CASE REPORT Year : 2022  |  Volume : 9  |  Issue : 5  |  Page : 401-403

A case of duplication of inferior vena cava and turner syndrome: Is it a rare association?

S BS Netam, Vishal Jain, Shubhkriti Agrawal, M Rashika
Department of Radiodiagnosis, Pt. JNMC and BRAMH, Raipur, Chhattisgarh, India

Date of Submission19-Oct-2022Date of Acceptance02-Nov-2022Date of Web Publication13-Jan-2023

Correspondence Address:
Dr. M Rashika
Department of Radiodiagnosis, Pt. JNMC and BRAMH, Raipur, Chhattisgarh
India

Source of Support: None, Conflict of Interest: None

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DOI: 10.4103/ijves.ijves_94_22

Turner syndrome is one of the most common sex chromosome disorders with many anatomical abnormalities that affect physiological systems of human body. The most common cardiovascular anomalies in Turner syndrome are bicuspid aortic valve and coarctation of aorta. The venous anomalies include partial anomalous pulmonary venous return and persistent left sided Superior vena cava. Duplication of inferior vena cava (IVC) is rare in occurrence. On extensive literature search, no such case of IVC duplication in Turner syndrome is found. We, herein, report an incidental finding of IVC duplication in a known case of Turner syndrome. Any patient with Turner syndrome should be evaluated for additional venous anomalies.

Keywords: Computed tomography, duplication of inferior vena cava, incidental finding, turner syndrome, venous anomaly

How to cite this article:
Netam S B, Jain V, Agrawal S, Rashika M. A case of duplication of inferior vena cava and turner syndrome: Is it a rare association?. Indian J Vasc Endovasc Surg 2022;9:401-3
How to cite this URL:
Netam S B, Jain V, Agrawal S, Rashika M. A case of duplication of inferior vena cava and turner syndrome: Is it a rare association?. Indian J Vasc Endovasc Surg [serial online] 2022 [cited 2023 Jan 14];9:401-3. Available from: https://www.indjvascsurg.org/text.asp?2022/9/5/401/367731   Introduction 

Turner syndrome occurs in about one in every 2500 newborn girls and is one of the most common sex chromosome abnormalities. The 45 XO karyotype accounts for about 40%–60% and is the most common type.[1]

The venous anomalies documented in Turner syndrome as far are partial anomalous pulmonary venous return (PAPVR) (13%) and persistent left-sided SVC (5%–13%).[2]

Inferior vena cava (IVC) duplication is a rare congenital anomaly with 1.5% prevalence which can remain undetected throughout life.[3] No significant correlation has been established yet between them. In this case, we found an incidental finding of IVC duplication in a patient of Turner syndrome.

  Case Report 

A 20-year-old female, a known case of Turner syndrome, came to the Department of Radiodiagnosis of Dr. B. R. A. M. Hospital, Raipur, for follow-up who is a diagnosed case of preductal coarctation of the aorta. She also had a history of recurrent otitis media and recurrent abdominal pain along with primary amenorrhea.

On karyotyping study, her chromosomal structure was XO.

On physical examination, the patient had short stature, webbed neck, kyphotic deformity, and pigeon-shaped chest.

On radiological investigation (contrast-enhanced computed tomography [CT] thorax), preductal coarctation of the aorta, right-sided cervical rib, vertebral osteochondrosis with kyphotic deformity, and pectus carinatum were noted [Figure 1]a, [Figure 1]b, [Figure 1]c.

Figure 1: (a) VRT image in sagittal plane showing preductal coarctation of aorta, (b) 3DCT reconstructed images showing right-sided cervical rib, (c) CT sagittal view showing pectus carinatum and Scheuermann disease (undulating surfaces, loss of disc height, and wedging of anterior vertebral body) leading to thoracic kyphosis. CT: Computed tomography, 3DCT: 3-Dimensional CT, VRT: Volume rendering technique

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On contrast-enhanced CT abdomen, infantile uterus with nonvisualization of the bilateral ovaries was noted [Figure 2].

Figure 2: CECT in sagittal view showing infantile uterus. CECT: Contrast-enhanced computed tomography

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An incidental finding, the left common iliac vein continues upward along the abdominal aorta as left IVC and terminates into the left renal vein at L2 vertebral level. There is an interiliac communication arising from the left common iliac vein draining into the right IVC at L5 vertebral level. Above features suggestive of duplication of IVC type 2b (According to Morita classification)[4][Figure 3]a and [Figure 3]b.

Figure 3: (a) VRT images and CECT on axial sections showing of Inferior vena cava IVC duplication at three levels (white line). Top to bottom, at the level of termination of left renal vein, at L3 vertebral level and at the level of interiliac communication, (b) CECT on coronal view showing inferior vena cava IVC duplication. CECT: Contrast-enhanced computed tomography, IVC: Inferior vena cava, VRT: Volume rendering technique

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  Discussion 

Turner syndrome usually presents with many associations such as cardiovascular, genitourinary, neurological, skeletal, otolaryngological, and craniofacial abnormalities that affect many physiological systems and are responsible for the reduced life expectancy of these patients.

The most common vascular anomalies in Turner syndrome are bicuspid aortic valve and coarctation of the aorta. However, additional arterial anomalies such as aberrant right subclavian artery, elongation of transverse aortic arch, and bovine aortic arch configuration are commonly seen. Venous anomalies such as PAPVR and persistent left-sided SVC can also be seen.

The development of IVC is a complex embryogenic process. It begins at 6th week of gestation. IVC is formed by pairs of subcardinal, supracardinal, and posterior cardinal veins along with right vitelline vein. This is the primary venous arrangement in the developing fetus. As the development proceeds, first, the left posterior cardinal vein disappears followed by subcardinal veins on each side except for a part of the subcardinal vein extending between right posterior cardinal vein and subcardinal–supracardinal anastomosis. With further development, the rest of the veins disappear and the final IVC is formed.[5] The four segments of IVC are formed as follows: hepatic segment by hepatocardiac channels from the right vitelline vein, suprarenal segment by the right subcardinal vein, renal segment by subcardinal–supracardinal anastomosis, and infrarenal segment by the right supracardinal vein [Figure 4]a.

Figure 4: (a) Schematic diagram showing Inferior vena cava IVC development 1: Hepatocardiac channel from right vitelline vein, 2: Supracardianal vein, 3: Subcardinal vein, 4: Posterior cardinal vein, 5: Supra-subcardinal anastomosis, 6: Intersubcardinal anastomoses, 7: Interposterior cardinal anastomosis, (b) Schematic diagram showing Inferior vena cava IVC duplication. IVC: Inferior vena cava

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IVC duplication is due to persistence of caudal portion of the left supracardinal vein. The left IVC typically ends at the left renal vein, which crosses anterior to the aorta to form a normal right sided prerenal IVC[5],[6][Figure 4]b.

The detection of this vascular anomaly holds significance when operating on the retroperitoneum. It is common to confuse the left infrarenal IVC with aortolumbar lymphadenopathy, left pyeloureteric dilatation, saccular aortic aneurysm, retroperitoneal cyst, or loops of small bowel. The duplicated IVC can lead to both misdiagnosis and life-threatening surgical complications in retroperitoneal and aortic surgeries. It may also increase the risk for development of deep vein thrombosis, especially in young adults due to slow flow, inadequate venous return, and increased venous pressure in the veins of both the lower limbs.[6]

Duplication of IVC usually occurs as a single vascular anomaly or associated with genitourinary and gastrointestinal anomalies. However, in this case, it is associated with Turner syndrome as explained below. The following are the case review of few associations with duplication of IVC[7],[8],[9],[10],[11],[12],[13],[14][Table 1].

  Conclusion 

There are multiple skeletal, cardiovascular, gonadal, and lymphatic anomalies noted associated with Turner syndrome. Duplication of IVC is rare in occurrence, not yet reported in a case of Turner syndrome. It can be an incidental finding or rare association but has increased risk of vascular injury and pulmonary thromboembolism. Hence, additional evaluation of venous anomalies in patients with Turner syndrome should be done during workup.

  Teaching Point 

Any case of Turner syndrome should also be evaluated for additional venous anomalies to avoid dreadful intraoperative complications and development of deep venous thrombosis.

Declaration of patient consent

Written informed consent is obtained from the patient for publishing her clinical information and radiographic images as a case report in this journal. She understands that her name and initial will not be published and due efforts will be made to conceal her identity

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 

  References 
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    8.Mani N, Venkataramu NK, Singh P, Suri S. Duplication of IVC and associated renal anomalies. Indian Journal of Radiology and Imaging 2000;10:157-58.  
    9.Kaur N, Saha S, Mriglani R, Saini P, Gupta A. Crosed Fused Renal Ectopia with a Single Ureter: A Rare Anomaly. Saudi Journal of Kidney Diseases and Transplantation 2013;24:773-76.  
    10.Dudekula A, Prabhu SD. A Rare Case of Right Retrocaval Ureter with Duplication of Infrarenal IVC. Case Reports in Radiology Hindawi Publishing Corporation 2014;2014:1-4.  
    11.Shaha P, Garg A, Sahoo K, Kothari N, Garg P. Duplication of Inferior vena cava with associated anomalies: A rare case report. J Clin Diagn Res 2016;10:TD01-4.  
    12.Vurucu S, Battal B, Kocaoglu M, Akin R. Klippel–Trenaunay syndrome with hemimegalencephaly, retroperitoneal lymphangioma and double inferior vena cava. Br J Radiol 2009;82:e10-e104.  
    13.Moosavi J, Sadeghipour P, Mohebbi B, Kalantari RK, Khalilipur E. A rare case of left inferior vena cava presenting with May-Thurner syndrome. CVIR Endovascular 2022;31:1-5.  
    14.Yoshida Rde A, Yoshida WB, Costa RF, Nacif MS, Sobreira ML, Jaldin RG. Nutcracker syndrome and deep venous thrombosis in a patient with duplicated inferior vena cava. J Vasc Surg Venous Lymphat Disord 2016;4:231-5.  
    
  [Figure 1], [Figure 2], [Figure 3], [Figure 4]
 
 
  [Table 1]