Laterality Disorders Flashcards
Situs solitus
Standard anatomy
Situs inversus totalis
Global situs defect where everything is mirror image
Asymetry of unpaired organs depends on
How they move from the midline
Asymetry of paired organs depends on
Their morphology more than their position
Situs ambiguus
Defect of asymetry of an unpaired organ, resulting in at least one organ with a reversed L/R oritentation
e.g. dextrocardia
Isomerism
Asymetry of a paired organ e.g. left isomerism causing the formation of two morphologically left lungs, 2 left atrial appendages, polysplenism
40yo M with R isomerism giving 2 morphologically R atrial appendages and 2 morphologically R lungs (tri-lobed) with asplenia
Died of cardiac failure
Raman., (2003)
3 steps in the establishment of the L/R axis
Peeters and Devriendt, (2006)
Situs inversus totalis usually clinically silent but other laterality disorders usually have functional consequences, especially regarding the heart
Up to 80% of children with situs ambiguus present with congenital heart disease
Levin, (2005)
Affects transplantation
Transplantation of a normal heart into a 6yo boy with dextrocardia, demonstrated dextrocardia on post-op radiograph
Deuse et al., (2010)
Situs anomalies may present with confusing symptoms relating to pathology especially regarding the abdomen
Fulcher and Turner, (2002)
May be part of a larger sydrome
Kartegener triad: bronchiectasis, sinusitis and situs inversus
Salama et al., (2013)