Situs inversus totalis

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Situs inversus totalis

Synonyms: Complete situs inversus | Complete situs inversus viscerum | Situs inversus

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.

Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview.

Reference: Access aggregated data from Orphanet at Orphadata.

Orphadata: Free access data from Orphanet. © INSERM 1999. Available on Data version April 2024

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Situs inversus totalis?

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Advocacy Organizations

Genetic Support Network of Victoria

The Genetic Support Network of Victoria is an organisation that supports people living with genetic, undiagnosed and rare conditions and those who support them including community and families, patient support organisations, health professionals and industry. Our vision is our community flourishing and living their best lives.

Clinical Trials

For a list of clinical trials in this disease area, please click here.