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<p>Polysplenia syndrome is a rare congenital condition within the heterotaxy spectrum, characterized by multiple splenic nodules and abnormal visceral arrangement. Its presentation varies widely and may include cardiovascular, gastrointestinal, and vascular anomalies. We report an asymptomatic child incidentally diagnosed with polysplenia associated with a cleft palate and a retroaortic right renal vein, an unusual combination not commonly described. Imaging revealed multiple spleens, left-sided interrupted inferior vena cava with azygos continuation, and a retroaortic renal vein without hemodynamic compromise for the first time in literature to our knowledge. This case emphasizes the importance of thorough systemic evaluation in patients with craniofacial anomalies, as early recognition of associated malformations can guide appropriate management and optimize long-term outcomes for affected children worldwide and the importance of radiologic evaluation.</p>

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Silent complexity: a rare combination of polysplenia, cleft palate, retroaortic renal vein, and dorsal pancreatic agenesis

  • 期刊论文

  • 发表日期：

    2026年3月23日

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  • Cenk Parlatan,
  • Senay Demir Kekec,
  • Elif Habibe Aktekin

摘要

Polysplenia syndrome is a rare congenital condition within the heterotaxy spectrum, characterized by multiple splenic nodules and abnormal visceral arrangement. Its presentation varies widely and may include cardiovascular, gastrointestinal, and vascular anomalies. We report an asymptomatic child incidentally diagnosed with polysplenia associated with a cleft palate and a retroaortic right renal vein, an unusual combination not commonly described. Imaging revealed multiple spleens, left-sided interrupted inferior vena cava with azygos continuation, and a retroaortic renal vein without hemodynamic compromise for the first time in literature to our knowledge. This case emphasizes the importance of thorough systemic evaluation in patients with craniofacial anomalies, as early recognition of associated malformations can guide appropriate management and optimize long-term outcomes for affected children worldwide and the importance of radiologic evaluation.