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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 -
World Neurosurgery May 2024Thoracic meningocele is a rare medical condition that is usually linked to neurofibromatosis type I. Respiratory and neurologic symptoms characterize it. Although there...
Thoracic meningocele is a rare medical condition that is usually linked to neurofibromatosis type I. Respiratory and neurologic symptoms characterize it. Although there have been some improvements in surgical techniques, the condition has a high recurrence rate, with most cases recurring within a year of surgery. A 56-year-old woman was observed due to respiratory and pyramidal signs. A chest computed tomography scan and magnetic resonance imaging revealed a thoracic meningocele, occupying the lower sectors of the right hemithorax, communicating with the cerebrospinal fluid space at the T10-T11 level. Multidisciplinary surgery was performed. After selectively intubating both bronchi, the patient was placed in prone position and a posterior median thoracic spine approach was performed. After T10-T11 laminectomy, 3 dural longitudinal incisions were performed. The first incision was placed in the middle to deflate the collection, the second was made on the right side to obtain a complete view of the meningocele, and the third was made on the right lateral side to exclude the meningocele. The lateral dura at the last incision was sutured to the dura propria lining the vertebral body of T11 and T10. The paramedian and median incisions were closed, with Tachosil placed above and below the sutures. Subsequently, the patient was placed in a supine position, the right lung was deflated, and a triportal thoracoscopic approach was performed to dissect and remove the lesion. The breach was closed using Tachosil (Baxter Healthcare Corp, Deerfield, Illinois, USA) and fibrin glue. An early 1-month computed tomography and magnetic resonance imaging confirmed the surgery was successful.
PubMed: 38777317
DOI: 10.1016/j.wneu.2024.05.075 -
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 -
Cureus Oct 2023We present a case of a two-year-old male with a history of congenital scoliosis and anterolateral thoracic meningocele. He was able to walk and run, but his parents...
We present a case of a two-year-old male with a history of congenital scoliosis and anterolateral thoracic meningocele. He was able to walk and run, but his parents reported left leg weakness and a frequent cough. The patient had normal neurological examination findings. Magnetic resonance imaging (MRI) of the spine without contrast showed left convex upper thoracic congenital scoliosis and rightward anterolateral meningocele inferiorly to T3, with the spinal cord tethered at this location. Neurosurgical cord detethering and repair of the meningocele were performed simultaneously with scoliosis repair by orthopedics. During the dissection of the meningocele, the bulging neural tissue was found to be a split cord ending in a blind stump. The split cord was determined to be nonfunctional via Prass probe (Medtronic, Minneapolis, MN) stimulation and was subsequently dissected. Detethering of the spinal cord was followed by repair of the dural outpouching and dural closure. The patient was stable post-surgery, but long-term results remain to be seen.
PubMed: 37927626
DOI: 10.7759/cureus.46496 -
Journal of Personalized Medicine Apr 2024Spontaneous orbital cephaloceles are a rare condition. The purpose of this study is to provide a description of a clinical case and to carry out a systematic literature... (Review)
Review
BACKGROUND
Spontaneous orbital cephaloceles are a rare condition. The purpose of this study is to provide a description of a clinical case and to carry out a systematic literature review.
METHODS
A systematic review of the English literature published on the Pubmed, Scopus, and Web of Science databases was conducted, according to the PRISMA recommendations.
RESULTS
A 6-year-old patient was admitted for right otomastoiditis and thrombosis of the sigmoid and transverse sinuses, as well as the proximal portion of the internal jugular vein. Radiological examinations revealed a left orbital mass (22 × 14 mm) compatible with asymptomatic orbital meningocele (MC) herniated from the superior orbital fissure (SOF). The child underwent a right mastoidectomy. After the development of symptoms and signs of intracranial hypertension (ICH), endovascular thrombectomy and transverse sinus stenting were performed, with improvement of the clinical conditions and reduction of the orbital MC. The systematic literature review encompassed 29 publications on 43 patients with spontaneous orbital MC. In the majority of cases, surgery was the preferred treatment.
CONCLUSIONS
The present case report and systematic review highlight the importance of ICH investigation and a pathophysiological-oriented treatment approach. The experiences described in the literature are limited, making the collection of additional data paramount.
PubMed: 38793047
DOI: 10.3390/jpm14050465 -
Indian Journal of Otolaryngology and... Dec 2023Fallopian canal meningocele is an extremely rare cause of cerebrospinal fluid rhinorrhoea. Also, due to complex anatomical relations and a lack of experience, its...
Fallopian canal meningocele is an extremely rare cause of cerebrospinal fluid rhinorrhoea. Also, due to complex anatomical relations and a lack of experience, its management remains a challenge. Here we report a case focusing on its clinical course, radiological features, and management.
PubMed: 38027530
DOI: 10.1007/s12070-023-03963-3 -
Journal of the Belgian Society of... 2023To investigate the computed tomography and magnetic resonance imaging manifestations of Joubert syndrome (JS).
OBJECTIVE
To investigate the computed tomography and magnetic resonance imaging manifestations of Joubert syndrome (JS).
METHOD
In this retrospective analysis, we investigated the clinical and imaging characteristics of JS in a cohort of twelve pediatric patients with confirmed diagnoses. Specifically, we analyzed both computed tomography (CT) and magnetic resonance imaging (MRI) manifestations in this population. CTs were performed on four patients and MRIs were performed on twelve, respectively.
RESULTS
JS is characterized by specific CT and MRI findings, including midline fissure, batwing, or triangular formations of the fourth ventricle between the bilateral cerebellar hemispheres, and molar sign at the midbrain level. All twelve cases in this cohort exhibited these traits, along with other cerebral abnormalities, such as dysplasia of the corpus callosum in two cases, gray matter heterotopia in one case, and occipital meningocele in one case.
CONCLUSION
JS has distinctive CT and MRI characteristics that can be clinically identified.
PubMed: 37781478
DOI: 10.5334/jbsr.3283 -
Neuro-Chirurgie May 2024Anterior sacral meningocele is a rare congenital disorder, occurring isolated or in syndromic disease.
INTRODUCTION
Anterior sacral meningocele is a rare congenital disorder, occurring isolated or in syndromic disease.
CASE REPORT
A 15-year-old patient who complained of abdominal pain and urinary dysfunction was managed surgically. Imaging diagnosed a giant presacral meningocele and agenesis of the coccyx.
DISCUSSION
The presentation of sacral meningocele can be poorly symptomatic, which is why some patients are diagnosed late. Sometimes, diagnosis is suggested by non-specific abdominal symptoms or complications. Abdominal-pelvic radiological examination and lumbar spine MRI are essential, and treatment must be surgical. There are several surgical approaches, but currently no consensus.
CONCLUSION
An unusual huge presacral cystic mass in a young patient may be isolated or part of a syndrome, and can be asymptomatic for a long time, leading to late diagnosis. The surgical approach should be based on multidisciplinary discussion. We operated on a giant anterior sacral meningocele in a child using a posterior approach, with a satisfactory result.
PubMed: 38820830
DOI: 10.1016/j.neuchi.2024.101571 -
Journal of Neurosurgery. Pediatrics Jul 2023The two main objectives of this study were to explore the rate of spinal dysraphism within bladder and cloacal exstrophy and to analyze the relationship between spinal...
OBJECTIVE
The two main objectives of this study were to explore the rate of spinal dysraphism within bladder and cloacal exstrophy and to analyze the relationship between spinal dysraphism surgery, including timing of spinal dysraphism surgery, with urological and neurological outcomes.
METHODS
A prospectively maintained IRB-approved database of pediatric exstrophy patients treated from 1982 to 2021 was retrospectively reviewed for patients with spinal dysraphism. Spinal dysraphism was categorized into the following 7 subtypes: lipoma-based closed defect, myelomeningocele, meningocele, diastematomyelia, myelocystocele, low-lying conus with tethered cord/fatty filum, and sacral bony defect. Other factors assessed included patient demographic characteristics, type of spinal dysraphism procedure, reoperation, complication, presence of other neurological problems (e.g., hydrocephalus, Chiari malformation), neurological status, and urological function.
RESULTS
Analysis revealed that 114/1401 patients had coexisting spinal dysraphism. Of these 114, sufficient records including type of dysraphism were available for 54. Spinal dysraphism was most common within cloacal exstrophy (83.3% [45/54 patients]), followed by cloacal exstrophy variants (9.3% [5/54]), classic bladder exstrophy (3.7% [2/54]), and classic bladder exstrophy variants (3.7% [2/54]). Within spinal dysraphism, lipoma-based closed defects (63.0% [34/54]) and low-lying conus with tethered cord/fatty filum (11.1% [6/54]) were most common. Hydrocephalus and Chiari malformation occurred in 24.1% (13/54) and 11.1% (6/54) of patients. All 13 patients with hydrocephalus underwent shunt placement. Among those who underwent neurosurgical intervention, the complication rate for spinal dysraphism was 14.6% (7/48). Motor function data were available for 41 patients and revealed that motor function declined for 2/41 (4.8%) patients and improved for 6/41 (14.6%) after neurosurgery. There was no statistical difference in lower-extremity motor outcome related to timing of neurosurgery and exstrophy closure.
CONCLUSIONS
The authors have reported the surgical management and outcomes of patients with exstrophy and coexisting spinal dysraphism (n = 54). In 54 patients, spinal dysraphism was most common in the subset of patients with cloacal exstrophy (83.3%). Lipoma-based closed defects (63.0%) and low-lying conus with tethered cord/fatty filum (11.1%) were the most common, and the rates of hydrocephalus and Chiari malformation were 24.1% and 11.1%, respectively. There was no difference in lower-extremity motor outcome related to timing of neurosurgery and exstrophy closure.
Topics: Humans; Child; Bladder Exstrophy; Retrospective Studies; Spinal Dysraphism; Neural Tube Defects; Meningomyelocele; Arnold-Chiari Malformation; Hydrocephalus; Digestive System Abnormalities; Lipoma
PubMed: 37119103
DOI: 10.3171/2023.3.PEDS22447 -
Cureus Dec 2023The present study aimed to review the treatment experience and outcomes of Currarino syndrome (CS) complicated with anorectal stenosis to evaluate the current treatment...
PURPOSE
The present study aimed to review the treatment experience and outcomes of Currarino syndrome (CS) complicated with anorectal stenosis to evaluate the current treatment strategies.
METHODS
Seven cases of CS complicated with anorectal stenosis, treated at our hospital between 1998 and 2021, were retrospectively investigated. This is a case series article from a single institution.
RESULTS
In six and three cases and one case, the presacral mass was a mature teratoma, meningocele, and lipoma, respectively. Resection of the lesion was performed in all six cases of mature teratoma, and duraplasty was performed before resection in all three cases of meningocele. Moreover, surgery for anorectal stenosis was performed simultaneously in four patients. Surgery was performed for six cases of anorectal stenosis, with the remaining case relieved by dilation using a metal bougie. The surgical methods used were a partial resection with end-to-end anastomosis, anorectal strictureplasty, pull-through, posterior sagittal anorectoplasty, and cutback after mass resection. Pathological analysis of the anorectal stenoses revealed disorganized and rough smooth muscle fibers and the replacement of the stroma by an increased quantity of collagen fibers.
CONCLUSIONS
The clinical outcomes of CS can be improved by establishing a treatment flow chart and understanding the complicated pathophysiology of the disease.
PubMed: 38226073
DOI: 10.7759/cureus.50512