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BMC Pediatrics May 2024Neural tube defects (NTDs) account for the largest proportion of congenital anomalies of the central nervous system and result from failure of the neural tube to close...
BACKGROUND
Neural tube defects (NTDs) account for the largest proportion of congenital anomalies of the central nervous system and result from failure of the neural tube to close spontaneously between the 3rd and 4th weeks of in utero development. Prognosis and treatment outcome depends on the nature and the pattern of the defect. The nature of treatment outcomes and its pattern associated with grave prognosis is not well known in the study area.
OBJECTIVE
The aim of study was to determine the patterns and short term neurosurgical management outcomes of newborns with neural tube defects admitted at Felege Hiwot Specialized Hospital.
METHODS
Institutional based retrospective cross-sectional study among neonates, who were admitted at Felege Hiwot Specialized Hospital with neural tube defects from January 1 to December, 30, 2018 was conducted. All Charts of Neonates with confirmed diagnosis of neural tube defects were included as part of the study. Trained data collectors (medical interns) supervised by trained supervisors (general practitioners) collected the data using a pretested data extraction format. Data were coded, entered and analyzed using SPSS version 23 software. Frequency and cross tabulations were used to summarize descriptive statistics of data, and tables and graphs were used for data presentation.
RESULT
About 109 patients had complete documentation and imaging confirmed neural tube defects. Myelomeningocele was the commonest pattern 70 (64.2%). Thoracolumbar spine was the commonest site of presentation 49(45%). The most common associated impairment was hydrocephalus 37(33.9%). Forty-five (41.1%) had multiple complications. The mortality rate was 7.3%, 44% were discharged with sequalae and 36.7% were discharged without impairment. The significant causes of death were infection 66.7% and Chiari crisis 33.3%.
CONCLUSION
Myelomeningocele was the most frequent clinical pattern of neural tube defect and thoracolumbar spine was the commonest site. Isolated neural tube defect was the commonest finding. There were multiple complications after surgery accompanied with meningitis and hydrocephalus. The mortality rate among neonates with neural tube defects was considerably high. The commonest causes of death were infection and Chiari crisis.
Topics: Humans; Infant, Newborn; Cross-Sectional Studies; Retrospective Studies; Ethiopia; Neural Tube Defects; Female; Male; Neurosurgical Procedures; Treatment Outcome; Hydrocephalus; Hospitals, Special; Meningomyelocele
PubMed: 38773409
DOI: 10.1186/s12887-024-04837-5 -
Neurology India Mar 2024Dural closure is an important part of neurosurgery, failure which may lead to wound infection, pseudomeningoceles, meningitis, severe morbidity to a patient, and even...
INTRODUCTION
Dural closure is an important part of neurosurgery, failure which may lead to wound infection, pseudomeningoceles, meningitis, severe morbidity to a patient, and even mortality. In cases where primary dura closure is not possible, a bovine pericardial patch is a good alternative with the benefits of suturability and the ability to provide watertight closure, hence preventing Cerebrospinal Fluid (CSF) leak. The present study demonstrates the use of the bovine pericardial patch in both cranial and spinal disorders for dural closure as well as for transposition technique in microvascular decompression.
OBJECTIVES
The aim of our study is to understand the advantages and feasibility of a bovine pericardial patch in various neurosurgical procedures.
MATERIAL METHODS
Fifty-one patients were analyzed prospectively and followed up in which glutaraldehyde-free bovine pericardial patch was used in various cranial and spinal disorders.
RESULTS
The most common indications where a bovine pericardial patch was used, in decreasing order of frequency, were meningioma excision surgery (47%), followed by Chiari malformation operated for foramen magnum decompression (17.6%), meningomyelocele (7.8%), spinal dural defects (7.8%), trigeminal neuralgia (5.8%), traumatic decompression with lax duraplasty (4%), glioma (4%), encephalocele (4%), and skull base defects (2%). Two patients had complications, one with CSF leak and the other had superficial wound infection, which were managed appropriately.
CONCLUSIONS
The use of a bovine pericardial patch as a dural substitute in various cranial and spinal disorders is feasible with good outcomes, and it can be considered an ideal dural substitute.
Topics: Humans; Pericardium; Cattle; Male; Female; Adult; Middle Aged; Neurosurgical Procedures; Animals; Dura Mater; Aged; Adolescent; Young Adult; Prospective Studies; Child
PubMed: 38691472
DOI: 10.4103/ni.ni_327_22 -
The Pan African Medical Journal 2024
Topics: Humans; Meningomyelocele; Female; Neural Tube Defects; Infant, Newborn; Male; Magnetic Resonance Imaging
PubMed: 38681107
DOI: 10.11604/pamj.2024.47.61.42654 -
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; Meningomyelocele; Neural Tube Defects; Spinal Nerve Roots; Male; Magnetic Resonance Imaging; Female; Spinal Cord; Cauda Equina
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