Role of Hepatobiliary Scintigraphy and Preoperative Liver Biopsy for Exclusion of Biliary Atresia in Neonatal Cholestasis Syndrome

2017 ◽  
Vol 84 (9) ◽  
pp. 685-690 ◽  
Author(s):  
Ankur Mandelia ◽  
Richa Lal ◽  
Nijagal Mutt
2017 ◽  
Vol 28 (03) ◽  
pp. 261-267 ◽  
Author(s):  
Ahmed Negm ◽  
Claus Petersen ◽  
Andrea Markowski ◽  
Birgit Luettig ◽  
Kristina Ringe ◽  
...  

Introduction Biliary atresia (BA) is a rare destructive inflammatory obliterative cholangiopathy of neonates. Early diagnosis is important in disease management. The aim was to evaluate the role of endoscopic retrograde cholangiopancreatography (ERCP) in diagnosing BA in a large cohort. In addition, we evaluated whether parameters such as bile trace, GGT, bilirubin, and laboratory values in combination can be used to develop a risk score that could indicate the referral to specialized centers. Materials and Methods All infants with neonatal cholestasis (2000–2014) who presented to our endoscopy unit for suspected BA were included. Demographics, laboratory parameters, ultrasound findings, liver biopsy results, ERCP diagnosis, and surgical outcome were collected. Value and safety of ERCP and risk factors for BA were retrospectively analyzed. Results We included 251 infants in our cohort (55% males, median age: 53 days). BA was intraoperatively diagnosed in 155 (83.4%) patients and was excluded in 30 (16.2%). Fifty-six cases were not operated due to the ERCP findings. ERCP was successful in 224/251 patients (89.2%) with no procedure-related complications. The operative and endoscopic diagnosis matched in 96.6% of the patients (positive predictive value: 92.2%, negative predictive value: 97.1%). In comparison to cases with excluded BA, the ones with this disease were significantly associated with absence of duodenal bile traces (98.4 vs. 1.6%, p < 0.001), higher bilirubin (p < 0.001, cutoff 7.3 mg/dL), and higher GGT (p < 0.001, cutoff 250 U/L). Conclusion ERCP is safe and accurate in the hands of experts in diagnosing BA if the cause of cholestasis is unclear. While evaluating the role of ERCP for diagnosing this disease, we found that the secondary parameters GGT > 250 U/L, bilirubin > 7.3 mg/dL (125 μmol/L), and the absence of bile traces are risk factors.


2017 ◽  
Vol 18 (1) ◽  
pp. 51-53
Author(s):  
Nasreen Sultana ◽  
Zeenat Jabin ◽  
Md Bashir ◽  
Rahima Parveen ◽  
Shamim MF Begum ◽  
...  

Objective: The purpose of this study was to determine whether gall bladder visualization can help to exclude the biliary atresia in hepatobiliary scintigraphic studies of infants with persistent jaundice.Methods: This is a retrospective study carried out at the National Institute of Nuclear Medicine and Allied Sciences (NINMAS). Study subjects include infants with neo-natal jaundice who underwent Hepatobiliary scintigraphies for suspected biliary atresia and study period was 2 years. Food was withheld for 4 hours before the examination. Anterior images of liver and gall bladder were taken after i/v administration of 2 -3 mci 99m Tc labeled Brida (HIDA) at 5 min interval for 2 hours then at 4 hours and 24 hours. Non-visualization of bowel activity in HIDA scan in 24hours delayed images was considered as cases of diagnosis of biliary atresia.Results: Thirty-six patients were included in this retrospective study. Patent biliary channels was seen by scintigraphies in 17(47%) patients and biliary atresia was seen in 19(52%) patients. By abdominal US non- visualization of gall bladder were found in 25(69%) cases and gall bladder visualized in 11(30%) cases. Eight (22%) of 36 patients had biopsy confirmed biliary atresia; all of these had positive scintigraphies and (60%) had positive sonographic findings. Among the 5 false-positive scintigraphies caused by hepatic dysfunction and 2 had normal sonography. Thirty-six patients had periscintigraphic sonography. There were 25/36 (61%) abnormal studies, which included cases with small gallbladder (n = 8) and non-visualized gallbladder (n = 17), but not periportal fibrosis.Conclusion: Gall bladder was usually visible on Hepatobiliary scintigraphy of fasting patients with biliary patency.  Both hepatobiliary scintigraphy and sonography are currently the standard imaging investigations for suspected biliary atresia. The complementary role, in which scintigraphy and sonography are important, and recommend follow-up imaging reassessment before making definitive surgical decisions. This will serve to decrease the frequency of false-positive imaging diagnoses of biliary atresia, and hence, avoid unnecessary surgeries.Bangladesh J. Nuclear Med. 18(1): 51-53, January 2015


2017 ◽  
Vol 5 ◽  
pp. 2050313X1769599 ◽  
Author(s):  
Noella Maria Delia Pereira ◽  
Ira Shah

Cholestasis can occur in newborns due to infections. However, the manifestations of the underlying infections usually dominate the presentation. We present a 2-month-old infant who presented with jaundice and no fever or signs of systemic illness. Liver biopsy was suggestive of cholangitis. He was subsequently detected to have urinary tract infection with Klebsiella pneumoniae. The child was treated with appropriate antibiotics for 2 weeks following which the cholestasis resolved. Thus, neonatal cholestasis due to infections can also occur in the post-neonatal period without clinical manifestations of an underlying infection.


2014 ◽  
Vol 41 (2) ◽  
pp. 34-39
Author(s):  
F Hamid ◽  
A Afroza ◽  
PC Ray

Cholestasis in young infants has a varied etiology. There is considerable delay in presentation of cholestatic cases, both in India (average delay of 3 months in referral centers) and Bangladesh (3.5 months). Early diagnosis is important as the effects of cholestasis are profound and wide-spread. EHBA comprises a significant proportion of cases of cholestatic diseases. If treatment of EHBA is delayed beyond the first 90 days of life, the only option thereafter is liver transplantation, which is not presently feasible on a large scale in developing countries. So, the aim and objective of this study is to determine the cause and to categorize the clinical profile and treatment options of conjugated hyperbilirubinaemia in infancy. A total of 30 patients, who fulfilled inclusion and exclusion criteria were included. A detailed history and physical examination was done daily. Complete blood count, liver function tests, HBsAg and TORCH screening, thyroid function test, Urine was tested for non-glucose reducing substances. Ultrasonography of the hepatobiliary system and hepatobiliary scintigraphy was done. Liver biopsy was done in appropriate patients. Patients were followed up daily during hospital stay. The management and its response were also monitored and recorded. Out of 30 patients 24 (80%) were male and 6 (20%) were female. 63% were term and 37% were preterm. Out of 12 patients in BA, 11 were term and only 1 was preterm, whereas in NH group, out of 18 babies, 10 were preterm. Most of the patients were male in both studied groups. Mean value of birth weight in BA was 2.65±0.13 and in NH was 2.40±0.31. Mean age (days) at onset of jaundice in 2 groups were 3.92 ± 2.43 in BA and 6.5 ±4.5 in NH. Most patients of BA (91%) had persistent acholic stool, whereas in Neonatal hepatitis group 83% had intermittent acholic stool. No statistically significant difference was observed when hepatosplenomegaly and ALT values were considered in 2 studied groups. In BA group 33% & In NH group 72% babies had positivity for CMV infection. Normal ultrasonic findings were seen in 2 patients of BA group, and 7 in NH group. No patient had shown contracted gall bladder after meal in BA, Choledocal cyst was found to be responsible for 5 (42%) patients in BA group and none in NH group. HIDA showed 47% had biliary atresia, 43% neonatal hepatitis and 10.0% had normal liver. Liver biopsy revealed that 12 (40.0%) had biliary atresia and 18 (60.0%) had neonatal hepatitis. Out of 12 babies in Biliary atresia group almost all, 11(92%) received surgical management where as in Neonatal hepatitis group; all 18 babies (100%) received medical treatments. Early detection of cholectatic cases by observing stool colour is very important for physicians to direct the very specific investigations to find out the cause and start appropriate treatment immediately. DOI: http://dx.doi.org/10.3329/bmj.v41i2.18804 Bangladesh Medical Journal 2012 Vol. 41 No. 2: 34-39


2009 ◽  
Vol 48 (03) ◽  
pp. 100-103 ◽  
Author(s):  
H. R. Kianifar ◽  
V. R. D. Kakhki ◽  
R. Zakavi ◽  
K. Ansari ◽  
R. Sadeghi

Summary Aim: Hepatobiliary scintigraphy is an integral part in the diagnostic work-up of the neonatal cholestasis syndrome. However, less than optimal specificity is its major disadvantage. Differentiation between biliary atresia and neonatal hepatitis is nearly impossible in some cases with poor hepatocellular function. 99mTc sestamibi (MIBI) is a cationic lipophilic agent which is a substrate of P-glycoprotein. This glycoprotein is normally expressed in biliary canalicular surfaces of hepatocytes. This property provides a hepatic excretory mechanism which is different from bilirubin excretion. In this study we evaluated the value of 99mTc MIBI in differential diagnosis of neonatal cholestasis. Patients, methods: 20 infants with a mean age of 2.41 months (range, 0.1–5 months) were included in the study. Ten infants turned out to have extrahepatic biliary atresia and the other ten had neonatal hepatitis. Hepatobiliary (with 99mTc BrIDA) and 99mTc MIBI scintigraphy were performed for all the patients. Results: 99mTc MIBI scintigraphy has shown bowel activity in all patients, including the patients with biliary atresia. Hepatobiliary scintigraphy revealed bowel activity only in five patients with neo natal hepatitis. Conclusion: Bowel visualization with 99mTc MIBI may be seen in patients with biliary atresia and 99mTc MIBI has limited value in differential diagnosis of neonatal chole stasis.


2006 ◽  
Vol 48 (5) ◽  
pp. 498-500 ◽  
Author(s):  
NUR ARSLAN ◽  
AYŞE MAVİ ◽  
SEMA KALKAN ◽  
AYSEL AYDIN ◽  
RAGIP ORTAÇ ◽  
...  

Author(s):  
Nsreen R. A. Mohamadien ◽  
Rania Makboul ◽  
Shereen M. Galal ◽  
Nadia M. Mostafa

Abstract Background Biliary atresia (BA) and neonatal hepatitis (NH) are the two major causes of neonatal cholestasis (NC). However, both conditions had entirely different therapeutic schedule and prognosis. Considering BA as a surgical emergency, it is pretty important to accurately differentiate the two entities. The aim of the study is to evaluate the diagnostic utility of hepatobiliary scintigraphy (HBS) using a semi-quantitative technique as well as 15-point histopathological scoring system in differentiating BA from NH. Results The sensitivity, specificity, and overall accuracy of HBS in the diagnosis of BA was 90.5%, 80%, and 83.6%, respectively. The median values of kidney-liver ratio (KLR), intestinal-liver ratio (ILR), and background-liver ratio (BLR) were significantly higher in patients with BA, while that of the liver-kidney ratio (LKR) was significantly lower in cases with BA. Moreover, KLR had the largest area under curve (AUC); advocates it to be the best of the semi-quantitative parameters that can predicts BA. Histopathological scoring using a cutoff point ≥ 7 was helpful in discriminating BA from NH with 85.7% sensitivity, 95% specificity and 91.8% accuracy. Conclusions HBS is a non-invasive diagnostic tool frequently used in diagnosis of BA, yet it has a relatively low specificity. To overcome this challenge, we kindly recommend the use of semi-quantitative parameters that could possibly improve the accuracy of HBS for diagnosing BA. Additionally, the use of 15-point scoring for liver biopsy was useful.


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