Evaluation of prognostic criteria in extrahepatic bile duct atresia

Extrahepatic bile duct atresias must be classified into four histopathological groups according to the characteristic numerical, metrical, and morphological alterations of the interlobular bile ducts. A prospective study based on an observation period of 5 and more years included 43 patients with extrahepatic biliary atresia. It showed that prognosis is generally dependent on three factors: (1) the duration of cholestasis; (2) the structure of the bile ducts in the praehilaeren Versclussplatte; and (3) the histopathologically defined features of the interlobular bile ducts. The total diameter of all bile duct structures in the praehilaeren Verschlussplatte is unmistakably the most significant finding. A total diameter of more than 400 m indicates a favorable prognostic subtype, while a total diameter of less than 400 m is unfavorable. Even if the prognostically favorable type of finding is present, the ultimate prognosis is determined by the histopathological features of the interlobular bile ducts. Therefore, four characteristic groups must be taken into consideration when evaluating prognosis.     

Isolated extrahepatic bile duct injury: diagnosis and surgical management

Isolated injuries of the extrahepatic bile ducts due to blunt trauma are rare. The diagnosis in these cases is often delayed and there are no firm guidelines in regard to the treatment. We present a 3-year-old child with partial disruption of the confluence of the left and right hepatic ducts. A hepatic scintiscan was used to confirm the diagnosis and identify the site of the bile leak. The available literature has been reviewed briefly to highlight the clinical features that should arouse suspicion of such injuries and the various surgical options are discussed.     

Acute vanishing bile duct syndrome after ibuprofen therapy in a child

We report the case of a 10 year-old girl who had Stevens-Johnson syndrome and cholestasis after ibuprofen therapy. Liver histology was compatible with vanishing bile duct syndrome. She received ursodeoxycholic acid, and liver tests normalized within 7 months. This report confirms that ibuprofen may induce acute vanishing bile duct syndrome.     

Ectopic drainage of the common bile duct into the lesser curvature of the gastric antrum in a newborn with pyloric atresia,annular pancreas and congenital short bowel syndrome

We report a newborn with bilious vomiting and the rare combination of pyloric atresia, annular pancreas and ectopic drainage of the common bile duct into the lesser curvature of the gastric antrum. Radiologic, sonographic and percutaneous transhepatic transcholecystic cholangiographic (PTTC) findings, with surgical correlation, are presented.     

Anterior abdominal wall defects and biliary obstruction

Three infants with anterior abdominal wall defects (gastroschisis and exomphalos) who presented with obstructive jaundice secondary to biliary obstruction, are described. All three infants had abnormal biliary systems, with mechanical distortion of the biliary tree. Biliary obstruction secondary to structural biliary anomalies should be considered in patients with abdominal wall defects and cholestasis, as prolonged unrelieved biliary obstruction may lead to biliary cirrhosis and portal hypertension.     

Spontaneous resolution of extrahepatic biliary obstruction in a neonate

A patient with spontaneous resolution of complete extrahepatic biliary obstruction shortly after the neonatal period is described. Liver biopsy prior to resolution revealed widened portal tracts and extensive fibrosis; these changes normalized following resolution. The aetiology was not established but possibilities included a choledochal cyst, localized inflammation; for example, pancreatitis, a calculus of the common bile duct or a congenital membrane. Management of this patient would have been advanced if there were a paediatric size side-viewing duodenoscope which would have diagnostic and therapeutic potentials.     

Combined chemotherapy and tracheobronchial stenting for life-threatening airway obstruction in a child with endobronchial non-Hodgkin lymphoma

Endobronchial involvement in non-Hodgkin lymphoma is rare even in the presence of advanced disease. A 15-year-old boy presented with progressively worsening dyspnea with occasional hemoptysis for 1 week prior to admission. Three days later, he was intubated due severe dyspnea with complete atelectasis of the right lung. Fiberoptic bronchoscopy disclosed an endobronchial mass almost occupying the right main bronchus. He underwent partial resection of the endobronchial tumor with rigid bronchoscopy. An airway stenting was used in this patient because he had severe tracheal obstruction from the tumor. The compromised airway was alleviated by combined chemotherapy and tracheobronchial stenting.     

Duodenal duplication with bile duct obstruction and keratinaceous casts

We report an unusual case of duodenal duplication presenting in a newborn with duodenal and bile duct obstruction. The duplication and biliary tract were filled with keratinaceous casts. To our knowledge, such an association has not been previously reported; this case demonstrates the importance of confirming bile duct patency during operations to remove duodenal duplications.     

The width of the common bile duct in childhood

The maximum diameter of the common bile duct was measured on 85 normal intravenous cholangiograms carried out on children between the ages of 1–14 years. There was a close correlation between bile duct width and the patient's age, but a poor correlation with weight. The range of bile duct widths in each age group is tabulated as a guide to diagnosing biliary dilatation in childhood.     

新生儿肝外胆管囊性病变的临床与病理特征

目的　了解新生儿不同类型肝外胆道囊性病变的临床表现与病理特征。方法　对 2 2例新生儿肝外胆道囊性病变 [Ⅰ型胆道闭锁 (BA) 4例 ,Ⅲ型BA 1例 ,胆总管囊肿 (CC) 17例 ]患儿临床表现、肝功能、B超、CT、光镜及电镜特征进行比较。结果　BA与新生儿CC均好发于女童 ,以梗阻性黄疸为主要表现 ,肝功能检查结果符合梗阻性黄疸的肝功能改变。二者肝脏组织学改变 (光镜与电镜 )也可能相似。但B超与CT检查发现 ,BA组囊肿与胆囊大小均小于CC组 (P均 <0 .0 5 )。术中胆道造影显示ⅢBA型组肝外胆管囊肿终止于肝门处 ,肝内胆管树无法显示 ;Ⅰ型BA组肝内胆管树变形、连续性中断且有左右肝管狭窄 ;而CC组肝内胆管树形态正常。结论　新生儿肝外胆道囊性病变可能是多种胆道疾病的局部表现 ,术中造影可有效鉴别这些疾病 ,并能为治疗方法的选择提供可靠依据     

Children presenting with hydatid cysts in common bile duct and choledochal cyst

Hydatid cysts developing primarily in the common bile duct are extremely rare. Occasionally, hydatid cysts rupture into the extrahepatic biliary ducts and cause obstruction [1–7]. Two children with intact hydatid cysts in the common bile duct and choledochal cysts were seen during a 2-year period in the Paediatric Surgery Division of the Children's Hospital, Banaras Hindu University, Varanasi. The details of their presentation and operative management are reported.     

敲除巨噬细胞对胆管结扎小鼠胆汁淤积的干预作用

目的探讨巨噬细胞对胆汁酸代谢的影响。方法以结扎7周龄雄性CD11b-DTR小鼠胆总管方法建立胆汁淤积模型,2周后测定肝脏及结肠内容物中胆汁酸谱及肠道菌群组成。将28只小鼠分为假手术(Sham)组、假手术干预(DT)组、胆管结扎(BDL)组、胆管结扎干预(BDL+DT)组。结果与Sham组相比,BDL组肝脏中初级胆汁酸及总胆汁酸含量均显著增加,BDL+DT组肝脏中非结合初级胆汁酸及总胆汁酸含量则较BDL组明显减少,差异有统计学意义(P均0.05)。在结肠内容物中,BDL+DT组较BDL组结合初级胆汁酸含量降低,差异有统计学意义(P0.05)。BDL+DT组肝内胆汁酸合成关键酶cyp7a1表达量较BDL组降低,差异有统计学意义(P0.05)。与Sham组相比,BDL组肠道Prevotella的属水平相对丰度增加,而BDL+DT组Escherichia、Clostridium和Segmented filamentous bacteria(SFB)较BDL组属水平相对丰度增加,差异均有统计学意义(P均0.05)。结论巨噬细胞条件性敲除可通过改变肠道菌群结构干预胆汁淤积。     

Ileo-caecal arterio-venous malformation associated with extrahepatic portal hypertension: a case report

This paper is a case report describing a boy with Down syndrome and a novel combination of multiple vascular anomalies: extrahepatic portal hypertension, an arterio-venous malformation (AVM) at the ileo-caecal junction, and caval/iliac vein anomalies and developing anal bleeding. We considered that the ileo-caecal AVM would be one of the causes of the repeated hematochezia. The patient underwent ileo-caecal resection with the AVM, and anastomosis of the left external iliac vein and the jejunal branch vein because of the stenosis of the superior mesenteric vein (Clatworthy mesocaval shunt). Intraoperative portal pressure measurement at the site of the right colic vein showed a moderate pressure reduction (42.5–31.5 cm H₂O). On the fourth month after operation, gastrointestinal fiberscopy showed no existence of esophageal varices. One year after operation, the patient was doing well without bleeding.     

Adenoma of the gallbladder associated with a gallstone in a child

We describe a rare case of adenoma of the gallbladder associated with a gallstone in a 9-year-old girl who was successfully treated by cholecystectomy. The diagnosis, which was suspected on repeated ultrasonographic examinations of the gallbladder, was confirmed at surgery and histologic examination. To our knowledge, this is the third documented case of adenoma of the gallbladder and the first case associated with a gallstone in childhood. Correspondence to: S. Y. Kim     

Ectopic pancreas associated with a choledochal cyst and extrahepatic biliary atresia

Two children with incidentally-diagnosed ectopic pancreatic tissue in the jejunum at surgery for extrahepatic biliary atresia (EHBA) and choledochal cyst (CC) are reported. No case has been reported in the literature describing the association of a CC with ectopic pancreas, and only one case of EHBA associated with ectopic pancreas has been reported. We believe that incidentally-detected ectopic pancreatic tissue should be excised, even though the patient is symptom-free, in order to prevent the risk of serious complications due to either the mass effect or the potential for acute pancreatitis, cystic degeneration, or malignant transformation at a later date. Accepted: 12 December 2000     

Congenital dilatation of the bile duct: Changes in diagnostic tools over the past 19 years

BACKGROUND: Because of recent improvements in diagnostic procedures, many cases of congenital dilatation of the bile duct (CDBD) have been diagnosed in early life. To determine the mode of presentation and changes in diagnostic tools, medical records of 34 children (25 girls, nine boys) with CDBD who were treated between 1982 and 2000 were reviewed. RESULTS: The age at presentation ranged from 0 to 16 years (median 4.9 +/- 0.7 years), with two patients being diagnosed using prenatal ultrasonography. Of the 32 patients diagnosed post-natally, 21 (66%) complained of abdominal pain. There were also 21 (66%) cases of vomiting. Sixteen patients (50%) experienced both these symptoms. Ten (31%) cases presented with jaundice and three (9%) presented with a palpable mass. Ultrasonography was used as a diagnostic tool in 29 cases (85%). The finding of a dilated common bile duct on abdominal ultrasonography was helpful for early diagnosis. Spiral computed tomography was also a useful and accurate imaging method for diagnosis. Anomalous pancreaticobiliary junction (PBJ) was demonstrated in 44% of patients with CDBD. Magnetic resonance cholangiopancreatography, which can be performed non-invasively, is useful for the diagnosis and pre-operative assessment of CDBD in children. The average length of time from presentation to diagnosis could be shortened from 46 days (1982-1988) to 13 days (1995-2000). CONCLUSION: Recent improvements in diagnostic procedures make immediate, non-invasive diagnosis of CDBD and PBJ possible.     

Acute hydrops of the gallbladder in a child

A 5-year-old girl with acute abdomen was diagnosed as suffering from hydrops of the gallbladder (HGB). She had typical clinical and laboratory characteristics of HGB including ultrasonography and radionuclide butylimidodiacetic acid (BIDA) scan.