Azygos continuation of the IVC
Diagnosis certain
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The reported incidence of this anomalyis estimated to be 0.6-2% of patients with congenital heart diseases and less than 0.3% in otherwise normal individuals 1 2 3-3.
This anomaly results from failure of right sub-cardinalsubcardinal-hepatic anastomosis with atrophy of right sub-cardinalsubcardinal vein and shunting of blood from supra-cardinal-sub-cardinalsupracardinal-subcardinal anastomosis to cranial portion of supra-cardinalsupracardinal vein (retro-crural(retrocrural azygos vein) 2.
Imaging findings include: hepatic veins drain into right atrium. Renal veins drain into azygousazygos (or hemi-azygoushemiazygos). This anomaly usually occurs with a right infra-renal IVC with azygos/hemi-azygos/hemiazygos continuation (4)4, but it may also occur with other two variants:
(1) Left infra-renal
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left infrarenal IVC with azygos
/hemi-azygos/hemiazygos continuation 5 (as in this case).(2) Double infra-renal -
double infrarenal IVCs with two possibilities:
EITHERORhemi-azygoshemiazygos vein) 7.
The clinical importance of this anomaly is inthe risk of misinterpretation of it as a right para-trachealparatracheal mass or retro-cruralretrocrural adenopathy, in being associated with heterotaxy syndromes and other congenital heart diseases and in pre-operativepreoperative knowledge of the anatomy prior to planning cardio-pulmonarycardiopulmonary bypass and to avoid difficulties in catheterizingcatheterising the heart 8 9,9.
-<p>The reported incidence of this <span style="line-height:20.8px">anomaly</span><span style="line-height:20.8px"> </span>is estimated to be 0.6-2 % of patients with congenital heart diseases and less than 0.3 % in otherwise normal individuals <sup>1 2 3</sup>.</p><p>This anomaly results from failure of right sub-cardinal-hepatic anastomosis with atrophy of right sub-cardinal vein and shunting of blood from supra-cardinal-sub-cardinal anastomosis to cranial portion of supra-cardinal vein (retro-crural azygos vein) <sup>2</sup>.</p><p>Imaging findings include: hepatic veins drain into right atrium. Renal veins drain into azygous (or hemi-azygous). This anomaly usually occurs with a right infra-renal IVC with azygos/hemi-azygos continuation <sup>(4)</sup>, but it may also occur with other two variants:</p><p>(<strong>1</strong>) Left infra-renal IVC with azygos/hemi-azygos continuation <sup>5 </sup>(as in this case).<sup> </sup> </p><p>(<strong>2</strong>) Double infra-renal IVCs with two possibilities: <strong><em>EITHER</em></strong> both IVCs join at the renal level and continue as azygos vein) <sup>6</sup> <strong><em>OR</em></strong> (the right IVC continues as the azygos vein and the left one continues as the hemi-azygos vein) <sup>7</sup>.</p><p>The clinical importance of this anomaly is in misinterpretation as right para-tracheal mass or retro-crural adenopathy, in being associated with heterotaxy syndromes and other congenital heart diseases and in pre-operative knowledge of the anatomy prior to planning cardio-pulmonary bypass and to avoid difficulties in catheterizing the heart <sup>8 9</sup>.</p>- +<p>The reported incidence of this anomaly is estimated to be 0.6-2% of patients with congenital heart diseases and less than 0.3% in otherwise normal individuals <sup>1-3</sup>.</p><p>This anomaly results from failure of right subcardinal-hepatic anastomosis with atrophy of right subcardinal vein and shunting of blood from supracardinal-subcardinal anastomosis to cranial portion of supracardinal vein (retrocrural azygos vein) <sup>2</sup>.</p><p>Imaging findings include: hepatic veins drain into right atrium. Renal veins drain into azygos (or hemiazygos). This anomaly usually occurs with a right infra-renal IVC with azygos/hemiazygos continuation <sup>4</sup>, but it may also occur with other two variants:</p><ol>
- +<li>left infrarenal IVC with azygos/hemiazygos continuation <sup>5 </sup>(as in this case).</li>
- +<li>double infrarenal IVCs with two possibilities: either both IVCs join at the renal level and continue as the azygos vein) <sup>6</sup> or (the right IVC continues as the azygos vein and the left one continues as the hemiazygos vein) <sup>7</sup>. </li>
- +</ol><p>The clinical importance of this anomaly is the risk of misinterpretation of it as a right paratracheal mass or retrocrural adenopathy, in being associated with heterotaxy syndromes and other congenital heart diseases and in preoperative knowledge of the anatomy prior to planning cardiopulmonary bypass and to avoid difficulties in catheterising the heart <sup>8,9</sup>.</p>
References changed:
- 3. Mehta AJ, Kate AH, Gupta N et-al. Interrupted inferior vena cava syndrome. J Assoc Physicians India. 2013;60: 48-50. <a href="http://www.ncbi.nlm.nih.gov/pubmed/23405525">Pubmed citation</a><span class="auto"></span>.
- 1. Dähnert W. Radiology Review Manual. LWW. (2011) ISBN:1609139437. <a href="http://books.google.com/books?vid=ISBN1609139437">Read it at Google Books</a> - <a href="http://www.amazon.com/gp/product/1609139437">Find it at Amazon</a><span class="auto"></span>.
- 2. Bass JE, Redwine MD, Kramer LA et-al. Spectrum of congenital anomalies of the inferior vena cava: cross-sectional imaging findings. Radiographics. 2000;20 (3): 639-52. <a href="http://dx.doi.org/10.1148/radiographics.20.3.g00ma09639">doi:10.1148/radiographics.20.3.g00ma09639</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/10835118">Pubmed citation</a><span class="auto"></span>.
- 4. Vijayaraghavan SB, Raja V, Chitra TV. Interrupted inferior vena cava and left-sided subrenal inferior vena cava: prenatal diagnosis. J Ultrasound Med. 2003;22 (7): 747-52. <a href="http://www.ncbi.nlm.nih.gov/pubmed/12862278">Pubmed citation</a><span class="auto"></span>.
- 5. Amato M, Biondetti PR. Left inferior vena cava, with hemiazygos continuation and atypical flow into the superior vena cava. A case report. Radiol Med. 2001;99 (6): 474-5. <a href="http://www.ncbi.nlm.nih.gov/pubmed/11262828">Pubmed citation</a><span class="auto"></span>.
- 6. Esposito S, Mansueto G, Amodio F et-al. Duplication of the vena cava inferior with a continuation into the vena azygos. A report of a rare case. Minerva Chir. 1999;54 (4): 261-5. <a href="http://www.ncbi.nlm.nih.gov/pubmed/10380526">Pubmed citation</a><span class="auto"></span>.
- 7. Mayo J, Gray R, St Louis E et-al. Anomalies of the inferior vena cava. AJR Am J Roentgenol. 1983;140 (2): 339-45. <a href="http://dx.doi.org/10.2214/ajr.140.2.339">doi:10.2214/ajr.140.2.339</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/6336872">Pubmed citation</a><span class="auto"></span>.
- 8. Ginaldi S, Chuang VP, Wallace S. Absence of hepatic segment of the inferior vena cava with azygous continuation. J Comput Assist Tomogr. 1980;4 (1): 112-4. <a href="http://www.ncbi.nlm.nih.gov/pubmed/7354162">Pubmed citation</a><span class="auto"></span>.
- 9. Mazzucco A, Bortolotti U, Stellin G et-al. Anomalies of the systemic venous return: a review. J Card Surg. 1992;5 (2): 122-33. <a href="http://www.ncbi.nlm.nih.gov/pubmed/2133830">Pubmed citation</a><span class="auto"></span>.
- 3- Mehta AJ, Kate AH, Gupta N et-al. Interrupted inferior vena cava syndrome. J Assoc Physicians India. 2013;60: 48-50. <a href="http://www.ncbi.nlm.nih.gov/pubmed/23405525">Pubmed citation</a><span class="auto"></span>.
- 1- Dähnert W. Radiology Review Manual. LWW. (2011) ISBN:1609139437. <a href="http://books.google.com/books?vid=ISBN1609139437">Read it at Google Books</a> - <a href="http://www.amazon.com/gp/product/1609139437">Find it at Amazon</a><span class="auto"></span>.
- 2- Bass JE, Redwine MD, Kramer LA et-al. Spectrum of congenital anomalies of the inferior vena cava: cross-sectional imaging findings. Radiographics. 2000;20 (3): 639-52. <a href="http://dx.doi.org/10.1148/radiographics.20.3.g00ma09639">doi:10.1148/radiographics.20.3.g00ma09639</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/10835118">Pubmed citation</a><span class="auto"></span>.
- 4- Vijayaraghavan SB, Raja V, Chitra TV. Interrupted inferior vena cava and left-sided subrenal inferior vena cava: prenatal diagnosis. J Ultrasound Med. 2003;22 (7): 747-52. <a href="http://www.ncbi.nlm.nih.gov/pubmed/12862278">Pubmed citation</a><span class="auto"></span>.
- 5- Amato M, Biondetti PR. Left inferior vena cava, with hemiazygos continuation and atypical flow into the superior vena cava. A case report. Radiol Med. 2001;99 (6): 474-5. <a href="http://www.ncbi.nlm.nih.gov/pubmed/11262828">Pubmed citation</a><span class="auto"></span>.
- 6- Esposito S, Mansueto G, Amodio F et-al. Duplication of the vena cava inferior with a continuation into the vena azygos. A report of a rare case. Minerva Chir. 1999;54 (4): 261-5. <a href="http://www.ncbi.nlm.nih.gov/pubmed/10380526">Pubmed citation</a><span class="auto"></span>.
- 7- Mayo J, Gray R, St Louis E et-al. Anomalies of the inferior vena cava. AJR Am J Roentgenol. 1983;140 (2): 339-45. <a href="http://dx.doi.org/10.2214/ajr.140.2.339">doi:10.2214/ajr.140.2.339</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/6336872">Pubmed citation</a><span class="auto"></span>.
- 8- Ginaldi S, Chuang VP, Wallace S. Absence of hepatic segment of the inferior vena cava with azygous continuation. J Comput Assist Tomogr. 1980;4 (1): 112-4. <a href="http://www.ncbi.nlm.nih.gov/pubmed/7354162">Pubmed citation</a><span class="auto"></span>.
- 9- Mazzucco A, Bortolotti U, Stellin G et-al. Anomalies of the systemic venous return: a review. J Card Surg. 1992;5 (2): 122-33. <a href="http://www.ncbi.nlm.nih.gov/pubmed/2133830">Pubmed citation</a><span class="auto"></span>.
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Images showed diffuse fatty infiltration of the liver, with no detectable focal lesions.
The infra-renalinfrarenal IVC is seen on the left side. It drains both renal veins and it ascends to join the hemi-azygoushemiazygos vein, which drains into the azygousazygos vein and this ascends in its usual retro-cruralretrocrural course to the chest.
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Imaged showed the infra-renalInfrarenal IVC is seen on the left side (long white arrows). It drains both renal veins (long red arrows) and it ascends to join the hemi-azygoushemiazygos vein (short red arrows), which drains into the azygousazygos vein and this ascends in its usual retro-cruralretrocrural course to the chest (long green arrows).
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