P-125 - CONGENITAL INFERIOR VENA CAVA AGENESIS PRESENTING AS UNILATERAL VARICOSE VEINS IN THE LOWER EXTREMITIES

TOPIC:
Case Reports
AUTHORS:
El Beyrouti H. (Department of Cardiac and vascular Surgery, University Medical Center, Johannes-Gutenberg University ~ Mainz ~ Germany) , Al-Saegh S. (Department of Cardiac and vascular Surgery, University Medical Center, Johannes-Gutenberg University ~ Mainz ~ Germany) , Nancy H. (Department of Cardiac and vascular Surgery, University Medical Center, Johannes-Gutenberg University ~ Mainz ~ Germany) , Kamchybekov U. (Department of Cardiac and vascular Surgery, University Medical Center, Johannes-Gutenberg University ~ Mainz ~ Germany) , Treede H. (Department of Cardiac and vascular Surgery, University Medical Center, Johannes-Gutenberg University ~ Mainz ~ Germany) , Omar M. (Department of Cardiac and vascular Surgery, University Medical Center, Johannes-Gutenberg University ~ Mainz ~ Germany)
Introduction:
Complete absence of Inferior Vena Cava (IVC) is a rare congenital anomaly with an estimated prevalence of 0.0005 % to 1 % in general population. The IVC agenesis usually asymptomatic with incidental detection. It may also present with deep venous thrombosis (DVT) in young adults, pulmonary thromboembolism, Pelvic Congestion Syndrome and causes diagnostic problems in the paravertebral area because of their tumor like appearance.
Methods:
We report a case of 15-years-old male presented to our outpatient clinic on August 2013 with right lower extremity aching pain, muscle cramps, sensation of throbbing, and fatigue especially with exertion and training. The patient couldn't participate in sport sessions in school more than 15 minutes then complaining from muscle cramps, fatigue, swelling and Blue discoloration of legs so he must to stop and elevate his legs for approximately 10 minutes until relief of pain. An ultrasound demonstrate no evidence of DVT and incompetent femoral-saphenous junction and magnetic resonance angiography (MRA) demonstrate IVC agenesis and enlarged azygous and hemiazygous veins. The right iliac veins is replaced with multiple tortuous venous channels, the left iliac veins showed absence of the common iliac vein, both are draining in the prominent azygous and hemiazygous veins.
Results:
Development of thrombosis in this case is possibly unprovoked since the patient had tolerated elastic stockings support and intermittent elevation of the leg and avoidance of risk factors over 8 years follow-up without any occurrence of deep vein thrombosis (DVT) or worsening the symptoms.
Conclusion:
It is reasonable to consider IVC agenesis as a differential diagnosis in young patients presenting with extensive varicose with no typical risk factors. Contrast enhanced CT scan or magnetic resonance imaging (MRI) studies should be considered in diagnosing IVC anomalies. The treatment strategy of IVC agenesis with varicose veins of lower limbs is individualized. Elastic stockings support and intermittent elevation of the leg and avoidance of risk factors like prolonged immobilization have been shown to be of benefit. The mainstay of treatment is conservative with anticoagulation to prevent venous thrombosis.