一名患有猪大小便失禁症的儿童颅内扩展的波特浮肿瘤:同名疾病的病例回顾。

IF 1.3 4区 医学 Q4 CLINICAL NEUROLOGY
Child's Nervous System Pub Date : 2024-11-01 Epub Date: 2024-08-24 DOI:10.1007/s00381-024-06577-4
Debajyoti Datta, Albert Tu
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引用次数: 0

摘要

背景:波特浮肿瘤(PPT)是一种不常见的累及额骨的感染,由珀西瓦尔-波特爵士(Sir Percival Pott)在 250 多年前首次描述。它可表现为颅内扩展和严重的神经系统疾病。猪失禁症(IP)是一种罕见的遗传性基因皮肤病,男性在产前发病致死,女性则表现为临床症状。IP 与反复感染和免疫功能障碍/抑制有关:方法:我们报告了一例帕特浮肿瘤患儿。我们还对与免疫功能障碍相关的 PPT 病例进行了文献综述。我们讨论了这些病变的临床表现、诊断和处理方法:我们发现了 12 例与免疫功能障碍/抑制相关的 PPT。糖尿病是最常见的病因,其次是先天性免疫抑制。手术是治疗 PPT 的标准治疗方法,我们还讨论了颅内受累和未受累 PPT 的治疗方法,尤其是在潜在免疫功能障碍/抑制的情况下:结论:PPT仍然是一种罕见但并非不常见的诊断,通常需要神经外科干预。免疫功能障碍/抑制是导致 PPT 的另一个风险因素。为获得最佳疗效,应及早采取积极的治疗措施。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

Pott's puffy tumor with intracranial extension in a child with incontinentia pigmenti: case based review of the eponymous disease.

Pott's puffy tumor with intracranial extension in a child with incontinentia pigmenti: case based review of the eponymous disease.

Background: Pott's puffy tumor (PPT) is an uncommon infection involving the frontal bone, first described by Sir Percival Pott more than 250 years ago. It can present with intracranial extension and serious neurological morbidity. Incontinentia pigmenti (IP) is a rare inherited genodermatosis that is lethal prenatally in males and manifests clinically in females. IP is associated with recurrent infections and immune dysfunction/suppression.

Methods: We report a case of Pott's puffy tumor presenting in a child with IP. We also performed a literature review of reported cases of PPT associated with immune dysfunction. We discuss the clinical presentation, diagnosis, and management of these lesions.

Results: We identified 12 cases of PPT associated with immune dysfunction/suppression. Diabetes was the most commonly identified cause followed by iatrogenic immunosuppression. Surgery is the standard treatment for managing PPT and the management of PPT with and without intracranial involvement, particularly in the context of underlying immune dysfunction/suppression, is discussed.

Conclusion: PPT remains a rare but not infrequent diagnosis, often requiring neurosurgical intervention. Immune dysfunction/suppression is an additional risk factor that may predispose to PPT. Early and aggressive management should be instituted for optimal outcome.

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来源期刊
Child's Nervous System
Child's Nervous System 医学-临床神经学
CiteScore
3.00
自引率
7.10%
发文量
322
审稿时长
3 months
期刊介绍: The journal has been expanded to encompass all aspects of pediatric neurosciences concerning the developmental and acquired abnormalities of the nervous system and its coverings, functional disorders, epilepsy, spasticity, basic and clinical neuro-oncology, rehabilitation and trauma. Global pediatric neurosurgery is an additional field of interest that will be considered for publication in the journal.
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