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Radiology Case Reports May 2024Arachnoid cysts, fluid-filled lesions within the central nervous system, pose diagnostic challenges. This study examines a unique case of a quadrigeminal arachnoid cyst...
Arachnoid cysts, fluid-filled lesions within the central nervous system, pose diagnostic challenges. This study examines a unique case of a quadrigeminal arachnoid cyst in a 13-year-old girl, emphasizing accurate identification and treatment. The patient's symptoms of blurred vision and headaches led to the discovery of papilledema and imaging revealing a sizable cyst causing obstructive hydrocephalus. Urgent surgical intervention involved suboccipital craniectomy and infratentorial-supracerebellar cyst drainage, resulting in favorable postoperative outcomes. Further analysis of anatomical variations, age-related factors, and etiological debates deepens understanding. Diagnostic advancements, notably MRI, are crucial for noninvasive characterization. This case offers nuanced insights into managing arachnoid cysts, highlighting the success of tailored surgical strategies. Recognizing clinical subtleties, utilizing diagnostic innovations, and customizing surgical techniques are essential for navigating complexities. This study underscores the importance of a comprehensive approach in addressing the challenges of arachnoid cysts within the central nervous system.
PubMed: 38523695
DOI: 10.1016/j.radcr.2024.02.048 -
Neuro-Chirurgie Mar 2024Syringomyelia associated with extensive spinal adhesive arachnoiditis (SAA) can be defined as a rare but progressive disease with potentially devastating clinical...
Syringomyelia associated with extensive spinal adhesive arachnoiditis (SAA) can be defined as a rare but progressive disease with potentially devastating clinical consequences. Diagnosis can be challenging due to the absence of specific clinical findings, confusion with other pathologies, and late imaging modalities. The treatment strategy for syringomyelia associated with extensive SAA should include direct drainage of the syringomyelia, and dissection of the adhesive arachnoid with expansive duraplasty. Hence, several approaches have been reported for arachnoid dissection and decompression of the subarachnoid space. The high risk of recurrence after the operation is one of the most challenging situations. First two cases were operated previously, and the cyst walls were removed and anastomosed to the intact subarachnoid space at the upper and lower ends by each other. However, in both cases, the cyst recurred after a short time and they were anastomosed again in our clinic. A subarachnoid-subarachnoid shunt was placed with a multi-hole silicone tube extending to the proximal and distal intact subarachnoid spaces and passing through the cyst removal area. In our third case, the shunt system we described was applied directly, and satisfactory results were obtained in the clinical follow-up of the patient. It has been demonstrated that subarachnoido-subarachnoid shunt is a very satisfactory treatment option for this type of pathology, which is difficult to follow and treat.
PubMed: 38520969
DOI: 10.1016/j.neuchi.2024.101555 -
Der Nervenarzt Jun 2024
Topics: Humans; Arachnoid Cysts; Alzheimer Disease; Diagnosis, Differential; Magnetic Resonance Imaging; Female; Male; Aged
PubMed: 38483549
DOI: 10.1007/s00115-024-01638-0 -
Head & Neck May 2024Anterior endoscopic access to middle cranial base lesions becomes feasible in the presence of infratemporal fossa (ITF) involvement. Various approaches, including...
BACKGROUND
Anterior endoscopic access to middle cranial base lesions becomes feasible in the presence of infratemporal fossa (ITF) involvement. Various approaches, including endoscopic endonasal, transoral sublabial, and transorbital methods, have been described for accessing the ITF through a transmaxillary corridor. Among these approaches, endonasal access is the most commonly preferred, while the transorbital approach is a novel technique gaining popularity. The transoral sublabial approach is considered suitable for selected lesions.
METHODS
Patients who underwent the anterior endoscopic transoral/sublabial transmaxillary approach to middle cranial base lesions at a single institute from 2016 to 2023 were included in this retrospective study. Malignant lesions were excluded from the study. The sublabial approach was exclusively performed in all cases, with the exception of one patient who required a combined approach.
RESULTS
The anterior endoscopic transoral sublabial transmaxillary approach to the infratemporal fossa, upper parapharyngeal space, and middle cranial fossa was performed on 14 patients. The underlying conditions for these patients were as follows: trigeminal schwannomas (n = 8), meningiomas (n = 2), juvenile nasopharyngeal angiofibroma, osteochondroma, arachnoid cyst and encephalocele (n = 1 each). Gross total resection was achieved in 11 cases. The most common complication was numbness in the territory of the maxillary and mandibular nerves (n = 4). Two patients needed endoscopic maxillary antrostomy for persistent suppuration. No wound problems or CSF rhinorrhea occurred. The average follow-up time was 26.6 months.
CONCLUSION
The endoscopic sublabial transmaxillary approach provides direct access to the infratemporal fossa and middle cranial base, enhancing the surgical range of maneuverability while sparing the sinonasal cavity. This procedure is safe, less invasive, and could be used as an efficient corridor for the resection of selected infratemporal fossa lesions with or without extension to the middle cranial base and parapharyngeal space.
Topics: Humans; Retrospective Studies; Endoscopy; Skull Base; Nasopharyngeal Neoplasms; Maxilla
PubMed: 38465500
DOI: 10.1002/hed.27725 -
Neurosurgical Review Mar 2024A cerebrospinal-fluid-related (CSF-related) problem occurred in 25-30% of frontoethmoidal encephalocele (FEE) cases. Since there was no algorithm or guideline, the...
A cerebrospinal-fluid-related (CSF-related) problem occurred in 25-30% of frontoethmoidal encephalocele (FEE) cases. Since there was no algorithm or guideline, the judgment to treat the CSF-related problem often relies upon the surgeon's experience. In our institution, the early shunt was preferable to treat the problem, but it added risks to the children. We developed an algorithm, "Shunt Algorithm for Frontoethmoidal Encephalocele" (SAFE), to guide the surgeon in making the most reasonable decision. To evaluate the SAFE's efficacy in reducing unnecessary early shunting for FEE with CSF-related intracranial abnormality. Medical records of FEE patients with CSF-related abnormalities treated from January 2007 to December 2019 were reviewed. The patients were divided into two groups: before the SAFE group as group 1 (2007 - 2011) and after the SAFE group as group 2 (2012 - 2019). We excluded FEE patients without CSF-related abnormalities. We compared the number of shunts and the complications between the two groups. One hundred and twenty-nine patient's medical records were reviewed. The males were predominating (79 versus 50 patients) with an average age of 58.2±7.1 months old (6 to 276 months old). Ventriculomegaly was found in 18 cases, arachnoid cysts in 46 cases, porencephalic cysts in 19 cases, and ventricular malformation in 46 cases. Group 1, with a score of 4 to 7 (19 cases), received an early shunt along with the FEE repair. Complications occurred in 7 patients of this group. Group 2, with a score of 4-7, received shunts only after the complication occurred in 3 cases (pseudomeningocele unresponsive with conservative treatment and re-operation in 2 cases; a sign of intracranial hypertension in 1 case). No complication occurred in this group. Groups 1 and 2, with scores of 8 or higher (6 and 8 cases, respectively), underwent direct shunt, with one complication (exposed shunt) in each group. The SAFE decision algorithm for FEE with CSF-related intracranial abnormalities has proven effective in reducing unnecessary shunting and the rate of shunt complications.
Topics: Child; Male; Humans; Child, Preschool; Encephalocele; Hydrocephalus; Brain; Intracranial Hypertension; Neurosurgical Procedures; Cerebrospinal Fluid Shunts; Retrospective Studies
PubMed: 38459217
DOI: 10.1007/s10143-024-02342-y -
International Journal of Surgery Case... Mar 2024Intracranial glioependymal cysts are an uncommon type of neuroepithelial cyst and are encountered much less frequently than arachnoid cysts. These cysts primarily...
INTRODUCTION AND IMPORTANCE
Intracranial glioependymal cysts are an uncommon type of neuroepithelial cyst and are encountered much less frequently than arachnoid cysts. These cysts primarily manifest within the parenchyma of the brain, although exceedingly rare instances have been reported in the lateral ventricles.
CASE PRESENTATION
We present a highly unusual case of a glioependymal cyst in a 7-year-old girl. The glioependymal cyst was located in the midline in the suprasellar region and extended to the upper clivus region. Its only manifestation was precocious puberty. We performed endoscopic fenestration of the cyst, leading to a return of hormonal levels to normal and a slight reduction in cyst size.
CLINICAL DISCUSSION
A comprehensive search of the Medline database revealed only a few documented cases of glioependymal cysts (fewer than 30 cases). Remarkably, the majority (if not all) were located laterally rather than in the midline of the brain. Endoscopic fenestration and biopsy are effective and confirm the diagnosis.
CONCLUSION
This instance of a rare glioependymal cyst located in the midline, spanning the suprasellar and retrosellar regions, is an uncommon occurrence. Its sole presentation was precocious puberty. The successful management of this condition was achieved through an endoscopic approach, leading to the normalization of endocrine abnormalities.
PubMed: 38387370
DOI: 10.1016/j.ijscr.2024.109360 -
European Annals of Otorhinolaryngology,... Feb 2024
PubMed: 38383168
DOI: 10.1016/j.anorl.2024.02.002 -
Child's Nervous System : ChNS :... May 2024Pulsatile CSF flow patterns include flow through the ventricles to the subarachnoid space and cisterns and from the infra- to the supratentorial subarachnoid space. In...
OBJECTIVE
Pulsatile CSF flow patterns include flow through the ventricles to the subarachnoid space and cisterns and from the infra- to the supratentorial subarachnoid space. In this study, we demonstrate how an obstruction at the level of the prepontine space may lead to obstructive hydrocephalus with specific radiological characteristics, as well as the implications for treatment options.
METHODS
We retrospectively collected data of patients who underwent surgery between February 2010 and December 2022 for hydrocephalus secondary to a suspected prepontine block. One additional patient diagnosed with prepontine block who did not undergo surgery was also included. We excluded patients with a background of previous unrelated neurosurgical procedures or CNS infections.
RESULTS
Six children and two adults were included. Three presented with hydrocephalus on imaging, without any other underlying pathology. Five had a suprasellar arachnoid cyst, with its lower border abating the pons and occluding the spinal subarachnoid space (SAS). All cases had an open aqueduct on T2 sagittal sequences, as well as an infracerebellar or retrocerebellar CSF collection. In most cases, a horizontal web was identified in the prepontine region. Seven cases were treated with an endoscopic fenestration. One patient subsequently underwent a shunt surgery. All the operated children reached normal developmental milestones after surgery.
CONCLUSIONS
This paper describes a rather small series of cases where clear obstruction was observed at the level of the prepontine subarachnoid space. We believe this anatomical subtlety adds to a better understanding of CSF pathways and the role of ETV in treating hydrocephalus, focusing on a small subgroup of patients without a clear obstruction.
Topics: Child; Adult; Humans; Retrospective Studies; Treatment Outcome; Hydrocephalus; Cerebral Ventricles; Cerebral Aqueduct; Ventriculostomy
PubMed: 38376529
DOI: 10.1007/s00381-024-06323-w -
Molecular Genetics & Genomic Medicine Feb 2024Intellectual disability (ID) refers to a childhood-onset neurodevelopmental disorder with a prevalence of approximately 1%-3%.
BACKGROUND
Intellectual disability (ID) refers to a childhood-onset neurodevelopmental disorder with a prevalence of approximately 1%-3%.
METHODS
We performed whole exome sequencing for the patient with ID. And the splicing variant we found was validated by minigene assay.
RESULTS
Here, we report a boy with ID caused by a variant of CNKSR2. His neurological examination revealed hypsarrhythmia via electroencephalography and a right temporal polar arachnoid cyst via brain magnetic resonance imaging. A novel splicing variant in the CNKSR2 gene (NM_014927.5, c.1657+1G>A) was discovered by exome sequencing. The variant caused a 166 bp intron retention between exons 14 and 15, which was validated by a minigene assay. The variant was not reported in public databases such as gnomAD and the Exome Aggregation Consortium.
CONCLUSIONS
The variant was predicted to be damaging to correct the translation of the CNKRS2 protein and was classified as likely pathogenic according to the ACMG guidelines.
Topics: Male; Child; Humans; Developmental Disabilities; Mental Retardation, X-Linked; Intellectual Disability; RNA Splicing; Neurodevelopmental Disorders; Adaptor Proteins, Signal Transducing
PubMed: 38337158
DOI: 10.1002/mgg3.2389