Document Type : Case Report
Authors
- Mohammad Amin Dolatkhah 1
- Mohammad Sadegh Gholami Farashah 1
- Nastaran Hesam Shariati 2
- khadije dizaji 1
- Susan Mohammadi 3
- Mohammad Bakhtiar Hesam Shariati 4
1 PhD student, Department of Anatomical Science, Faculty of Medicine, Tabriz University of Medical Sciences, Tabriz, Iran
2 Department of Biomedical Sciences, University of Sydney, Lidcombe, Australia
3 Assistant Professor, Department of Radiology, Faculty of Medicine, Kurdistan University of Medical Sciences, Sanandaj, Iran
4 Assistant Professor, Department of Anatomical Sciences and Histology, School of Medicine, Kurdistan University of Medical Sciences, Sanandaj, Iran
Abstract
Heterotaxy syndrome or situs ambiguous is a rare congenital disease in which the pattern of anatomical organization of the thoraco-abdominal visceral and vascular structures is not arranged in normal position. Patients with heterotaxy syndrome represent a wide range of anatomical variations including thoraco-abdominal structures. Here we report a rare case of asymptomatic heterotaxy syndrome in an elderly female with multiple accessory spleens, stomach on right side of the abdomen, midline liver, azygos continuation of Inferior Vena Cava (IVC) and intestinal malrotation.
Keywords
- Stanger P, Rudolph AM, Edwards JE. Cardiac malpositions. An overview based on study of sixty-five necropsy specimens. Circulation. 1977;56(2):159-72.
- Van Praagh R. Terminology of congenital heart disease. Glossary and commentary. Circulation. 1977;56(2):139-43.
- Uemura H, Ho SY, Devine WA, Anderson RH. Analysis of visceral heterotaxy according to splenic status, appendage morphology, or both. The American journal of cardiology. 1995;76(11):846-9.
- Mujo T, Finnegan T, Joshi J, Wilcoxen KA, Reed JC. Situs ambiguous, levocardia, right sided stomach, obstructing duodenal web, and intestinal nonrotation: a case report. Journal of radiology case reports. 2015;9(2):16.
- Tortajada M, Moreno M, Gracia M, Sanchis A. Rare disease: Situs ambiguous in a schoolchild. BMJ case reports. 2010;2010.
- Mortari EP, Baban A, Cantarutti N, Bocci C, Adorisio R, Carsetti R. Heterotaxy syndrome with and without spleen: Different infection risk and management. Journal of Allergy and Clinical Immunology. 2017;139(6):1981-4. e1.
- Rameshbabu CS, Gupta KK, Qasim M, Gupta OP. Heterotaxy polysplenia syndrome in an adult with unique vascular anomalies: case report with review of literature. Journal of radiology case reports. 2015;9(7):22.
- Ma L, Tierney ESS, Lee T, Lanzano P, Chung WK. Mutations in ZIC3 and ACVR2B are a common cause of heterotaxy and associated cardiovascular anomalies. Cardiology in the Young. 2012;22(2):194-201.
- Farashah MSG, Shariati NH, Asghari R, Mohamadi S, Dolatkhah MA, Shariati MBH. Pulmonary Sequestration: A Case Report. Acta Medica Iranica. 2019;57(6):398-401.
- Mohammadi S, Farashah MSG, Frounchi N, Shoorei H, Shariati MBH. Retro Aortic Left Renal Vein: A Case Report and Brief Literature of Review.
- Yoneyama H, Kondo C, Yamasaki A, Nakanishi T, Sakai S. Comparison of situs ambiguous patterns between heterotaxy syndromes with polysplenia and asplenia. European journal of radiology. 2015;84(11):2301-6.
- Radiopaedia. Radiopaedia. org. 2015.
- Catana A, Apostu AP. The determination factors of left-right asymmetry disorders-a short review. Clujul Medical. 2017;90(2):139.
- Kim S-J. Heterotaxy syndrome. Korean circulation journal. 2011;41(5):227-32.
- Agarwal H, Mittal SK, Kulkarni CD, Verma AK, Srivastava SK. Right isomerism with complex cardiac anomalies presenting with dysphagia-A case report. Journal of radiology case reports. 2011;5(4):1.
- Loomba RS, Anderson RH. Polysplenia or Left Isomerism? Internal Medicine. 2016;55(5):555-.
- Kothari SS. Non-cardiac issues in patients with heterotaxy syndrome. Annals of pediatric cardiology. 2014;7(3):187.
- Shiraishi I, Ichikawa H. Human heterotaxy syndrome. Circulation Journal. 2012;76(9):2066-75.
- Low J, Williams D, Chaganti J. Polysplenia syndrome with agenesis of the dorsal pancreas and preduodenal portal vein presenting with obstructive jaundice—a case report and literature review. The British journal of radiology. 2011;84(1007):e219-e22.
- Rose V, Izukawa T, Moes C. Syndromes of asplenia and polysplenia. A review of cardiac and non-cardiac malformations in 60 cases withspecial reference to diagnosis and prognosis. Heart. 1975;37(8):840-52.
- Yılmaz G, Akpınar SH, Alıcıoğlu B. Polysplenia syndrome detected after chest symptoms in two adult patients: case report and review of literature. Polish journal of radiology. 2014;79:311-4.
- Gatrad A, Read A, Watson G. Consanguinity and complex cardiac anomalies with situs ambiguus. Archives of disease in childhood. 1984;59(3):242-5.
- Vijayvergiya R, Bhat MN, Kumar RM, Vivekanand SG, Grover A. Azygos continuation of interrupted inferior vena cava in association with sick sinus syndrome. Heart (British Cardiac Society). 2005;91(4):e26-e.
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