Splenic Trauma in a Patient with Het-Erotaxy: A Case Report and Brief Review of Laterality Defects

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Critical Care Medicine


Introduction: Heterotaxy broadly describes a spectrum of anatomic left-right lateralization irregularities. Among the varying subtypes of heterotaxy, there can be associated anomalies of multiple organ systems, namely the heart and spleen. These anomalies can have significant implications for the evaluation and management of trauma patients. We present one of the first reported cases of blunt abdominal trauma with isolated splenule laceration in a patient with heterotaxy syndrome with polysplenia. Description: A twelve year-old male presented to the emergency center with severe abdominal pain after diving for a soccer ball and landing onto his right side. Due to a positive FAST exam, a CT scan was obtained which showed abdominal situs-inversus with right-sided polysplenia, a normal cardiac configuration, multiple rib fractures, and a laceration of the caudal most splenule with moderate hemoperitoneum. He was admit-ted to the pediatric ICU for close hemodynamic monitoring, with involvement of both pediatric surgery and interventional radiology should operative or endovascular intervention be required. He ultimately remained stable and was managed non-operatively. Discussion: The incidence of anatomic laterality defects ranges from 1:6,000 to 1:20,000 in the general population. Patients may present with a spectrum of laterality malformations ranging from situs inversus totalis (complete mirror-image reversal of all thoracoabdominal organs and structures) to situs ambiguous (or heterotaxy) which involves incomplete laterality variations. The latter can manifest with a broad range of functional and anatomic abnormalities, including vascular abbarencies, which can affect multiple organ systems. However, heterotaxy is almost always associated with anomalies of the spleen, and the most common variations include asplenia, polysplenia, or a single right-sided spleen. It is imperative to accurately and adequately characterize a heterotaxy patient’s anatomic features, especially in a trauma setting, as this can have significant impli-cations on diagnosis and treatment. Furthermore, there are no published guidelines for the management of splenic trauma in the polysplenia population; therefore, we recommend a multi- disciplinary team approach.





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