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儿童颈胸段脊柱裂

Spinal dysraphisms of the cervicothoracic region in childhood.

作者信息

Orakdogen Metin, Turk Cezmi Cagri, Ersahin Mehmet, Biber Necat, Berkman Zafer

机构信息

Haydarpaşa Numune Research and Educational Hospital, Clinic of Neurosurgery, Istanbul, Turkey.

出版信息

Turk Neurosurg. 2009 Oct;19(4):400-5.

Abstract

PURPOSE

Spinal dysraphisms are the most common congenital anomalies of the CNS. Spinal dysraphism (SD) of cervical and upper thoracic region are rare, demonstrating distinct clinical and structural configurations compared to lumbar counterparts.

METHODS

In Haydarpasa Numune Hospital, a total of 7 cases (3 male and 4 female) ranging between 20 days to 9 years of age with cervicothoracic SD were operated on between 2002 and 2008. The sacs were located in the cervical and thoracic region in 4 and 3 of the cases, respectively. The associated anomalies were diagnosed in 3 cases, including SCM type 2, Chiari type 2, hyrocephalus and hydromyelia. All cases underwent surgical treatment that involves excision of the sac and intradural exploration providing untethering of the spinal cord. Postoperative follow up of these patients was uneventful and neither neurological deficits nor complications were observed.

CONCLUSION

Cervicothoracic SD has more favorable outcome in respect to neurological, orthopaedic and urologic problems compared to lumbar counterparts. In order to prevent forthcoming neurological deterioration, surgical treatment consisting of intradural exploration of the lesion, untethering of the spinal cord and excision of potential adhesions should be performed in the early period.

摘要

目的

脊柱裂是中枢神经系统最常见的先天性异常。颈段和上胸段脊柱裂(SD)较为罕见,与腰段脊柱裂相比,其临床和结构形态各异。

方法

在海达尔帕萨努穆内医院,2002年至2008年间,共对7例年龄在20天至9岁之间的颈胸段脊柱裂患者进行了手术(3例男性,4例女性)。其中4例患者的囊肿位于颈部,3例位于胸部。3例患者被诊断出伴有其他异常,包括2型脊髓脊膜膨出、2型Chiari畸形、脑积水和脊髓空洞症。所有患者均接受了手术治疗,包括囊肿切除和硬膜内探查以松解脊髓。这些患者术后随访情况良好,未观察到神经功能缺损或并发症。

结论

与腰段脊柱裂相比,颈胸段脊柱裂在神经、骨科和泌尿系统问题方面预后更佳。为防止未来出现神经功能恶化,应尽早进行手术治疗,包括对病变进行硬膜内探查、松解脊髓和切除潜在粘连。

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