Surg Radiol Anat DOI 10.1007/s00276-015-1460-5
ANATOMIC VARIATIONS
Multiple regenerative nodular hyperplasia in the left infrarenal vena cava accompanied by abernethy malformation Zuhal Gu¨ls¸ en1 • Hasan Yig˘it1 • Pelin Demir1
Received: 12 May 2014 / Accepted: 16 March 2015 Ó Springer-Verlag France 2015
Abstract The current study presents the case of a 19-year-old male patient who was detected with an increased alanine aminotransferase and aspartate transaminase levels during a preoperative evaluation of the right inguinal hernia operation and was later found to have Abernethy malformation accompanied by multiple regenerative nodular hyperplasia and left intra renal inferior vena cava. Regenerative nodular hyperplasia accompanying these two abnormalities is extremely rare and to the best of our knowledge, such a case has not been reported to date. Abdominal ultrasound (US) and color Doppler US, dynamic abdominal magnetic resonance imaging (MRI), and portography examinations were performed and a type 2 abernethy malformation, partial malrotation of the inferior vena cava, and regenerative nodular hyperplasia were detected. We aimed to discuss the radiological signs of these two accompanying abnormalities with a literature review. Keywords Abernethy malformation Inferior vena cava transposition Regenerative nodular hyperplasia Magnetic resonance imaging
Introduction The Abernethy malformation is a congenital extrahepatic anastomosis of the portal vein and systemic circulation. It was described by John Abernethy [2]. Morgan and Superina further classified the congenital extrahepatic & Zuhal Gu¨ls¸ en [email protected] 1
Department of Radiology, Ankara Education and Training Hospital, Altindag, Ankara, Turkey
portosystemic shunt into two types, according to the type of anastomosis and presence of the portal vein [9]. All portal venous blood empties into the inferior vena cava (IVC) in type 1 abernethy malformations; while, it drains partially into the inferior vena cava, since there is a side-to-side anastomosis [3] (Fig. 1). Accompanying abnormalities are more frequent in type 1 malformations compared to type 2 malformations. Female and male predominance is generally seen in types 1 and 2 cases [10]. Complications of uncorrected abernethy malformations are hepatic encephalopathy, hepatopulmonary syndrome, portopulmonary syndrome, and hepatic tumors. Anomalies of the inferior vena cava (IVC) and its tributaries have been known to anatomists since 1793, when Abernethy described a congenital mesocaval shunt and azygos continuation of the IVC in a 10-month-old infant with polysplenia and dextrocardia [2]. The infrahepatic IVC develops between the 6th and 8th weeks of embryonic life as a composite structure formed from the continuous appearance and regression of three paired embryonic veins. In order of appearance, they are the posterior cardinal, the subcardinal, and the supracardinal veins. Persistence of infra renal part of left supracardinal vein, results in formation of left inferior vena cava [5]. The embryogenesis of the IVC is a complex process involving the formation of several anastomoses between three paired embryonic veins [3] (Figs. 2, 3). A 19-year-old male patient who had Abernethy malformation accompanied by an inferior vena cava with a left infrarenal course is presented herein. Regenerative nodular hyperplasia is extremely rare, accompanying these two abnormalities and no other case has been reported up to date to the best of our knowledge. We aimed to discuss the radiological signs of these two accompanying abnormalities with a literature review.
123
Surg Radiol Anat
Fig. 1 Abernethy malformation
Fig. 2 Conceptual framework for development of the IVC. Composite schematic shows the relative positions and interrelationships of the three paired embryonic vessels that contribute to development of the IVC. The pictured veins are not all present simultaneously. card cardinal, post posterior, SMA superior mesenteric artery, v vein, 1 intersubcardinal anastomosis, 2 intersupracardinal anastomosis
Case report A 19-year-old male patient presented to our hospital with complaints of a right inguinal hernia and was detected to have elevated alanine aminotransferase (ALT) and
123
aspartate transaminase (AST) levels preoperatively; an abdominal US was ordered. The abdominal US revealed hepatomegaly and multiple hyperechoic lesions with the largest diameter of 8 cm. These lesions had thin hypoechoic halo around the periphery.
Surg Radiol Anat
the advancing phases, which were iso- slight hyperintense compared to the liver in the hepatic phase, were diagnosed in favor of regenerative nodular hyperplasia (Fig. 7a, b). The patient was observed conservatively. At the 6-month follow-up, the dimensions of the lesions were found to be unchanged by ultrasonography.
Discussion Congenital extra hepatic portosystemic shunt is a rare congenital anomaly that was first described by Abernethy [2]. To the best of our knowledge, only 22 cases of type 2 abernethy malformations have been reported to date [10].
Fig. 3 A left IVC terminating at the left renal vein
In addition, a side-to-side anastomosis was present between the extrahepatic portion of the portal vein and IVC (Fig. 4). Doppler examination revealed a hepatopedal flow inside the PV and a flow in the direction of the IVC in the portocaval shunt. The distal IVC raised at the left side up to the level of the renal vein and crossed to the right, passing anterior to the aorta at this level. The IVC was in its natural course at the suprarenal level. No IVC was seen in the right side at the infrarenal level. This situation was diagnosed as vena cava transposition (Figs. 5a, b, 6). MRI and portography were performed to provide a more detailed image of the lesions in the liver and the vascular structures. Multiple lesions in the T2A series in the liver with a peripheral hypointense rim were detected. The hyperintense lesions which opacify in the early arterial phase in the contrast enhanced series and become isointense in
Fig. 4 Color Doppler ultrasonographic image of anastomosis between the portal vein and IVC
Fig. 5 a, b MRI portography shows inferior vena cava progressed on the left up to the level of renal vein
123
Surg Radiol Anat
Fig. 6 IVC observed on the left of the aorta in dynamic-contrast series
Fig. 7 a Portocaval shunt (small arrow) and hyperintense lesion with hypointense rim (long arrow) at T2A. b Regenerative nodular hyperplasia in portal venous phase, which is iso-slight hyperintense compared to the liver (arrow)
The simultaneous presence of the transposition of the IVC (also known as left-sided IVC) has not been reported in previously published case reports in the literature.
123
The portal venous system develops between the fourth and tenth weeks of embryonic life by selective apoptosis of some portions of the vitelline veins. The IVC also develops at this time, which leads to the potential for congenital portosystemic shunts [10]. Portosystemic shunt anomalies have been classified into two types [9]. Type 1 shunts are characterized by the absence of the intrahepatic PV and complete end-to-side shunt, and have two subtypes, (1) separate drainage of the superior mesenteric and splenic veins into the IVC, iliac veins, or renal veins (subtype Ia), and (2) superior mesenteric and splenic veins joining to form a short extrahepatic PV which drains into the IVC (subtype Ib). Type 2 shunts are marked by presence of a patent intrahepatic PV and a partial side-to-side shunt. The patient presented herein had a type 2 malformation with a side-to-side portocaval shunt. Congenital extra hepatic portosystemic shunts are also associated with an increased frequency of hepatic neoplasms, which may be benign (focal nodular hyperplasia, hepatocellular adenoma, or nodular regenerative hyperplasia) or malignant (hepatocellular carcinoma or hepatoblastoma) [7, 10, 13]. It has been suggested that the disequilibrium of hepatic circulation between the hepatic artery and PV may provide an environment for the development of neoplastic tumor in these patients [8]. Malignant transformation of benign neoplasms has also been reported in this setting, and therefore, long-term follow-up and monitoring are recommended for these patients [8]. The development of regenerative nodular hyperplasia is due to the abnormal hepatic cell response that evolves in response to the anastomosis in portal vein. In some cases, the fact that there are blood vessels flowing through the lesions observed in the liver made us consider that there is a perfusion anomaly rather than a neoplasm [6]. A biopsy from one of the largest lesions detected in the liver revealed a benign lesion. Currently, a diagnosis of Abernethy malformation is usually made by noninvasive cross-sectional imaging techniques such as ultrasound, computed tomography or MRI which show the shunt and any intrahepatic PV branches. However, liver biopsy may be necessary in patients with suspected type 1 malformation, since an occasional patient may have small PV radicals which cannot be seen on ultrasound but can be observed on liver biopsy [4]. Determining the type of shunt is particularly important in the planning of treatment. In patients with type I malformation, occlusion of the shunt is not an option, since it represents the only drainage route for the mesenteric venous blood. Hence, these patients merit clinical, biochemical, and imaging follow-up; for those who develop severe hepatic encephalopathy or malignant liver nodules,
Surg Radiol Anat
liver transplantation is the only treatment option [4, 10]. For patients with type 2 malformations and serious symptoms such as hepatic encephalopathy, shunt occlusion can be performed, either surgically or by percutaneous transcatheter coil placement [4]. Congenital intra-hepatic portosystemic shunts can present in the early neonatal period with growth restriction, galactosemia, neonatal cholestasis. In countries having neonatal screening programmes, some cases can be diagnosed by neonatal screening tests due to presence of galactosemia [11]. Many patients are diagnosed due to associated defects like heart disease, which are present in up-to 60 % of the patients [14]. Anomalies of the inferior vena cava (IVC) and its tributaries have been known to anatomists since 1793, when Abernethy described a congenital mesocaval shunt and azygos continuation of the IVC in a 10-month-old infant with polysplenia and dextrocardia [2]. Composite schematic shows the relative positions and interrelationships of the three paired embryonic vessels that contribute to development of the IVC (Fig. 2). Under ordinary circumstances, the prerenal division is formed from union of the hepatic segment (purple area), a vitelline vein derivative, and the right subcardinal vein (lavender area). The renal segment is formed from the suprasubcardinal anastomosis (pink area) and the postsubcardinal anastomosis (blue area). The infrarenal segment derives from the right supracardinal vein (goldenrod area). The iliac veins form from the posterior cardinal veins (violet area). card: cardinal, post: posterior, SMA: superior mesenteric artery, v: vein, 1: intersubcardinal anastomosis, 2: intersupracardinal anastomosis. Embryologically, the normal IVC is composed of four segments: hepatic, suprarenal, renal and infrarenal [1]. A left IVC results from regression of the right supracardinal vein with persistence of the left supracardinal vein. The prevalence is 0.2–0.5 % [12]. Typically, the left IVC joins the left renal vein, which crosses anterior to the aorta in the normal fashion, uniting with the right renal vein to form a normal right-sided prerenal IVC. The distal inferior vena cava in our case progressed on the left, up to the level of renal vein and at this level, it passed towards the right, in front of the aorta along with the left renal vein. Woodle et al. [15] described a 10-year-old girl with congenital portocaval shunt type 1, biliary atresia, cardiac and inferior vena cava anomalies, and polysplenia. Wojcicki et al. described a 45-year-old man with congenital portocaval shunt type 1 and end-stage renal insufficiency due to focal segmental glomerulopathy, who developed therapyresistant encephalopathy with intermittently high ammonia levels. The patient underwent a combined liver and kidney transplantation and histopathological examination of the
native liver showed no portal vein branches in the portal tracts [14]. Due to the widespread use of cross-sectional imaging, the presence of congenital IVC abnormalities has increasingly been reported in asymptomatic patients. The major clinical significance of this anomaly is the potential for misdiagnosis as left-sided paraaortic adenopathy [3]. Also, the clinical importance of the left IVC is during transjugular access of the infrarenal left IVC (for placement of an IVC filter may be difficult) or during left-sided donor nephrectomy. Acknowledgments I would like to thank Dr. Seda Uyg˘un, who is an anatomist in Faculty of Medicine, Hacettepe University for drawing figures 1–3. Conflict of interest The authors certify that they have no affiliation with or financial involvement in any organization or entity with a direct financial interest in the subject matter or materials discussed in the manuscript (e.g., employment, consultancies, stock ownership, honoraria).
References 1. Abdullah A, Williamson K, Lewis T et al (2011) Variant ventral intrahepatic course of inferior vena cava: volume rendering and maximum intensity projection CT findings. Br J Radiol 84:e135– e137 2. Abernethy J (1793) Account of two instances of uncommon formation in the viscera of the human body. Phil Trans R Soc 83:59–66 3. Bass JE, Redwine MD, Kramer LA et al (2000) Spectrum of congenital anomalies of the inferior vena cava: cross-sectional imaging findings. Radiographics 20:639–652 4. Collard B, Maleux G, Heye S et al (2006) Value of carbon dioxide wedged venography and transvenous liver biopsy in the definitive diagnosis of Abernethy malformation. Abdom Imaging 31:315–319 5. Eid N, Ito Y, Otsuki Y (2013) The left inferior vena cava: a surgically important variant tributary of left renal vein. Surg Radiol Anat 35(5):455–456 6. Grazioli L, Alberti D, Olivetti L et al (2000) Congenital absence of portal vein with nodular regenerative hyperplasia of the liver: a case report. Eur Radiol 10:820–825 7. Kanamori Y, Hashizume K, Kitano Y et al (2003) Congenital extrahepatic portacaval shunt (abernethy type 2), huge liver mass, and patent ductus arteriosus: a case report of its rare clinical presentation in a young girl. J Pediatr Surg 38:E15 8. Kondo F, Nagao T, Sato T et al (1998) Etiological analysis of focal nodular hyperplasia of the liver, with emphasis on similar abnormal vasculatures to nodular regenerative hyperplasia and idiopathic portal hypertension. Pathol Res Pract 194:487–495 9. Morgan G, Superina R (1994) Congenital absence of the portal vein: two cases and a proposed classification system for portosystemic vascular anomalies. J Ped Surg 29:1239–1241 10. Murray CP, Yoo SJ, Babyn PS (2003) Congenital extrahepatic portosystemic shunts. Pediatr Radiol 33:614–620 11. Pathak A, Agarwal N, Mandliya J et al (2012) Abernethy malformation: a case report. BMC Pediatrics 12:57 12. Phillips E (1969) Embryology, normal anatomy, and anomalies. In: Ferris EJ, Hipona FA, Kahn PC, Phillips E, Shapiro JH (eds)
123
Surg Radiol Anat Venography of the inferior vena cava and its branches. Williams & Wilkins, Baltimore, pp 1–32 13. Turkbey B, Karcaaltincaba M, Demir H et al (2006) Multiple hyperplastic nodules in the liver with congenital absence of portal vein: MRI findings. Pediatr Radiol 36:445–448 14. Wojcicki M, Haagsma EB, Gouw AS et al (2004) Orthotopic liver transplantation for portosystemic encephalopathy in an adult
123
with congenital absence of the portal vein. Liver Transplant 10(9):1203–1207 15. Woodle ES, Thistlethwaite JR, Emond JC et al (1990) Successful hepatic transplantation in congenital absence of recipient portal vein. Surgery 107:475–479
ANATOMIC VARIATIONS
Multiple regenerative nodular hyperplasia in the left infrarenal vena cava accompanied by abernethy malformation Zuhal Gu¨ls¸ en1 • Hasan Yig˘it1 • Pelin Demir1
Received: 12 May 2014 / Accepted: 16 March 2015 Ó Springer-Verlag France 2015
Abstract The current study presents the case of a 19-year-old male patient who was detected with an increased alanine aminotransferase and aspartate transaminase levels during a preoperative evaluation of the right inguinal hernia operation and was later found to have Abernethy malformation accompanied by multiple regenerative nodular hyperplasia and left intra renal inferior vena cava. Regenerative nodular hyperplasia accompanying these two abnormalities is extremely rare and to the best of our knowledge, such a case has not been reported to date. Abdominal ultrasound (US) and color Doppler US, dynamic abdominal magnetic resonance imaging (MRI), and portography examinations were performed and a type 2 abernethy malformation, partial malrotation of the inferior vena cava, and regenerative nodular hyperplasia were detected. We aimed to discuss the radiological signs of these two accompanying abnormalities with a literature review. Keywords Abernethy malformation Inferior vena cava transposition Regenerative nodular hyperplasia Magnetic resonance imaging
Introduction The Abernethy malformation is a congenital extrahepatic anastomosis of the portal vein and systemic circulation. It was described by John Abernethy [2]. Morgan and Superina further classified the congenital extrahepatic & Zuhal Gu¨ls¸ en [email protected] 1
Department of Radiology, Ankara Education and Training Hospital, Altindag, Ankara, Turkey
portosystemic shunt into two types, according to the type of anastomosis and presence of the portal vein [9]. All portal venous blood empties into the inferior vena cava (IVC) in type 1 abernethy malformations; while, it drains partially into the inferior vena cava, since there is a side-to-side anastomosis [3] (Fig. 1). Accompanying abnormalities are more frequent in type 1 malformations compared to type 2 malformations. Female and male predominance is generally seen in types 1 and 2 cases [10]. Complications of uncorrected abernethy malformations are hepatic encephalopathy, hepatopulmonary syndrome, portopulmonary syndrome, and hepatic tumors. Anomalies of the inferior vena cava (IVC) and its tributaries have been known to anatomists since 1793, when Abernethy described a congenital mesocaval shunt and azygos continuation of the IVC in a 10-month-old infant with polysplenia and dextrocardia [2]. The infrahepatic IVC develops between the 6th and 8th weeks of embryonic life as a composite structure formed from the continuous appearance and regression of three paired embryonic veins. In order of appearance, they are the posterior cardinal, the subcardinal, and the supracardinal veins. Persistence of infra renal part of left supracardinal vein, results in formation of left inferior vena cava [5]. The embryogenesis of the IVC is a complex process involving the formation of several anastomoses between three paired embryonic veins [3] (Figs. 2, 3). A 19-year-old male patient who had Abernethy malformation accompanied by an inferior vena cava with a left infrarenal course is presented herein. Regenerative nodular hyperplasia is extremely rare, accompanying these two abnormalities and no other case has been reported up to date to the best of our knowledge. We aimed to discuss the radiological signs of these two accompanying abnormalities with a literature review.
123
Surg Radiol Anat
Fig. 1 Abernethy malformation
Fig. 2 Conceptual framework for development of the IVC. Composite schematic shows the relative positions and interrelationships of the three paired embryonic vessels that contribute to development of the IVC. The pictured veins are not all present simultaneously. card cardinal, post posterior, SMA superior mesenteric artery, v vein, 1 intersubcardinal anastomosis, 2 intersupracardinal anastomosis
Case report A 19-year-old male patient presented to our hospital with complaints of a right inguinal hernia and was detected to have elevated alanine aminotransferase (ALT) and
123
aspartate transaminase (AST) levels preoperatively; an abdominal US was ordered. The abdominal US revealed hepatomegaly and multiple hyperechoic lesions with the largest diameter of 8 cm. These lesions had thin hypoechoic halo around the periphery.
Surg Radiol Anat
the advancing phases, which were iso- slight hyperintense compared to the liver in the hepatic phase, were diagnosed in favor of regenerative nodular hyperplasia (Fig. 7a, b). The patient was observed conservatively. At the 6-month follow-up, the dimensions of the lesions were found to be unchanged by ultrasonography.
Discussion Congenital extra hepatic portosystemic shunt is a rare congenital anomaly that was first described by Abernethy [2]. To the best of our knowledge, only 22 cases of type 2 abernethy malformations have been reported to date [10].
Fig. 3 A left IVC terminating at the left renal vein
In addition, a side-to-side anastomosis was present between the extrahepatic portion of the portal vein and IVC (Fig. 4). Doppler examination revealed a hepatopedal flow inside the PV and a flow in the direction of the IVC in the portocaval shunt. The distal IVC raised at the left side up to the level of the renal vein and crossed to the right, passing anterior to the aorta at this level. The IVC was in its natural course at the suprarenal level. No IVC was seen in the right side at the infrarenal level. This situation was diagnosed as vena cava transposition (Figs. 5a, b, 6). MRI and portography were performed to provide a more detailed image of the lesions in the liver and the vascular structures. Multiple lesions in the T2A series in the liver with a peripheral hypointense rim were detected. The hyperintense lesions which opacify in the early arterial phase in the contrast enhanced series and become isointense in
Fig. 4 Color Doppler ultrasonographic image of anastomosis between the portal vein and IVC
Fig. 5 a, b MRI portography shows inferior vena cava progressed on the left up to the level of renal vein
123
Surg Radiol Anat
Fig. 6 IVC observed on the left of the aorta in dynamic-contrast series
Fig. 7 a Portocaval shunt (small arrow) and hyperintense lesion with hypointense rim (long arrow) at T2A. b Regenerative nodular hyperplasia in portal venous phase, which is iso-slight hyperintense compared to the liver (arrow)
The simultaneous presence of the transposition of the IVC (also known as left-sided IVC) has not been reported in previously published case reports in the literature.
123
The portal venous system develops between the fourth and tenth weeks of embryonic life by selective apoptosis of some portions of the vitelline veins. The IVC also develops at this time, which leads to the potential for congenital portosystemic shunts [10]. Portosystemic shunt anomalies have been classified into two types [9]. Type 1 shunts are characterized by the absence of the intrahepatic PV and complete end-to-side shunt, and have two subtypes, (1) separate drainage of the superior mesenteric and splenic veins into the IVC, iliac veins, or renal veins (subtype Ia), and (2) superior mesenteric and splenic veins joining to form a short extrahepatic PV which drains into the IVC (subtype Ib). Type 2 shunts are marked by presence of a patent intrahepatic PV and a partial side-to-side shunt. The patient presented herein had a type 2 malformation with a side-to-side portocaval shunt. Congenital extra hepatic portosystemic shunts are also associated with an increased frequency of hepatic neoplasms, which may be benign (focal nodular hyperplasia, hepatocellular adenoma, or nodular regenerative hyperplasia) or malignant (hepatocellular carcinoma or hepatoblastoma) [7, 10, 13]. It has been suggested that the disequilibrium of hepatic circulation between the hepatic artery and PV may provide an environment for the development of neoplastic tumor in these patients [8]. Malignant transformation of benign neoplasms has also been reported in this setting, and therefore, long-term follow-up and monitoring are recommended for these patients [8]. The development of regenerative nodular hyperplasia is due to the abnormal hepatic cell response that evolves in response to the anastomosis in portal vein. In some cases, the fact that there are blood vessels flowing through the lesions observed in the liver made us consider that there is a perfusion anomaly rather than a neoplasm [6]. A biopsy from one of the largest lesions detected in the liver revealed a benign lesion. Currently, a diagnosis of Abernethy malformation is usually made by noninvasive cross-sectional imaging techniques such as ultrasound, computed tomography or MRI which show the shunt and any intrahepatic PV branches. However, liver biopsy may be necessary in patients with suspected type 1 malformation, since an occasional patient may have small PV radicals which cannot be seen on ultrasound but can be observed on liver biopsy [4]. Determining the type of shunt is particularly important in the planning of treatment. In patients with type I malformation, occlusion of the shunt is not an option, since it represents the only drainage route for the mesenteric venous blood. Hence, these patients merit clinical, biochemical, and imaging follow-up; for those who develop severe hepatic encephalopathy or malignant liver nodules,
Surg Radiol Anat
liver transplantation is the only treatment option [4, 10]. For patients with type 2 malformations and serious symptoms such as hepatic encephalopathy, shunt occlusion can be performed, either surgically or by percutaneous transcatheter coil placement [4]. Congenital intra-hepatic portosystemic shunts can present in the early neonatal period with growth restriction, galactosemia, neonatal cholestasis. In countries having neonatal screening programmes, some cases can be diagnosed by neonatal screening tests due to presence of galactosemia [11]. Many patients are diagnosed due to associated defects like heart disease, which are present in up-to 60 % of the patients [14]. Anomalies of the inferior vena cava (IVC) and its tributaries have been known to anatomists since 1793, when Abernethy described a congenital mesocaval shunt and azygos continuation of the IVC in a 10-month-old infant with polysplenia and dextrocardia [2]. Composite schematic shows the relative positions and interrelationships of the three paired embryonic vessels that contribute to development of the IVC (Fig. 2). Under ordinary circumstances, the prerenal division is formed from union of the hepatic segment (purple area), a vitelline vein derivative, and the right subcardinal vein (lavender area). The renal segment is formed from the suprasubcardinal anastomosis (pink area) and the postsubcardinal anastomosis (blue area). The infrarenal segment derives from the right supracardinal vein (goldenrod area). The iliac veins form from the posterior cardinal veins (violet area). card: cardinal, post: posterior, SMA: superior mesenteric artery, v: vein, 1: intersubcardinal anastomosis, 2: intersupracardinal anastomosis. Embryologically, the normal IVC is composed of four segments: hepatic, suprarenal, renal and infrarenal [1]. A left IVC results from regression of the right supracardinal vein with persistence of the left supracardinal vein. The prevalence is 0.2–0.5 % [12]. Typically, the left IVC joins the left renal vein, which crosses anterior to the aorta in the normal fashion, uniting with the right renal vein to form a normal right-sided prerenal IVC. The distal inferior vena cava in our case progressed on the left, up to the level of renal vein and at this level, it passed towards the right, in front of the aorta along with the left renal vein. Woodle et al. [15] described a 10-year-old girl with congenital portocaval shunt type 1, biliary atresia, cardiac and inferior vena cava anomalies, and polysplenia. Wojcicki et al. described a 45-year-old man with congenital portocaval shunt type 1 and end-stage renal insufficiency due to focal segmental glomerulopathy, who developed therapyresistant encephalopathy with intermittently high ammonia levels. The patient underwent a combined liver and kidney transplantation and histopathological examination of the
native liver showed no portal vein branches in the portal tracts [14]. Due to the widespread use of cross-sectional imaging, the presence of congenital IVC abnormalities has increasingly been reported in asymptomatic patients. The major clinical significance of this anomaly is the potential for misdiagnosis as left-sided paraaortic adenopathy [3]. Also, the clinical importance of the left IVC is during transjugular access of the infrarenal left IVC (for placement of an IVC filter may be difficult) or during left-sided donor nephrectomy. Acknowledgments I would like to thank Dr. Seda Uyg˘un, who is an anatomist in Faculty of Medicine, Hacettepe University for drawing figures 1–3. Conflict of interest The authors certify that they have no affiliation with or financial involvement in any organization or entity with a direct financial interest in the subject matter or materials discussed in the manuscript (e.g., employment, consultancies, stock ownership, honoraria).
References 1. Abdullah A, Williamson K, Lewis T et al (2011) Variant ventral intrahepatic course of inferior vena cava: volume rendering and maximum intensity projection CT findings. Br J Radiol 84:e135– e137 2. Abernethy J (1793) Account of two instances of uncommon formation in the viscera of the human body. Phil Trans R Soc 83:59–66 3. Bass JE, Redwine MD, Kramer LA et al (2000) Spectrum of congenital anomalies of the inferior vena cava: cross-sectional imaging findings. Radiographics 20:639–652 4. Collard B, Maleux G, Heye S et al (2006) Value of carbon dioxide wedged venography and transvenous liver biopsy in the definitive diagnosis of Abernethy malformation. Abdom Imaging 31:315–319 5. Eid N, Ito Y, Otsuki Y (2013) The left inferior vena cava: a surgically important variant tributary of left renal vein. Surg Radiol Anat 35(5):455–456 6. Grazioli L, Alberti D, Olivetti L et al (2000) Congenital absence of portal vein with nodular regenerative hyperplasia of the liver: a case report. Eur Radiol 10:820–825 7. Kanamori Y, Hashizume K, Kitano Y et al (2003) Congenital extrahepatic portacaval shunt (abernethy type 2), huge liver mass, and patent ductus arteriosus: a case report of its rare clinical presentation in a young girl. J Pediatr Surg 38:E15 8. Kondo F, Nagao T, Sato T et al (1998) Etiological analysis of focal nodular hyperplasia of the liver, with emphasis on similar abnormal vasculatures to nodular regenerative hyperplasia and idiopathic portal hypertension. Pathol Res Pract 194:487–495 9. Morgan G, Superina R (1994) Congenital absence of the portal vein: two cases and a proposed classification system for portosystemic vascular anomalies. J Ped Surg 29:1239–1241 10. Murray CP, Yoo SJ, Babyn PS (2003) Congenital extrahepatic portosystemic shunts. Pediatr Radiol 33:614–620 11. Pathak A, Agarwal N, Mandliya J et al (2012) Abernethy malformation: a case report. BMC Pediatrics 12:57 12. Phillips E (1969) Embryology, normal anatomy, and anomalies. In: Ferris EJ, Hipona FA, Kahn PC, Phillips E, Shapiro JH (eds)
123
Surg Radiol Anat Venography of the inferior vena cava and its branches. Williams & Wilkins, Baltimore, pp 1–32 13. Turkbey B, Karcaaltincaba M, Demir H et al (2006) Multiple hyperplastic nodules in the liver with congenital absence of portal vein: MRI findings. Pediatr Radiol 36:445–448 14. Wojcicki M, Haagsma EB, Gouw AS et al (2004) Orthotopic liver transplantation for portosystemic encephalopathy in an adult
123
with congenital absence of the portal vein. Liver Transplant 10(9):1203–1207 15. Woodle ES, Thistlethwaite JR, Emond JC et al (1990) Successful hepatic transplantation in congenital absence of recipient portal vein. Surgery 107:475–479
