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Biomedicines Jan 2023Fetal and maternal risks associated with open fetal surgery (OFS) in the management of meningomyelocele (MMC) are considerable and necessitate improvement. A modified...
Decreased Maternal Morbidity and Improved Perinatal Results of Magnesium-Free Tocolysis and Classical Hysterotomy in Fetal Open Surgery for Myelomeningocele Repair: A Single-Center Study.
Fetal and maternal risks associated with open fetal surgery (OFS) in the management of meningomyelocele (MMC) are considerable and necessitate improvement. A modified technique of hysterotomy (without a uterine stapler) and magnesium-free tocolysis (with Sevoflurane as the only uterine muscle relaxant) was implemented in our new magnesium-free tocolysis and classical hysterotomy (MgFTCH) protocol. The aim of the study was to assess the introduction of the MgFTCH protocol in reducing maternal and fetal complications. The prospective study cohort (SC) included 64 OFS performed with MgFTCH at the Fetal Surgery Centre Bytom (FSCB) (2015-2020). Fetal and maternal outcomes were compared with the retrospective cohort (RC; = 46), and data from the Zurich Center for Fetal Diagnosis and Therapy (ZCFDT; = 40) and the Children's Hospital of Philadelphia (CHOP; = 100), all using traditional tocolysis. The analysis included five major perinatal complications (Clavien-Dindo classification, C-Dc) which developed before the end of 34 weeks of gestation (GA, gestational age). None of the newborns was delivered before 30 GA. Only two women presented with grade 3 complications and none with 4th or 5th grade (C-Dc). The incidence of perinatal death (3.3%) was comparable with the RC (4.3%) and CHOP data (6.1%). MgFTCH lowers the risk of major maternal and fetal complications.
PubMed: 36830929
DOI: 10.3390/biomedicines11020392 -
BMJ Open Feb 2023Hydrocephalus and myelomeningocele (MMC) place disproportionate burdens of disease on low-income and middle-income countries (LMICs). MMC-associated hydrocephalus and...
INTRODUCTION
Hydrocephalus and myelomeningocele (MMC) place disproportionate burdens of disease on low-income and middle-income countries (LMICs). MMC-associated hydrocephalus and its sequelae result in a spectrum of severely devastating clinical manifestations, for which LMICs are disproportionately unprepared in terms of human, capital and technological resources. This study aims to review and compare the management and outcomes of infant MMC-associated hydrocephalus in LMICs and high-income countries.
METHODS AND ANALYSIS
This systematic review and meta-analysis will follow the Preferred Reporting Items for Systematic Reviews and Meta-Analyses 2020 guidelines. The following databases will be searched without restrictions on language, publication date or country of origin: EMBASE, MEDLINE, The Cochrane Library, Global Index Medicus, African Journals Online and SciELO. All peer-reviewed studies of primary data reporting management and outcomes of infant MMC-associated hydrocephalus will be included. Where high-quality homogeneous studies exist, meta-analyses will be conducted to compare the management and outcomes of MMC-associated hydrocephalus across socioeconomic and geographical regions of the world. The primary outcome will be treatment failure of the first-line hydrocephalus treatment, which we defined operationally as the performance of a second intervention for the same reason as the first. Secondary outcomes include time to failure, rates of mortality and postoperative complications.
ETHICS AND DISSEMINATION
Ethical approval was not applicable because this study does not involve human participants. Dissemination strategies will include publication in a peer-reviewed journal, oral and poster presentations at conferences and an interactive web application to facilitate interaction with the findings and promote the discussion and sharing of findings on social media.
PROSPERO REGISTRATION NUMBER
CRD42021285850.
Topics: Infant; Humans; Developing Countries; Meningomyelocele; Income; Treatment Failure; Research Design; Hydrocephalus; Meta-Analysis as Topic; Systematic Reviews as Topic
PubMed: 36750288
DOI: 10.1136/bmjopen-2022-066339 -
International Journal of Surgery Case... Feb 2023Benign cystic teratoma considered an extragonadal germ cell tumor that can present at any age and mostly located in the anterior mediastinum, only 3 %-8 % are in the...
INTRODUCTION AND IMPORTANCE
Benign cystic teratoma considered an extragonadal germ cell tumor that can present at any age and mostly located in the anterior mediastinum, only 3 %-8 % are in the posterior mediastinum. Meningomyelocele is an abnormal herniation of the meninges that located in most cases posteriorly in lumbosacral spine. Cervicothoracic meningomyelocele are rare entities resemble only 1 %-5 % of all neural tube defects. The presence of both anterior meningomyelocele (MMC) an benign teratoma is very rare and this association in the thoracic column has never been mentioned before in the medical literature.
CASE PRESENTATION
We present the case of a one-year-old child, who was admitted to our hospital with a complaint recurrent vomiting episode, and respiratory distress, with no improvement in symptoms after conservative treatment. Computed tomography showed an anterior heterogeneous meningomyelocele that extend posteriorly to the upper lobe of right lung tissue. MRI confirmed the presence of the meningomyelocele in addition to a heterogenous cystic structure within. Thoracotomy was indicated and the meningomyelocele was carefully resected and sent to histopathology analysis which showed the presence of a benign teratoma accompanying the meningomyelocele.
CLINICAL DISCUSSION
Meningomyeloceles and teratoma are rarely associated, especially in the thoracic spine. Mediastinal tumors should be taken into consideration when a mass is found. A thorough imaging investigations is crucial in establishing the diagnosis along with histopathology after complete resection.
CONCLUSION
In the presence of posterior mediastinal meningomyelocele with heterogeneity, a histopathological examination of the specimen should be performed to exclude the mediastinal tumors.
PubMed: 36737867
DOI: 10.1016/j.ijscr.2023.107914 -
Pediatric Neurosurgery 2023
Letter to the Editor regarding the Article "Comparison of Follow-Up Length-Matched Single-Center Myelomeningocele Postnatal Closure Cohort to the Management of Myelomeningocele Study (MOMS) Trial Results".
Topics: Humans; Meningomyelocele; Follow-Up Studies; Fetus; Hydrocephalus
PubMed: 36626880
DOI: 10.1159/000529013 -
Ultrasound in Obstetrics & Gynecology :... Jun 2023In-utero repair of open neural tube defects (ONTD) is an accepted treatment option with demonstrated superior outcome for eligible patients. While current guidelines... (Review)
Review
OBJECTIVE
In-utero repair of open neural tube defects (ONTD) is an accepted treatment option with demonstrated superior outcome for eligible patients. While current guidelines recommend genetic testing by chromosomal microarray analysis (CMA) when a major congenital anomaly is detected prenatally, the requirement for an in-utero repair, based on the Management of Myelomeningocele Study (MOMS) criteria, is a normal karyotype. In this study, we aimed to evaluate if CMA should be recommended as a prerequisite for in-utero ONTD repair.
METHODS
This was a retrospective cohort study of pregnancies complicated by ONTD that underwent laparotomy-assisted fetoscopic repair or open-hysterotomy fetal surgery at a single tertiary center between September 2011 and July 2021. All patients met the MOMS eligibility criteria and had a normal karyotype. In a subset of the pregnancies (n = 77), CMA testing was also conducted. We reviewed the CMA results and divided the cohort into two groups according to whether clinically reportable copy-number variants (CNV) were detected (reportable-CNV group) or not (normal-CMA group). Surgical characteristics, complications, and maternal and early neonatal outcomes were compared between the two groups. The primary outcomes were fetal or neonatal death, hydrocephalus, motor function at 12 months of age and walking status at 30 months of age. Standard parametric and non-parametric statistical tests were employed as appropriate.
RESULTS
During the study period, 146 fetuses with ONTD were eligible for and underwent in-utero repair. CMA results were available for 77 (52.7%) patients. Of those, 65 (84%) had a normal CMA and 12 (16%) had a reportable CNV, two of which were classified as pathogenic. The first case with a pathogenic CNV was diagnosed with a 749-kb central 22q11.21 deletion spanning low-copy-repeat regions B-D of chromosome 22; the second case was diagnosed with a 1.3-Mb interstitial deletion at 1q21.1q21.2. Maternal demographics, clinical characteristics, operative data and postoperative complications were similar between those with normal CMA results and those with reportable CNVs. There were no significant differences in gestational age at delivery or any obstetric and early neonatal outcome between the study groups. Motor function at birth and at 12 months of age, and walking status at 30 months of age, were similar between the two groups.
CONCLUSIONS
Standard diagnostic testing with CMA should be offered when an ONTD is detected prenatally, as this approach has implications for counseling regarding prognosis and recurrence risk. Our results indicate that the presence of a clinically reportable CNV should not a priori affect eligibility for in-utero repair, as overall pregnancy outcome is similar in these cases to that of cases with normal CMA. Nevertheless, significant CMA results will require a case-by-case multidisciplinary discussion to evaluate eligibility. To generalize the conclusion of this single-center series, a larger, multicenter long-term study should be considered. © 2023 International Society of Ultrasound in Obstetrics and Gynecology.
Topics: Infant, Newborn; Female; Pregnancy; Humans; Child, Preschool; Retrospective Studies; Prenatal Care; Fetus; Meningomyelocele; Microarray Analysis; Prenatal Diagnosis; Multicenter Studies as Topic
PubMed: 36610024
DOI: 10.1002/uog.26152 -
Journal of Pediatric Rehabilitation... 2022This study aimed to describe health care use by type of health providers and care settings visited by children with spina bifida (SB) and to compare this use between...
PURPOSE
This study aimed to describe health care use by type of health providers and care settings visited by children with spina bifida (SB) and to compare this use between children with and without a shunt.
METHODS
Health care use data were extracted from a larger study on the health and functioning of children with SB aged 3-6 years. The present study focused on the medical information subsection of a parent-reported survey related to SB care, general care, specialty care (e.g., neurosurgery), emergency care, and complications related to SB and shunts.
RESULTS
Parents of 101 children with SB participated. Most of the children were male with myelomeningocele and had a shunt. They visited a health care provider for SB care an average of 7.4 times and a specialist an average of 11.9 times in the previous 12 months. Most visited a multidisciplinary clinic for SB-related care and a private physician's office for general care. Children with a shunt had more SB-related medical visits, more visits to a specialist, and a greater number of different types of specialists than those without it. Frequency of emergency room visits did not differ between the two groups. Health providers informed parents about headaches, vomiting, and fever as signs of complications, and some parents did report shunt-related complications.
CONCLUSION
SB is a complex medical condition requiring that children receive medical care from various medical specialists, especially for children with a shunt. Findings on health care use suggest high levels of monitoring and care coordination that parents navigate to care for their child.
Topics: Child; Humans; Male; Child, Preschool; Female; Spinal Dysraphism; Meningomyelocele; Parents; Surveys and Questionnaires; Delivery of Health Care
PubMed: 36530103
DOI: 10.3233/PRM-220027 -
Pediatric Surgery International Dec 2022A TCS after primary closure of meningomyeloceles is a known complication of the spina bifida disease. Data on the outcome after SSCU surgery is heterogeneous and lacking...
INTRODUCTION
A TCS after primary closure of meningomyeloceles is a known complication of the spina bifida disease. Data on the outcome after SSCU surgery is heterogeneous and lacking standardization. Thus we aimed to find a reliable system for assessment of the bladder function before and after SSCU surgery and document postoperative outcome.
METHODS
A retrospective study was performed on a cohort of patients with spina bifida diagnosis. In total, 130 patients underwent 182 SSCU surgeries, 56 of those met our inclusion criteria. A classification system, including two different methods, was used. The AC system used baseline pressure and detrusor over activity to define three levels of bladder dysfunction, the second method ranked the severity of bladder dysfunction by awarding points from 0 to 2 for bladder capacity, maximal detrusor pressure during autonomous contractions, leak point pressure and vesicoureteral reflux A high score is correlated with a severe bladder dysfunction.
RESULTS
Gender distribution was equally (male: n = 29; 51.8%; female: n = 27; 48.2%). The median age at SSCU was 902 years (range 0.5-22.8 years). After SSCU, the stage improved in 11 patients (19.6%), worsened in 11 (19.6%) patients and remained the same in 34 patients (60.7%) after intervention (AC score). Non-worsening was observed in a total of 45 cases (80.4%) (p < 0.001). MHS score (n = 27, 48.2%) improved, remained unchanged (n = 12, 21.4%), 17 patients worsened (30.4%). Non-worsening in postoperative bladder functional outcome was demonstrated in 39 cases (69.6%) over all (p < 0.005). Regardless of whether bladder function is categorized by AC or MHS, postoperative outcome worsened significantly when SSCU was performed due to increasing deterioration in motor function alone (p < 0.05). Of the 24 cases with NOD as indication, 22 (91.7%) had an unchanged (n = 10; 41.7%) or improved (n = 12; 50.0%), meaning positive neuro-orthopedic outcome, only 2 (8.3%) deteriorated (p < 0.001).
CONCLUSION
Our study presents reliable evaluation systems for bladder function in spina bifida patients. Since indications for SSCU surgery differ, it is important to know the possible effects on bladder function after this surgical procedure. Even a mild impairment of bladder function has a risk to deteriorate after SSCU surgery. Particularly interesting becomes this with regard to the fact that the prevalence of TCS might become more frequent with the rising numbers of prenatal closures of meningomyeloceles.
Topics: Pregnancy; Humans; Female; Male; Infant; Child, Preschool; Child; Adolescent; Young Adult; Adult; Urodynamics; Meningomyelocele; Urinary Bladder; Retrospective Studies; Spinal Dysraphism
PubMed: 36454325
DOI: 10.1007/s00383-022-05297-7 -
Journal of Pediatric Rehabilitation... 2022The purpose of this study was to evaluate associations of ethnicity and adaptive function with health-related quality of life (HRQOL) in youths with spina bifida...
PURPOSE
The purpose of this study was to evaluate associations of ethnicity and adaptive function with health-related quality of life (HRQOL) in youths with spina bifida myelomeningocele (SBM).
METHODS
Participants included Hispanic (n = 75) and non-Hispanic White (n = 86) children and adolescents with SBM. Participants completed ratings of adaptive function and SBM-specific HRQOL. A series of linear multiple regression models was computed to investigate whether ethnicity moderates the relation between adaptive function and HRQOL.
RESULTS
Results showed that greater adaptive function was associated with higher HRQOL. While no relations were found between ethnicity and HRQOL, a significant interaction was observed between adaptive function and ethnicity over and above other terms. Although groups did not differ on HRQOL at lower levels of adaptive function, Hispanic participants rated higher HRQOL relative to non-Hispanic White participants at higher levels of adaptive function. Further analysis showed this was true of both nonmotor and motor aspects of adaptive function.
CONCLUSION
Results suggested complex relations between ethnicity and adaptive function with HRQOL, highlighting the importance of optimizing adaptive functioning in youth with SBM.
Topics: Adolescent; Child; Humans; Ethnicity; Hispanic or Latino; Meningomyelocele; Quality of Life; Spinal Dysraphism; White
PubMed: 36442218
DOI: 10.3233/PRM-220021 -
Cureus Oct 2022We present a 21-year-old female with a previously known myelomeningocele who underwent myelomeningocele repair 10 years ago. She presented to the orthopedic outpatient...
We present a 21-year-old female with a previously known myelomeningocele who underwent myelomeningocele repair 10 years ago. She presented to the orthopedic outpatient clinic with bilateral calcaneovalgus deformity, causing non-healing ulcers and multiple hospitalizations for pressure ulcers, cellulitis, and osteomyelitis. She had successful tibialis anterior transfer surgery on her right foot six years ago. The patient arrived for treatment of her left foot deformity to underwent three hours of surgery that was uneventful without any complications and recovered well postoperatively and was discharged on day 2. On week 1, the patient came to the clinic for follow-up; the wound was healthy, placed in the full cast in the equinus position, and referred to physiotherapy. In the third month postoperatively, she was able to tolerate her weight with her foot back to a neutral position with full dorsiflexion.
PubMed: 36420251
DOI: 10.7759/cureus.30576 -
Pediatrics Dec 2022Describe the distribution of weight status categories and determine factors associated with overweight and obesity (OW/OB) in children and adolescents with spina bifida...
OBJECTIVES
Describe the distribution of weight status categories and determine factors associated with overweight and obesity (OW/OB) in children and adolescents with spina bifida (SB) using the National Spina Bifida Patient Registry.
METHODS
Demographic, anthropometric, and clinical data collected from 2009 through 2018 was used to describe the prevalence of OW/OB. The generalized estimating equation model (GEE) identified factors associated with OW/OB among individuals with SB.
RESULTS
Participants (n = 7215) were aged 2 to 19 years (mean = 11.1; standard error, 0.06) and 51.4% female. The majority were non-Hispanic white (57.2%) followed by Hispanic or Latino (25.1%) and non-Hispanic Black (7.5%). The myelomeningocele (MMC) subgroup accounted for 76.3%. Most (60.2%) were community ambulators. The overall percentage of OW/OB was 45.2%, with 49.2% of MMC and 32.0% of nonmyelomeningocele OW/OB. Following the Centers for Disease Control Obesity Severity Classification System, 19.7% of MMC were in class 1, 6.6% in class 2, and 3.5% in class 3. Univariate analysis of MMC participants demonstrated demographic (age, sex, race/ethnicity, and clinic region) and clinical variables (functional level of lesion, ambulation, and number of shunt surgeries) were associated with OW/OB. The GEE model showed that OW/OB was independently, and significantly, associated with age, sex, race/ethnicity, lesion levels, and geographic location of the clinics.
CONCLUSIONS
The demographic and clinical factors associated with OW/OB in children and adolescents with SB further our understanding of factors contributing to the higher prevalence of OW/OB in this population and may inform OW/OB prevention and treatment strategies.
Topics: Adolescent; Child; Female; Humans; Male; Spinal Dysraphism; Overweight; Meningomyelocele; Obesity; Registries
PubMed: 36416007
DOI: 10.1542/peds.2022-057007