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

Abstract

All diagnostic algorithms for Neonatal Cholestasis Syndrome (NCS) focus on differentiating numerous medical causes from Biliary Atresia (BA). No preoperative diagnostic algorithm has 100% diagnostic accuracy for BA and yet, timely diagnosis is crucial to optimize surgical outcome. Markers for high index of clinical suspicion for BA are: a "usually" well thriving infant with conjugated hyperbilirubinemia, raised gamma glutamyl transpeptidase, persistently "acholic" stools, firm hepatomegaly with dysmorphic, hypoplastic gall bladder. In the presence of above 'red flag' signs, there has been much debate on diagnostic accuracy of percutaneous liver biopsy (PLB) vs. hepatobiliary scintigraphy (HBS) to substantiate or exclude BA. Recent guidelines suggest a shift towards PLB (91.6% overall diagnostic accuracy) as the diagnostic cornerstone with key differentiating feature being 'bile ductular proliferation'. HBS has a high (98.7%) sensitivity but low specificity (37-74%) with an overall diagnostic accuracy of 67% for BA. Severe hepatocellular disease without anatomic obstruction would also have a non-excretory scan. Thus, while excretory HBS excludes BA, non-excretion does not confirm BA. Hence, diagnostic algorithms relying on non-excretory HBS as the primary standalone benchmark for surgical exploration would be mired by a high negative laparotomy rate revealing a normal peroperative cholangiogram (POC). However, an excretory HBS obviates need for laparotomy in case of equivocal stool color or PLB. A POC continues to be the ultimate gold standard. Hence, with high index of clinical suspicion but equivocal ultrasonography or PLB and a non-excretory HBS, the baby should not be denied a POC within time frame crucial for successful hepatoportoenterostomy.

Development and validation of bile acid profile-based scoring system for identification of biliary atresia: a prospective study

“…Presumably, the isotopes could pass through the slit-like lumen and transit into the duodenum in a few patients, which produced false-negative results, as demonstrated in this study. Also, according to a previous study, HBS has a high (98.7%) sensitivity but low (37-74%) specificity for BA diagnosis, with an overall diagnostic accuracy of 67% for BA [28]. A positive finding could also be found in severe intrahepatic cholestasis, such as CMV hepatitis, which reflects obstruction in the intrahepatic bile ducts affecting bile excretion in the intestine.…”

Serum MMP-7 in the Diagnosis of Biliary Atresia

“…17 Although radiologic investigations (such as ultrasound, MRI, and hepatobiliary scintigraphy) were reported to be helpful in differentiating BA from other obstructive cholestasis, they are time consuming and costly. [18][19][20][21][22][23] Therefore, an effective biomarker would be extremely valuable for the preoperative diagnosis of BA.…”

Role of hepatobiliary scintigraphy with different semi-quantitative parameters along with histopathological scoring in differentiating biliary atresia from neonatal hepatitis

“…NC is always pathologic and signifies hepatobiliary disorder. Differential diagnoses of neonatal cholestatic jaundice include hepatocellular causes [neonatal hepatitis (NH), metabolic diseases (galactosemia, tyrosinemia, alpha one antitrypsin deficiency, parenteral nutritionrelated cholestasis, and storage disorders)], ductal paucity, and idiopathic and obstructive causes [biliary atresia (BA) and choledochal cyst] [3]. BA and NH are the two most common causes of neonatal cholestatic jaundice, yet they have similar clinical manifestations.…”