Treatment of Chiari type I malformation in patients with and without syringomyelia: a consecutive series of 66 cases

2001 ◽  
Vol 11 (1) ◽  
pp. 1-9 ◽  
Author(s):  
Juan C. Alzate ◽  
Karl F. Kothbauer ◽  
George I. Jallo ◽  
Fred J. Epstein
Keyword(s):  
Chiari I Malformation ◽  
Neurological Deficits ◽  
Motor Deficits ◽  
Consecutive Series ◽  
Spinal Cord Tissue ◽  
Type I ◽  
Excellent Outcome ◽  
Dural Graft ◽  
Chiari I

Object The authors describe the results of performing a standard posterior craniovertebral decompression and placement, if indicated, of a syringosubarachnoid shunt for the treatment of patients with Chiari I malformation with and without syringomyelia. Methods This is a retrospectively analyzed consecutive series of 66 patients (mean patient age 15 years, range 1–53 years). The uniform posterior craniovertebral decompression consisted of a small suboccipital craniectomy, a C-1 laminectomy, microsurgical reduction of the cerebellar tonsils, and dural closure with a synthetic dural graft to increase the cerebrospinal fluid space at the craniocervical junction. The presence of a large syrinx, with significant thinning of the spinal cord tissue and obliteration of the spinal subarachnoid space, particularly when combined with syrinx-related symptoms, was an indication for the placement of a syringosubarachnoid shunt. In 32 patients Chiari I malformation alone was present, and 34 in patients it was present in combination with syringomyelia. Clinical findings included pain, neurological deficits, and spinal deformity. The presence of syringomyelia was significantly associated with the presence of scoliosis (odds ratio 74.4 [95% confidence interval 8.894–622.4]). All patients underwent a posterior craniovertebral decompression procedure. In 22 of the 34 patients with syringomyelia a syringosubarachnoid shunt was also placed. The mean follow-up period was 24 months (range 3–95 months). Excellent outcome was achieved in 54 patients (82%) and good outcome in 12 (18%). In no patient were symptoms unchanged or worse at follow-up examination, including four patients who initially required a second operation for persistent syringomyelia. Pain was more likely to resolve than sensory and motor deficits after decompressive surgery. Radiological examination revealed normalization of tonsillar position in all patients. The syrinx had disappeared in 15 cases, was decreased in size in 17, and remained unchanged in two. Conclusions Posterior craniovertebral decompression and selective placement of a syringosubarachnoid shunt in patients with Chiari I malformation and syringomyelia is an effective and safe treatment. Primary placement of a shunt in the presence of a sufficiently large syrinx appears to be beneficial. The question of if and when to place a shunt, however, requires further, preferably prospective, investigation.

scholarly journals The surgical procedure of syringomyelie with Chiari I in adults regarding the intrapial aspiration of cerebellar tonsils: does this procedure improve symptoms with less complication?

2019 ◽  
pp. 37-48
Author(s):  
Ait Bachir Mustapha ◽  
T. Benbouzid
Keyword(s):  
Clinical Signs ◽  
Foramen Magnum ◽  
Chiari I Malformation ◽  
Spinal Fluid ◽  
Motor Deficits ◽  
Common Denominator ◽  
Consecutive Series ◽  
Dural Graft ◽  
Chiari I ◽  
Cerebellar Tonsils

Introduction. Cranio-vertebral decompression remains the common denominator for the treatment of syringomyelia associated with Chiari I. On the other hand, the details of the procedure, remains controversial. The success of the surgery is to restore the circulation of cerebro-spinal fluid at the level of the foramen magnum. How is this circulation restored to the level of foramen is the question? We offer our attitude towards the treatment of syringomyelia with Chiari I. Material and method. Consecutive series of 32/121 patients benefiting from cranio-vertebral decompression associated with intrapial aspiration of cerebellar tonsils treated for syringomyelia with a Chiari I malformation in adults. Result. Motor deficits were present in 20 / 22 patients, representing 90% of the entire patient group. these motor deficits are improved in 16 out of 22 cases, and remained unchanged in 06cas. no motor aggravation occurred. in our study, bone decompression and intra pial aspiration of cerebellar tonsils (sub arachnoids manipulation) were found to be associated with favourable results on clinical signs and symptoms. However, sub arachnoids manipulation and intra pial aspiration of cerebellar tonsils showed a little more complication compared with bone decompression with dural plasty. Conclusion. The bone decompression with dural graft and intradural dissection of adhesions and reduction by intra pial aspiration or resection of the tonsils is indicated on the MRI aspect of cerebellar tonsils of considerable size totally obstructing the foramen Magnum, the intraoperative finding, through the arachnoid, of the absence of passage of the cerebro-spinal fluid because of the bulging of the cerebellar tonsils.

scholarly journals Surgical Experience in Pediatric Patients with Chiari-I Malformations Aged ≤18 Years

2019 ◽  
Vol 10 (01) ◽  
pp. 85-88 ◽  
Author(s):  
Ghanshyam Das Singhal ◽  
Shakti Singhal ◽  
Gunjan Agrawal ◽  
Deepti Singhal ◽  
Vipin Arora
Keyword(s):  
Pediatric Patients ◽  
Foramen Magnum ◽  
Chiari I Malformation ◽  
Neurological Deficits ◽  
Resonance Imaging ◽  
Surgical Experience ◽  
Limb Weakness ◽  
Lower Limb Weakness ◽  
Chiari I

ABSTRACT Objective: The objective of this study was to retrospectively study Chiari I malformation patients (<18 years) treated surgically. Materials and Methods: Chiari I malformation patients (<18 years) treated surgically at our institute were retrospectively studied. Results: During the study period between January 1999 and June 2011, fifty patients, aged ≤18 years with Chiari malformation, were treated surgically and formed the basis for this series. There were 21 female children (42%) and 29 male children (58%), with a female-to-male ratio of 1:1. At the last follow-up, oropharyngeal symptoms were improved in 33% (n = 3/9). Headache/neck/back pain improved in 69.56% of children (n = 16/23). Upper-extremity pain/weakness/numbness improved in 73.91% of children (n = 17/23). Ataxia improved in 66.66% of children (n = 4/6). Lower-limb weakness/hyperreflexia improved in 83.33% of children (n = 5/6). At follow-up, magnetic resonance imaging for patients with syrinx was available for 75% of patients (n = 30/50) and not available for 25% of patients (n = 10/40). Syrinx was diminished in size or resolved in 66.33% of patients (n = 19/30) and the remaining was same for 36.66% of patients (n = 11/30). Conclusions: The main goal of surgery is to arrest the progression of neurological deficits. Foramen magnum decompression with a lax duroplasty is the surgical procedure of choice.

Cystic dilation of the ventriculus terminalis

2012 ◽  
Vol 17 (1) ◽  
pp. 86-92 ◽  
Author(s):  
Mario Ganau ◽  
Andrea Talacchi ◽  
Paolo C. Cecchi ◽  
Claudio Ghimenton ◽  
Massimo Gerosa ◽  
...  
Keyword(s):  
Surgical Treatment ◽  
Clinical Status ◽  
Relevant Literature ◽  
University Hospital ◽  
Neurological Deficits ◽  
Herniated Disc ◽  
Treatment Modalities ◽  
Consecutive Series ◽  
Type I

Object The ventriculus terminalis, an embryological remnant consisting of the ependymal-lined space of the conus medullaris, can occasionally become symptomatic after cystic dilation. In the existing literature, consisting of 32 cases, the preferred type of management (conservative vs surgical) is still debated. The object of this study was to report the surgical results in a consecutive series of 10 adult patients with cystic dilation of the ventriculus terminalis (CDVT), to match them with data retrieved from the relevant literature, and specifically to validate a new recent clinical classification. Methods The authors reported 13 new cases of CDVT treated in the Department of Neurosurgery at University Hospital in Verona, Italy. Treatment modalities and clinical and radiological outcomes, both early and at follow-up, were analyzed and compared with a preoperative classification of clinical presentation, as established by de Moura Batista and colleagues (2008). Results Surgical treatment seemed to guarantee the resolution of CDVT. Dorsolumbar laminotomy, myelotomy, and cystic drainage were performed in 10 patients. Patients with Type I symptoms (nonspecific complaints) often presented with comorbidities (herniated disc or facet hypertrophy) confusing their clinical status. The surgical treatment of patients with Type I symptoms promoted good results only if the diagnosis of CDVT was definitive and symptoms had rapidly evolved. In patients with Type II (focal neurological deficits) and III (sphincter disturbances) symptoms, surgical treatment sustained improvement even at the late follow-up. Conclusions While confirming the usefulness of de Moura Batista and colleagues' classification in its impact on prognosis, the authors propose a revision of the classification with subgroups Type Ia (nonspecific symptoms without clear relation to CDVT), which is best treated conservatively, and Type Ib (rapid onset and invalidating unspecific complaints without comorbidities), which may benefit from surgical evacuation.

Management and outcomes of isolated interhemispheric subdural hematomas associated with falx syndrome

2019 ◽  
Vol 131 (6) ◽  
pp. 1920-1925
Author(s):  
Daniel A. Tonetti ◽  
William J. Ares ◽  
David O. Okonkwo ◽  
Paul A. Gardner
Keyword(s):  
Neurological Deterioration ◽  
Neurological Deficits ◽  
Motor Deficits ◽  
Study Cohort ◽  
Venous Infarction ◽  
Nonsurgical Management ◽  
Subdural Hematomas ◽  
Poor Outcomes ◽  
Contemporary Management

OBJECTIVELarge interhemispheric subdural hematomas (iSDHs) causing falx syndrome are rare; therefore, a paucity of data exists regarding the outcomes of contemporary management of iSDH. There is a general consensus among neurosurgeons that large iSDHs with neurological deficits represent a particular treatment challenge with generally poor outcomes. Thus, radiological and clinical outcomes of surgical and nonsurgical management for iSDH bear further study, which is the aim of this report.METHODSA prospectively collected, single-institution trauma database was searched for patients with isolated traumatic iSDH causing falx syndrome in the period from January 2008 to January 2018. Information on demographic and radiological characteristics, serial neurological examinations, clinical and radiological outcomes, and posttreatment complications was collected and tallied. The authors subsequently dichotomized patients by management strategy to evaluate clinical outcome and 30-day survival.RESULTSTwenty-five patients (0.4% of those with intracranial injuries, 0.05% of those with trauma) with iSDH and falx syndrome represented the study cohort. The average age was 73.4 years, and most patients (23 [92%] of 25) were taking anticoagulants or antiplatelet medications. Six patients were managed nonoperatively, and 19 patients underwent craniotomy for iSDH evacuation; of the latter patients, 17 (89.5%) had improvement in or resolution of motor deficits postoperatively. There were no instances of venous infarction, reaccumulation, or infection after evacuation. In total, 9 (36%) of the 25 patients died within 30 days, including 6 (32%) of the 19 who had undergone craniotomy and 3 (50%) of the 6 who had been managed nonoperatively. Patients who died within 30 days were significantly more likely to experience in-hospital neurological deterioration prior to surgery (83% vs 15%, p = 0.0095) and to be comatose prior to surgery (100% vs 23%, p = 0.0031). The median modified Rankin Scale score of surgical patients who survived hospitalization (13 patients) was 1 at a mean follow-up of 22.1 months.CONCLUSIONSiSDHs associated with falx syndrome can be evacuated safely and effectively, and prompt surgical evacuation prior to neurological deterioration can improve outcomes. In this study, craniotomy for iSDH evacuation proved to be a low-risk strategy that was associated with generally good outcomes, though appropriately selected patients may fare well without evacuation.

Chiari Type I Malformation Presenting as Hemifacial Spasm: Case Report

Neurosurgery ◽  
2005 ◽  
Vol 57 (2) ◽  
pp. E371-E371 ◽  
Author(s):  
Mustafa Efkan Colpan ◽  
Zeki Sekerci
Keyword(s):  
Facial Nerve ◽  
Posterior Fossa ◽  
Hemifacial Spasm ◽  
Chiari I Malformation ◽  
Posterior Fossa Decompression ◽  
Type I ◽  
Chiari Type I Malformation ◽  
Downward Displacement ◽  
Facial Spasm ◽  
Chiari I

ABSTRACT OBJECTIVE AND IMPORTANCE: We report on a patient with a Chiari I malformation presenting with right hemifacial spasm. Clinicians should consider the downward displacement of the hindbrain as a rare cause of hemifacial spasm in Chiari I malformation. CLINICAL PRESENTATION: An 18-year-old man was admitted with right hemifacial spasm. The results of the neurological examination were normal except for the facial spasm. Magnetic resonance imaging demonstrated a Chiari I malformation without syringomyelia. After surgery, the hemifacial spasm completely resolved. INTERVENTION: Posterior fossa decompression, C1 laminectomy, and duraplasty were performed. CONCLUSION: The hemifacial spasm could be attributed to compression and/or traction of the facial nerve because of downward displacement of the hindbrain in Chiari I malformation. Compression and/or traction might create irritation of the facial nerve that causes hemifacial spasm. Resolution of the hemifacial spasm after posterior fossa decompression could explain the facial nerve irritation in Chiari I malformation. Clinicians should consider Chiari malformation as a cause of hemifacial spasm and posterior fossa decompression as a potential treatment.

scholarly journals Acquired Chiari I malformation due to lumboperitoneal shunt: A case report and review of literature

2019 ◽  
Vol 10 ◽  
pp. 78
Author(s):  
Aslam Hentati ◽  
Mohamed Badri ◽  
Kamel Bahri ◽  
Ihsen Zammel
Keyword(s):  
Chiari Malformation ◽  
Rare Complication ◽  
Chiari I Malformation ◽  
Clinical Feature ◽  
Type I ◽  
Cerebral Magnetic Resonance Imaging ◽  
Surgical Closure ◽  
Lumboperitoneal Shunt ◽  
Cerebral Mri ◽  
Chiari I

Background: The Type I malformations are supposed to be the result of mesodermal defects that create a congenitally small posterior fossa. However, Chiari malformation could be also “iatrogenic” and then called “acquired” Chiari I malformation. In this study, the authors report the clinical feature of a patient who developed a Chiari I malformation after lumboperitoneal shunt. Case Description: A 35-year-old woman has been suffering from idiopathic intracranial hypertension and rhinorrhea due to an anterior skull base defect. A valveless lumboperitoneal shunt followed by surgical closure of the defect was performed. Six months later, she suffered from major continuous occipital headaches. The neurological examination found a mild cerebellar gait ataxia and cerebellar dysarthria. The cerebral magnetic resonance imaging (MRI) showed a ptosis of the cerebellar tonsils and a disappearance of the cisterna magna; there was no syringomyelia. This herniation was not present before shunt was performed. A replacement of the lumboperitoneal shunt with a pressure-regulated valve chamber was performed. After a 1-year follow-up, the patient reports a marked decrease of the headache as well as the ataxia, and the last cerebral MRI showed resolution of the Chiari I malformation. Conclusions: Symptomatic acquired Chiari malformation with or without syringomyelia as a delayed complication after lumbar shunting is a rare complication, particularly reported in the pediatric population, but could also occur to adult patients. Treating these patients by correcting the shunt’s valve could be enough, but should be monitored, as it may fail to resolve the Chiari malformation even years after treatment.

Chiari Malformation with Syringomyelia

2019 ◽  
pp. 57-64
Author(s):  
Yiping Li ◽  
Bermans J. Iskandar
Keyword(s):  
Foramen Magnum ◽  
Chiari I Malformation ◽  
Neurological Deficits ◽  
Csf Leakage ◽  
Craniocervical Instability ◽  
Chiari I ◽  
Spine Mri ◽  
Operative Monitoring ◽  
Associated Abnormalities ◽  
Cerebellar Tonsils

Chiari I malformation (CIM) is defined by pathological herniation of the cerebellar tonsils below the foramen magnum. Operative intervention for CIM is generally undertaken to treat neurological deficits associated with tonsillar herniation or with associated syringomyelia. A complete brain and spine MRI is indicated to rule out associated abnormalities and to identify and the presence and extent of syringomyelia. The type of surgical decompression remains controversial but may include bone-only decompression, bony decompression followed by duraplasty, and bony decompression followed by duraplasty and tonsillar shrinkage. Post-operative monitoring for CSF leakage, inadequate decompression, subtle chronic craniocervical instability, and hydrocephalus is critical.

*Siringomielia asociada a Malformación de Chiari I. Reporte de caso. [Syringomyelia associated with Chiari I Malformation. Case report.]

2021 ◽  
Vol 40 (03) ◽  
Author(s):  
Flor Wilson Giraldo ◽  
Hector Lezcano ◽  
Leonardo Barrios
Keyword(s):  
Cranial Nerves ◽  
Chiari I Malformation ◽  
Clinical Suspicion ◽  
Unknown Etiology ◽  
Type I ◽  
Imaging Studies ◽  
Structural Alterations ◽  
Truncal Ataxia ◽  
Chiari I ◽  
The Right

<p>Introducción: Las Malformaciones de Chiari (M.C.) son alteraciones estructurales a nivel del cerebelo de etiología en estudio. Muy raras, afectan al 0,5% de la población. Más de la mitad de los pacientes cursan con siringomielia. Caso clínico: Femenina de 47 años con antecedente de M.C. Tipo I, acude con cuadro de dos semanas de evolución de cefalea holocraneana de intensidad 8/10, asociado a mareo e inestabilidad a la marcha. Hallazgos positivos de alteración en los pares craneales III, VIII, IX; hipertonía, signo de Babinski y Hoffman positivos bilaterales; ataxia truncal; nistagmus a la derecha; fuerza muscular disminuida en miembros superiores e hiperreflexia. Discusión: Ante la sospecha clínica, y las múltiples alteraciones a nivel del cerebelo y bulbo, se procede a realizar estudios de imagen y se confirma el diagnóstico de siringomielia.</p><p><strong>Abstract: </strong></p><p>Introduction: Chiari malformations (C.M.) are structural alterations in cerebellum. They are of unknown etiology, at present in study. They are very rare and affect 0,5 % of the population. More than half of the patients have syringomyelia. Clinical case: A 47-year-old female with medical record of C.M. Type I, presents with a two-week evolution holocranial headache, 8/10 in intensity, associated with dizziness and gait instability. Positive findings of alteration in cranial nerves III, VIII, IX; hypertonia, Babinski and Hoffman bilateral sign; truncal ataxia; nystagmus on the right; decreased muscle strength in the upper limbs and hyperreflexia. Discussion: Due to clinical suspicion and multiple alterations in cerebellum and bulb, imaging studies are performed and the diagnosis of syringomyelia is confirmed.</p>

Cystic Fibrosis and Chiari Type I Malformation: Autopsy Study of Two Infants with a Rare Association

2003 ◽  
Vol 6 (1) ◽  
pp. 88-93 ◽  
Author(s):  
Dinesh Rakheja ◽  
Yin Xu ◽  
Dennis K. Burns ◽  
Daniel L. Veltkamp ◽  
Linda R. Margraf
Keyword(s):  
Cystic Fibrosis ◽  
Mutational Analysis ◽  
Chiari I Malformation ◽  
Cftr Gene ◽  
Type I ◽  
Autopsy Study ◽  
Recurrent Pneumonia ◽  
Cftr Mutations ◽  
Chiari I ◽  
American Children

Cystic fibrosis (CF), an epithelial cell transport disorder caused by mutations of the cystic fibrosis transmembrane conductance regulator (CFTR) gene, is not generally associated with malformations of the central nervous system (CNS). This report describes two African-American children who died at less than 2 years of age with known Chiari I malformations and were found, unexpectedly at autopsy, to have the classic pancreatic and respiratory changes of CF. Both patients had suffered from failure to thrive that had been attributed to their CNS malformations. One child also had recurrent pneumonia and died with Pseudomonas sepsis. Mutational analysis for > 70 common CFTR mutations identified a single delta F508 mutation in one patient and a single 3120 + 1G to A mutation in the other. Their second CFTR mutations were not identified. The association of CF with Chiari I malformation is not likely to be purely coincidental, as the probability of such an occurrence in African-Americans is greater than one in 7,500,000 patients. It is possible that the CFTR gene may play a previously unrecognized role in CNS development. Alternatively, this CNS abnormality may be acquired due to the metabolic and electrolyte imbalances that characteristically occur in CF.

Intramedullary ependymoma of the spinal cord

1990 ◽  
Vol 72 (4) ◽  
pp. 523-532 ◽  
Author(s):  
Paul C. McCormick ◽  
Roland Torres ◽  
Kalmon D. Post ◽  
Bennett M. Stein
Keyword(s):  
Spinal Cord ◽  
Mr Imaging ◽  
Tumor Recurrence ◽  
Neurological Deficits ◽  
Functional Improvement ◽  
Consecutive Series ◽  
Intramedullary Spinal Cord ◽  
Content Type ◽  
Fine Print

✓ A consecutive series of 23 patients underwent operative removal of an intramedullary spinal cord ependymoma between January, 1976, and September, 1988. Thirteen women and 10 men between the age of 19 and 70 years experienced symptoms for a mean of 34 months preceding initial diagnosis. Eight patients had undergone treatment prior to tumor recurrence and referral. Mild neurological deficits were present in 22 patients on initial examination. The location of the tumors was predominantly cervical or cervicothoracic. Radiological evaluation revealed a wide spinal cord in all cases. Magnetic resonance (MR) imaging was the single most important radiological procedure. At operation, a complete removal was achieved in all patients. No patient received postoperative radiation therapy. Histological examination revealed a benign ependymoma in all cases. The follow-up period ranged from 6 to 159 months (mean 62 months) with seven patients followed for a minimum of 10 years after surgery. Fourteen patients underwent postoperative MR imaging at intervals ranging from 8 months to 10 years postoperatively. No patient has been lost to follow-up review and there were no deaths. No patient showed definite clinical or radiological evidence of tumor recurrence during the follow-up period. Recent neurological evaluation revealed functional improvement from initial preoperative clinical status in eight patients, no significant change in 12 patients, and deterioration in three patients. The data support the belief that long-term disease-free control of intramedullary spinal ependymomas with acceptable morbidity may be achieved utilizing microsurgical removal alone.

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