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Agenesis of the Suprarenal Inferior Vena Cava With Azygous Continuation
October 2013, Vol 144, No. 4_MeetingAbstracts Signs and Symptoms of Chest Diseases | October 2013
Agenesis of the Suprarenal Inferior Vena Cava With Azygous Continuation Hamdi Gabr Amar, MD Royal Bournemouth Hospital, Bournemouth, United Kingdom Chest. 2013;144(4_MeetingAbstracts):933A. doi:10.1378/chest.1701357
Abstract SESSION TITLE: Miscellaneous Global Case Reports SESSION TYPE: Global Case Report PRESENTED ON: Tuesday, October 29, 2013 at 01:30 PM - 02:30 PM INTRODUCTION: Inferior Vena Cava Agenesis (IVCA) , also called IVC atresia or aplasia ,is a very rare congenital anomaly that is mostly asymptomatic.However, if not considered it may be a diagnostic mystery.It may also cause risks during surgical and radiological procedures.We describe a case report of IVCA to enhance awareness of this rare condition CASE PRESENTATION: A 40 year old man was referred with a CXR reported as bulky right hilum following a month history of coughing up blood tinged sputum.He had no alarming symptoms,never smoked,had normal clinical examination and normal bloods.A staging chest and abdominal CT scans ,performed to rule out malignancy,revealed Inferior vena cava agenesis/atresia (IVCA). DISCUSSION: IVCA is a rare congenital abnormality with an estimated prevalence of 0.00051% in the general population.It was found in 2%of patients undergoing heart catheterization for congenital heart lesions.Its etiology is controversial,but embryological dysgenesis seems to be the most commonly proposed origin.However,other authors demonstrated that IVCA could result from intrauterine or perinatal thrombosis.IVCA must be differentiated from congenital membranes.Several anomalies of IVCA were described based on the phase of dysgenesis;total IVCA,Absence of the Infrarenal IVC,Absence of suprarenal IVC(Interruption of the IVC with Azygous or Hemiazygous Continuation),Membranous Obstruction of the Intrahepatic IVC,Left IVC and Double IVC.Although it mostly remains asymptomatic,clinical awareness is important as it may present with thromboembolic complications in young patients with no other reason for thromboembolism.Dilated azygos/hemiazygos system on chest or abdominal X-rays can be misinterpreted as a mediastinal or retroperitoneal neoplasm, lymphadenopathy or aortic dissection.Finally,awareness of their existence helps avoid pitfalls in preoperative surgical and interventional radiological planning e.g.Cardiopulmonary bypass,IVC filter placement CONCLUSIONS: This case not only adds to the scarcely reported cases of IVCA , but it is also meant to enhance awareness in the respiratory and the whole medical community about this rare anomaly that may cause a diagnostic dilemma as well as potential risks if not anticipated. Reference #1: 1Ferris,E.J.et al.Baltimore,Md:Williams&Wilkins1969;1-32
Reference #2: 2Kellman,G.M.et al.RadioGraphics1988;8(3):533-556 DISCLOSURE: The following authors have nothing to disclose: Hamdi Gabr Amar No Product/Research Disclosure Information
Agenesis of the Suprarenal Inferior Vena Cava With Azygous Continuation Hamdi Gabr Amar, MD Royal Bournemouth Hospital, Bournemouth, United Kingdom Chest. 2013;144(4_MeetingAbstracts):933A. doi:10.1378/chest.1701357
Abstract SESSION TITLE: Miscellaneous Global Case Reports SESSION TYPE: Global Case Report PRESENTED ON: Tuesday, October 29, 2013 at 01:30 PM - 02:30 PM INTRODUCTION: Inferior Vena Cava Agenesis (IVCA) , also called IVC atresia or aplasia ,is a very rare congenital anomaly that is mostly asymptomatic.However, if not considered it may be a diagnostic mystery.It may also cause risks during surgical and radiological procedures.We describe a case report of IVCA to enhance awareness of this rare condition CASE PRESENTATION: A 40 year old man was referred with a CXR reported as bulky right hilum following a month history of coughing up blood tinged sputum.He had no alarming symptoms,never smoked,had normal clinical examination and normal bloods.A staging chest and abdominal CT scans ,performed to rule out malignancy,revealed Inferior vena cava agenesis/atresia (IVCA). DISCUSSION: IVCA is a rare congenital abnormality with an estimated prevalence of 0.00051% in the general population.It was found in 2%of patients undergoing heart catheterization for congenital heart lesions.Its etiology is controversial,but embryological dysgenesis seems to be the most commonly proposed origin.However,other authors demonstrated that IVCA could result from intrauterine or perinatal thrombosis.IVCA must be differentiated from congenital membranes.Several anomalies of IVCA were described based on the phase of dysgenesis;total IVCA,Absence of the Infrarenal IVC,Absence of suprarenal IVC(Interruption of the IVC with Azygous or Hemiazygous Continuation),Membranous Obstruction of the Intrahepatic IVC,Left IVC and Double IVC.Although it mostly remains asymptomatic,clinical awareness is important as it may present with thromboembolic complications in young patients with no other reason for thromboembolism.Dilated azygos/hemiazygos system on chest or abdominal X-rays can be misinterpreted as a mediastinal or retroperitoneal neoplasm, lymphadenopathy or aortic dissection.Finally,awareness of their existence helps avoid pitfalls in preoperative surgical and interventional radiological planning e.g.Cardiopulmonary bypass,IVC filter placement CONCLUSIONS: This case not only adds to the scarcely reported cases of IVCA , but it is also meant to enhance awareness in the respiratory and the whole medical community about this rare anomaly that may cause a diagnostic dilemma as well as potential risks if not anticipated. Reference #1: 1Ferris,E.J.et al.Baltimore,Md:Williams&Wilkins1969;1-32
Reference #2: 2Kellman,G.M.et al.RadioGraphics1988;8(3):533-556 DISCLOSURE: The following authors have nothing to disclose: Hamdi Gabr Amar No Product/Research Disclosure Information