Situs ambiguous anomaly during laparoscopic cholecystectomy in an adult female

Anatomy, embryology, and imaging of situs ambiguous with polysplenia and left IVC

“…On the other hand, situs ambiguous or heterotaxia is defined as a clinical situation in which there is an alteration in the usual left-right distribution of the organs without a fixed or specific pattern. It is a very rare situation with an associated incidence of 0.0001% [9] . It can be divided into 2 categories according to the presence or absence of spleen: Situs ambiguous with asplenia or right atrial isomerism (RAI); absence of spleen.…”

“…Patients affected with RAI syndrome usually have cyanosis, since almost all the supply to the pulmonary circulation depends on the ductus arteriosus and drainage to the pulmonary circulation is usually severely compromised; the mortality rate during the first year of life in these patients is 80%-90% [15] . On the other hand, those affected by LAI syndrome, in whom the alteration of the related structures of the pulmonary circulation is not so severe, most newborns will not have cyanosis associated with lower mortality rates during the pediatric phase [9] .…”