-
The Spine Journal : Official Journal of... Dec 2023Spinal arachnoid cysts (SACs) are rare, cerebrospinal fluid-filled sacs lined by an arachnoid membrane in the spinal canal. Symptoms can develop due to pressure on the...
BACKGROUND CONTEXT
Spinal arachnoid cysts (SACs) are rare, cerebrospinal fluid-filled sacs lined by an arachnoid membrane in the spinal canal. Symptoms can develop due to pressure on the spinal cord or adjacent spinal nerves by the cyst itself or by interrupted flow of cerebrospinal fluid. If noninvasive management fails or neurological deterioration occurs, surgical treatment is recommended. However, data is lacking on long-term outcomes after surgery.
PURPOSE
To determine long-term outcomes in patients surgically treated for SACs.
STUDY DESIGN
Population-based cohort-study.
PATIENT SAMPLE
All consecutive patients treated for either intra- or extradural SACs with surgery between 2005 and 2020 at the author's institution were included.
OUTCOME MEASURES
American Spinal Injury Association Impairment Scale (AIS) and modified Japanese Orthopedic Association score (mJOA).
METHODS
Data was primarily extracted from electronic patient medical notes. Telephone interviews were performed to assess long-term postoperative outcomes. All analyses were conducted using the statistical software program R version 4.0.5. Statistical significance was set at p<.05.
RESULTS
Thirty-four patients were included. Cyst excision was performed in 11 (32%) cases, and fenestration in the remaining 23 (68%). The median follow-up time was 8.0 years. Surgery resulted in a significant long-term improvement in both AIS (p=.012) and mJOA (p=.005). Sensory deficit was the symptom that most often improved (81%), followed by pain (74%) and motor function (64%). AIS deteriorated in two patients, of which one case was attributed to a surgical complication. Local cyst recurrence requiring reoperation was seen in 4 (12%) cases, all of them following cyst fenestration. One patient (3%) required reoperation for progression of the cyst progression at a different level.
CONCLUSION
This study reports outcomes of surgically treated SACs with the longest follow-up time to date. Microsurgical cyst excision or fenestration were safe treatment options, and the neurological improvements seen in the immediate postoperative phase were maintained at long-term follow-up.
Topics: Humans; Arachnoid Cysts; Cohort Studies; Spinal Cord Diseases; Laminectomy
PubMed: 37604309
DOI: 10.1016/j.spinee.2023.08.011 -
Case Report: Novel biallelic moderately damaging variants in in a patient with cerebellar dysplasia.Frontiers in Pediatrics 2023Rotatin, encoded by the gene, is a centrosomal protein with multiple, emerging functions, including left-right specification, ciliogenesis, and neuronal migration....
Rotatin, encoded by the gene, is a centrosomal protein with multiple, emerging functions, including left-right specification, ciliogenesis, and neuronal migration. Recessive variants in are associated with a neurodevelopmental disorder with microcephaly and malformations of cortical development known as "Microcephaly, short stature, and polymicrogyria with seizures" (MSSP, MIM #614833). Affected individuals show a wide spectrum of clinical manifestations like intellectual disability, poor/absent speech, short stature, microcephaly, and congenital malformations. Here, we report a subject showing a distinctive neuroradiological phenotype and harboring novel biallelic variants in : the c.5500A>G, p.(Asn1834Asp), (dbSNP: rs200169343, ClinVar ID:1438510) and c.19A>G, p.(Ile7Val), (dbSNP: rs201165599, ClinVar ID:1905275) variants. In particular brain magnetic resonance imaging (MRI) showed a peculiar pattern, with cerebellar hypo-dysplasia, and multiple arachnoid cysts in the lateral cerebello-medullary cisterns, in addition to left Meckel cave. Thus, we compare his phenotypic features with current literature, speculating a possible role of newly identified variants in his clinical picture, and supporting a relevant variability in this emerging condition.
PubMed: 38178912
DOI: 10.3389/fped.2023.1326552 -
Revista de Neurologia Jul 2023
Topics: Humans; Hydrocephalus; Arachnoid Cysts; Intracranial Hypertension
PubMed: 37403245
DOI: 10.33588/rn.7702.2022390 -
Operative Neurosurgery (Hagerstown, Md.) Jan 2024
PubMed: 38198191
DOI: 10.1227/ons.0000000000001061 -
Asian Journal of Neurosurgery Dec 2023Posterior fossa localization of a hydatid cyst is uncommon; in these cases, the cyst usually locates in the cerebellum. Localization within the subarachnoid spaces or...
Posterior fossa localization of a hydatid cyst is uncommon; in these cases, the cyst usually locates in the cerebellum. Localization within the subarachnoid spaces or the cerebrospinal fluid ventricular system is exceptional. In the present report, which appears to be the seventh in the literature, we describe two cases of a hydatid cyst in the cerebellopontine cistern. Magnetic resonance imaging findings revealed a nonneoplastic cystic lesion mimicking an arachnoid cyst. The hydatid nature of the cyst was unexpected preoperatively. In both cases, the cyst was successfully removed using the puncture, aspiration, irrigation, and resection technique via a retrosigmoid approach. Histopathological examination confirmed the cysts to be in nature. Hydatid cyst may be considered in the differential diagnosis of arachnoid cysts of the cerebellopontine cistern to determine which surgical procedure to perform and to avoid unexpected complications. Previous published cases were also discussed.
PubMed: 38161603
DOI: 10.1055/s-0043-1775860 -
Acta Neurochirurgica Sep 2023Management options for treatment of quadrigeminal arachnoid cysts (QAC) include microsurgical/endoscopic fenestration or shunt. There is an open debate about which...
BACKGROUND
Management options for treatment of quadrigeminal arachnoid cysts (QAC) include microsurgical/endoscopic fenestration or shunt. There is an open debate about which method is the best. Microsurgical fenestration is well suited for treatment of QAC with predominant infratentorial component and without hydrocephalus making endoscopic procedures more challenging.
METHOD
We describe the microsurgical technique and related anatomy to fenestrate infratentorial QAC through supracerebellar infratentorial approach. We also discuss our experiences with this approach, some of the drawbacks and nuances.
CONCLUSION
Navigation-guided microsurgical fenestration of infratentorial QAC is the authors' surgical approach of choice for treating these rare challenging lesions when not associated with hydrocephalus.
Topics: Humans; Arachnoid Cysts; Neuronavigation; Endoscopy; Hydrocephalus; Otologic Surgical Procedures
PubMed: 36849831
DOI: 10.1007/s00701-023-05531-8 -
Clinical Neurology and Neurosurgery Jul 2024The Liliequist membrane (LM) represents a crucial yet challenging anatomical structure in neuroanatomy. First observed in 1875 and later elucidated by Bengt Liliequist... (Review)
Review
BACKGROUND
The Liliequist membrane (LM) represents a crucial yet challenging anatomical structure in neuroanatomy. First observed in 1875 and later elucidated by Bengt Liliequist in 1956, the LM's precise anatomical description and boundaries remain complex. Its significance extends to neurosurgery, impacting various procedures like endoscopic third ventriculocisternostomies, aneurysm and tumor surgeries, treatment of suprasellar arachnoid cysts, and managing perimesencephalic hemorrhages. However, a comprehensive understanding of the LM is hindered by inconsistent anatomical descriptions and limitations in available literature, warranting a systematic review.
METHODS
A systematic review was conducted by searching PubMed, Science Direct, and Google Scholar for articles pertaining to Liliequist's membrane. The search employed Mesh terms like "Liliequist membrane," "Liliequist's diaphragm," and related variations. Inclusion criteria encompassed studies exploring the historical evolution, anatomical structure, radiological characteristics, and clinical implications of the LM in neurosurgery.
RESULTS
The search yielded 358 articles, with 276 unique articles screened based on relevance. Following a meticulous screening process, 72 articles underwent full-text assessment, resulting in the inclusion of 5 articles meeting the eligibility criteria. The selected studies varied in methodology, including anatomical dissections, radiological evaluations, and clinical significance in neurosurgical procedures. Insights were derived on LM's anatomical variations, radiological visualization, and its critical role in guiding neurosurgical interventions.
CONCLUSIONS
Despite advancements in understanding its clinical significance and radiological visualization, challenges persist in precisely delineating its boundaries. Further research, especially on embryological development and histological characterization, is essential. Enhancing comprehension of LM-related pathologies is crucial for accurate preoperative planning and optimizing patient outcomes in neurosurgery.
Topics: Humans; Neurosurgical Procedures; History, 20th Century; History, 19th Century; Clinical Relevance
PubMed: 38795689
DOI: 10.1016/j.clineuro.2024.108322 -
British Journal of Neurosurgery Aug 2023We describe an 81-year-old gentleman presenting with mild myelopathic symptoms in the upper limbs. Imaging showed a C1-3 intradural extramedullary lesion initially...
We describe an 81-year-old gentleman presenting with mild myelopathic symptoms in the upper limbs. Imaging showed a C1-3 intradural extramedullary lesion initially thought to be an arachnoid cyst. A C1 + 2 hemilaminectomy and partial excision was performed with histology revealing a neurenteric cyst (NC). NCs are congenital tumours that usually present within the third decade of life, they account for 1% of all spinal tumours. A literature search was conducted and we found that the age of presentation might actually be earlier than previously described. We also found that there has never before been a case described in the eighth decade of life, making this the oldest known symptomatic presentation of this rare condition.
Topics: Male; Humans; Aged, 80 and over; Neural Tube Defects; Laminectomy; Spinal Cord Neoplasms; Arachnoid Cysts; Magnetic Resonance Imaging
PubMed: 31825252
DOI: 10.1080/02688697.2019.1681361 -
Acta Neurochirurgica May 2024In recent years there has been a re-evaluation regarding the clinical implications of temporal lobe arachnoid cysts (temporal arachnoid cysts) in children. These cysts...
BACKGROUND AND PURPOSE
In recent years there has been a re-evaluation regarding the clinical implications of temporal lobe arachnoid cysts (temporal arachnoid cysts) in children. These cysts have often been considered asymptomatic, or if symptomatic, only causing focal neurological symptoms or signs of increased intracranial pressure. However, several studies have more recently reported on cognitive symptoms improving after surgery. This study aimed to evaluate if reported cognitive improvement after surgery of temporal arachnoid cysts were stable after five years.
METHOD
Ten consecutive children (m = 14.65; range 12.1-19.415 were assessed cognitively five years after micro-neurosurgical fenestration of a temporal arachnoid cyst. Results were compared to results from their pre- and post-surgical evaluations. Evaluations included the Wechsler-scales, Boston Naming Test (BNT), Rey Auditory Verbal Learning Test (RAVLT), verbal fluency test (FAS) and Rey Complex Figure Test (RCFT).
RESULTS
The analysis revealed significant postsurgical improvement compared to baseline on the Wechsler-scales measures of general intelligence (FSIQ), verbal abilities (VCI) and processing speed (PSI). Mean differences after surgery were 8.3 for FSIQ, (p = 0.026), 8.5 for VI (p = < .01) and 9.9 for PSI (p = 0.03). There were no significant differences in mean test results when comparing postsurgical scores with scores five years after surgery, indicating long-term stability of improvements.
CONCLUSION
The results indicate that affected cognitive functions in children with temporal arachnoid cysts improve after surgery and that the improvements remain stable five years later. The improvements and long term stability were also consistent with the experience of both parents and children. The findings provide a strong argument for neurosurgical fenestration of temporal arachnoid cysts in children.
Topics: Humans; Arachnoid Cysts; Male; Female; Child; Follow-Up Studies; Adolescent; Cognition; Young Adult; Neurosurgical Procedures; Microsurgery; Neuropsychological Tests; Treatment Outcome; Temporal Lobe
PubMed: 38777952
DOI: 10.1007/s00701-024-06120-z -
Revista Paulista de Pediatria : Orgao... 2023To report a rare case of a patient with a molecular diagnosis of Kleefstra syndrome (KS) who has four other chromosomal alterations involving pathogenic variants.
OBJECTIVE
To report a rare case of a patient with a molecular diagnosis of Kleefstra syndrome (KS) who has four other chromosomal alterations involving pathogenic variants.
CASE DESCRIPTION
Male patient, two years old, with global delay, including in neuropsychomotor development, ocular hypertelorism, broad forehead, brachycephaly, hypotonia, ligament laxity, unilateral single palmar crease and arachnoid cyst. The microarray-based comparative genomic hybridization (a-CGH) identified copy number variations (CNVs) in five regions: 9q34.3, 6p22.1, Yq11.223, Yp11.23, and 2q24.1. The heterozygous microdeletion in 9q34.3 involving the EHMT1 gene confirms the diagnosis of KS.
COMMENTS
The presence of pathogenic CNVs and/or those of uncertain significance, located on chromosomes 2, 6 and Y, may be contributing to a variability in the patient's clinical condition (arachnoid cyst, single palmar fold and ligament laxity), compared to other individuals with only KS genetic alteration, making the dignosis of the disease harder.
Topics: Male; Humans; Child, Preschool; DNA Copy Number Variations; Comparative Genomic Hybridization; Heterozygote; Cysts
PubMed: 37729241
DOI: 10.1590/1984-0462/2024/42/2022230