Home > D. General pathology > Genetic and developmental anomalies > situs inversus
situs inversus
Wednesday 29 June 2005
Definition: Situs inversus totalis is a defect in global situs orientation. It results in a complete mirror image of situs solitus. In this condition, occurring in one out of 6000–8000 newborns, all organs maintain their normal relative position with respect to each other.
Synopsis
cardiovascular anomalies
- dextrocardia with right aorta
- cardiac malformations
- vascular malformations
pulmonary malformations
complete visceral heterotaxy (mirror image)
- trilobar left lung
- bilobar right lung
- left liver
- inversed stomach
- right spleen
Associations
renal malformations
- renal agenesis
- non-obstructive renal dysplasia
- bilateral renal dysplasia (12605452)
- multicytic kidneys (polycyctic kidneys)
- renal ectopia
- horseshoe kidney,
- renal hypoplasia (15877680)
renal cell carcinoma
situs inversus totalis with reno-pancreatic dysplasia (10678653, 9934983)
congenital pancreatic fibrosis (pancreatic dysplasia)
congenital hepatic fibrosis
short rib polydactyly syndromes (1481807, 1519646)
- short rib polydactyly syndrome type 3 (1481807)
NB: Children with situs inversus totalis have a much lower risk of cardiovascular malformations (3–9%) than children with situs ambiguus (over 80%). The risk of cardiovascular malformations in situs inversus totalis (3–9%) is higher than in the normal situation, situs solitus (0.6%).
Case records
[Case 10041->10041]: Situs inversus totalis with sirenomelia
See also
laterality
heterotaxy (situs anomalies, laterality disorders)
- situs ambiguus
- situs inversus totalis
- isomerisms
- left isomerism
- right isomerism
Associations
Ellis-van Creveld syndrome (#5770269 #)
ciliopathies
References
Peeters H, Devriendt K. Human laterality disorders. Eur J Med Genet. 2006 Sep-Oct;49(5):349-62. Epub 2006 Jan 3. PMID: 16461029
Aylsworth AS. Clinical aspects of defects in the determination of laterality. Am J Med Genet. 2001 Jul 15;101(4):345-55. PMID: 11471158
