Choledochal cyst

Description:

The choledochal cyst represents its expansion. The gall bladder, a vesical channel and hepatic channels above a cyst are not expanded unlike strictures at which all biliary tree above a stricture extends. The combination of a choledochal cyst to Karoli's disease is possible. At a histologic research the wall of a cyst is presented by the fibrous fabric which is not containing an epithelium and unstriated muscles. At patients with a choledochal cyst anomaly of its merge to a pancreatic channel (a long general segment) is described. Assume that formation of a cyst is caused by a reflux of enzymes of a pancreas in bilious ways.
Choledochal cysts classify as follows:
Type I - segmented or diffusion spindle-shaped dilatation.
Type II - a diverticulum.
Type III - to the holedokhotsela of a distal part of the general bilious canal, preferential in a duodenum wall.
Type IV - the anatomic changes inherent to type I are combined with a cyst of intra hepatic bilious channels (IVa; the type was Punished) or with the holedokhotsel (IVB). If allocate type V, understand Karoli's disease as it.
Most often the spindle-shaped extrahepatic cyst (type I) following on frequency a form - a combination inside - and extrahepatic changes meets (IVa type). Still there is no consensus about whether the holedokhotsela should consider (type III) as a cyst.
The isolated clustery dilatation of intra hepatic bilious channels is occasionally observed.
The cyst like I comes to light as partially retroperitoneal cystous tumorous education which amount is subject to considerable fluctuations: from 2-3 cm to reaching the volume of 8 l. The cyst contains dark brown liquid. She is sterile, but can be infected for the second time. The cyst can be broken off.
At late stages the disease can be complicated by biliary cirrhosis. Cysts of a holedokh can squeeze a portal vein that leads to portal hypertensia. Development in a cyst or in bilious channels of malignant tumors is possible. The biliary papillomatosis with K-ras gene mutation is described.

Reasons of a choledochal cyst:

Frequency of a cyst of a holedokh very small in spite of the fact that it is about the most frequent reason of a cholestasia of an extrahepatic origin at the child. The disease is shown mainly at children's and teenage age. Among 19 cases studied by us 8 children were aged younger than 1 year that allowed to think of an atresia of extrahepatic bilious ways also; in 10 cases the age of children was from 1 year to 10 years and only one child was more senior than 10 years. However at inquiry almost at a half of these children it turned out that symptoms of a disease appeared at them within 1 year of life. Female explicit dominance is noted: 75% of girls according to Tsuchida and Kasai and 68,2% - according to our data. It is necessary to emphasize special exposure to this anomaly of the Japanese race. In Japan more than 200 cases were described; according to Kasai, cystous expansion of a holedokh is one of the most important surgical diseases at children. The inborn nature of this pathology admits most of authors; the cases observed at adults, apparently, represent the cysts which are not distinguished in the childhood.

Symptoms of a choledochal cyst:

At babies the disease is shown by a long cholestasia. Perforation of a cyst with development of bilious peritonitis is possible. At children of more advanced age and at adults the disease is usually shown by passing episodes of jaundice, pains against the background of volume education in a stomach. At children more often than at adults (respectively in 82 and 25% of cases), at least two signs from this "classical" triad are noted. Though earlier the disease was carried to the category of nurseries, now to its thicket diagnosed for adults. In a quarter of cases as the first manifestations serve pancreatitis symptoms. Choledochal cysts develop at Japanese and other east people more often.
Jaundice has the alternating character, belongs to cholestatic type and is followed by fever. Pain is kolikoobrazny, it is localized generally in the right upper quadrant of a stomach. Volume education is caused by a cyst, comes to light in the right upper quadrant of a stomach, has the different sizes and density.
Choledochal cysts can be combined with inborn fibrosis of a liver or Karoli's disease. Disturbances of a drainage of bile and a secret of a pancreas, especially are important if channels connect under a right or acute angle.

Diagnosis:

At a survey X-ray analysis of an abdominal cavity reveal myagkotkanny education. At babies when scanning with derivatives of iminodiacetate or at ultrasonography a cyst sometimes it is possible to reveal in utero or in a puerperal period. At children of more advanced age and adults the cyst is revealed at ultrasonography or KT. All diagnostic methods can yield false-negative result. The diagnosis is confirmed at a transdermal or endoscopic holangiografiya.

Treatment of a choledochal cyst:

Treatment of inborn cystous expansion of a holedokh always surgical. Operational data various. Now method of the choice is экзерес cysts with a gepatoyeyunalny anastomosis of a pas a loop in a form Y. Always the possible degeneration of the pockets left on site forces to prefer this method. Other authors consider as a method of final treatment of a cyst imposing of a kistoyeyunalny anastomosis on a loop in a form Y across Ru. The outside bilious drainage like cystifellotomy has to be redundant for exclusively urgent situations. Holetsistoektomiya was combined with treatment of a cyst in our 3 observations. It is obviously important to us to verify passability of portal circulation during operation.

At big children the surgery of a choledochal cyst most often has favorable effects: jaundice disappears during a pedela after operation, coloring of excrements appears after recovery of passability of bilious ways. Aggravations like a cholangitis arose only at 2 children and demanded repeated intervention after a partial ekzerez in one case and a cholecystoduodenostomy - in another.

Clinical recovery with return to norm of the sizes and a consistence of a liver was observed at 6 children. Hepatic tests were always normalized. The histologic control of a liver which is carried out in 7 cases showed total disappearance of changes in 4 of them with preservation of cicatricial fibrosis which expressiveness it is difficult to estimate as the biopsy was made by means of a needle.

Thus, it is possible to oppose the excellent postoperative forecast of inborn cystous expansions at big children of big weight of this pathology at newborns who have septic postoperative complications rather frequent and heavy.