstone.

[23], [24], [25], [26]

Intrahepatic location of gallbladder

The gallbladder is normally surrounded by a tissue of the liver before the second month of intrauterine development; in the future he occupies a position outside the liver. In some cases, the intrahepatic location of the gallbladder can persist. The gallbladder is higher than normal, and more or less surrounded by a liver tissue, but not completely. It often develops pathological processes, as its contractions are difficult, which contributes to infection and the subsequent formation of gallstones.

Congenital spasms of the gallbladder

Congenital spasms of the gallbladder are very common. They are sheets of peritoneum that form during embryonic development when the anterior mesentery stretches, forming a small gland. Adhesions can pass from the common bile duct in the lateral direction above the gallbladder to the duodenum, to the hepatic bend of the colon and even to the right lobe of the liver, probably closing the gland opening (Wislov hole). With less pronounced changes, the spikes spread from the small omentum through the cystic duct and in front to the gallbladder or form a mesentery of the gallbladder (a "wandering" gallbladder).

These adhesions have no clinical significance. When surgical interventions, they should not be taken for inflammatory adhesions.

[27], [28], [29], [30], [31]

Wandering gallbladder and twist of the gallbladder

In 4-5% of cases, the gallbladder has a membrane supporting it. The peritoneum encloses the gallbladder and converges in the form of two leaves, forming a fold or mesentery, fixing the gallbladder to the lower surface of the liver. This fold can allow the gallbladder to "hang" 2-3 cm below the surface of the liver.

The mobile gallbladder can rotate, which leads to its twisting. At the same time, the blood supply of the bladder is disrupted, as a result of the infarction.

Torsion of the gallbladder usually occurs in lean older women. As the aging fatty layer of the omentum decreases, and a decrease in the tone of the muscles of the abdominal cavity and pelvis leads to a significant displacement of the abdominal cavity in the caudal direction. The gallbladder, having a mesentery, can be twisted. This complication can develop in any, including in childhood, age.

Torsion is manifested by sudden strong pains of a persistent nature in the epigastric region and right hypochondrium, radiating into the back and accompanied by vomiting and collapse. Palpable tumor-like formation resembling an enlarged gallbladder, which can disappear within a few hours. Cholecystectomy is indicated.

Relapses of incomplete torsion are accompanied by acute episodes of the symptoms described above. When an ultrasound or CT scan, the gallbladder is located in the lower part of the abdomen and even in the pelvic cavity, resting on a long, curved downstream bladder duct. Cholecystectomy is shown in early life.

Anomalies of the bladder duct and the cystic artery

In 20% of cases, the bladder duct connects to the common hepatic duct not immediately, being parallel to it in one connective tissue tunnel. Sometimes it spirals around the common hepatic duct.

This anomaly is very important for surgeons. Until the vesicular duct is carefully separated and the site of its connection to the common hepatic duct is identified, the risk of ligation of the common hepatic duct remains with catastrophic consequences.

Bubble artery can not depart from the right hepatic artery, as in the norm, but from the left liver or even from the gastroduodenal artery. Additional vesical arteries usually move away from the right hepatic artery. In this case, the surgeon should also be alert, highlighting the bladder artery.

[32], [33], [34], [35], [36], [37]

Benign biliary stricture

Benign bile duct strictures are rare, usually after surgery, especially after laparoscopic or "open" cholecystectomy. In addition, they can develop after liver transplantation, with primary sclerosing cholangitis, chronic pancreatitis and abdominal injuries.

Clinical signs of strictures of the bile ducts are cholestasis, which can be accompanied by sepsis, and pain. The diagnosis is made using cholangiography. In most cases, the cause of the disease can be established based on the clinical picture.

[38], [39], [40]