开放性胎儿修复和产后髓膜膨出闭合后囊性病变的详细分析。

IF 1.3 4区 医学 Q4 CLINICAL NEUROLOGY Child's Nervous System Pub Date : 2025-01-03 DOI:10.1007/s00381-024-06735-8
Sanjana R Salwi, Sierra D Land, Taryn Gallagher, Tom A Reynolds, Deborah M Zarnow, Angela Viaene, Julie S Moldenhauer, N Scott Adzick, Tracy M Flanders, Gregory G Heuer
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引用次数: 0

摘要

目的:我们试图评估脊髓脊膜膨出/髓裂闭合后囊性脊髓病变的发生率、自然病史和处理。方法:我们对2013年至2018年所有接受脊髓脊膜膨出/髓裂闭合的患者进行了单中心回顾性研究,随访至5岁。结果:我们分析了305例手术中100例胎儿修复和81例产后闭合。该队列中的患者系统地接受了腰椎的连续MRI扫描,并进行了临床随访,直到至少5岁。93例(51.2%)出现囊性病变的影像学证据,28例(30.1%)需要手术干预。胎儿修复时出现囊肿的比例(67/100,67%)高于出生后(26/81,32.1%;结论:髓脊膜膨出/髓裂患者的囊性病变是导致神经根栓系的常见表现。我们建议对有症状和无症状的患者进行定期筛查,以避免神经损伤。大多数囊性病变不需要手术切除,但胎儿修复与手术囊肿的发生率较高有关。然而,这些病变可以在维持行走和泌尿功能的情况下安全地手术切除。
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Detailed analysis of cystic lesions in patients after open fetal repair and postnatal myelomeningocele closure.

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.

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来源期刊
Child's Nervous System
Child's Nervous System 医学-临床神经学
CiteScore
3.00
自引率
7.10%
发文量
322
审稿时长
3 months
期刊介绍: The journal has been expanded to encompass all aspects of pediatric neurosciences concerning the developmental and acquired abnormalities of the nervous system and its coverings, functional disorders, epilepsy, spasticity, basic and clinical neuro-oncology, rehabilitation and trauma. Global pediatric neurosurgery is an additional field of interest that will be considered for publication in the journal.
期刊最新文献
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