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Congential Extrahepatic Portocaval Shunt: Growth in Vain
Congential Extrahepatic Portocaval Shunt: Growth in Vain
A
12-year-old boy was evaluated for jaundice, loss of appetite, and weight loss. He denied abdominal pain, fever, vomiting, diarrhea, and night sweats. He was afebrile, and his blood pressure and heart rate were within normal limits. Findings of the physical examination revealed pallor and hepatomegaly without tenderness. The patient was mildly anemic with a hemoglobin measurement of 11.1 g/dL. Liver functions tests were abnormal, with alanine aminotransferase and aspartate aminotransferase levels measuring 60 and 71 IU/L, respectively, serum bilirubin measuring 1.3 g/dL, serum alkaline phosphatase 550 IU/L, and gamma glutamyl transferase 165 IU/L. The total protein was 6.8 g/dL with a slightly low albumin of 3.1 g/dL and reversed albumin/globulin ratio. Quadriphasic contrastenhanced computed tomography (CECT) of the abdomen revealed an extrahepatic portocaval shunt (Figures 1 and 2; Figure 1 available at www.jpeds.com), the absence of the intrahepatic portal vein, and several circumscribed nodules in the liver that remained hypoattenuating on all phases of the CT study (Figure 3). A diagnosis of congenital
Figure 3. Axial CECT image of the liver shows absence of the intrahepatic portal vein tributaries and hypodense regenerative nodules. No evidence of biliary dilation is seen.
extrahepatic portocaval shunt with regenerative nodules in the liver was made. The Abernethy malformation is a congenital anomaly in which splanchnic blood drains directly into the inferior vena cava.1 The diversion of splanchnic blood into the inferior vena cava may be complete in the form of an end-toside portocaval anastomosis with deficient intrahepatic portal veins (Abernethy type 1) or partial, via a side-to-side portocaval union (Abernethy type 2).2 Patients may be asymptomatic or may develop mild liver dysfunction with transaminitis.3 Growth retardation may also be a presentation, as highlighted in our case. Uncommon clinical presentations include hyperammonemia, hepatic encephalopathy, pulmonary hypertension, and hepatopulmonary syndrome.4,5 Regenerative nodules and hepatic neoplasms have been reported in 40% of published cases; therefore, some patients present with a hepatic mass.6,7 Definitive treatment includes liver transplantation for type I malformation and surgical or transcatheter shunt occlusion in type II malformation.8,9 n
Figure 2. Reformatted coronal maximum intensity projection CECT image demonstrates an extrahepatic shunt connecting the portal vein to the inferior vena cava.
Prabhakaran Velayutham, MD Ankur Dev, FRCR Ankur Arora, FRCR Department of Radiology Institute of Liver and Biliary Sciences New Delhi, India
J Pediatr 2013;162:1076. 0022-3476/$ - see front matter. Copyright ª 2013 Mosby Inc. All rights reserved. http://dx.doi.org/10.1016/j.jpeds.2012.11.066
1076
References available at www.jpeds.com
Vol. 162, No. 5 May 2013
References 1. Abernethy J. Account of two instances of uncommon formation in the viscera of the human body. Phil Trans R Soc 1793;83:59-66. 2. Morgan G, Superina R. Congenital absence of the portal vein: two cases and a proposed classification system for portosystemic vascular anomalies. J Pediatr Surg 1994;29:1239-41. 3. Murray CP, Yoo SJ, Babyn PS. Congenital extrahepatic portosystemic shunts (review). Pediatr Radiol 2003;33:614-20. 4. Shinkai M, Ohhama Y, Nishi T, Yamamoto H, Fujita S, Take H, et al. Congenital absence of the portal vein and role of liver transplantation in children. J Pediatr Surg 2001;36:1026-31.
5. Osorio MJ, Bonow A, Bond GJ, et al. Abernethy malformation complicated by hepatopulmonary syndrome and a liver mass successfully treated by liver transplantation. Pediatr Transplant 2011;15:E149-51. 6. Chandler TM, Heran MK, Chang SD, Parvez A, Harris AC. Multiple focal nodular hyperplasia lesions of the liver associated with congenital absence of the portal vein. Magn Reson Imaging 2011;29:881-6. 7. Mistinova J, Valacsai F, Varga I. Congenital absence of the portal vein— Case report and a review of literature. Clin Anat 2010;23:750-8. 8. Woodle ES, Thistlethwaite JR, Emond JC, et al. Successful hepatic transplantation in congenital absence of recipient portal vein. Surgery 1990; 107:475-9. 9. Ikeda S, Sera Y, Ohshiro H, et al. Surgical indications for patients with hyperammonemia. J Pediatr Surg 1999;34:1012-5.
Figure 1. Axial CECT maximum intensity projection image of the abdomen shows anomalous drainage of the portal vein into the infrahepatic inferior vena cava. 1076.e1
A
12-year-old boy was evaluated for jaundice, loss of appetite, and weight loss. He denied abdominal pain, fever, vomiting, diarrhea, and night sweats. He was afebrile, and his blood pressure and heart rate were within normal limits. Findings of the physical examination revealed pallor and hepatomegaly without tenderness. The patient was mildly anemic with a hemoglobin measurement of 11.1 g/dL. Liver functions tests were abnormal, with alanine aminotransferase and aspartate aminotransferase levels measuring 60 and 71 IU/L, respectively, serum bilirubin measuring 1.3 g/dL, serum alkaline phosphatase 550 IU/L, and gamma glutamyl transferase 165 IU/L. The total protein was 6.8 g/dL with a slightly low albumin of 3.1 g/dL and reversed albumin/globulin ratio. Quadriphasic contrastenhanced computed tomography (CECT) of the abdomen revealed an extrahepatic portocaval shunt (Figures 1 and 2; Figure 1 available at www.jpeds.com), the absence of the intrahepatic portal vein, and several circumscribed nodules in the liver that remained hypoattenuating on all phases of the CT study (Figure 3). A diagnosis of congenital
Figure 3. Axial CECT image of the liver shows absence of the intrahepatic portal vein tributaries and hypodense regenerative nodules. No evidence of biliary dilation is seen.
extrahepatic portocaval shunt with regenerative nodules in the liver was made. The Abernethy malformation is a congenital anomaly in which splanchnic blood drains directly into the inferior vena cava.1 The diversion of splanchnic blood into the inferior vena cava may be complete in the form of an end-toside portocaval anastomosis with deficient intrahepatic portal veins (Abernethy type 1) or partial, via a side-to-side portocaval union (Abernethy type 2).2 Patients may be asymptomatic or may develop mild liver dysfunction with transaminitis.3 Growth retardation may also be a presentation, as highlighted in our case. Uncommon clinical presentations include hyperammonemia, hepatic encephalopathy, pulmonary hypertension, and hepatopulmonary syndrome.4,5 Regenerative nodules and hepatic neoplasms have been reported in 40% of published cases; therefore, some patients present with a hepatic mass.6,7 Definitive treatment includes liver transplantation for type I malformation and surgical or transcatheter shunt occlusion in type II malformation.8,9 n
Figure 2. Reformatted coronal maximum intensity projection CECT image demonstrates an extrahepatic shunt connecting the portal vein to the inferior vena cava.
Prabhakaran Velayutham, MD Ankur Dev, FRCR Ankur Arora, FRCR Department of Radiology Institute of Liver and Biliary Sciences New Delhi, India
J Pediatr 2013;162:1076. 0022-3476/$ - see front matter. Copyright ª 2013 Mosby Inc. All rights reserved. http://dx.doi.org/10.1016/j.jpeds.2012.11.066
1076
References available at www.jpeds.com
Vol. 162, No. 5 May 2013
References 1. Abernethy J. Account of two instances of uncommon formation in the viscera of the human body. Phil Trans R Soc 1793;83:59-66. 2. Morgan G, Superina R. Congenital absence of the portal vein: two cases and a proposed classification system for portosystemic vascular anomalies. J Pediatr Surg 1994;29:1239-41. 3. Murray CP, Yoo SJ, Babyn PS. Congenital extrahepatic portosystemic shunts (review). Pediatr Radiol 2003;33:614-20. 4. Shinkai M, Ohhama Y, Nishi T, Yamamoto H, Fujita S, Take H, et al. Congenital absence of the portal vein and role of liver transplantation in children. J Pediatr Surg 2001;36:1026-31.
5. Osorio MJ, Bonow A, Bond GJ, et al. Abernethy malformation complicated by hepatopulmonary syndrome and a liver mass successfully treated by liver transplantation. Pediatr Transplant 2011;15:E149-51. 6. Chandler TM, Heran MK, Chang SD, Parvez A, Harris AC. Multiple focal nodular hyperplasia lesions of the liver associated with congenital absence of the portal vein. Magn Reson Imaging 2011;29:881-6. 7. Mistinova J, Valacsai F, Varga I. Congenital absence of the portal vein— Case report and a review of literature. Clin Anat 2010;23:750-8. 8. Woodle ES, Thistlethwaite JR, Emond JC, et al. Successful hepatic transplantation in congenital absence of recipient portal vein. Surgery 1990; 107:475-9. 9. Ikeda S, Sera Y, Ohshiro H, et al. Surgical indications for patients with hyperammonemia. J Pediatr Surg 1999;34:1012-5.
Figure 1. Axial CECT maximum intensity projection image of the abdomen shows anomalous drainage of the portal vein into the infrahepatic inferior vena cava. 1076.e1