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Journal of Neurosurgery. Case Lessons Mar 2024Anterior cervicothoracic myelomeningoceles are a rare pathology. In reported cases, treatment has included shunting, isolated resection and repair without deformity...
BACKGROUND
Anterior cervicothoracic myelomeningoceles are a rare pathology. In reported cases, treatment has included shunting, isolated resection and repair without deformity correction, or isolated deformity correction without meningocele repair. The authors describe a pediatric patient with an anterior cervicothoracic myelomeningocele presenting with progressive neurological decline, who underwent simultaneous treatment of the myelomeningocele to detether the spinal cord and achieve major correction of the scoliotic deformity.
OBSERVATIONS
A 15-year-old girl was born with C7-T1-T2 hemivertebrae and anterior cervical myelomeningocele at C7-T1. She developed progressive cervical thoracic scoliosis, left hemiparesis initially, and additional right hemiparesis eventually. She underwent surgical repair via C7, T1, and T2 corpectomies with intradural detethering of the spinal cord. The scoliosis was treated with C7-T2 Ponte osteotomies and C2-T5 posterior fixation, followed by anterior reconstruction with a titanium cage and anterior plate from C6 to T3. The myelomeningocele was adequately treated with good correction of the patient's deformity. The patient had postoperative improvement in her strength and solid arthrodesis on postoperative imaging.
LESSONS
The authors describe the successful treatment of an anterior cervicothoracic myelomeningocele and associated scoliosis in a child. This is a unique report of a combined strategy to achieve both deformity correction and detethering of the spinal cord.
PubMed: 38437675
DOI: 10.3171/CASE23710 -
Pediatrics and Neonatology May 2024
Topics: Humans; Meningocele; Infant, Newborn; Female; Male; Cervical Vertebrae; Magnetic Resonance Imaging
PubMed: 38423867
DOI: 10.1016/j.pedneo.2023.12.005 -
Journal of Pediatric Urology Jun 2024Children and adolescents with neurogenic bladder often need clean intermittent catheterization (CIC) over a long period. Our study aimed to identify factors that affect...
INTRODUCTION
Children and adolescents with neurogenic bladder often need clean intermittent catheterization (CIC) over a long period. Our study aimed to identify factors that affect CIC compliance and to determine if CIC compliance affected short-term urological outcomes among patients in Malaysia.
STUDY DESIGN
50 patients aged 2-18 years who perform CIC were included in this cohort study. Patient compliance with CIC was evaluated using the validated Intermittent Catheterization Adherence Scale (ICAS). CIC difficulties were assessed using the validated Intermittent Catheterization Difficulty Questionnaire (ICDQ). Data was obtained on patients' co-morbidity, caregiver factors, socio-economic factors, CIC technique, access to catheters and facilities, urinary tract infections, incontinence, urology tests and treatment. Statistical analysis was performed.
RESULTS
Mean age was 6.68 ± 4.34 years. 32 (64%) patients commenced CIC within the first month of life. Mean daily CIC frequency was 4.70 ± 1.33.30 (60%) participants showed strong adherence to CIC. 39 (78%) participants were able to catheterize with no or minor difficulties. Pain (6, 12%), transient blocking sensation (6.12%), and urinary incontinence (3, 6%) were the predominant difficulties encountered. CIC performed by caregiver was associated with improved adherence compared to patient self-catheterization (p = 0.039). The mean age of participants who self-catheterized was 10.7 ± 3.7 years. Strong adherence was also observed among patients who purchased their own CIC catheters (p = 0.007). Participants with lower ICDQ score were more likely to be compliant with CIC (p = 0.007). CIC adherence was not affected by patient's age, gender, co-morbidity, mobility, caregiver factors, socio-economic factors, and age at initiation of CIC. There was no significant association between CIC adherence and febrile urinary tract infections, upper tract deterioration, and bladder stones at 6 months follow-up.
DISCUSSION
There is lower CIC adherence when a child begins to self-catheterize and healthcare providers should be alert during this period of transition. Though most patients with spina bifida have decreased urethral sensation, some patients do experience significant pain during CIC which may impact their compliance. These patients would need a review of their catheterization techniques to improve adherence. The limitations of our study are its modest sample size from a single center and short study period. Our study provides insights into the feasibility of instituting CIC in developing countries.
CONCLUSION
Strong CIC adherence was observed among patients who were catheterized by their caregiver, purchased their own CIC catheters, and encountered minimal difficulties during catheterization. CIC adherence had no effect on short-term urological outcomes.
Topics: Humans; Urinary Bladder, Neurogenic; Child; Male; Female; Child, Preschool; Adolescent; Intermittent Urethral Catheterization; Spinal Dysraphism; Patient Compliance; Malaysia; Meningocele; Surveys and Questionnaires; Meningomyelocele; Abnormalities, Multiple; Sacrococcygeal Region
PubMed: 38413297
DOI: 10.1016/j.jpurol.2024.02.009 -
Child's Nervous System : ChNS :... Jun 2024Sacral agenesis (SA) includes a range of clinical presentations of varying severity, with implications for function and quality of life (QoL). Diagnosis is often made...
INTRODUCTION
Sacral agenesis (SA) includes a range of clinical presentations of varying severity, with implications for function and quality of life (QoL). Diagnosis is often made perinatally, and prognostic discussions become an important aspect of parental counselling. This study engaged SA sufferers and their caregivers to obtain objective, long-term patient reported outcome data.
METHOD
Patients with radiologically confirmed SA from a single tertiary spinal unit underwent retrospective medical record review. Patients were then contacted by telephone to complete QoL questionnaires including EQ-ED-5L for adults and EQ-ED-Y for < 16-year-olds. Additional information including Renshaw grade, employment, living situation and bladder function was also collected.
RESULTS
Twenty-six patients with SA were identified. Mean age is 23.35 years (range 0.92-63.53), 13 M:17F. Renshaw grade ranged from 1 to 4. Sixty-eight percent had associated kyphoscoliotic deformities. The majority (70%) had either impaired or absent bladder control, and 80% need walking aids to mobilise. Twenty patients completed the questionnaire (10 adults and 10 < 16-year-olds). Mean EQ-ED-5L index for adults was +0.474 (range -0.1 to +0.089, 1 = best), with a lower mean value of +0.287 (range -0.54 to +1) for the < 16-year cohort. Those undergoing spinal fusion procedures had significantly lower scores (-0.08 v +0.44, p = 0.022).
CONCLUSION
This study provides an objective record of the QoL of individuals with SA, illustrating a wide variety of outcomes, with differences between younger and older individuals which may reflect the results of a long-term adaptive process. The implications for individuals should be carefully tailored to the specific deformity and the likely underlying neurological deficits.
Topics: Humans; Female; Male; Adolescent; Adult; Young Adult; Child; Quality of Life; Child, Preschool; Middle Aged; Retrospective Studies; Infant; Sacrum; Surveys and Questionnaires; Treatment Outcome; Abnormalities, Multiple; Meningocele; Sacrococcygeal Region
PubMed: 38411707
DOI: 10.1007/s00381-024-06326-7 -
The Laryngoscope May 2024We undertook a systematic review of the literature with meta-analysis to identify the role of obesity (BMI ≥30) in the patient characteristics presenting with... (Meta-Analysis)
Meta-Analysis Review
OBJECTIVE
We undertook a systematic review of the literature with meta-analysis to identify the role of obesity (BMI ≥30) in the patient characteristics presenting with spontaneous cerebrospinal fluid (sCSF) leaks of the lateral skull base and the outcomes of their repair.
DATA SOURCES
A Systematic Review of English Articles using MEDLINE, EMBASE, and Cochrane Library.
REVIEW METHODS
The research algorithm included the following keywords: "spontaneous CSF leak," "lateral skull base," "temporal bone," "meningocele," "encephalocele," and "otorrhea." We also manually searched the references of included studies, to identify possible studies missed during our literature search.
RESULTS
More than two-thirds of the patients were female (69.2%) and often were obese (mean BMI 36.5 kg/m) with a mean age of 57. Most common presenting symptoms were otorrhea and hearing loss. Most authors did not report a routine use of a post-operative lumbar drain. Most patients had a single skull base defect and encephaloceles prolapsing through, across obese and non-obese groups. Median length of stay in hospital was 3.2 days, and the majority of patients did not have any recurrence during their follow-up (89.6%), which was not affected by obesity.
CONCLUSION
Obesity does not affect length of hospital stay or recurrence rate following surgical repair of lateral skull base sCSF leaks. Surgical repair is a safe and viable approach in the management of obese patients with sCSF leaks in the temporal bone.
LEVEL OF EVIDENCE
NA Laryngoscope, 134:2012-2018, 2024.
Topics: Humans; Female; Middle Aged; Male; Retrospective Studies; Treatment Outcome; Skull Base; Cerebrospinal Fluid Leak; Obesity; Temporal Bone; Encephalocele
PubMed: 38400793
DOI: 10.1002/lary.31349 -
Cureus Jan 2024Meningoceles refer to the protrusion of meninges filled with cerebrospinal fluid (CSF) through a bone defect. There is scarce literature on the management of multiple...
Meningoceles refer to the protrusion of meninges filled with cerebrospinal fluid (CSF) through a bone defect. There is scarce literature on the management of multiple giant anterior sacral meningoceles (ASMs). We report the case of a patient with Marfan syndrome presenting with gait disturbances and dizziness triggered by posture changes due to multiple giant ASMs. The patient was managed through an anterior approach involving a multidisciplinary team of surgeons. Care was taken to limit the persistence of CSF leak using an omental pedicled flap. This technique has only been mentioned twice in the literature for such cases. A literature review was conducted focusing on the evolution course and surgical strategy of meningoceles.
PubMed: 38384626
DOI: 10.7759/cureus.52724 -
JA Clinical Reports Feb 2024Diagnosed intrathoracic meningocele is an uncommon complication of neurofibromatosis type 1. We report an anesthesia management for a rare case undergoing thoracoscopic...
BACKGROUND
Diagnosed intrathoracic meningocele is an uncommon complication of neurofibromatosis type 1. We report an anesthesia management for a rare case undergoing thoracoscopic resection of a huge intrathoracic meningocele.
CASE PRESENTATION
A 51-year-old woman was scheduled for thoracoscopic meningectomy under general anesthesia. We monitored intrathecal pressure during anesthesia to prevent a decrease in intrathecal pressure. During surgery, the intrathecal pressure occasionally increased by around 5 cmHO immediately after the insertion of the drainage tube and occasionally decreased by up to 10 cmHO during the careful slow aspiration of the cerebrospinal fluid (CSF). The pressure rapidly recovered after the interruption of the procedures. She was discharged on postoperative day 4 without major complications.
CONCLUSIONS
The CSF pressure was fluctuated by procedures during thoracoscopic resection of a huge meningocele. A CSF pressure monitoring was useful to detect the sudden change of CSF pressure immediately, which can cause intracranial hemorrhage.
PubMed: 38369643
DOI: 10.1186/s40981-024-00697-1 -
BMJ Case Reports Feb 2024Spontaneous meningoencephaloceles (MECs) are sparsely documented in the literature. Those occurring in the frontal sinus are an exceedingly rare entity. MECs are...
Spontaneous meningoencephaloceles (MECs) are sparsely documented in the literature. Those occurring in the frontal sinus are an exceedingly rare entity. MECs are commonly associated with cerebrospinal fluid (CSF) rhinorrhoea. CSF rhinorrhoea is frequently misdiagnosed, causing delays in diagnosis and management. The subsequently increased risk of bacterial meningitis can be life-threatening to patients. We report the case of a woman in her late 70s with a spontaneous frontal sinus MEC, presenting with a 6-month history of CSF rhinorrhoea. The patient was successfully treated using the novel Carolyn's window approach endoscopically; 9-month follow-up revealed no skull-base breach. Our case emphasises the importance of considering MEC as a differential diagnosis for clear rhinorrhoea and demonstrates successful repair through a novel surgical approach.
Topics: Female; Humans; Cerebrospinal Fluid Rhinorrhea; Frontal Sinus; Tomography, X-Ray Computed; Encephalocele; Meningocele
PubMed: 38355205
DOI: 10.1136/bcr-2023-258886 -
Frontiers in Neurology 2024Fetal-neonatal neurologists (FNNs) consider diagnostic, therapeutic, and prognostic decisions strengthened by interdisciplinary collaborations. Bio-social perspectives... (Review)
Review
Fetal-neonatal neurologists (FNNs) consider diagnostic, therapeutic, and prognostic decisions strengthened by interdisciplinary collaborations. Bio-social perspectives of the woman's health influence evaluations of maternal-placental-fetal (MPF) triad, neonate, and child. A dual cognitive process integrates "fast thinking-slow thinking" to reach shared decisions that minimize bias and maintain trust. Assessing the science of uncertainty with uncertainties in science improves diagnostic choices across the developmental-aging continuum. Three case vignettes highlight challenges that illustrate this approach. The first maternal-fetal dyad involved a woman who had been recommended to terminate her pregnancy based on an incorrect diagnosis of an encephalocele. A meningocele was subsequently identified when she sought a second opinion with normal outcome for her child. The second vignette involved two pregnancies during which fetal cardiac rhabdomyoma was identified, suggesting tuberous sclerosis complex (TSC). One woman sought an out-of-state termination without confirmation using fetal brain MRI or postmortem examination. The second woman requested pregnancy care with postnatal evaluations. Her adult child experiences challenges associated with TSC sequelae. The third vignette involved a prenatal diagnosis of an open neural tube defect with arthrogryposis multiplex congenita. The family requested prenatal surgical closure of the defect at another institution at their personal expense despite receiving a grave prognosis. The subsequent Management of Myelomeningocele Study (MOMS) would not have recommended this procedure. Their adult child requires medical care for global developmental delay, intractable epilepsy, and autism. These three evaluations involved uncertainties requiring shared clinical decisions among all stakeholders. Falsely negative or misleading positive interpretation of results reduced chances for optimal outcomes. FNN diagnostic skills require an understanding of dynamic gene-environment interactions affecting reproductive followed by pregnancy exposomes that influence the MPF triad health with fetal neuroplasticity consequences. Toxic stressor interplay can impair the neural exposome, expressed as anomalous and/or destructive fetal brain lesions. Functional improvements or permanent sequelae may be expressed across the lifespan. Equitable and compassionate healthcare for women and families require shared decisions that preserve pregnancy health, guided by person-specific racial-ethnic, religious, and bio-social perspectives. Applying developmental origins theory to neurologic principles and practice supports a brain health capital strategy for all persons across each generation.
PubMed: 38352135
DOI: 10.3389/fneur.2024.1335933 -
ClinicoEconomics and Outcomes Research... 2024The prevention of myelomeningocele (MMC) and meningocele (MC) is a public health concern. A systematic review on economic factors associated with MMC and MC can help the... (Review)
Review
BACKGROUND
The prevention of myelomeningocele (MMC) and meningocele (MC) is a public health concern. A systematic review on economic factors associated with MMC and MC can help the policy makers to evaluate the cost-effectiveness of screening and treatment. To our knowledge, this is the first systematic review to provide up-to date pharmacoeconomic evidence of all economic studies present in literature on different aspects of MMC and MC.
METHODS
We searched in the National Health Service Economic Evaluation Database (NHSEED), PubMed, Cost-effectiveness Analysis Registry (CEA Registry), Centre for Reviews and Dissemination (CRD), Health Technology Assessment Database (HTAD), Cochrane Library, and Econlit. The PRISMA guidelines were followed in the search and evaluation of literature. Only articles in English not limited by the year of publication that fulfilled the eligibility criteria were included in this systematic review.
RESULTS
Nineteen papers were included in the study. The studies were very heterogeneous and reported a comparison of the costs between prenatal versus postnatal repair, the cost of fetoscopic approach versus open surgery, the cost of ventriculoperitoneal shunting (VPS) versus endoscopic third ventriculostomy (ETV), and ETV with choroid plexus cauterization (ETV/CPC), the cost of hospitalization, and the cost of diagnosis for MMC.
CONCLUSION
The results of this study can help in implementing new policies in different countries to assist MC and MMC patients with the cost of treatment and screening.
PubMed: 38352115
DOI: 10.2147/CEOR.S443120