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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 -
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 -
Journal of Neurosurgery. Case Lessons Feb 2024Developmental meningoceles of the sphenoid sinus are uncommon. When encountered, they are often associated with cerebrospinal fluid (CSF) rhinorrhea.
BACKGROUND
Developmental meningoceles of the sphenoid sinus are uncommon. When encountered, they are often associated with cerebrospinal fluid (CSF) rhinorrhea.
OBSERVATIONS
The authors present the case of a 27-year-old female with a large meningocele eroding through the sella turcica and sphenoid sinus into the nasopharynx. The patient presented with intractable headaches and amenorrhea without CSF rhinorrhea.
LESSONS
The patient underwent an endoscopic endonasal transsphenoidal reduction of the meningocele with reelevation of the pituitary gland and skull base reconstruction with abdominal fat graft and nasoseptal flap.
PubMed: 38346300
DOI: 10.3171/CASE23700 -
Surgical Neurology International 2024Delayed cerebrospinal fluid (CSF) leaks are a known complication following intradural spinal tumor surgery. The placement of subfascial drains in these patients...
BACKGROUND
Delayed cerebrospinal fluid (CSF) leaks are a known complication following intradural spinal tumor surgery. The placement of subfascial drains in these patients undergoing requisite intradural surgery is controversial. Here, we demonstrated that placing a subfascial drain on partial suction for 48 h, with early ambulation, proved to be safe and effective in preventing early/delayed recurrent CSF fistulas.
METHODS
Medical records of 17 patients undergoing surgery for intradural spinal tumors over a 30-month were reviewed. All patients underwent intradural tumor resection followed by primary dural closure, placement of Gelfoam in a non-compressive fashion, application of fibrin sealant, and utilization of a subfascial drain placed on partial suction for 48 h postoperatively. Patients are mobilized the morning following surgery. We tracked the incidence of postoperative recurrent CSF leaks, over drainage, infection, wound dehiscence, pseudo meningocele formation, and the reoperation rate.
RESULTS
For the 17 patients, our programmed average utilization of subfascial drains was 48 h. Moreover, the average drain output was 165 mL. Over the 1-year follow-up period, no patient developed a recurrent early/ delayed CSF leak, there were no wound complications, nor need for revision surgery.
CONCLUSION
Utilizing subfascial drains on partial suction following the resection of intradural spinal tumors with primary dural closure proved to be safe and effective.
PubMed: 38344094
DOI: 10.25259/SNI_934_2023 -
Journal of Radiology Case Reports Oct 2023Persistent craniopharyngeal canal (PCC) is a rare congenital anomaly that appears as a linear well-corticated canal running from the sella through the clivus and into...
Persistent craniopharyngeal canal (PCC) is a rare congenital anomaly that appears as a linear well-corticated canal running from the sella through the clivus and into the nasopharynx. Case reports of this anomaly have shown it is associated with a range of craniofacial defects, pituitary abnormalities, and meningoencephaloceles. It predisposes patients to bacterial meningitis. In this case a 46-year-old gentleman presenting for preoperative planning for surgical drainage of Potts Puffy tumor was found to have a PCC on CT and MRI. Imaging also demonstrated the presence of chronic inflammation and a fistula extending from the tract into the sphenoid sinus. This unusual presentation of a PCC with a sphenoid sinus fistula broadens the potential clinical presentations of PCC and further emphasizes the ability of this anomaly to serve as a conduit for CNS infection.
Topics: Male; Humans; Middle Aged; Sphenoid Sinus; Encephalocele; Meningocele; Magnetic Resonance Imaging
PubMed: 38343882
DOI: 10.3941/jrcr.v17i8.4707 -
Frontiers in Surgery 2024To study the classification, diagnosis, and treatment strategies of complex tethered cord syndrome (C-TCS) on the basis of the patients' clinical symptoms, imaging...
OBJECTIVE
To study the classification, diagnosis, and treatment strategies of complex tethered cord syndrome (C-TCS) on the basis of the patients' clinical symptoms, imaging findings, and therapeutic schedule.
METHODS
The clinical data of 126 patients with C-TCS admitted to our department from January 2015 to December 2020 were retrospectively analyzed. Classification criteria for C-TCS were established by analyzing the causes of C-TCS. Different surgical strategies were adopted for different types of C-TCS. The Kirollos grading, visual analogue scale (VAS), critical muscle strength, and Japanese Orthopaedic Association (JOA) scores were used to evaluate the surgical outcomes and explore individualized diagnosis and treatment strategies for C-TCS.
RESULTS
C-TCS was usually attributable to three or more types of tether-causing factors. The disease mechanisms could be categorized as pathological thickening and lipomatosis of the filum terminal (filum terminal type), arachnoid adhesion (arachnoid type), spina bifida with lipomyelomeningocele/meningocele (cele type), spinal lipoma (lipoma type), spinal deformity (bone type), and diastomyelia malformation (diastomyelia type). Patients with different subtypes showed complex and varied symptoms and required individualized treatment strategies.
CONCLUSION
Since C-TCS is attributable to different tether-related factors, C-TCS classification can guide individualized surgical treatment strategies to ensure complete release of the tethered cord and reduce surgical complications.
PubMed: 38322409
DOI: 10.3389/fsurg.2024.1277322 -
Cureus Jan 2024Transient global amnesia (TGA) constitutes a rare clinical entity that manifests with temporary memory without any other neurological manifestation. Several pathogenetic...
Transient global amnesia (TGA) constitutes a rare clinical entity that manifests with temporary memory without any other neurological manifestation. Several pathogenetic mechanisms have been implicated, including temporal hypoperfusion, venous congestion, and cortical spreading potentials. Accordingly, the only relevant imaging findings are hippocampal CA1 areas of restricted diffusion on diffusion-weighted images. In the current case report, we present the rare case of a patient with TGA associated with bilateral petrous apex cephalocele (PAC). A 63-year-old female presented with a single episode of transient memory. The brain MRI showed a bilateral PAC and an empty sella. The patient was neurologically intact upon examination and was conservatively managed. There was no symptom recurrence during the six months of follow-up. We hypothesize that the presence of the meningocele could be associated with the pathogenesis of TGA. To the best of our knowledge, this is the first case of a petrous apex meningocele presenting with TGA. Most previously reported patients were females in their fourth decade of life, usually presenting with headaches or incidentally. Almost half of the cases were bilateral, with an empty sella. Surgical treatment was reserved for symptomatic patients with cerebrospinal fluid leaks and excruciating trigeminal neuralgia. Patients with TGA may be associated with temporal lesions, including PAC. Likewise, PAC is an extremely rare clinical entity that could occasionally present with TGA.
PubMed: 38322086
DOI: 10.7759/cureus.51778 -
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 -
International Journal of Women's Health 2024Microphthalmia is a rare autosomal recessive condition commonly known as Waardenburg anophthalmia syndrome (WAS) or oculo-acromal formation syndrome (MIM#206920).
BACKGROUND
Microphthalmia is a rare autosomal recessive condition commonly known as Waardenburg anophthalmia syndrome (WAS) or oculo-acromal formation syndrome (MIM#206920).
CASE DESCRIPTION
Here, we report the case of a woman whose fetal ultrasonography at 12 weeks of pregnancy revealed multiple fetal abnormalities. These included the absence of the left upper limb, an unclear display of the right orbit, a visible maxillary space, and a round, echoless appearance measuring 4 mm in diameter in the middle of the forehead. There was also a significant echo in the sac wall. The possibility of a frontal meningocele or a proboscis-like nose was considered. The fetus was delivered with absence of the left upper limb, absence of the right eye, a cleft lip on the right side, and a milky white sac with a diameter of 5 mm on the forehead after the pregnancy was terminated at the hospital. Pathological investigation revealed a mature cystic teratoma. The conclusion was microphthalmia with limb anomalies (MLA) after missing limbs, absence of eyes, and cleft lip were input into the Online Mendelian Inheritance in Man database. The case was diagnosed with fetal microphthalmia with limb anomalies and an interfrontal teratoma.
CONCLUSION
In this case, the entire exon analysis was not conducted, and as a result, the final diagnosis remains unclear. Based exclusively on the phenotype of the induced fetus, MLA was diagnosed. It is proposed that cases satisfying the requirements for a pathological diagnosis should undergo a pathological examination to establish a definitive diagnosis.
PubMed: 38222311
DOI: 10.2147/IJWH.S441452