Various anatomic anomalies have been considered the causes of nutcracker syndrome (NCS). Posterior NCS refers to the condition, in which vascular narrowing was secondary to the compression of the retroaortic left renal vein while it is crossing between the aorta and the vertebral column. Here, we report an unusual case of posterior NCS associated with a complicated malformation of the interrupted left inferior vena cava with azygos continuation and retroaortic right renal vein, diagnosed by both color Doppler ultrasonography and CT angiography.
Adult ; Azygos Vein/*abnormalities ; Diagnosis, Differential ; Female ; Humans ; Renal Nutcracker Syndrome/*radiography/*ultrasonography ; Renal Veins/*abnormalities ; Tomography, X-Ray Computed ; Vena Cava, Inferior/*abnormalities

Congenital extrahepatic portocaval shunt (CEPS) is a rare anomaly of the mesenteric vasculature in which the intestinal and splenic venous drainage bypasses the liver and drains directly into the inferior vena cava, the left hepatic vein or the left renal vein. This uncommon disease is frequently associated with other malformations and mainly affects females. Here we report a case of pulmonary arterial hypertension associated with CEPS (Abernethy type 1b shunt) in a 20-yr-old man who was incidentally diagnosed during evaluation of multiple nodules of the liver. The patient was treated by inhalation of iloprost (40 microg/day) with improved condition and walking test. Physicians should note that congenital portocaval shunt may cause pulmonary hypertension.
Echocardiography, Doppler ; Humans ; Hypertension, Pulmonary/*diagnosis/drug therapy ; Iloprost/therapeutic use ; Liver/blood supply/radiography ; Magnetic Resonance Imaging ; Male ; Thoracic Arteries/ultrasonography ; Tomography, X-Ray Computed ; Vasodilator Agents/therapeutic use ; Vena Cava, Inferior/*abnormalities/ultrasonography ; Young Adult