x

Search for a rare disease

* (*) mandatory field

Other search option(s)

Suggest an update

(*) Required fields.

Attention

Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted. For all other comments, please send your remarks via contact us. Only comments written in English can be processed.

Captcha image

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.

ORPHA:101063

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
  • OMIM: -
  • UMLS: C0037221
  • MeSH: -
  • GARD: 4883
  • MedDRA: -

Additional information

Further information on this disease

Specialised Social Services

The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.