Soman Shardul, Tharadara G D, Chhatrala Naitik, Jain Shubham
Department of Spine, Government Spine Institute, Ahmedabad, India.
Department of Orthopaedics, B.J. Medical College and Civil Hospital Ahmedaba, India.
Int J Spine Surg. 2016 Jun 30;10:25. doi: 10.14444/3025. eCollection 2016.
Extramedullary hematopoeisis (EMH) is defined as formation of blood cells outside the bone marrow. It occurs most commonly in the liver and spleen in patients having disorders that lead to chronic anaemia. EMH in spinal canal is a very rare site and cauda equina syndrome due to EMH has very few cases presented in literature.
A 28 year old male patient presented with complain of incontinenance of bladder and bowel along with saddle anaesthesia from 10 days. Patient was a known case of beta-thalassemia intermedia. And MRI scan of the spine showed multiple well circumscribed, enhancing lesions in the epidural space extending from L5 to S3 and resulting in compression of the cauda equina. Patient underwent posterior neural decompression by a laminectomy from L5 to S3. At 3 months follow up patient had partial recovery of his bladder control and complete recovery of sensation.
EMH should be recognized early on the basis of clinical features and MRI findings. The various modalities available for treatment of such cases includes blood transfusion, low dose radiotherapy, hydroxyurea and surgical decompression. There are very few cases noted in the literature of such phenomenon in the lumbosacral spine. In cases of acute presentations like cauda equine surgical decompression is a treatment modality of choice.
髓外造血(EMH)被定义为在骨髓外形成血细胞。它最常见于患有导致慢性贫血疾病的患者的肝脏和脾脏。椎管内的EMH是一个非常罕见的部位,因EMH导致的马尾综合征在文献中报道的病例极少。
一名28岁男性患者,因膀胱和肠道失禁以及鞍区感觉缺失10天前来就诊。患者为中间型β地中海贫血已知病例。脊柱MRI扫描显示硬膜外间隙有多个边界清晰的强化病灶,从L5延伸至S3,导致马尾受压。患者接受了L5至S3的椎板切除后路神经减压术。在3个月的随访中,患者膀胱控制部分恢复,感觉完全恢复。
应根据临床特征和MRI表现尽早识别EMH。治疗此类病例的各种方法包括输血、低剂量放疗、羟基脲和手术减压。腰骶部脊柱出现这种现象的文献报道极少。在急性表现如马尾综合征的病例中,手术减压是首选的治疗方式。