Lin Kunzhe, Fan Yong, Lin Zhehui, He Xiangzhong, Huang Shaokuan, Zhang Fan
Department of Neurosurgery, Affiliated Fuzhou First Hospital of Fujian Medical University, No. 190, Dadao Road, Fuzhou 350009, Fujian, China.
Department of Central Laboratory, Affiliated Fuzhou First Hospital of Fujian Medical University, Fuzhou 350009, Fujian, China.
Open Med (Wars). 2021 Mar 22;16(1):435-439. doi: 10.1515/med-2021-0245. eCollection 2021.
In this study, a rare case with hemophilia pseudotumor in the skull was reported.
The case was a 34-year-old male patient who was admitted to the hospital, with the complaint of dizziness for more than 1 month. The physical examination indicated that the patient was conscious, who could give right answers to the questions. Moreover, there was a bulge on the left frontal-temporal parietal bone. The head CT showed abnormal density lesions on the left frontal-temporal parietal bone, with multiple irregular calcifications within the border. Skull MRI showed a large clump-like mixed signal at the top of the left frontal ridge. After admission, the patient was subjected to complete preoperative preparation and surgical treatment. Neurological navigation was used to determine the extent of skull defect before surgery to make a surgical incision. Clotting factor VIII substitution therapy was used for the intraoperative and postoperative treatments. The lesion was completely removed.
These results suggest that the skull hemophilia pseudotumor has been rarely seen. According to imaging examination, in combination with family history, the diagnosis can be confirmed. If there is no obvious occupying effect, conservative treatment can be tried. On the contrary, if there is an obvious occupying effect, surgical treatment might be effective, and coagulation factor VIII should be supplemented during the perioperative period.
本研究报告了一例罕见的颅骨血友病性假瘤病例。
该病例为一名34岁男性患者，因头晕1个多月入院。体格检查显示患者意识清醒，能正确回答问题。此外，左额颞顶骨有一隆起。头部CT显示左额颞顶骨密度异常病变，边界内有多个不规则钙化。头颅MRI显示左额嵴顶部有一大团块状混合信号。入院后，患者接受了完善的术前准备和手术治疗。术前采用神经导航确定颅骨缺损范围以进行手术切口。术中及术后采用凝血因子VIII替代治疗。病变被完全切除。
这些结果表明颅骨血友病性假瘤较为罕见。根据影像学检查，结合家族史可确诊。若没有明显占位效应，可尝试保守治疗。相反，若有明显占位效应，手术治疗可能有效，围手术期应补充凝血因子VIII。
