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儿童小肠梗阻的罕见病因:钙化性纤维瘤病例报告

A rare cause of small intestinal obstruction in children: a case report of calcifying fibrous tumor.

作者信息

Yang Zesheng, Xie Xiaoying, Wang Shicheng, Xu Weijun, Pei Guanghua, Wan Jun

机构信息

Department of Ultrasound, Tianjin Children's Hospital (Children's Hospital of Tianjin University), No. 238 Longyan Road, Beichen District, Tianjin, 300134, China.

Department of Emergency and Traumatic Surgery, Tianjin Children's Hospital(Children's Hospital of Tianjin University), Tianjin, China.

出版信息

BMC Pediatr. 2025 Sep 19;25(1):683. doi: 10.1186/s12887-025-06149-8.

DOI:10.1186/s12887-025-06149-8
PMID:40973955
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12447610/
Abstract

BACKGROUND

Calcifying fibrous tumor (CFT) is a rare benign mesenchymal tumor. Primary small intestinal CFT causing intestinal obstruction in children is exceptionally rare. Preoperative diagnosis remains challenging due to its nonspecific clinical manifestations. Ultrasound holds potential advantages in pediatric abdominal imaging, though further studies are needed to validate its utility for rare entities like CFT.

CASE PRESENTATION

A 3-year-old girl presented with progressive abdominal distension for 2 months, accompanied by occasional periumbilical restlessness (suggestive of mild discomfort). No bilious vomiting was noted, and bowel movements remained regular with normal consistency. High-frequency abdominal ultrasound revealed dilated small bowel loops, focal jejunal wall thickening (6 mm) with calcified foci and posterior acoustic shadowing, and punctate blood flow signals on color Doppler imaging, collectively indicating mechanical obstruction secondary to localized calcified wall thickening. CT confirmed small bowel dilation and high-density calcified lesions. Laparoscopy-confirmed obstructive jejunal stenosis further prompted mini-laparotomy with curative jejunal resection and primary anastomosis. Histopathology and immunohistochemistry confirmed CFT diagnosis. The patient recovered uneventfully and showed no recurrence at 2-month follow-up.

CONCLUSIONS

Small intestinal CFT is a rare cause of pediatric intestinal obstruction. High-frequency ultrasound demonstrated diagnostic capability in this specific case by identifying the pathognomonic triad of bowel wall thickening with calcifications and obstructive transition zone, facilitating timely preoperative assessment as a radiation-free initial imaging tool, with CT providing complementary confirmation of calcified lesions. Complete surgical resection achieved symptom resolution with no recurrence observed during available follow-up (2 months). This case supports considering small intestinal CFT in the differential diagnosis of children with unexplained chronic intestinal obstruction when the aforementioned pathognomonic triad (bowel wall thickening, calcifications, and obstructive transition zone) is observed, though larger pediatric cohort studies are required to validate this diagnostic approach.

摘要

背景

钙化性纤维瘤(CFT)是一种罕见的良性间叶组织肿瘤。原发性小肠CFT导致儿童肠梗阻极为罕见。由于其临床表现不具特异性,术前诊断仍具有挑战性。超声在儿科腹部成像中具有潜在优势,不过还需要进一步研究来验证其对CFT等罕见病变的应用价值。

病例介绍

一名3岁女孩出现进行性腹胀2个月,伴有偶尔的脐周不安(提示轻度不适)。未观察到胆汁性呕吐,排便规律且质地正常。高频腹部超声显示小肠肠袢扩张,空肠壁局限性增厚(6毫米)伴有钙化灶及后方声影,彩色多普勒成像显示点状血流信号,综合提示为局限性钙化性肠壁增厚继发的机械性梗阻。CT证实小肠扩张及高密度钙化性病变。腹腔镜检查确诊为梗阻性空肠狭窄,进一步促使行迷你剖腹术,进行根治性空肠切除及一期吻合术。组织病理学和免疫组化确诊为CFT。患者恢复顺利,随访2个月无复发。

结论

小肠CFT是儿童肠梗阻的罕见病因。高频超声通过识别肠壁增厚伴钙化及梗阻过渡区这一特征性三联征,在该特定病例中显示出诊断能力,有助于作为无辐射的初始成像工具进行及时的术前评估,CT则对钙化性病变提供补充性确认。完整的手术切除使症状得以缓解,在现有随访期(2个月)内未观察到复发。本病例支持在鉴别诊断不明原因慢性肠梗阻的儿童时,若观察到上述特征性三联征(肠壁增厚、钙化及梗阻过渡区),应考虑小肠CFT,不过还需要更大规模的儿科队列研究来验证这种诊断方法。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9778/12447610/754348f8297a/12887_2025_6149_Fig9_HTML.jpg
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https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9778/12447610/754348f8297a/12887_2025_6149_Fig9_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9778/12447610/143dfecce26b/12887_2025_6149_Fig1_HTML.jpg
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https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9778/12447610/6396fd954e41/12887_2025_6149_Fig5_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9778/12447610/21d0842733b2/12887_2025_6149_Fig6_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9778/12447610/2c82a912bbab/12887_2025_6149_Fig7_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9778/12447610/debc16901506/12887_2025_6149_Fig8_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9778/12447610/754348f8297a/12887_2025_6149_Fig9_HTML.jpg

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