Dréau Axel, Barthomeuf Clémence, Balesdent Marion, Fumery Mathurin, Sabbagh Charles, Chatelain Denis
Service d'anatomopathologie, CHU Amiens Picardie, site Nord (hôpital Nord), 1, place Victor-Pauchet, 80054 Amiens cedex 1, France.
Service d'anatomopathologie, CHU Amiens Picardie, site Nord (hôpital Nord), 1, place Victor-Pauchet, 80054 Amiens cedex 1, France.
Ann Pathol. 2024 Feb;44(1):65-68. doi: 10.1016/j.annpat.2023.07.002. Epub 2023 Aug 25.
Enteritis cystica profunda is a rare and benign disease defined as the invagination of the intestinal epithelium into the submucosa and more profound layers of intestinal wall leading to the formation of mucin-filled cystic spaces. We reported the case of a 45-year-old female, suffering from a Crohn's disease, with a Koenig's syndrome, diarrhea, abdominal pain and weight loss. The colonoscopy and the abdominopelvic scan showed a terminal ileal stenosis, with parietal calcifications. A surgical ileocecal resection was decided. Gross examination of the ileocecal resection showed a thickening of the ileal wall, with many mucin-filled cysts measuring 1mm to 2cm, with some calcifications. The ileal mucosa was ulcerated, and showed a stenotic sector extending over 3cm. Histological examination showed acute ulcerated ileitis lesions, with chronic ileitis lesions and stenosis, compatible with the known diagnosis of Crohn's disease. There were also many cysts into the ileal wall. They were lined with a regular ileal epithelium. The cysts contained mucus, with some calcifications. Some cysts were ruptured, with extravasation of mucus within the wall. Cystica profunda can be found anywhere along the digestive tract. The physiopathology is not yet well understood, but it seems to be favored by chronic aggression of the intestinal wall. This pathology most often coexists with Crohn's disease. The main differential diagnosis is mucinous adenocarcinoma. Cystica profunda does not require any specific treatment.
深部囊性肠炎是一种罕见的良性疾病，定义为肠上皮向黏膜下层及肠壁更深层内陷，导致形成充满黏液的囊性间隙。我们报告了一例45岁女性病例，该患者患有克罗恩病，伴有克尼格综合征、腹泻、腹痛及体重减轻。结肠镜检查和腹部盆腔扫描显示回肠末端狭窄，伴有肠壁钙化。遂决定行外科回盲部切除术。回盲部切除标本大体检查显示回肠壁增厚，有许多大小为1毫米至2厘米的充满黏液的囊肿，伴有一些钙化。回肠黏膜溃疡，可见一个超过3厘米的狭窄区域。组织学检查显示急性溃疡性回肠炎病变、慢性回肠炎病变及狭窄，与已知的克罗恩病诊断相符。回肠壁内还有许多囊肿。囊肿内衬规则的回肠上皮。囊肿内含有黏液，伴有一些钙化。一些囊肿破裂，黏液溢入肠壁内。深部囊性肠炎可发生于消化道的任何部位。其病理生理学尚未完全明确，但似乎与肠壁的慢性侵袭有关。这种病理情况常与克罗恩病共存。主要鉴别诊断为黏液腺癌。深部囊性肠炎无需特殊治疗。
