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Cysts of bilious channels - Diseases of digestive organs at children

Table of contents
Diseases of digestive organs at children
Oral cavity
Diseases of teeth
Malformations of the sky and soft tissues of an oral cavity
Diseases of a mucous membrane of an oral cavity and gums
Diseases of lips and language
Digestive tract
Basic reasons of gastrointestinal frustration
Atresia and esophageal and tracheal fistula
Guttural and tracheal and esophageal crevice, inborn stenosis of a gullet
Other diseases of a gullet
Stomach and intestines
Peptic ulcer
Inborn hypertrophic pyloric stenosis
Inborn impassability of intestines
Inborn impassability of a duodenum
Disturbances of turn of intestines
Inborn impassability of a small bowel
Inborn megacolon
Diverticulums and duplikatura
The acquired impassability of intestines
Intestines invagination
Foreign bodys of a stomach and intestines
Motive frustration. stomach and intestines
Anomalies of a structure of anorectal area
Infectious diseases of intestines
Nonspecific ulcer colitis
Illness Krone
Necrotic coloenteritis of newborns
The coloenteritis connected with treatment by antibiotics
Gastrointestinal symptoms of anaphylactoid purpura, gemolitiko-uraemic syndrome
Intolerance of food proteins
Eosinophilic gastroenteritis
Absorption disturbance syndromes
Immunodeficiency and intestines
Syndrome of "a congestive loop"
Syndrome of a short small bowel
Gee's disease
Sprue after acute enteritis
Tropical to a spr
Whipple's illness, intestines lymphangiectasia, Uolmap's illness, idiopathic diffusion defeat of mucous
Enzymopathies and disturbances of mechanisms of transport of nutrients
Irritable colon
Acute appendicitis
Diseases of an anus, direct and large intestine
Tumors of a digestive tract at children
Hernias of a digestive tract at children
Exocrine part of a pancreas
Embryonic development of structure and function of a liver
Diagnosis of diseases of a liver
Cholestatic states at babies
Parenchymatous jaundices at children of chest age
Disturbances of a metabolism of a liver and zhelchevydelitelny system
Anomalies of a structure of bilious ways
Cysts of bilious channels
Cholestasia and diseases of a liver connected with completely parenteral food
Medicinal damage of a liver
Ray's syndrome
Chronic hepatitis
Wilson's illness — Konovalova
Indian juvenile cirrhosis
Damages of a liver at chronic colitis
Cirrhosis and chronic liver failure
Portal hypertensia and varicosity of a gullet
Fatty infiltration of a liver
Peritoneum diseases
Peritoneum hernias

Cysts of bilious channels can arise in any part of a biliary tree; clinical manifestations depend mainly on their localization. Not reported (often solitary) cysts and cysts which are located in the field of terminal channels usually almost do not influence current of bile, a bessimptomna and do not demand treatment. Big solitary cysts are shown by symptoms of a prelum of surrounding fabrics, they are localized mainly in the right upper quadrant. They should be differentiated from a tumor, an echinococcal cyst and abscess. Excision or drainage of a cyst can sometimes be required.
Expansions of large intra hepatic and extrahepatic channels are followed by stagnation of bile and the progressing abnormal liver function. Many cystous formations of a biliary path have the general etiology. The same influence, but at different stages of an organogenesis or on different sites can lead to formation of a choledochal cyst, expansion of large intra hepatic channels (Karoli's illness), inborn fibrosis of a liver, a polycystosis of a liver and kidneys or to a combination of these anomalies. The scheme of cystous gepatobiliarny anomalies, their bonds with renal anomalies and the corresponding clinical aspects are presented on fig. 12-30.

Печень при гипоплазии внутрипеченочных желчных путей

Fig. 12-29. and. A liver at a hypoplasia of intra hepatic bilious ways. Bioptat is received at the age of 7 weeks at the child represented in fig. 28 at the age of 8 months. On the right: a hepatic segment with considerably expanded portal zone (P), the central vein (Ts) and elements of an erythrogenesis (arrow). X 60. At the left bigger increase in the fragment concluded in a frame is given, the epithelium of an interlobular channel is thickened, has degeneration signs. The channel is surrounded with concentric layers of connecting fabric I inflammatory cells (pericholangitis). X 200. Bioptat of a liver taken from the same child at the age of 2 flyings. On the right: the portal zone (the fragment concluded in a frame) is reduced (in comparison with and). There are no signs of an inflammation, a necrosis, fibrosis. The central vein (Ts) is visible on the right below. X 60. At the left: big increase in the fragment concluded in a frame. Hypoplasia and atrophy of bilious channels (PR), lack of elements of an inflammation. The branch of a portal vein (V) and hepatic arteriole (A) in a portal zone are deformed by star-shaped bunches of fibrous fabric (arrow)
X 200.


Cysts of extrahepatic bilious channels can have an appearance of diverticulums or spindle-shaped expansions of the general bilious channel, the general hepatic channel, a gall bladder or to look as to a holedokhotsela of an intra duodenal site of the general bilious canal. Most often cysts arise in free part of the general bilious canal. This type of cysts in 33 — 100% of cases is combined with merge of the general bilious and pancreatic channels before their falling into Oddi's sphincter. Owing to more high pressure in a pancreatic channel its contents can flow into the general bilious canal, causing its inflammation, a stenosis and an atony.
The cyst can remain asymptomatic until arises obturation of a bilious channel the stone formed in a cyst or the cholangitis will not develop. Passing impassability of a channel is followed by jaundice, pains and emergence of the palpated consolidation. Only at very few patients the cyst is distinguished aged till 1 year, and approximately in 50% of cases it is diagnosed for children after 10 flyings. The main symptoms — pains in right hypochondrium, vomiting and fever — are observed a long time (from 1 to 5 month). An important role in diagnosis of cysts at children of chest age is played by ultrasonic scanning, especially if the classical triad of symptoms — pain, jaundice, consolidation — is not expressed. The cyst can be suspected if there is long mechanical jaundice with rise in level of an alkaline phosphatase in serum to 200 — 300 ME or if find signs of the extrahepatic block of bilious ways in bioptata of a liver, and at duodenal sounding or in Calais find bilious pigments. The diagnosis is made on the basis of results of X-ray inspection of an upper part of a digestive tract, an intravenous holangiografiya or computer tomography.
Treatment consists in excision of a cyst or a holedokhokistoyeyunostomiya with a cholecystostomy. After operation the recurrent cholangitis, an anastomosis stricture, the progressing damage of a liver and impassability of intestines can develop.

bilious channel

Cystous expansion
bilious channels (Naroli's illness)

Inborn polycystosis of a liver and kidneys

Почки интантны

Кистозное расширение

Врожденный поликистоз печени и почек

Почки поражены

Intantna kidneys
The cysts connected with bilious channels the Cholestasia (jaundice)
The forecast is defined by a condition of a liver

Kidneys are affected
Cysts are usually not connected with bilious protons Portal hypertensia is not present the Cholestasia
The forecast is defined by a condition of kidneys

Fig. 12-30. Cystous formations of a liver and bilious ways subdivide into 2 main categories: 1) choledochal cysts and cystous expansions of intra hepatic channels (Karoli's illness), 2) inborn fibrosis of a liver and polycystosis of a liver and kidneys. Disturbances of the 1st category cause delay of current of bile in the main bilious ways that is followed by a chronic or recurrent cholestasia, a cholangitis and the progressing damage of a liver. The forecast depends on extent of defeat of tissue of liver and complications as kidneys at Karoli's illness are involved only in rare instances (the asterisk specifies that cases of an illness of Karoli at which expansion of renal tubules was combined with inborn fibrosis of a liver were observed). Disturbances of the second category have focal character, they or are not connected with the bilious courses, or limited to interlobular channels. The main signs of this anomaly are considerable development of fibrous fabric and close connection with a polycystosis of kidneys. Fibrous fabric is especially expressed at inborn fibrosis of a liver (it is designated on the scheme by the dark strokes surrounding cysts). At patients with inborn fibrosis, a polycystosis of a liver and kidneys of a cholestasia is not present, functions of a liver are not broken. Except for portal hypertensia at inborn fibrosis of a liver, the forecast is defined by renal displays of a disease. Allocate types I and III of a polycystosis of kidneys (classification of Potter). Type I — medullary ("spongy") kidneys, type III — the polycystic damage of kidneys which is found usually at adults.

Cystous expansion of intra hepatic bilious channels (Karoli's illness) is characterized by existence of the vishneobrazny cysts covered by a cubical epithelium; these cysts are continuation of primary branches of an intra hepatic biliary tree. Thus, among cysts of bilious channels Karoli's illness is intermediate between choledochal cysts and expansions of interlobular ductules and intra lobular capillaries at inborn fibrosis of a liver. The fact that these 3 diseases are associated with each other is caused by their general prenatal reason, and options reflect influence temporary, hereditary, and also teratogens.
The leading clinical displays of an illness of Karoli — gallstones in expansions of intra hepatic bilious channels, flashes of a recurrent cholangitis and abscessing. Usually children and young adults are ill, however the illness can be shown for the first time at any age. During the periods of an aggravation fever, a skin itch, slight jaundice, morbidity and insignificant increase in a liver, increase of level of serumal bilirubin, aminotransferases and an alkaline phosphatase are noted. The preliminary diagnosis is made on the basis of results of ultrasonic research, i.e. detection in a parenchyma of a liver of multiple consolidations. Finally the diagnosis can be made by means of a transdermal transhepatic holangiografiya. In bioptata it is possible to find the accompanying inborn fibrosis of a liver, and at an intravenous piyelografiya — expansion of uric ways.
Treatment consists in introduction of antibiotics, however they often do not give desirable effect. At defeat of one of hepatic lobes successful operations of a partial gepatektomiya were made. Attempts to delete stones from liver sites, adjacent to a surface, were made. The forecast is uncertain. Quite often liver abscess, sepsis, a cholangiocarcinoma and an amyloidosis is a cause of death.
Inborn fibrosis of a liver is inherited on autosomal recessively type. The illness is characterized by gamartomatozny changes including interlobular bilious channels. Portal triads are surrounded with dense accumulations of fibrous fabric in which the deformed structures covered by a biliary epithelium lie. The set of small cysts of the fibrous plaques surrounded with bunches and closely adjacent to a liver parenchyma is usually observed. Cystous educations are, as a rule, not connected with system of small bilious channels and do not break bile current, except for exceptional cases, not distinguishable from Karoli's illness. The parenchyma of a liver is not changed and does not contain inflammation elements.
The illness is usually diagnosed for children of advanced age when increase in a liver and spleen is found or there is gastrointestinal bleeding owing to portal hypertensia. Functional indicators of a liver are usually not changed or slightly deviate norm; at the same time the sharp thrombocytopenia caused by a hypersplenism is possible. In 60 — 80% of cases inborn fibrosis is combined with cystous expansions of collective tubules of the kidneys seen at an intravenous piyelografiya.
Treatment consists in the prevention of bleeding from varicose veins of a gullet. To liquidate thrombocytopenia) and to redeem normal situation of the bodies displaced by a big spleen, the splenectomy can be required. During a splenectomy create the splenorenalny shunt with the purpose to weaken portal hypertensia. The forecast is favorable if function of kidneys is not broken.
Liver polycystosis — rare frustration of an unknown etiology which differs from inborn fibrosis in lack of vyrazhenpy accumulations of connecting fabric in a liver parenchyma. Cysts vary from microscopic bubbles to the cavities having several centimeters in the diameter; they are not connected with a biliary path. The parenchyma of a liver is kept and functions it are not broken. At a palpation the liver is painless, but its surface can be uneven because of existence of big cysts under a capsule. Ultrasonic scanning and an axial computer tomography reveal the cavities filled with liquid in a liver and kidneys.
The polycystosis of kidneys in 50% of cases accompanies a liver polycystosis. At most of patients the symptomatology is defined by the disturbance of functions of kidneys reaching heavy, even lethal renal failure. Sometimes the rupture of a big cyst or the hemorrhage in a cyst demanding surgical intervention becomes the first display of an illness; however in most cases cysts of a bessimptomna also only accidentally are found on autopsy. Treatment is not required, cysts are not recommended to be deleted even if they are accidentally found at the laparotomy made in other occasion.

"Diseases of bodies of an urinary system at children   Diseases of the lacrimal bodies"