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1.
Cureus ; 16(3): e55676, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-38586684

RESUMO

Chiari malformation (CM) type 1 is a complex neurological disorder characterized by the displacement of the cerebellar tonsils into the upper spinal canal. Hydrosyringomyelia (HSM), which frequently coexists with this condition, presents diagnostic and treatment problems due to its broad spectrum of symptoms. There are various forms of CMs, with CM type 1 (CM1) being the most common type. Magnetic resonance imaging (MRI) is the best imaging technique to properly identify and diagnose CM1 and HSM. Important imaging findings include downward displacement of the cerebellar tonsils across the foramen magnum, the appearance of the syrinx in the spinal cord, and the alteration of the flow dynamics of the cerebrospinal fluid. This study was conducted at Datta Meghe Medical College, Nagpur, and Government Medical College & Super Speciality Hospital, Nagpur, India. It focuses on the diagnostic use of MRI in CM1 and its variations associated with HSM. Individuals who are asymptomatic may not need any treatment; however, those who are symptomatic or have HSM may require surgical decompression and restoration of the flow. We discuss the findings of MRI of six cases of CM1 and its variants with HSM and search for possible underlying causes. We conclude that magnetic resonance imaging is an imaging modality for the identification and evaluation of CM1 in cases of HSM.

2.
Front Neurol ; 15: 1309718, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38327627

RESUMO

Background: Acquired prolapse of the cerebellar tonsils in spontaneous intracranial hypotension (SIH) patients is rare. This study aims to evaluate neuroimaging changes of acquired prolapse of the cerebellar tonsils below the foramen magnum in SIH patients due to spontaneous spinal cerebrospinal fluid leakage, which was treated by targeted epidural blood patches (EBP). Methods: We retrospectively reviewed clinical and neuroimaging characteristics of 5 cases of SIH with acquired prolapse of the cerebellar tonsils that received targeted EBP in our institution from January 2013 to December 2016. Results: Of these SIH patients, all of them suffered from an orthostatic headache. Initial cranial MRI demonstrated descent of the cerebellar tonsils ≥5 mm. Intrathecal gadolinium-enhanced spinal MR myelography and/or spinal MR hydrography were performed to evaluate the level of spinal cerebrospinal fluid leakage. Symptoms were alleviated in all 5 patients after two (n = 4), or three (n = 1) targeted EBP during hospitalization. Follow-up cranial MRI revealed that the descent of cerebellar tonsils was reversed after EBP treatment. Conclusion: Acquired tonsillar herniation can occur in patients with SIH and spinal cerebrospinal fluid leakage. Symptoms of these patients may be resolved and radiologic findings may be reversed after EBP treatment.

3.
BMC Neurol ; 23(1): 59, 2023 Feb 03.
Artigo em Inglês | MEDLINE | ID: mdl-36737721

RESUMO

BACKGROUND: Gorham-Stout disease (GSD) is a very rare disorder characterized by massive osteolysis of poorly understood aetiology. The association between GSD involving the skull base and cerebrospinal fluid (CSF) leakage has been reported in the literature. However, few cases of CSF leakage and Chiari-like tonsillar herniation in GSD involving the spine have been reported. CASE PRESENTATION: We present the case of a 20-year-old man with GSD involving the thoracic and lumbar spine, which caused CSF leakage and Chiari-like tonsillar herniation. The patient underwent four spinal surgeries for osteolytic lesions of the spine over a 10-year period. Here, we discuss the possible aetiology of the development of CSF leakage. Epidural blood patch (EBP) was performed at the T11-T12 level to repair the CSF leakage. After EBP treatment, rebound intracranial hypertension (RIH) developed, and tonsillar herniation disappeared 2 months later. CONCLUSIONS: GSD involving the spine with CSF leakage and Chiari-like tonsillar herniation is relatively rare. For patients who have undergone multiple spinal surgeries, minimally invasive treatment is an alternative treatment for CSF leakage. EBP can repair CSF leakage secondary to GSD and improve chronic brain sagging, with reversibility of Chiari-like malformations.


Assuntos
Malformação de Arnold-Chiari , Osteólise Essencial , Masculino , Humanos , Adulto Jovem , Adulto , Osteólise Essencial/complicações , Osteólise Essencial/cirurgia , Osteólise Essencial/patologia , Encefalocele/complicações , Encefalocele/cirurgia , Encefalocele/patologia , Vazamento de Líquido Cefalorraquidiano/diagnóstico por imagem , Vazamento de Líquido Cefalorraquidiano/etiologia , Vazamento de Líquido Cefalorraquidiano/cirurgia , Encéfalo/patologia , Malformação de Arnold-Chiari/complicações , Malformação de Arnold-Chiari/cirurgia
4.
Br J Neurosurg ; 37(5): 963-966, 2023 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-30522360

RESUMO

Tonsillar herniation is a rare and seldom reported complication after lumboperitoneal (LP) shunting. There have been only few reports that have presented possible options for treatment with varying degrees of success. In this report, we describe a rare case of tonsillar herniation after LP shunting and review related literature.A 17-year-old girl with hydrocephalus related to a traumatic brain injury underwent implantation of an adjustable pressure shunt (valve setting2.5) and a small lumen peritoneal catheter via the L4-L5 interspinal space. One month later, she was admitted to the emergency room with a Glasgow Coma Scale score ofE1M1Vt and dilated pupil. Image studies demonstrated new-onset tonsillar herniation and a mild cervical syrinx. Emergent suboccipital decompressive craniectomy, C1 laminectomy, and duraplasty were performed. This was followed with ligation of the LP shunt and implantation of a ventriculoperitoneal (VP) shunt a few days later. The patient's Glasgow Coma Scale score gradually recovered to 6, which was her previous neurologic status.Tonsillar herniation as a complication after LP shunting is best treated with decompression, ligation or removal of the LP shunt, and a shift to a VP shunt. The tonsillar herniation should be rapidly addressed to avoid persistent symptoms or progression of the neurologic deficits.


Assuntos
Malformação de Arnold-Chiari , Hidrocefalia , Humanos , Feminino , Adolescente , Encefalocele/diagnóstico por imagem , Encefalocele/etiologia , Encefalocele/cirurgia , Malformação de Arnold-Chiari/cirurgia , Derivação Ventriculoperitoneal/efeitos adversos , Hidrocefalia/cirurgia , Hidrocefalia/complicações , Procedimentos Neurocirúrgicos/efeitos adversos
5.
Asian J Neurosurg ; 17(3): 515-520, 2022 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-36398186

RESUMO

Syringomyelia is a fluid-filled cyst within the spinal cord and is usually associated with conditions that obstruct the cerebrospinal fluid (CSF) (flow at the foramen magnum or spinal levels such as Chiari malformations, arachnoiditis, and basilar invaginations). Very rarely, posterior cranial fossa tumors can lead to tonsillar herniation and secondary syringomyelia. There are only nine reported cases in the literature. We report a rare case of a 56-year-old female with posterior cranial meningioma and secondary syringomyelia, admitted with headache, nausea, vomiting, and ataxic gait. Magnetic resonance imaging (MRI) demonstrated a large posterior fossa lesion causing early ventriculomegaly and a giant cervicothoracic syrinx within the upper spinal cord extending from the hindbrain inferiorly to the level of T8. She underwent a posterior fossa craniectomy with left C1 hemilaminectomy and complete excision of the tumor. In 6 months following her procedure, an MRI scan showed a significant reduction in the caliber of the syringomyelia throughout its length, and there was a significant improvement in symptoms. Although the pathophysiology of syrinx formation is still poorly understood, the alteration of CSF dynamic flow has been implicated. A common unifying cause appears to be increased transcranial difference in intracranial pressure across the foramen magnum causing tonsillar herniation, irrespective of location in the posterior fossa. With high syrinx pressure, mechanical stress-induced structural change of the spinal cord occurs, allowing the persistence and progression of the syrinx in the spinal cord. Syringomyelia appears to be resolved partially or completely after craniotomy and excision of the posterior fossa lesion.

6.
J Neurosurg Case Lessons ; 3(15)2022 Apr 11.
Artigo em Inglês | MEDLINE | ID: mdl-36303496

RESUMO

BACKGROUND: Cerebrospinal fluid (CSF) venous fistulas are a recently discovered and underdiagnosed cause of spontaneous spinal CSF leak, which may lead to spontaneous intracranial hypotension. Most cases occur in the thoracic spine, and only 2 cases were reported in the cervical spine. Treatments include the epidural blood patch, fibrin glue injection, and surgical ligation of the fistula. OBSERVATIONS: The authors report the treatment of a C6-7 CSF venous fistula, for which direct ligation was not feasible, with suboccipital decompression, leading to the complete resolution of the symptoms. Based on the clinical course and outcome in our patient, the authors summarize the previous theory and propose a hypothesis for the pathophysiology of headache and other symptoms in patients with CSF venous fistulas. LESSONS: The symptoms of CSF venous fistulas may be linked not only to intracranial hypotension but also to the altered CSF dynamics induced by tonsillar herniation. Suboccipital decompression should be considered as a potential treatment option, especially in patients with Valsalva-induced headache who show a poor response to surgical ligation, patients in whom surgical ligation is not feasible, and patients with foramen magnum obstruction. Further investigation of the pathophysiology of CSF venous fistulas is warranted and should be performed in the future.

7.
J Neurosurg Pediatr ; 30(2): 210-216, 2022 08 01.
Artigo em Inglês | MEDLINE | ID: mdl-35916100

RESUMO

OBJECTIVE: Chiari malformation type I (CM-I) is frequent in children and remains a surgical challenge. Several techniques have been described for posterior fossa decompression. No decision algorithm has been validated, and strategies are highly variable between institutions. The goal of this study was to define therapeutic guidelines that take into consideration patient specificities. METHODS: The authors retrospectively collected data from patients who were < 18 years of age, were diagnosed with CM-I, and were treated surgically between 2008 and 2018 in 8 French pediatric neurosurgical centers. Data on clinical features, morphological parameters, and surgical techniques were collected. Clinical outcomes at 3 and 12 months after surgery were assessed by the Chicago Chiari Outcome Scale. The authors used a hierarchical clustering method to define clusters of patients by considering their anatomical similarities, and then compared outcomes between surgical strategies in each of these clusters. RESULTS: Data from 255 patients were collected. The mean age at surgery was 9.6 ± 5.0 years, syringomyelia was reported in 60.2% of patients, the dura mater was opened in 65.0% of patients, and 17.3% of patients underwent a redo surgery for additional treatment. The mean Chicago Chiari Outcome Scale score was 14.4 ± 1.5 at 3 months (n = 211) and 14.6 ± 1.9 at 12 months (n = 157). The hierarchical clustering method identified three subgroups with potentially distinct mechanisms underlying tonsillar herniation: bony compression, basilar invagination, and foramen magnum obstruction. Each cluster matched with specific outcomes. CONCLUSIONS: This French multicenter retrospective cohort study enabled the identification of three subgroups among pediatric patients who underwent surgery for CM-I, each of which was associated with specific outcomes. This morphological classification of patients might help in understanding the underlying mechanisms and providing personalized treatment.


Assuntos
Malformação de Arnold-Chiari , Malformação de Arnold-Chiari/complicações , Criança , Estudos de Coortes , Descompressão Cirúrgica/métodos , Dura-Máter/cirurgia , Humanos , Estudos Retrospectivos , Resultado do Tratamento
8.
J Neurosurg Pediatr ; : 1-12, 2022 Jul 22.
Artigo em Inglês | MEDLINE | ID: mdl-35901679

RESUMO

OBJECTIVE: The authors' objective was to measure the effect of posterior cranial vault distraction (PCVD) plus foramen magnum decompression (FMD) on dural sinus volume and venous flow in patients with syndromic craniosynostosis. METHODS: The volumes of the sagittal, straight, transverse, and sigmoid sinuses of 5 consecutive patients with syndromic craniosynostosis who underwent PCVD+FMD were calculated in cubic centimeters with T2-weighted volumetric MRI sequences before surgery, immediately after surgery, and after the end of the distraction process. Tridimensional reconstructions of phase-contrast magnetic resonance angiography (PC-MRA) images were obtained with multiplanar reconstruction (MPR). RESULTS: The average total volume of all dural sinuses increased immediately after surgery (from 10.06 cm3 to 12.64 cm3) and continued to increase throughout the 30-day distraction period (from 12.64 cm3 to 14.71 cm3) (p = 0.04), except that the right sigmoid sinus remained stable after the initial increase. The most important increases were observed for the left transverse sinus (+113.2%), right transverse sinus (+104.3%), left sigmoid sinus (+91.3%), and sagittal sinus (+41.8%). Less important modifications were evident for the right sigmoid sinus (+33.7%) and straight sinus (+23.4%). Significant improvements in venous flow were noted on the tridimensional reconstructions of the PC-MRA images. Venous obstruction grading score improved in 4 patients (average [range] 2.4 [ 2-5]) (p = 0.023) and remained stable in 1 patient. All patients had chronic tonsillar herniation (CTH) (mean [range] 16.6 [8-26] mm), and 3 had syringomyelia. CTH showed improvement on the last follow-up MRI evaluation in 4 patients (mean [range] 10.5 [0-25] mm) and worsened from 15 mm to 19 mm in 1 patient. Syringomyelia improved in 2 patients and remained unchanged in 1. CONCLUSIONS: This study has provided the first radiological evidence of the impact of craniofacial surgery on dural sinus anatomy and venous drainage. The venous anomalies described in patients with syndromic craniosynostosis are not static, and PCVD+FMD triggers a dynamic process that can lead to significant modifications of intracranial venous drainage. The traction exerted by the distracted bone flap onto the occipitoparietal dura mater adherent to the inner calvaria may account for the enlargement of the dural sinus throughout the distraction period. The impact of these modifications on venous pressure, intracranial pressure, CTH, and hydrocephalus remains to be determined.

9.
Cureus ; 14(2): e22233, 2022 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-35340478

RESUMO

Well delineated precipitating factors of migraine or incapacitating headaches are well known in the literature. Few peculiar and under-recognized precipitants are crying, shouting, straining in stools, urination, orgasm, childbirth, powerlifting. We present a case of a young student whose laughing aloud is a potent headache precipitant and is consistently reproducible despite normal brain imaging. It is worth mentioning here that laugh-induced headache has recently been assigned a place in the International Classification of Headache Disorders (ICHD-III) in 2018. The proposed pathophysiology in our case could be loud laugh induced Valsalva maneuver raising intra-abdominal and intra-thoracic pressure momentarily causing venous congestion of head presenting as episodic headache. Another plausible explanation related to craniospinal pressure dissociation and the concept of dural elasticity and compliance needs to be explored if the symptoms persist and repeat scans show no pathology. Momentarily rise of intracranial pressure due to vigorous laugh could press the tonsils or distal cerebellar portion to herniate down transiently, causing symptoms and may be back to normal position once the laugh ceases. Social laughter releases enormous endogenous opioids, which is supported using positron emission tomography (PET) and u-opioid-receptor (MOR)-specific ligand carfentanil. A mirthful laugh could trigger a primary laugh headache. The role of modulated opioidergic activity and social mirthful laugh, if connected with such rare headaches requires further study.

10.
J Plast Reconstr Aesthet Surg ; 75(2): 797-805, 2022 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-34799294

RESUMO

PURPOSE: Cerebellar tonsillar herniation (TH) occurs frequently in syndromic craniosynostosis; however, the exact pathogenesis is unknown. This study evaluates the association between skull base deformities and TH in syndromic craniosynostosis. METHODS: Retrospective study MRI study comparing syndromic craniosynostosis to controls. Measured parameters included clivus length, skull base angle, Boogard's angle, foramen magnum area, and cerebellar tonsillar position (TP). The association between skull base parameters and TP was evaluated with linear mixed models, correcting for age and risk factors for TH in craniosynostosis (hydrocephalus, intracranial hypertension, craniocerebral disproportion, and lambdoid synostosis). RESULTS: Two hundred and eighty-two scans in 145 patients were included, and 146 scans in 146 controls. The clivus was smaller at birth, and its growth was retarded in all syndromes. The skull base angle was smaller at birth in Apert and Crouzon syndromes, and the evolution through time was normal. Boogard's angle was smaller at birth in Apert syndrome, and its evolution was disturbed in Apert and Saethre-Chotzen syndromes. The foramen magnum was smaller at birth in Crouzon and Saethre-Chotzen syndromes, and its growth was disturbed in Apert, Crouzon, and Saethre-Chotzen syndromes. TP was higher at birth in Apert syndrome, but lowered faster. In Crouzon syndrome, TP was lower at birth and throughout life. A smaller clivus and larger foramen magnum were associated with a lower TP in controls (p<0.001, p=0.007), and in Crouzon syndrome, this applied to only foramen magnum size (p=0.004). CONCLUSION: The skull base and its growth are significantly different in syndromic craniosynostosis compared to controls. However, only foramen magnum area is associated with TP in Crouzon syndrome.


Assuntos
Acrocefalossindactilia , Disostose Craniofacial , Craniossinostoses , Disostose Craniofacial/diagnóstico por imagem , Craniossinostoses/complicações , Humanos , Lactente , Recém-Nascido , Estudos Retrospectivos , Base do Crânio/diagnóstico por imagem , Síndrome
11.
Ann Med Surg (Lond) ; 70: 102776, 2021 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-34540210

RESUMO

INTRODUCTION: and Importance: Acute ischemic stroke is a rare event in children with leukemia, yet with long-term morbidity, substantial health, and economic cost. Central nervous system (CNS) leukemic involvement and chemotherapy-related stroke are the most common causes. Hyperleukocytosis induced stroke is very rarely reported. CASE PRESENTATION: A 2-year-old male child presented with hyperleukocytosis (leukocyte count was 320x109/L). Bone marrow evaluation revealed T-cell acute lymphoblastic leukemia. He was treated with dexamethasone, vincristine and daunorubicin, and on day 4 of chemotherapy, he developed abnormal movements, altered mental status, limb weakness and mutism. Magnetic resonance imaging of the brain showed multifocal infarctions involving left pons and both cerebellar hemispheres causing tonsillar herniation with restricted diffusion and mild hydrocephalus but no leptomeningeal enhancement or leukemic infiltrates. Magnetic resonance angiography did not show any arterial stenosis. He was intubated, sedated and managed conservatively with dexamethasone. Cytologic analysis of cerebrospinal fluid showed no blasts. Thrombophilia work up was negative. Five weeks later, the patient had significant improvement in overall neurologic status. He is free of leukemia. MRI showed interval resolution of previous infarcts. CLINICAL DISCUSSION: Hyperviscosity secondary to hyperleukocytosis was considered to be the most likely explanation for this patient stroke after excluding thrombophilia and leukemic infiltration. Prompt management with hydration and careful chemotherapy resulted in good outcome in our patient. CONCLUSION: This case demonstrate the value of early recognition and prompt management of posterior circulation ischemic stroke in children with leukemia and hyperleukocytosis at presentation.

14.
World Neurosurg ; 155: e453-e459, 2021 11.
Artigo em Inglês | MEDLINE | ID: mdl-34455094

RESUMO

BACKGROUND: Radiographic characterization of Chiari malformation (CM) has historically focused on caudal tonsillar herniation (CH) below the foramen magnum. Previously, we published evidence linking ventral tonsillar herniation (VH) and medullary symptoms in very young children. We sought to extend that investigation by studying the radiographic and clinical significance of VH in adults diagnosed with CM. METHODS: We retrospectively reviewed adults with cerebellar ectopia who underwent posterior fossa decompression with or without duraplasty (PFD/D) at our institution. VH was defined as tonsils crossing a line bisecting the caudal medulla at the level of the foramen magnum on axial MRI. Degree of VH was measured as distance between this bisecting line and the ventral tip of the herniated tonsil. Dorsal brainstem compression was qualitatively determined by assessing for obliteration of CSF space between the dorsal brainstem and the tonsils. RESULTS: Out of 89 cases reviewed, 54 had some degree of VH. Compared with those without VH, the VH group was significantly older in age and more likely to also present with dorsal brainstem compression and headaches. No correlation was observed between degrees of CH and VH in the VH group. The degree of VH significantly decreased 3 months after PFD/D. CONCLUSIONS: VH is relatively common in CM patients and might be an important independent radiographic metric to evaluate and consider as part of the decision-making process, especially in those presenting with Chiari-like symptomatology referable to the medulla but who do not meet the traditional criteria of cerebellar ectopia greater than 5 mm.


Assuntos
Malformação de Arnold-Chiari/diagnóstico por imagem , Malformação de Arnold-Chiari/cirurgia , Cerebelo/diagnóstico por imagem , Cerebelo/cirurgia , Cefaleia/diagnóstico por imagem , Cefaleia/cirurgia , Adulto , Malformação de Arnold-Chiari/complicações , Estudos de Coortes , Fossa Craniana Posterior/diagnóstico por imagem , Fossa Craniana Posterior/cirurgia , Feminino , Seguimentos , Cefaleia/etiologia , Humanos , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Estudos Retrospectivos
15.
J Neurosurg Pediatr ; 28(3): 260-267, 2021 Jun 25.
Artigo em Inglês | MEDLINE | ID: mdl-34171843

RESUMO

OBJECTIVE: Chiari malformation type I (CM-I) is a congenital and developmental abnormality that results in tonsillar descent 5 mm below the foramen magnum. However, this cutoff value has poor specificity as a predictor of clinical severity. Therefore, the authors sought to identify a novel radiographic marker predictive of clinical severity to assist in the management of patients with CM-I. METHODS: The authors retrospectively reviewed 102 symptomatic CM-I (sCM-I) patients and compared them to 60 age-matched normal healthy controls and 30 asymptomatic CM-I (aCM-I) patients. The authors used the fourth ventricle roof angle (FVRA) to identify fourth ventricle "bowing," a configuration change suggestive of fourth ventricle outlet obstruction, and compared these results across all three cohorts. A receiver operating characteristic (ROC) curve was used to identify a predictive cutoff for brainstem dysfunction. Binary logistic regression was used to determine whether bowing of the fourth ventricle was more predictive of brainstem dysfunction than tonsillar descent, clival canal angle, or obex position in aCM-I and sCM-I patients. RESULTS: The FVRA had excellent interrater reliability (intraclass correlation 0.930, 95% CI 0.905-0.949, Spearman r2 = 0.766, p < 0.0001). The FVRA was significantly greater in the sCM-I group than the aCM-I and healthy control groups (59.3° vs 41.8° vs 45.2°, p < 0.0001). No difference was observed between aCM-I patients and healthy controls (p = 0.347). ROC analysis indicated that an FVRA of 65° had a specificity of 93% and a sensitivity of 50%, with a positive predictive value of 76% for brainstem dysfunction. FVRA > 65° was more predictive of brainstem dysfunction (OR 5.058, 95% CI 1.845-13.865, p = 0.002) than tonsillar herniation > 10 mm (OR 2.564, 95% CI 1.050-6.258, p = 0.039), although increasing age was also associated with brainstem dysfunction (OR 1.045, 95% CI 1.011-1.080, p = 0.009). A clival canal angle < 140° (p = 0.793) and obex below the foramen magnum (p = 0.563) had no association with brainstem dysfunction. CONCLUSIONS: The authors identified a novel radiographic measure, the FVRA, that can be used to assess fourth ventricular bowing in CM-I and is more predictive of brainstem dysfunction than tonsillar herniation. The FVRA is easy to measure, has excellent interrater variability, and can be a reliable universal radiographic measure. The FVRA will be useful in further describing CM-I radiographically and clinically by identifying patients more likely to be symptomatic as a result of brainstem dysfunction.

16.
World Neurosurg ; 148: 44-53, 2021 04.
Artigo em Inglês | MEDLINE | ID: mdl-33385608

RESUMO

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.


Assuntos
Cistos Aracnóideos/cirurgia , Malformação de Arnold-Chiari/cirurgia , Vértebra Cervical Áxis/cirurgia , Derivações do Líquido Cefalorraquidiano/métodos , Descompressão Cirúrgica/métodos , Hidrocefalia/cirurgia , Adolescente , Cistos Aracnóideos/complicações , Malformação de Arnold-Chiari/complicações , Fossa Craniana Posterior , Humanos , Hidrocefalia/etiologia , Lactente , Masculino , Neuroendoscopia , Cavidade Peritoneal , Ventriculostomia/métodos
17.
J Neurosurg Case Lessons ; 2(23): CASE21118, 2021 Dec 06.
Artigo em Inglês | MEDLINE | ID: mdl-36061084

RESUMO

BACKGROUND: Spontaneous intracranial hypotension (SIH) is a well-documented condition that typically follows a defined clinical course. Previously published studies describing the pathophysiology of SIH have demonstrated extensive evidence of low intracranial pressure (ICP) driving the clinical features of the condition. Through lumbar puncture and use of intracranial monitoring devices, however, both low and normal cerebrospinal fluid (CSF) pressures have been documented. This report outlined and discussed the unique finding of elevated ICP associated with clinical features of SIH. OBSERVATIONS: Here, the authors presented a case of a patient with spontaneous spinal CSF leak who developed tonsillar herniation, cerebral edema, and subsequent episodes of elevated ICP. Although more diverse presentations of SIH are being reported, the authors believed the case to be unique because SIH was accompanied by elevated ICP. LESSONS: This case adds to the growing body of literature surrounding SIH by demonstrating that patients can develop elevated CSF pressures associated with acute encephalopathy.

18.
Acta Neurol Belg ; 121(6): 1547-1554, 2021 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-32524535

RESUMO

INTRODUCTION: The widespread use of imaging has increased Chiari malformation (CM) diagnosis. CM shows clinical heterogeneity that makes management controversial. We aimed to evaluate the occurrence and clinical and radiographic presentation of children with CM-1 and CM-1.5, reporting possible differences according to age and management. METHODS: We retrospectively reviewed 46 children diagnosed with CM-1 or CM-1.5, between 2006 and 2019 at our institute. We evaluated for each subject: reason for hospital admission, clinical presentation, age at diagnosis, extent of cerebellar tonsillar herniation (CTH) and type of treatment when carried out. Affected children were assigned to three age groups. In some patients, a clinical follow-up was carried out. RESULTS: Mean age at diagnosis was 7.61 years. Mean CTH was 8.72 mm. Syringomyelia was found in 10.9%. Twenty-six individuals (56.5%) were symptomatic. The most frequent symptom was headache (34.8%). There were no statistically significant differences between the age groups with regard to the amount of CTH (p = 0.81). Thirteen children (28.3%) underwent surgical treatment. CTH was significantly higher in the surgical group (p < 0.01). Twenty-three patients (50%) performed a 3-year mean follow-up, 17 of whom had no surgery treatment. CTH was stable in 58.8%, reduced in three and increased in three, without any change in symptoms. Only one child showed a worsening in herniation and symptoms, then requiring surgery. CONCLUSION: Frequency and type of symptoms were consistent with those reported in the literature. Conservative approach is a viable option for minimally symptomatic patients, most of whom did not show clinical worsening at follow-up.


Assuntos
Malformação de Arnold-Chiari/diagnóstico por imagem , Malformação de Arnold-Chiari/epidemiologia , Gerenciamento de Dados/métodos , Gerenciamento Clínico , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Itália/epidemiologia , Masculino , Estudos Retrospectivos
19.
Cureus ; 12(9): e10418, 2020 Sep 13.
Artigo em Inglês | MEDLINE | ID: mdl-33062533

RESUMO

Spinal fluid overdrainage with cerebellar hemorrhage is a rare complication of spinal surgery that can have severe consequences if not detected quickly. We present the case of a 72-year-old Caucasian female who underwent thoracolumbar fixation for flatback syndrome. Intraoperatively, the patient suffered a dural injury that was repaired. In the immediate postoperative period, the patient's neurological status rapidly deteriorated within an hour and Jackson-Pratt (JP) drain output measured 300 ml of serosanguinous fluid. A stat CT scan revealed cerebellar hemorrhage, pneumocephalus, and tonsillar herniation. The postoperative drain was immediately removed, and a ventriculostomy tube was placed, confirming low intracranial pressure. Postoperatively, the patient was electively ventilated for three days, continued with remote cerebellar hemorrhage (RCH) treatment and precaution, and extubated on the third day as the patient's neurological function continued to improve. The patient was discharged home nine days after the initial surgery, with a complete recovery. This case indicates that wound drainage in the face of durotomy can induce cerebellar herniation as early as within an hour postoperatively following spine surgery with a dural tear, even after dural repair. This case also suggests that early recognition and appropriate management of RCH is the key to a full recovery. Even in the event of tonsillar herniation and cerebellar hemorrhage, a complete recovery is possible with early recognition and proper management.

20.
Cureus ; 12(8): e9994, 2020 Aug 24.
Artigo em Inglês | MEDLINE | ID: mdl-32983693

RESUMO

Bacterial meningitis is a fatal infectious disease with an annual incidence of four to six cases per 100,000 adults. The most common pathogens associated with this condition are Streptococcus pneumoniae, Neisseria meningitides, and Hemophilus influenzae. Mortality rates range between 10 and 40% despite the availability of highly effective antibiotic therapy, and severe neurological damage affects 30-52% of survivors. The causes of death in patients with pneumococcal meningitis are multifactorial and involve both neurological complications such as cerebral edema, hydrocephalus, infarction, and septic sinus or venous thrombosis and systemic complications such as septic shock, disseminated intravascular coagulation, and acute respiratory distress syndrome. We present an unfortunate case of a 42-year-old woman with asplenia and sickle cell disease, admitted for pneumococcal meningitis, who developed diffuse cerebral edema leading to tonsillar herniation and aneurysmal subarachnoid hemorrhage (SAH) with a fatal outcome. To the best of our knowledge, this is the only case ever reported of meningitis complicated by both SAH and brain herniation.

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