658 CORRESPONDENCE

from high sodium and bicarbonate load are well-tolerated and are spontaneously balanced within several hours.

Richard Skhba, MD Dept of Pediatric Surgery Charles University in Prague V fivalu 84 151 12 PRAGUE 5-Motol Czechoslovakia

To the Editor:

I recently noted an article iq the Journal o f Pediatric Surgery, Vol. 18, No. 2 (April), pages 138-140, 1983, entitled "Hydrops of the Gallbladder in Children." The authors present a case of presumed idiopathic hydrops of the gallbladder and discuss differential diagnosis and manage- ment.

In addition to the disorders mentioned, acute viral hepatitis and Wilson's Disease need to be mentioned as two of the etiologies that may be associated with acute hydrops. In these settings, surgery would be contraindicated, and nonoperative management with nasogastric suction, fluid resuscitation, antibiotics, and other supportive measures would be the treatment of choice. The same can be said for the hydrops occurring with the mucocutaneous lymph node syndrome, in which operative management is usually not necessary.

Richard J. Grand, MD Chief, Division of Pediatric Gastroenterology and Nutrition New England Medical Center, Inc. 171 Harrison Avenue Boston, MA 02111

To the Editor."

A new classification of biliary obstructive disorders was proposed in a recent editorial (Journal o f Pediatric Surgery 18:107, 1983). A principle argument for deleting the adjec- tives correctable and uncorrectable is that the latter is now routinely treated with the operation of Kasai, with some success. An additional reason to delete these adjectives from modern terminology is that the former may not in fact be correctable. This point is illustrated by a recent case treated by the undersigned. A 22-year-old female presented with abdominal pain and jaundice. At 6 weeks of age she was treated at another hospital for jaundice. At exploration at that time atresia of the distal common bile duct was found and treated with a distal choledochoduodenostomy. She did well until her late teen years when she consulted several different physicians with abdominal pain and itching but was treated only symptomatically. On the present admission physical examination was remarkable only for jaundice. Laboratory studies revealed a bilirubin of 8.5, an SGOT of 144 and an alkaline phosphatase of 1675. A pereutaneous

Fig. 1. Postoperative cholangiogram via stent showing marked attenuation, clubbing, and irregularity of hepatic biliary radicles.

transhepatic cholangiogram was attempted but only two small ducts were able to be opacified despite multiple passes. She was reexplored with a preoperative diagnosis of stricture of the choledochoduodenostomy or primary choledocholithia- sis. At operation the gallbladder was decompressed and the choledochoduodenostomy was not strictured. However, the proper hepatic and the left and right hepatic ducts were extremely sclerotic and narrowed. After dilatation numerous intrahepatic stones were removed from the left ductal system. The right hepatic duct had a dense stricture with no more than a 1 mm lumen at its junction with the left duct. Operative and postoperative cholangiography (Fig. 1) revealed marked sclerosis and clubbing of all intrahepatic ducts. After dilatation the junction of the left and right ducts were drained over stents into a Roux limb. Pathology on a biopsy of the duct revealed severe fibrosis without evidence of malignancy. A liver biopsy showed severe secondary biliary cirrhosis with bile duct proliferation, canalicular bile plug- ging and bridging portal fibrosis. Postoperatively the biliru- bin rose to 26 but after several weeks fell to the preoperative level.

This case is nearly identical to one previously described (Berenson et al, Gastroenterology 66:260, 1974) and both illustrate that in at least some cases of congenital correctable biliary atresia the sclerosing disease progresses, albeit slowly, even in the face of surgical "correction."

Richard McCann, MD Duke University Medical Center Dept of Surgery Durham, NC 27710