Duplication of inferior vena cava with an interrupted segment and DVT: A case report

Meenakshi Paramasivan

HOD – Department of Radiology, Kauvery Hospital, Tirunelveli

Case Presentation

49-year-old male came to ER with chief complaints of Leg pain and swelling. No H/o chest pain/abdominal pain/breathlessness. A known diabetic since 1 year on medication. On examination his vitals were stable.

Blood Investigations

Elevated Total Count: 14100, INR: 1.37, RBS: 265, Urea: 43, Creatinine: 1.40, D Dimer >10, CRP: 32, ESR: 18, APPT: 52.

Other Investigations:

  • B2 macroglobulin: 3120, ANA Profile done.
  • Homocysteine: 23.3 – Hyperhomocysteinemia

Radiological Investigations

  • Chest X-ray: Normal, Bilateral Lower Limb
  • Arterial Doppler: Normal. Bilateral Lower Limb
  • Venous Doppler: Acute Deep Venous Thrombosis of Bilateral Lower Limb Veins extending into IVC.
  • HRCT Chest: Grossly Normal. Bilateral basal atelectasis & Small bronchocoele in right lung upper lobe.
  • CT Pulmonary Angio and CECT of Abdomen were done. The representative images and descriptions are given below in pulmonary angio, arterial, venous and venous phases.

CT Lower Limb Venogram

No evidence of pulmonary thrombo-embolism. Features of deep venous thrombosis involving bilateral proximal anterior, posterior tibial veins, popliteal, superficial femoral, common femoral, external iliac, common iliac veins and left internal iliac vein noted.

Double inferior vena cava (IVC) extending just proximal to common iliac venous confluence. Patent left IVC is seen to drain into azygos and then into superior vena cava. Reformed right IVC seen to drain into right atrium.

Long segment thrombosis of right inferior vena cava noted extending from common iliac venous confluence involving infrarenal and renal segments.

Thinning of IVC in the suprarenal segment and non-opacification with associated thinning of hepatic veins. Suprahepatic segment of IVC reformed by hepatic veins which is seen to drain into right atrium. Left renal vein draining into left SVC, thin right renal vein seen to drain into thrombosed right IVC.

Schematic representation of DIVC

Discussion

Aim of Presentation

Complex presentation of congenital anomalous IVC in this case with many findings in one single patient is the reason why the case is on article.

Duplication of Inferior Vena Cava with Azygos Continuation and DVT

  • Congenital Anomaly: Duplication of IVC
  • Anatomical Variant: Interrupted suprarenal segment of Right IVC.
  • Acquired Pathology: Bilateral Ilio-Femoral Thrombosis with Thrombosed Infrarenal & Renal Right IVC.
  • Associated Anomaly: Multicystic right kidney.

Duplicated IVC with azygos continuation, another name of duplicated ivc with interrupted segment.

Case of Duplication of Inferior Vena Cava with an Interrupted Segment and DVT

Congenital anomaly with an anatomical variant an acquired pathology & an associated anomaly.

Embryology

The inferior vena cava has a convoluted development during the 7-10th weeks of gestation. Posterior cardinal vein appears first but forms only the distal IVC i.e. iliac bifurcation. Subcardinal veins appear next, left subcardinal vein regresses and right subcardinal vein forms the suprarenal IVC. Supracardinal veins appear last, left supracardinal vein regresses, and right supracardinal vein forms infrarenal IVC.

Etiology

IVC duplication results from persistent left supracardinal vein. Regression of right subcardinal vein and persistence of left subcardinal vein: Cause of Interrupted suprarenal segment in right IVC.

Incidence

The incidence of inferior vena cava duplication ~1.5% (range 0.2-3%)

Associations

Horseshoe kidney, circumaortic renal collar, crossed fused ectopia, retroaortic left renal vein, cloacal exstrophy, ureteropelvic junction anomaly and retrocaval ureters.

Types of IVC

Implications of DIVC

Increased risk of venous thromboembolism (VTE), complications during surgery, potential misdiagnosis.

Venous Thromboembolism (VTE)

Duplication can lead to venous stasis and increased pressure in lower extremity veins, potentially increasing the risk of deep vein thrombosis (DVT) and pulmonary embolism.

Surgical Complications

During procedures involving the abdomen or retroperitoneum (e.g., nephrectomy, adrenalectomy), identifying the correct vessels can be challenging due to the presence of the duplicated IVC, potentially leading to accidental damage or hemorrhage.

Nutcracker Syndrome

In rare cases, a duplicated IVC can coexist with nutcracker syndrome, where the left renal vein is compressed, leading to haematuria, abdominal pain, and other symptoms.

Nut Cracker Syndrome

Misdiagnosis

  • A duplicated IVC can be mistaken for other conditions, such as mediastinal massesor aortolumbar lymphadenopathy, particularly in patients with certain types of tumors.
  • Impaired Venous Return: In some cases, duplication can lead to impaired venous return, especially if it’s associated with other congenital anomalies or if one of the IVC vessels is obstructed.
  • IVC Filter Implantation: In patients with VTE requiring IVC filter implantation, the presence of a duplicated IVC can affect filter placement and potential complications.

Aim of the article

  • Early Detection: Awareness of this anomaly can help prevent complications during surgery and guide appropriate management of VTE.
  • Detailed Imaging: Careful review of imaging studies can help identify the presence of a duplicated IVC and its relationship with other structures.
  • Individualized Management: Treatment strategies for VTE and surgical procedures should be tailored to the individual patient’s anatomy and specific circumstances.

Conclusion

We do encounter anomalies in adulthood in many. This patient however carries a congenital anomaly with an anatomical variant, an acquired pathology and an associated anomaly – Just reminds us the age old medical school teaching that “Common things are always common; However, uncommon things are not always uncommon !!”

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