儿科白内障卷风,非洲退行性肌肉病的罕见表现 - 一份病例报告
Francesca Palmisani1,2, Emanuele Trovalusci3,4, Sphamandla Zulu3
1Department of Paediatric Surgery, Medical University of Vienna, Vienna, Austria. francesca.palmisani@meduniwien.ac.at.
International journal of colorectal disease
|January 26, 2026
概括
儿童的卷发可能表明儿科肠道伪阻塞 (PIPO). 这一案例突出了非洲退行性肌肉病变 (ADL) 作为PIPO的罕见原因,导致白内障卷积和不良结果.
科学领域:
- 胃肠病学 胃肠病学
- 儿科手术 儿科手术
- 病理学 病理学 病理学
背景情况:
- 便秘卷发症在儿童中很少见,通常与结肠固定异常或从慢性便秘或赫施普朗格病 (HD) 等疾病引起的膨胀有关.
- 非洲退行性肌肉病变 (ADL) 是一种导致PIPO的内脏肌肉病变,是撒哈拉以南非洲的特有病,具有严重的后果.
- 这是首个报告的白内障卷发病例,导致ADL的诊断.
研究的目的:
- 报告小儿病患患中唯一的白内障卷积病例.
- 突出了白内障卷风和非洲退行性肌肉病 (ADL) 之间的关联.
- 强调在儿科白内障中考虑儿科肠道伪阻塞 (PIPO) 的重要性.
主要方法:
- 一个11岁的女性患有白内障卷发症的病例介绍.
- 诊断工作包括腹部放射,CT扫描,探索性腹腔切除术和组织学.
- 进行了直肠活检,以排除希尔施普隆病 (HD).
主要成果:
- 患者接受了白内障卷积的血液切除术和胆管切除术.
- 组织学证实了与ADL一致的肌病变化.
- 尽管接受了治疗,但患者经历了反复出现的伪阻塞,并死于腹腔综合征.
结论:
- 儿童的卷发需要对潜在的PIPO进行调查.
- ADL是一种罕见的,往往致命的疾病,导致间歇性肠道阻塞和全身问题.
- 在儿科白内障卷发病例中早期识别ADL对于管理至关重要.
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