Pre-operative Non-Invasive Imaging for Neonatal Cholestasis in a Child with Extrahepatic Biliary Atresia

@#Hepatobiliary iminodiacetic acid (HIDA) scintigraphy is a non-invasive, functional imaging of the hepatobiliary system that serves as an adjunct imaging modality for neonatal cholestasis work-up. In view of the urgency to diagnose biliary atresia and restore bile flow through surgery, HIDA scintigraphy could help to distinguish between neonatal cholestasis due to biliary atresia and neonatal hepatitis of various causes. We describe a full-term male infant with jaundice beyond the physiological period in which HIDA scintigraphy showed absent tracer excretion from the biliary system into the intestines up to 5 hours on follow-up imaging. The intraoperative diagnosis confirmed the diagnosis of biliary atresia. The prognosis of the patient with biliary atresia depends on early surgical planning and intervention. Therefore, non-invasive diagnostic tools play an important role in the evaluation of a child with neonatal cholestasis.

Clinical Profile and Outcome of Young Patients with Extrahepatic Biliary Obstruction at A High-Volume Tertiary Care Centre in Northern India

Background and Aim: The etiology of extrahepatic biliary obstruction (EHBO) ranges from benign disorders to pancreaticobiliary malignancy. We studied the demographic, clinical, laboratory, and endoscopic features of young patients with EHBO undergoing Endoscopic retrograde cholangiopancreatography (ERCP). Methods: We retrospectively analyzed one-year data of ERCP procedures performed on adult patients under 40 years of age. The diagnosis was based on a clinical and radiological basis with histopathological confirmation. Results: A total of 180 patients were included with a mean(±SD) age of 33.4(±5.8, range: 15-40) years, and 67.8% being female. Benign (67.2%) findings included choledocholithiasis (57.8%), benign biliary stricture (9.44%, post cholecystectomy stricture 82.3%), and malignant (32.8%) causes were gallbladder carcinoma (24.4%), cholangiocarcinoma (4.4%), periampullary carcinoma (2.8%), pancreatic head carcinoma (1.1%). Clinical presentation included jaundice (66.1%), pain abdomen (59.4%), pruritis (26.1%), weight loss (19.4%), anorexia, fever, and cholangitis (24.44%). Mean bilirubin levels (16.9 ±6.8 vs 4.6 ±4.1 mg/dl) and alkaline phosphatase (1170 ± 260.7 vs 439.3 ± 362.7 IU/mL) were higher in malignant causes, in comparison to benign. Stone retrieval using balloon during ERCP was successful in 79% of cases. Large (>15 mm) or impacted stones or those with biliary stricture failed stone extraction. In gallbladder carcinoma, adequate endoscopic biliary drainage was achieved in 68% of patients. Endoscopic biliary drainage was achieved in 75%, 80%, and 50% cases of cholangiocarcinoma, periampullary carcinoma, and pancreatic head carcinoma, respectively. Conclusion: Choledocholithiasis and gallbladder carcinoma are the most common benign and malignant causes of EHBO in young patients. The successful endoscopic therapeutic intervention could be achieved in most patients.

Laparoscopic classification and treatment of extrahepatic biliary dilatations / 中华肝胆外科杂志

Objective To establish a laparoscopic classification of extrahepatic biliary dilatations (EHBD) that can guide minimally invasive treatment.Methods According to inclusion criteria,124 patients with EHBD who were admitted and treated from July 2001 to July 2017 in the First Hospital Affiliated to Army Military Medical University were included in this study.A new laparoscopic classification of EHBD was proposed based on the preoperative imaging data and laparoscopic findings of the position and extent of EHBD.The minimally invasive diagnosis and treatment strategies were made based on the new classification.Results According to the preoperative imaging data and intraoperative laparoscopic findings,124 patients with EHBD were divided into the following groups:type A (upper segment,34 cases),type B (middle segment,27 cases),type C (lower segment,20 cases),and type D (entire bile duct,43 cases).The clinical symptoms (abdominal pain,jaundice and mass) and reoperation rates were not significantly different among the 4 groups(both P＞0.05).The incidences of comorbidities (calculus or inflammation) were significantly different (P＜0.05).The operative time(type A:237.6±66.7 min,type B:259.2±60.0 min,type C:286.1 ± 74.7 min,type D:347.5±94.4 min) and blood loss (type A:192.6±102.2 ml,type B:201.5±120.2 ml,type C:297.5±162.1 ml,type D:305.8±237.3 ml) were significantly different among the groups (P＜ 0.05).The short-term complication rates after surgery (5.9％ ～ 20.0％) were significantly different (P＜ 0.05),while the long-term complication rates after surgery (7.4％ ～ 10.0％) were not significantly different.The conversion rates to open surgery were significantly higher in patients with type C and D lesions than in those with type A and B lesions (P＜0.05).Conclusion This laparoscopic classification predicted the difficulty of laparoscopic surgery for EHBD and had a guiding significance in the minimally invasive treatment for this disease entity.

The research on diagnosis and treatment of intrahepatic and extrahepatic biliary stones / 中国医师杂志

The clinical and fundamental research for intrahepatic and extrahepatic biliary stones were still inadequate currently.It was difficult for treat and has the following clinical features:extensive he-patic lesion,complex conditions,more complications,higher recurrence rate,etc.Satisfactory results were hard to gained if just depend on conventional surgery recently.As the methods of diagnosis and treatment are continuously increasing:Ultrasound,CT,MRCP,Choledochoscope,Cholangiography and 3D imaging of hepa-tobiliary system.From the traditional open operation to a variety of minimally invasive treatment.Different examination and treatment methods has its advantages and disadvantages.How to choose the effective,less trauma,appropriate pathway method is the main direction for the current research.

Right Posterior Segmental Hepatic Duct: Role of Intraoperative Cholangiogram to Prevent Extrahepatic Biliary Duct Injuries

Extrahepatic bile ducts constitute a significant anatomic site for surgeons when performing hepatobiliary operations from minor procedure such as cholecystectomy to major operation such as hepatectomy. The challenges for surgeon in performing such cases increase when congenital variance of biliary tree occurs which may lead to unintentional bile duct injuries. We reported a case of a lady who presented with obstructive jaundice secondary to huge distal common bile duct stone. The presence of right posterior segmental hepatic duct, which rarely occurs, was discovered during intraoperative cholangiogram before common bile duct exploration. The operation was successful without incidence of biliary duct injury

Value of hepatobiliary scintigraphy combined with total bile acid and γ-glutamyltransferase detection in etiological diagnosis of the persistent jaundice in infants / 中华实用儿科临床杂志

Objective To investigate the value of hepatobiliary scintigraphy combined with total bile acid (TBA) and γ-glutamyhransferase(γ-GT) detection in the differential diagnosis of persistent jaundice induced by infantile hepatitis syndrome(IHS) and congenital extrahepatic biliary atresia(EHBA).Methods A retrospective analysis of 60 infants with persistent jaundice undertaking 99Tcm-diethylacetanilide iminodiacetic acid (EHIDA) hepatobiliary scintigraphy was done in Nanfang Hospital by single photon emission computed tomography(SPECT).Meanwhile,these infants' sera were collected and separately detected by AU5431 automatic biochemical assay;the sensitivity,specificity and accuracy of hepatobiliary scintigraphy with TBA and γ-GT were evaluated.Results The sensitivity to 99Tcm-EHIDA hepatobiliary scintigraphy in the diagnosis of IHS and EHBA were 100.00％ (17/17 cases) and 67.57％ (25/37 cases),the specificity was 67.57％ (25/37 cases) and 100.00％ (17/17 cases),and the accuracy was 77.78％ (42/54cases) and 77.78％ (42/54 cases),respectively.The levels of TBA and γ-GT were higher in infants with EHBA than those with IHS(U =209.0,19.5,all P ＜0.05),and ROC curve analysis indicated that TBA in the IHS group and γ-GT in EHBA group had some diagnostic value[area under curve (AUC) =0.736,0.968,respectively].99Tcm-EHIDA hepatobiliary scintigraphy combined with TBA and γ-GT analysis suggested when intestinal non-radioactive imaging was shown,TBA was 98.5 μmol/L and γ-GT was 298 U/L,the sensitivity,specificity and accuracy of diagnosis of EHBA were 100.00.00％ (17/17 cases),100.00％ (37/37 cases) and 100.00％ (54/54 cases) in a serial test.Conclusions Hepatobiliary scintigraphy combined with TBA and γ-GT examination can effectively identify EHBA and IHS earlier,noninvasively and safely,which have important role in further treatment in infants with persistent jaundice.

Intraductal Papillary Mucinous Neoplasm with Associated Invasive Carcinoma Arising from Remnant Cystic Duct: A Case Report

Intraductal papillary mucinous neoplasm (IPMN) involving the biliary system is rare. To date, only a few cases of IPMN arising from the extrahepatic bile duct have been reported. In our case, extrahepatic IPMN arose in the remnant cystic duct after cholecystectomy, and to the best of our knowledge, this is the first report in the remnant cystic duct after cholecystectomy. A 74-year-old woman was referred for right upper quadrant pain lasting one day. Ultrasonography (US) showed a lobulated and hyperechoic mass with an outer linear hypoechoic lesion located adjacent to the dilated common bile duct. Contrast enhanced computed tomography showed a heterogeneously enhancing mass. Magnetic resonance imaging (MRI) showed a heterogenous mass with an outer semicircular high signal portion indicating remnant cystic duct.

Ultrasonography of Neonatal Cholestasis

Ultrasonography (US) is as an important tool for differentiation of obstructive and non-obstructive causes of jaundice in infants and children. Beyond two weeks of age, extrahepatic biliary atresia and neonatal hepatitis are the two most common causes of persistent neonatal jaundice; differentiation of extrahepatic biliary atresia, which requires early surgical intervention, is very important. Meticulous analysis should focus on size and configuration of the gallbladder and anatomical changes of the portahepatis. In order to narrow the differential diagnosis, combined approaches using hepatic scintigraphy, MR cholangiography, and, at times, percutaneous liver biopsy are necessary. US is useful for demonstrating choledochal cyst, bile plug syndrome, and spontaneous perforation of the extrahepatic bile duct.

Tratamento endoscópico das lesões biliares / Endoscopic treatment of the biliary injuries

As técnicas cirúrgicas convencionais ofertam uma apropriada condição de cura na maioria dos pacientes com estreitamento biliar benigno. Nesta condição, no entanto, o reparo cirúrgico está associado com recorrência tardia da re-estenose em 10 por cento a 30 por cento dos pacientes. Neste contexto, os avanços tecnológicos na endoscopia terapêutica promoveram a possibilidade alternativa do tratamento efetivo destas obstruções benignas. Considerações em relação ao tempo de reospitalização e de procedimentos devem ser averiguados em detalhes e ponderados em relação á cirurgia. Estenoses malignas estão relacionadas á colangite, icterícia e dor e, consequentemente, com as alterações sistêmicas relacionadas com a sepsis biliar. A conduta endoscópica cria uma derivação do suco biliar para o duodeno, sendo uma verdadeira derivação biliodigestiva endoscópica e utilizando-se próteses plásticas ou metálicas. O propósito desta revisão é ofertar aos leitores a eficácia do tratamento endoscópico na estenose benigna e maligna biliopancreática.

Standard surgical techniques offer a good chance of cure forthe majority of patients affected by extrahepatic benign biliarystricture. Nevertheless, operative repair has a long-termrecurrence rate of stricture in 10 percent to 30 percent of patients. Advancesin endoscopic procedures have provided alternative options ofrelieving biliary obstructions, but prolonged length of treatmentand rehospitalization have to be considered if endoscopy isperformed. Malignant stenoses are an important factor determining cholangitis, jaundice and pain and it's each systemic consequences. The endoscopic approach intends to create a deviation of bile juice to duodenum by means of metal or plastic stents aiming a safe palliation. The purpose of this review is to show to the readerseffectiveness of endoscopic treatments of benign and malignat stenosis of the bile duct and pancreatic.

Clinical significance of preoperative magnetic resonance cholangiopancreatography in the evaluation of anatomic variants of the extrahepatic biliary tract / 中华消化外科杂志

Objective To evaluate the feasibility and clinical significance of magnetic resonance cholangiopancreatography (MRCP) in the diagnosis of anatomic variants of the extrahepatic biliary tract. Methods The MRCP images of 535 patients who were admitted to The Second Affiliated Hospital of Suzhou University from March 2008 to March 2009 were retrospectively analyzed by two reviewers independently. The anatomic variants of the extrahepatic biliary tract were evaluated by studying the T2-weighted magnetic resonance images. Results The consistency between the two reviewers in the diagnosis of low choledochal joint, medial cystic duct insertion, aberrant hepatic duct, accessory hepatic duct and other anatomic variants was 98% , 99% , 89% , 90% and 100%, respectively (K = 0.86, 0.93, 0.81, 0.82, 1.00). The extrahepatic biliary system was clearly displayed by MRCP in 500 patients, and anatomic variants were observed in 240 patients, including low choledochal joint in 85, parallel cystic duct in 37, medial cystic duct insertion in 82, aberrant hepatic duct in nine, accessory hepatic duct in 10, choledochal cyst in three and high cystic duct in seven. A total of 259 patients underwent operation, and 168 patients had anatomic variants of the extrahepatic biliary tract. Conclusion MRCP imaging enables the accurate assessment of anatomic variants of the extrahepatic biliary system and avoids bile duct injury.

Efficacy of endoscopic papillary balloon dilation in the treatment of non-dilated extrahepatic biliary stones / 中华消化外科杂志

Objective To investigate the efficacy of endoscopic papillary balloon dilation (EPBD) and endoscopic sphincterotomy (EST) in the treatment of non-dilated extrahepatic biliary stones. Methods The clinical data of 317 patients with non-dilated extrahepatic biliary stones who were admitted to the Taiyuan Iron and Steel Corporation Hospital from January 1999 to April 2008 were retrospectively analyzed. Of all patients, 119 received EPBD treatment, and the remaining 198 received EST treatment. Sixty-two patients treated by EPBD (EPBD group) and 62 by EST (EST group) were selected to conduct the matched-pair analysis. In the EPBD group, a cylindrical balloon was inserted under the guidance of a guidewire after the conclusive diagnosis by endoscopic retrograde cholangiopancreatography. After the dilation, stones were extracted by a stone basket or a balloon, and then conventional endoscopic nasobiliary drainage was carried out. In the EST group, stones were extracted by a stone basket or a balloon under the monitor of the digital subtraction radiography after incision of the duodenal papilla. The efficacy of the two methods was compared using the chi-square test. Results The stonefree rate in one session was 92% (57/62) in the EPBD group and 94% (58/62) in the EST group, with no significant difference between the two groups ( χ 2 = 0.222, P > 0.05 ). The number of patients who were complicated with hyperamylasemia and pancreatitis was slightly greater, and the number of patients who were complicated with duodenal perforation was smaller in the EPBD group than those in the EST group, while there was no significant difference between the two groups ( χ 2 = 0. 286, 1. 000, 2.000, P > 0.05 ). The numbers of patients who were complicated with duodenal papilla bleeding or biliary infection in the EPBD group were significantly smaller thanthose in the EST group ( χ 2 = 4. 000, 7. 000, P < 0.05 ). All patients were followed up for 24 months. The recurrence rate of bile duct stone and the incidence of biliary infection in the EPBD group were significantly lower than those in the EST group at the end of 6, 12 and 24 months (χ2 =4. 000, 5. 000, 6.000; 6.000, 8.000,11. 000, P < 0.05 ). Conclusions Combined application of EPBD and ENBD has the advantages of minor trauma and quick recovery of patients. It can preserve the function of duodenal papilla, so it is the first choice for treating non-dilated extrahepatic biliary stones.

Reversal of nail changes after liver transplantation in a child.

We report a 6-year-old girl who received a left-lobe live-related liver transplant for decompensated liver disease after a failed Kasai’s surgery for biliary atresia. Preoperatively, her nails were white, dystrophic, brittle with severe onycholysis, clubbing and watch-glass deformities. Nail scrapings were negative for fungus. Five months after transplantation, her nails had become near normal. There is only one such documented case in literature on reversal of nail changes in an adult.

The development of intrapulmonary arteriovenous shunts as a poor prognostic factor following surgery for biliary atresia.

Background & Aims: Chronic liver disease requiring liver transplantation is a common occurrence following corrective surgery for extrahepatic biliary atresia (EHBA). The formation of intrapulmonary arteriovenous shunts (IPS) is a well-known feature of chronic liver disease. The aim of this study was to investigate the development of IPS and its prognostic significance in postoperative patients with EHBA. Methods: Fourteen patients who underwent Kasai’s portoenterostomy during 1993-2005 were included in the study. The clinical features, hepatobiliary scintigraphy and biochemical liver function tests were recorded. A transthoracic contrast enhanced echocardiogram using a four-chamber view was performed in all patients within a week of the Kasai’s procedure by injecting 5 mL of hand-agitated saline solution into a peripheral vein. The opacification of microbubbles in the left atrium 3-6 minutes after their emergence in the right atrium was considered diagnostic of IPS. The contrast enhanced echocardiogram was repeated 6 months after the Kasai’s procedure in all patients Results: Nine patients were clinically asymptomatic after surgery. HIDA scan was excretory in all 14 patients at the time of the study, although 5 patients were jaundiced. The serum bilirubin increased in 2 patients after surgery; both these patients were jaundiced and developed ascites and 1 expired one year after surgery. Contrast enhanced echocardiogram was negative for IPS in all 14 initially. In the follow-up evaluation the only patient who died was the one who had developed IPS. Conclusions: There is a risk of developing IPS following a failed Kasai’s portoenterostomy in patients of EHBA. Contrast enhanced echocardiography can be used to serially monitor these patients for early detection of this complication. It can be used to predict prognosis after hepatic portoenterostomy and can be extremely useful in selecting patients who will need liver transplant on priority.

A Case of Congenital Agenesis of the Gallbladder without Biliary Atresia associated with Duodenal Web

Congenital absence of the gall bladder without extrahepatic biliary atresia is an extremely rare congenital malformation with a reported incidence ranging between 0.01 and 0.02%. It is thought to be occurred from failure of the gallbladder bud to develop in utero and frequent association with other malformations. Agenesis can be randomly discovered at autopsy or operations for symptoms suggestive of biliary tract disease. The authors report a case of agenesis of the gallbladder without extrahepatic biliary atresia in a neonate which was incidentally found at laparotomy for presumed duodenal obstruction, with a review of the literature.

Biliary Atresia with Extrahepatic Biliary Cyst

Biliary atresia (BA) with extrahepatic biliary cysts (EHBC) is a rare disease. It has been generally recognized as type I (correctable with cystic dilatation), which means a good prognosis. From a total of 73 patients with BA who underwent operation from September 1988 to September 2003 at our institute, 7 (9.6 %) cases of type III BA with EHBC (uncorrectable with cystic dilatation) are reviewed. Clinical findings, laboratory data, radiologic findings, treatment methods and outcomes were reviewed. Female was more prevalent (male to female ratio; 2:5). All cases were type III with EHBC according to the intraoperative cholangiography, and underwent Kasai's portoenterostomy. The mean age was 57 days at operation. Three patients(42.9 %) are long term survivors. Further evaluation is needed to determine the correlation between prognostic factors and outcome for.

Lesão complexa da via biliar principal: a ligadura como opção associada a colecistojejunostomia: [relato de caso] / Common bile duct injury: ligation and cholecystojejunostomy as surgical option: [case report]

The authors present a case of distal common bile duct injury. Ligation of the bile duct and a bypass cholecystojejunostomy were chosen as treatment. Diagnosis of blunt traumatic injury to the extrahepatic biliary ducts may be difficult due to the benign nature of initial bile peritonitis. Surgical treatment for associated abdominal injuries usually makes the diagnosis possible. One of the challenges in the treatment of these injuries relates to the small diameter of the, usually, normal common bile duct. Primary repair and T tube drainage is the best option for non-complex injuries. End-to-end anastomosis and, preferentially, biliary-enteric anastomosis are the best surgical options for more complex injuries. Severe injuries have high complication rates, especially when the distal portion of the common bile duct is affected. Early leaks and late strictures are likely to develop in these situations. Cholecistojejunostomy and ligation of the injuried common bile duct are good surgical options for complex injuries. They carry a low complication rate and consequently low morbidity.

The Correlation between Acholic Stool and the Result of Tc(99m) DISIDA Hepatobiliary Scintigraphy and Biochemical Test in Neonatal Cholestasis / 대한소아소화기영양학회지

PURPOSE: The most common causes of neonatal cholestasis are neonatal hepatitis (NH) and extrahepatic biliary atresia (EHBA). Since neonatal cholestasis presents with variable expression of same pathologic process and has similar clinical, biochemical, and histologic features between EHBA and idiopathic neonatal hepatitis (NH), differential diagnosis is often difficult. We reviewed the differences of clinical characteristics and laboratory data to find out any correlation between the results of Tc(99m) DISIDA scan and presence of acholic stool. METHODS: Between June 1993 and January 2001, total 29 infants younger than 4 month-old underwent Tc(99m) DISIDA scan. Their biochemical tests and clinical course were reviewed retrospectively. RESULTS: Patients who had negative intestinal activity on Tc(99m) DISIDA scan showed acholic stool and revealed higher serum direct bilirubin and urine bilirubin level. 18.2% of patients with acholic stool showed intestinal activity on Tc(99m) DISIDA scan and 81.8% of them did not. All the patients without acholic stool showed positive intestinal activity on Tc(99m) DISIDA scan. The result of Tc(99m) DISIDA scan and the presence of acholic stool showed high negative correlation (r :-0.858). Patients with acholic stool and negative intestinal activity on Tc(99m) DISIDA scan showed higher serum total bilirubin level. Patients without acholic stool and positive intestinal activity on Tc(99m) DISIDA scan showed higher serum level of ALT. CONCLUSION: Patients with acholic stool and negative intestinal activity showed high correlation, but 18.2% of patients with acholic stool showed positive intestinal activity. So operative cholangiogram or transcutaneous liver biopsy should be performed for confirmation.

Atresia of the extrahepatic biliary system (a report and review)

A case report of a complete atresia of the extra hepatic biliary system is made and a brief review of the many aspects involved in the management of the case is given. An early diagnosis should be the objective as early as possible during the first six months of life, if we are to offer a chance of salvage to the newborn. Unfortunately, it is not always so attained. A dilemma in the diagnosis arises such that an early diagnostic laparotomy is indicated. (Summary)

Clinical and Pathological Comparison of Neonatal Hepatitis and Extrahepatic Biliary Atresia in Korean Children

PURPOSE: Neonatal hepatitis and congenital extrahepatic biliary atresia are two major causes of neonatal cholestasis. But the method of therapeutic trials used for each disease is essentially different. Nonetheless, it is very difficult to differentiate these diseases clinically and histologically. This study is aimed to find out major differences between the two by clinical characteristics and scoring of various histological parameters. METHODS: Clinical and histologically assessments were carried out in 8 cases with neonatal hepatitis and 11 cases with extrahepatic biliary atresia, who were admitted to the Department of Pediatrics, Pusan National University Hospital, from January 1991 to June 1997. RESULTS: By sex distribution, males were more commonly had neonatal hepatitis but females were more commonly had biliary atresia. Hepatosplenomegaly and acholic stool were more frequent in biliary atresia.. Serial determinations of serum bilirubin concentrations showed that a steady fall occured in neonatal hepatitis whereas, progressive increase or stability of bilirubin level was noted in biliary atresia. Serum direct bilirubin level of more than 4mg/dL was found more frequently in biliary atresia. Serum aspartate aminotransferase level above 400IU/L was found more frequently in biliary atresia. Bile duct proliferation was more frequent in biliary atresia but Kupffer cell proliferation was more frequent in neonatal hepatitis. There was a significant difference in the total score in the liver biopsy scoring system between the two diseases. CONCLUSION: Females with hepatosplenomegaly and acholic stool, serum direct bilirubin level higher than 4mg/dL, serum aspartate aminotransferase level above 400IU/L, prominent bile duct proliferation and a higher total pathological score in biopsy specimen was found more frequently in biliary atresia.