title: Heterotaxy Polysplenia Syndrome In An Adult With Unique Vascular Anomalies: Case Report With Review Of Literature.
abstract: 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.
authors.affiliation: Department of Anatomy, Muzaffarnagar Medical College, N.H.-58, Muzaffarnagar- 251203 (U.P), India.
authors.forename: Chittapuram Srinivasan
authors.initials: CS
authors.lastname: Rameshbabu
authors.affiliation: Dr. O.P.Gupta Imaging Center, 387, Sumer Bhawan, Bachcha Park, Meerut- 250002. (U.P), India.
authors.forename: Kanchan Kumar
authors.initials: KK
authors.lastname: Gupta
authors.affiliation: Dr. O.P.Gupta Imaging Center, 387, Sumer Bhawan, Bachcha Park, Meerut- 250002. (U.P), India.
authors.forename: Muhammad
authors.initials: M
authors.lastname: Qasim
authors.affiliation: Dr. O.P.Gupta Imaging Center, 387, Sumer Bhawan, Bachcha Park, Meerut- 250002. (U.P), India.
authors.forename: Om Prakash
authors.initials: OP
authors.lastname: Gupta
mesh_terms: D000328:Adult; D001013:Aorta, Thoracic / Q000002:abnormalities*; D001980:Bronchi / Q000098:blood supply; D006499:Hepatic Artery / Q000002:abnormalities; D059446:Heterotaxy Syndrome / Q000150:complications* / Q000000981:diagnostic imaging / Q000453:epidemiology / Q000209:etiology; D006801:Humans; D007084:Iliac Vein / Q000002:abnormalities; D007669:Kidney Calculi / Q000150:complications / Q000175:diagnosis / Q000601:surgery; D008168:Lung / Q000002:abnormalities; D008297:Male; D011859:Radiography; D012077:Renal Artery / Q000002:abnormalities*; D012082:Renal Veins / Q000002:abnormalities*; D013348:Subclavian Artery / Q000002:abnormalities; D014682:Vena Cava, Inferior / Q000002:abnormalities
keywords: Heterotaxy syndrome; Interrupted inferior vena cava; Intestinal malrotation; Polysplenia Syndrome; Preduodenal portal vein; Situs ambiguous; Truncated pancreas