Significance of the anomalous arrangement of the pancreaticobiliary duct in the etiology of biliary atresia.

BACKGROUND/PURPOSE: The anomalous arrangement of the pancreaticobiliary duct (AAPBD) is one theory used to explain the etiology of biliary atresia. We investigated whether AAPBD could be involved and evaluated its significance for the etiology of biliary atresia. MATERIALS AND METHODS: Of 43 patients with biliary atresia, the area between the common bile duct and the duodenum could be visualized by operative cholangiogram in 5 patients with an uncorrectable type of biliary atresia. Three of the 5 showed an anomalous arrangement of the pancreaticobiliary duct. In these 3 patients, the type of anomalous arrangement of the pancreaticobiliary duct and the length of the common channel were studied by operative cholangiogram. Histological findings of the gallbladder and the common bile duct were examined in addition to the measurement of the serum amylase levels. RESULTS: All 3 patients showed AAPBD with the P-C type of pancreaticobiliary junction. The length of the common channel ranged from 7 mm to 12 mm. Two of the 3 cases did not show an elevated serum amylase level. Epithelial hyperplasia of the gallbladder was observed in one patient, while the other two showed no hyperplasia. Inflammatory changes in the mucosa of the gallbladder and the common bile duct were not remarkable in these 3 patients. CONCLUSIONS: From these results it seems that AAPBD in biliary atresia might not be an etiological factor for atresia of the extrahepatic bile duct, but might be an associated anomaly in biliary atresia. Other factors should be examined to clarify the etiological factor leading to lumenal obstruction of the extrahepatic bile duct.     

Clinical and pathological characteristics of cystic lesions of extrahepatic bile duct in neonates

Aim: To investigate the differences in clinical and pathological manifestations between biliary atresia with extrahepatic biliary cyst and choledochal cyst in neonates. Methods: Symptoms and clinical signs in 5 neonates with biliary atresia with extrahepatic biliary cyst (4 of type I and 1 of type III) and 17 neonates with choledochal cyst were recorded. The levels of serum alkaline phosphatase, bilirubin, direct bilirubin, transaminase, gamma-glutamyl transpeptidase were analysed. Width and length of gallbladder and choledochal cyst were measured on B-mode ultrasound before surgery. Intrahepatic or extrahepatic biliary ducts were visualized with intraoperative cholangiography. The pathologic features in specimens of the liver were studied with light- and electron transmission microscopy. Results: All malformations occurred more commonly in girls, and obstructive jaundice was the main manifestation in both groups. Laboratory tests showed similar results for all patients in this study. With regard to pathological features, no significant difference was seen in either light microscopy or transmission electron microscopy, but it was shown with ultrasound that the length and width of the cysts and the gallbladder in neonates with biliary atresia were all shorter than the measurements in patients with choledochal cyst. The intrahepatic bile ducts could not be visualized on intraoperative cholangiography in type III biliary atresia. Deformation of the biliary ducts within the liver and stricture of the portal bile duct were the predominant features in type I biliary atresia, while the bile duct within the liver was normal or dilated in neonates with choledochal cyst.

Conclusions: Cystic lesions of the extrahepatic bile duct might be a common manifestation of biliary atresia and choledochal cyst. Intraoperative cholangiography is a precise and effective technique in the differential diagnosis of those lesions and helps decide on the most rational method of treatment.     

The use of ultrasonography in the diagnosis of biliary atresia

Preoperative upper abdominal ultrasonograms of babies with biliary atresia were reviewed in order to determine the efficacy of this technique in the differential diagnosis of biliary atresia and neonatal hepatitis. In 4 patients with neonatal hepatitis and 8 normal controls, ultrasonograms showed echoes of the gallbladder. In 14 patients with biliary atresia, echoes of the gallbladder were not apparent. It is concluded that preoperative ultrasonograms provide an efficient diagnostic method for differentiating biliary atresia from neonatal hepatitis.Offprint requests to: T. Okasora     

Hitching the gallbladder in laparoscopic-assisted cholecysto-cholangiography: a simple technique

Neonatal cholestatic disorder in the late neonatal period requires often cholangiography to differentiate between biliary atresia and other causes of prolonged neonatal jaundice. A simplified method of a laparoscopic-assisted cholecysto-cholangiography is presented. Retrospective chart review was conducted of all patients who from May 2002 to April 2012 underwent a laparoscopic-assisted cholecysto-cholangiography with routine fixation of the fundus of the gallbladder to the lateral aspect of the abdominal wall. A total of 18 infants (8 boys) aged 41–104 (median 64) days underwent laparoscopic-assisted cholecysto-cholangiography for prolonged jaundice. The technique identified ten cases of a patent bile duct system and eight biliary atresias. (Thirty-two cases of suspected biliary atresia were confirmed by laparoscopy alone.) Two cases required suturing of a bile leak at the puncture site. Hitching the gallbladder to the lateral abdominal wall is a simple method allowing an optimal radiographic assessment of the extra- and intra-hepatic bile duct anatomy.     

Diagnostic laparoscopy-assisted cholangiography in infants with prolonged jaundice

Cholangiography is often crucial for establishing the definitive cause of neonatal jaundice. We present our protocol for using laparoscopy-assisted cholangiography in infants with prolonged jaundice and discuss its benefits. Firstly, a 5 mm supra-umbilical trocar is introduced to create a port for a 0° laparoscope. A 5 mm trocar is then inserted through a right subcostal incision to allow the liver and gallbladder to be visualized. If the gallbladder is of good size, the fundus is exteriorized through the right subcostal trocar site and a catheter is inserted into the gallbladder for cholangiography. If the gallbladder is atretic, the fundus is not exteriorized and a laparotomy is performed for open intraoperative cholangiography because the lumen of an atretic gallbladder is usually not fully patent and cholangiography through its exteriorized fundus often fails. We reviewed 18 jaundiced infants thought to have biliary atresia (BA) who had laparoscopy-assisted cholangiography. At laparoscopy, four patients had good sized gallbladders and minimal to mild liver fibrosis. They underwent cholangiography via the exteriorized fundus, and BA in two cases and biliary hypoplasia in two cases were identified. The remaining 14 had atretic gallbladders and varying degrees of liver fibrosis. Cholangiography via the exteriorized fundus was performed in one patient, but failed and converted to open cholangiography. Open intraoperative cholangiography identified BA in all 14 cases. All BA cases progressed to Kasai portoenterostomy directly after diagnosis. Laparoscopy is used to determine the type of cholangiography to be performed based on the appearance of the gallbladder and this simple, accurate, and safe protocol allows the anatomical structure of the biliary tree to be obtained accurately with minimal surgical intervention.     

Definitive exclusion of biliary atresia in infants with cholestatic jaundice: the role of percutaneous cholecysto-cholangiography

Definitive exclusion of biliary atresia in the infant with cholestatic jaundice usually requires operative cholangiography. This approach suffers from the disadvantage that sick infants are subjected to a time-consuming and potentially negative surgical exploration. The purpose of this study was to determine if percutaneous cholecystocholangiography (PCC) prevents unnecessary laparotomy in infants whose cholestasis is caused by diseases other than biliary atresia. This study is a 10 year retrospective review of all infants with persistent direct hyperbilirubinemia and inconclusive biliary nuclear scans who underwent further evaluation for suspected biliary atresia. A gallbladder ultrasound (US) was obtained in all patients. When the gallbladder was visualized, further imaging by PCC was done under intravenous sedation; otherwise, the standard operative cholangiogram (OCG) was performed, with liver biopsy as indicated. The primary outcome was the diagnostic accuracy of PCC, especially with respect to preventing a laparotomy. There were 35 infants with suspected biliary atresia, with a mean age of 8 weeks (range 1-14 weeks). Nine infants whose gallbladder was visualized by ultrasound underwent PCC that definitively excluded biliary atresia. Of this group, the most frequent diagnosis (five patients) was total parenteral nutrition-associated cholestasis. The other 26 infants with absent or decompressed gallbladder had laparotomy and OCG, which identified biliary atresia in 16 patients (61%). Laparotomy was avoided in all 9 patients who underwent PCC, thus reducing the negative laparotomy rate by 47%. There were no complications associated with PCC. Several alternative techniques to operative cholangiogram have been described for the definitive exclusion of biliary atresia, but many of these have distinct drawbacks. Advances in interventional radiology techniques have permitted safe percutaneous contrast evaluation of the biliary tree. Identification of a normal gall bladder on sonogram is highly predictive of the absence of biliary atresia. Further confirmation can be accurately obtained by a combination of PCC and percutaneous liver biopsy.     

Serial ultrasonic examination to differentiate biliary atresia from neonatal hepatitis — special reference to changes in size of the gallbladder

We performed serial ultrasonic examinations to differentiate biliary atresia from neonatal hepatitis. The subjects studied were 144 children (100 normal neonates and infants, 31 patients with neonatal hepatitis and 13 patients with biliary atresia). They were examined by ultrasound before, during and after feeding. In 97 out of 100 normal children and all patients with neonatal hepatitis, the gallbladder was identified, and the change in size following oral feeding was observed. In four children with severe neonatal hepatitis which could not be differentiated from biliary atresia by clinical and laboratory data, we readily identified the gallbladder and observed the change in the size following oral feeding. In 8 of 13 patients with biliary atresia, we identified a small gallbladder whose size was not affected by oral feeding. In the other patients the gallbladder was not identified before, during or after oral feeding. On the basis of these results, we consider that serial ultrasonic examination with oral feeding aids in a differential diagnosis of biliary atresia and neonatal hepatits.     

Making the diagnosis of biliary atresia using the triangular cord sign and gallbladder length

Background. To evaluate the accuracy and utility of the triangular cord sign and gallbladder length in diagnosing biliary atresia by sonography.?Materials and methods. Sixty fasted infants with cholestatic jaundice aged 2–12 weeks were examined sonographically using a 5–10 MHz linear array transducer, focusing on the triangular cord sign (as described by Choi et al. [1]), the gallbladder, and ducts. The triangular cord is defined as a triangular or tubular echogenic density seen immediately cranial to the portal vein bifurcation; it represents the fibrotic remnant of the obliterated cord in biliary atresia. The findings were blinded to blood chemistry, ^{99 m}Tc-DISIDA hepatobiliary scintigraphy, and liver biopsy. Diagnosis of biliary atresia was confirmed at surgery and histology. Non-biliary atresia infants resolved medically. Comparative charges of the various investigations was made.?Results. Twelve infants had biliary atresia, and ten demonstrated a definite triangular cord. The two false-negatives had small or nonvisualized gallbladders. No false-positives were recorded. Gallbladder length ranged from 0–1.45 cm with a mean of 0.52 cm in biliary atresia compared to a mean of 2.39 cm in nonbiliary atresia infants. ^{99 m}Tc-DISIDA hepatobiliary scintigraphy showed no excretion (false-positive) in 23 % of nonbiliary atresia cases. Scintigraphy and liver biopsy charges were 2 and 6 times that of sonography, respectively.?Conclusion. The triangular cord sign and gallbladder length together are noninvasive, inexpensive, and very useful markers for biliary atresia. Received: 26 May 1999/Accepted: 28 July 1999     

Clinical and Postmortem Findings of Two Cases With Karyotype: 48, XXX, +18

Clinical and postmortem findings of two cases with karyotype: 48, XXX, +18 are presented. In both patients the usual characteristics of trisomy 18 were found. Case 1 was complicated with congenital esophageal atresia, hypoplastic left auricle with external auditory canal atresia, left congenital dislocation of hip, and left club foot. The patient died on the 9th day after birth. Autopsy revealed mitral atresia, aortic stenosis, ventricular septal defect, patent ductus arteriosus, coarctation of aorta, bicuspid aortic and pulmonary valves, patent foramen ovale, and congenital esophageal atresia (type C). Case 2 died on the 8th day after birth. Postmortem examination revealed ventricular septal defect, but there was no other noteworthy malformation. Dermatoglyphic findings were more prominent on the right hand than the left. The severity of malformations may be biased to either the right or the left side.     

Apparent gut excretion of Tc-99m-DISIDA in a case of extrahepatic biliary atresia

Our patient had DISIDA scan at 39 days of age to exclude biliary atresia. Gut excretion was seen 18h after injection and the diagnosis of neonatal hepatitis was made. Because of continued elevated liver function values, liver biopsy was performed and demonstrated findings consistent with biliary atresia. Exploratory laparotomy performed 8 days after the scan showed patent cystic duct, bile duct, and gall-bladder, but an atretic common hepatic duct. This case is an example of documented biliary atresia demonstrating gut excretion on the DISIDA scan.     

Biliary atresia: making the diagnosis by the gallbladder ghost triad

OBJECTIVE: To describe the gallbladder ghost triad and evaluate its usefulness in the sonographic diagnosis of extrahepatic biliary atresia (BA). METHODS: From October 1997 to February 2002, 217 fasted infants with cholestatic jaundice aged 2-12 weeks were examined sonographically. We defined the gallbladder ghost triad as gallbladder length <1.9 cm, lack of smooth/complete echogenic mucosal lining with an indistinct wall and irregular/lobular contour, and used it as a criteria for BA. Gallbladder wall thickness, triangular cord, diffuse periportal echogenicity and hepatic artery calibre were also recorded. Diagnosis of BA was confirmed surgically and histologically. RESULTS: Thirty of 31 babies with BA demonstrated the gallbladder ghost triad. No false-positives were recorded. The 31st BA baby showed a normal gallbladder at 6 weeks, but developed the ghost triad at 8 weeks. Gallbladder wall thickening was seen in 46/186 non-BA babies, but not in BA. Triangular cord was observed in 24/31 babies. Twenty-two of 186 non-BA babies and 5/31 BA babies showed diffuse periportal echogenicity. The hepatic artery appeared more prominent in BA. All 31 babies diagnosed sonographically as BA had surgery. Three non-BA babies had "negative" laparotomies showing hypoplastic bile ducts. CONCLUSIONS: The gallbladder ghost triad is a very accurate sign of BA. Indeterminate cases require close follow-up.     

Value of sonography in prolonged neonatal jaundice. Findings in 13 cases]

BACKGROUND: Different conditions are associated with a prolonged cholestatic jaundice in the neonatal period: viral hepatitis, biliary atresia and choledocal cyst are the most frequent causes. Laboratory findings are necessary, although they do not permit an etiologic diagnosis in all cases. Serial ultrasonographic study could be proposed for the evaluation of biliary excretion before and after feeding, in order to differentiate between these three conditions. PATIENTS AND METHODS: Between February 1993 and January 1997, 13 newborns (seven girls and six boys) aged from 30 to 186 days, presented with jaundice and conjugated hyperbilirubinemia. They were evaluated by laboratory tests; serial ultrasonographic examinations were performed after 4 hours fasting then 1 and 2 hours after meal. RESULTS: The gallbladder (GB) was visualized in nine patients. In five of these patients, it contracted after feeding suggesting the diagnosis of neonatal hepatitis, that was confirmed by the clinical evolution. In three patients, the GB did not change in size and the diagnosis of biliary atresia was surgically proven. In one patient, a choledocal cyst was visualized and confirmed by surgery. The GB was not identified after 4 hours of fasting in four patients; biliary atresia was suspected and confirmed by surgery. CONCLUSION: Serial ultrasound of the GB is an easy and non-invasive method. It was useful in identifying those conditions requiring surgery in eight patients. We recommend its use as the initial method in the evaluation of neonatal jaundice before the other invasive methods.     

A multicenter study of the outcome of biliary atresia in the United States, 1997 to 2000

OBJECTIVE: To determine the prognostic factors and optimal approaches to the diagnosis and management of biliary atresia, the leading indication for liver transplantation in children. STUDY DESIGN: A retrospective study was performed of all children who underwent hepatoportoenterostomy (HPE) for biliary atresia between 1997 and 2000 at 9 centers in the United States. Outcome at age 24 months was correlated with demographic and clinical parameters. RESULTS: A total of 104 children underwent HPE; 25% had congenital anomalies, and outcome was worse in those with biliary atresia splenic malformation syndrome. Diagnostic and clinical approaches varied, although specific approaches did not appear to correlate with outcome. The average age at referral was 53 days, and the average age at HPE was 61 days. At age 24 months, 58 children were alive with their native liver, 42 had undergone liver transplantation (37 alive, 5 dead), and 4 had died without undergoing transplantation. Kaplan-Meier analysis of survival without liver transplantation revealed markedly improved survival in children with total bilirubin level<2 mg/dL at 3 months after HPE (84% vs 16%; P<.0001). CONCLUSIONS: Outcome in the study centers was equivalent to that reported in other countries. Total bilirubin in early follow-up after HPE was highly predictive of outcome. Efforts to improve bile flow after HPE may lead to improved outcome in children with biliary atresia.     

Type I biliary atresia without extrahepatic biliary cyst

Currently, magnetic resonance cholangiography (MRC) is used for the differentiation of biliary atresia (BA) from other causes of infantile cholestasis. The authors present a case of type I BA without an extrahepatic biliary cyst in a 2-month-old girl. MRC clearly visualized the patency of the gallbladder, cystic duct, and hepatic ducts with disappearance of the common bile duct. Intraoperative cholangiography demonstrated a cloudy appearance of the intrahepatic bile ducts, confirming the diagnosis of type I BA. We believe that this is the first reported case of type I BA without an extrahepatic biliary cyst diagnosed by MRC.     

Non-correctable biliary atresia with large extrahepatic cyst: a report of two cases.

Biliary atresia constitutes a serious problem in early infancy, due to the concomitant cholestasis. The conventional classification falls into 3 main types: type I, atresia of the common bile duct; type II, atresia of the hepatic duct; and type III, atresia of the porta hepatis. We report 2 unusual cases of biliary atresia type III with a cystic structure that could be mistaken for correctable biliary atresia or choledochal cyst.     

Hepatobiliary scintigraphy with technetium-99m disofenin in the evaluation of neonatal cholestasis

To assess the reliability of technetium-99m disofenin scanning in evaluating neonatal cholestasis, 33 neonates (less than 3 months of age) with direct hyperbilirubinemia were evaluated prospectively by cholescintigraphy. Results of this test were compared to those of standard serum tests of liver function, ultrasonography, and liver biopsy. The diagnosis of biliary atresia was suggested by a serum gamma-glutamyl transpeptidase (gamma-GTP) greater than 300 units/L, absence of the gallbladder on ultrasonography, and a lack of detectable radioisotope in the gastrointestinal and/or extrahepatic biliary tract on cholescintigraphy. Each of these tests lacked sensitivity and/or specificity when compared to liver biopsy. Of the nine neonates with biliary atresia, three had gallbladders identified by ultrasonography and two had gamma-GTP less than 300 units/L. Of the 24 neonates without biliary atresia, eight had cholescintigraphy without detectable radioisotope excretion, four had ultrasonography that failed to visualize the gallbladder, and nine had gamma-GTP greater than 300 units/L. Cholescintigraphy excluded the diagnosis of biliary atresia when gut and/or extrahepatic biliary excretion of isotope was seen. However, cholescintigraphy required more time, 6-8 days, and was less specific than ultrasonography and liver biopsy. We recommend that cholescintigraphy should not be routinely used in evaluating neonatal cholestasis, especially if it delays surgical intervention.     

Gallbladder Changes in Neonatal Hepatitis: Markedly Thickened Wall and Lack of Contractility

The gallbladder wall changes were observed on ultrasonography during the course of a patient with neonatal hepatitis. The gallbladder was not detected at 53 days of age, but on the next day its wall was observed to be markedly thickened and without contraction following the administration of cerulein. It had a thinner wall at 57 days of age and reacted to cerulein. The wall thickness and contractility went together with the improvement of jaundice and liver function tests. Histological diagnosis was compatible with neonatal hepatitis.
Ultrasonographic detection of the gallbladder has been helpful to differentiate neonatal hepatitis from biliary atresia. It is reported to be compatible with neonatal hepatitis to detect a normal-sized gallbladder or its contraction following cerulein administration or feeding. Since this case did not meet these criteria initially, thickened wall of the gallbladder may be an additional finding indicating neonatal hepatitis. The importance of repeated ultrasonography and clinical correlation was stressed.     

产前诊断胆道闭锁影像学研究

目的：胆道闭锁早期诊断困难,而产前诊断更是极少发现。本文对产前诊断的胆道闭锁影像学特点进行探讨。方法回顾我院2010年至2012年收治的产前诊断9例产前诊断胆道畸形患儿,入院手术年龄24 d至2岁。全部患儿行腹腔镜胆道造影,4例诊断胆道闭锁,5例诊断先天性胆管扩张症,胆道闭锁患儿中2例接受腹腔镜下肝管空肠ROUX-Y吻合术,2例接受开腹肝门空肠吻合术。观察其临床表现,超声和实验室指标,术中情况,术后恢复情况等。结果4例患儿产前超声未见胆囊或胆囊显示不清。产前超声发现肝门部囊肿的3例患儿,囊肿小且均无明显增大,张力较高,呈规则圆形。产前诊断发现肝门囊肿的3例患儿术中证实为胆总管远端闭锁（Ⅰ型胆道闭锁）,未发现胆囊也未发现囊肿的1例患儿证实为Ⅲ型胆道闭锁。生后全部胆道闭锁患儿出现黄疸,最早出现在生后第2天,但都未出现陶土样便。全部胆道闭锁患儿囊肿大小形态无明显变化。全部患儿查ALT、AST、rGGT、直接胆红素和总胆红素进行性升高。2例接受腹腔镜下肝管空肠ROUX-Y吻合术,2例接受开腹肝门空肠吻合术。全部4例患儿术后恢复好。结论产前超声检查可以确定胆道闭锁,如果发现肝门部囊肿的胎儿,应定期接受超声检查,如果囊肿在孕期变化不明显,应怀疑囊肿型胆道闭锁。如产前超声未发现胆囊结构,则应怀疑为Ⅲ型胆道闭锁。生后应密切观察、超声、生化、黄疸情况。如果黄疸进行性加重可及早进行腹腔镜胆道造影及手术治疗。     

Computerized three-dimensional study of a rotavirus model of biliary atresia: comparison with human biliary atresia

Biliary atresia is a panbiliary disease causing obstructive jaundice in neonates and infants. The clinical spectrum can be broadly categorized into the fetal and perinatal types. A consistent animal model that accurately mimics the whole clinical spectrum of biliary atresia is not yet available. However, rotavirus infection of neonatal mice has been shown to produce atresia in the biliary system. This study investigates the three-dimensional computerized morphology of the murine neonatal model comparing with age-matched control mice. Newborn Balb/c mice were injected intraperitoneally with rhesus rotavirus within 24–48 h after birth. Control mice received 0.9% NaCl. Pups with symptoms of cholestasis were sacrificed from the 5th to the 15th postinjection day, as were age-matched controls. Their hepatobiliary tissues were prepared for three-dimensional computerized image reconstruction. Rotavirus infection caused obliteration of the intrahepatic bile ducts and single to multiple atresias in the extrahepatic bile duct. At 15 days postinjection, intrahepatic ductal proliferation appeared, and the three-dimensional appearances of the intrahepatic biliary structures were similar to the human disease. Cystic duct and gallbladder dilatation was frequently seen in this model, and this feature distinguishes it from the human disease in which the gallbladder is almost always atretic. This rotavirus murine model demonstrates many of the features of human perinatal biliary atresia, and can be used as an investigative tool to further study the pathogenesis of biliary atresia.     

Unusual gallbladder findings in two brothers with metachromatic leukodystrophy

Characteristic biliary tree abnormalities in metachromatic leukodystrophy (MLD) include gallbladder polyposis and haemobilia. We report two brothers with MLD, who presented with uncommon biliary complications. One presented with gastric outlet obstruction secondary to gallbladder enlargement, which was treated by percutaneous aspiration. He later developed gallbladder carcinoma with liver metastases. His brother demonstrated US findings consistent with gallstones. Received: 20 August 1997 Accepted: 23 February 1998