Citation, DOI, disclosures and article data
At the time the article was created Mohammadtaghi Niknejad had no recorded disclosures.View Mohammadtaghi Niknejad's current disclosures
Polysplenia is seen predominantly in female patients. It is usually diagnosed in childhood or adulthood, later than asplenia syndrome, since associated congenital heart diseases tend to be less severe than those encountered in the latter.
It is related to the associated pathology.
Polysplenia results from failure of development of the usual left-right asymmetry of organs and consists of situs ambiguus, with features of bilateral left-sidedness.
The exact cause of polysplenia is unknown. However, it is suggested that it is caused by various factors 10:
It is characterized by multiple splenules without a parent spleen. It occurs on the patient’s left side but may be bilateral. The most common associated feature is inferior vena cava interruption with azygos or hemiazygos continuation 3.
Other characteristic features include:
Treatment and prognosis
It carries 75% mortality by age 5 though. The presence of congenital heart diseases increases mortality rate. 1-year mortality is approximately 85% with right isomerism and more than 50% with polysplenia syndrome as the latter has less association with congenital heart diseases 11.
- 1. Gayer G, Zissin R, Apter S et-al. CT findings in congenital anomalies of the spleen. Br J Radiol. 2001;74 (884): 767-72. Br J Radiol (full text) - Pubmed citation
- 2. McGahan JP, Goldberg BB. Diagnostic ultrasound. Informa Health Care. (2008) ISBN:1420069780. Read it at Google Books - Find it at Amazon
- 3. Applegate KE, Goske MJ, Pierce G et-al. Situs revisited: imaging of the heterotaxy syndrome. Radiographics. 1999;19 (4): 837-52. Radiographics (full text) - Pubmed citation
- 4. Herman TE, Siegel MJ. Polysplenia syndrome with congenital short pancreas. AJR Am J Roentgenol. 1991;156 (4): 799-800. AJR Am J Roentgenol (citation) - Pubmed citation
- 5. Jelinek JS, Stuart PL, Done SL et-al. MRI of polysplenia syndrome. Magn Reson Imaging. 1990;7 (6): 681-6. Pubmed citation
- 6. Hadar H, Gadoth N, Herskovitz P et-al. Short pancreas in polysplenia syndrome. Acta Radiol. 1991;32 (4): 299-301. Pubmed citation
- 7. Low JP, Williams D, Chaganti JR. Polysplenia syndrome with agenesis of the dorsal pancreas and preduodenal portal vein presenting with obstructive jaundice--a case report and literature review. Br J Radiol. 2011;84 (1007): e217-20. doi:10.1259/bjr/27680217 - Pubmed citation
- 8. Kobayashi H, Kawamoto S, Tamaki T et-al. Polysplenia associated with semiannular pancreas. Eur Radiol. 2001;11 (9): 1639-41. Eur Radiol (link) - Pubmed citation
- 9. Yildiz AE, Ariyurek MO, Karcaaltincaba M. Yildiz AE, Ariyurek MO, Karcaaltincaba M. ScientificWorldJournal. 2013 Apr 21;2013:321810. doi: 10.1155/2013/321810. Print 2013. get pdf
- 10. de la Monte SM, Hutchins GM. Sisters with polysplenia. (1985) American journal of medical genetics. 21 (1): 171-6. doi:10.1002/ajmg.1320210125 - Pubmed
- 11. Kim S. Heterotaxy Syndrome. Korean Circ J. 2011;41(5):227. doi:10.4070/kcj.2011.41.5.227 - Pubmed