CTICasesKartagener Syndrome with Heterotaxy, Polysplenia, and Azygos Continuation
Imaging findings
Patient presents with Kartagener syndrome, exhibiting situs inversus totalis, panbronchiectasis, and widespread bronchiolitis with small airways shadows. Abdominal imaging reveals heterotaxy with polysplenia and azygos continuation of an interrupted abdominal inferior vena cava. The pulmonary airway anatomy also appears to be inverted and symmetrical.
Key takeaways
Kartagener syndrome, a subset of primary ciliary dyskinesia, can manifest not only with situs inversus totalis but also with heterotaxy in about 12% of cases, leading to anomalies like polysplenia and azygos continuation of an interrupted IVC. This highlights that organ arrangement may not be a uniform mirror image in these patients.
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