Learning Tables







Learning Tables







Learning Tables







Learning Tables






Bookmark and Share

From the manuscript

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

Free full text article: 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.






References



1. Jacobs JP, Anderson RH. The nomenclature, definition and classification of cardiac structures in the setting of heterotaxy. Cardiology in the Young, 2007; 17 (Suppl S4): 1-28
Get full text
Find similar topics on Read this article on PubMed :: Find similar articles on Google scholarScholar :: Search for similar topics with the Radiology specific search engine Radiology search engine


2. Kim SJ. Heterotaxy syndrome. Korean Circ J., 2011; 41:227-232
Get full text
Find similar topics on Read this article on PubMed :: Find similar articles on Google scholarScholar :: Search for similar topics with the Radiology specific search engine Radiology search engine


3. Ghosh S, Yarmish G, Godelman A, Haramati LB, Spindola-Franco H. Anomalies of visceroatrial situs. AJR 2009; 193: 1107-1117
Get full text
Find similar topics on Read this article on PubMed :: Find similar articles on Google scholarScholar :: Search for similar topics with the Radiology specific search engine Radiology search engine


4. Peoples WM, Moller JH, Edwards JE. Polysplenia: a review of 146 cases. Pediatr Cardiol., 1983; 4(2): 129-137
Get full text
Find similar topics on Read this article on PubMed :: Find similar articles on Google scholarScholar :: Search for similar topics with the Radiology specific search engine Radiology search engine


5. Gayer G, Apter S. Polysplenia syndrome detected in adulthood: report of eight cases and review of the literature. Abdom Imaging 1999; 24(2): 1768-184
Get full text
Find similar topics on Read this article on PubMed :: Find similar articles on Google scholarScholar :: Search for similar topics with the Radiology specific search engine Radiology search engine


6. Zhu L, Belmont JW, Ware SM. Genetics of human heterotaxias. Eur J Human Genet., 2006;14(1): 17-25
Get full text
Find similar topics on Read this article on PubMed :: Find similar articles on Google scholarScholar :: Search for similar topics with the Radiology specific search engine Radiology search engine


7. Shiraishi I, Ichikawa H. Human heterotaxy syndrome- from molecular genetics to clinical features, management and prognosis.Circ J., 2012; 76(9):2066-2075.
Get full text
Find similar topics on Read this article on PubMed :: Find similar articles on Google scholarScholar :: Search for similar topics with the Radiology specific search engine Radiology search engine


8. Low JP, Williams D, Chaganti JR. Polysplenia syndrome with agenesis of dorsal pancreas and preduodenal portal vein presenting with obstructive jaundice- a case report asnd literature review. Br J Radiol., 2011; 84: e219-e222
Get full text
Find similar topics on Read this article on PubMed :: Find similar articles on Google scholarScholar :: Search for similar topics with the Radiology specific search engine Radiology search engine


9. Rose V, Izukawa T, Moes CAF. Syndromes of asplenia and polysplenia. A review of cardiac and non-cardiac malformations in 60 cases with special reference to diagnosis and prognosis Br Heart J., 1975; 37: 840-852
Get full text
Find similar topics on Read this article on PubMed :: Find similar articles on Google scholarScholar :: Search for similar topics with the Radiology specific search engine Radiology search engine


10. Abut E, Arman A, Guveli H. Malposition of internal organs: A case of situs ambiguous anomaly in an adult. Turk J Gastroenterol., 2003; 14(2): 151-155
Get full text
Find similar topics on Read this article on PubMed :: Find similar articles on Google scholarScholar :: Search for similar topics with the Radiology specific search engine Radiology search engine


11. Gatrad AR, Read AP, Watson GH. Consanguinity and complex cardiac anomalies with situs ambiguous. Arch Dis Child 1984; 59:242-245
Get full text
Find similar topics on Read this article on PubMed :: Find similar articles on Google scholarScholar :: Search for similar topics with the Radiology specific search engine Radiology search engine


12. Applegate KE, Goske MJ, Pierce G, Murphy D. Situs revisited: Imaging of the heterotaxy syndrome. Radiographics 1999; 19: 832-852
Get full text
Find similar topics on Read this article on PubMed :: Find similar articles on Google scholarScholar :: Search for similar topics with the Radiology specific search engine Radiology search engine


13. Tawfik AM, Batouty NM, Zaky MM, Eladalany MA, Elmokadem AH. Polysplenia syndrome: a review of the relationship with viscero-atrial situs and the spectrum of extra- cardiac anomalies. Surg Radiol Anat 2013; 35: 647-653
Get full text
Find similar topics on Read this article on PubMed :: Find similar articles on Google scholarScholar :: Search for similar topics with the Radiology specific search engine Radiology search engine


14. Celik KM, Celik T, Iyisoy A, Guler A. An unusual combination of congenital anomalies in an adult patient: Patent ductus arteriosus, Kommerell`s diverticulum with aberrant right subclavian artery and heterotaxy syndrome. Hellenic J Cardiol., 2011; 52(5): 469- 472
Get full text
Find similar topics on Read this article on PubMed :: Find similar articles on Google scholarScholar :: Search for similar topics with the Radiology specific search engine Radiology search engine


15. Ergun T, Lakadamyali H, Lakadamyali H, Eldem O. Adult polysplenic syndrome accompanied by aberrant right subclavian artery and hemangioma in a cleft spleen: a case report. Ann vasc Surg., 2008; 22(4): 579-581
Get full text
Find similar topics on Read this article on PubMed :: Find similar articles on Google scholarScholar :: Search for similar topics with the Radiology specific search engine Radiology search engine


16. Jo DS, Jung SS, Joo CU. A case of unusual visceral heterotaxy syndrome with isolated levocardia. Korean Circ J., 2013; 43: 705-709
Get full text
Find similar topics on Read this article on PubMed :: Find similar articles on Google scholarScholar :: Search for similar topics with the Radiology specific search engine Radiology search engine


17. Muneta S, Sakai S, Fukuda H, Imamura Y, Matsumoto I. Polysplenia syndrome with various visceral anomalies in an adult: Embryological and clinical considerations. Internal Medicine, 1992; 31: 1026-1031
Get full text
Find similar topics on Read this article on PubMed :: Find similar articles on Google scholarScholar :: Search for similar topics with the Radiology specific search engine Radiology search engine


18. Burton EC, Olson M, Rooper L. Defects in laterality with emphasis on heterotaxy syndromes with asplenia and polysplenia: an autopsy case series at a single institution. Pediatr Dev Pathol, 2014; 17(4): 250-264
Get full text
Find similar topics on Read this article on PubMed :: Find similar articles on Google scholarScholar :: Search for similar topics with the Radiology specific search engine Radiology search engine


19. Van de Perre , Vanhoenacker FM, Petre C, Van Doorn J, De Schepper AM. Heterotaxy syndrome. JBR-BTR, 2004; 87: 158-159
Get full text
Find similar topics on Read this article on PubMed :: Find similar articles on Google scholarScholar :: Search for similar topics with the Radiology specific search engine Radiology search engine


20. Oto A, Akpman E, Surucu HS. Right internal iliac vein joining the left common iliac vein: case report demonstrated by CT angiography. Surg radiol Anat,2003; 25: 339-341
Get full text
Find similar topics on Read this article on PubMed :: Find similar articles on Google scholarScholar :: Search for similar topics with the Radiology specific search engine Radiology search engine


21. Cardinot TM, Aragao AHBM, Babinski MA. Rare variation in course and affluence of internal iliac vein due to its anatomical and surgical significance. Surg Radiol Anat, 2006; 28: 422-425
Get full text
Find similar topics on Read this article on PubMed :: Find similar articles on Google scholarScholar :: Search for similar topics with the Radiology specific search engine Radiology search engine


22. Fulcher AS, Turner MA. Abdominal manifestations of situs anomalies in adults. Radiographics 2002; 22:1439-1456
Get full text
Find similar topics on Read this article on PubMed :: Find similar articles on Google scholarScholar :: Search for similar topics with the Radiology specific search engine Radiology search engine


23. Plata-Munoz JJ, Hernandez-Ramirez D, Anthon FJ, Podgatez E, Avila-Flores F, Chan C. Polysplenia syndrome in the adult patient. Case report with review of literature Ann Hepatol., 2004; 3(3): 114-117
Get full text
Find similar topics on Read this article on PubMed :: Find similar articles on Google scholarScholar :: Search for similar topics with the Radiology specific search engine Radiology search engine


24. Ito H, Ohgi S, Kanno T, Ishibashi T. Heterotaxy syndrome with pancreatic malrotation: CT features. Abdom Imaging 2003; 28(6): 856-858
Get full text
Find similar topics on Read this article on PubMed :: Find similar articles on Google scholarScholar :: Search for similar topics with the Radiology specific search engine Radiology search engine


25. Nichols DM, Li DK. Superior mesenteric vein rotation: A CT sign of midgut malrotation. AJR 1983; 141: 707-708
Get full text
Find similar topics on Read this article on PubMed :: Find similar articles on Google scholarScholar :: Search for similar topics with the Radiology specific search engine Radiology search engine


26. Tsutsumi R, Nagata Y, Enjoji A. Situs ambiguous with gastric cancer: Report of a case. Surg Today 2007; 37:676-679
Get full text
Find similar topics on Read this article on PubMed :: Find similar articles on Google scholarScholar :: Search for similar topics with the Radiology specific search engine Radiology search engine


28. Vanhoenacker FM, De Ruysscher D, De Backer AI, De Schepper AM. Heterotaxy syndrome in adult with polysplenia, visceral and cardiovascular malposition. JBR-BTR, 2001; 84(1): 1-4
Get full text
Find similar topics on Read this article on PubMed :: Find similar articles on Google scholarScholar :: Search for similar topics with the Radiology specific search engine Radiology search engine


29. Kapa S, Gleeson FC, Vege SS. Dorsal pancreas agenesis and polysplenia / Heterotaxy syndrome: A novel association with aortic coarctation and review of the literature. J Pancreas (online) 2007; 8(4): 433-437
Get full text
Find similar topics on Read this article on PubMed :: Find similar articles on Google scholarScholar :: Search for similar topics with the Radiology specific search engine Radiology search engine


30. Maier M, Weisner W, Mengiardi B. Annular pancreas and agenesis of the dorsal pancreas in a patient with polysplenia syndrome. AJR 2007; 188: W150-W153
Get full text
Find similar topics on Read this article on PubMed :: Find similar articles on Google scholarScholar :: Search for similar topics with the Radiology specific search engine Radiology search engine


31. Seo HI, Jeon TY, Sim MS, Kim S. Polysplenia syndrome with preduodenal portal vein detected in adults. World J Gastroenterol., 2008; 14: 6418-6420
Get full text
Find similar topics on Read this article on PubMed :: Find similar articles on Google scholarScholar :: Search for similar topics with the Radiology specific search engine Radiology search engine


32. Kim JG, Youn HJ, Kim GH. Incidentally detected situs ambiguous in adults. J Cardiovasc Ultrasound 2011; 19(4): 211-215
Get full text
Find similar topics on Read this article on PubMed :: Find similar articles on Google scholarScholar :: Search for similar topics with the Radiology specific search engine Radiology search engine


33. Jeong JH, Kim GH, Song GA. Polysplenia syndrome with congenital agenesis of dorsal pancreas presenting as acute pancreatitis and the role of endoscopic ultrasonography in its diagnosis. Korean J Gastroenterol., 2012; 60(1): 47-51
Get full text
Find similar topics on Read this article on PubMed :: Find similar articles on Google scholarScholar :: Search for similar topics with the Radiology specific search engine Radiology search engine


34. Mehta AJ, Kate AH, Gupta N, Chhajed PN. Interrupted inferior vena cava syndrome. JAPI., 2012; 60: 48-50
Get full text
Find similar topics on Read this article on PubMed :: Find similar articles on Google scholarScholar :: Search for similar topics with the Radiology specific search engine Radiology search engine


35. Turkalj I, Petrovic K, Stojanivic S, Mikov I. Polysplenia in an adult patient. Intern Med., 2012; 51:3219
Get full text
Find similar topics on Read this article on PubMed :: Find similar articles on Google scholarScholar :: Search for similar topics with the Radiology specific search engine Radiology search engine


36. Dilli A, Gultekin SS, Ayaz UY, Kaplanoglu H, Hekimoglu B. A rare variation of the heterotaxy syndrome. Case Reports in Medicine 2012
Get full text
Find similar topics on Read this article on PubMed :: Find similar articles on Google scholarScholar :: Search for similar topics with the Radiology specific search engine Radiology search engine


37. Brahmbhatt P, Barad B, Panchal M, Bhavsar V, Saleem A, Klosterman L. Left-sided liver and right sided polysplenia: A 77 -year-old patient with heterotaxy syndrome. Tenn Med 2014; 107(1): 35-36
Get full text
Find similar topics on Read this article on PubMed :: Find similar articles on Google scholarScholar :: Search for similar topics with the Radiology specific search engine Radiology search engine




Search for articles with similar topics

Keyword search:

1. Heterotaxy syndrome

2. Polysplenia Syndrome

3. Situs ambiguous

4. Truncated pancreas

5. Interrupted inferior vena cava

6. Preduodenal portal vein

7. Intestinal malrotation


This article belongs to the GI section.


Search for more in the Internet

Peer-reviewed Radiology Search




Discuss it!

Bookmark and Share




© Journal of Radiology Case Reports - The Interactive Radiology Journal
Affiliated journal of Radiolopolis - The International Radiology Community


The Journal of Radiology Case Reports