延迟表现的先天性sigmoid结肠狭窄症:一个罕见的实体
Prajwal Dahal1, Kapil Dawadi1, Sabina Parajuli2
1Department of Radiology and Imaging, Grande International Hospital, Tokha, Kathmandu, Bagmati, 11796, Nepal.
BJR case reports
|February 14, 2024
概括
先天性西格结肠狭窄症是一种罕见的疾病,可以被误认为是赫斯普隆病. 这一案例突出了由于其类似的呈现而导致2岁儿童的延迟诊断.
科学领域:
- 儿科手术 儿科手术
- 胃肠病学 胃肠病学
- 医疗成像医学成像
背景情况:
- 先天性sigmoid结肠狭窄症是婴儿远端肠道阻塞的一个罕见原因.
- 它经常表现出模仿赫斯普隆病的症状,导致诊断挑战.
研究的目的:
- 报告一个先天性sigmoid结肠狭窄症的病例,该病例发生在2岁的孩子身上.
- 强调在儿科肠道阻塞的差异诊断中考虑这种罕见实体的重要性.
- 为了说明对比度增强CT和对比度浸泡剂的诊断实用性.
主要方法:
- 一个2岁的孩子患有慢性便秘和腹部膨胀的案例报告.
- 诊断工作包括对比增强型计算机断层扫描 (CT) 扫描和对比浸泡.
- 狭窄片段的手术切除,然后进行体内病理学检查.
主要成果:
- 最初的临床诊断是赫施普隆病.
- 脑电图扫描和对比液囊揭示了近端西格结肠的短段狭窄,有一个清晰的过渡点.
- 胰腺病理学证实了切除的狭窄分段中有足够的质细胞,排除了赫施普朗格病.
结论:
- 先天性sigmoid结肠狭窄症可以在婴儿期晚些时候出现,具有远端肠道阻塞的特征.
- 多模式成像对于将其与希尔施普朗格病区分开来至关重要.
- 对于诊断狭窄症和排除希尔施普朗格病而言,质细胞的组织病理学确认是最终的.
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