Situs inversus totalis

Orpha code: 101063OMIM code:

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.

Disease data
Classification

Morphological anomaly

Synonyms
Complete situs inversus
Całkowite odwrócenie trzewi
Odwrócenie trzewi
Complete situs inversus viscerum
Situs inversus
ORPHA code
101063
OMIM code
-
ICD10 code
Q89.3
ICD11 code
LA82

No additional description.

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