Handbook of congenital malformations / edited by Alan Rubin.

Date:
1967
Catalogue details

Licence: Attribution-NonCommercial 4.0 International (CC BY-NC 4.0)

Credit: Handbook of congenital malformations / edited by Alan Rubin. Source: Wellcome Collection.

    22/424 (page 2)
    BILIARY ATRESIA (continued) nosis, but proof is only by exploration and liver biopsy. Death may be prompt, but 50 per cent live one year and 25 per cent live for 18 months. Survival for several years is not rare. A few have con- genitally hypoplastic bile ducts which produce the same poor outlook. Prevalence: Relatively rare. Associated Anomalies: Rare. Hereditary Factors: The anomaly occurs very rarely in siblings. Treatment and Outlook: Exploration with anastomosis to bowel (Roux-Y) of any dilated duct above the atretic area (only 4 to 6 per cent of cases can be so treated, and hence cured) ; liver biopsy to confirm histologic picture. No other operative measures offer any hope. If surgical correction is possible it should be curative. Process is otherwise uniformly fatal. Reference: Ghadimi, H., and Sass-Kortsak, A.: Evaluation of the radioactive rose-bengal test for the differential diagnosis of obstruc¬ tive jaundice in infants. New England J. Med. 265:351, 1961. CLOACA, EXSTROPHY OF. See page 302. CHOLEDOCHAL CYST (Choledochus Cyst, Cyst of Common Bile Ducf, Idiopathic Dilatation of Common Bile Duct). Cystic swelling of com¬ mon bile duct which may cause pain, jaundice, fever or vomiting or may be present as an asymptomatic mass. It may be due to unequal proliferation of epithelial cells, which form a solid mass filling the bile duct, or to duplication of the common duct or a wide range of other causes. X-ray study of the upper bowel may suggest the diagnosis— confirmation is by exploration. Prevalence: Rare. Associated Anomalies: None known. Hereditary Factors: Unknown. Treatment and Outlook: Surgical exploration with anastomosis of cyst to intestine (Roux-Y) should lead to an excellent result without residua. COLON, MALROTATION OF (Incomplete Rotation of Bowels, Incomplete Rotation of Colon). Failure of the bowel to undergo normal migra¬ tion and attachment on return from physiological omphalocele. Con¬ dition is due to arrest of development during the tenth or eleventh week of fetal life. Cecum usually rests in right upper quadrant with bands running laterally, sometimes significantly occluding the de¬ scending duodenum. Often seen in neonatal period with volvulus of midgut, due to short root of mesentery, and vascular damage. Vomit¬ ing and abdominal distention are danger signs—should prompt x-ray examination. Barium enema may be helpful in demonstrating mis¬ placed cecum. Prevalence: Rare. 2