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Diagnostics 2016, 6(1), 5; doi:10.3390/diagnostics6010005

Potential Pitfalls on the 99mTc-Mebrofenin Hepatobiliary Scintigraphy in a Patient with Biliary Atresia Splenic Malformation Syndrome

1
Department of Clinical Physiology, Nuclear Medicine & PET, Rigshospitalet–Glostrup, Copenhagen University Hospital, Nordre Ringvej 57, DK-2600 Glostrup, Denmark
2
Department of Clinical Physiology, Nuclear Medicine & PET, Rigshospitalet, Copenhagen University Hospital, Blegdamsvej 9-KFNA 4011, DK-2100 Copenhagen, Denmark
*
Author to whom correspondence should be addressed.
Academic Editor: Andreas Kjaer
Received: 11 December 2015 / Revised: 29 December 2015 / Accepted: 31 December 2015 / Published: 7 January 2016
(This article belongs to the Collection Hybrid Imaging in Medicine)
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Abstract

Biliary atresia (BA) is an obliterative cholangiopathy affecting 1:10.000–14.000 of newborns. Infants with Biliary Atresia Splenic Malformation syndrome (BASM) are a subgroup of BA patients with additional congenital anomalies. Untreated the disease will result in fatal liver failure within the first years of life. Kasai portoenterostomy restores bile flow and delay the progressive liver damage thereby postponing liver transplantation. An early diagnosis is of most importance to ensure the effectiveness of the operation. The 99mTc-Mebrofenin hepatobiliary scintigraphy is part of the diagnostic strategy when an infant presents jaundice due to conjugated hyperbilirubinemia (>20 µmol/L total bilirubin of which 20% is conjugated) with its high sensitivity of 97%–100% in refuting BA. Rapid extraction of tracer by the liver and no visible tracer in the small bowl after 24 h is indicative of BA. Laparotomy with antegrade cholangiography is then performed giving the final diagnosis when the remains of the obliterated biliary tree are revealed in the case of BA. We present a case demonstrating some of the challenges of interpreting the 99mTc-Mebrofenin hepatobiliary scintigraphy in an infant with BASM and stress the importance that the 99mTc-Mebrofenin hepatobiliary scintigraphy is part of a spectrum of imaging modalities in diagnosing BA. View Full-Text
Keywords: biliary atresia; biliary atresia splenic malformation syndrome; hepatobiliary scintigraphy; magnetic resonance imaging biliary atresia; biliary atresia splenic malformation syndrome; hepatobiliary scintigraphy; magnetic resonance imaging
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This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. (CC BY 4.0).

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Maestri Brittain, J.; Borgwardt, L. Potential Pitfalls on the 99mTc-Mebrofenin Hepatobiliary Scintigraphy in a Patient with Biliary Atresia Splenic Malformation Syndrome. Diagnostics 2016, 6, 5.

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