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Cirrhosis Froni Congenital Obliteration of Tiie Bile Ducts

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CIRRHOSIS FRONI CONGENITAL OBLITERATION OF TIIE BILE DUCTS Congenital obliteration of the bile ducts is a not unusual malfor mation. The cirrhosis is brought about by simple stagnation of the bile, such as can be produced experimentally. In two of my cases the common bile duct was replaced by a fibrous cord. The gall bladder was absent. The liver was much enlarged and finely granular, the peri toneum much thickened with fibrous deposits, and the liver tissue showed cirrhotic changes. Some of the children are born jaundiced, sometimes the yellow tint appears first a few. days after birth. The jaundice persists to the end. The abdomen becomes markedly dis tended. Severe toxic symptoms arise; convulsions, haunorrhage from the navel and vomiting of blood, often as early as the first week. Um bilical hcnorrhage caused death after 14 days in one of my cases. Should the child survive longer, ascites appears; a breast-fed child whom I observed, survived for 6 months. In most cases this malforma tion depends on a primary defeet of development, in other cases dis ase of the fmtus has been thought to be the cause.

The treatment of cirrhosis must be directed against the causative factor when possible. If misuse of alcohol can be demonstrated we can do away with this factor and order unirritating diet, principally milk. Should the cirrhosis depend on syphilis, or should this factor even be suspected we must institute antisyphilitic treatment with iodide of potash or with mercury. Extract of the liver has been given in France, according to Gaillard, with success itt an advanced ease of atrophic cirrhosis, d'Espine has seen marked improvement in the car diac form of cirrhosis follow a sojourn by the sea.

Talma's operation, to relieve congestion by opening new paths for the portal circulation has been frequently reeonunended, and several times performed on children. I have the following case-report. (See Fig. 75, page 2S3.) A four-year-old child was taken sick acutely in the fall of 1897 with fever, slight icterus and marked aseites. Repeated abdominal punctures were made the next year, and in the early months of 1899 ten quarts of fluid were frequently evacuated. Talmals opera tion was performed in June 1S99. The ascites collected again after the operation, and twelve days later five quarts of fluid were evacu ated by puncture. A second puncture five days later evacuated three quarts. In the subsequent period the aseites did not appear and the child felt well enough to visit school from January to May 1900. Then the ascites returned necessitating puncture in July .S quarts) and August (14 quarts). After a second Talma operation, pneu monia developed and the child succumbed. The post-morteni findings were £L very small, rough liver, only half the normal size, and a buttdle of large veins at the point of anastomosis between the omentum and the abdominal walls.

The favorable result from the operation was unmistakable but not of long duration. Since the prognosis is bad, operation should be advised; there is always a chanee of obtaining good results.