Polysplenia syndrome
Citation, DOI, disclosures and article data
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View Mohammadtaghi Niknejad's current disclosuresAt the time the article was last revised Hoe Han Guan had no financial relationships to ineligible companies to disclose.
View Hoe Han Guan's current disclosures- Left isomerism
- Polysplenia
- Bilateral left-sidedness
- Left sided isomerism
Polysplenia syndrome, also known as left isomerism, is a type of heterotaxy syndrome where there are multiple spleens congenitally as part of left-sided isomerism.
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Epidemiology
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.
Etiology
The exact cause of polysplenia is unknown. However it is suggested that it is caused by various factors 10:
embryogenic
genetic
teratogenic
Clinical manifestation
Clinical presentation is related to the associated pathology.
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.
Radiographic features
General
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:
bilateral hyparterial bronchi
bilateral bilobed lungs
bilateral pulmonary/left atria
midline liver
Associations
-
congenital heart disease (>50%): especially non-cyanotic, and less complex/severe than in asplenia syndrome 3
dextrocardia (37%)
-
gastrointestinal
semi-annular pancreas / dorsal pancreatic agenesis 4,6,8
midgut malrotation (80%) 7,8
gallbladder agenesis (50%) and biliary atresia
absent C-loop of duodenum
-
genitourinary
-
vascular
-
inferior vena cava anomalies 7,8
intrahepatic IVC interruption with azygos/hemiazygos continuation
-
portal vein anomalies 7,8
-
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See also
References
- 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
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