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World Neurosurgery Apr 2021Arachnoid cysts are benign cerebrospinal fluid collection within a duplication of arachnoid membrane and, when found in the retrocerebellar site, they may be associated...
Coexisting Retrocerebellar Arachnoid Cyst and Chiari Type 1 Malformation: 3 Pediatric Cases of Surgical Management Tailored to the Pathogenic Mechanism and Systematic Review of the Literature.
INTRODUCTION
Arachnoid cysts are benign cerebrospinal fluid collection within a duplication of arachnoid membrane and, when found in the retrocerebellar site, they may be associated with tonsils herniation. This rare situation of coexisting retrocerebellar arachnoid cyst (AC) and Chiari malformation type 1 (CM-1) have been previously reported in few cases (10 patients) with syringomyelia and hydrocephalus described to be the most relevant issues. The aim of this paper is to describe 3 pediatric cases of this condition with a systematic review of the literature, underlining the importance of surgical management tailored to the pathogenic mechanism.
METHODS
A restrospective analysis of patients treated for coexisting CM-1 and ACs at the authors' institution has been carried out.
RESULTS
A case of a 10-month-old baby with coexisting AC and CM-1 with tri-ventricular hydrocephalus treated with endoscopic third ventriculostomy, a case of a 1-year-old child with a huge retrocerebellar AC and CM-1 treated with a cysto-peritoneal shunt, and a case of a 15-year-old child with retrocerebellar AC causing symptomatic CM-1 treated with C0-C2 decompression, AC fenestration and duraplasty are described. A long-term follow-up is reported.
CONCLUSIONS
Surgical management of coexisting ACs and CM-1 should not aim at the complete resolution of the cyst or of tonsil herniation, especially when pediatric patients are treated. Rather, the purpose of the neurosurgeon should be to understand the underlying pathogenic mechanism, and then restoring both the cerebrospinal fluid flow in the posterior fossa and the dynamic equilibrium between ventricles, cyst, and subarachnoid space.
Topics: Adolescent; Arachnoid Cysts; Arnold-Chiari Malformation; Axis, Cervical Vertebra; Cerebrospinal Fluid Shunts; Cranial Fossa, Posterior; Decompression, Surgical; Humans; Hydrocephalus; Infant; Male; Neuroendoscopy; Peritoneal Cavity; Ventriculostomy
PubMed: 33385608
DOI: 10.1016/j.wneu.2020.12.094 -
Neurosurgical Review Feb 2021Foramen magnum decompression (FMD) is widely accepted as the standard treatment for syringomyelia associated with Chiari type I malformation (CMI). Despite extensive... (Meta-Analysis)
Meta-Analysis
Foramen magnum decompression (FMD) is widely accepted as the standard treatment for syringomyelia associated with Chiari type I malformation (CMI). Despite extensive clinical investigations, relevant surgical details are still matter of debate. The authors performed a systematic review and meta-analysis of the literature examining the radiological outcome of syringomyelia in adult patients with CMI after different surgical strategies. PRISMA guidelines were followed. A systematic search of three databases was performed for studies published between 1990 and 2018. Our systematic review included 13 studies with a total of 276 patients with CMI associated with syringomyelia. Overall, the rate of post-operative radiological improvement at last follow-up was 81.1% (95% CI 73.3-88.9%; p < 0.001; I = 71.4%). The rate of post-operative syrinx shrinkage did not differ significantly among both groups of decompression with the extra-arachnoidal technique and arachnoid dissection (90%, 95% CI 85.1-94.8%, I = 0% vs 79.8%, 95% CI 61.7-98%, I = 85.5%). A lower rate of post-operative radiological syrinx shrinkage was observed after decompression with splitting of the outer layer of the dura (55.6% 95% CI 40.5-70.8%, I = 0%). CSF-related complications and infections were similar among the different groups. Our meta-analysis found that FMD with the extra-arachnoidal technique and arachnoid dissection provides similar results in terms of post-operative shrinkage of syringomyelia. Patients undergoing decompression with splitting of the dura presented the lower rate of syrinx reduction. These data should be considered when choosing the surgical approach in adult patients with CMI associated with syringomyelia.
Topics: Arnold-Chiari Malformation; Decompression, Surgical; Foramen Magnum; Humans; Neurosurgical Procedures; Syringomyelia; Treatment Outcome
PubMed: 31953784
DOI: 10.1007/s10143-020-01239-w -
Neurosurgical Review Jun 2024Aim of the present study was to conduct a comprehensive review of surgical strategies that can be offered to patients with trigeminal neuralgia undergoing microvascular... (Review)
Review
A systematic review on the efficacy of adjunctive surgical strategies during microvascular decompression for trigeminal neuralgia without intraoperative evidence of neurovascular conflict.
Aim of the present study was to conduct a comprehensive review of surgical strategies that can be offered to patients with trigeminal neuralgia undergoing microvascular decompression (MVD) surgery and without intraoperative evidence of neurovascular conflict, with a high pre-operative suspicion of conflict lacking intraoperative confirmation, or individuals experiencing recurrence after previous treatment. This systematic review followed established guidelines (PRISMA) to identify and critically appraise relevant studies. The review question was formulated according to the PICO (P: patients; I: intervention; C: comparison; O: outcomes) framework as follows. For patients with trigeminal neuralgia (P) undergoing MVD surgery (I) without demonstrable preoperative neurovascular conflict, high suspicion of conflict but no intraoperative confirmation or recurrence after previous treatment (C), do additional surgical techniques (nerve combing, neurapraxia, arachnoid lysis) (O) improve pain relief outcomes (O)? The search of the literature yielded a total of 221 results. Duplicate records were then removed (n = [76]). A total of 143 papers was screened, and 117 records were excluded via title and abstract screening; 26 studies were found to be relevant to our research question and were assessed for eligibility. Upon full-text review, 17 articles were included in the review, describing the following techniques; (1) internal neurolysis (n = 6) (2) arachnoid lysis/adhesiolysis (n = 2) (3) neurapraxia (n = 3) (4) partial rhizotomy of the sensory root (n = 4) (5) pontine descending tractotomy (n = 2). The risk of bias was assessed using the ROBINS-I (Risk of Bias in Non-randomized Studies - of Interventions) assessment tool. While the described techniques hold promise, further research is warranted to establish standardized protocols, refine surgical approaches, and comprehensively evaluate long-term outcomes.
Topics: Trigeminal Neuralgia; Humans; Microvascular Decompression Surgery; Treatment Outcome
PubMed: 38884812
DOI: 10.1007/s10143-024-02498-7 -
British Journal of Neurosurgery Apr 2023Non-communicating extradural spinal arachnoid cysts (NEACs) are extremely rare aetiology of symptomatic spinal cord compression. The aim of this study was to address...
OBJECTIVE
Non-communicating extradural spinal arachnoid cysts (NEACs) are extremely rare aetiology of symptomatic spinal cord compression. The aim of this study was to address their pathogenesis, optimum management strategy and outcome through systematic review of existing published studies.
MATERIALS AND METHOD
We have found 13 eligible publications by searching through PubMed, ScienceDirect, and Google Scholar databases, published from inception to December 2020. We have analysed the data of 21 patients extracted from those 13 publications by IBM SPSS version 23.
RESULTS
According to our analysis congenital predisposition, trauma, and previous surgery history are the aetiology of NEAC. Clinical presentation of cyst depends upon the location and extent of compression or involvement of the neurovascular structures. Paraparesis with variable degree of sensory disturbance was seen among patients. Based on neuroimaging findings, NEACs are most commonly found at dorsal and dorsolumbar region. Magnetic resonance imaging (MRI) is the diagnostic modalities of choice and CT myelography can demonstrate the communication with the subarachnoid space. Recurrence rate of cyst after surgery is very low as only one out of twenty patients showed recurrence. If dural defect is not accurately addressed, the recurrence rate increased significantly.
CONCLUSIONS
Our study has highlighted aetiology, treatment strategies, and neurological outcome of NEAC. These findings may help neurosurgeons to manage this rare surgical entity for favourable outcome.
Topics: Humans; Arachnoid Cysts; Spinal Cord Diseases; Spinal Cord Compression; Magnetic Resonance Imaging; Tomography, X-Ray Computed
PubMed: 35766304
DOI: 10.1080/02688697.2022.2090505 -
Clinical Neurology and Neurosurgery May 2021Large interhemispheric cysts (IHC) with partial or complete agenesis of corpus callosum (ACC) constitute a heterogeneous group of rare disorders. Neurosurgical...
OBJECTIVE
Large interhemispheric cysts (IHC) with partial or complete agenesis of corpus callosum (ACC) constitute a heterogeneous group of rare disorders. Neurosurgical treatment, in the terms of if, when and how to operate, remains unclear METHODS: We performed a surgical literature review of series or reports of IHCs with callosal anomalies; we evaluated whether revision surgeries were necessary and considered the dimensional change in the cyst postoperatively and the developmental outcome. We also reported our experience with sfour patients treated by programmable cysto-peritoneal (CP) shunting. Patients' clinical history, neuroradiological and neuropsychological performances were evaluated pre and post operatively.
RESULTS
The review included 133 patients with surgically-treated IHCs. Although most authors are in agreement to perform surgery if the patients present signs of raised ICP and to not intervene in those completely asymptomatic, for other signs and symptoms the debate is still open; only few authors performed cognitive tests pre and post-operatively. Shunting procedures were successful in 60% of our reviewed cases and often lead to a major cyst collapse. Craniotomy achieves good results but is extremely invasive. Endoscopy is minimally invasive and our review demonstrated a success rate of 66%. However, endoscopy does not ensue a complete cyst collapse. Our series and review seem to suggest a possible link between parenchymal re-expansion and cognitive outcome.
CONCLUSIONS
Early and effective surgery seems to obtain a greater cerebral parenchyma re-expansion and long-term cognitive evolution. Endoscopy is safe and reliable, but more data is needed on the impact of uncomplete cyst collapse on neurocognitive outcome.
Topics: Agenesis of Corpus Callosum; Central Nervous System Cysts; Cerebrospinal Fluid Shunts; Craniotomy; Humans; Treatment Outcome
PubMed: 33799088
DOI: 10.1016/j.clineuro.2021.106600 -
Anatomical Record (Hoboken, N.J. : 2007) Jun 2024The existence of a previously unrecognized subarachnoid lymphatic-like membrane (SLYM) was reported in a recent study. SLYM is described as an intermediate... (Review)
Review
The existence of a previously unrecognized subarachnoid lymphatic-like membrane (SLYM) was reported in a recent study. SLYM is described as an intermediate leptomeningeal layer between the arachnoid and pia mater in mouse and human brains, which divides the subarachnoid space (SAS) into two functional compartments. Being a macroscopic structure, having missed detection in previous studies is surprising. We systematically reviewed the published reports in animals and humans to explore whether prior descriptions of this meningeal layer were reported in some way. A comprehensive search was conducted in PubMed/Medline, EMBASE, Google Scholar, Science Direct, and Web of Science databases using combinations of MeSH terms and keywords with Boolean operators from inception until 31 December 2023. We found at least eight studies that provided structural evidence of an intermediate leptomeningeal layer in the brain or spinal cord. However, unequivocal descriptions for this layer all along the central nervous system were scarce. Obscure names like the epipial, intermediate meningeal, outer pial layers, or intermediate lamella were used to describe it. Its microscopic/ultrastructural details closely resemble the recently reported SLYM. We further examined the counterarguments in current literature that are skeptical of the existence of this layer. The potential physiological and clinical implications of this new meningeal layer are significant, underscoring the urgent need for further exploration of its structural and functional details.
PubMed: 38924700
DOI: 10.1002/ar.25524 -
Neurosurgical Review Mar 2023A series of 5 patients treated with the fourth ventricle to spinal subarachnoid space stent (FVSSS) is presented. Indication for surgery, surgical technique,...
A series of 5 patients treated with the fourth ventricle to spinal subarachnoid space stent (FVSSS) is presented. Indication for surgery, surgical technique, pre-operative and post-operative images, and outcome are analyzed. A systematic review of the pertinent literature has also been performed. This is a retrospective cohort review of a series of 5 consecutive patients with refractory syringomyelia who underwent a fourth ventricle to spinal subarachnoid space shunt surgery. The surgical indication was based on the presence of refractory syringomyelia in patients already treated for Chiari malformation or in patients who developed scarring at the level of the outlets of the fourth ventricle following posterior fossa tumor surgery. The mean age at FVSSS was 11.30 ± 5.88 years. Cerebral MRI revealed crowded posterior fossa, with a membrane at the level of the foramen of Magendie. Spinal MRI showed syringomyelia in all patients. Before surgery, the averages of the craniocaudal and the anteroposterior diameter were 22.66 and 1.01 cm, respectively, whereas the volume was 28.16 cm. The post-operative period was uneventful in 4 out of 5 patients; one child died on the 1st post-operative day due to complications unrelated to surgery. In remaining cases, syrinx marked improvement. The post-operative volume was 1.47 cm with an overall reduction of 97.61%. With regard to literature, 7 articles with a total of 43 patients were analyzed. After FVSSS, syringomyelia reduction was observed in 86.04% of cases. Three patients underwent reoperation due to syrinx recurrence. Four patients presented a catheter displacement, one a wound infection and meningitis and one CSF leak requiring placement of a lumbar drain. FVSSS is highly effective in restoring CSF dynamics, with dramatic improvement of syringomyelia. In all our cases, the volume of the syrinx was reduced by at least 90%, with improvement/resolution of accompanying symptomatology. This procedure should be reserved to patients in which other causes of gradient pressure between the fourth ventricle and subarachnoid space are excluded, for example, tetraventricular hydrocephalus. Surgical procedure is not simple, because it requires meticulous microdissection of cerebello-medullary fissure and upper cervical spine, in already operated patients. To avoid migration of the stent, it should be carefully sutured to the dura mater or thick arachnoid membrane.
Topics: Adolescent; Child; Child, Preschool; Humans; Arnold-Chiari Malformation; Decompression, Surgical; Fourth Ventricle; Magnetic Resonance Imaging; Retrospective Studies; Stents; Subarachnoid Space; Syringomyelia; Treatment Outcome
PubMed: 36905420
DOI: 10.1007/s10143-023-01972-y -
World Neurosurgery Feb 2024The role of surgical management of arachnoid cyst (AC) of the cerebellopontine angle (CPA) is uncertain. This topic has remained controversial with varying contradictory...
OBJECTIVE
The role of surgical management of arachnoid cyst (AC) of the cerebellopontine angle (CPA) is uncertain. This topic has remained controversial with varying contradictory recommendations in the literature, which is limited to mostly case reports. We aimed to provide a comprehensive summary and analysis of symptoms, operative techniques, outcomes, and recurrence of all available surgical cases of AC of the CPA to date.
METHODS
A systematic literature search was performed in May 2022 querying several scientific databases. Inclusion criteria specified all studies and case reports of patients with AC located at the CPA for which any relevant surgical procedures were performed.
RESULTS
A total of 55 patients from the literature and 5 treated at our institution were included. Mean patient age was 29 years (range, 0.08-79 years), with nearly twice (1.7×) as many female as male patients (37 female, 22 male). Headaches (35%), hearing loss (30%), vertigo (22%), and ataxia (22%) were the most common presentations. Following surgery, 95% experienced symptom improvement, with complete resolution in 64%. Of patients with hearing loss, 44% reported a return to normal. The rate of mortality was 1.69%, and 10% of tumors recurred (mean follow-up 2.3 years [range, 0-15 years].
CONCLUSIONS
Symptomatic AC of the CPA is rare. It exhibits a proclivity for females and commonly manifests with headache, hearing loss, vertigo, and ataxia. While careful selection for surgical candidacy is needed and intervention should be reserved for patients with severe symptoms, surgical decompression is an effective tool for symptom alleviation and recovery.
Topics: Humans; Male; Female; Infant, Newborn; Infant; Child, Preschool; Child; Adolescent; Young Adult; Adult; Middle Aged; Aged; Cerebellopontine Angle; Hearing Loss; Headache; Vertigo; Deafness; Arachnoid Cysts; Ataxia
PubMed: 38070740
DOI: 10.1016/j.wneu.2023.12.018 -
World Neurosurgery Jul 2020Idiopathic ventral spinal cord herniation (ISCH) is a rare disease; however, it is an important differential diagnosis. Its treatment presents some controversies.
BACKGROUND
Idiopathic ventral spinal cord herniation (ISCH) is a rare disease; however, it is an important differential diagnosis. Its treatment presents some controversies.
CASE DESCRIPTION
We report on a 55-year-old woman who had been presenting with relevant back pain and leg weakness for the past 3 years and urinary incontinence for the past 3 months. Clinical examination disclosed paresis on the right inferior limb and right foot, as well as a T6-level painful hypoesthesia. Magnetic resonance imaging disclosed a T4/T5 ISCH. The patient underwent surgical decompression. During the procedure, we opened the arachnoid and cut the dentate ligaments of the spine, which considerably improved the mobility and safety of the procedure. In the early follow-up, our patient presented a partial improvement regarding the paresis grades and hypoesthesia pain relief on the left side. A video showing the surgical procedure and case evolution is presented. We also assembled literature reviews to compare our case with others. ISCH is becoming a more recognized cause of progressive thoracic myelopathy. However, this condition is still frequently misdiagnosed. Magnetic resonance imaging is the key for diagnosis. The objective of surgical treatment is to prevent myelopathy progression. The technique presented in this report is an appropriate surgical option, once it is a safer way to identify and treat the defect. The neurologic condition usually improves greatly after surgical treatment, especially when the patient presents positive predictive factors.
CONCLUSIONS
ISCH is being more recognized. The technique presented is an appropriate surgical option.
Topics: Female; Hernia; Herniorrhaphy; Humans; Middle Aged; Spinal Cord; Thoracic Vertebrae
PubMed: 32376383
DOI: 10.1016/j.wneu.2020.04.190 -
Frontiers in Endocrinology 2024Acromegaly is a rare endocrine disorder caused by hypersecretion of growth hormone (GH) from a pituitary adenoma. Elevated GH levels stimulate excess production of...
UNLABELLED
Acromegaly is a rare endocrine disorder caused by hypersecretion of growth hormone (GH) from a pituitary adenoma. Elevated GH levels stimulate excess production of insulin-like growth factor 1 (IGF-1) which leads to the insidious onset of clinical manifestations. The most common primary central nervous system (CNS) tumors, meningiomas originate from the arachnoid layer of the meninges and are typically benign and slow-growing. Meningiomas are over twice as common in women as in men, with age-adjusted incidence (per 100,000 individuals) of 10.66 and 4.75, respectively. Several reports describe co-occurrence of meningiomas and acromegaly. We aimed to determine whether patients with acromegaly are at elevated risk for meningioma. Investigation of the literature showed that co-occurrence of a pituitary adenoma and a meningioma is a rare phenomenon, and the majority of cases involve GH-secreting adenomas. To the best of our knowledge, a systematic review examining the association between meningiomas and elevated GH levels (due to GH-secreting adenomas in acromegaly or exposure to exogenous GH) has never been conducted. The nature of the observed coexistence between acromegaly and meningioma -whether it reflects causation or mere co-association -is unclear, as is the pathophysiologic etiology.
SYSTEMATIC REVIEW REGISTRATION
https://www.crd.york.ac.uk/prospero/, identifier CRD42022376998.
Topics: Humans; Meningioma; Acromegaly; Meningeal Neoplasms; Human Growth Hormone; Risk Factors; Adenoma
PubMed: 38919490
DOI: 10.3389/fendo.2024.1407615