The curious case of the disappearing IVC: a case report and review of the aetiology of inferior vena cava agenesis

Abstract

We report the case of a previously well 18-year-old male who presented to the Emergency Department with lower limb pain. An ultrasound demonstrated extensive left sided deep vein thrombosis and computed tomography demonstrated inferior vena cava agenesis, leading to the diagnosis of inferior vena cava agenesis associated deep vein thrombosis. The aetiology of inferior vena cava agenesis is explored in depth.

Keywords: IVC; Inferior vena cava agenesis; deep vein thrombosis; inferior vena cava agenesis associated deep vein thrombosis; inferior vena cava anomalies; venous thromboembolism.

Figure 1

18-year-old male with extensive DVT and infrarenal IVC agenesis. FINDINGS: Sagittal ultrasound image shows thrombus in the left common iliac vein (green arrow) with good blood flow in the artery overlying. TECHNIQUE: Ultrasonography was performed using a Philips IU22 (Philips Healthcare UK) ultrasound machine with linear array and curvilinear 5 - 2 megahertz (MHz) probes.

Figure 2

18-year-old male with extensive DVT and infrarenal IVC agenesis. FINDINGS: Normal posterior-anterior chest radiograph appearances.

Figure 3

18-year-old male with extensive DVT and infrarenal IVC agenesis. FINDINGS: Venous phase axial (a) and coronal (b) enhanced computed tomography of the abdomen demonstrating the small right sided suprarenal IVC cava (white arrows; solid white arrow in magnification (b)). TECHNIQUE: The patient was imaged on a Siemens Sensation 16 CT Scanner (Siemens Medical, Forchheim Germany) scanned helically with axial reconstruction at 1.5mms, matrix size 512×512, with 120kVp and modulated mAs varying from 70 to 205. An initial scan was acquired after administration of non ionic Iopamidol (Niopam 300mg Iodine/ml Bracco UK Limited) IV Contrast triggered from the aorta with 25 second delayed imaging performed from pelvis to lung apices with a second study of the pelvis visually triggered over the IVC as initial opacification of pelvic veins was poor. A pump injection was used with a speed of 4 mls/sec. The legs were not imaged as ultrasound had provided diagnostic information regarding the presence of thrombus on the left side.

Figure 4

18-year-old male with extensive DVT and infrarenal IVC agenesis. FINDINGS: Coronal views of the abdomen at different levels (a posterior to b) demonstrating a large collateral vessel (white arrows; hollow white arrow in magnification (a)) containing thrombus, draining the right kidney. TECHNIQUE: The patient was imaged on a Siemens Sensation 16 CT Scanner (Siemens Medical, Forchheim Germany) scanned helically with axial reconstruction at 1.5mms, matrix size 512×512, with 120kVp and modulated mAs varying from 70 to 205. An initial scan was acquired after administration of non ionic Iopamidol (Niopam 300mg Iodine/ml Bracco UK Limited) IV Contrast triggered from the aorta with 25 second delayed imaging performed from pelvis to lung apices with a second study of the pelvis visually triggered over the IVC as initial opacification of pelvic veins was poor. A pump injection was used with a speed of 4 mls/sec. The legs were not imaged as ultrasound had provided diagnostic information regarding the presence of thrombus on the left side.

Figure 5

18-year-old male with extensive DVT and infrarenal IVC agenesis. FINDINGS: Computed tomography axial scan (a) of the abdomen showing a lumbar vein (blue arrow in main image (a); solid blue arrow in magnification (a)) forming bulbous left ‘inferior vena cava’ (white arrow in main image; solid white arrow in magnification (a)). Coronal image (b) demonstrating a left gonadal vein (blue arrow in main image (b); solid blue arrow in magnification (b)) with left sided collaterals (orange arrow in main image (b); solid orange arrow in magnification (b)) draining into the bulbous left ‘inferior vena cava’ which crosses the aorta to join the suprarenal portion of the right sided inferior vena cava (white arrow in main image (b); solid white arrow in magnification (b)). TECHNIQUE: The patient was imaged on a Siemens Sensation 16 CT Scanner (Siemens Medical, Forchheim Germany) scanned helically with axial reconstruction at 1.5mms, matrix size 512×512, with 120kVp and modulated mAs varying from 70 to 205. An initial scan was acquired after administration of non ionic Iopamidol (Niopam 300mg Iodine/ml Bracco UK Limited) IV Contrast triggered from the aorta with 25 second delayed imaging performed from pelvis to lung apices with a second study of the pelvis visually triggered over the IVC as initial opacification of pelvic veins was poor. A pump injection was used with a speed of 4 mls/sec. The legs were not imaged as ultrasound had provided diagnostic information regarding the presence of thrombus on the left side.

Figure 6

18-year-old male with extensive DVT and infrarenal IVC agenesis. FINDINGS: Computed tomography axial scan of the abdomen demonstrating the right sided inferior vena cava (white arrow; solid white arrow in magnification) and left sided collaterals containing thrombus (blue arrow; blue solid arrow in magnification). TECHNIQUE: The patient was imaged on a Siemens Sensation 16 CT Scanner (Siemens Medical, Forchheim Germany) scanned helically with axial reconstruction at 1.5mms, matrix size 512×512, with 120kVp and modulated mAs varying from 70 to 205. An initial scan was acquired after administration of non ionic Iopamidol (Niopam 300mg Iodine/ml Bracco UK Limited) IV Contrast triggered from the aorta with 25 second delayed imaging performed from pelvis to lung apices with a second study of the pelvis visually triggered over the IVC as initial opacification of pelvic veins was poor. A pump injection was used with a speed of 4 mls/sec. The legs were not imaged as ultrasound had provided diagnostic information regarding the presence of thrombus on the left side.

Figure 7

18-year-old male with extensive DVT and infrarenal IVC agenesis. FINDINGS: A three-dimensional volumetric reconstruction demonstrating the left sided sacral bony defect (white arrow). TECHNIQUE: The patient was imaged on a Siemens Sensation 16 CT Scanner (Siemens Medical, Forchheim Germany) scanned helically with axial reconstruction at 1.5mms, matrix size 512×512, with 120kVp and modulated mAs varying from 70 to 205. An initial scan was acquired after administration of non ionic Iopamidol (Niopam 300mg Iodine/ml Bracco UK Limited) IV Contrast triggered from the aorta with 25 second delayed imaging performed from pelvis to lung apices with a second study of the pelvis visually triggered over the IVC as initial opacification of pelvic veins was poor. A pump injection was used with a speed of 4 mls/sec. The legs were not imaged as ultrasound had provided diagnostic information regarding the presence of thrombus on the left side.