Sakamoto Yushi, Ozaki Tomonori, Tahata Shogo, Fujimoto Toru, Naruo Seiichiro
Department of Spine Surgery, Naruo Orthopedic Hospital, Kumamoto, Japan.
JMA J. 2024 Oct 15;7(4):638-641. doi: 10.31662/jmaj.2024-0005. Epub 2024 Aug 23.
Hemophilia B is a quantitative or qualitative factor IX anomaly that manifests as an X-linked recessive inheritance pattern in which females are carriers. Postoperative epidural hematoma emerges as a typical complication in spinal surgery, although its incidence is infrequent. No documentation of postoperative epidural hematoma in carriers of hemophilia B exists. A 64-year-old female patient presented with progressive pain and muscle weakness in both lower limbs. Despite a history of childbirth and prior colorectal cancer surgery, the patient displayed no abnormal bleeding tendencies. Subsequently undergoing decompression surgery for lumbar spinal canal stenosis, the patient experienced paralysis and pain in both legs within 5 hours postoperatively. A magnetic resonance imaging scan revealed severe spinal canal compression attributed to a postoperative epidural hematoma, prompting emergency decompression surgery that ameliorated symptoms. The application of gelatin-thrombin matrix sealants (GTMS) facilitated hematoma removal, resulting in an uneventful recovery. In a postoperative interview, it was revealed that her grandson was undergoing treatment for hemophilia B. Additionally, she exhibited diminished factor IX levels and was diagnosed as a hemophilia B carrier. A definitive preoperative diagnosis of the carrier status is imperative. In instances where surgical intervention is warranted, the implementation of factor IX replacement and intraoperative hemostasis with GTMS is promising for potentially averting the onset of postoperative epidural hematoma.
血友病B是一种定量或定性的因子IX异常,表现为X连锁隐性遗传模式,女性为携带者。术后硬膜外血肿是脊柱手术中的一种典型并发症,尽管其发生率较低。目前尚无血友病B携带者术后硬膜外血肿的相关报道。一名64岁女性患者出现双下肢进行性疼痛和肌肉无力。尽管有分娩史和既往结直肠癌手术史,但该患者未表现出异常出血倾向。随后因腰椎管狭窄接受减压手术,患者术后5小时内出现双腿瘫痪和疼痛。磁共振成像扫描显示,术后硬膜外血肿导致严重的椎管受压,促使进行紧急减压手术,症状得以缓解。应用明胶-凝血酶基质密封剂(GTMS)有助于清除血肿,患者恢复顺利。在术后访谈中发现,她的孙子正在接受血友病B治疗。此外,她的因子IX水平降低,被诊断为血友病B携带者。术前明确携带者状态至关重要。在需要进行手术干预的情况下,实施因子IX替代治疗并在术中使用GTMS止血,有望避免术后硬膜外血肿发作。