Abstract
Heterotaxy syndrome with polysplenia is an extremely rare congenital disorder caused by a disruption in the embryonic development that results in an abnormal arrangement of the abdominal and thoracic organs. We present the case of a 59-year-old female patient with invasive ductal carcinoma of the right breast (luminal A type) and CT findings of heterotaxy syndrome with polysplenia. The most remarkable anomalies identified were a left inferior vena cava draining into the hemiazygos vein, absent inferior vena cava at the thoracic level, and hepatic veins directly draining into the right atrium. Moreover, an atrial septal defect was identified, explaining the pulmonary hypertension of unknown cause previously detected in the patient. The relevance of this case lies in the unusual anatomical abnormalities found and the large patient survival, having in to account the great rate of heterotaxy syndrome mortality in the first years of life.
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Abbreviations
- Body-CT:
-
Body-Computed Tomography
- IVC:
-
Inferior vena cava.
- ASD:
-
Atrial septal defect
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This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors. Internal funding from CTS-107 Group was used.
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KD, VC and JP, carried out the study concepts, data acquisition, data analysis and manuscript editing; MCP, IM and CM were dedicated to the data analysis and manuscript preparation. All authors have read and approved this article.
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All protocols and guidelines established by the Medical Oncology Service of Virgen de las Nieves Hospital (Granada) in the treatment of patient data have been followed. The research was conducted ethically in accordance with The Code of Ethics of the World Medical Association (Declaration of Helsinki).
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Doello, K., Conde, V., Perez, M.C. et al. Unusual long survival in a case of heterotaxy and polysplenia. Surg Radiol Anat 43, 607–611 (2021). https://doi.org/10.1007/s00276-020-02586-5
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DOI: https://doi.org/10.1007/s00276-020-02586-5