Datta Debajyoti, Tu Albert
Division of Pediatric Neurosurgery, Children's Hospital of Eastern Ontario (CHEO), Rm 3359, 401 Smyth Rd, Ottawa, ON, K1H 8L1, Canada.
University of Ottawa, Ottawa, ON, Canada.
Childs Nerv Syst. 2024 Nov;40(11):3505-3510. doi: 10.1007/s00381-024-06577-4. Epub 2024 Aug 24.
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.
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.
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.
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.
波特氏浮肿性骨炎（PPT）是一种累及额骨的罕见感染，250多年前由珀西瓦尔·波特爵士首次描述。它可出现颅内扩展并导致严重的神经功能障碍。色素失禁症（IP）是一种罕见的遗传性皮肤病，男性胎儿期致死，女性有临床症状。IP与反复感染及免疫功能障碍/抑制有关。
我们报告一例患有IP的儿童出现波特氏浮肿性骨炎的病例。我们还对已报道的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、诊断和治疗。
我们确定了12例与免疫功能障碍/抑制相关的PPT病例。糖尿病是最常见的病因，其次是医源性免疫抑制。手术是治疗PPT的标准方法，并讨论了有无颅内受累情况下PPT的治疗，特别是在存在潜在免疫功能障碍/抑制的情况下。
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/抑制是可能易患PPT的另一个危险因素。应尽早积极治疗以获得最佳结果。
