U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Polysplenia heterotaxy syndrome

MedGen UID:
768727
•Concept ID:
C3640087
•
Disease or Syndrome
Synonym: Heterotaxy Syndrome with Polysplenia
SNOMED CT: Polysplenia heterotaxy syndrome (8641000119101)

Definition

A congenital defect in which there is an abnormal spatial arrangement of the internal thoraco-abdominal organs; this manifestation of the disorder presents with many accessory spleens instead of one. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPolysplenia heterotaxy syndrome

Professional guidelines

PubMed

Practical approach to imaging diagnosis of biliary atresia, Part 1: prenatal ultrasound and magnetic resonance imaging, and postnatal ultrasound.
Napolitano M, Franchi-Abella S, Damasio MB, Augdal TA, Avni FE, Bruno C, Darge K, Ključevšek D, Littooij AS, Lobo L, Mentzel HJ, Riccabona M, Stafrace S, Toso S, Woźniak MM, Di Leo G, Sardanelli F, Ording Müller LS, Petit P
Pediatr Radiol 2021 Feb;51(2):314-331. Epub 2020 Nov 17 doi: 10.1007/s00247-020-04840-9. PMID: 33201318
Bacteremia in Patients with Heterotaxy: A Review and Implications for Management.
Loomba RS, Geddes GC, Basel D, Benson DW, Leuthner SR, Hehir DA, Ghanayem N, Shillingford AJ
Congenit Heart Dis 2016 Dec;11(6):537-547. Epub 2016 Jul 18 doi: 10.1111/chd.12395. PMID: 27425254
Perinatal and infant outcomes of prenatal diagnosis of heterotaxy syndrome (asplenia and polysplenia).
Escobar-Diaz MC, Friedman K, Salem Y, Marx GR, Kalish BT, Lafranchi T, Rathod RH, Emani S, Geva T, Tworetzky W
Am J Cardiol 2014 Aug 15;114(4):612-7. Epub 2014 Jun 6 doi: 10.1016/j.amjcard.2014.05.042. PMID: 24996551Free PMC Article

Recent clinical studies

Diagnosis

Aortic pseudocoarctation associated with polysplenia/heterotaxy syndrome.
Duarte R, Morais H
Rev Port Cardiol 2015 Jan;34(1):73.e1-3. Epub 2015 Jan 10 doi: 10.1016/j.repc.2014.08.012. PMID: 25583627
Dorsal pancreas agenesis and polysplenia/heterotaxy syndrome: a novel association with aortic coarctation and a review of the literature.
Kapa S, Gleeson FC, Vege SS
JOP 2007 Jul 9;8(4):433-7. PMID: 17625295

Supplemental Content

Table of contents

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Related information

    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...