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.

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.

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.

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.

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.

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.

Prognosis of Congenital Extrahepatic Biliary Atresia After Hepatic Portoenterostomy

PURPOSE: The surgical results for congenital extrahepatic biliary atresia have been improved since Kasai first described hepatic portoenterostomy(Kasai operation) in 1959. This study was conducted to evaluate the postoperative prognosis of congenital extrahepatic biliary atresia after hepatic portoenterostomy. METHODS: This clinical study was done by reviewing the medical records of 30 patients who have undergone hepatic portoenterostomy at the Severance Hospital, Yonsei University College of Medicine between January 1984 to June 1994 due to congenital extrahepatic biliary atresia. RESULTS: 1) The ages of the 30 patients ranged from 45 to 242 days and the mean age at operation was 90.8 days. Seventeen patients were male and 13 were female, and the male to female ratio was 1.3:1. 2) Of the total 30 patients, 9 cases(30%) had died, 12 cases(40%) were still being followed up, and 9 cases(30%) never returned for during follow-up. The calculated survival rate of more than 3 years was 53%. 3) Jaundice was improved in 8 patients(27%) after portoenterostomy, but the remaining 22 patients(73%) showed no improvement. The two year survival rate of the jaundice-free group was 100%, compared with 42% in the jaundice-persistent group, which showed a statistically significant difference (p<0.05). 4) Hepatic portoenterostomy was performed in 7 patients(23%) before the age of 60 days, and done in the remaining 23 patients(77%) after 60 days. The two year survival rate of the former group was 54%, compared with 70% in the latter group, which did not show a statistically significant difference. 5) Portal hypertension was complicated in 17 patients(57%), but not in the remaining 13 patients(43%). The two year survival rate of the former group was 54%, compared with 100% in the latter group, which showed a statistically significant difference between the two groups (p<0.05). 6) Cholangitis was complicated in 15 patients(50%), but not in the remaining 15 patients(50%). The two year survival rate of the former group was 53%, compared with 81% in the latter group, which did not show a statistically significant difference. CONCLUSIONS: The 3 year survival rate in patients who performed hepatic portoenterostomy due to congenital extrahepatic biliary atresia was 53%. The persistent jaundice and the development of portal hypertension after surgery were important determinants of long term survival of exteahepatic biliary atresia in this study.