Hoang Thomas, Karavelic Adnan, Sunil Aswathy, Murray Timothy, Telford Jennifer, Clement Elizabeth, Gador Anthony
Department of Medicine, Faculty of Medicine, University of British Columbia, Vancouver, British Columbia, Canada.
Department of Pathology and Laboratory Medicine, Faculty of Medicine, University of British Columbia, Vancouver, British Columbia, Canada.
ACG Case Rep J. 2025 Mar 19;12(3):e01637. doi: 10.14309/crj.0000000000001637. eCollection 2025 Mar.
Sclerosing mesenteritis is a rare spectrum of disease that results in chronic inflammation and fibrosis of the abdominal mesentery. Its heterogeneous presentation, nondiagnostic imaging, and pathology findings make diagnosis challenging. In this study, we present the first reported case of hilar sclerosing mesenteritis resulting in chylous ascites, gastric outlet obstruction, and duodenal perforation. Diagnosis was made after extensive investigations including computed tomography imaging, magnetic resonance cholangiopancreatography, positron emission tomography scan, bidirectional endoscopy, endoscopic ultrasound, and diagnostic laparoscopy. While initially mistaken for hilar cholangiocarcinoma, the patient has significant clinical improvement with corticosteroids and now remains in symptomatic and radiographic remission on low-dose prednisone and tamoxifen.
硬化性肠系膜炎是一种罕见的疾病谱，可导致腹部肠系膜的慢性炎症和纤维化。其表现多样、影像学检查无特异性及病理结果不典型，使得诊断具有挑战性。在本研究中，我们报告了首例导致乳糜性腹水、胃出口梗阻和十二指肠穿孔的肝门部硬化性肠系膜炎病例。通过包括计算机断层扫描成像、磁共振胰胆管造影、正电子发射断层扫描、双向内镜检查、内镜超声和诊断性腹腔镜检查在内的广泛检查后作出诊断。该患者最初被误诊为肝门胆管癌，使用皮质类固醇治疗后临床症状有显著改善，目前在低剂量泼尼松和他莫昔芬治疗下症状缓解，影像学检查结果也保持缓解。
