Adenovirus Ascending Cholangiohepatitis

Three children, two with liver transplants and one with acquired human immunodeficiency virus (HIV) infection, presented with hepatitis accompanied by elevated gamma glutamyl transpeptidase. Biopsies revealed cholangiohepatitis caused by adenovirus infection. There was a progressive loss of interlobular bile ducts in two of the patients. In one patient, infection of the biliary tree was marked by a necrotizing cholangitis, with adenoviral inclusions noted in the biliary epithelium. In each patient, there was evidence of adenovirus gastrointestinal infection. This is the first report of adenoviral infection of the biliary tree in humans. It is hypothesized that adenovirus cholangiohepatitis occurs as a result of ascending infection from the gastrointestinal tract to the biliary tree.     

Endoscopic biliary drainage management for children with serious cholangitis caused by congenital biliary dilatation

Congenital biliary dilatation (CBD) is usually associated with complications such as recurrent cholangitis, manifested as abdominal pain, vomiting, and jaundice. If cholangitis cannot be controlled by conservative treatment, a good therapeutic effect can be obtained through percutaneous biliary drainage or open T-tube drainage. We aimed to evaluate our experiences in biliary drainage through endoscopic retrograde cholangiopancreatography in children with cholangitis caused by CBD. From January 2014 to December 2017, 167 children with CBD were treated in our hospital. 17 patients (10.18%) with serious cholangitis caused by CBD underwent ERCP. There were 4 males and 13 females with an age range of 10–120 months (average 56.4 months). Placement of a biliary stent was attempted for biliary drainage through endoscopic retrograde cholangiopancreatography. Of the 17 patients studied, 13 children had jaundice and 15 had elevated aminotransferases. ERCP showed CBD in all patients and a common biliopancreatic duct in 12 of 17 patients (70.6%). Five patients underwent nasobiliary drainage and 12 patients underwent biliary drainage through double pigtail tubes. All patients achieved successful biliary drainage. Postoperative pancreatitis occurred in one patient. Biochemical indicators decreased significantly in 12 patients (70.6%) on the second postoperative day. The average length of hospital stay after surgery was 4.5 (range 3–7) days. No major complications related to ERCP were observed and all children had a good prognosis so far. Endoscopic biliary drainage is a safe, simple, and reliable technique. It can be used to resolve CBD-associated cholangitis, evaluate the biliary tract and pancreatobiliary duct junction, and guide pediatric surgeons to choose the right time and the correct procedure for CBD.     

Treatment and prognosis of symptomatic gallbladder disease in patients with cystic fibrosis

Twenty-four (3.6%) of 670 patients with cystic fibrosis seen over a 25-year period developed symptomatic gallbladder disease. Only four patients were less than 16 years old. Four patients presented with unusual problems, including one with acute cholangitis and two with atonic gallbladder, one of whom required cholecystectomy. Another patient was found to have cholangiocarcinoma of the gallbladder when an exploratory laparotomy was performed to investigate biliary obstruction. Twenty patients had cholelithiasis, 15 of whom underwent cholecystectomy. Only one patient had substantial pulmonary difficulties postoperatively. Patients who presented with classic biliary colic had no further symptoms after cholecystectomy. One patient developed intrahepatic stones 6 years later and required a choledochoduodenostomy. As the pulmonary status of most cystic fibrosis patients will eventually deteriorate, we recommend that serious consideration be given to performing a cholecystectomy as soon as practical after the diagnosis of symptomatic cholelithiasis. Our experience indicates that surgery can be performed safely unless pulmonary status is already extremely compromised and the patient is in overt respiratory failure.     

胆道闭锁葛西手术后肝内胆管扩张的治疗与预后分析

目的 探讨胆道闭锁葛西手术后肝内胆管扩张（Intrahepatic biliary cysts,IBCs）的治疗选择和预后情况.方法 回顾性分析8例肝内胆管囊性扩张患儿的临床表现、治疗过程和转归,其中2例单发囊肿型胆管扩张患儿分别予手术和抗感染治疗;6例多发复杂囊肿型胆管扩张患儿均行经皮肝胆管引流术（PTCD）治疗.结果 2例单发囊肿型胆管扩张患儿疗效好,预后良好.6例多发复杂囊肿型胆管扩张患儿经PTCD引流后,2例再次开腹手术吻合;6例患儿中,3例死亡,1例行肝脏移植,1例等待肝移植,1例合并门静脉高压存活.结论 胆道闭锁葛西术后多发肝内胆管扩张（C型）常提示存在严重肝脏损伤,PTCD治疗或手术再吻合治疗多数情况下仅是姑息治疗.PTCD有助于减轻黄疸,改善肝移植术前条件;而无条件进行肝脏移植的患者,单纯PTCD很难长期控制临床症状,应选择再次手术行扩张胆管肠吻合手术.单发肝内胆管扩张（A型）临床过程和预后明显优于C型扩张,可能与肝脏受损程度较轻有关.     

Diagnostic and therapeutic ERCP in the pediatric age group

The role and value of endoscopic retrograde cholangiopancreatography (ERCP) in the pediatric age group is not well established, because pancreatic and biliary diseases are less common in children. This however is not the case in areas like the Eastern Province of Saudi Arabia where sickle cell disease (SCD) and other hemoglobinopathies are common, with increased frequency of cholelithiasis and choledocholithiasis. The purpose of this study was to evaluate the indications, findings, safety and therapies of ERCP in children. One hundred and twenty five children had diagnostic and/or therapeutic ERCP as part of their management at our hospital. Their medical records were reviewed for: age at diagnosis, sex, Hb electrophoresis, indication for ERCP, findings, therapy and complications. There were 77 males and 48 females. Their age at presentation ranged from 5–18 year (mean 13.25 year). The majority of them had sickle cell disease (77.6%). The indications for ERCP were: obstructive jaundice (67.2%), recurrent biliary colic with or without jaundice (10.4%), acute and chronic pancreatitis (7.2%), postoperative bile leak (2.4%), cholangitis with obstructive jaundice (2.4%), hepatitis of unknown etiology (3.2%), cirrhosis of unknown etiology (4%), thalassemia with jaundice (0.8%), hemobilia (0.8%), acute cholecystitis with jaundice (0.8%), and sickle cell disease with ulcerative colitis and obstructive jaundice (0.8%). In six children, ERCP was done following laparoscopic cholecystectomy. ERCP was carried out under sedation in 91 (72.8%) children and under general anesthesia in 34. It was successful in 121 (96.8%) children while cannulation of the Ampulla failed in four. ERCP was normal in 43 children, but eight of them showed evidence of recent stone passage and in six, there were gallstones. In the remaining children, ERCP revealed: normal CBD with stones (18 patients), dilated CBD with stones (17 patients), dilated CBD without stones (19 patients), dilated biliary tree with stones (10 patients), dilated biliary tree without stones (six patients), bile leak (two patients), dilated biliary tree with stones and choledocho-duodenal fistula (one patient), choledochal cyst (two patients), septate gallbladder (one patient), normal ERCP with multiple pancreatic cysts (one patient) and biliary stricture (one patient). The following procedures were carried out: 35 had endoscopic sphincterotomy and stone extraction, 20 had endoscopic sphincterotomy, four had CBD stenting, one underwent removal of a stent, two had insertion of a nasobiliary tube and one had biliary endoprosethesis. There was no mortality. One had bleeding from the site of sphincterotomy which stopped after adrenaline injection. Four patients (3.2%) developed transient mild pancreatitis which settled conservatively. ERCP in the pediatric age group is safe both as a diagnostic and therapeutic procedure. ERCP can provide valuable information which aid in the diagnosis of biliary and pancreatic diseases in children as well as therapy with the technical feasibility of endoscopic sphincterotomy. This is specially so in the era of laparoscopic cholecystectomy, where ERCP should be the treatment of choice in children with CBD stones who are going or have previously undergone laparoscopic cholecystectomy.     

Percutaneous removal of biliary stones post‐liver transplant in a pediatric patient: Case report and review of the literature

This case report describes an 8‐year‐old girl who underwent a segmental LT for a primary diagnosis of citrullinemia at the age of 12 months. She presented with cholangitis secondary to stenosis of the biliary‐enteric anastomosis. MRI revealed dilatation of intrahepatic bile ducts associated with multiple stones. An endoscopic approach failed to decompress the bile ducts and remove the stones. A percutaneous approach was then undertaken. After placement of a temporary external biliary drain for 12 days, a 26 French sheath was placed to access the bile ducts. Using a 14Fr flexible cystoscope, 80%‐90% of the biliary stones were removed. This was followed by antegrade balloon dilatation of the biliary‐enteric anastomosis. Two months later, the procedure was repeated, resulting in complete clearance of the biliary stones. An internal‐external biliary drain was maintained in placed for 10 months. The patient has been asymptomatic, with no evidence of stone recurrence for 13 months after drain removal. Percutaneous biliary stone removal is commonly performed in adults with non‐transplanted livers, especially in complex cases, and has also been shown to be successful in the pediatric population. However, it is rarely reported in transplanted livers in adults, and to the best of our knowledge, no pediatric cases have been reported. This case illustrates that this technique can be successfully utilized in pediatric LT patients.     

Is perioperative cholangiography necessary in children undergoing elective laparoscopic cholecystectomy?

BACKGROUND: The necessity of carrying out pre- or intra-operative imaging of the biliary tree to rule out a possible anatomical abnormality or the presence of common bile duct (CBD) stones in patients undergoing laparoscopic cholecystectomy (LC) is debated. We prospectively assessed the risk of developing symptoms related to bile duct injury or CBD stones after LC in children not receiving peri-operative cholangiography. MATERIALS AND METHODS: All patients 相似文献   

A treatment for biliary hypoplasia: tube cholecystostomy and irrigation.

PURPOSE: Biliary Hypoplasia (BH), characterised by a small ductal system and reduction in the number of interlobular bile ducts, has a bad prognosis. It has been claimed that other treatment methods apart from liver transplantation are not effective. Seven patients with BH underwent tube cholecystostomy, decompression and saline irrigation of the biliary tree via tube cholecystostomy. We present our treatment method, together with the early and late biochemical and histopathological results of these patients. PATIENTS AND METHODS: The records of seven patients with BH were reviewed retrospectively. BH was proved by operative cholangiography. Irrigation was performed intermittently with warm saline to the biliary tract via tube cholecystostomy over two to three months. SGOT, SGPT, alkaline phosphatase (AP) and bilirubin levels were evaluated preoperatively and postoperatively. Histopathological findings were also evaluated. RESULTS: Median age at operation was 46 days (range 20 - 90 days). There were six males and one female. Five patients recovered completely. There was a statistical difference between preoperative and postoperative SGOT, SGPT, AP, and bilirubin levels of patients who recovered (p < 0.05). CONCLUSION: Similar to biliary atresia, many factors such as the patient's age, postoperative bilirubin level, histopathology of liver, treatment method, and cholangitis as a complication of the surgical procedure affect the prognosis of BH. Operation before the age of 70 days, normal bilirubin levels after operation, no cholangitis attack, intracellular and intracanalicular cholestasis, and mild mixed cellular infiltration are favourable factors. We believe that decompression and irrigation of the biliary tract is an effective treatment method for suitable cases of BH.     

Perioperative factors affecting the outcome following repair of biliary atresia

The records of all patients with biliary atresia seen at the Childrens Hospital of Los Angeles during a 14-year period were reviewed. Of the 41 patients who could be evaluated, 32 were treated with trimethoprim and sulfamethoxazole prophylaxis, five were given other agents, and four received no prophylaxis after surgery. At least one episode of cholangitis occurred in nine patients. With one exception, all patients destined to have cholangitis did so within 9 months of surgery. Of the nine patients, five were receiving prophylaxis at the time the disease developed, but two were no longer receiving any prophylaxis. The other two patients in whom cholangitis developed never received antibiotic prophylaxis. In the 24 patients who had a Kasai type of portoenterostomy, cholangitis developed in three of the five (60%) with a Roux-en-Y limb length less than 40 cm and in two of the 19 (10.5%) with limb lengths greater than 40 cm (P less than .02). When performed earlier than 61 days after birth, surgery resulted in adequate bile flow in 64.7% (11/17) of patients who could be evaluated as compared with 31.8% (7/22) for surgery at 61 days or later (P less than .05). Of the patients with adequate biliary drainage 11 had no apparent liver disease, but only two of the patients with poor drainage were free of clinical liver disease. The conclusion from this series is that a combination of timely surgery, intestinal conduit at least 40 cm in length, and subsequent long-term antibiotic prophylaxis favors the best bile flow and reduces the occurrence of cholangitis, resulting in the best outcome.     

Cholangitis associated with cystic dilatation of the intrahepatic bile ducts after antireflux valve construction in biliary atresia

 An intussusception-type antireflux valve (ARV) has been introduced to prevent postoperative ascending cholangitis in the management of biliary atresia (BA). We investigated the characteristics of cholangitis in the management of BA using the ARV in 38 patients who had undergone an operation at our institution; 29 underwent ARV construction at the same time as portenterostomy (PEO) or hepaticojejunostomy. One patient underwent ARV construction for refractory cholangitis with cystic dilatation of the intrahepatic bile ducts (CDIB) long after the PEO. Five of 29 patients who had ARV construction developed CDIB complicated by severe, refractory cholangitis. One or two episodes of mild cholangitis were observed in 5 (20.8%) of 24 patients who did not show CDIB. An ARV created for postoperative recurrent cholangitis associated with CDIB was ineffective. Preoperative cholangitis associated with a type I choledochal cyst and CDIB was observed in 1 patient. In conclusion, the ARV was effective in preventing refractory cholangitis without CDIB, but ineffective in preventing cholangitis with CDIB. Our findings suggest that CDIB resulting from the ongoing process of BA could be a potential target of bacterial infection through other routes than bilioenteric reflux. Accepted: 14 April 2000     

Autoimmune hepatitis as a late complication of liver transplantation

BACKGROUND: The development of de novo autoimmune hepatitis as a long-term complication after liver transplantation has been recently reported. The authors describe five liver allograft recipients who developed chronic hepatitis associated with autoimmune features. METHODS: Five of 155 liver transplant recipients at risk (2.5%) developed this particular form of graft dysfunction. The authors review the clinical records, liver histology, therapy, and outcome of these five patients. RESULTS: Patients included two boys and three girls. Median age at transplantation was 3.5 years (range, 0.5-14 years), median age at presentation was 9 years (range, 2-17 years), and median interval after transplantation was 5.1 years (range, 1.5-9 years). Indications for liver transplant included biliary atresia in four patients and primary sclerosing cholangitis in one patient. At the time of presentation, all patients were receiving cyclosporine as their primary immunosuppressive agent. Only one patient had a history of rejection, which had resolved. All patients presented with increased transaminase levels, and one had a mildly elevated conjugated bilirubin level. Only one patient had constitutional complaints. Acute and chronic rejection, viral hepatitis, vascular insufficiency, and biliary tract obstruction were excluded. Antinuclear antibody levels were elevated in four patients (titer range, 1:160-1:640), one of whom also had positive antismooth muscle antibody (titer 1:80) results. The fifth patient had an elevated serum total protein level. Histologic analysis of liver biopsy samples from the five patients showed findings consistent with chronic autoimmune hepatitis. All patients were treated with standard therapy for autoimmune hepatitis, which included daily steroids and azathioprine. Cyclosporine doses were reduced in three patients and eliminated in two. All patients responded with normalization (n = 2) or improvement (n = 3) of liver transaminases within the first 3 months of therapy. Histologic analysis of the 3-month follow-up liver biopsy was normal (n = 2) or showed improvement in inflammation (n = 2). Two patients developed acute allograft rejection within 6 to 12 months after discontinuation or reduction in cyclosporine. CONCLUSIONS: Autoimmune hepatitis occurs after liver transplantation in patients without a previous history of autoimmune hepatitis. The risk of developing autoimmune hepatitis appears to be greater in children after liver transplantation than in the general pediatric population. Standard therapy for autoimmune hepatitis is effective.     

Kasai术后肝内胆管囊性扩张的诊治与预后

目的 总结胆管闭锁术后肝内胆管囊性扩张的诊治经验,分析其对患儿预后的影响.方法 1998年6月至2008年3月,对胆管闭锁行葛西手术的患儿进行追踪随访.通过超声检查,发现8例患儿存在肝内胆管扩张.其中3例再行MRI检查,6例再行CT平扫加增强检杳以明确诊断.此8例患儿术后均有不同程度胆管炎症状.5例行PTCD显示肝内胆管囊性扩张.3例患儿行囊肿与空肠胆支再吻合术,2例患儿仪行PTCD置管引流,2例患儿暂未予任何处理.结果 3例经手术治疗的患儿,术后黄疸消退或减轻.仅行PTCD的患儿肝内胆管扩张长期存在.结论 对反复发作的胆管炎应定期行超声检查,尽早发现肝内囊肿;对肝内胆管囊性扩张,无论其影像学分型如何,应根据其具体临床表现进行相应的积极治疗;PTCD无法长期放置,仅是暂时性的治疗,但对手术时寻找囊肿有指示作用,使手术时囊肿的定位相对容易;囊肿的大小、位置与门静脉的关系,压迫的时间、治疗是否及时有效都可影响患儿的预后.     

Congenital dilatation of extrahepatic bile ducts in children. Experience in the central hospital of Hue, Vietnam.

BACKGROUND/PURPOSE: The authors present a study of a series of cases in children with congenital dilatation of the extrahepatic bile ducts (CDEBD). METHODS: Between November 1998 and October 2002, 38 children aged between 50 days and 15 years suffering from CDEBD, admitted to the Central Hospital in Hue, Vietnam, were treated surgically with a minimum follow-up of one year. RESULTS: Diagnosis was based only on ultrasonography which was 100% accurate. According to Miyano's classification, 26 cases presented as cystic dilatation of the main bile duct (MBD) associated with dilatation of the intrahepatic bile ducts, while in the other 12 cases the dilatation of the MBD was of the fusiform type. The surgical treatment of choice was extensive excision of the dilatated extrahepatic bile ducts and biliary drainage according to the Roux-en-Y method in 36/38 patients. The other two patients underwent surgery for internal biliary drainage without removing the cysts. During follow-up we observed one case of pancreatitis out of the 36 patients who underwent excision of the dilatated extrahepatic bile ducts, while the two patients who had internal biliary drainage without removal of the cysts suffered from numerous attacks of cholangitis. CONCLUSIONS: The reflux of bile in the dilated biliary tree plays an important role in the etiopathogenesis of CDEBD. Excision of the extrahepatic bile ducts and internal biliary drainage by Roux-en-Y has proved a satisfactory surgical method.     

Biliary atresia with a “cyst at porta”: management and outcome as per the cholangiographic anatomy

The purpose of this study is to classify biliary atresia (BA) with a "cyst at porta" according to the cholangiographic anatomy and to define management strategy and outcome in each group. A cyst at porta was identified in 13 of 58 babies (22.4%) with BA at first presentation. The cholangiographic anatomy was classified as; Group A (n = 7), type III BA with extrahepatic cyst; Group B (n = 2), type I or II BA with extrahepatic biliary cyst; and Group C (n = 4), type I or II BA with both extrahepatic and intrahepatic biliary cysts. The remaining 45 patients were comprised of type III BA without a cyst. A Kasai's portoenterostomy (PE) was performed for all Group A patients. Groups B and C were treated by hepaticojejunostomy (n = 5) or portoenterostomy (n = 1). All 45 patients with type III BA without a cyst were treated by a Kasai's PE. The median age at surgery was 92 days (ranges 28-342 days). There were three early post-operative deaths, all in patients with type III BA without cyst. Overall 18/55 (32.7%) patients achieved a jaundice free state. In Group A, 5/7 (71.4%) patients had bile flow, 2/7 (28.6%) are anicteric and 2/7(28.6%) had 1-2 episodes of post-operative cholangitis. In Group B, both patients are anicteric and none had post-operative cholangitis. In Group C, all four babies had bile flow but, significant morbidity because of recurrent severe cholangitis. Only one patient reached a jaundice free state. Of the remaining 42 patients with type III BA without a cyst, 27 (64.3%) had bile flow, 13 (31%) became jaundice free and 14 (33.3%) have had 1-2 episodes of post-operative cholangitis. In conclusion, thirteen of 58 (22.4%) babies with BA had a "cyst at porta" at first presentation in this series. The outcome was most satisfactory in type I BA without intrahepatic cystic dilatation (Group B) in terms of achieving a jaundice free state and freedom from recurrent cholangitis. However, intrahepatic biliary cysts (Group C) were associated with recurrent severe cholangitis and a poor eventual outcome despite a good initial bile flow. The outcome in type III BA with extrahepatic cyst was comparable to type III BA without cyst.     

Recurrent intrahepatic pigmented stones after liver transplantation in a patient with hemoglobin SC disease: case report and review of the literature

Patients with hemoglobinopathies may have hepatic involvement, which if severe, can lead to chronic liver disease and a need for liver transplant. Here, we present a case of a 16-yr-old female adolescent who presented to our center with hemoglobin SC disease, obstructive jaundice because of pigmented intrahepatic biliary stones, and progressive liver disease. She underwent a successful liver transplant but a few years later, she developed recurrent cholangitis and graft dysfunction because of recurrent intrahepatic biliary stones. Recurrent formation of intrahepatic stones after liver transplant is a rare and severe complication in patients with hemoglobinopathies. We recommend hypertransfusion therapy and surveillance imaging studies after liver transplant for early detection and prevention of this complication.     

Chronic sclerosing cholangitis and recurrent pulmonary infections in two brothers associated with cellular immunodeficiency and increased cytokine production

Primary sclerosing cholangitis has occasionally been associated with congenital immunodeficiency. Recently, sclerosing cholangitis has been found in the course of AIDS and cryptosporidium or cytomegalovirus have been observed chronically in the biliary tract, suggesting their role in the evolution to sclerosing cholangitis. We report on 2 brothers with immunodeficiency without any evidence of AIDS, who showed sclerosing cholangitis and recurrent pulmonary infections in the course of their disease. No bacterial microorganism could be cultured from the biliary juice. The serum of both patients contained measurable levels of TNFα. Supernatants from cultured peripheral blood mononuclear cells of the two brothers contained higher amounts of tumor necrosis factor (TNFα), interleukins 1α and 1β than did supernatants from cultures of normal individuals. The role of cytokines as an aggravating factor in immunodeficiency is discussed as well as their role in the inflammatory process.     

Intrahepatic biliary cysts after hepatic portoenterostomy in four children with biliary atresia

We report our experience with 4 cases of cystic dilatation of intrahepatic bile ducts following hepatic portoenterostomy for biliary atresia. Two of the cases did not achieve satisfactory bile excretion and all four cases developed recurrent cholangitis after hepatic portoenterostomy. The attacks of cholangitis seemed to be associated with the presence of intrahepatic cysts. Although one case resulted in death from hepatic failure, three other cases are now outpatients. Patients who develop recurrent cholangitis following hepatic portoenterostomy, should be examined to exclude the presence of intrahepatic biliary cysts. Ultrasonography, computed tomography and percutaneous transhepatic cholangiography were all effective in detecting cysts and provided valuable information for planning treatment. Percutaneous transhepatic or surgical drainage of the bile ducts was effective in reducing jaundice, and recurrent cholangitis.     

Polymerase chain reaction-based diagnosis of infection with Cryptosporidium in children with primary immunodeficiencies

BACKGROUND: Patients with deficient cell-mediated immunity are prone to chronic biliary tract infection with Cryptosporidium, which can lead to the development of sclerosing cholangitis and acute cryptosporidiosis after bone marrow transplantation (BMT). The organism is very difficult to detect during asymptomatic periods. METHODS: PCR techniques were compared with standard microscopy for detecting the organism in such patients. Amplification targets were two fragments of the 18S ribosomal RNA gene (unnested) and part of the Cryptosporidium oocyst wall protein gene (nested and unnested). Twenty eight-patients with primary immunodeficiencies were studied including: CD40 ligand deficiency (13); undefined combined immunodeficiency (10); major histocompatibility complex II deficiency (2); and other defects (3). Samples analyzed included stool, bile and liver tissue. RESULTS: Of 25 patients tested prospectively, Cryptosporidium could be detected by PCR but not by microscopy in 12, only 3 of whom had a known history of infection. Five of this group had sclerosing cholangitis. Nine of the PCR-positive patients subsequently underwent BMT and 5 developed acute posttransplant diarrhea and cholangiopathy associated with Cryptosporidium excretion. Of the 13 PCR-negative patients, 3 had cholangiopathy (sclerosing cholangitis in 1 and minor changes in 2). Four of these underwent BMT and none developed cryptosporidiosis. In 3 patients, studied only after developing post-BMT cholangiopathy and diarrhea, Cryptosporidium was detected by PCR but not by microscopy. Genotyping and sequencing showed multiple types of Cryptosporidium in approximately one-third of positive cases. CONCLUSIONS: These results indicate that PCR-based procedures are more sensitive than microscopy for detecting Cryptosporidium in patients with immunodeficiencies.     

Biliary tract dilatation and abnormal choledocho-pancreatic ductal junction in children

Nine children aged between 2 months and 11 years, who had congenital dilatation of extrahepatic biliary tract, with or without intrahepatic dilatation, are presented. The radiological studies performed in eight patients demonstrated abnormal choledochopancreatico ductal junction. Thus, a long common channel is formed out of the duodenal wall, leading to a continuous reflux of pancreatic juice into the biliary system, chronic inflammation of biliary tract wall, fibrosis of muscular layer and finally different types of dilatations. The main clinical findings were upper abdominal pain, vomiting, or obstructive jaundice. In only one child the dilatation was cystic type and the clinical manifestation was palpable abdominal mass. In eight patients the initial diagnosis of biliary tract dilatation was made through abdominal ultrasound and in one case by direct visualization through emergent exploratory laparotomy. The final confirmation of the presence of long common channel was made by preoperative endoscopic retrograde cholangiopancreatography or intraoperative cholangiogram. The radiologic investigation was not performed in only one case due to bad clinical conditions. The surgical treatment indicated to all cases was Roux- en- Y hepatic- jejunostomy. The late follow up demonstrated that all patients are well and assymptomatic.     

Long-term prognosis of patients with extrahepatic biliary atresia successfully treated with surgery. Our experience

The long-term prognosis of extrahepatic biliary atresia after surgical restoration of bile flow is still controversial. An ongoing process of cirrhosis and the development of portal hypertension continue to create frequent and frustrating management problems. Clinical features, hepatic function, echotomography aspect, calcium-phosphorus metabolism and serum levels of 25-OH-D-3 were evaluated in 12 anicteric patients with extrahepatic biliary atresia successfully treated in a period from 1974 through 1987. Seven of these children had a total of 21 episodes of cholangitis. In five patients liver biopsy, obtained at the time of the external diversion closure, showed a biliary cirrhosis. Growth, development and hepatic function were normal in all children studied; one patient had esophageal varices. The serum levels of 25-OH-D3 in patients without oral supplementation of vitamin D are lower than normal. This deficit can be corrected by oral administration of vitamin D. Our study revealed that the children with successful portoenterostomy appeared to thrive normally and that they tolerated the relatively mild liver damage. We believe that Kasai operation should be done in all patients with extrahepatic biliary atresia and that the liver transplantation is to be reserved only in those with unsuccessful Kasai. In our experience external diversion was not useful to prevent cholangitis and moreover it complicates the hepatectomy in case of transplantation.