孤立性弥漫性肠道神经节瘤病表现为乙状结肠冗长：一例报告
Isolated diffuse intestinal ganglioneuromatosis presented as a redundant sigmoid colon: a case report.
作者信息
Al Samaan Fadi, Alhimyar Maryam, Alhalbouni Mohammad, Aldirani Alaa, Assad Lina
机构信息
Departments of Pathology.
General Surgery, Damascus Hospital.
出版信息
Ann Med Surg (Lond). 2023 Feb 7;85(2):219-224. doi: 10.1097/MS9.0000000000000195. eCollection 2023 Feb.
UNLABELLED
Diffuse intestinal ganglioneuromtosis is a benign tumor of the enteric nervous system, that almost always occurs in children with systemic syndromes. Whereas isolated cases in adults are exceedingly rare.
CASE PRESENTATION
A 38-year-old man presented with refractory chronic constipation. An abdominal computed tomography scan revealed a redundant sigmoid colon, then he underwent a sigmoid colectomy. Histopathologic examination showed diffuse ganglioneuromatosis. However, the patient was in good health 18 months after surgery.
CLINICAL DISCUSSION
Intestinal ganglioneuromas commonly occur in children with systemic syndromes such as multiple endocrine neoplasia type 2B and neurofibromatosis type 1. The most frequent symptoms are abdominal discomfort, constipation, ileus, weight loss, appendicitis, and obstruction in more severe cases. surgical resection is the standard management in diffuse ganglioneuromatosis.
CONCLUSION
Although diffuse ganglioneuromatosis is uncommon, it should be considered in patients with refractory constipation.
未标注
弥漫性肠道神经节瘤病是一种肠道神经系统的良性肿瘤，几乎总是发生在患有全身性综合征的儿童中。而在成人中孤立病例极为罕见。
病例报告
一名38岁男性因难治性慢性便秘就诊。腹部计算机断层扫描显示乙状结肠冗长，随后他接受了乙状结肠切除术。组织病理学检查显示弥漫性神经节瘤病。然而，患者术后18个月健康状况良好。
临床讨论
肠道神经节瘤常见于患有全身性综合征的儿童，如2B型多发性内分泌肿瘤和1型神经纤维瘤病。最常见的症状是腹部不适、便秘、肠梗阻、体重减轻、阑尾炎，在更严重的情况下会出现梗阻。手术切除是弥漫性神经节瘤病的标准治疗方法。
结论
虽然弥漫性神经节瘤病不常见，但对于难治性便秘患者应考虑此病。
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