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Background
Situs solitus is defined as the normal position of organs. Situs ambiguous is a rare congenital defect where most organs are heterogeneously distributed in the chest and abdomen. In addition to the left/right axis inversion with partial or complete situs inversus, laterality can be left (left isomerism) or right (right isomerism). Isomerism is defined as a defect in the asymmetry of paired organs. 1 The left-right axis is defined in the early stages of embryogenesis, 2 - 5 approximately around day 15. Isomerisms can be isolated, but can also be part of various syndromes, the best known of which-for its respiratory disorders-is immotile cilia, that includes Kartagener syndrome. This syndrome shows a recessive inheritance and is associated with situs inversus, heart defects, bronchiectasis, sinusitis, and otitis media as well as sterility in males. The main disorder includes structural anomalies of the cilia of the respiratory mucosa. 6
Abnormal intestinal rotations are commonly found in patients with heterotaxy syndrome. 1 , 7 The pancreas, duodenum, stomach, liver and spleen are involved in some form of rotation in the early stages of embryonic development, generally between weeks 5 and 6 of pregnancy. 8 It is likely that the effect of a nosological factor during this period can cause incomplete or reverse rotation in some organs. Therefore, heterotaxy can also cause polysplenia or asplenia associated with congenital heart defects. The heterotaxy syndrome associated with asplenia has a worse prognosis, because it is commonly associated with significant heart anomalies. 9 , 10 However, the advances made in recent years in heart surgery and the early detection of heart disorders by prenatal ultrasonography have prolonged survival in patients with heterotaxy. 7 , 11 It has also been shown that some maternal diseases, such as diabetes mellitus, increase the risk of cardiac malformations and heterotaxy in newborns. 12 Situs ambiguous can be considered as a symptom complex rather than a diagnosis. The pathophysiology of syringomyelia changes with different types. In the cases associated with the malformation of the cranium cervical, the cerebrospinal fluid cannot flow freely from the ventricle IV towards the subarachnoid space. The most common reason is the decrease of the cerebellar tonsils below the foramen magnum in the Chiari.
We report the case of a child who,...