ELECTRONIC CLINICAL CHALLENGES AND IMAGES IN GI A Rare Cause of Neonatal Obstructive Jaundice María José de Castro López, José María Fraga Bermúdez, and Maria Luz Couce Pico Neonatal Unit, Department of Pediatrics, Hospital Clínico Universitario de Santiago de Compostela, Santiago de Compostela, Spain
Question: A 10-day-old, full-term male infant born by vaginal delivery after an uneventful pregnancy presents to the Pediatric Emergency Department with irritability, poor feeding, and jaundice. Complete blood count, chemistry profile, and coagulation tests showed direct hyperbilirubinemia (total bilirubin, 4.1 mg/dL; direct bilirubin, 2.9 mg/ dL), gamma-glutamyl transpeptidase of 459 UI/L and alkaline phosphatase of 440 UI/L, without other alterations. Abdominal ultrasounds (US; Figure A) examination revealed a cystic duct dilated by biliary sludge (pink asterisk) with a normal gallbladder (green arrow head) and the newborn was hospitalized. A hepatobiliary scintigraphy was performed and showed nonvisualization of the intrahepatic–extrahepatic biliary tract and no activity in the bowel for 1 hour after injection. These findings indicated biliary atresia. On hospital day 7, a MR cholangiopancreatography (MRC; Figure B) was done, revealing a cystic duct (pink asterisk) inserted into the distal choledochus (near to the ampulla of Vater) and a filling defect of the cystic duct (green arrow head) that was causing dilatation of it and external compression of the choledochus (blue asterisk). Colagogues, fat soluble vitamins, and ursodeoxicolic acid were administered according to the standard management of neonatal cholestasis. The patient’s parents preferred to continue with medical follow up and conservative management, and they refused invasive treatments. Despite this, clinical evolution was satisfactory over the first three months. What is your diagnosis? See the Gastroenterology web site (www.gastrojournal.org) for more information on submitting your favorite image to Clinical Challenges and Images in GI.
Conflicts of interest The authors disclose no conflicts. © 2015 by the AGA Institute 0016-5085/$36.00 http://dx.doi.org/10.1053/j.gastro.2014.10.061
Gastroenterology 2015;148:e12–e13
ELECTRONIC CLINICAL CHALLENGES AND IMAGES IN GI Answer to the Clinical Challenges and Images in GI Question: Image 6: Mirizzi Syndrome These findings are consistent with a biliary sludge impaction in an anatomic variant of the cystic duct1 that produces external compression of the extrahepatic bile duct, resulting in biliary obstruction, which is known as Mirizzi syndrome (MS). MS is a rare cause of obstructive jaundice in adults,2 and never reported in newborns. The clinical diagnosis is difficult since there are no pathognomonic patterns of presentation. Abdominal US is used widely for the initial screening and it may show a contracted gallbladder with a gallstone impacted in the infundibulum and an hepatic duct dilated above the level of obstruction, but the reported diagnostic accuracy for US in MS is 29%. To confirm the diagnosis, endoscopic retrograde cholangiopancreatography and/or MRC must be performed, because both tests allow an optimal visualization of the cystic duct and the biliary tract. In this case, MRC was done to avoid the complications of direct cholangiography and led to a diagnosis of MS.3 In conclusion, physicians should be aware that MS, although rare, may occur during the neonatal period and should be considered in the differential diagnosis of cholestatic jaundice.
References 1. 2. 3.
Turner MA, Fulcher AS. The cystic duct: normal anatomy and disease processes. Radiographics 2001;21:3–22. Lai EC, Lau WY. Mirizzi syndrome: history, present and future development. ANZ J Surg 2006;76:251–257. Beltrán MA. Mirizzi syndrome: history, current knowledge and proposal of a simplified classification. World J Gastroenterol 2012;18:4639–4650.
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Question: A 10-day-old, full-term male infant born by vaginal delivery after an uneventful pregnancy presents to the Pediatric Emergency Department with irritability, poor feeding, and jaundice. Complete blood count, chemistry profile, and coagulation tests showed direct hyperbilirubinemia (total bilirubin, 4.1 mg/dL; direct bilirubin, 2.9 mg/ dL), gamma-glutamyl transpeptidase of 459 UI/L and alkaline phosphatase of 440 UI/L, without other alterations. Abdominal ultrasounds (US; Figure A) examination revealed a cystic duct dilated by biliary sludge (pink asterisk) with a normal gallbladder (green arrow head) and the newborn was hospitalized. A hepatobiliary scintigraphy was performed and showed nonvisualization of the intrahepatic–extrahepatic biliary tract and no activity in the bowel for 1 hour after injection. These findings indicated biliary atresia. On hospital day 7, a MR cholangiopancreatography (MRC; Figure B) was done, revealing a cystic duct (pink asterisk) inserted into the distal choledochus (near to the ampulla of Vater) and a filling defect of the cystic duct (green arrow head) that was causing dilatation of it and external compression of the choledochus (blue asterisk). Colagogues, fat soluble vitamins, and ursodeoxicolic acid were administered according to the standard management of neonatal cholestasis. The patient’s parents preferred to continue with medical follow up and conservative management, and they refused invasive treatments. Despite this, clinical evolution was satisfactory over the first three months. What is your diagnosis? See the Gastroenterology web site (www.gastrojournal.org) for more information on submitting your favorite image to Clinical Challenges and Images in GI.
Conflicts of interest The authors disclose no conflicts. © 2015 by the AGA Institute 0016-5085/$36.00 http://dx.doi.org/10.1053/j.gastro.2014.10.061
Gastroenterology 2015;148:e12–e13
ELECTRONIC CLINICAL CHALLENGES AND IMAGES IN GI Answer to the Clinical Challenges and Images in GI Question: Image 6: Mirizzi Syndrome These findings are consistent with a biliary sludge impaction in an anatomic variant of the cystic duct1 that produces external compression of the extrahepatic bile duct, resulting in biliary obstruction, which is known as Mirizzi syndrome (MS). MS is a rare cause of obstructive jaundice in adults,2 and never reported in newborns. The clinical diagnosis is difficult since there are no pathognomonic patterns of presentation. Abdominal US is used widely for the initial screening and it may show a contracted gallbladder with a gallstone impacted in the infundibulum and an hepatic duct dilated above the level of obstruction, but the reported diagnostic accuracy for US in MS is 29%. To confirm the diagnosis, endoscopic retrograde cholangiopancreatography and/or MRC must be performed, because both tests allow an optimal visualization of the cystic duct and the biliary tract. In this case, MRC was done to avoid the complications of direct cholangiography and led to a diagnosis of MS.3 In conclusion, physicians should be aware that MS, although rare, may occur during the neonatal period and should be considered in the differential diagnosis of cholestatic jaundice.
References 1. 2. 3.
Turner MA, Fulcher AS. The cystic duct: normal anatomy and disease processes. Radiographics 2001;21:3–22. Lai EC, Lau WY. Mirizzi syndrome: history, present and future development. ANZ J Surg 2006;76:251–257. Beltrán MA. Mirizzi syndrome: history, current knowledge and proposal of a simplified classification. World J Gastroenterol 2012;18:4639–4650.
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