Polysplenia

Common Name(s)

Polysplenia

Polysplenia is a disorder in which an individual has multiple spleens that are about equal in size. This disorder is caused by improper connection between parts of the spleen during fetal development. Polysplenia is usually accompanied by other developmental problems, particularly severe heart defects, which often results in death before the age of 5. Polysplenia is believed to be caused by environmental factors that disturb development in utero (teratogenic) or genetic factors. Female infants are affected more often than males.

Complications that are commonly seen with polysplenia include intestinal malrotation, biliary atresia, and cardiac abnormalities. Intestinal malrotation occurs when the intestines do not rotate properly during normal fetal development, and may lead to poor blood supply or physical blockages that prevent the intestines from functioning normally. Biliary atresia is another developmental abnormality in which the bile ducts that normally drain bile from the liver are blocked, and may lead to the accumulation of bile that damages the liver. Cardiac problems include severe defects in the major veins and arteries, displacement of the chambers of the heart, and a heart that is located on the opposite side of the chest. Only about 10% of affected individuals have either minor or no cardiac problems, allowing them to survive into adulthood without symptoms.

Polysplenia is often diagnosed in infants after heart problems are noticed. In less severe cases, polysplenia is diagnosed incidentally after medical imaging or surgery for other reasons. Medication or surgery may be used to treat the symptoms of polysplenia. Surgery is more complicated in affected individuals due to abnormalities in the arrangement and placement of multiple organs. If you or your child has been diagnosed with polysplenia, talk to your doctor about the most current treatment options.

Source: Advocacy organizations associated with the condition.

 

Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Polysplenia" for support, advocacy or research.

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Heterotaxy Foundation

The Heterotaxy Foundation is a non-profit organization created to raise funds for research and promote awareness of a rare disease called Heterotaxy Syndrome.

Last Updated: 12 Feb 2013

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How do you compare to others with this condition?

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Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Polysplenia" for support, advocacy or research.

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Heterotaxy Foundation

The Heterotaxy Foundation is a non-profit organization created to raise funds for research and promote awareness of a rare disease called Heterotaxy Syndrome.

http://www.heterotaxy.org

Last Updated: 12 Feb 2013

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General Support Organizations

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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Polysplenia" returned 31 free, full-text research articles on human participants. First 3 results:

Polysplenia or Left Isomerism?
 

Author(s): Rohit S Loomba, Robert H Anderson

Journal: Intern. Med.. 2016 ;55(5):555.

 

Last Updated: 3 Mar 2016

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[Biliary atresia and polysplenia syndrome].
 

Author(s): Yosra Kerkeni, Amine Ksia, Hayet Zitouni, Mohsen Belghith, Sahnoun Lassad, Imed Krichene, Mongi Mekki, Abdellatif Nouri

Journal: Tunis Med. ;93(8-9):494-6.

 

Polysplenia syndrome is a rare malformation characterized by the association of multiple rates and other congenital anomalies dominated by cardiac, vascular, intestinal and bile malformations. We report the observation of a patient operated in the neonatal period (3 days) for an upper ...

Last Updated: 28 Jan 2016

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An adult case of polysplenia syndrome associated with sinus node dysfunction, dextrocardia, and systemic venous anomalies.
 

Author(s): Kentaro Fukuda, Toshihito Onda, Yuki Kimura, Seiji Miura, Rie Matsumori, Yoshiyuki Masaki, Akihisa Nishino, Kenji Inoue, Yasumasa Fujiwara, Masataka Sumiyoshi

Journal: Intern. Med.. 2015 ;54(9):1071-4.

 

A 54-year-old woman was referred to our hospital for symptomatic sinus bradyarrhythmia with a sinus pause of 8 seconds. She was diagnosed with dextrocardia during childhood and discovered to have heterotaxy syndrome when she had an appendectomy during her teenager years. Chest and ...

Last Updated: 7 May 2015

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Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
The terms "Polysplenia" returned 4 free, full-text review articles on human participants. First 3 results:

Polysplenia Syndrome With Splenic and Skeletal Muscle Metastases From Thyroid Carcinoma Evaluated by FDG PET/CT: Case Report and Literature Review: A Care-Compliant Article.
 

Author(s): Zu-Gui Li, Zhi-Chun Lin, Hai-Yu Mu

Journal: Medicine (Baltimore). 2016 Jan;95(4):e2532.

 

Polysplenia syndrome (PSS) is a rare congenital abnormality. Metastases to spleen and skeletal muscle from differentiated thyroid cancer (DTC) are also extremely rare. Our case report aims to present an interesting case of PSS associated with splenic metastasis (SM) and skeletal muscle ...

Last Updated: 30 Jan 2016

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

Author(s): Chittapuram Srinivasan Rameshbabu, Kanchan Kumar Gupta, Muhammad Qasim, Om Prakash Gupta

Journal:

 

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 ...

Last Updated: 2 Dec 2015

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Polysplenia syndrome with agenesis of the dorsal pancreas and preduodenal portal vein presenting with obstructive jaundice--a case report and literature review.
 

Author(s): J P Low, D Williams, J R Chaganti

Journal: Br J Radiol. 2011 Nov;84(1007):e217-20.

 

Polysplenia, as part of the heterotaxy syndrome, is a rare embryological disorder which results from failure of development of the usual left-right asymmetry of organs. It is often associated with cardiac and biliary abnormalities, which are the usual causes of death in early neonatal ...

Last Updated: 20 Oct 2011

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Symptoms, Diagnosis, and Treatment

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Clinical Trial Information This information is provided by ClinicalTrials.gov

Molecular Genetics of Heterotaxy and Related Congenital Heart Defects
 

Status: Recruiting

Condition Summary: Heterotaxy Syndrome; Congenital Heart Defects

 

Last Updated: 25 Aug 2016

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