Heterotaxy Polysplenia Syndrome In An Adult With Unique Vascular Anomalies: Case Report With Review Of Literature.

The pattern of anatomical organization of the thoraco-abdominal visceral and vascular structures which is not the expected normal arrangement, is called as situs ambiguous or heterotaxy syndrome. Patients with heterotaxy syndrome exhibit a wide spectrum of anatomical variations involving thoraco-abdominal structures. We present here an incidental finding of heterotaxy syndrome associated with unique vascular anomalies in a 35 year old male patient evaluated initially for nephrolithiasis by ultrasonography, and intravenous pyelography. Further evaluation by multidetector row computed tomography showed bilateral bilobed lungs with hyparterial bronchi, cardiac apex to the left, five branches from left-sided aortic arch with retroesophageal right subclavian artery, interrupted inferior vena cava with azygos continuation, left renal vein continuing as hemiazygos vein and replaced common hepatic artery arising from the superior mesenteric artery. Other vascular anomalies include right internal iliac vein joining the left common iliac vein and precaval course of the single main right renal artery. Anomalies involving abdominal organs include right-sided stomach, midline liver, multiple splenules (polysplenia) in right upper quadrant of abdomen, short truncated pancreas, intestinal malrotation, inversion of superior mesenteric vessels and a preduodenal portal vein. To the best of our knowledge this is the first report of association of left renal vein continuing as hemiazygos vein, precaval right renal artery and anomalous branching pattern of aortic arch with heterotaxy syndrome.

Bilateral asymmetrical duplicated origin of vertebral arteries: Multidetector row CT angiographic study.

Bilateral duplicated origin of V-1 segment of vertebral arteries is an extremely rare vascular variant and only two such cases have been reported so far. Presence of this vascular abnormality was observed incidentally in a 36-year-old male patient, with a complaint of dizziness, evaluated by multidetector row computed tomography (CT) angiography. Two limbs of the right vertebral artery arose from the right subclavian artery and fused to form a single vessel at the interval between fourth and fifth cervical vertebrae, which entered the foramen transversarium of fourth cervical vertebra. On the left side, the medial limb originated directly from the arch of aorta and the lateral limb from the left subclavian artery, and both united at the interval between fifth and sixth cervical vertebrae to form a single vessel which entered the foramen transversarium of fifth cervical vertebra. No other cerebrovascular pathology like aneurysm, fenestration, dissection, and stenosis was detected, which could be correlated with the symptoms of the patient. This rare congenital vascular anomaly has diagnostic and therapeutic implications in any intervention involving the vertebral artery.