Bile Duct In Infancy Research Articles

Spontaneous Perforation of the Extrahepatic Biliary Tree in Infancy

Spontaneous perforation of the bile duct in infancy is rare, with less than 55 cases described in the literature to date. The authors report the case of a 30-day-old neonate who presented with a 2-week history of progressive abdominal distension and intermittent jaundice. Disofenin technicium 99m sequential scintiscanning provided a preoperative noninvasive confirmation of the diagnosis of biliary ascites secondary to spontaneous perforation of the extrahepatic biliary tract. Distal common bile duct atresia was identified intraoperatively, and end-to-side Roux-en-Y hepaticojejunostomy was performed, with an uneventful postoperative recovery. The etiology, diagnosis, and treatment of spontaneous neonatal biliary perforation is discussed.

Spontaneous perforation of the common bile duct in infancy: D.C. Dunn and V.C. Lees. Br J Surg 73:929, (November), 1986

Spontaneous perforation of the common bile duct in infancy: D.C. Dunn and V.C. Lees. Br J Surg 73:929, (November), 1986

Pigment gallstones of the common bile duct in infancy.

Ten infants of less than 6 months of age presented with cholestatic jaundice and gallstones. Jaundice occurred after a lag period, and sepsis was present in three children. Ultrasound examination showed dilatation of intrahepatic and extrahepatic bile ducts in eight patients and detected cholelithiasis in three. Percutaneous transhepatic cholangiography and/or surgery allowed separation of the patients into two groups: (i) six children with lithiasis in the distal common bile duct, and (ii) four children with lithiasis associated with bile duct perforation at the junction between the cystic and common bile ducts with gallstones probably secondary to bile stasis and infection. Surgical treatment was confined to removal of calculi and drainage in eight children; biliary reconstructive surgery was necessary in the other two who had serious biliary duct lesions. No recurrence was observed after 1 to 7 years. The pigmentary nature of cholelithiasis was established by stone morphology in all cases, and by bile and stone analysis in several cases.

Manifestations of nonsuppurative cholangitis in chronic hepatobiliary diseases: morphologic spectrum, clinical correlations and terminology.

The features of nonsuppurative cholangitis were studied in liver biopsy specimens from 185 patients with chronic active hepatitis (CAH), 280 patients with primary biliary cirrhosis (PBC), and 55 patients with primary sclerosing cholangitis (PSC). Specimens from patients with other liver diseases in which the presence of nonsuppurative cholangitis had been recorded were also studied. We identified four types of nonsuppurative cholangitis: granulomatous cholangitis, lymphoid cholangitis, fibrous cholangitis, and pleomorphic cholangitis. Granulomatous cholangitis almost always seemed to be destructive; the other types were either destructive or nondestructive. Granulomatous cholangitis was, for all practical purposes, diagnostic of PBC and the obliterative form of fibrous cholangitis was similarly diagnostic for the hepatic manifestations of PSC in adults and paucity of intrahepatic bile ducts in infants. All other types of cholangitis were found in CAH, PBC, PSC, and other liver diseases. Thus, the term "nonsuppurative cholangitis" describes a spectrum of morphologic lesions that differ in incidence, morphogenesis, usefulness for liver biopsy diagnosis, and, probably, pathogenesis.

Arteriohepatic dysplasia in infancy and childhood: a longitudinal study of six patients.

Arteriohepatic dysplasia (syndromatic ductular hypoplasia, Alagille syndrome) is a condition of chronic cholestasis dating from infancy accompanied by characteristic facies, pulmonic stenosis, and other somatic abnormalities. The pathologic hallmark of arteriohepatic dysplasia is a paucity or absence of intrahepatic bile ducts, wildely regarded as a congenital deficiency. We present a longitudinal study of six infants and children with arteriohepatic dysplasia, stressing evolution of the characteristic pathology on liver biopsy. All patients were biopsied twice. All five liver biopsies performed during infancy (<6 months old) showed intrahepatic cholestasis and portal inflammation, and two infants had, in addition, giant cell transformation. No infant showed congenital absence of interlobular bile ducts. Three of five infants had normal numbers of interlobular bile ducts (0.5 to 1.0 interlobular bile ducts per triad) and two infants had a paucity of interlobular bile ducts (<0.5 per triad). Three infants showed focal destructive inflammation of interlobular bile ducts. All biopsies performed later in childhood, between 3 and 20 years of age, showed a paucity or absence of interlobular bile ducts. At this time cholestasis and inflammation had largely resolved but some fibrosis persisted. The number of interlobular bile ducts decreased with age in each patient but there was no characteristic age at which interlobular bile ducts disappeared completely. The presence of interlobular bile ducts in infancy followed later in childhood by paucity or absence of interlobular bile ducts, suggests that intrahepatic bile duct deficiency in some patients with arteriohepatic dysplasia is acquired rather than congenital. The mechanism of bile duct disappearance in these patients may have been destructive inflammation of duct epithelium. Histopathologic diagnosis of arteriohepatic dysplasia may be difficult or impossible in infancy since interlobular bile ducts may be present at that time. However, arteriohepatic dysplasia should be considered in the differential diagnosis of all infants with cholestasis and such infants should have careful clinical evaluation for the syndromatic features of arteriohepatic dysplasia. Evaluation of their liver biopsies should include specific morphometric assessment of interlobular bile ducts.

Spontaneous perforation of the bile ducts in infancy : J. Koo, A. B. Jones, and F. W. Turner. Can J Surg 20:41–44 (January), 1977

Spontaneous perforation of the bile ducts in infancy : J. Koo, A. B. Jones, and F. W. Turner. Can J Surg 20:41–44 (January), 1977

Spontaneous rupture of the extrahepatic bile ducts in infancy : J. M. Hartong. Am Surg 42: 795–800 (October), 1976

Spontaneous rupture of the extrahepatic bile ducts in infancy : J. M. Hartong. Am Surg 42: 795–800 (October), 1976

Massive bile peritonitis in infancy due to spontaneous bile duct perforation with portal vein occlusion

A case of massive bile peritonitis due to spontaneous slough of the junction of the cystic, common, and common hepatic ducts in a 5-wk-old male infant is reported. This was managed successfully by a Roux-en-Y jejunal anastomosis to the remnant of common hepatic duct. Subsequent ascites (4 mo postoperatively) was relieved by peritoneovenous drainage by a Holter valve from the peritoneal cavity to the right atrium. Comments are made relative to an etiologic commonality between spontaneous perforation of the bile ducts in infancy, “congenital” choledochal cyst, and biliary atresia and a possible role for birth-acquired viral infection, particularly with a herpes type of virus, in these disorders.

Choledochal cyst and dilatation of the bile ducts in infancy and childhood : K. Somasundaram. Aust. New Zeal. J. Surg. 42:163–167 (November), 1972

Choledochal cyst and dilatation of the bile ducts in infancy and childhood : K. Somasundaram. Aust. New Zeal. J. Surg. 42:163–167 (November), 1972

Choledochal cyst and dilatation of the bile ducts in infancy and childhood.

Choledochal cyst, though not common, has become recognized as a distinct clinical entity. Almost half of them are diagnosed before the patient reaches the age of ten years. This paper reports four instances of choledochal cyst occurring in childhood which were encountered by the author during the past two years. A fifth instance of intermittent neonatal jaundice due to a different cause is also reported in order to illustrate the problems of diagnosis and management.