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开放性胎儿修复和产后脊髓脊膜膨出闭合术后患者囊性病变的详细分析。

Detailed analysis of cystic lesions in patients after open fetal repair and postnatal myelomeningocele closure.

作者信息

Salwi Sanjana R, Land Sierra D, Gallagher Taryn, Reynolds Tom A, Zarnow Deborah M, Viaene Angela, Moldenhauer Julie S, Adzick N Scott, Flanders Tracy M, Heuer Gregory G

机构信息

Division of Neurosurgery, Department of Surgery, Children's Hospital of Philadelphia, Philadelphia, PA, USA.

Richard D. Wood Jr. Center for Fetal Diagnosis and Treatment, Children's Hospital of Philadelphia, Philadelphia, PA, USA.

出版信息

Childs Nerv Syst. 2025 Jan 3;41(1):78. doi: 10.1007/s00381-024-06735-8.

Abstract

PURPOSE

We sought to evaluate the incidence, natural history, and management of cystic spinal lesions following myelomeningocele/myeloschisis closure.

METHODS

We performed a single-center retrospective review of all patients who underwent myelomeningocele/myeloschisis closure from 2013 to 2018 with follow-up to 5 years old.

RESULTS

We analyzed 100 fetal repairs and 81 postnatal closures from 305 total surgeries. Patients within this cohort systematically underwent serial MRI scans of the lumbar spine and had clinical follow-up until at least 5 years of age. Ninety-three (51.2%) developed radiographic evidence of cystic lesions with 28 (30.1%) requiring surgical intervention. Presence of cysts was higher in fetal repair (67/100, 67%) compared with postnatal (26/81, 32.1%; p < 0.01). Of the 93 patients with radiographic cysts, 28 (30.1%) underwent surgical resection at a median age of 27.6 months old ([Q1, Q3], [13.0, 48.6 months]). Fetal repair patients had a higher rate (26/67, 38.8%) of cysts requiring surgical resection compared with postnatal closure (2/26, 7.7%, p value < 0.01). Pathology demonstrated 18 of resected cysts were dermoid, 8 were epidermoid, and 2 were fibrous tissue. Post-operatively, no patients experienced a worsened ambulation status. Bladder compliance showed a non-significant trend toward improvement.

CONCLUSIONS

Cystic lesions in myelomeningocele/myeloschisis patients are common findings that result in nerve root tethering. We propose regular screening in both symptomatic and asymptomatic patients to circumvent nerve injury. Most cystic lesions do not require surgical resection though fetal repair is associated with a higher incidence of operative cysts. However, these lesions can be safely surgically resected with maintenance of ambulation and urologic function.

摘要

目的

我们旨在评估脊髓脊膜膨出/脊髓裂闭合术后囊性脊柱病变的发生率、自然病史及治疗方法。

方法

我们对2013年至2018年期间接受脊髓脊膜膨出/脊髓裂闭合术且随访至5岁的所有患者进行了单中心回顾性研究。

结果

我们分析了305例手术中的100例胎儿修复术和81例产后闭合术。该队列中的患者系统地接受了腰椎的系列磁共振成像扫描,并进行了临床随访直至至少5岁。93例(51.2%)出现了囊性病变的影像学证据,其中28例(30.1%)需要手术干预。胎儿修复术后囊肿的发生率(67/100,67%)高于产后(26/81,32.1%;p<0.01)。在93例有影像学囊肿的患者中,28例(30.1%)在中位年龄27.6个月([第一四分位数,第三四分位数],[13.0,48.6个月])接受了手术切除。与产后闭合术相比,胎儿修复术患者中需要手术切除的囊肿发生率更高(26/67,38.8%对比2/26,7.7%,p值<0.01)。病理显示,切除的囊肿中有18例为皮样囊肿,8例为表皮样囊肿,2例为纤维组织。术后,没有患者的行走状态恶化。膀胱顺应性有改善的非显著趋势。

结论

脊髓脊膜膨出/脊髓裂患者的囊性病变是导致神经根牵拉的常见发现。我们建议对有症状和无症状患者进行定期筛查以避免神经损伤。大多数囊性病变不需要手术切除,尽管胎儿修复术与手术相关囊肿的发生率较高有关。然而,这些病变可以通过手术安全切除,同时保持行走和泌尿功能。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cc43/11698761/9a65a9b5eca1/381_2024_6735_Fig1_HTML.jpg

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