Orphanet: Situs inversus totalis

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Situs inversus totalis

Disease definition

A rare, genetic, developmental defect during embryogenesis characterized by total mirror-image transposition of both thoracic and abdominal viscera across the left-right axis of the body. Congenital abnormalities, such as primary ciliary dyskinesia, Kartagener type, polysplenia syndrome, biliary atresia, congenital heart disease, and midgut malrotation, as well as vascular anomalies (e.g. absence of retrohepatic inferior vena cava, preduodenal portal vein, aberrant hepatic arterial anatomy) and malignancy, are frequently associated.


Classification level: Disorder
  • Synonym(s):
    • Complete situs inversus
    • Complete situs inversus viscerum
    • Situs inversus
  • Prevalence: -
  • Inheritance: Autosomal recessive or Autosomal dominant or Not applicable 
  • Age of onset: -
  • ICD-10: Q89.3
  • ICD-11: LA82
  • OMIM: -
  • UMLS: C0037221
  • MeSH: -
  • GARD: 4883
  • MedDRA: -

Detailed information


ERN : produced/endorsed by ERN(s)
FSMR : produced/endorsed by FSMR(s)
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