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小儿脊髓硬膜下血肿揭示甲型血友病:一例报告。

Spinal subdural hematoma revealing hemophilia A in a child: A case report.

作者信息

Eftekhar Behzad, Ghodsi Mohammad, Ketabchi Ebrahim, Bakhtiari Abbas, Mostajabi Pardis

机构信息

Department of Neurosurgery, Sina Hospital, Tehran University, Iran.

出版信息

BMC Blood Disord. 2003 Aug 7;3(1):2. doi: 10.1186/1471-2326-3-2.

Abstract

BACKGROUND

Intraspinal bleeding especially in the form of subdural hematoma is rare in hemophiliacs. In the present case, we report a neglected hemophilic A child with such a problem and discuss its management options. CASE PRESENTATION: A 9-year old hemophilic A boy presented with quadriparesis, confusion and meningismus after a fall 4 days previously. There was no sign of direct trauma to his back. His CT Scan and MRI showed spinal extramedullary hematoma extended from C5 to L2. We corrected the factor VIII level, but two days later, the patient's lower limbs weakened to 1/5 proximally as well as distally. We performed a laminectomy from T11 to L2, according to the level of the maximal neurological deficit and recent deterioration course. The subdural hematoma was evacuated. The hematoma in other spinal levels was managed conservatively. In the week following the operation, the patient's neurological status approached normal. CONCLUSION: This case calls attention to the clinical manifestation, radiological features and management options of the rarely reported intraspinal hematoma in hemophilic children. Although this case has been managed operatively for its hematoma in the thoracolumbar region, at the same time it can be considered a successful case of conservative management of intraspinal hematoma in the cervicothoracic region. Both conservative and surgical management could be an option in managing these patients considering their neurological course.

摘要

背景

脊髓内出血,尤其是硬膜下血肿形式,在血友病患者中罕见。在本病例中,我们报告了一名患有此类问题但被忽视的甲型血友病儿童,并讨论其治疗方案。病例介绍:一名9岁甲型血友病男孩在4天前摔倒后出现四肢瘫痪、意识模糊和颈项强直。其背部无直接外伤迹象。他的CT扫描和MRI显示脊髓外血肿从C5延伸至L2。我们纠正了凝血因子VIII水平,但两天后,患者双下肢近端和远端肌力减弱至1/5。根据最大神经功能缺损水平和近期病情恶化过程,我们从T11至L2进行了椎板切除术。硬膜下血肿被清除。其他脊髓节段的血肿采取保守治疗。术后一周,患者神经状态接近正常。结论:本病例提请关注血友病儿童中罕见报道的脊髓内血肿的临床表现、影像学特征及治疗方案。尽管本病例因胸腰段血肿接受了手术治疗,但同时它也可被视为颈胸段脊髓内血肿保守治疗的成功案例。考虑到患者的神经病程,保守治疗和手术治疗均可作为管理这些患者的选择。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cd00/194670/6d8bc271b12c/1471-2326-3-2-1.jpg

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