ArticleSurgical and radiologic anatomy : SRA2021
Unusual long survival in a case of heterotaxy and polysplenia.
Article in Surgical and radiologic anatomy : SRA, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 4 papers.
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Who cites it
4 citing papers in PubMed.
- Abdominal aortic aneurysm in a patient with situs ambiguous and polyspleny (heterotaxy syndrome): a very rare occurrence in vascular surgery! Short literature review.Surgical and radiologic anatomy : SRA · 2026Review
- Characterization of ultrasound and postnatal pathology in fetuses with heterotaxy syndrome.Frontiers in cardiovascular medicine · 2023Article
- Cholangitis secondary to obstructive choledocholithiases in an elderly woman with heterotaxy syndrome with polysplenia: report of a case and brief review of the literature.Clinical journal of gastroenterology · 2022Review
- Splenic Infarct with Polysplenia Syndrome.Journal of the Belgian Society of Radiology · 2022Article
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Authors and funding
6 authors.
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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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