Compressive Symptoms Due to Thrombosed or Hypertrophic Collateral Circulation in Infrarenal Inferior Vena Cava Agenesis

Anomalous development of the inferior vena cava: Case reports of agenesis and hypoplasia

“…In patients with agenesis of the infrarenal IVC collateral circulation development is common, usually through the four large ways above mentioned: the gonadal venous system, which drains to the suprarenal cava; the paravertebral venous plexus, which drains to the superior vena cava through the azygous–hemiazygous system; the hemorrhoidal plexus, draining to the portal vein; and the superficial pathway, which drains to the subclavian veins and superior cava through superficial abdominal veins [4] , [5] .…”

“…Symptoms associated to collateral pathways are uncommon, however lower extremity neurologic symptoms, resulting from compression of the lumbosacral nerve roots [4] , [22] , [23] , and abdominal symptoms, caused by compression of visceral structures related to the gonadal venous system, are frequently observed [4] , [24] .…”

“…These malformations can be unique or can be associated with other abnormalities such as situs inversus, dextrocardia, or polysplenia and asplenia [4] .…”

Absence of the suprarenal segment of the inferior vena cava with a coexisting absence of the right brachiocephalic vein in a 22-year-old Caucasian male with arterial hypertension

“…If a venous collateral circulation is efficient enough, this anomaly is usually asymptomatic [11,13]. In the literature some clinical manifestations of the disease have been described, such as isolated iliac vein thrombosis in small children and young subjects, varicose veins of the internal organs and intravertebral venous plexus, pathological mediastinal or abdominal masses, cyanosis of the abdomen and lower extremities [5,7,10,17].…”

“…It is estimated that they occur in 0.3% of otherwise healthy individuals, and in 0.6% to 2% of patients with coexisting various cardiovascular abnormalities, although these figures likely represent underestimates [15]. Congenital anomaly of the IVC may be a stand-alone defect or it may coexist with disturbances in the development of other vessels and organs, especially the heart and the spleen [7,10,17]. Generally, the majority of the anatomical anomalies of the IVC are usually recognised incidentally during imaging, operations or dissection studies.…”