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一例罕见的由孤立性肠道肿瘤引起的新生儿结肠梗阻病例。

A rare case of neonatal colonic obstruction caused by a solitary intestinal tumor.

作者信息

Sugai Yu, Hirayama Yutaka, Iinuma Yasushi, Nakaya Kengo, Aikou Takato, Taki Shotaro, Hashidate Hideki, Kinoshita Yoshiaki

机构信息

Department of Pediatric Surgery, Niigata-City General Hospital, 463-7 Shumoku, Chuo-ku, Niigata City, Niigata, 950-1197, Japan.

Department of Pediatric Surgery, Nagaoka Red Cross Hospital, 2-297-1 Senshu, Nagaoka City, Niigata, 940-2085, Japan.

出版信息

Surg Case Rep. 2021 Jan 19;7(1):26. doi: 10.1186/s40792-021-01107-9.

DOI:10.1186/s40792-021-01107-9
PMID:33464419
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7815852/
Abstract

BACKGROUND

Intestinal obstruction caused by a tumor is very rare in newborns, and the preoperative diagnosis is difficult. We herein report a rare case of neonatal colonic obstruction due to solitary intestinal myofibroma with characteristic findings on gastrografin enema and the surgical strategy.

CASE PRESENTATION

A 4-day-old female infant presented to our neonatal intensive-care unit with abdominal distention and bilious vomiting after feeding. A gastrografin enema showed that the transverse colon near the hepatic flexure was not delineated at the oral side. When pressure was applied, a small amount of contrast material moved into the mouth in the form of threads. Microcolon was not observed, and stenosis of the transverse colon was found 9 cm from the Bauhin valve. Partial resection and end-to-end anastomosis were performed. A pathological examination of the resected specimen suggested gastrointestinal stromal tumor (GIST). After obtaining a second opinion, the histology and immunohistological markers were deemed characteristic of infantile myofibroma.

CONCLUSION

If string sign and a napkin ring appearance are found in a case of neonatal intestinal obstruction, surgery should be performed with a tumor in mind. In cases of neonatal intestinal obstruction caused by a tumor, the lesion should be resected with a sufficient surgical margin before the pathological examination.

摘要

背景

肿瘤引起的新生儿肠梗阻非常罕见,术前诊断困难。我们在此报告一例罕见的新生儿结肠梗阻病例,该病例由孤立性肠肌纤维瘤引起,在泛影葡胺灌肠检查中有特征性表现及手术策略。

病例介绍

一名4日龄女婴因喂养后腹胀和胆汁性呕吐入住我们的新生儿重症监护病房。泛影葡胺灌肠检查显示肝曲附近的横结肠在口侧未显影。施加压力时,少量造影剂呈线状进入口腔。未观察到小结肠,在距鲍氏瓣9厘米处发现横结肠狭窄。进行了部分切除和端端吻合术。对切除标本的病理检查提示为胃肠道间质瘤(GIST)。在获得第二种意见后,组织学和免疫组织化学标记物被认为具有婴儿肌纤维瘤的特征。

结论

如果在新生儿肠梗阻病例中发现线状征和餐巾环外观,应考虑肿瘤进行手术。在肿瘤引起的新生儿肠梗阻病例中,应在病理检查前切除病变组织,并保留足够的手术切缘。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1e53/7815852/f900668e2b12/40792_2021_1107_Fig3_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1e53/7815852/78efee9b003d/40792_2021_1107_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1e53/7815852/bed5944ab690/40792_2021_1107_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1e53/7815852/f900668e2b12/40792_2021_1107_Fig3_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1e53/7815852/78efee9b003d/40792_2021_1107_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1e53/7815852/bed5944ab690/40792_2021_1107_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1e53/7815852/f900668e2b12/40792_2021_1107_Fig3_HTML.jpg

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本文引用的文献

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A Solitary Intestinal Myofibroma: A Rare Cause of Neonatal Anemia.孤立性肠道肌纤维瘤:新生儿贫血的罕见病因
Case Rep Oncol. 2017 Oct 17;10(3):890-896. doi: 10.1159/000481305. eCollection 2017 Sep-Dec.
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Recurrent Somatic PDGFRB Mutations in Sporadic Infantile/Solitary Adult Myofibromas But Not in Angioleiomyomas and Myopericytomas.散发性婴儿/孤立性成人肌纤维瘤中存在复发性体细胞PDGFRB突变,而血管平滑肌瘤和肌周细胞瘤中则不存在。
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