Department of Surgery, Nara Medical University, 840 Shijo-cho, Kashihara, Nara, 634-8522, Japan.
Division of Endoscopy, Nara Medical University Hospital, 840 Shijo-cho, Kashihara, Nara, 634-8522, Japan.
Clin J Gastroenterol. 2022 Oct;15(5):934-940. doi: 10.1007/s12328-022-01678-6. Epub 2022 Jul 21.
Cronkhite-Canada syndrome (CCS) is a non-hereditary disorder characterized by non-neoplastic gastrointestinal polyposis and ectodermal changes. While corticosteroids are considered effective, some cases are refractory. A 48-year-old woman presented with diarrhea, anorexia, and epigastralgia lasting for 3 months. She suffered from alopecia and nail dystrophy. Gastrointestinal endoscopy with histological examination confirmed non-neoplastic polyposis from the stomach to the rectum, confirming the diagnosis of CCS. Linked color imaging (LCI) with magnified endoscopy revealed a ribbon-like proliferation of capillaries surrounding the pits in the colonic mucosa. Histologically, the polyps had dilated glands, edematous stroma with inflammatory cell infiltrates and increased capillaries just beneath the epithelium. Immunohistochemical examination confirmed the expression of vascular endothelial growth factor (VEGF), mainly in the superficial epithelial and crypt cells. Steroid therapy was ineffective, and concomitant infliximab therapy provided symptomatic relief. Although symptoms rapidly improved with combination therapy, capillary hyperplasia and slight inflammation persisted in the colon mucosa after polyp resolution. Withdrawal of steroid treatment resulted in flare-ups of symptoms and polyps. Repeated magnified observations at LCI during post-relapse retreatment clearly captured the resolution process of both neovascularization and inflammation. Once the capillary hyperplasia and inflammation subsided, the steroid could be tapered off without relapse. To our knowledge, this is the first report describing the involvement of VEGF-induced angiogenesis and LCI findings in CCS; LCI observations are useful not only in the active phase of CCS, but also in determining subtle capillary hyperplasia and residual inflammation in remission, which may be an indicator of continued treatment.
Cronkhite-Canada 综合征（CCS）是一种非遗传性疾病，其特征为非肿瘤性胃肠道息肉病和外胚层改变。皮质类固醇被认为是有效的，但有些病例是难治性的。一位 48 岁女性因腹泻、食欲不振和上腹痛持续 3 个月就诊。她患有脱发和指甲营养不良。胃肠镜检查和组织学检查证实从胃到直肠的非肿瘤性息肉，确诊为 CCS。放大内镜下的链接颜色成像（LCI）显示出围绕结肠黏膜陷窝的毛细血管呈带状增生。组织学上，息肉有扩张的腺体、水肿的基质，伴有炎症细胞浸润和上皮下毛细血管增多。免疫组织化学检查证实血管内皮生长因子（VEGF）的表达，主要在浅表上皮和隐窝细胞中。类固醇治疗无效，同时使用英夫利昔单抗治疗提供了症状缓解。虽然联合治疗后症状迅速改善，但在息肉消退后，结肠黏膜中的毛细血管增生和轻微炎症仍持续存在。停用类固醇治疗导致症状和息肉复发。在复发后再次治疗期间，通过 LCI 进行放大观察清楚地捕捉到新生血管形成和炎症的消退过程。一旦毛细血管增生和炎症消退，就可以逐渐减少类固醇用量而不会复发。据我们所知，这是第一份描述 VEGF 诱导的血管生成和 LCI 发现参与 CCS 的报告；LCI 观察不仅在 CCS 的活动期有用，而且在确定缓解期的细微毛细血管增生和残留炎症方面也有用，这可能是继续治疗的指标。
