Raras
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Mielosquise
ORPHA:645398CID-10 · Q06.8CID-11 · LA02.YDOENÇA RARA

Espinha bífida é uma malformação congênita relativamente comum caracterizada por um fechamento incompleto do tubo neural. Ela faz parte do amplo espectro dos defeitos abertos do tubo neural.

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Introdução

O que você precisa saber de cara

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Forma rara de espinha bífida/defeito do tubo neural (DTN) aberto caracterizada por ausência de um componente quístico, meninges displásicas e placóide neural exposto através de um defeito nos arcos vertebrais posteriores (espinha bífida) que são contíguos à pele circundante. O placóide encontra-se no plano da pele ou abaixo dele e está tipicamente associado a uma malformação de Chiari II. É normalmente isolada ou raramente associada a uma malformação do cordão dividido.

Pesquisas ativas
2 ensaios
3 total registrados no ClinicalTrials.gov
Publicações científicas
239 artigos
Último publicado: 2026 Apr 16
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SUS: Cobertura mínimaScore: 15%
CID-10: Q06.8
🇧🇷Dados SUS / DATASUS
PROCEDIMENTOS SIGTAP (5)
0202010503
Cariótipo — bandas G, Q ou Rgenetic_test
0202010600
Pesquisa de microdeleções/microduplicações por FISHlab_test
0202010694
Sequenciamento completo do exoma (WES)rehabilitation
0202010260
Dosagem de alfa-fetoproteína
0301070040
Atendimento em reabilitação — doenças raras
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Anos de pesquisa1desde 2026
Total histórico239PubMed
Últimos 10 anos131publicações
Pico202124 papers
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2026Hoje · 2026🧪 2020Primeiro ensaio clínico📈 2021Ano de pico
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Publicações mais relevantes

Timeline de publicações
141 papers (10 anos)
#1

Myelomeningocele versus myeloschisis: comparison of brain development and need for CSF diversion.

Journal of neurosurgery. Pediatrics2026 Mar 13

How hydrocephalus rates and brain development differ between myelomeningocele (MMC; with overlying sac) and myeloschisis (without overlying sac) is poorly understood. In this study, the authors compare rates of and age at CSF diversion as well as brain anatomy in patients with MMC and myeloschisis after prenatal and postnatal repair. Demographic and clinical data were retrospectively collected from open spinal dysraphism patients who underwent prenatal or postnatal repair between 2015 and 2024 at Washington University in St. Louis. The need for and timing of permanent CSF diversion and brain imaging characteristics on the first postnatal MRI examination were compared by lesion type (myeloschisis vs MMC). The following imaging characteristics were examined: ventricular anatomy; corpus callosum, massa intermedia, tectum, and septum pellucidum morphology; the presence of gray matter heterotopias; hindbrain herniation; and medullary kinking/compression/displacement below foramen magnum. A total of 98 patients (48% female, 88% Caucasian) were included. There were 21 (21%; 9 prenatal repair, 12 postnatal repair) myeloschisis and 77 (79%; 29 prenatal repair, 48 postnatal repair) MMC patients with similar distribution of lesion levels (p = 0.115). After both prenatal (relative risk [RR] 2.17, 95% CI 1.14-5.62) and postnatal (RR 1.33, 95% CI 1.07-1.65) repair, more patients with myeloschisis than MMC required CSF diversion. Those with prenatally repaired MMC (median 94 days, range 15-154 days) underwent CSF diversion earlier than those with myeloschisis (median 210 days, range 13-357 days) (Hodges-Lehmann Δ114, 95% CI 2-226). However, this was no longer significant after adjusting for fetal atrial diameter, the presence of hindbrain herniation, and medullary compression (β = 75 days, 95% CI -78 to 229). There were minimal differences in brain imaging characteristics except for an increased presence of hindbrain herniation (4 [44%] myeloschisis patients vs 6 [21%] MMC patients; Δ23.8, 95% CI -11.9 to 59.4) and a decreased presence of posterior fossa crowding (2 [22%] myeloschisis patients vs 15 [52%] MMC patients; Δ30.3, 95% CI 3.0-62.1) in patients with myeloschisis versus MMC that was repaired prenatally. Patients with myeloschisis have an increased need for CSF diversion after both prenatal and postnatal repair compared to those with MMC, which may be a consequence of increased rates of hindbrain herniation. The timing of CSF diversion after prenatal repair occurs later in patients with myeloschisis, which may be a consequence of lower rates of medullary compression. These findings suggest that there may be differences in pathophysiology between lesion types and may help with patient counseling.

#2

Complex Type Split Cord Malformation: A Two-Center Study.

Pediatric neurosurgery2026 Mar 17

Complex spinal dysraphism is the condition where multiple dysraphic pathologies are observed in the same patient. Complex type SCM is a compound dysraphic phenotype involving disruptions at multiple developmental stages, characterized by the co-occurrence of SCM and spina bifida aperta. This study aimed to understand the differences between complex SCMs from classical SCM cases in terms of embryological aspects, perioperative management, and follow-up. The study included 44 patients who underwent surgery for complex SCM. Of the patients, 81.8% were operated on during the neonatal period in the same session with spina bifida aperta surgery. Surgery was planned for 11.4% of patients for follow-up purposes, and for 6.8% following detection of SCM on MRI performed due to gait disturbances. It was observed that 68.2% of patients were plegic/severely paretic, 22.7% had moderate paresis, and 9.1% had mild paresis/normally. Regarding the type of spina bifida aperta, 72.7% of patients had myelomeningocele, and 27.3% had myeloschisis. Type 1 SCM was detected in 79.5% of patients, Type 2 SCM in 13.6%, and Type 1.5 SCM in 6.8%. Additionally, 93.2% had tethered cord/thickened-fatty filum terminale, 90.9% had posterior fusion defects, 77.3% had hydrocephalus, 68.2% had kyphoscoliosis, 65.9% had Chiari malformation, 54.5% had syringomyelia, 45.5% had intracranial pathologies other than hydrocephalus, and 4.6% had lipoma. Complex SCMs should not be viewed solely as a spinal pathology; they should be evaluated multidisciplinarily in the perioperative period in terms of anomalies accompanying spina bifida aperta. In these cases are diagnosed at a younger age, the incidence of Type 1 SCM increases, while the frequency of occurrence in the lumbar region decreases and increases in the thoracic region. Furthermore, it has been determined that additional anomalies accompanying spina bifida aperta occur more frequently with complex SCMs.

#3

Prenatal Closure of Myeloschisis vs Myelomeningocele: Insights From the fMMC Consortium Registry.

Neurosurgery2026 Feb 05

Myelomeningocele is defined by the presence of a fluid-filled sac that contains the neural placode and cerebrospinal fluid (CSF) at the level of the spinal defect. Alternatively, the term myeloschisis is used when the neural placode is not contained within a CSF-filled sac. While both are eligible for prenatal closure, few studies have characterized the impact of the type of lesion on postnatal outcomes. This study compares the outcomes of these 2 types of open spinal dysraphism using data from the Fetal Myelomeningocele Consortium registry sponsored by the North American Fetal Therapy Network. The prospective observational Fetal Myelomeningocele Consortium registry was used to extract deidentified data corresponding to patients who underwent fetal closure of myelomeningocele vs myeloschisis. Demographics, operative characteristics, perioperative complications, and postnatal outcomes were collected. A total of 1327 patients were identified, including 991 (74.7%) with myelomeningocele and 336 (25.3%) with myeloschisis. Compared with myelomeningocele, myeloschisis was associated with lower rates of prenatal talipes (11% vs 24%, P < .001) and higher rates of favorable prenatal movement (94% vs 86%, P < .001). Postnatally, myeloschisis conferred higher rates of leg movement (97% vs 90%, P = .002) and an increased likelihood of ambulation in the household (10.4% vs 4.1%, P = .009) and outside (7.2% vs 1.9%, P = .004) at 1 year. However, myeloschisis was also linked to greater use of skin patches during closure (49% vs 27%, P < .001), higher CSF leak rates among those with wound dehiscence (26% vs 8%, P = .014), and more frequent CSF diversion and tethered cord surgeries at follow-up. Myeloschisis and myelomeningocele demonstrate distinct clinical profiles. Although patients with myeloschisis may have favorable motor outcomes, they are also characterized by higher rates of CSF leakage, CSF diversion, and tethered cord surgery. These findings highlight the need for lesion-specific prognostication and may inform surgical planning and parental counseling in the context of prenatal spina bifida closure.

#4

Prenatal Diagnosis to Postnatal Outcomes of Saccular Forms of Closed Spina Dysraphism: A Single Center Retrospective Study.

Prenatal diagnosis2026 Feb

To describe prenatal imaging findings and postnatal outcomes in fetuses diagnosed with saccular forms of closed spinal dysraphism (CSD). This retrospective single-centre study included fetuses diagnosed with non-genetic, non-syndromic CSD between January 2018 and June 2023. Prenatal ultrasound and MRI findings were reviewed, with postnatal outcomes assessed in those managed expectantly, focusing on neurosurgical interventions, motor function, and urologic and bowel outcomes. Eighteen cases were identified; twelve opted for expectant management and had postnatal follow-up. The most common lesion was dysraphic spinal cord lipoma (10/18, 55.6%), followed by limited dorsal myeloschisis (3/18, 16.7%), myelocystocele (3/18, 16.7%) and meningocele (2/18, 11.0%). Chiari malformation and related brain abnormalities were absent at the time of diagnosis in all cases. Most fetuses (88.9%, 16/18) had a low-lying conus medullaris, and 70.6% (12/17) had a sac wall thickness ≥ 2 mm. Postnatally, 75% (9/12) underwent surgical detethering. At ≥ 30 months, all children could walk independently with or without orthoses. However, urologic complications were common in children age ≥ 48 months: 80% (4/5) required clean intermittent catheterization (CIC), 60% (3/5) had urinary incontinence, and 40% (2/5) experienced bowel incontinence. Prenatal imaging features at the lesion level are key for diagnosing CSD. While motor outcomes are favorable, urologic and bowel dysfunctions are frequent in postnatal life.

#5

Serum Prolidase and Ischemia-Modified Albumin Levels in Neural Tube Defects: A Comparative Study of Myelomeningocele, Meningocele, and Myeloschisis.

Medical science monitor : international medical journal of experimental and clinical research2025 Apr 25

BACKGROUND Neural tube defects (NTDs) are congenital malformations resulting from incomplete neural tube closure, leading to severe neurological impairments. Despite advances in prenatal screening and surgical interventions, the biochemical mechanisms underlying NTDs remain unclear. Prolidase, an enzyme involved in collagen metabolism, and ischemia-modified albumin (IMA), a marker of oxidative stress, may play roles in NTD pathogenesis. This study aimed to compare serum prolidase and IMA levels in infants with NTDs and healthy controls to assess their potential contribution to NTD development. MATERIAL AND METHODS A case-control study was conducted, including 45 infants diagnosed with NTDs (myelomeningocele, meningocele, and myeloschisis) and 45 age- and sex-matched healthy controls. Serum prolidase and IMA levels were measured using validated spectrophotometric methods. Statistical analyses were performed to compare biomarker levels between groups and among NTD subtypes. RESULTS Serum prolidase levels were significantly elevated in NTD patients (2.21±0.06 IU/L) compared to controls (1.07±0.04 IU/L, p<0.001). Similarly, serum IMA levels were higher in NTD patients (0.40±0.01 ABSU) than in controls (0.22±0.01 ABSU, p<0.001). No significant differences were observed in biomarker levels among the different NTD subtypes (p>0.05). CONCLUSIONS Elevated prolidase and IMA levels in NTD patients suggest a potential role in NTD pathogenesis, possibly through impaired collagen metabolism and oxidative stress. Further research is needed to explore their diagnostic and therapeutic implications in neural tube defect management.

Publicações recentes

Ver todas no PubMed

📚 EuropePMCmostrando 127

2026

Complex Type Split Cord Malformation: A Two-Center Study.

Pediatric neurosurgery
2026

Myelomeningocele versus myeloschisis: comparison of brain development and need for CSF diversion.

Journal of neurosurgery. Pediatrics
2026

Prenatal Closure of Myeloschisis vs Myelomeningocele: Insights From the fMMC Consortium Registry.

Neurosurgery
2025

Saccular Limited Dorsal Myeloschisis with a Short Stalk at the Base of the Sac Causing Spinal Cord Deviation out of the Spinal Canal.

NMC case report journal
2026

Prenatal Diagnosis to Postnatal Outcomes of Saccular Forms of Closed Spina Dysraphism: A Single Center Retrospective Study.

Prenatal diagnosis
2025

Uncovering the Genetic Landscape of Spinal Dysraphism: A Retrospective Analysis of 150 Fetal Cases.

Prenatal diagnosis
2025

Incomplete cervical limited dorsal myeloschisis with membranous sac: a case report and literature review.

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
2025

Development and Evaluation of a Conceptual Framework for the Use of Fetal Myelomeningocele Repair.

Journal of obstetric, gynecologic, and neonatal nursing : JOGNN
2025

High-fidelity trainer for fetoscopic spina bifida repair.

American journal of obstetrics &amp; gynecology MFM
2025

Morphometric analysis of spina bifida after fetal repair shows new subtypes with associated outcomes.

Ultrasound in obstetrics &amp; gynecology : the official journal of the International Society of Ultrasound in Obstetrics and Gynecology
2025

Isolation and characterization of human cKIT positive amniotic fluid stem cells obtained from pregnancies with spina bifida.

Scientific reports
2025

[Limited dorsal myeloschisis: From antenatal screening to postnatal evolution. About a series of cases from the Grand Est, Bourgogne and Franche-Comté].

Gynecologie, obstetrique, fertilite &amp; senologie
2025

Saccular Limited Dorsal Myeloschisis: A Distinct Prenatal Entity.

Fetal diagnosis and therapy
2025

Serum Prolidase and Ischemia-Modified Albumin Levels in Neural Tube Defects: A Comparative Study of Myelomeningocele, Meningocele, and Myeloschisis.

Medical science monitor : international medical journal of experimental and clinical research
2025

Detailed analysis of hydrocephalus patterns and associated variables in patients after open fetal repair and postnatal myelomeningocele/myeloschisis closure.

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
2025

Outcome of Children With Prenatally Diagnosed Saccular Limited Dorsal Myeloschisis: The Importance of Accurate Diagnosis.

Prenatal diagnosis
2025

Cervical wart-like cutaneous appendage with a contiguous stalk of limited dorsal myeloschisis treated with untethering after long-term follow-up.

Surgical neurology international
2025

Fetoscopic repair of open spina bifida between 26 0/7 and 27 6/7 gestational weeks and postnatal cerebrospinal fluid diversion.

The journal of maternal-fetal &amp; neonatal medicine : the official journal of the European Association of Perinatal Medicine, the Federation of Asia and Oceania Perinatal Societies, the International Society of Perinatal Obstetricians
2025

Perinatal Outcomes in Patients With Neural Tube Defects in a Middle-Income Setting.

Birth defects research
2025

Neonatal diagnosis of cervical limited dorsal myeloschisis.

Anales de pediatria
2025

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

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
2025

Atypical Variants of Spinal Dysraphism: A Case Series.

Journal of the West African College of Surgeons
2025

Acrania with exencephaly and spinal Myeloschisis in a young pregnant woman from Rural Nepal: A curious case report.

Radiology case reports
2025

The clinical significance of lack of hindbrain herniation in fetal myelomeningocele/myeloschisis patients.

Journal of neurosurgery. Pediatrics
2025

Pituitary Gland Duplication Syndrome: An International Imaging Analysis.

AJNR. American journal of neuroradiology
2024

The effect of using synthetic vs. biological dural substitutes during prenatal and postnatal repair of spina bifida on spinal cord tethering-a review of literature.

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
2024

Proposal for standardized prenatal assessment of fetal open dysraphisms by the European reference network for Intellectual disability, TeleHealth, Autism and Congenital Anomalies (ITHACA) and eUROGEN.

Prenatal diagnosis
2024

Spinal epidermoid cyst associated with limited dorsal myeloschisis.

Surgical neurology international
2024

Periventricular nodular heterotopia in patients with a prenatal diagnosis of myelomeningocele/myeloschisis: associations with seizures and neurodevelopmental outcomes during early childhood.

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
2024

Immunohistochemical and Histopathological Characterization of Spina Bifida Defect Tissues Removed After Prenatal and Postnatal Surgical Repair.

Fetal and pediatric pathology
2024

Mirror movements associated with cervical limited dorsal myeloschisis: a unique case study.

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
2025

Differences between Myeloschisis and Myelomeningocele in Patients Undergoing Prenatal Repair of Open Spina Bifida.

Fetal diagnosis and therapy
2024

Revisiting MOMS criteria for prenatal repair of spina bifida: upper gestational-age limit should be raised and assessment of prenatal motor function rather than anatomical level improves prediction of postnatal function.

Ultrasound in obstetrics &amp; gynecology : the official journal of the International Society of Ultrasound in Obstetrics and Gynecology
2024

Cervical saccular limited dorsal myeloschisis, so-called "cervical myelomeningocele": long-term follow-up of a single-center series and systematic review.

Journal of neurosurgery. Pediatrics
2023

Parental perception of contrast enhanced voiding ultrasonography urodynamics vs fluoroscopic urodynamics.

Journal of pediatric urology
2023

Focal Spinal Nondisjunctional Disorders: Including a Discussion on the Embryogenesis of Cranial Focal Nondisjunctional Lesions.

Advances and technical standards in neurosurgery
2024

Ruptured saccular limited dorsal myeloschisis: good indication for fetal repair.

Ultrasound in obstetrics &amp; gynecology : the official journal of the International Society of Ultrasound in Obstetrics and Gynecology
2024

Limited exposure to preserve stability and achieve complete excision of limited dorsal myeloschisis - the "Skip-Hop Laminectomy" technique: a technical note.

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
2023

Assessment of longitudinal brain development using super-resolution magnetic resonance imaging following fetal surgery for open spina bifida.

Ultrasound in obstetrics &amp; gynecology : the official journal of the International Society of Ultrasound in Obstetrics and Gynecology
2023

Biocellulose patch technique for fetoscopic repair of open spina bifida in twin pregnancy.

Ultrasound in obstetrics &amp; gynecology : the official journal of the International Society of Ultrasound in Obstetrics and Gynecology
2023

Prenatal predictors of need for cerebrospinal fluid diversion in infants following prenatal repair of open spina bifida; systematic review and meta-analysis.

American journal of obstetrics &amp; gynecology MFM
2023

Limited dorsal myeloschisis associated with intramedullary infantile hemangioma in the conus medullaris: illustrative case.

Journal of neurosurgery. Case lessons
2023

Midline suprapineal pseudocyst in brain of fetuses with open spina bifida.

Ultrasound in obstetrics &amp; gynecology : the official journal of the International Society of Ultrasound in Obstetrics and Gynecology
2023

Absence of a Hernia Sack in Patients Undergoing Prenatal Repair of Spina Bifida Increases the Risk of Developing Shunt-Dependent Hydrocephalus.

Diagnostics (Basel, Switzerland)
2023

Fetal MRI prior to intrauterine surgery of open neural tube defects: What does the radiologist need to know.

La Radiologia medica
2023

Early outcomes of a myofascial repair technique for fetal myelomeningocele.

Journal of pediatric surgery
2021

Atlantoaxial limited dorsal myeloschisis: A report of two cases and review of literature.

Brain &amp; spine
2023

Fetal spinal cord tractography in a case of limited dorsal myeloschisis.

Ultrasound in obstetrics &amp; gynecology : the official journal of the International Society of Ultrasound in Obstetrics and Gynecology
2022

Teaching NeuroImage: Prenatal Diagnosis of Limited Dorsal Myeloschisis.

Neurology
2022

Disorders of Secondary Neurulation: Suggestion of a New Classification According to Pathoembryogenesis.

Advances and technical standards in neurosurgery
2023

Limited dorsal myeloschisis without extradural stalk continuity to coexisting congenital dermal sinus.

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
2022

Limited dorsal myeloschisis involving one hemicord of a split cord malformation - a "hemi-LDM".

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
2022

Percutaneous/mini-laparotomy fetoscopic repair of open spina bifida: a novel surgical technique.

American journal of obstetrics and gynecology
2022

Primary vs patch-based skin closure for in-utero spina bifida repair.

Ultrasound in obstetrics &amp; gynecology : the official journal of the International Society of Ultrasound in Obstetrics and Gynecology
2022

Giant limited dorsal myeloschisis may be associated with normal functional prognosis.

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
2021

Saccular Limited Dorsal Myeloschisis with Spinal Cord Deviation out of the Spinal Canal to the Sac.

NMC case report journal
2022

The Absence of the Neuronal Component in Limited Dorsal Myeloschisis: A Case Report.

Pediatric neurosurgery
2021

The Myelic Limited Dorsal Malformation: Prenatal Ultrasonographic Characteristics of an Intermediate Form of Dysraphism.

Fetal diagnosis and therapy
2021

Congenital Cervical Tethered Spinal Cord in Adults: Recognition, Surgical Technique, and Literature Review.

World neurosurgery
2021

Postnatal Repair of Open Neural Tube Defects: A Single Center with 90-Month Interdisciplinary Follow-Up.

Journal of clinical medicine
2021

Spinal cord deformity with aggravation of tethering in saccular limited dorsal myeloschisis during the first 2 months of life.

Surgical neurology international
2022

Cerebral Abnormalities in Spina Bifida: A Neuropathological Study.

Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society
2021

Surgical histopathology of a filar anomaly as an additional tethering element associated with closed spinal dysraphism of primary neurulation failure.

Surgical neurology international
2022

Prevalence of supratentorial anomalies assessed by magnetic resonance imaging in fetuses with open spina bifida.

Ultrasound in obstetrics &amp; gynecology : the official journal of the International Society of Ultrasound in Obstetrics and Gynecology
2022

Extensive cervicothoracic posterior arch defects from C1 to T6 leading to myelopathy due to thoracic kyphosis in an adolescent boy.

Spine deformity
2021

Factors Affecting the Intensive Care Stay of Patients with Spinal Neural Tube Defects.

Turkish neurosurgery
2021

Impact of the cystic neural tube defects on fetal motor function in prenatal myelomeningocele repairs: A retrospective cohort study.

Prenatal diagnosis
2021

Experience of 300 cases of prenatal fetoscopic open spina bifida repair: report of the International Fetoscopic Neural Tube Defect Repair Consortium.

American journal of obstetrics and gynecology
2021

Open fetal surgery for myelomeningocele repair in France.

Journal of gynecology obstetrics and human reproduction
2021

Disorders of Secondary Neurulation : Mainly Focused on Pathoembryogenesis.

Journal of Korean Neurosurgical Society
2021

Percutaneous fetoscopic spina bifida repair: effect on ambulation and need for postnatal cerebrospinal fluid diversion and bladder catheterization.

Ultrasound in obstetrics &amp; gynecology : the official journal of the International Society of Ultrasound in Obstetrics and Gynecology
2021

Focal Spinal Nondisjunction in Primary Neurulation : Limited Dorsal Myeloschisis and Congenital Spinal Dermal Sinus Tract.

Journal of Korean Neurosurgical Society
2021

Maternal serum alpha-fetoprotein level and the relationship to ventriculomegaly in fetal neural tube defect: A retrospective cohort study.

European journal of obstetrics, gynecology, and reproductive biology
2021

Systematic Classification of Spina Bifida.

Journal of neuropathology and experimental neurology
2021

Fetal surgery for spina bifida in Zurich: results from 150 cases.

Pediatric surgery international
2020

Congenital Dermal Sinus Elements in Each Tethering Stalk of Coexisting Thoracic Limited Dorsal Myeloschisis and Retained Medullary Cord.

Pediatric neurosurgery
2020

Open Fetal Surgical Outcomes for Myelomeningocele Closure Stratified by Maternal Body Mass Index in a Large Single-Center Cohort.

Fetal diagnosis and therapy
2021

Systematic classification of maternal and fetal intervention-related complications following open fetal myelomeningocele repair - results from a large prospective cohort.

BJOG : an international journal of obstetrics and gynaecology
2023

Split cord malformation associated with congenital dermoid cyst and myeloschisis - case-based literature review on possible embryonic derivation and implications.

British journal of neurosurgery
2021

Prenatal predictors of motor function in children with open spina bifida: a retrospective cohort study.

BJOG : an international journal of obstetrics and gynaecology
2020

Development of a Core outcome set for fetal Myelomeningocele (COSMiC): study protocol.

Trials
2021

Early de-tethering: analysis of urological and clinical consequences in a series of 40 children.

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
2020

Limited dorsal myeloschisis in three cats: a distinctive form of neural tube defect.

JFMS open reports
2021

Shunt timing in meningomyelocele and clinical results: analysis of 80 cases.

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
2020

Neurosurgical Pathology and Management of Limited Dorsal Myeloschisis Associated with Congenital Dermal Sinus in Infancy.

Pediatric neurosurgery
2021

Amniotic band syndrome associated with limited dorsal myeloschisis: a case report of an unusual case and review of the literature.

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
2020

Limited dorsal myeloschisis with a contiguous stalk to human tail-like cutaneous appendage, associated with a lipoma of conus medullaris: A case report.

International journal of surgery case reports
2021

Impact of the size of the lesion in prenatal neural tube defect repair on imaging, neurosurgical and motor outcomes: a retrospective cohort study.

BJOG : an international journal of obstetrics and gynaecology
2020

Perspectives on Spinal Dysraphism : Past, Present, and Future.

Journal of Korean Neurosurgical Society
2020

Enlargement of Extraspinal Cysts in Spinal Dysraphism : A Reason for Early Untethering.

Journal of Korean Neurosurgical Society
2020

Spinal Dysraphism in the Last Two Decades : What I Have Seen during the Era of Dynamic Advancement.

Journal of Korean Neurosurgical Society
2020

Myelomeningocele sac associated with worse lower-extremity neurological sequelae: evidence for prenatal neural stretch injury?

Ultrasound in obstetrics &amp; gynecology : the official journal of the International Society of Ultrasound in Obstetrics and Gynecology
2020

Terminal syringomyelia associated with lumbar limited dorsal myeloschisis.

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
2020

Retained medullary cord with sacral subcutaneous meningocele and congenital dermal sinus.

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
2020

Benchmarking against the MOMS Trial: Zurich Results of Open Fetal Surgery for Spina Bifida.

Fetal diagnosis and therapy
2019

MOMS Plus: Single-Institution Review of Outcomes for Extended BMI Criteria for Open Fetal Repair of Myelomeningocele.

Fetal diagnosis and therapy
2019

Human tail-like cutaneous appendage with a contiguous stalk of limited dorsal myeloschisis.

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
2020

Commentary: Limited Dorsal Myeloschisis: Reconsideration of its Embryological Origin.

Neurosurgery
2019

Limited Dorsal Myeloschisis with and without Type I Split Cord Malformation: Report of 3 Cases and Surgical Nuances.

Medicina (Kaunas, Lithuania)
2020

Limited Dorsal Myeloschisis: Reconsideration of its Embryological Origin.

Neurosurgery
2019

Slender Stalk with Combined Features of Saccular Limited Dorsal Myeloschisis and Congenital Dermal Sinus in a Neonate.

Pediatric neurosurgery
2018

Myelomeningocele Versus Myelocele on Fetal MR Images: Are There Differences in Brain Findings?

AJR. American journal of roentgenology
2019

Dermoid cyst with cerebellar meningoencephalocele at different locations accompanied by posterior fossa abnormalities: case report.

Sao Paulo medical journal = Revista paulista de medicina
2018

Limited dorsal myeloschisis with no extradural stalk linking to a flat skin lesion: a case report.

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
2019

Surgical histopathology of limited dorsal myeloschisis with flat skin lesion.

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
2019

Congenital Dermal Sinus and Limited Dorsal Myeloschisis: "Spectrum Disorders" of Incomplete Dysjuction Between Cutaneous and Neural Ectoderms.

Neurosurgery
2018

A case of junctional neural tube defect associated with a lipoma of the filum terminale: a new subtype of junctional neural tube defect?

Journal of neurosurgery. Pediatrics
2018

Percutaneous fetoscopic closure of large open spina bifida using a bilaminar skin substitute.

Ultrasound in obstetrics &amp; gynecology : the official journal of the International Society of Ultrasound in Obstetrics and Gynecology
2018

In utero Plastic Surgery in Zurich: Successful Use of Distally Pedicled Random Pattern Transposition Flaps for Definitive Skin Closure during Open Fetal Spina Bifida Repair.

Fetal diagnosis and therapy
2018

Neurosurgical pathology of limited dorsal myeloschisis.

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
2017

Fetal surgery for myelomeningocele: After the Management of Myelomeningocele Study (MOMS).

Seminars in fetal &amp; neonatal medicine
2018

A case of prenatally diagnosed limited dorsal myeloschisis with good prognosis.

Journal of clinical ultrasound : JCU
2018

Amniotic Fluid Concentrations of Glial Fibrillary Acidic Protein Do Not Correlate with Prenatal Metrics in Fetuses with Myelomeningocele.

Fetal diagnosis and therapy
2017

Prenatal surgery for myelomeningocele: review of the literature and future directions.

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
2017

Evolution of posterior fossa and brain morphology after in utero repair of open neural tube defects assessed by MRI.

European radiology
2017

Modification of surgical procedure for "probable" limited dorsal myeloschisis.

Journal of neurosurgery. Pediatrics
2017

Multiple neural tube defects: a rare combination of limited dorsal myeloschisis, diplomyelia with dorsal bony spur, sacral meningocoele, syringohydromyelia, and tethered cord.

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
2017

Limited Dorsal Myeloschisis and Congenital Dermal Sinus: Comparison of Clinical and MR Imaging Features.

AJNR. American journal of neuroradiology
2017

Limited dorsal myeloschisis associated with dermoid elements.

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
2016

Cryopreserved Human Umbilical Cord for In Utero Myeloschisis Repair.

Obstetrics and gynecology
2017

Limited Dorsal Myeloschisis: A Diagnostic Pitfall in the Prenatal Ultrasound of Fetal Dysraphism.

Fetal diagnosis and therapy
2015

Pediatric encephaloceles: A series of 20 cases over a period of 3 years.

Journal of pediatric neurosciences
2016

Hemimyeloschisis associated with hydrocephalus.

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
2016

[Preliminary results from the French study on prenatal repair for fetal myelomeningoceles (the PRIUM study)].

Journal de gynecologie, obstetrique et biologie de la reproduction
2015

A practical clinical classification of spinal neural tube defects.

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
2015

Missed limited dorsal myeloschisis: an unfortunate cause for recurrent tethered cord syndrome.

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
2015

Factors affecting infection development after meningomyelocele repair in newborns and the efficacy of antibiotic prophylaxis.

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery

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Referências e fontes

Bases de dados externas citadas neste artigo

Publicações científicas

Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.

  1. Myelomeningocele versus myeloschisis: comparison of brain development and need for CSF diversion.
    Journal of neurosurgery. Pediatrics· 2026· PMID 41825066mais citado
  2. Complex Type Split Cord Malformation: A Two-Center Study.
    Pediatric neurosurgery· 2026· PMID 41843714mais citado
  3. Prenatal Closure of Myeloschisis vs Myelomeningocele: Insights From the fMMC Consortium Registry.
    Neurosurgery· 2026· PMID 41642007mais citado
  4. Prenatal Diagnosis to Postnatal Outcomes of Saccular Forms of Closed Spina Dysraphism: A Single Center Retrospective Study.
    Prenatal diagnosis· 2026· PMID 41419435mais citado
  5. Serum Prolidase and Ischemia-Modified Albumin Levels in Neural Tube Defects: A Comparative Study of Myelomeningocele, Meningocele, and Myeloschisis.
    Medical science monitor : international medical journal of experimental and clinical research· 2025· PMID 40275547mais citado
  6. Saccular Limited Dorsal Myeloschisis: A New Indication for Prenatal Surgery? Case Series and Scoping Review.
    Fetal Diagn Ther· 2026· PMID 41989971recente
  7. Fetoscopic myelomeningocele repair: standard technique and approaches for closure of large defects.
    Neurosurg Focus Video· 2026· PMID 41953229recente
  8. Hybrid approach to closure in fetoscopic myelomeningocele repair.
    Neurosurg Focus Video· 2026· PMID 41953228recente
  9. Open fetal myeloschisis closure: pearls for uterine exposure and closure.
    Neurosurg Focus Video· 2026· PMID 41953226recente
  10. Fetoscopic repair of myeloschisis: optimizing the three-layer closure.
    Neurosurg Focus Video· 2026· PMID 41953225recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:645398(Orphanet)
  2. MONDO:0958076(MONDO)
  3. Busca completa no PubMed(PubMed)
  4. Artigo Wikipedia(Wikipedia)

Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.

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ORPHA:645398 · MONDO:0958076
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Q06.8 · Outras malformações congênitas especificadas da medula espinal
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