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<p>Heterotaxy syndrome (situs ambiguus) is a rare congenital syndrome with an incidence of approximately 0.0001%, characterized by abnormal positioning of thoracoabdominal organs that does not fully conform to the normal anatomical arrangement (situs solitus) or mirror-image configuration (situs inversus) and includes a wide spectrum of anatomical variations. These cases are mostly diagnosed during examinations, radiological evaluations, or surgical procedures performed for other reasons. In this case report, a 34-year-old patient who presented to the emergency department with abdominal pain and was diagnosed with acute appendicitis through diagnostic workup, and who was incidentally found to have heterotaxy with polysplenia, is presented. Imaging demonstrated increased appendiceal diameter and surrounding fat stranding; the liver was centrally located extending to the left, the gallbladder was left-sided, and the stomach and spleen were right-sided, with an additional spleen on the right. The patient underwent laparoscopic appendectomy, and the perioperative course was uneventful. This case demonstrates that heterotaxy syndrome may remain clinically silent and can be incidentally detected during evaluation for acute abdominal pathologies. During surgical intervention, especially in patients scheduled for laparoscopic procedures, anatomical variations of the organs must be considered.</p>

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Incidental Diagnosis of Abdominal Heterotaxy with Polysplenia in an Adult Presenting with Acute Appendicitis

  • 期刊论文

  • 发表日期：

    2026年3月30日

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  • Muhammed Furkan Arslan,
  • Mehmet Saban Korkmaz,
  • Murat Demir,
  • Huseyin Kilavuz

摘要

Heterotaxy syndrome (situs ambiguus) is a rare congenital syndrome with an incidence of approximately 0.0001%, characterized by abnormal positioning of thoracoabdominal organs that does not fully conform to the normal anatomical arrangement (situs solitus) or mirror-image configuration (situs inversus) and includes a wide spectrum of anatomical variations. These cases are mostly diagnosed during examinations, radiological evaluations, or surgical procedures performed for other reasons. In this case report, a 34-year-old patient who presented to the emergency department with abdominal pain and was diagnosed with acute appendicitis through diagnostic workup, and who was incidentally found to have heterotaxy with polysplenia, is presented. Imaging demonstrated increased appendiceal diameter and surrounding fat stranding; the liver was centrally located extending to the left, the gallbladder was left-sided, and the stomach and spleen were right-sided, with an additional spleen on the right. The patient underwent laparoscopic appendectomy, and the perioperative course was uneventful. This case demonstrates that heterotaxy syndrome may remain clinically silent and can be incidentally detected during evaluation for acute abdominal pathologies. During surgical intervention, especially in patients scheduled for laparoscopic procedures, anatomical variations of the organs must be considered.