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Nigerian Journal of Clinical Practice Apr 2024Tethered cord syndrome is a neurological disorder closely associated with congenital spinal dysraphism. Aberrant dorsal nerve roots may be one of the possible and...
Tethered cord syndrome is a neurological disorder closely associated with congenital spinal dysraphism. Aberrant dorsal nerve roots may be one of the possible and relatively rare tethering pathologies, especially in the complex form of occult spinal dysraphism such as caudal regression syndrome or split cord malformation. We report an illustrative case of caudal regression syndrome with spinal cord tethering due to a combination of a contiguous bundle of an aberrant dorsal nerve root, and a dorsal-type lipomyelomeningocele, with a thickened filum terminale treated with microneurosurgical untethering.
Topics: Humans; Male; Cauda Equina; Magnetic Resonance Imaging; Meningomyelocele; Neural Tube Defects; Spinal Cord; Spinal Nerve Roots; Infant
PubMed: 38679779
DOI: 10.4103/njcp.njcp_838_23 -
CEN Case Reports Apr 2024Distal renal tubular acidosis (dRTA) is a clinical picture of hyperchloremic hypokalemic metabolic acidosis with a normal anion gap. It can be caused by a variety of...
Distal renal tubular acidosis (dRTA) is a clinical picture of hyperchloremic hypokalemic metabolic acidosis with a normal anion gap. It can be caused by a variety of conditions including obstructive uropathy such as vesicoureteral reflux (VUR). We report a rare case of dRTA secondary to VUR in a 4-year-old girl with a history of meningomyelocele, neurogenic bladder and recurrent urinary tract infections. She was admitted to the hospital with complaints of polydipsia, polyuria, and inability to gain weight for the last 1 year. She was on prophylactic antibiotic treatment with clean intermittent catheterization and anticholinergic drug. She had a history of subureteral injection of various agents and botulin toxin injection into the bladder. Her voiding cystourethrogram revealed grade 5 VUR in the left kidney, tortuosity in the left ureter, and the bladder had a dome-like appearance and was trabeculated. When all laboratory values of the patient since birth were examined, it was observed that urine pH was high despite hypokalemic hyperchloremic metabolic acidosis for the last year; these abnormalities became more severe in the last few months. In conclusion, the development of hypokalemia and nephrolithiasis/nephrocalcinosis along with metabolic acidosis in a patient diagnosed with VUR should be considered as an indicator of impaired tubular functions. Also, the possibility of an underlying VUR in the presence of recurrent urinary tract infection in a patient diagnosed with dRTA should not be ignored.
PubMed: 38637460
DOI: 10.1007/s13730-024-00873-3 -
European Journal of Medical Research Mar 2024The current convention for treatment of children with myelomeningocele (MMC) is timely surgical intervention combined with long-term follow-up by a multidisciplinary...
BACKGROUND
The current convention for treatment of children with myelomeningocele (MMC) is timely surgical intervention combined with long-term follow-up by a multidisciplinary specialized team. This study aims to investigate the outcomes of MMC patients treated at Namazi Hospital.
METHODS
All children presenting to Namazi Hospital with myelomeningocele between May 2001 and August 2020 were eligible for this study. For those with a documented telephone number, follow-up phone surveys with the patient's caregivers, on top of the review of the medical documents were carried out to assess mortality, morbidities, and the functional outcome of the care provided to them.
RESULTS
A total of 125 patients were studied (62 females). All of the patients were followed up for a mean duration of 6.28 years (range 1-23 years). The majority were located in the lumbosacral area. All of the patients underwent postnatal surgical intervention for MMC in Namazi Hospital. Mean age at surgery was 9.51 days. There were statistically significant differences between urinary and bowel incontinence and presence of scoliosis, MMT grading of the lower limbs, school attendance, number of readmissions, and requirement of laminectomy at the initial surgical intervention.
CONCLUSIONS
This study is the first to characterize the long-term outcomes of MMC patients in Iran. This study illustrates that there is a great need for improved access to and coordination of care in antenatal, perioperative, and long-term stages to improve morbidity and mortality.
Topics: Child; Humans; Female; Pregnancy; Infant, Newborn; Meningomyelocele; Follow-Up Studies; Iran; Referral and Consultation; Retrospective Studies
PubMed: 38528560
DOI: 10.1186/s40001-024-01667-0 -
Brazilian Journal of Anesthesiology... 2024Myelomeningocele (MMC) is a neural tube defect disease. Antenatal repair of fetal MMC is an alternative to postnatal repair. Many agents can be used as tocolytics during... (Comparative Study)
Comparative Study
BACKGROUND
Myelomeningocele (MMC) is a neural tube defect disease. Antenatal repair of fetal MMC is an alternative to postnatal repair. Many agents can be used as tocolytics during the in utero fetal repair such as β2-agonists and oxytocin receptor antagonists, with possible maternal and fetal repercussions. This study aims to compare maternal arterial blood gas analysis between terbutaline or atosiban, as tocolytic agents, during intrauterine MMC repair.
METHODS
Retrospective cohort study. Patients were divided into two groups depending on the main tocolytic agent used during intrauterine MMC repair: atosiban (16) or terbutaline (9). Maternal arterial blood gas samples were analyzed on three moments: post induction (baseline, before the start of tocolysis), before extubation, and two hours after the end of the surgery.
RESULTS
Twenty-five patients were included and assessed. Before extubation, the terbutaline group showed lower arterial pH (7.347 ± 0.05 vs. 7.396 ± 0.02 for atosiban, p = 0.006) and higher arterial lactate (28.33 ± 12.76 mg.dL vs. 13.06 ± 6.35 mg.dL, for atosiban, p = 0.001) levels.
CONCLUSIONS
Patients who received terbutaline had more acidosis and higher levels of lactate, compared to those who received atosiban, during intrauterine fetal MMC repair.
Topics: Humans; Retrospective Studies; Terbutaline; Female; Meningomyelocele; Adult; Tocolytic Agents; Pregnancy; Vasotocin; Cohort Studies; Blood Gas Analysis
PubMed: 38521500
DOI: 10.1016/j.bjane.2024.844495 -
Cognition and emotional distress in middle-aged and older adults with spina bifida myelomeningocele.PloS One 2024To investigate cognitive functioning and emotional distress in adults aged 55 to 68 years old with spina bifida myelomeningocele (SBM), both with and without...
PURPOSE
To investigate cognitive functioning and emotional distress in adults aged 55 to 68 years old with spina bifida myelomeningocele (SBM), both with and without hydrocephalus. A secondary aim was to explore the associations between psychosocial factors in relation to emotional distress.
MATERIALS AND METHODS
Cross-sectional study of eleven females and eight males with SBM, five with and twelve without hydrocephalus. Cognitive functioning was investigated with neuropsychological tests and self-report measures. Furthermore, participants completed questionnaires regarding resilience, access to social support, coping, and emotional distress. Descriptive statistics were applied, and Spearman Rho correlation coefficients were used to explore the relationships between psychosocial factors and emotional distress.
RESULTS
Eleven exhibited normal cognitive functioning. An observed difference was seen between participants with and without hydrocephalus, where six and five persons reported clinical levels of depression and anxiety, respectively. Positive perceptions of self and future were associated with lower levels of depression and anxiety.
CONCLUSION
This study adds important information about cognitive functioning and emotional distress in an understudied population. The results indicated normal cognitive functioning in adults aged 55 to 68 years with SBM without hydrocephalus. Prevalence of emotional distress was comparable with previous studies of younger adults with SBM. There is a need for longitudinal studies investigating cognition and psychological health to fully capture important aspects of the life course of SBM with and without hydrocephalus.
Topics: Female; Male; Middle Aged; Humans; Aged; Meningomyelocele; Cross-Sectional Studies; Spinal Dysraphism; Psychological Distress; Cognition; Hydrocephalus
PubMed: 38422087
DOI: 10.1371/journal.pone.0298891 -
Journal of Orthopaedic Surgery and... Feb 2024Neural tube defects are the most common congenital disorders after cardiac anomalies. Lumbar kyphosis deformity is observed in 8-15% of these patients. This deformity...
OBJECTIVE
Neural tube defects are the most common congenital disorders after cardiac anomalies. Lumbar kyphosis deformity is observed in 8-15% of these patients. This deformity severely limits the daily lives of these patients. In our study, we aimed to correct the kyphosis angle of the patients with lumbar kyphosis associated with myelomeningocele (MMC) and allow them to continue their growth without limiting their lung capacity by applying kyphectomy and sliding growing rod technique.
PATIENTS AND METHODS
In this study, we retrospectively evaluated 24 patients with congenital lumbar kyphosis deformity associated with MMC, aged between 4 and 9 years, and who applied to Umraniye Training and Research Hospital between the dates of 2018 and 2021. We evaluated preoperative and postoperative kyphosis angles, correction rates, bleeding during operations, operation time, level of instrumentation, number of the resected vertebrae, initial levels of the posterior defects, duration of hospital stays, annual lengthening, and weight of the patients.
RESULTS
Mean age was 5.04 (between 4 and 9). Mean preoperative and early postoperative kyphosis angles were 129.8° (87-175°) and 0.79° (- 20-24°), respectively. The kyphotic deformity correction rate was 99.1%. A difference was found regarding kyphosis measurements between preoperative and early period values (p < 0.05). The annual height lengthening of patients was calculated as 0.74 cm/year and 0.77 cm/year between T1-T12 and T1-S1, respectively. Mean preoperative level of hemoglobin (Hgb) was 11.95, postoperative Hgb value was 10.02, and the decrease was significant (p < 0.05). In terms of complications, 50% (12) had broken/loosen screws, 50% (12) had undergone debridement surgery, 37.5% (9) had vacuum-assisted closure therapy, and 33.3% (8) had to get all of their implants removed.
CONCLUSION
We believe that our sliding growing rod technique is a new and updated surgical method that can be applied in these patient groups, facilitating the life, rehabilitation process, and daily care of MMC patients with lumbar kyphosis. This technique seems to be a safe and reliable method which preserves lung capacity and allows lengthening.
Topics: Humans; Child, Preschool; Child; Meningomyelocele; Retrospective Studies; Kyphosis; Spine; Scoliosis; Treatment Outcome; Lumbar Vertebrae; Spinal Fusion
PubMed: 38308272
DOI: 10.1186/s13018-024-04577-3 -
AJOG Global Reports Feb 2024Prepregnancy obesity and racial-ethnic disparities has been shown to be associated with meningomyelocele.
BACKGROUND
Prepregnancy obesity and racial-ethnic disparities has been shown to be associated with meningomyelocele.
OBJECTIVE
This study aimed to investigate the association of maternal periconceptional factors, including race-ethnicity and prepregnancy body mass index, with the prevalence of isolated fetal myelomeningocele.
METHODS
This was a population-based cross-sectional study using Centers for Disease Control and Prevention birth data from 2016 to 2021. Major structural anomalies or chromosomal abnormalities were excluded. Race-ethnicity was classified as non-Hispanic White (reference population), non-Hispanic Black, non-Hispanic Asian, Hispanic, and others. Maternal prepregnancy body mass index was classified as underweight (<18.5 kg/m), normal (reference group; 18.5-24.9 kg/m), overweight (25-29.9 kg/m), and class I (30-34.9 kg/m), class II (35-39.9 kg/m), and class III obesity (≥40 kg/m). A chi-square test of independence was performed to identify factors significantly associated with myelomeningocele. These factors were then stratified into 3 adjusted clusters/levels. The prevalence was calculated per 10,000 live births. The Cochran-Armitage test for trend was used to detect any significant increasing or decreasing trends.
RESULTS
A total of 22,625,308 pregnancies with live birth, including 2866 pregnancies with isolated fetal myelomeningocele, were included in the analysis. The prevalence of isolated fetal myelomeningocele per 10,000 live births varied among different racial/ethnic groups, with the highest prevalence found among the non-Hispanic White (1.60 [1.52-1.67]) and lowest among the non-Hispanic Asian (0.50 [0.40-0.64]) population. The prevalence significantly increased with body mass index, with the highest prevalence found in the population with class III obesity (1.88 per 10,000 live births). Subgroup analysis of the associations between the significant variables (obesity, diabetes, hypertension, and education) and each ethnicity in cases with myelomeningocele showed significant variations in prevalence of these variables among different racial/ethnic groups. Following the model with the 3 levels of adjustment described in the Methods section, prepregnancy overweight and class I, II, and III obesity remained significantly associated with the odds of isolated fetal myelomeningocele. The adjusted odds ratios were 1.32 (95% confidence interval, 1.19-1.46; <.001) for overweight, 1.55 (95% confidence interval, 1.38-1.75; <.001) for class I obesity, 1.68 (95% confidence interval, 1.45-1.94; <.001) for class II obesity, and 1.73 (95% confidence interval, 1.47-2.04; <.001) for class III obesity. Similarly, following the 3-level adjustment model, the obesity-mediated effect of maternal race-ethnicity on the odds of myelomeningocele remained significant (non-Hispanic Black: adjusted odds ratio, 1.03; 95% confidence interval, 1.02-1.05; <.001; non-Hispanic Asian: adjusted odds ratio, 1.02; 95% confidence interval, 1.01-1.03; <.001; Hispanic: adjusted odds ratio, 1.5; 95% confidence interval, 1.03-1.6; <.001). The test for trend among different racial/ethnic groups did not show significant results across the past 6 years. However, the test for trend showed a significant increase in the prevalence of isolated myelomeningocele associated with class II and III obesity over the past 6 years.
CONCLUSION
There has been a rising trend of fetal isolated myelomeningocele in pregnancies with maternal class II and III obesity over the past 6 years after adjusting for other covariates. Prepregnancy obesity, a modifiable risk factor, is a significant driver of racial/ethnic disparities in the overall risk for isolated fetal myelomeningocele.
PubMed: 38274636
DOI: 10.1016/j.xagr.2023.100290 -
Cureus Dec 2023Myelomeningoceles with soft tissue defects are often difficult to close primarily and require reconstructive surgery. Furthermore, cases with large skin defects or cases...
Myelomeningoceles with soft tissue defects are often difficult to close primarily and require reconstructive surgery. Furthermore, cases with large skin defects or cases with kyphosis of the spine require a large area to be covered, making reconstruction even more difficult. We performed closure of soft tissue defects using a bilateral bipedicle flap and application of artificial dermis to the flap donor area in three cases in which surgery was difficult. The bilateral bipedicle flap was able to easily and reliably close the soft tissue defect even in highly difficult emergency myelomeningocele surgery. We believe that applying artificial dermis to the flap donor area is a useful method that avoids autologous skin grafting and facilitates wound management. There have been no cases of major donor wound contracture. The healing period of the flap donor area may be predicted to some extent by the width immediately after surgery.
PubMed: 38192914
DOI: 10.7759/cureus.50141 -
Fetal Diagnosis and Therapy 2024The Management of Myelomeningocele Study (MOMS) eligibility criteria preclude in utero surgery for fetal spina bifida (fSB) when the maternal body mass index (BMI) is...
INTRODUCTION
The Management of Myelomeningocele Study (MOMS) eligibility criteria preclude in utero surgery for fetal spina bifida (fSB) when the maternal body mass index (BMI) is ≥35 kg/m2. Some centers still respect this criterion, while others, like ours, do not. This study aimed to assess whether maternal and fetal safety is compromised with higher maternal BMIs.
METHODS
Data of 192 patients with open fSB repair at our center were retrospectively analyzed. According to their BMI, patients were divided into three groups: group 1 (BMI <30 kg/m2), group 2 (BMI 30-35 kg/m2), and group 3 (BMI >35 kg/m2). Subgroup analysis was performed to assess differences in maternal and fetal outcomes. Additionally, complications were divided into grades 1 to 5 according to their severity and outcome consequences and compared among groups.
RESULTS
Out of 192 patients, 146 (76.0%) had a BMI <30 kg/m2, 28 (14.6%) had a BMI 30-35 kg/m2, and 18 (9.4%) had a BMI >35 kg/m2. Significant differences occurring more often in either group 2 or 3 compared to group 1 were maternal wound seroma (50% or 56% vs. 32%, p = 0.04), amniotic fluid leakage (14% or 6% vs. 2%, p = 0.01) as well as vaginal bleeding (11% or 35% vs. 9%, p = 0.01). On the contrary, duration of tocolysis with atosiban was shorter in patients with BMI >30 kg/m2 (4 or 5 vs. 6 days, p = 0.01). When comparing severity of maternal or fetal complications, grade 1 intervention-related complications occurred significantly more often in group 3 compared to group 1 or 2 (78% vs. 45% or 57%, p = 0.02). Gestational age at delivery was around 36 weeks in all groups without significant differences.
CONCLUSION
This investigation did not identify clinically relevant maternal and/or fetal outcome problems related to BMIs >35 kg/m2. Additional studies are however needed to confirm our results.
Topics: Pregnancy; Female; Humans; Infant; Retrospective Studies; Fetus; Meningomyelocele; Obesity; Spinal Dysraphism; Spina Bifida Cystica
PubMed: 38190813
DOI: 10.1159/000536071