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The value of ultrasonography in interrupted inferior vena cava with azygos continuation
European Journal of Ultrasound 14 (2001) 179– 182 www.elsevier.com/locate/ejultrasou
Case Report
The value of ultrasonography in interrupted inferior vena cava with azygos continuation Ismail Mihmanli a,*, Nail Bulakbasi b, Fatih Kantarci a, Ibrahim Adaletli a, Yuksel Pabuscu b a
Department of Radiology, Cerrahpasa Medical Faculty, Istanbul Uni6ersity, 34300 I: stanbul, Turkey b Department of Radiology, Gu¨lhane Military School of Medicine, Ankara, Turkey Received 21 May 2001; received in revised form 30 July 2001; accepted 15 August 2001
Abstract The cardiosplenic syndromes represent a complex set of abnormalities of the thorax and viscera resulting from abnormal folding of the embryo during early development. Anomalies of venous development include interruption of inferior vena cava (IVC), and in combination with situs inversus referred to as the polysplenia syndrome. We present a 23-year-old male previously diagnosed as having a persistent left IVC on venography whereas ultrasonographic examination clearly demonstrated interrupted IVC with azygos continuation along with situs inversus. © 2001 Elsevier Science Ireland Ltd. All rights reserved. Keywords: Agenesis; Inferior vena cava; Ultrasonography; Situs inversus
1. Case report A 23-year-old male presented with recurrent thrombophlebitis of the right lower extremity. Pelvic venography in addition to bilateral lower extremity venographic examination showed that the common iliac veins were draining to a left paravertebrally located venous structure (Fig. 1), thought to be a persistent left inferior vena cava (IVC). One year after the venographic examina* Corresponding author. Tel.: + 90-212-586-1586; fax: + 90-212-632-0044. E-mail address: [email protected] (I. Mihmanli).
tion the patient presented with abdominal discomfort and abdominal ultrasonography (US) showed absence of the hepatic and infrahepatic segments of the IVC. The hepatic veins were draining directly into the right atrium (Fig. 2). In the left paravertebral location, a large vascular structure draining both common iliac veins could be followed up to the diaphragm and was diagnosed as the azygos vein. Both renal veins were draining into this large vascular structure. Echocardiographic examination demonstrated agenesis of the IVC and a dilated azygos vein, which was draining into the superior vena cava, and prolapse of the mitral and tricuspid valves. In addition to
0929-8266/01/$ - see front matter © 2001 Elsevier Science Ireland Ltd. All rights reserved. PII: S 0 9 2 9 - 8 2 6 6 ( 0 1 ) 0 0 1 6 0 - 4
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I. Mihmanli et al. / European Journal of Ultrasound 14 (2001) 179–182
these findings, US demonstrated situs inversus and multiple small rounded hypoechoic structures consistent with splenic tissues on the right side. These findings were confirmed on abdominopelvic Computed Tomography (CT). CT also showed clearly the retrocrurally located dilated azygos vein and multiple splenic tissues (Fig. 3). Routine clinical examination was normal, and posteroanterior chest X-ray revealed dextrocardia.
2. Discussion
Fig. 1. Conventional venography. Bilateral common iliac veins are draining to a venous structure situated at the left paravertebral location.
Abdominal variations of the IVC or agenesis of one or multiple segments and drainage of the associated veins to the azygos vein is extremely rare (Meyer et al., 1998; Dietz and Reinheimer, 1991). IVC agenesis associated with mega azygos can be diagnosed prenatally by US and is known as the ‘double vessel’ sign. In these cases, polysplenia, rarely asplenia, situs inversus and cardiac anomalies may be associated. Therefore, cardiosplenic syndrome is an accepted terminology for this anomaly (Sheley et al., 1995). Polysplenia syndrome is associated with abnormalities in the visceral lateralization during the embryologic period. It is characterized by the presence of accessory splenic tissue in the location of spleen. The splenic tissues can be well documented on both US and CT examinations, as was
Fig. 2. Ultrasonographic image at level of hepatic veins. The hepatic veins are forming a confluence and draining directly into the right atrium.
I. Mihmanli et al. / European Journal of Ultrasound 14 (2001) 179–182
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done in our case. This malformation is commonly seen along with IVC agenesis, a preduodenal portal vein, intestinal malrotation, aberrant hepatic artery, and situs inversus (Gandin et al., 1993; Falchetti et al., 1991). US demonstrated interrupted IVC with azygos continuation and, situs inversus well in our case. Sometimes Kartagener’s syndrome may accompany this syndrome (Tabeta and Hiroshima, 1991). Cardiac defects are generally seen in situs anomalies associated with asplenia, whereas vascular malformations are seen in situs anomalies associated with polysplenia (Ruscazio et al., 1998; Van Praagh et al., 1992). In our case, although there were situs anomalies associated with polysplenia, mitral and tricuspid valve prolapse was also seen besides vascular malformations such as IVC agenesis. In cases of liver transplantation, because of the surgical difficulties encountered in vascular malformations, the anatomy must be clearly demonstrated (Vazguez et al., 1995). The agenetic conditions of the IVC should also taken into consideration when interventional procedures are planned, such as IVC filter placement (Dietz and Reinheimer, 1991). The differentiation between persistent left IVC and interrupted IVC with azygos continuation may be impossible on venographic examination, especially in cases with situs inversus. In such cases sectional radiologic modalities such as US or CT should be used to make the correct diagnosis. US can elegantly demonstrate the agenetic segment of the IVC, hepatic veins draining into the right atrium, and retrocrurally lying mega azygos, excluding the diagnosis of persistent left IVC. In conclusion, cases diagnosed as persistent left IVC on venographic examination should at least have abdominal US performed, in order to differentiate persistent left IVC from interrupted IVC with azygos continuation.
Fig. 3
Fig. 3. (A) Computed tomographic image at the level of upper abdomen shows situs inversus, polysplenia, and the absent inferior vena cava. Dilated azygos vein located retrocrurally is also seen. AA, abdominal aorta; AZ, azygos vein; D, splenic tissues. (B and C) Two consecutive computed tomographic images at the level of upper thorax depict the dilated azygos vein draining into the superior vena cava. a, azygos vein; s, superior vena cava; j, junction.
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References Dietz R, Reinheimer G. CT diagnosis of the inferior vena cava anomalies. The azygos continuation. Radiologe 1991;31:352 –4. Falchetti D, de Carvallo FB, Clapuyt P, et al. Liver transplantation in children with biliary atresia and polysplenia syndrome. J Pediatr Surg 1991;26:528 –31. Gandin F, Bazzocchi M, Raodini M, Degano G. The echographic and computed tomographic diagnosis of polysplenia. Radiol Med Torino 1993;85:75 –8. Meyer DR, Huppe T, Andresen R, Friedrich M. Intra and infra hepatic agenesis of the inferior vena cava with azygos continuation accompanied by duplication of the postrenal segment. Invest Radiol 1998;33:113 –6. Sheley RC, Nyberg DA, Kapur R. Azygos continuation of the interrupted inferior vena cava: a clue to prenatal diagnosis
of the cardiosplenic syndromes. J Ultrasound Med 1995;14:381 – 7. Ruscazio M, Van Praagh S, Marras Ar, Catani G, Iliceto S, Van Praagh R. Interrupted inferior vena cava in asplenia syndrome and a review of the hereditary patterns of visceral situs abnormalities. Am J Cardiol 1998;81:111 – 6. Tabeta H, Hiroshima K. A case of Kartagener’s syndrome with bilateral superior vena cava and absent inferior vena cava. Nippon Kyobu Shikkan Gakkai Zasshi 1991;29:507 – 11. Van Praagh S, Santini F, Sanders SP. Cardiac malpositions with special emphasis on visceral heterotaxy (asplenia and polysplenia syndromes). In: Fyler DC, editor. Nadas’ Pediatric Cardiology. Philadelphia: Hanley and Belfus, 1992:589 – 608. Vazguez J, Lopez Gutierrez JC, Gamez M, et al. Biliary atresia and polysplenia syndrome: its impact on final outcome. Pediatr Surg 1995;30:485 – 7.
Case Report
The value of ultrasonography in interrupted inferior vena cava with azygos continuation Ismail Mihmanli a,*, Nail Bulakbasi b, Fatih Kantarci a, Ibrahim Adaletli a, Yuksel Pabuscu b a
Department of Radiology, Cerrahpasa Medical Faculty, Istanbul Uni6ersity, 34300 I: stanbul, Turkey b Department of Radiology, Gu¨lhane Military School of Medicine, Ankara, Turkey Received 21 May 2001; received in revised form 30 July 2001; accepted 15 August 2001
Abstract The cardiosplenic syndromes represent a complex set of abnormalities of the thorax and viscera resulting from abnormal folding of the embryo during early development. Anomalies of venous development include interruption of inferior vena cava (IVC), and in combination with situs inversus referred to as the polysplenia syndrome. We present a 23-year-old male previously diagnosed as having a persistent left IVC on venography whereas ultrasonographic examination clearly demonstrated interrupted IVC with azygos continuation along with situs inversus. © 2001 Elsevier Science Ireland Ltd. All rights reserved. Keywords: Agenesis; Inferior vena cava; Ultrasonography; Situs inversus
1. Case report A 23-year-old male presented with recurrent thrombophlebitis of the right lower extremity. Pelvic venography in addition to bilateral lower extremity venographic examination showed that the common iliac veins were draining to a left paravertebrally located venous structure (Fig. 1), thought to be a persistent left inferior vena cava (IVC). One year after the venographic examina* Corresponding author. Tel.: + 90-212-586-1586; fax: + 90-212-632-0044. E-mail address: [email protected] (I. Mihmanli).
tion the patient presented with abdominal discomfort and abdominal ultrasonography (US) showed absence of the hepatic and infrahepatic segments of the IVC. The hepatic veins were draining directly into the right atrium (Fig. 2). In the left paravertebral location, a large vascular structure draining both common iliac veins could be followed up to the diaphragm and was diagnosed as the azygos vein. Both renal veins were draining into this large vascular structure. Echocardiographic examination demonstrated agenesis of the IVC and a dilated azygos vein, which was draining into the superior vena cava, and prolapse of the mitral and tricuspid valves. In addition to
0929-8266/01/$ - see front matter © 2001 Elsevier Science Ireland Ltd. All rights reserved. PII: S 0 9 2 9 - 8 2 6 6 ( 0 1 ) 0 0 1 6 0 - 4
180
I. Mihmanli et al. / European Journal of Ultrasound 14 (2001) 179–182
these findings, US demonstrated situs inversus and multiple small rounded hypoechoic structures consistent with splenic tissues on the right side. These findings were confirmed on abdominopelvic Computed Tomography (CT). CT also showed clearly the retrocrurally located dilated azygos vein and multiple splenic tissues (Fig. 3). Routine clinical examination was normal, and posteroanterior chest X-ray revealed dextrocardia.
2. Discussion
Fig. 1. Conventional venography. Bilateral common iliac veins are draining to a venous structure situated at the left paravertebral location.
Abdominal variations of the IVC or agenesis of one or multiple segments and drainage of the associated veins to the azygos vein is extremely rare (Meyer et al., 1998; Dietz and Reinheimer, 1991). IVC agenesis associated with mega azygos can be diagnosed prenatally by US and is known as the ‘double vessel’ sign. In these cases, polysplenia, rarely asplenia, situs inversus and cardiac anomalies may be associated. Therefore, cardiosplenic syndrome is an accepted terminology for this anomaly (Sheley et al., 1995). Polysplenia syndrome is associated with abnormalities in the visceral lateralization during the embryologic period. It is characterized by the presence of accessory splenic tissue in the location of spleen. The splenic tissues can be well documented on both US and CT examinations, as was
Fig. 2. Ultrasonographic image at level of hepatic veins. The hepatic veins are forming a confluence and draining directly into the right atrium.
I. Mihmanli et al. / European Journal of Ultrasound 14 (2001) 179–182
181
done in our case. This malformation is commonly seen along with IVC agenesis, a preduodenal portal vein, intestinal malrotation, aberrant hepatic artery, and situs inversus (Gandin et al., 1993; Falchetti et al., 1991). US demonstrated interrupted IVC with azygos continuation and, situs inversus well in our case. Sometimes Kartagener’s syndrome may accompany this syndrome (Tabeta and Hiroshima, 1991). Cardiac defects are generally seen in situs anomalies associated with asplenia, whereas vascular malformations are seen in situs anomalies associated with polysplenia (Ruscazio et al., 1998; Van Praagh et al., 1992). In our case, although there were situs anomalies associated with polysplenia, mitral and tricuspid valve prolapse was also seen besides vascular malformations such as IVC agenesis. In cases of liver transplantation, because of the surgical difficulties encountered in vascular malformations, the anatomy must be clearly demonstrated (Vazguez et al., 1995). The agenetic conditions of the IVC should also taken into consideration when interventional procedures are planned, such as IVC filter placement (Dietz and Reinheimer, 1991). The differentiation between persistent left IVC and interrupted IVC with azygos continuation may be impossible on venographic examination, especially in cases with situs inversus. In such cases sectional radiologic modalities such as US or CT should be used to make the correct diagnosis. US can elegantly demonstrate the agenetic segment of the IVC, hepatic veins draining into the right atrium, and retrocrurally lying mega azygos, excluding the diagnosis of persistent left IVC. In conclusion, cases diagnosed as persistent left IVC on venographic examination should at least have abdominal US performed, in order to differentiate persistent left IVC from interrupted IVC with azygos continuation.
Fig. 3
Fig. 3. (A) Computed tomographic image at the level of upper abdomen shows situs inversus, polysplenia, and the absent inferior vena cava. Dilated azygos vein located retrocrurally is also seen. AA, abdominal aorta; AZ, azygos vein; D, splenic tissues. (B and C) Two consecutive computed tomographic images at the level of upper thorax depict the dilated azygos vein draining into the superior vena cava. a, azygos vein; s, superior vena cava; j, junction.
182
I. Mihmanli et al. / European Journal of Ultrasound 14 (2001) 179–182
References Dietz R, Reinheimer G. CT diagnosis of the inferior vena cava anomalies. The azygos continuation. Radiologe 1991;31:352 –4. Falchetti D, de Carvallo FB, Clapuyt P, et al. Liver transplantation in children with biliary atresia and polysplenia syndrome. J Pediatr Surg 1991;26:528 –31. Gandin F, Bazzocchi M, Raodini M, Degano G. The echographic and computed tomographic diagnosis of polysplenia. Radiol Med Torino 1993;85:75 –8. Meyer DR, Huppe T, Andresen R, Friedrich M. Intra and infra hepatic agenesis of the inferior vena cava with azygos continuation accompanied by duplication of the postrenal segment. Invest Radiol 1998;33:113 –6. Sheley RC, Nyberg DA, Kapur R. Azygos continuation of the interrupted inferior vena cava: a clue to prenatal diagnosis
of the cardiosplenic syndromes. J Ultrasound Med 1995;14:381 – 7. Ruscazio M, Van Praagh S, Marras Ar, Catani G, Iliceto S, Van Praagh R. Interrupted inferior vena cava in asplenia syndrome and a review of the hereditary patterns of visceral situs abnormalities. Am J Cardiol 1998;81:111 – 6. Tabeta H, Hiroshima K. A case of Kartagener’s syndrome with bilateral superior vena cava and absent inferior vena cava. Nippon Kyobu Shikkan Gakkai Zasshi 1991;29:507 – 11. Van Praagh S, Santini F, Sanders SP. Cardiac malpositions with special emphasis on visceral heterotaxy (asplenia and polysplenia syndromes). In: Fyler DC, editor. Nadas’ Pediatric Cardiology. Philadelphia: Hanley and Belfus, 1992:589 – 608. Vazguez J, Lopez Gutierrez JC, Gamez M, et al. Biliary atresia and polysplenia syndrome: its impact on final outcome. Pediatr Surg 1995;30:485 – 7.