The Diagnosis of Cronkhite-Canada Syndrome with CT Enterography: A Clinical Case Analysis
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摘要: Cronkhite-Canada综合征（CCS）是一组以胃肠道弥漫性息肉和外胚层变化为特征的综合征，其临床表现主要为慢性腹泻和吸收不良，由于发病率罕见，早期发现和诊断对医生来说是一个挑战。本文报告1例CCS病例，58岁男性，临床表现为慢性水样腹泻、便血、体重减轻和皮肤变化，包括指甲营养不良和色素沉着；实验室检查结果提示贫血和低蛋白血症；CT小肠造影检查发现全消化道黏膜息肉样增生，高度怀疑CCS，随后行内镜检查证实了该诊断。患者经治疗后，症状明显好转，在近1年的随访中，CT小肠造影和内镜检查均提示明显缓解。本文对该疾病进行文献综述，旨在总结其CT小肠造影表现，以提高临床医生对本病的认识，为CCS的早期诊断提供一定的思路。
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关键词:
- CT小肠造影 /
- 息肉-色素沉着-脱发-指(趾)甲营养不良综合征 /
- 诊断
Abstract: Cronkhite-Canada syndrome (CCS) is a rare cause of chronic diarrhea and malabsorption where patients develop multiple polyps throughout the gastrointestinal (GI) tract, accompanied by ectodermal changes. Due to its rarity, its early detection and diagnosis can be challenging for physicians. This case report described a 58-year-old male patient with CCS who presented with chronic watery diarrhea, hematochezia, weight loss, and skin changes including nail dystrophy and hyperpigmen-tation. Laboratory results showed anemia and hypoalbuminemia. He underwent CT enterography (CTE) which identified diffuse edematous polyposis in the GI tract. The CTE results were highly suspicious of CCS and a subsequent endoscopic examination confirmed the diagnosis. The patient received supportive treatment which improved his symptoms. Based on CTE and endoscopy at 1-year follow-up, the patient was deemed to be in remission. We included a literature review of CCS. The case report aimed to improve the understanding of CCS and explored the key CTE features relevant to its early diagnosis.-
Key words:
- CT enterography /
- Cronkhite-Canada syndrome /
- diagnosis
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图 1 CCS患者脚趾和手指图像
显示指（趾）甲萎缩，甲板从甲床远端虫蚀样脱落，指间关节色素沉着。
Figure 1. Images of toes and fingers of the patient with CCS
图 2 CCS患者CTE图像
（a）～（d）为静脉期横断面增强图像，依次显示胃体、十二指肠球降段、末端回肠、乙状结肠和直肠黏膜多发息肉样增生，呈弥漫性分布，胃体和末端回肠呈现息肉样黏膜皱襞；（e）和（f）为冠状面重建图像，更加清晰显示息肉分布范围、形态，以末端回肠黏膜息肉样增生强化更明显（白箭）。
Figure 2. CT enterographic images of the patient with CCS
图 3 CCS患者胃镜和经肛双气囊小肠镜图像
（a）～（c）依次为胃体、末端回肠和升结肠，显示黏膜息肉样增生，表面腺体增生间裂，头端充血水肿。
Figure 3. Gastroscopic and transanal double balloon enteroscopic images of the patient with CCS
图 4 CCS患者治疗后随访CTE图像
（a）～（d）为静脉期横断面增强图像，依次显示经过治疗后，胃体、十二指肠球降段、末端回肠、乙状结肠和直肠黏膜多发息肉样增生明显好转，息肉变稀疏甚至消失，部分结肠仍可见黏膜息肉样增生；（e）和（f）为冠状面重建图像，更加清晰显示整个病变范围。
Figure 4. CTE images after treatment in patient with CCS
图 5 CCS患者治疗后病理图像
（a）和（b）分别为胃体和升结肠（HE 20×），显示腺上皮无明显异型性，间质一定量淋巴细胞、浆细胞和嗜酸性粒细胞浸润。
Figure 5. Pathological images after treatment in patient with CCS
表 1 CCS CTE表现
Table 1. CT enterographic features of CCS
息肉 CTE特点 部位 胃底、胃体、胃角、胃窦、十二指肠球降段、空肠、回肠下段、末端回肠、回盲部、大肠 大小 大小不等，小者4 mm，大者2 cm，小肠中以末端回肠为著，大肠中以右半结肠为著 形态 椭圆形、指状，基底宽，蒂不明显 分布 弥漫性分布、地毯式覆盖 边界（与黏膜分界） 与黏膜分界不清，形成巨大息肉样黏膜皱襞 供血动脉 未见明显肠系膜供血动脉与其相连 强化方式 明显强化，尤以末端回肠强化更明显 并发症 末端回肠有套入右半结肠趋势，其他部位未见明显套叠；未见明显癌变征象；其他脏器未
见异常肠管周围及肠系膜淋巴结 肠管周围脂肪间隙清晰，未见肠系膜淋巴结增大 
下载: 导出CSV
表 2 CCS患者实验室检查结果前后对照
Table 2. Laboratory results of the patient with CCS before and after treatment
实验室检查项目 第一次入院 随访 血红蛋白/（g/L） 128 141 总蛋白/（g/L） 45 65 白蛋白/（g/L） 29 39
下载: 导出CSV
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