EJVES Extra 14,8e13 (2007) doi:10.1016/j.ejvsextra.2007.02.007, available online at http://www.sciencedirect.com on SHORT REPORT Congenital Anomalies of the Inferior Vena Cava and their Clinical Manifestation A.A. Baeshko,1* H.V. Zhuk,1 E.A. Ulezko,2 I.V. Goresckaya,2 E.G. Oganova,3 V.S. Dudarev4 and Y.N. Orlovski1 1Belorussian State Medical University, Minsk, Belarus 2State Establishment Republic Scientific-Practical Center ‘‘Mother and Child’’, Minsk, Belarus 39 City Clinical Hospital, Minsk, Belarus 4Scientific-Research Institute of Oncology, Minsk, Belarus Congenital anomalies of IVC are rare. They are seen more often in young males. They are latent for a long time. Peripheral venous thrombosis or CVI are often the first symptoms of a congenital IVC anomaly. We present 5 patients aged 20 to 43 with congenital anomalies of the IVC. The diagnosis and the level of hypoplasia have been determined by compression ultrasonography with color Doppler assessment, spiral computer tomography, pelvic phlebography and retrograde cavag- raphy. In three out of five patients the disease presented as a deep venous thrombosis, in two by temperature rise, chills and subsequent edema of both legs. Two patients had hypoplasia of the infrarenal segment of the IVC. Two others had abnormal of infra-, renal and suprarenal regions of the IVC and one had almost complete vena cava aplasia. In case of DVT or CVI, especially in young males, a potential IVC abnormality should be excluded by ultrasonography of the infra-, renal and suprarenal areas of the IVC. In case of recognized abnormalities a spiral CT scan is indicated. Treatment should comprise vasotonic drugs, elastic compression stockings and use of anticoagulants in cases with peripheral thrombosis. Keywords: Congenital anomaly; Inferior vena cava; Helical computer tomography. Introduction color Doppler assessment (US) and helical computer tomography (HCT).3,4 Hypo- and aplasia of the inferior vena cava is a rela- Differentiating congenital pathology of the IVC tively rare congenital anomaly. Despite being associ- from acquired diseases such as thrombosis or com- ated with deep vein thrombosis (DVT) or chronic pression by a tumor is important in choosing a treat- venous insufficiency (CVI), the correct diagnosis is ment method and further prognosis. often made late during the assessment of patients We aim to present our results of investigation and with suspected peripheral venous thrombosis. It is treatment of patients with congenital IVC anomalies. sometimes suspected on a chest X-ray examination due to an enlargement of mediastinal shadow caused by azygos veindilatation.1,2 Congenital anomalies of the IVC are a problem due Materials and Methods to development of CVI in such patients. The interest shown to it has increased due to improved diagnostic We present 5 cases of patients with congenital IVC methods, such as compression ultrasonography with abnormalities (Table 1). We performed an US of veins of lower extremities, pelvis and retroperitoneal space, a HCT scan of the abdominal cavity and chest, pelvic phlebography and retrograde cavagraphy, echocardi- *Corresponding author. A. A. Baeshko, Dzerjinskogo av., 83, Minsk 220116, Belarus. ography and ultrasound investigation of the abdomi- E-mail addresses: [email protected], [email protected] nal cavity in our patients in order to determine the 1533–3167/000008 + 06 $32.00/0 Ó 2007 Elsevier Ltd. All rights reserved. Table 1. Basic clinical data Age/ DVT: primary/ Temperature Aplasia extension Blood flow collateral tracts Other Therapy type Prognosis sex relapse rise, fever abnormalities 20/m Primary e Infrarenal, renal, Ascending lumbar veins, e Anticoagulants, Recovery of partly suprarenal azygos and hemiazygos veins, phlebotonics, working abilities vertebral veins, paravertebral elastic compression plexus, left testicular, suprarenal and phrenic veins, veins of abdominal wall 26/m d // d þ Infrarenal, renal, d // d Pyelocaliceal d // d Invalidi-zation suprarenal, major duplication of part of retrohepatic the left kidney 20/m e þ Infrarenal Left testicular vein, ascending e Phlebotonics, Recovery of lumbar veins, paravertebral elastic compression working abilities plexus, veins of abdominal wall 32/m Relapse þ Infrarenal, renal, Ascending lumbar veins, azygos e Anticoagulants, d // d partly suprarenal and hemiazygos veins, vertebral phlebotonics, Anomalies of the Inferior Vena Cava veins, paravertebral plexus, left elastic compression testicular, suprarenal and phrenic veins, veins of abdominal wall 43/m e þ Infrarenal Left testicular vein, ascending Pulmonary artery Phlebotonics, d // d lumbar veins, vertebral veins, stenosis, atrial elastic compression paravertebral plexus, veins of septal defect abdominal wall View) with aAcuson 128 5 XP 10 Scannerin (Acuson the IVC Inc. andcharacter Mountain to and identify prevalence other anomalies. of the pathological process CT images were obtained fromwith the the pelvis patient to the in Th ner the supine position. (HiSpeed; Nonenhanced General Electricflow velocity Medical was registered(red also. Systems) color) bloodpump. flow Valvular incompetence during was decompression. shown bysion Blood reversed and decompressionflow if the simulates venous valve thesalva is incompetent. manoeuvre calf Calf provokes compres- reversed muscle The (red color) increased blood abdominalusing the pressure Valsalva test during and the manual calf Val- compression. vein, the inferior venailiac cava. vein, thefemoral external vein, iliac theliteal common vein, vein, femoral the vein the commonveins, the long perforators, iliac internal the saphenous shortstool. vein, saphenous vein, the The the superficial veins pop- sition, examined the knee were flexed the and posterior the feet tibial resting on a foot- were examined with the individual(minimum in a 45 measurable velocity 1 cm/s). All veins times per day and elastic compression. In case of tion of 6 mmthe and superior a vena cava 2.5 junctiondelay, by the means of IVC a system collima- tal was imaged brachial from the vein.Medrad) pelvis at After a to rate of ainjected 4.35 ml/sec 70-second with through the and use antecubi- iohexol of 5 minutes (Omnipaque; a power Nycomed)contrast-enhanced injector was (Envision HCT, automatically a CT; pitch 100 ml of 1:1 of using a 350 mg slice thickness I/ml of 6 mm. For diosmin lies of IVC includedchest vasotonic and abdomen drugs wereContrast performed. was (500 mg enteredcava and of tributaries X-ray and examinationa the of catheter the right waspatient atrium inserted with into the through the patient the IVC. in superiorthe the vena pelvis supine were position made. (Omnipaque; and Nycomed) wascatheterized injected just and below X-rays the of groin,hypoplasia contrast were medium evaluated. and volume rendering,reconstructions, the including presenceval multiplanar or at absence a reformation pitch of of 1:1. With use of three-dimensional US was conducted by the same operator using an HCT scanning was performed with a helical scan- Venous valvular incompetence was examined The treatment of patients with congenital anoma- Retrograde cavagraphy was carried out in one For pelvic phlebography both femoral veins were e Daflon 500, Les Laboratoires Servier) two e 7 MHz probe in low-flow setting e 3.2-mm reconstruction inter- EJVES Extra Vol 14, July 2007 sitting po- 4 at 9 10 A. A. Baeshko et al. DVT we used of IV heparin for 5 days followed by cases partial organized thrombotic masses were warfarin for life. visualized in the lumen. The internal iliac veins were The follow-up lasted from 0.5 to 3.5 years. tortuous, distended to 13e15 mm, with retrograde blood flow. The IVC was partially invisible on US of the retro- Results peritoneal space in all patients. In 2 patients no blood flow could be determined in the infrarenal segment All patients examined were male, aged from 20 to and in 3 patients in the infra-, renal and suprarenal 43 years old. The medical history revealed that the segments. Dilated ascending lumbar veins were seen. disease first presented at 17 till 39 years of age respec- In 3 patients dilated renal veins (18e20 mm in diame- tively. One of the patients underwent a heart surgery ter, linear blood flow velocity up to 10 cm/sec) and for congenital malformation, pulmonary artery steno- their segmental tributaries in the portals were revealed. sis and atrial septal defect, at the age of 5. An other Also ultrasound showed duplication of the pyelocali- one suffered from varicose disease of the lower ceal system of the left kidney in one of the patients. extremities from the age of 15. In all patients US demonstrated dilatation of the In 2 out of 5 patients the pathology presented by anterior abdominal wall veins with the diameter of symptoms of the right iliofemoral thrombosis, and the superficial epigastric vein measuring 9 mm in one in 2 others by a temperature rise, chills and subse- of the patients. quent edema of both legs, and one presented with Pelvic phlebography showed in all patients symptoms of deep vein thrombosis of the right calf. postthrombotic stenosis of external and common iliac Thus, in 3 out of 5 patients the disease presented veins with obscure margins (Fig. 1), as well as dilated with the clinical picture of a DVT. internal iliac veins. Retrograde cavagraphy was per- Retrospective analysis of the US data of the patients formed in one patient for clear illustration of retrohe- with peripheral vein thrombosis in acute stage of dis- patic IVC area and its data showed the constricted ease showed thrombus in one or both common iliac (7.7e9.4 mm in diameter) part of retrohepatic segment veins. It extended to the superficial femoral and popli- of IVC (Fig. 2). teal veins, and in one patient to the posterior tibial vein. Echocardiography did not identify heart- or In one out of three patients the development of vessels-related pathology in any of the patients. thrombosis was contributed to by surgery for a left- The most thorough information about the character sided ureterohydronephrosis.