What is situs ambiguous?
Situs ambiguous describes an abnormal position of the thoracoabdominal organs and can be categorized between two extremes; right-sided isomerism with asplenia and left sided isomerism with polysplenia.
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 are situs anomalies?
Situs anomalies are among the least common forms of congenital heart disease. This study defines situs and describes the variations of fetal situs anomalies. Situs refers to the arrangement of the viscera, atria, and vessels within the body.
How rare is situs inversus?
Situs inversus totalis has an incidence of 1 in 8,000 births. Situs inversus with levocardia is less common, with an incidence of 1 in 22,000 births. When situs cannot be determined, the patient has situs ambiguous or heterotaxy.
What is the difference between heterotaxy and situs inversus?
Situs inversus totalis involves the complete transposition of all visceral organs, while heterotaxy (situs ambiguus) is used to describe any deviation from the standard placement of organs in the abdomen.
What does polysplenia mean?
Polysplenia is a congenital disease manifested by multiple small accessory spleens. [ from HPO]
Can you live with heterotaxy syndrome?
Prognosis. Without corrective surgery, most children with heterotaxy syndrome and significant heart problems will not survive beyond the first year of life. Prognosis is difficult to determine for the group as a whole because of the varying degree of heart defects.
Is Heterotaxy the same as 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.
Can you survive 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.
Can you live with situs inversus?
Signs and symptoms 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.
Does situs inversus affect the brain?
One possibility is that asymmetries in the structure of the brain may influence the subsequent function of the organ. Yet individuals with anatomical reversals in brain structure, due to a condition called situs inversus totalis, still retain left-sided language processing [4].
What causes polysplenia?
The exact cause of polysplenia has not been defined. The probable hypotheses are embryonic (accelerated curvature of the embryonic body), genetic causes and teratogenic factors [7]. There is slight female preponderance and rare familial association has also been found [7].
Is heterotaxy syndrome fatal?
The severity of heterotaxy syndrome varies depending on the specific abnormalities involved. 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.