OBJECTIVE: Agenesis of the inferior vena cava (IVC) is a rare vascular malformation. Deep vein thrombosis (DVT) and bilateral pelvic thrombosis develop quite frequently, making surgical therapy necessary. PATIENTS AND METHODS: Between 1982 and 2006, 15 patients (nine male, six female, mean age 28 standard deviance 9 years) with agenesis of the IVC (IVCA) were treated surgically because of acute or subacute DVT. These patients underwent bilateral transfemoral ante- and retrograde thrombectomy of the iliofemoral and sometimes popliteal veins and replacement of the IVC with an external ring supported PTFE-graft. Bi- or unilateral arteriovenous fistulae were created in the femoral region. The fistulae were closed, on average, 8 months after trans-arterial venography was performed. These patients were examined clinically and by duplex ultrasound imaging during follow-up to assess graft patency and to allow CEAP classification. Patients were assessed for the development of post-thrombotic syndrome (PTS). RESULTS: No patient died during any part of their treatment or within 60 days. Primary patency of the venous reconstruction was 53%, secondary and long time follow-up patency was 83%. The mean duration of follow-up was 41 SD 12 months. Minor complications were observed in five cases (33%). PTS showed no progression during a follow-up of 41 SD 12 months in all patients. There was no change in the CEAP clinical stage during follow-up nor did any leg ulcer develop. CONCLUSION: A surgical approach to restore venous patency is effective and appears to prevent the deterioration of CVI over time. Copyright (c) 2010 European Society for Vascular Surgery. Published by Elsevier Ltd. All rights reserved.
OBJECTIVE: Agenesis of the inferior vena cava (IVC) is a rare vascular malformation. Deep vein thrombosis (DVT) and bilateral pelvic thrombosis develop quite frequently, making surgical therapy necessary. PATIENTS AND METHODS: Between 1982 and 2006, 15 patients (nine male, six female, mean age 28 standard deviance 9 years) with agenesis of the IVC (IVCA) were treated surgically because of acute or subacute DVT. These patients underwent bilateral transfemoral ante- and retrograde thrombectomy of the iliofemoral and sometimes popliteal veins and replacement of the IVC with an external ring supported PTFE-graft. Bi- or unilateral arteriovenous fistulae were created in the femoral region. The fistulae were closed, on average, 8 months after trans-arterial venography was performed. These patients were examined clinically and by duplex ultrasound imaging during follow-up to assess graft patency and to allow CEAP classification. Patients were assessed for the development of post-thrombotic syndrome (PTS). RESULTS: No patient died during any part of their treatment or within 60 days. Primary patency of the venous reconstruction was 53%, secondary and long time follow-up patency was 83%. The mean duration of follow-up was 41 SD 12 months. Minor complications were observed in five cases (33%). PTS showed no progression during a follow-up of 41 SD 12 months in all patients. There was no change in the CEAP clinical stage during follow-up nor did any leg ulcer develop. CONCLUSION: A surgical approach to restore venous patency is effective and appears to prevent the deterioration of CVI over time. Copyright (c) 2010 European Society for Vascular Surgery. Published by Elsevier Ltd. All rights reserved.
Authors: Paul Monagle; Anthony K C Chan; Neil A Goldenberg; Rebecca N Ichord; Janna M Journeycake; Ulrike Nowak-Göttl; Sara K Vesely Journal: Chest Date: 2012-02 Impact factor: 9.410
Authors: Tolga Atilla Sagban; Rüdiger E Scharf; Markus U Wagenhäuser; Alexander Oberhuber; Hubert Schelzig; Klaus Grabitz; Mansur Duran Journal: Orphanet J Rare Dis Date: 2015-01-21 Impact factor: 4.123
Authors: Alexandra Leitão; Joana M Esteves; João Pedro Abreu; Ana Francisca Pereira; Maria Teresa Boncoraglio; Manuela Certo; Rodolfo Abreu Ferreira Journal: Eur J Case Rep Intern Med Date: 2019-03-15