Idiopathic hypereosinophilic syndrome complicated by computed tomography-negative cerebral infarction, strangulated intestinal obstruction, and hepatic portal venous gas: a case report of multi-system involvement

特发性嗜酸性粒细胞增多症并发CT阴性脑梗死、绞窄性肠梗阻和肝门静脉积气:一例多系统受累病例报告

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Abstract

BACKGROUND: Idiopathic hypereosinophilic syndrome (HES) is a rare disorder characterized by persistent eosinophilia, which can lead to multi-organ dysfunction. Despite its highly heterogeneous clinical manifestations, cardiac and neurological complications are the most significant contributors to patient disability and mortality. CASE DESCRIPTION: We report the case of a 66-year-old man who was emergently admitted to the hospital with the chief complaint of "sudden unsteady walking accompanied by deviation of the corner of the mouth for 15 hours". Laboratory tests revealed a significantly elevated eosinophil percentage (47.4%, count: 6.76×10(9)/L). Initial diagnostic evaluation, including head computed tomography (CT) combined with CT angiography and CT perfusion, showed no significant abnormalities. However, magnetic resonance imaging confirmed an acute infarct in the left basal ganglia-paraventricular area. To control hypereosinophilia, dexamethasone (10 mg/day) was initiated. Due to the patient's suboptimal therapeutic response, treatment was escalated to prednisolone (15 mg three times a day) for intensive management 2 days later. Following clinical stabilization, the regimen was transitioned back to dexamethasone for maintenance therapy (2.25 mg once daily at discharge), in accordance with standard steroid tapering protocols in HES. On the seventh day, the patient developed sudden signs of generalized peritonitis, and imaging revealed intestinal ischemia. By the tenth day, the classic triad of strangulated intestinal obstruction had emerged; that is, progressive abdominal pain, positive fecal occult blood (+++), and CT findings of a pneumoportogram with terminal ileal wall edema. Emergency exploratory laparotomy confirmed extensive intestinal necrosis, necessitating radical intestinal resection and jejunostomy. Following stepwise management, digestive tract reconstruction (jejunostomy reduction and end-to-side anastomosis of the small intestine and colon) was successfully performed. Current follow-up indicates a good prognosis. CONCLUSIONS: This case provides valuable clinical insights into the early identification and management of thrombotic critical conditions secondary to idiopathic HES. It also highlights multi-dimensional intervention strategies for short bowel syndrome, expanding post-intestinal resection management pathways.

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