• Yuanjun Liu, Yi Dai, Fan Xiao, Shuang Liu, Yakun Wu, and Enrong Ran.
• Department of Hepatobiliary Surgery, Suining Central Hospital, Suining, Sichuan Province, China.
• Medicine (Baltimore). 2022 Aug 5; 101 (31): e29636e29636.

RationaleUpper gastrointestinal hemorrhage (UGIH) is defined as hemorrhage originating from the gastrointestinal tract proximal to the ligament of Treitz. The causes of UGIH include esophagitis, gastritis, peptic ulcers, Mallory-Weiss syndrome, and cancer. However, a rare cause of UGIH, such as an accessory spleen, may lead to serious complications if left untreated and can sometimes be very difficult to diagnose preoperatively.Patient ConcernsAn 18-year-old man was admitted to the Department of Gastroenterology of our hospital due to "repeated black stool for 2 months with aggravation, accompanied by hematemesis for 9 days." He denied any history of hepatitis, trauma, or surgery.DiagnosisLaboratory evaluation revealed severe anemia (hemoglobin, 6.4 g/dL). Computed tomography revealed a mass measuring 127 mm in its largest dimension, located in the upper left abdomen, with varicose veins in the gastric fundus. Moreover, distended blue-purple tortuous veins were observed by gastroscopy in the gastric fundus. We believed the mass was likely an abnormally proliferating accessory spleen; however, the causes of severe anemia and gastrointestinal hemorrhage were unknown.InterventionsAfter discussion in a multidisciplinary conference, the mass was completely resected laparoscopically, and the subserosal veins in the gastric fundus were sutured using absorbable threads.OutcomesAfter the surgery, the patient recovered uneventfully without any complications. Clinicopathological examination showed that the mass was chronic congestive splenomegaly. Gastrointestinal hemorrhage secondary to an abnormally proliferating accessory spleen was confirmed as the diagnosis. Laboratory evaluation revealed hemoglobin at 12.1 g/dL 2 months after surgery. At the 12-month follow-up, the patient showed no recurrence of gastrointestinal hemorrhage.LessonsUGIH caused by accessory spleen is extremely rare. This entity should be considered in differential diagnosis of gastrointestinal hemorrhage. Surgical intervention is necessary for timely diagnosis and treatment in case of gastrointestinal hemorrhage in critical clinical situations.Copyright © 2022 the Author(s). Published by Wolters Kluwer Health, Inc.

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