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终末脊髓脊膜膨出:30例病例系列及文献综述

Terminal myelocystocele: a series of 30 cases and review of the literature.

作者信息

Tandon Vivek, Garg Kanwaljeet, Mahapatra Ashok Kumar

机构信息

Department of Neurosurgery, Cardioneurosciences Centre, All India Institute of Medical Sciences, New Delhi, India.

出版信息

Pediatr Neurosurg. 2012;48(4):229-35. doi: 10.1159/000349915. Epub 2013 Mar 23.

DOI:10.1159/000349915
PMID:23548822
Abstract

OBJECTIVE

Terminal myelocystocele is a rare form of spinal dysraphism. We report on the clinical and radiological features, surgical procedures and outcome of myelocystocele.

METHODS

Thirty patients, with an age range of 1 month to 15 years, which included 16 (53.3%) male children, had undergone surgery for terminal myelocystocele between 2000 and 2010. We had retrospectively analyzed their data. All patients had a swelling in the lumbosacral region which had a healthy skin cover. Twenty (66.7%) patients had presented with weakness, while 10 (33.7%) patients had no deficits at all. Bladder bowel involvement was evident in 12 (40%) cases. All patients had undergone excision of the meningocele sacs, the tethering bands were lysed, and filum was detethered. Ventriculoperitoneal shunt was done in 3 (10%) patients. Complications like pseudomeningocele were seen in 6 (20%) patients, cerebrospinal fluid leak in 4 (13.3%) patients, and meningitis and surgical site infection in 1 (3.3%) patient each. The mean follow-up period was 15.2 (1-60) months. The status of all patients with no preoperative deficits remained unchanged. Out of 20 patients with motor weakness, 12 (60%) had improvement; worsening was observed in 1 patient.

CONCLUSIONS

Though rare, the possibility of myelocystocele should be kept in mind when treating lumbosacral region masses as they have a better prognosis when compared with other masses in the region due to spinal dysraphism. We recommend early surgery in all diagnosed cases.

摘要

目的

终末型脊髓脊膜膨出是一种罕见的脊柱裂畸形。我们报告脊髓脊膜膨出的临床和放射学特征、手术方法及结果。

方法

2000年至2010年间,30例年龄在1个月至15岁之间的患者接受了终末型脊髓脊膜膨出手术,其中包括16例(53.3%)男童。我们对他们的数据进行了回顾性分析。所有患者腰骶部均有肿胀,表面皮肤正常。20例(66.7%)患者出现肌无力,10例(33.7%)患者无任何功能缺损。12例(40%)患者有膀胱直肠受累表现。所有患者均接受了脊膜膨出囊切除、松解栓系带及切断终丝。3例(10%)患者进行了脑室腹腔分流术。6例(20%)患者出现假性脊膜膨出等并发症,4例(13.3%)患者出现脑脊液漏,1例(3.3%)患者出现脑膜炎和手术部位感染。平均随访时间为15.2(1 - 60)个月。所有术前无功能缺损的患者病情无变化。20例肌无力患者中,12例(60%)病情改善,1例病情恶化。

结论

尽管终末型脊髓脊膜膨出罕见,但在治疗腰骶部肿块时应考虑到这种可能性,因为与该区域因脊柱裂畸形导致的其他肿块相比,其预后较好。我们建议对所有确诊病例尽早进行手术。

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