Congenital absence of inferior vena cava and thrombosis: a case report

Abstract

IntroductionA congenitally absent Inferior Vena Cava (IVC) is a rare anomaly that is recognised to be associated with idiopathic Deep Venous Thrombosis (DVT), particularly in the young. It may not be apparent until later in life. Retrospectively, as discussed in this case, there can be clues indicating the presence of such an anomaly from a young age. However, it is not clear whether early recognition of this condition would affect the prognosis and treatment.Case presentationA 54 year old gentleman was admitted with 3 weeks of abdominal pain and localised swelling over the right flank. Examination revealed palpable 'snake-like' tortuous, tender lumps on the abdominal wall. Past history revealed chronic non-healing venous leg ulcers, and varicose veins necessitating varicose vein ligation at a very young age. The ulcers eventually needed skin grafting.During this, current admission he was investigated and diagnosed with Deep Vein Thrombosis (DVT). CT scan, performed to search for intra-abdominal cancer, revealed absence of the Inferior Vena Cava with extensive thrombosed collaterals of the superficial abdominal and azygous veins and a congenitally atrophic left kidney.ConclusionThis is a case of one of the oldest patient described in the literature to be diagnosed with absence of the IVC. It is thought that IVC anomalies are under-diagnosed, and may be commoner than once believed. However there were vital clues in his previous medical history suspicious for an underlying venous anomaly. Idiopathic DVT in a relatively young person with a past history of chronic leg ulceration or varicose veins should be investigated for congenital anomalies of the IVC. This is best achieved by CT scan of the abdomen.

Agenesia de veia cava inferior associada à trombose venosa profunda

“…Some authors suggest that thrombosis on the IVC during the perinatal period is the origin of its disappearance, hence no embryologic abnormalities are seen 1,3,9 . IVC agenesis or hypoplasia may be accompanied by other congenital abnormalities such as splenic anomalies, disorders of intestinal rotation, pulmonary dysgenesis, renal agenesis, dextrocardia and other congenital heart diseases 1,3,4,9,11 .…”

“…To date, 15 different types have been reported, and the most common ones are left IVC, double The embryogenesis of the IVC is a complex event that involves formation, regression and fusion of three pairs of embryonic veins 1,3,7,8,10 . The infrahepatic segment of the IVC may be divided in three parts: suprarenal, renal and infrarenal; the right subcardinal vein becomes the suprarenal segment; the supracardinal anastomoses with the subcardinal vein originates the renal segment, and the infrarenal segment emerges from the right supracardinal vein 1,5,8,9 .…”

“…The best imaging methods for the diagnosis of IVC anomalies are CT angiography and MRI and the diagnosis is considered to be difficult when ultrasonography is used alone 3,4,6,13,14 .…”

“…Its prevalence in the western population is estimated to be 1:1,000 individuals per year [1][2][3] . The prevalence varies according to age, being ten times lower in 20 to 40 year-old individuals than in older age groups 2,3 .…”

Agenesia da veia cava inferior

“…2,3 If the originally paired structures are not joined between the sixth and eighth weeks of gestation, malformations can occur, such as a duplicated inferior vena cava, inferior vena cava agenesis, and the interruption of a certain segment (infrahepatic, prerenal, renal, or infrarenal), among others. 3,4 Inferior vena cava malformations are present in 0.07% to 8.7% of the population 3 and may be asymptomatic or associated with nonspecific symptoms.…”