Liver disease and common bile duct stenosis in cystic fibrosis

Liver disease and common bile duct stenosis in cystic fibrosis

138 INTERNATIONAL ABSTRACTS Blood Flow in Experimental Short Bowel Syndrome. M.E. Holl- warth, M.G. Ulrich-Baker, P.R. Kvietys, et al. Pediatr Surg...

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INTERNATIONAL ABSTRACTS

Blood Flow in Experimental Short Bowel Syndrome. M.E. Holl-

warth, M.G. Ulrich-Baker, P.R. Kvietys, et al. Pediatr Surg Internat 3:242-246, (May), 1988. Mechanisms of intestinal adaptation after major loss of small bowel have been studied extensively, but little attention has been given to related changes in blood flow. In this study, an 80% midintestinal resection was performed in rats (n = 54). Blood flows to the jejunal and ileal remnants, the anastomosis, and the cecum and colon were measured with the radioactive microsphere method on days 1, 2, 3, 5, and 28 after surgery. Results were compared with blood flow estimates after jejunal or ileal transection (n = 48) and with normal controls (n = 9). The results showed that the operative stress of intestinal transection is followed by a brief increase in intestinal blood flow. After 80% small bowel resection, intestinal blood flow remains more significantly elevated for at least three days. Blood flow to the ileal remnant remains elevated for at least 4 weeks after surgery. The mechanisms responsible for the increase in blood flow remain to be defined.--Prem Puri

Mechanisms of Intestinal Adaptation. J.B. Bristol and R.C.N.

Williamson. Pediatr Surg Internat 3:233-241, (May), 1988. The control of intestinal adaptation is complex and involves different mechanisms at different sites. The principal basic stimuli to adaptive growth are the presence of food in the gut lumen, endogenous secretions, and circulating hormones and peptides. Lesser factors include neural and vascular influences. Much effort has been devoted to the search for a systemic tropic factor responsible for the direct stimulation of mucosal cell proliferation. Conclusive proof is lacking, but evidence suggests that enteroglucagon or other gut-glucagon peptide fragments may be the elusive enterotropic substance. Attention has recently turned from the study of the phenomenon of intestinal adaptation itself, now well defined, to the possible intracellular mechanisms responsible for enhanced cell division. This review highlights the way these endeavors have led to our current appreciation of the mechanisms of intestinal adaptation.--Prem Puri ABDOMEN Plain Abdominal Radiographs and Acute Abdominal Pain, J.P.M.

Campbell and A.A. Gunn. Br J Surg 75:554-556, (June), 1988. The authors reviewed the records of over 5,000 patients presenting to a district general hospital with acute abdominal pain over a 4-year period. The contribution of the abdominal radiographs in the assessment of those patients with suspected appendicitis, urinary tract infection, and nonspecific abdominal pain was evaluated. These conditions accounted for just under half of the patients presenting with abdominal pain and a third of those with abdominal radiographs. It was concluded that any positive information from the radiographs was likely to be potentially misleading, and that in suspected appendicitis, urinary tract infection, or nonspecific abdominal pain, there is little value in the routine use of abdominal radiographs. It was emphasized that unnecessary radiographs have costs in financial terms, in patient discomfort, and in gonadal irradiation.--John D. Orr

The Continued Value of Angiography in Planning Surgical Resection of Benign and Malignant Hepatic Tumors in Children. LL.D.

Tonkin, E.L. Wrenn, and R.S. Hollabaugh. Pediatr Radiol 18:3544, (January), 1988. Believing that the exact vascular anatomy of the liver and its contained tumor is important, the authors reviewed their 10-year

experience with 27 children, aged five days to 16 years, who had primary liver tumors. Malignancy was present in 22 of the patients, with hepatoblastoma predominating in 18. The five benign tumors included two hemangioendotheliomas, two cystic mesenchymal hamartomas, and one focal nodular hyperplasia. The angiograms were analyzed retrospectively and were found to precisely localize the tumor and accurately establish the tumor as benign or malignant in each of the 27 cases. Criteria for judging a mass as being benign or malignant on the angiogram are reviewed and contrasted with findings of ultrasonography and computerized tomography.-Thomas V. Whalen Liver Disease and Common Bile Duct Stenosis in Cystic Fibrosis.

K.J. Gaskin, D.L.M. Waters, R. Howman-Giles, et al. N Engl J Med 318:340-346, (February 11), 1988. Hepatobiliary scanning in 50 of 61 patients with cystic fibrosis and clinical evidence of liver disease was compared with similar scanning in 31 of 92 patients with cystic fibrosis and "normal livers." Evaluation of liver disease was first done by clinical examination and liver function studies. Clinical liver disease was defined as the presence of firm hepatomegaly, splenomegaly, or both. Biochemical liver disease was defined as the presence of one or more elevations of transaminase, transpeptidase, alkaline phophatase, bilirubin, and fasting bile acids. Percutaneous transhepatic cholangiography was performed in 30 children with clinical and biochemical evidence of liver disease. Of the 153 patients evaluated, 36 had both clinical and biochemical manifestations of liver disease, 11 had clinical disease only, 14 had biochemical disease, and 92 had neither clinical nor biochemical disease. Hepatobiliary scanning was performed in 45 patients with clinical liver disease, five with biochemical disease only, and 31 patients without liver disease. All 45 patients with clinical liver disease had biliary tract obstruction characterized by common bile duct tapering (31), retention of tracer in the parenchyma or intrahepatic duct (41), or enlarged gallbladders with a delay in emptying of up to three hours (31). In five patients with biochemical liver disease alone, two scans appeared normal except for nonfunctioning gallbladders. Among the 31 patients without liver disease, there was no evidence of retention of tracer in the gallbladder or the intrahepatic or common bile ducts. However, in 20 of these patients, the gallbladder was not visualized. Twenty-nine of the 47 patients with clinical liver disease and one with only biochemical disease had evidence of biliary obstruction on scanning and underwent cholangiography. Twenty-eight were found to have strictures of the distal common bile duct of various lengths and tortuosity, with some having an abnormal insertion into the third portion of the duodenum. All 18 of 31 patients with tapering of the common bile duet on hepatobiliary scanning who underwent cholangiography had strictures of the distal common duct. In ten patients in whom the distal common duct was not adequately visualized on scanning, but with retention of tracer in the gallbladder, parenchyma, or intrahepatic ducts, eight had strictures of the distal duct on cholangiography, whereas the other two had beaded irregularly-narrowed distal ducts. Only 73% of 48 patients with evidence of obstruction on scanning had abnormal liver chemistries. Fourteen patients with common duct strictures underwent surgery; nine with functioning gall bladders had cholecystojejunostomies, and five with contracted gall bladders had choledochojejunostomies. Preoperatively, all had upper abdominal colicky pain which was relieved postoperatively. Several of these patients had been considered to have meeonium ileus equivalent as the cause of the pain. This study suggests that distal common duct stenosis, probably secondary to pancreatic fibrosis, is a common occurence in children with cystic fibrosis and especially in those children with hepatomegaly and biochemical abnormalities. The presence of abdominal pain of a recurring nature, especially in view of any of the above findings, even without

INTERNATIONAL ABSTRACTS

biochemical abnormalities, suggests the need for hepatobiliary scanning as an excellent screening study to identify the presence of distal common duct obstruction.--Eugene S. Wiener Mesobiileal Shunt in an Infant for Control of Bleeding From Gastroesophageal Varicas. T.C. Putnam, Surgery 104:108-111,

(July), 1988. A type of pertosystemic shunt is described that will remain patent in an infant. After controlling the inferior vena cava inferior to the renal veins, each common iliac vein is divided and the distal ends ligated. Each iliac limb of the resulting "Y" is anastomosed in end-to-end fashion to each end of the divided superior mesenteric vein with interrupted 6-0 Prolene sutures. The procedure was performed successfully on a 5-month-old infant weighing 34.6 kg. The child did well until death from sepsis 6 months later. The autopsy showed a patent shunt.--John N. Schullinger Histopathologic Studies of Congenital Dilatation of the Bile Duct as Related to an Anomalous Junction of the Pancreaticobiliary Ductal System: Clinical and Experimental Studies. Y. Oguchi, A./kada, T.

Nakamura, et al. N Engl J Med 103:168-173, (February), 1988. Histopathologic studies of congenital dilatation of the bile duct (CDBD) as related to an anomalous junction of the pancreaticobiliary ductal system (AJPBDS) was performed both clinically in humans and in an animal model. Forty patients with CDBD, 29 children (1 month to 13 years of age), and 11 adults (21 to 59 years) were studied. Common bile ducts were excised in all. Thirty-one patients had cystic and nine patients had cylindrical dilatation. AJPBDS was clearly demonstrated radiographically in all of the 35 patients studied. Epithelial hyperplasia instead of a single columnar epithelial layer, accompanied by small round cell infiltration and an increasing number of glands, occured in 23 of 40 patients (57.5%). In 17, no epithelial hyperplasia or small round cell or glands were seen, but thickness of the wall and fibrosis were prominent. An experimental model was created in ten mongrel dogs using a duodenal patch with the main pancreatic duct attached and anastomosed to the gallbladder. Similar histologic changes were present in all animals. These findings suggest that an anomalous junction of the pancreaticobiliary ductal system and the reflux of pancreatic juice into the bile ducts may be important in the etiology, as well as the pathologic and clinical findings in patients with congenital dilatation of the bile ducts.--Eugene S. Wiener Time-Space Distribution of Extrahepatic Biliary Atresia in The Netherlands and West Germany. R.H.J. Houwen, LLA. Klerre-

marts, H.A. vain Steensel-Moll, et al. Z Kinderchir 43:68-71, (April), 1988. Extrahepatic hiliary atresia (EHBA) is an infrequent disease confined to early infancy. Its etiology has not been elucidated yet, but an infectious agent may be implicated. Demonstration of timespace clustering would support this hypothesis. The authors investigated the time-space distribution in 89 patients with EHBA born in a 10-year period in The Netherlands, and 130 patients with EHBA born in West Germany between 1969 and 1986. There was no evidence of clustering in specific years or in a specific period of the year. The birth places of the patients were also randomly distributed over rural areas, villages, and towns. It is concluded that this random distribution of patients with EHBA suggests a pathogenetically heterogenous etiology.--Thomas A. Angerpointner Studies on Subtype "'d'" Biliary Atresia. T. Chiba, 1. Mochizuki and M. Kasai. Z Kinderchir 43:88-91, (April), 1988.

There are various types of obstruction of the extrahepatic bile duct in biliary atresia, totaling about 60 different combinations of

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obstruction at the portahepatis. Nine cases of a specific type (subtype "d" according to Kasai) are presented and classified in three groups on the basis of their clinical and pathologic findings. Group 1 cases are defined as those showing obstruction on the side of the hepatic duct and cystic dilatation of the common bile duct communicating with the gallbladder. Group 2 cases are those presenting with dilatation similar to group 1, but measuring <20 mm, and with a thick covering of the dilated parts with connective tissue. Group 3 cases are those showing isolated dilatation in the hepatic duct or the common bile duct, but without communication with the gallbladder. Postoperative courses were satisfactory in group 1 patients, while jaundice was prolonged in group 2 patients. In two group 3 patients, jaundice disappeared in one who died later on, and the other patient showed recurrence of slight jaundice. Subtype "d" is a specific type with a low frequency of occurrence. This study shows that subtype "d" can be further subdivided into three groups.--Thomas A. Angerpointner

Ictoric Flare-Up in Patients With Biliary Atresia After Hepatic Portoenterostomy. H. Takemoto, Y. lnomata, Y. Matsukawa, et

al. Z Kinderchir 43:92-94, (April), 1988. The recurrence of icterus may be a sign of serious postoperative complication after hepatic portoenterostomy for biliary atresia. To analyze the cause of such icteric flare-up, the authors performed a retrospective study of 27 patients. A total of 38 episodes of icteric flare-up occurred in 19 patients. An accompanying disease, which had not directly involved the biliary tract, was assumed to be the cause in 11 episodes (29%). Fourteen episodes (37%) were due to biliary tract infection caused by cysts formed at the portahepatis or ascending cholangitis. The remaining 13 episodes (34%) were free from both accompanying disease and signs of biliary infections. Among them, the cause of icteric flare-up was a cyst at the portahepatis in one case, scar tissue formation at the portahepatis in nine episodes, and undetermined in three episodes. In conclusion, a patient suffering from an afebrile episode of icteric flare-up might require a reoperation. However, since some episodes can be managed with routine postoperative medication within 2 weeks, observation for this period may be justified.--Thomas A. Angerpointner

Efficacy of HepatobUiary Scintigraphy in the Differential Diagnosis of Neonatal Chotestasis and Postoperative Follow-Up After Hepatoportodigestive Anastomosis. J. Dressier, I. Kerremans, and P.

Schweizer. Z Kinderchir 43:81-87, (April), 1988. In the differential diagnosis of unclear neonatal and infantile cholestasis, the formerly employed 131-I-Rose Bengal scintigraphy has been widely abandoned in favor of hepatobiliary scintigraphy by means of 99"Tc-labeled IDA derivatives excreted with the bile. The authors present the results in 32 jaundiced children, aged 2 weeks to 32 weeks, which show high sensitivity of scintigraphy. Diagnostic accuracy can be further increased if additional criteria, such as hepatocellular clearance and patient age, are considered. This method is of equally high importance for postoperative follow-up, where it is superior to all other imaging procedures in the evaluation of bile flow situations. In 52 examinations of portoenterostomies, the prognostic value can be demonstrated not only with a good correlation of scintigraphic data with the bilirubin level, but also the degree of liver fibrosis at the time of operation. Early and late complications of hepatoportojejunostomy can usually be recognized and localized. Since the prognosis of extrahepatic biliary atresia depends on early operation, hepatobiliary scintigraphy must be carried out earlier and more frequently in all unclear cases of cholestasis.--Thomas A. Angerpointner