What is situs ambiguous?
Introduction. Heterotaxy (or situs ambiguus) is defined as the abnormal arrangement of thoracic or abdominal organs, or both, across the left-right axis (Video 90.1 ); it is different from complete situs inversus, which involves all organs.
Can you live with polysplenia?
Some affected individuals have only mild health problems related to the condition. At the other end of the spectrum, heterotaxy syndrome can be life-threatening in infancy or childhood, even with treatment [1]. The majority of patients with polysplenia syndrome die by age of 5 years.
What is the difference between Heterotaxy and situs inversus?
Heterotaxy syndrome is an arrangement of internal organs somewhere between situs solitus and situs inversus; this condition is also known as “situs ambiguus.” Unlike situs inversus, the abnormal arrangement of organs in heterotaxy syndrome often causes serious health problems.
How common is situs Ambiguus?
Heterotaxy syndrome with atrial isomerism occurs in 1 out of every 10,000 live births and is associated with approximately 3% of congenital heart disease cases. Additional estimation of incidence and prevalence of isomerism proves difficult due to failure to diagnose and underestimation of the disease by clinicians.
What does levocardia mean?
Strictly speaking, levocardia means that the cardiac apex is left sided. Isolated levocardias are those hearts that are left sided when situs inversus is present.
How common is polysplenia?
It is a rare congenital disease initially described by Helwig in 1929 [2], [3]. Since then, few cases were described in the literature with an incidence of 1/250,000 live births [4]. The exact cause of polysplenia has not been clearly defined.
How is situs inversus treated?
How is situs inversus treated? For many patients, situs inversus doesn’t cause any other symptoms. If a person with situs inversus does have complications like a heart defect, a doctor will treat the symptoms. Surgery to reverse the organs’ positioning is usually not recommended.
Can you live with situs inversus?
In the absence of congenital heart defects, individuals with situs inversus are homeostatically normal, and can live standard healthy lives, without any complications related to their medical condition.
What is situs inversus with levocardia?
Situs inversus has two main subtypes: dextrocardia and levocardia. A person is said to have dextrocardia if the point of the heart is on the right side. A person is said to have levocardia if the heart is on the left side, yet the other organs are flipped.
What is Situs Solitus with levocardia?
Situs solitus is the normal arrangement of abdominal organs with levocardia (Fig. 3) and is associated with a less than 1% incidence of congenital heart disease.
What causes Mesocardia?
Etiology: Cardiac location is affected by many factors including underlying cardiac malformation, abnormalities of mediastinal and thoracic structures (eg., Bronchogenic cysts, lung mass, tumors, esophageal atresia, diaphragmatic hernia, kyphoscoliosis, abnormalities of the diaphragm ecc.), Otherwise mesocardia …
Which is the correct description of situs ambiguus?
Situs ambiguus or situs ambiguous, also known as heterotaxy or heterotaxia, is a rare congenital defect in which the major visceral organs are distributed abnormally within the chest and abdomen. Heterotaxy in general refers to any defect of left-right laterality and arrangement of the visceral organs.
Is the mirror image symmetry normal in situs inversus?
In isolated situs inversus (occurring alone with no other abnormalities), there is a complete mirror image transposition of the thoracic (chest) and abdominal organs, and anterior-posterior (front-back) symmetry is normal. Many affected people have no associated health issues when the condition is isolated.
What causes malrotation of the kidneys on imaging 5?
It is asymptomatic and is virtually always discovered incidentally on imaging 5. The exact cause of malrotation of the kidneys is unclear, though it has been hypothesized that malrotation may occur if the ureteric bud inserts into an abnormal region of metanephric mesenchyme.
Where are the visceral organs located in situs ambiguus?
Situs ambiguus is a rare congenital defect in which the major visceral organs are distributed abnormally within the chest and abdomen.
https://www.youtube.com/watch?v=oJLsZ5-2yKE