Abstract

A 38-year-old man with Ehlers-Danlos syndrome was examined for abdominal pain and fever. An In-111 leukocyte scan performed to detect the possible source of occult sepsis revealed intense activity above the liver. The presence of the intrathoracic spleen was confirmed on a Tc-99m sulfur colloid scan. Computed tomography showed herniation of the stomach, colon, and spleen through a large diaphragmatic defect into the chest. The mechanism of a wandering spleen in this patient is most likely ligamentous laxity caused by Ehlers-Danlos syndrome, which also accounts for the hiatus hernia.

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