血友病患者枕骨肿块的诊断与治疗策略：1例罕见病例报告及文献复习
The diagnosis and treatment strategy of occipital skull mass in hemophilic patients: a rare case report and literature review.
作者信息
Liu Qingyuan, Wu Jun, Li Chunde, Wang Shuo
机构信息
Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, No.119 South 4th Ring West Road, Fengtai District, Beijing, 100070 China.
China National Clinical Research Center for Neurological Diseases, Beijing, People's Republic of China.
出版信息
Chin Neurosurg J. 2019 Apr 3;5:8. doi: 10.1186/s41016-019-0155-x. eCollection 2019.
BACKGROUND
Cranial hemophilic pseudotumor (cHPT) is a very rare disease, which is easy to misdiagnose. It is also difficult to manage such patients. We reported the first case of occipital cHPT.
CASE PRESENTATION
Here, we presented a rare case of an occipital bone mass in a 3-year-old boy who was diagnosed with hemophilia A. The mass was misdiagnosed as an aneurysmal bone cyst by pathological examination. After resection, the patient underwent one-stage cranioplasty. However, the patient was admitted again for hematoma caused by an invasive procedure. A second surgery and one-stage cranioplasty were performed at the same time. A follow-up 3 months after discharging showed the patient was uneventful, and the titanium mesh was well fixed.
CONCLUSION
The diagnosis of cHPT requires the combining of history, radiological examination, and pathological examination. Resection is the best choice for symptomatic cHPT. Replacement treatment and less invasive treatment can make perioperative management safer. One-stage cranioplasty for resection of an occipital cHPT can improve the quality of life.
背景
颅骨血友病性假瘤（cHPT）是一种非常罕见的疾病，容易误诊，治疗此类患者也很困难。我们报告了首例枕部cHPT病例。
病例介绍
在此，我们展示了一例罕见的3岁男孩枕部骨肿物病例，该男孩被诊断为甲型血友病。经病理检查，该肿物被误诊为动脉瘤样骨囊肿。切除术后，患者接受了一期颅骨成形术。然而，患者因侵入性操作导致血肿再次入院。同时进行了二次手术和一期颅骨成形术。出院3个月后的随访显示患者情况良好，钛网固定良好。
结论
cHPT的诊断需要结合病史、影像学检查和病理检查。对于有症状的cHPT，手术切除是最佳选择。替代治疗和微创治疗可使围手术期管理更安全。一期颅骨成形术切除枕部cHPT可提高生活质量。
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