制度・支援
指定難病 — No.118

脊髄髄膜瘤

検索語 Myelomeningocele ・ 最終更新 2026-09-17 13:07 ・ 最新に更新

Data Sheet
指定 No.118
Src PubMed · CT.gov · jRCT

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( 01 )EVIDENCE / PUBMED · 5件

世界の論文

直近の研究を、やさしい日本語で

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観察研究
MK-01 · PMID 42728560

Physical therapy interventions for children and adolescents with myelomeningocele: A systematic review

Abstract / 原文

AIM: To systematically review the current evidence on physical therapy interventions for children and adolescents with myelomeningocele, the most complex and common presentation of spina bifida, to explore intervention components and the outcomes according to International Classification of Functioning, Disability and Health (ICF) domains, and to evaluate the level of evidence and methodological quality of the studies. METHODS: The MEDLINE/PubMed, Embase, Cochrane, Scopus, Web of Science, PEDro, and BVS databases were searched from inception to May 2026. Methodological quality was appraised using JBI critical appraisal tools, and levels of evidence were based on recommendations of the Oxford Centre for Evidence-Based Medicine. RESULTS: A total of 36 studies were included, involving 532 children and adolescents aged from 1 month to 18 years. The most frequently investigated interventions were electrical stimulation (n = 9), treadmill training (n = 4), and whole-body vibration (n = 3). Eighteen studies were rated as high methodological quality, ten as moderate quality, and eight as low quality. INTERPRETATION: A wide range of physical therapy interventions have been investigated for children and adolescents with myelomeningocele across multiple ICF domains. Although interventions such as electrical stimulation, treadmill training, serial casting, and laser therapy showed favorable outcomes in specific domains, the available evidence remains limited and heterogeneous, precluding definitive conclusions about their effectiveness.

Journal
Developmental medicine and child neurology(2026 Sep)
Authors
4名
Type
Journal Article, Review
PubMedで原文を見る
不明
MK-02 · PMID 42728023

Exploring the Legal Landscape of Intradural Spine Procedures: A Retrospective Analysis of US Malpractice Claims

Abstract / 原文

INTRODUCTION: Intradural spine procedures carry substantial neurological risk and technical complexity, yet their associated medicolegal landscape remains poorly characterized. Improved understanding of their litigation patterns may help inform patient counseling, perioperative communication, and risk-reduction strategies. METHODS: A retrospective analysis was conducted of malpractice litigation involving common intradural spine procedures: myelomeningocele repair, tethered cord release, and intradural mass resection, using the Westlaw legal database. Inclusion required that one of the specified procedures was central to the litigation and had been performed. Data were extracted on plaintiff demographics, allegations, and outcomes. RESULTS: From an initial review of 417 cases, 22 met the inclusion criteria. Most cases (63.6%) occurred after 2005. The estimated annual litigation frequency was 1 per 1200 intradural mass resection procedures, 1 per 2950 tethered cord release procedures, and 1 per 4600 myelomeningocele repairs. Common allegations included improper informed consent (63.6%) and procedural error (50.0%). Physicians were named in 86.4% of cases, followed by hospitals or clinics (72.7%) and government entities (27.3%). At the time of review, only 4.5% had resulted in a plaintiff verdict. The mean time from surgery to legal resolution was 70.2 months. CONCLUSION: Intradural spine litigation represents an underexplored area of medical malpractice. Informed consent and procedural error were most frequently cited, highlighting the potential benefit of improved preoperative communication and surgical protocols. Intradural mass resection had the highest estimated annual frequency and involved only adult plaintiffs, reflecting possible differences between adult and pediatric populations, such as differences in baseline functional status and the role of guardians in pediatric claims.

Journal
International journal of spine surgery(2026 Sep)
Authors
6名
Type
Journal Article
PubMedで原文を見る
症例報告
MK-03 · PMID 42720059

Bilateral Lumbar Artery Perforator (LAP) Flaps for Extensive Lumbosacral Defects: A Report of Two Cases

Abstract / 原文

Extensive lumbosacral defects remain a major reconstructive challenge because of limited regional tissue mobility, contamination risk, proximity to neural structures, and frequent association with prior surgery or spinal instrumentation. Although lumbar artery perforator (LAP) flaps are recognized as muscle-sparing options for posterior trunk reconstruction, their capacity to safely reconstruct very large midline lumbosacral defects and their applicability in rare adult presentations of lumbosacral myelomeningocele remain insufficiently defined. We report two adult female patients who underwent reconstruction of extensive midline lumbosacral defects using bilateral pedicled LAP flaps to illustrate the applicability of this approach in exceptionally large post-instrumentation and adult dysraphic defects. The first patient, a 19-year-old woman, presented with a 36 × 28 cm post-instrumentation defect with exposed L4-S1 hardware following multi-trauma and repeated soft-tissue necrosis. After serial debridements and prolonged culture-directed intravenous antibiotic therapy, bilateral LAP flaps measuring 22 × 12 cm were elevated based on bilateral L4 perforators identified by computed tomography angiography and Doppler ultrasonography. The second patient, a 28-year-old paraplegic woman with spina bifida, presented with recurrent cerebrospinal fluid leakage associated with a 22 × 16 cm lumbosacral myelomeningocele defect. Following neurosurgical dural repair, bilateral LAP flaps measuring 18 × 10 cm were harvested based on medially displaced L4 perforators. In both cases, the flaps were designed according to defect geometry and perforator location, then rotated approximately 90° medially to achieve tension-reduced midline closure while facilitating primary donor-site closure. Complete flap survival was achieved in both patients without venous congestion or total flap loss. In the first case, limited wound dehiscence related to hardware prominence was successfully revised with a local V-Y advancement flap, and a small donor-site tension area was managed with split-thickness skin grafting. In the second case, a limited lateral donor defect also required split-thickness skin graft coverage. At 13 months, spinal instrumentation was electively removed in the first patient after adequate osseous stabilization, while flap stability was preserved. At 15 and 9 months of follow-up, respectively, both patients demonstrated durable soft-tissue coverage without recurrent breakdown or cerebrospinal fluid leakage. These cases suggest that bilateral pedicled LAP flaps may facilitate stable, muscle-sparing reconstruction in selected extensive lumbosacral defects, including exceptionally large post-instrumentation wounds and rare adult presentations of lumbosacral myelomeningocele.

Journal
Microsurgery(2026 Sep)
Authors
7名
Type
Case Reports, Journal Article
PubMedで原文を見る
観察研究
MK-04 · PMID 42713086

Retrospective Comparative Analysis of Ponseti Method of Treatment for Idiopathic and Nonidiopathic Clubfoot: A 4 year follow-up study

Abstract / 原文

INTRODUCTION: This study sought to critically evaluate the effectiveness of the Ponseti method in treating idiopathic and non-idiopathic clubfoot, and compared clinical outcomes over a 4-year follow-up. MATERIALS AND METHODS: A total of 75 children (105 clubfeet) were treated with the Ponseti casting protocol at an institution-based clubfoot clinic. Group I comprised 45 patients (63 clubfeet) with idiopathic clubfoot, while Group II included 30 patients (42 clubfeet) with non-idiopathic clubfoot, including arthrogryposis multiplex congenita, flail myelomeningocele, spina bifida, and various syndromic clubfoot. Demographic characteristics, number of casts, tenotomy rate, recurrence, need for minimally invasive adjunct procedures in recurrent or residual cases, and treatment success were compared. Clinical outcomes were assessed using the Pirani score at each follow-up, while brace compliance was based on subjective parent-reported adherence at brace initiation and treatment completion. RESULTS: Group I required significantly fewer casts than Group II (median 5.0 vs. 8.0; P < 0.001), whereas tenotomy rates were comparable (75.5% vs. 73.3%). Multivariable linear regression identified higher initial Pirani score, older age at presentation, rigid non-idiopathic foot type, and recurrent presentation as independent predictors of increased casting requirements. The flail subtype did not differ significantly from idiopathic clubfoot in casting burden. Recurrence was significantly lower in Group I (5/63 [7.9%]) than in Group II (9/42 [21.4%]; P = 0.042). A significant Spearman correlation between initial Pirani score and casting requirement was observed only in the flail subgroup (P = 0.007) of Group II. At final follow-up, treatment success (Pirani score ≤0.5) was achieved in 98.82% of Group I and 95.41% of Group II, P < 0.001. CONCLUSION: The Ponseti method is an effective and reliable treatment for both idiopathic and non-idiopathic clubfoot. In recurrent cases, timely minimally invasive adjunctive procedures may restore correction and reduce the need for extensive surgical release at later stages.

Journal
Journal of orthopaedic case reports(2026 Sep)
Authors
6名
Type
Journal Article
PubMedで原文を見る
不明
MK-05 · PMID 42700585

Total intravenous anesthesia for open fetal myelomeningocele repair: a case series from a single-center in Peru (2017-2025)

Abstract / 原文

BACKGROUND: Open fetal myelomeningocele repair requires maternal general anesthesia and profound uterine relaxation. Total intravenous anesthesia may be an alternative to volatile anesthetics. We describe our anesthetic approach and maternal, fetal, surgical, and neonatal outcomes. METHODS: We retrospectively reviewed 25 cases of open fetal myelomeningocele repairs performed from January 2017 to July 2025. The anesthetic protocol comprised target-controlled infusions of propofol and remifentanil guided by bispectral index monitoring, neuromuscular blockade, intravenous uterine relaxation, and intramuscular fetal atropine, fentanyl, and vecuronium. RESULTS: All 25 procedures were completed under total intravenous anesthesia without use of volatile anesthetics. Combined magnesium sulfate and nitroglycerin were used for uterine relaxation in 60% of cases. Fetal bradycardia occurred in one case (4%), and no cases of maternal pulmonary edema occurred. Median gestational age at delivery was 36 weeks. DISCUSSION: Total intravenous anesthesia combined with intravenous uterine relaxants was feasible for open fetal myelomeningocele repair in this case series.

Journal
International journal of obstetric anesthesia(2026 Aug)
Authors
3名
Type
Journal Article
PubMedで原文を見る
( 02 )TRIALS / JAPAN · 0件

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( 03 )REGISTRY / jRCT

治験をもっと探す

日本の公式レジストリで全件を確認

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