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• W2012633708 abstract "The timing of hepaticoportoenterostomy (HPE) continues to be one of the main prognostic factors for outcome in infants with biliary atresia (BA). Improvement in bile flow may be effective in 80% to 90% of cases when surgery is performed before 60 days and in less than 20% after 90 days (1). Even then, 27% to 54% of BA patients eventually require liver transplantation (LTX) for decompensated cirrhosis after unsuccessful HPE (1). Cystic dilatation of intrahepatic bile ducts (CDIB) has been described in 17.5% to 25% of cases of BA after HPE, developing between 4 months and 14 years of age (2). However, CDIB is uncommon before HPE in BA; only five cases were previously reported (2-8). Bu et al. (2) described 154 patients with BA of whom 2 (1.2%) developed multiple CDIB before surgery at an age of 8 months or more. We report a 4 month old diagnosed with BA associated with CDIB that developed before HPE. CASE REPORT A 4-month-old girl was referred to Hôpital Sainte-Justine for cholangitis. After an uneventful pregnancy, she was born at term by caesarean section for pelvic presentation. APGAR scores were normal, and birth weight was 2,845 g. Although her parents noted jaundice a few days after birth, this disorder was investigated in another hospital only the day before referral. At admission, her weight was 5.330 kg (15%), length 57 cm (<5%), and temperature 39.5°C. Physical examination revealed jaundice with acholic stools, hepatosplenomegaly with a firm liver consistency, ascites, and inguinal and umbilical herniae. Initial investigations showed elevated alanine aminotransferase of 150 IU/L (normal 5-25 IU/L), aspartate aminotransferase of 208 IU/L (normal 5-70 IU/L), γ-glutamyl-transpeptidase of 590 IU/L (normal 3-87 IU/L), total bilirubin of 174 μmol/L, direct bilirubin of 115 μmol/L, International Normalized Ratio of 2.12, C-reactive protein of 33 mg/L (normal < 6 mg/L), and reduced hemoglobin of 75 g/L and albumin of 19 g/L (normal > 30 g/L). Abdominal ultrasonography (US) revealed an enlarged, heterogeneous liver, without intrahepatic bile duct dilatation, a small and thickened gallbladder, ascites, a slightly enlarged spleen, and normal kidneys. 99mTc-DISIDA hepatobiliary scintigraphy did not identify intra- or extrahepatic bile ducts, and excretion into the small bowel was not demonstrated. She was treated with intravenous ticarcillin/clavulanic acid and tobramycin. Her ascites was managed with a combination of furosemide, spironolactone, intravenous albumin, and abdominal paracentesis. Ursodeoxycholic acid (UDCA) and vitamin K were started. Other etiologies for neonatal cholestasis were ruled out: bacterial infection (negative blood and urine cultures) and syphilis, viral infection (negative serologic tests for cytomegalovirus, herpes simplex virus, varicella-zoster virus, Epstein-Barr virus, rubella, measles virus, viral hepatitis A, B, C), toxoplasmosis, alpha-1-antitrypsin deficiency, neonatal hemochromatosis, tyrosinemia, galactosemia, hypothyroidism, immunodeficiency, and Alagille's syndrome. A percutaneous liver biopsy showed marked fibrosis and expansion of the portal tracts, micronodular regeneration, bile ductular proliferation with bile plugs, and mild to moderate acute peribiliary inflammatory infiltration indicating cholangitis. CDIB was first seen on US 9 days after admission and confirmed by magnetic resonance cholangiography (Fig. 1A); extrahepatic bile ducts were not seen. A short portal vein was noted. Percutaneous cholangiography also showed serpiginous intrahepatic bile duct dilatation that did not communicate with intrahepatic or extrahepatic bile ducts (Fig. 1B).FIG. 1: (A) Magnetic resonance cholangiography showing cystic dilatations of intrahepatic bile ducts (arrows). (B) Percutaneous cholangiography after injection into dilated intrahepatic bile ducts shows same large biliary cysts. Contrast does not communicate with the intrahepatic biliary tree or with intestine.Bile cultures were negative, yet she remained febrile despite the subsequent addition of vancomycin and, eventually, Amphotericin B after the culture of Candida parapsilosis from stools. Three weeks later, at 5 months of age, she underwent LTX, indicated for a late diagnosis of BA associated with a persistent cholangitis and CDIB that were unlikely to benefit from HPE. The explanted recipient liver confirmed the diagnosis of BA and the presence of noncommunicating, multilocular biliary cysts filled with stagnant bile that were localized mainly at the porta hepatis and centrally throughout both lobes, with only rare cysts peripherally (Fig. 2). These observations correlated completely with the radiologic findings. Histologic findings included cystic dilatation of bile ducts in the hilar region, with the lumen frequently filled with bile engorged macrophages. The biliary epithelium was characterized by ulceration, granulation tissue, and fibrosis. The extent of fibrosis and inflammation suggested a long-standing inflammatory process. More peripherally, well-established micronodular cirrhosis was evident with bile ductular proliferation, intracellular cholestasis, and bile ductular plugging. Many of the peripheral cysts showed extensive inflammation and complete loss of the epithelium. Some bile structures showed modifications compatible with the ductal plate malformation (DPM), a disturbance in the regular remodeling of the fetal ductal plate leaving the intrahepatic bile ducts in the fetal configuration (9) (Fig. 3).FIG. 2: Macroscopic appearance of liver removed at time of transplantation and sectioned between right and left lobes: cholestatic liver with noncommunicating, multilocular biliary cysts. Larger cysts, filled with stagnant bile, are located near the hilum (arrows).FIG. 3: Histologic section of explanted liver showing persistence of embryological bile duct configuration, ductal plate malformation (arrows) with extensive fibrosis, and irregularly dilated bile ducts. Note mild chronic inflammatory infiltrate (arrowhead) (hematoxylin-phloxine-saffron, magnification ×10).DISCUSSION The etiology of CDIB in BA remains speculative. Moreover, it is not clear whether the same mechanisms pertain to cysts that develop in unoperated children as to those in children after HPE. Most of the cases described have been discovered after surgery, at times many years later. Cholangitis is a common complication of BA after HPE but is uncommon in unoperated children. It is generally assumed to result from ascending infection possibly associated with bacterial overgrowth in a poorly motile or excessively long Roux-en-Y loop (7,10). Antireflux valves have been placed to prevent ascending cholangitis with mixed success (11). However, the presence of an antireflux valve does not protect against cholangitis if intrahepatic cysts are present, suggesting that ascending infection may not be the principal contributing factor (7). Other hypotheses include impaired lymph drainage, portal venous infection, coexisting cholestasis, bacterial translocation, and hematogenous spread (1,11). After HPE, CDIB may appear rapidly at the time of cholangitis or shortly afterward, suggesting that cholangitis may be an initiating process (2,5,10). The incidence of postoperative cholangitis is approximately 40% to 60% (3), but can reach 60% to 100% if CDIB is present (12). A history of cholangitis was reported significantly more frequently in patients with cysts (50%) versus those without cysts (17%) (5). Cholangitis may temporarily potentiate an inflammatory destruction of the bile duct epithelium. Indeed, antibiotic therapy has been associated with reduction in size of the cysts and, rarely, even with complete resolution of cysts (2,7,10). Alternatively, CDIB with sterile contents may appear, as in our patient, independently without a clear history of cholangitis, suggesting that CDIB may result from ongoing intrahepatic bile duct inflammation, fibrosis, and obstruction rather than infection per se (2,5,10). Indeed, as early as 1975, Fonkalsrud et al. (13) suggested that CDIB developed as a result of progression of bile duct destruction, late in the course of the disease, after 6 months of age. The development of CDIB before HPE may be associated with a more severe form of biliary disease. Our patient developed early neonatal jaundice associated with a shortened portal vein and intestinal malrotation. This constellation, along with other extrahepatic malformations, is found in about 20% of infants with BA and is associated with the congenital form of the disease (9). DPM may be observed in 21% to 38% of BA, but insufficient clinical data are available to conclusively associate DPM with the congenital, early, severe form of BA (9,14). Nevertheless, failure of remodeling of the ductal plate in the first trimester of gestation has been proposed to lead to rupture of the bile duct, leakage of bile, and subsequent inflammation and fibrosis (15). Moreover, the DPM has been associated with a poor prognosis in BA (15). It is tempting to speculate that the DPM is a risk factor for future development of CDIB. The role of choleretic therapy in the development of CDIB cannot be discounted. We postulate that UDCA may have been contributory given that cysts appeared only 9 days after the beginning of therapy. Possibly, increased bile secretion caused by UDCA treatment in a patient with a poor biliary drainage may have helped to accelerate the development of CDIB. This effect may be particularly pronounced in the absence of bile drainage after HPE. Betz et al. (5), however, although observing that those with cysts were more likely to have received choleretic therapy, found that the difference was not statistically significant. In conclusion, cholangitis and CDIB can appear before HPE and may be associated with severe forms of BA. The development of intrahepatic cysts may be favored by the presence of the DPM and by the use of UDCA in the presence of complete biliary obstruction." @default.
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• W2012633708 title "Intrahepatic Biliary Cysts Presenting before Hepatic Portoenterostomy in Biliary Atresia" @default.
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