Posttraumatic syringomyelia associated with heavy weightlifting exercises: case report.

PubMed

Balmaseda, M T; Wunder, J A; Gordon, C; Cannell, C D

1988-11-01

Posttraumatic syringomyelia is a well-recognized late sequel to spinal trauma occurring in 1% to 3.2% of spinal cord injured patients. Its clinical presentation is usually marked by pain, ascending sensory loss, increased muscle weakness, and depressed deep tendon reflexes. The case of a 25-year-old man with C8 complete quadriplegia, who developed a syrinx five years after his initial injury, is presented. This patient kept a log of his daily physical workout which consisted of lifting weights of 50 to 60 pounds with his neck extensors and biceps. The diagnosis was made clinically and confirmed by magnetic resonance imaging. Repeated valsalva maneuvers from daily heavy weightlifting exercises most likely predisposed this patient to the development and extension of his syringomyelia. Dramatic improvement followed surgical placement of a subarachnoid shunt.

Treatment of selected syringomyelias with syringo-pleural shunt: the experience with a consecutive 26 cases.

PubMed

Fan, Tao; Zhao, XinGang; Zhao, HaiJun; Liang, Cong; Wang, YinQian; Gai, QiFei; Zhang, Fangyi

2015-10-01

It is well established that syringomyelia can cause neurological symptoms and deficit by accumulation of fluid within syrinx cavities that lead to internal compression within the spinal cord. When other intervention treating the underlying etiology failed to yield any improvement, the next option would be a procedure to divert the fluid from the syrinx cavity, such as syringo-subarachnoid, syringo-peritoneal or syringo-pleural shunting. The indications and long term efficacy of these direct shunting procedures are still questionable and controversial. To investigate the clinical indication, outcome and complication of syringe-pleural shunt (SPS) as an alternative for treatment of syringomyelia. We reported a retrospective 26 cases of syringomyelia were found to have indication for a diversion procedure. SPS was offered. Patients' symptoms, mJOA score, and MRI were collected to evaluate the change of the syringomyelia and prognosis of the patients. 2-tailed wilcoxon signed-rank test was used to perform the statistical analysis of the mJOA scores. All 26 patients underwent SPS. The clinical information was collected, the mean follow-up time was 27.4 months, 2-tailed wilcoxon signed-rank test was used to perform the statistical analysis of the mJOA scores. The key surgical technique, outcome and complications of SPS were reported in detail. No mortality and severe complications occurred. Postoperative MRIs revealed near-complete resolution of syrinx in 14 patients, significant shrinkage of syrinx in 10 patients, no obvious reduction or unchanged in remaining 2 patient. Postoperatively, the symptoms improved in 24 cases (92.3%). Statistical analysis of the mJOA scores showed a statistical significance (P<0.001) between the preoperative group and the 2-week postoperative group. No further significant improvement between 2 weeks to the final follow up at 27 months. Collapse or remarkable shrinkage of the syrinx by SPS could ameliorate or at least stabilize the symptoms for the patient. We recommend small laminectomy and a less than 3mm myelotomy either at PML or DREZ. The SPS procedure can be an effective and relatively long-lived treatment for the idiopathic syringomyelia and those that failed other options. Copyright © 2015 Elsevier B.V. All rights reserved.

Cervical spinal canal narrowing in idiopathic syringomyelia.

PubMed

Struck, Aaron F; Carr, Carrie M; Shah, Vinil; Hesselink, John R; Haughton, Victor M

2016-08-01

The cervical spine in Chiari I patient with syringomyelia has significantly different anteroposterior diameters than it does in Chiari I patients without syringomyelia. We tested the hypothesis that patients with idiopathic syringomyelia (IS) also have abnormal cervical spinal canal diameters. The finding in both groups may relate to the pathogenesis of syringomyelia. Local institutional review boards approved this retrospective study. Patients with IS were compared to age-matched controls with normal sagittal spine MR. All subjects had T1-weighted spin-echo (500/20) and T2-weighted fast spin-echo (2000/90) sagittal cervical spine images at 1.5 T. Readers blinded to demographic data and study hypothesis measured anteroposterior diameters at each cervical level. The spinal canal diameters were compared with a Mann-Whitney U test. The overall difference was assessed with a Friedman test. Seventeen subjects were read by two reviewers to assess inter-rater reliability. Fifty IS patients with 50 age-matched controls were studied. IS subjects had one or more syrinxes varying from 1 to 19 spinal segments. Spinal canal diameters narrowed from C1 to C3 and then enlarged from C5 to C7 in both groups. Diameters from C2 to C4 were narrower in the IS group (p < 0.005) than in controls. The ratio of the C3 to the C7 diameters was also smaller (p = 0.004) in IS than controls. Collectively, the spinal canal diameters in the IS were significantly different from controls (Friedman test p < 0.0001). Patients with IS have abnormally narrow upper and mid cervical spinal canal diameters and greater positive tapering between C3 and C7.

Post-traumatic syringomyelia refractory to surgical intervention: a series of cases on recurrent syringomyelia.

PubMed

Leahy, H P; Beckley, A A; Formal, C S; Fried, G W

2015-01-01

The objective of this study was to analyze a series of traumatic spinal cord injury (SCI) patients with a diagnosis of syrinx who had recurrence of symptomatic syrinx following surgical intervention. This is a patient series. The study was conducted in an acute inpatient rehabilitation facility. Participants included patients ( N =6) with post-traumatic syringomyelia (PTS) who had recurrent syrinx despite surgical repair. Not applicable. The main outcome measures were time period between injury and clinical manifestations of syringomyelia, time to recurrence and presenting symptoms. Among the six patients, there is great variability between time of the initial SCI and the development of syrinx. In terms of time periods between diagnosis of syrinx and recurrence of syrinx, there is also some variability (ranging from 6 to 936 weeks). The median length of time to recurrence was 104 weeks. In all cases, the presenting symptom was ultimately weakness, and in most cases it was associated with rising sensory deficits. Notably, all patients were male, aged 31-55 years, had suffered diffuse traumatic SCI and four of six patients lost the ability to ambulate because of syrinx formation. This series suggests that there may be risk factors for developing post-traumatic syringomyelia. We question whether there is a relationship between American Spinal Injury Association Impairment Scale grade and recurrence of PTS. We need to look closely at these patients to see whether there are modifiable risk factors that may minimize their chance of developing PTS. Once these are identified, there may be a role in routine screening of all patients and particularly those who may be at an increased risk for PTS to avoid loss of ambulatory function.

Syringomyelia and Craniocervical Junction Abnormalities in Chihuahuas.

PubMed

Kiviranta, A-M; Rusbridge, C; Laitinen-Vapaavuori, O; Hielm-Björkman, A; Lappalainen, A K; Knowler, S P; Jokinen, T S

2017-11-01

Chiari-like malformation (CM) and syringomyelia (SM) are widely reported in Cavalier King Charles Spaniels and Griffon Bruxellois dogs. Increasing evidence indicates that CM and SM also occur in other small and toy breed dogs, such as Chihuahuas. To describe the presence of SM and craniocervical junction (CCJ) abnormalities in Chihuahuas and to evaluate the possible association of CCJ abnormalities with SM. To describe CM/SM-related clinical signs and neurologic deficits and to investigate the association of CM/SM-related clinical signs with signalment, SM, or CCJ abnormalities. Fifty-three client-owned Chihuahuas. Prospective study. Questionnaire analyses and physical and neurologic examinations were obtained before magnetic resonance and computed tomography imaging. Images were evaluated for the presence of SM, CM, and atlantooccipital overlapping. Additionally, medullary kinking, dorsal spinal cord compression, and their sum indices were calculated. Scratching was the most common CM/SM-related clinical sign and decreased postural reaction the most common neurologic deficit in 73 and 87% of dogs, respectively. Chiari-like malformation and SM were present in 100 and 38% of dogs, respectively. Syringomyelia was associated with the presence of CM/SM-related clinical signs (P = 0.034), and medullary kinking and sum indices were higher in dogs with clinical signs (P = 0.016 and P = 0.007, respectively). Syringomyelia and CCJ abnormalities are prevalent in Chihuahuas. Syringomyelia was an important factor for the presence of CM/SM-related clinical signs, but many dogs suffered from similar clinical signs without being affected by SM, highlighting the clinical importance of CCJ abnormalities in Chihuahuas. Copyright © 2017 The Authors. Journal of Veterinary Internal Medicine published by Wiley Periodicals, Inc. on behalf of the American College of Veterinary Internal Medicine.

Comparison of spinal deformity in children with Chiari I malformation with and without syringomyelia: matched cohort study.

PubMed

Godzik, J; Dardas, A; Kelly, M P; Holekamp, T F; Lenke, L G; Smyth, M D; Park, T S; Leonard, J R; Limbrick, D D

2016-02-01

To describe curve patterns in patients with Chiari malformation I (CIM) without syringomyelia, and compare to patients with Chiari malformation with syringomyelia (CIM + SM). Review of medical records from 2000 to 2013 at a single institution was performed to identify CIM patients with scoliosis. Patients with CIM were matched (1:1) by age and gender to CIM + SM. Radiographic curve patterns, MRI-based craniovertebral junction parameters, and associated neurological signs were compared between the two cohorts. Eighteen patients with CIM-associated scoliosis in the absence of syringomyelia were identified; 14 (78 %) were female, with mean age of 11.5 ± 4.5 years. Mean tonsillar descent was 9.9 ± 4.1 mm in the CIM group and 9.1 ± 3.0 mm in the CIM + SM group (p = 0.57). Average syrinx diameter in the CIM + SM group was 9.0 ± 2.7 mm. CIM patients demonstrated less severe scoliotic curves (32.1° vs. 46.1°, p = 0.04), despite comparable thoracic kyphosis (43.7° vs. 49.6°, p = 0.85). Two (11 %) patients with CIM demonstrated thoracic apex left deformities compared to 9/18 (50 %) in the CIM + SM cohort (p = 0.01). Neurological abnormalities were only observed in the group with syringomyelia (6/18, or 33 %; p = 0.007). In the largest series specifically evaluating CIM and scoliosis, we found that these patients appear to present with fewer atypical curve features, with less severe scoliotic curves, fewer apex left curves, and fewer related neurological abnormalities than CIM + SM. Notably, equivalent thoracic kyphosis was observed in both groups. Future studies are needed to better understand pathogenesis of spinal deformity in CIM with and without SM.

Quantitative Analysis of Chiari-Like Malformation and Syringomyelia in the Griffon Bruxellois Dog

PubMed Central

Knowler, Susan P.; McFadyen, Angus K.; Freeman, Courtenay; Kent, Marc; Platt, Simon R.; Kibar, Zoha; Rusbridge, Clare

2014-01-01

This study aimed to develop a system of quantitative analysis of canine Chiari-like malformation and syringomyelia on variable quality MRI. We made a series of measurements from magnetic resonance DICOM images from Griffon Bruxellois dogs with and without Chiari-like malformation and syringomyelia and identified several significant variables. We found that in the Griffon Bruxellois dog, Chiari-like malformation is characterized by an apparent shortening of the entire cranial base and possibly by increased proximity of the atlas to the occiput. As a compensatory change, there appears to be an increased height of the rostral cranial cavity with lengthening of the dorsal cranial vault and considerable reorganization of the brain parenchyma including ventral deviation of the olfactory bulbs and rostral invagination of the cerebellum under the occipital lobes. PMID:24533070

Dandy-Walker complex and syringomyelia in an adult: case report and discussion.

PubMed

Hammond, Christopher J; Chitnavis, Bhupal; Penny, Christopher C; Strong, Anthony J

2002-01-01

Syringomyelia in association with the Dandy-Walker complex is rare and only 17 cases have been reported, mostly as autopsy findings or incidental reports in series for other conditions. We present a further case report and discuss the possible pathogenetic mechanisms of syrinx formation in the presence of the Dandy-Walker complex. We emphasize the role of foramen magnum obstruction and alteration in cerebrospinal fluid flow dynamics in syrinx formation. The therapeutic importance of disimpaction of the foramen magnum is stressed. A 39-year-old man presented with headache. As a child, he had had cystoperitoneal and ventriculoperitoneal shunts inserted for hydrocephalus in association with the Dandy-Walker complex. On examination, the patient had limited upgaze but was otherwise normal. Magnetic resonance imaging revealed a large cervicothoracic syrinx and a Dandy-Walker cyst obstructing the foramen magnum. The cystoperitoneal shunt was revised. The patient's headaches resolved, and follow-up imaging demonstrated resolution of the syrinx and disimpaction of the foramen magnum. Disimpaction of the foramen magnum can resolve syringomyelia in patients with the Dandy-Walker complex. This can be achieved by a shunt procedure or by formal foramen magnum decompression. The rarity of the association between the Dandy-Walker complex and syringomyelia prevents firm conclusions from being made regarding the best management strategy for this condition.

Symptomatic arrhythmias due to syringomyelia-induced severe autonomic dysfunction.

PubMed

Riedlbauchová, Lucie; Nedělka, Tomáš; Schlenker, Jakub

2014-10-01

Syringomyelia is characterized by cavity formation in the spinal cord, most often at C2-Th9 level. Clinical manifestation reflects extent and localization of the spinal cord injury. 20-year old woman was admitted for recurrent rest-related presyncopes with sudden manifestation. Paroxysms of sinus bradycardia with SA and AV blocks were repeatedly documented during symptoms. There was normal echocardiographic finding, (para) infectious etiology was not proved. Character of the ECG findings raised suspicion on neurogenic cause. Autonomic nervous system testing demonstrated abnormalities reflecting predominant sympathetic dysfunction. Suspicion on incipient myelopathy was subsequently confirmed by MRI, which discovered syringomyelia at Th5 level as the only pathology. A 52-year old man with hypotrophic quadruparesis resulting from perinatal brain injury was sent for 2-years lasting symptoms (sudden palpitation, sweating, muscle tightness, shaking) with progressive worsening. Symptoms occurred in association with sudden increase of sinus rhythm rate and blood pressure that were provoked by minimal physical activity. Presence of significant autonomic dysregulation with baroreflex hyperreactivity in orthostatic test and symptomatic postural orthostatic tachycardia with verticalization-associated hypertension were proved. MRI revealed syringomyelia at C7 and Th7 level affecting sympathetic centers at these levels. Sympathetic fibers dysfunction at C-Th spinal level may cause significant autonomic dysfunction with arrhythmic manifestation.

Chiari malformation, syringomyelia and bulbar palsy in X linked hypophosphataemia

PubMed Central

Watts, Laura; Wordsworth, Paul

2015-01-01

X linked hypophosphataemia (XLH) is a rare condition with numerous musculoskeletal complications. It may mimic other more familiar conditions, such as vitamin D deficiency, ankylosing spondylitis or diffuse idiopathic skeletal hyperostosis. We describe two cases with Chiari type 1 malformations and syringomyelia, neither of which is well recognised in XLH. The first presented late with the additional complications of spinal cord compression, pseudofracture, renal stones and gross femoroacetabular impingement requiring hip replacement. The second also had bulbar palsy; the first case to be described in this condition, to the best of our knowledge. We wish to raise awareness of the important neurological complications of syringomyelia, Chiari malformation, spinal cord compression and bulbar palsy when treating these patients. We also wish to draw attention to the utility of family history and genetic testing when making the diagnosis of this rare but potentially treatable condition. PMID:26561226

Chiari malformation, syringomyelia and bulbar palsy in X linked hypophosphataemia.

PubMed

Watts, Laura; Wordsworth, Paul

2015-11-11

X linked hypophosphataemia (XLH) is a rare condition with numerous musculoskeletal complications. It may mimic other more familiar conditions, such as vitamin D deficiency, ankylosing spondylitis or diffuse idiopathic skeletal hyperostosis. We describe two cases with Chiari type 1 malformations and syringomyelia, neither of which is well recognised in XLH. The first presented late with the additional complications of spinal cord compression, pseudofracture, renal stones and gross femoroacetabular impingement requiring hip replacement. The second also had bulbar palsy; the first case to be described in this condition, to the best of our knowledge. We wish to raise awareness of the important neurological complications of syringomyelia, Chiari malformation, spinal cord compression and bulbar palsy when treating these patients. We also wish to draw attention to the utility of family history and genetic testing when making the diagnosis of this rare but potentially treatable condition. 2015 BMJ Publishing Group Ltd.

Syringomyelia and Arnold-Chiari malformation associated with neck pain and left arm radiculopathy treated with spinal manipulation

PubMed Central

Tieppo Francio, Vinicius

2014-01-01

An 18-year-old female patient presented with left dominant neck pain after a motor vehicle collision. Her cervical spine MRI revealed syringomyelia with associated Type I Arnold-Chiari malformation. Some researchers have reported that these might be considered contraindications to spinal manipulation. Nevertheless, her benign and functional clinical examination suggested otherwise and she underwent four manipulative treatments in 2 weeks. By the end of the treatment plan and after 1-month follow-up, she was asymptomatic, no adverse effects were noted and her outcome assessment score decreased from 56% to 0%. This case illustrates that spinal manipulation may be a useful adjunctive treatment procedure for spinal pain, even in the presence of syringomyelia and Chiari malformation, which may not necessarily be a contraindication to spinal manipulation, when performed by a skilled and well-trained physician. PMID:25385566

Syringomyelia with intramedullary ectopic choroid plexus: Case report.

PubMed

Duan, Hongzhou; Zhang, Jiayong; Xu, Feifan; Zhang, Zongqiang; Zhao, Xiaowen

2018-06-01

Intramedullary ectopic choroid plexus is rarely reported, here, we reported a rare case of symptomatic syringomyelia resulted of intramedullary ectopic choroid plexus. The patient was a 30-year-old female who presented with a 2-month history of progressive pain of upper back and bilateral ankle joint and progressive loss of upper-extremity function. MRI examination showed an intramedullary cystic lesion at T2-T4 without enhancement. Operative exploration was indicated. A reddish vascular villus-like lesion was found being located in the left dorsal part of the cyst, which was enblock removed and was confirmed as an ectopic choroid plexus tissue by pathological examination. The patient recovered uneventful and the symptom resolved during follow-up. Although ectopic choroid plexus is extremely rare, it should be taken into acount in the differential diagnosis of pathogenesis in syringomyelia or intramedullary cyst, aggressive surgical exploration should be considered when necessary. Copyright © 2018 Elsevier B.V. All rights reserved.

Pan, Syrinx and syringomyelia.

PubMed

Palacios-Sánchez, Leonardo; Botero-Meneses, Juan Sebastián; Vélez-Flórez, María Camila

2017-12-01

Many myths and legends have had a deep influence on modern language, and on modern medical vernacular. The terms "syrinx" and "panic"are two of the most characteristic examples and their use in neurology and other specialties is well known. This article reviews the history of these words in Greek mythology and their use in modern medicine. It is known by very few that clinical symptoms or conditions, such as syringomyelia and panic attacks, have a mythological origin in their definition and naming.

The quality of life and psychiatric morbidity in patients operated for Arnold-Chiari malformation type I.

PubMed

Bakim, Bahadir; Goksan Yavuz, Burcu; Yilmaz, Adem; Karamustafalioglu, Oguz; Akbiyik, Meral; Yayla, Sinan; Yuce, Ismail; Alpak, Gokay; Tankaya, Onur

2013-10-01

There are some case reports that highlight the association of Arnold-Chiari malformation (ACM) with psychiatric symptoms. We assessed the association between ACM and psychiatric symptoms and risk factors in terms of psychiatric morbidity and evaluated the quality of life after surgery. This study consisted of sixteen patients who underwent decompression operation at the Department of Neurosurgery of Sisli Etfal Hospital. The MINI plus, Short-Form McGill Pain Questionnaire and WHOQOL-BREF-TR were administered to patients. About 43.8% of the patients had a psychiatric disorder. About 50% of the patients had co-existing syringomyelia of which 50% with syringomyelia had a psychiatric disorder. Patients with syringomyelia without any psychiatric disorder had significantly lower scores on physical domain of WHOQOL-BREF-TR (p = 0.02) than the patients without syringomyelia and psychiatric disorder. Subjects with a psychiatric disorder had lower scores on four domains of WHOQOL-BREF-TR. The patients with psychiatric diagnoses had significantly higher scores on affective pain index (p = 0.021) and total pain index (p = 0.037) than the patients without any psychiatric disorder. The presence of a psychiatric condition influences not only the physical aspect but also deteriorates the psychological and social relations and environmental aspect. Moreover the presence of a psychiatric disorder increases the perception of pain and causes more discomfort.

A critical appraisal of drainage in syringomyelia.

PubMed

Sgouros, S; Williams, B

1995-01-01

The use of drains in the treatment of syringomyelia has a simple and immediate appeal and has been practiced widely since the report of Abbe and Coley over 100 years ago. Good short-term results have been claimed in the past, but long-term outcome is largely unknown. An experience in Birmingham, England is reviewed in which 73 patients who had had some form of syrinx drainage procedure performed were subsequently followed up. In these cases, a total of 56 syringopleural and 14 syringosubarachnoid shunts had been inserted. Ten years after the operations, only 53.5% and 50% of the patients, respectively, continued to remain clinically stable. A 15.7% complication rate was recorded, including fatal hemorrhage, infection, and displacement of the drain from the pleural and syrinx cavities. At second operation or necropsy, at least 5% of shunts were discovered to be blocked. The effect of other drainage procedures that do not use artificial tubing, such as syringotomy and terminal ventriculostomy, was analyzed but found not to offer any substantial benefit. These results indicate that drainage procedures are not an effective solution to remedying the progressive, destructive nature of syringomyelia. It is suggested that, rather than attempting to drain the syrinx cavity, disabling the filling mechanism of the syrinx is more appropriate. Most forms of syringomyelia have a blockage at the level of the foramen magnum or in the subarachnoid space of the spine. Surgical measures that aim to reconstruct the continuity of the subarachnoid space at the site of the block are strongly recommended. Lowering the overall pressure of the cerebrospinal fluid is advocated when reestablishment of the pathways proves impossible. Syrinx drainage as an adjuvant to more physiological surgery may have a place in the treatment of syringomyelia. If two procedures are done at the same time, however, it is difficult to ascribe with certainty a success or failure, and it is suggested that the drainage procedure be reserved for a later attempt if the elective first operation fails.

Association between the findings on magnetic resonance imaging screening for syringomyelia in asymptomatic Cavalier King Charles spaniels and observation of clinical signs consistent with syringomyelia in later life.

PubMed

Ives, E J; Doyle, L; Holmes, M; Williams, T L; Vanhaesebrouck, A E

2015-01-01

A questionnaire-based study was used to investigate the association between the findings on magnetic resonance imaging (MRI) screening for syringomyelia (SM) in 79 asymptomatic Cavalier King Charles spaniels (CKCS) and the subsequent development of clinical signs consistent with SM in later life. Owners reported clinical signs consistent with SM in 13/79 (16%) dogs at the time of the questionnaire. A significantly greater proportion of CKCS with a syrinx visible on MRI screening showed clinical signs in later life (9/25, 36%) than dogs without a visible syrinx (4/54, 7%; odds ratio 6.9). Whether the findings of MRI screening can be used to indicate the likelihood of an asymptomatic CKCS developing clinical signs consistent with SM in later life warrants further prospective study in a larger cohort of dogs. Copyright © 2014 Elsevier Ltd. All rights reserved.

Sustained high-pressure in the spinal subarachnoid space while arterial expansion is low may be linked to syrinx development.

PubMed

Clarke, Elizabeth C; Fletcher, David F; Bilston, Lynne E

2017-04-01

Syringomyelia (a spinal cord cyst) usually develops as a result of conditions that cause cerebrospinal fluid (CSF) obstruction. The mechanism of syrinx formation and enlargement remains unclear, though previous studies suggest that the fluid enters via the perivascular spaces (PVS) of the penetrating arteries of the spinal cord, and that alterations in the CSF pulse timing and pressure could contribute to enhanced PVS inflow. This study uses an idealised computational model of the PVS to investigate the factors that influence peri-arterial fluid flow. First, we used three sample patient-specific models to explore whether changes in subarachnoid space (SAS) pressures in individuals with and without syringomyelia could influence PVS inflow. Second we conducted a parametric study to determine how features of the CSF pulse altered perivascular fluid, including alterations to timing and magnitude of the peak SAS pressure, the timing of reversal from high to low pressure (diastolic phase), and the area under the pressure-time curve. The model for the patient with syringomyelia had higher net CSF inflow to the PVS than the two subjects without syringomyelia. In the parametric study, only increasing the area under the high pressure region of the SAS pulse substantially increased PVS inflow, when coupled with a temporal shift in arterial and SAS pulses. This suggests that a period of sustained high SAS pressure while arterial diameter is low may increase net CSF pumping into the PVS.

Fourth Ventriculostomy in Occlusion of the Foramen of Magendie Associated with Chiari Malformation and Syringomyelia

PubMed Central

Orakdogen, Metin; Emon, Selin Tural; Erdogan, Baris; Somay, Hakan

2015-01-01

We present four cases of hydrocephalus caused by occlusion of foramen of Magendie associated with Chiari Type I malformation and syringomyelia. The aim of this study is to evaluate the results of surgical treatment via fourth ventriculostomy with catheter from the fourth ventricle to the upper cervical subarachnoid space. Obstructive tetraventricular hydrocephalus due to occlusion of the foramina of Luschka and Magendie can be treated with cerebrospinal fluid shunting, opening the membranes with suboccipital craniotomy, placement of a catheter, endoscopic third ventriculostomy, and endoscopic fourth ventriculostomy. Our aim was to solve all the pathologies such as Chiari malformation, hydrocephalus, and syringomyelia in one approach. Thus, the treatment consisted of posterior fossa decompression and exploration. All the patients were treated with suboccipital craniectomy and C1 laminectomy with excision of the membrane obstructing the foramen of Magendie. Fourth ventriculostomy with cathetering from fourth ventricle to upper cervical subarachnoid space was performed. The postoperative period was uneventful in all the patients. Neurological status of all the patients improved. Tetraventricular hydrocephalus and syrinx were reduced in the control cranial magnetic resonance imaging. Complications such as infection and catheter migration were not observed during the follow-up period. Treatment with fourth ventriculostomy using a catheter from fourth ventricle to upper cervical subarachnoid space could be a treatment of choice in cases with hydrocephalus caused by occlusion of the foramina of Magendie, with associated Chiari Type I malformation and syringomyelia. PMID:28663969

Prenatal diagnosis of Chiari malformation with syringomyelia in the second trimester.

PubMed

Iruretagoyena, Jesus Igor; Trampe, Barbara; Shah, Dinesh

2010-02-01

Routine anatomic ultrasound performed in the second trimester has a detection rate of approximately 70-90% for fetal congenital abnormalities (Nyberg and Souter, J Ultrasound Med 2001;6:655-674). The central nervous system abnormalities are one of the most common ones detected. Chiari malformation is among the CNS abnormalities diagnosed in the fetal period (Bianchi et al., Fetology - diagnosis and management of the fetal patient, McGraw-Hill, 2000). The Arnold-Chiari malformation was first described in 1883 by Cleland (Romero et al., Prenatal diagnosis of congenital anomalies, Appleton and Lange, 1988). It is characterised by the prolapse of the hindbrain structures below the level of the foramen magnum. It can be associated with skeletal abnormalities and neurological dysfunction. In type I, a lip of cerebellum is downwardly displaced with the tonsils, but the fourth ventricle remains in the posterior fossa. This condition may coexist with syringomyelia, which is a cyst formation on the cervical portion of the spinal cord (Creasy et al., Maternal fetal medicine principles and practice, 2004). We present a case where Chiari type 1 and syringomyelia detected at 18 weeks of gestation. The reason for referral to our center was an abnormal inward posturing of both upper and lower extremities (minimal gross movement and almost inexistent range of motion on fetal joints). On further fetal evaluation, an abnormal brain ultrasound was identified. Prenatal diagnosis of Chiari type 1 malformation and syringomyelia is almost nonexistent when reviewing the literature is the reason why this case is presented.

[Oropharyngeal dysphagia associated with Chiari I malformation and syringomyelia J].

PubMed

Cortés-Medina, Julio César; Cárdenas-Lara, Armando; Guerrero-Rascón, Carlos Alberto; Rodríguez-Bautista, Heber

2014-01-01

Dysphagia associated with neurological disease is an important clinical manifestation in the diagnosis of injury that justifies the compression of the brainstem and lower cranial nerves. To emphasize the study of dysphagia in a patient with Chiari I malformation associated with syringomyelia in the absence of primary gastroenterological symptoms. We describe the case of a 62 year-old woman with oropharyngeal dysphagia of six years of evolution, cervicobrachialgia, ptosis and facial diplexia. Magnetic resonance imaging is an essential element for establishing the etiologic diagnosis of neurogenic dysphagia.

Three cases of communication syringomyelia secondary to midbrain gliomas.

PubMed Central

Williams, B; Timperley, W R

1977-01-01

Three cases of midbrain gliomas are descrbied clinically and pathologically. In each case high pressure symptoms were followed by visual disturbance and the onset of syringomyelia symptoms before death. All the patients had hydrocephalus. In one case with concomitant syringobulbia, the syrinx appeared to due to CSF communicating with the cord cavity through the tissues of the brain stem. In the other cases the communication between the CSF pathways and the syrinx was at the usual site, through the central canal at the obex. Images PMID:845611

Definitions and Anatomic Considerations in Chiari I Malformation and Associated Syringomyelia.

PubMed

Tubbs, R Shane

2015-10-01

Current understanding of the hindbrain hernias known as Chiari I malformations is based on more than 100 years of pathologic and clinical experience. Over time, the definition of this finding has been analyzed and altered. The term Chiari I malformation is currently used to describe tonsillar ectopia in a wide range of patients with varying embryonic derailments. This article discusses this malformation, its various definitions, and varied anatomic traits. In addition, the morphology of the commonly associated syringomyelia is reviewed. Copyright © 2015 Elsevier Inc. All rights reserved.

Changes in CSF flow after one-stage posterior vertebral column resection in scoliosis patients with syringomyelia and Chiari malformation type I.

PubMed

Wang, Yingsong; Xie, Jingming; Zhao, Zhi; Zhang, Ying; Li, Tao; Si, Yongyu

2013-05-01

Phase contrast-cine MRI (PC-cine MRI) studies in patients with syringomyelia and Chiari malformation Type I (CM-I) have demonstrated abnormal CSF flow across the foramen magnum, which can revert to normal after craniocervical decompression with syrinx shrinkage. In order to investigate the mechanisms leading to postoperative syringomyelia shrinkage, the authors studied the hydrodynamic changes of CSF flow in the craniocervical junction and spinal canal in patients with scoliosis associated with syringomyelia after one-stage deformity correction by posterior vertebral column resection. Preoperative and postoperative CSF flow dynamics at the levels of the foramen magnum, C-7, T-7 (or apex), and L-1 were assessed by electrocardiogram-synchronized cardiac-gated PC-cine MRI in 8 adolescent patients suffering from severe scoliosis with syringomyelia and CM-I (scoliosis group) and undergoing posterior vertebral column resection. An additional 8 patients with syringomyelia and CM-I without spinal deformity (syrinx group) and 8 healthy volunteers (control group) were also enrolled. Mean values were obtained for the following parameters: the duration of a CSF cycle, the duration of caudad CSF flow (CSF downflow [DF]) and cephalad CSF flow (CSF upflow [UF]), the ratio of DF duration to CSF cycle duration (DF%), and the ratio of UF duration to CSF cycle duration (UF%). The ratio of the stationary phase (SP) duration to CSF cycle duration was calculated (SP%). The maximum downflow velocities (VD max) and maximum upflow velocities (VU max) were measured. SPSS (version 14.0) was used for all statistical analysis. Patients in the scoliosis group underwent one-stage posterior vertebral column resection for deformity correction without suboccipital decompression. The mean preoperative coronal Cobb angle was 102.4° (range 76°-138°). The mean postoperative Cobb angle was 41.7° (range 12°-75°), with an average correction rate of 59.3%. During the follow-up, 1 patient with hypermyotonia experienced a significant decrease of muscle tension and 1 patient with reduced anal sphincter tone manifested recovery. A total of 5 patients demonstrated a significant decrease (> 30%) in syrinx size. With respect to changes in CSF flow dynamics, the syrinx group was characterized by slower and shorter downflow than the control group, and the difference was more significant at the foramen magnum and C-7 levels. In patients with scoliosis, CSF downflow at the foramen magnum level was significantly restricted, and a prolonged stationary phase indicated increased obstruction of CSF flow. After posterior vertebral column resection, the peak velocity of CSF flow at the foramen magnum increased, and the downflow phase duration was markedly prolonged. The parameters showed a return to almost normal CSF dynamics at the craniocervical region, and this improvement was maintained for 6-12 months of follow-up. There were distinct abnormalities of CSF flow at the craniocervical junction in patients with syringomyelia. Abnormal dynamics of downflow could be aggravated by associated severe spinal deformity and improved by correction via posterior vertebral column resection.

Pulse wave myelopathy: An update of an hypothesis highlighting the similarities between syringomyelia and normal pressure hydrocephalus.

PubMed

Bateman, Grant A

2015-12-01

Most hypotheses trying to explain the pathophysiology of idiopathic syringomyelia involve mechanisms whereby CSF is pumped against a pressure gradient, from the subarachnoid space into the cord parenchyma. On review, these theories have universally failed to explain the disease process. A few papers have suggested that the syrinx fluid may originate from the cord capillary bed itself. However, in these papers, the fluid is said to accumulate due to impaired fluid drainage out of the cord. Again, there is little evidence to substantiate this. This proffered hypothesis looks at the problem from the perspective that syringomyelia and normal pressure hydrocephalus are almost identical in their manifestations but only differ in their site of effect within the neuraxis. It is suggested that the primary trigger for syringomyelia is a reduction in the compliance of the veins draining the spinal cord. This reduces the efficiency of the pulse wave dampening, occurring within the cord parenchyma, increasing arteriolar and capillary pulse pressure. The increased capillary pulse pressure opens the blood-spinal cord barrier due to a direct effect upon the wall integrity and interstitial fluid accumulates due to an increased secretion rate. An increase in arteriolar pulse pressure increases the kinetic energy within the cord parenchyma and this disrupts the cytoarchitecture allowing the fluid to accumulate into small cystic regions in the cord. With time the cystic regions coalesce to form one large cavity which continues to increase in size due to the ongoing interstitial fluid secretion and the hyperdynamic cord vasculature. Crown Copyright © 2015. Published by Elsevier Ltd. All rights reserved.

Syringomyelia following surgery for a spontaneous spinal subdural hematoma in a 13-year-old girl with congenital von Willebrand disease: case report and literature review.

PubMed

Ben Nsir, A; Boubaker, A; Jemel, H

2016-04-01

Spontaneous spinal subdural hematomas are rare. Their occurrence in a child with congenital von Willebrand disease and the complication of their surgery by a large secondary syringomyelia have never been previously reported. A 13-year-old girl with congenital von Willebrand disease presented to our emergency department in January 2011 for sudden onset of severe back pain centered in her thoracic spine rapidly aggravated by signs of acute myelopathy without any precipitating factor. MRI scan revealed a thoracic subdural collection anterior to the spinal cord at the T7-T9 level, hyperintense on T1- and T2-weighted sequences consistent with an acute spinal subdural hemorrhage. Evacuation of the subdural hematoma was realized immediately after hemostasis parameter correction, and post-operative course was uneventful with full functional recovery. One year later, the patient presented once again but with progressive and more severe myelopathy caused by a large syringomyelia extending from the T5 level to the conus medullaris. A syringopleural shunting was performed and the patient was unrolled under an intensive care and rehabilitation program. Her condition remarkably improved and she became able to walk independently within 2 weeks post-operatively. von Willebrand disease should be included as a possible factor of spontaneous spinal subdural hemorrhage. Surgery is advised in emergency and can be associated with remarkable recovery especially in children. Delayed syringomyelia can complicate the post-operative course and can be successfully addressed by syringopleural shunting. Long-term clinical and radiological follow-up is advocated.

Evaluation of operative procedures for symptomatic outcome after decompression surgery for Chiari type I malformation.

PubMed

Kalb, Samuel; Perez-Orribo, Luis; Mahan, Mark; Theodore, Nicholas; Nakaji, Peter; Bristol, Ruth E

2012-09-01

The wide spectrum of symptoms and radiographic findings in patients with Chiari I malformation makes the decision to proceed with intervention controversial. We evaluated symptomatic outcomes using diverse surgical techniques in 104 patients who underwent decompression surgery. The symptoms of most patients improved. Patients with syringomyelia showed less symptomatic improvement; however, syringomyelia was not associated with postoperative symptomatic worsening. Durotomy was performed in 97.1% and arachnoid opening was performed in 60.6% with visualization of the fourth ventricle in 51.9% of patients. Neither arachnoid opening nor fourth ventricle visualization was associated with the clinical outcome. Duraplasty was performed in 94.2% of patients. A Chiari plate was used in 13.4% of patients and was associated with favorable outcomes. Use of postoperative steroids or muscle relaxants was not associated with outcome. Syringomyelia showed a 62.5% improvement rate on postoperative MRI. In conclusion, bony decompression and dural opening are important aspects of Chiari I surgery, with symptomatic improvement observed in most patients. Copyright © 2012. Published by Elsevier Ltd.

Sleep Disordered Breathing in Children and Adolescents with Chiari Malformation Type I

PubMed Central

Losurdo, Anna; Dittoni, Serena; Testani, Elisa; Di Blasi, Chiara; Scarano, Emanuele; Mariotti, Paolo; Paternoster, Giovanna; Di Rocco, Concezio; Massimi, Luca; Della Marca, Giacomo

2013-01-01

Study Objectives: Chiari malformation Type I (CM-I) has been associated with sleep disordered breathing (SDB). The aim of this study was to evaluate the prevalence of SDB in CM-I and its clinical correlates in a population of children and adolescents. Methods: Fifty-three consecutive children and adolescents affected by CM-I were enrolled (27 girls and 26 boys, mean age 10.3 ± 4.3, range: 3-18 years). All patients underwent neurological examination, MRI, and polysomnography (PSG). Otorhinolaryngologic clinical evaluation was performed in patients with polysomnographic evidence of sleep-related upper airway obstruction. Results: Mean size of the herniation was 9.5 ± 5.4 mm. Fourteen patients had syringomyelia, 5 had hydrocephalus, 31 presented neurological signs, 14 had epileptic seizures, and 7 reported poor sleep. PSG revealed SDB in 13 subjects. Patients with SDB, compared to those without SDB, had a higher prevalence hydrocephalus (p = 0.002), syringomyelia (p = 0.001), and neurological symptoms (p = 0.028). No significant difference was observed in age, gender, prevalence of epilepsy, and size of the herniation. Obstructive SDB was associated with syringomyelia (p = 0.004), whereas central SDB was associated with hydrocephalus (p = 0.034). Conclusions: In our population of CM-I patients the prevalence of SDB was 24%, lower than that reported in literature. Moreover, our findings suggest that abnormalities in cerebrospinal fluid dynamics in CM-I, particularly syringomyelia and hydro-cephalus, are associated with SDB. Citation: Losurdo A; Dittoni S; Testani E; Di Blasi C; Scarano E; Mariotti P; Paternoster G; Di Rocco C; Massimi L; Della Marca G. Sleep disordered breathing in children and adolescents with Chiari malformation Type I. J Clin Sleep Med 2013;9(4):371-377. PMID:23585753

Craniovertebral junction realignment for the treatment of basilar invagination with syringomyelia: preliminary report of 12 cases.

PubMed

Goel, Atul; Sharma, Praveen

2005-10-01

Twelve selected patients, eight males and four females aged 14 to 50 years, with syringomyelia associated with congenital craniovertebral bony anomalies including basilar invagination and fixed atlantoaxial dislocation, and associated Chiari I malformation in eight, were treated by atlantoaxial joint manipulation and restoration of the craniovertebral region alignment between October 2002 and March 2004. Three patients had a history of trauma prior to the onset of symptoms. Spastic quadriparesis and ataxia were the most prominent symptoms. The mean duration of symptoms was 11 months. The atlantoaxial dislocation and basilar invagination were reduced by manual distraction of the facets of the atlas and axis, stabilization by placement of bone graft and metal spacers within the joint, and direct atlantoaxial fixation using an inter-articular plate and screw method technique. Following surgery all patients showed symptomatic improvement and restoration of craniovertebral alignment during follow up from 3 to 20 months (mean 7 months). Radiological improvement of the syrinx could not be evaluated as stainless steel metal plates, screws, and spacers were used for fixation. Manipulation of the atlantoaxial joints and restoring the anatomical craniovertebral alignments in selected cases of syringomyelia leads to remarkable and sustained clinical recovery, and is probably the optimum surgical treatment.

Three-dimensional constructive interference in steady-state magnetic resonance imaging in syringomyelia: advantages over conventional imaging.

PubMed

Roser, Florian; Ebner, Florian H; Danz, Søren; Riether, Felix; Ritz, Rainer; Dietz, Klaus; Naegele, Thomas; Tatagiba, Marcos S

2008-05-01

Neuroradiology has become indispensable in detecting the pathophysiology in syringomyelia. Constructive interference in steady-state (CISS) magnetic resonance (MR) imaging can provide superior contrast at the sub-arachnoid tissue borders. As this region is critical in preoperative evaluation, the authors hypothesized that CISS imaging would provide superior assessment of syrinx pathology and surgical planning. Based on records collected from a database of 130 patients with syringomyelia treated at the authors' institution, 59 patients were prospectively evaluated with complete neuroradiological examinations. In addition to routine acquisitions with FLAIR, T1- and T2-weighted, and contrast-enhanced MR imaging series, the authors obtained sagittal cardiac-gated sequences to visualize cerebrospinal fluid (CSF) pulsations and axial 3D CISS MR sequences to detect focal arachnoid webs. Statistical qualitative and quantitative evaluations of spinal cord/CSF contrast, spinal cord/CSF delineation, motion artifacts, and artifacts induced by pulsatile CSF flow were performed. The 3D CISS MR sequences demonstrated a contrast-to-noise ratio significantly better than any other routine imaging sequence (p < 0.001). Moreover, 3D CISS imaging can detect more subarachnoid webs and cavitations in the syrinx than T2-weighted MR imaging with less flow-void artifact. The limitation of 3D CISS imaging is a susceptibility to motion artifacts that can cause reduced spatial resolution. Lengthy acquisition times for axial segments can be reduced with multiplanar reconstruction of 3D CISS-generated sagittal images. Constructive interference in steady-state imaging is the MR sequence of choice in the preoperative evaluation of syringomyelia, allowing significantly higher detection rates of focal subarachnoid webs, whereas standard T2-weighted MR imaging shows turbulent CSF flow voids. Constructive interference in steady-state MR imaging enables the neurosurgeon to accurately identify cases requiring decompression for obstructed CSF. Motion artifacts can be eliminated with technical variations.

Hydrocephalus: a rare initial manifestation of sporadic intramedullary hemangioblastoma : Intramedullary hemangioblastoma presenting as hydrocephalus.

PubMed

Morais, Barbara Albuquerque; Cardeal, Daniel Dante; Ribeiro E Ribeiro, Renan; Frassetto, Fernando Pereira; Andrade, Fernanda Goncalves; Matushita, Hamilton; Teixeira, Manoel Jacobsen

2017-08-01

Intramedullary hemangioblastomas are rare benign vascular tumors, infrequent in pediatric patients. Clinical symptoms vary according to the age of presentation, tumor size, location, and concomitant syringomyelia. This is the second reported case of hemangioblastoma presenting with acute hydrocephalus. A 3-month-old infant with acute hydrocephalus was asymptomatic after a ventriculoperitoneal shunt was placed. She returned 3 months later with irritability, acute paraplegia, and respiratory distress. Magnetic resonance imaging (MRI) showed an intramedullary T8-T9 tumor with syringomyelia. She underwent surgical resection with good results during the 6-month follow-up. Intramedullary tumors may present as hydrocephalus and other nonspecific symptoms, with invariably delayed diagnosis in children, but must be considered in suspicious cases.

Post-traumatic syringomyelia: CSF hydrodynamic changes following spinal cord injury are the driving force in the development of PTSM.

PubMed

Shields, Christopher B; Zhang, Yi Ping; Shields, Lisa B E

2012-01-01

Post-traumatic syringomyelia (PTSM) is a disorder that occurs infrequently following spinal cord injury (SCI), characterized by progressive neurological deterioration resulting from syrinx expansion originating in proximity to the traumatic epicenter. Several pathogenetic factors are associated with this disorder, however, the precise mechanism of the development of PTSM is controversial. Combined anatomical alterations and molecular changes following trauma to the spinal cord and arachnoid participate in the development of this condition. These factors include narrowing or obstruction of the subarachnoid space (SAS), central canal occlusion, myelomalacia, and alterations in intramedullary water permeability. If a patient sustains a SCI with delayed progressive deterioration in neurological function, in association with the MRI appearance of syringomyelia (SM), the diagnosis of PTSM is straightforward. The treatment of PTSM has not undergone any significant changes recently. The surgical treatment of PTSM consists of reconstructing the SAS or shunting fluid away from the syrinx to other locations. The advantages and disadvantages of each procedure will be discussed. With greater understanding of the mechanisms contributing to the development of SM, including advanced diagnostic methods and further advances in the development of artificial dural and shunting tubing, future therapies of PTSM will be more effective and long-lasting. Incorporation of alterations of AQP4 expression provides an intriguing possibility for future treatment of PTSM. Copyright © 2012 Elsevier B.V. All rights reserved.

Hydromyelia

MedlinePlus

... children with hydrocephalus or birth defects such as Chiari Malformation II and Dandy-Walker syndrome. Syringomyelia, however, features ... primarily in adults, the majority of whom have Chiari Malformation type 1 or have experienced spinal cord trauma. ...

Syringomyelia

MedlinePlus

... are symptoms, they may include: Headache Loss of muscle mass ( wasting , atrophy), often in the arms and hands Spasms or tightness in the leg or hand and arm muscles Muscle function loss , loss of ability to use ...

Comparison of the scoliosis curve patterns and MRI syrinx cord characteristics of idiopathic syringomyelia versus Chiari I malformation.

PubMed

Zhu, Zezhang; Sha, Shifu; Chu, Winnie C C; Yan, Huang; Xie, Dingding; Liu, Zhen; Sun, Xu; Zhu, Weiguo; Cheng, Jack C Y; Qiu, Yong

2016-02-01

Although the more readily available MR imaging has brought about more incidental findings of idiopathic syringomyelia (IS), no published study has specifically addressed the clinical and imaging features of IS-associated scoliosis. Since IS and Chiari I malformation (CMI)-type syringomyelia are hypothesized to share a common underlying developmental pathomechanism, this study aimed to investigate the scoliosis curve patterns and MRI syrinx cord characteristics of patients with IS comparing with those seen in CMI. Sixty-one patients with scoliosis secondary to IS were identified and reviewed retrospectively. The curve pattern and specific curve features were recorded and compared with historic CMI controls. Location, size, and morphological appearance of the syrinx were systematically assessed on MR images. The maximal syrinx/cord ratio and rostrocaudal length of the syrinx in IS averaged 0.43 ± 0.16 (range 0.17-0.78) and 4.6 ± 2.5 (range 2-15) vertebral levels, respectively, both of which were smaller than those reported in CMI-type syringomyelia. Regarding the characteristics of IS-related scoliosis, sagittal profiles as well as the frequency of curve patterns and atypical features were all found to resemble those in patients with CMI (P > .05). Among the 47 individuals with a single thoracic curve, Fisher exact test revealed a significant correlation between curve convexity and the dominant side of deviated syrinx (83.3 % concordance rate, P = .021). In addition, apex of the thoracic curve trended toward being significantly correlated with the level of maximum expansion of the syrinx (P = .066). Radiological characteristics of scoliosis were found to be similar between idiopathic and CMI-type syrinx in both the coronal and sagittal planes, adding further evidence to the concept that these entities may be part of a spectrum of disease sharing a common pathophysiological mechanism. The thoracic spine in IS patients tended to be convex to the deviated side of syrinx, which indirectly supported the likely role of spinal cord dysfunction in the pathogenesis of syrinx-associated spinal deformities.

American Syringomyelia & Chiari Alliance Project

MedlinePlus

... and the families and friends who support them — information and support are vital in understanding the conditions and making the best treatment and lifestyle decisions. In this section, you’ll find all the ...

Syringomyelia: A review of the biomechanics

NASA Astrophysics Data System (ADS)

Elliott, N. S. J.; Bertram, C. D.; Martin, B. A.; Brodbelt, A. R.

2013-07-01

Syringomyelia is a neurological disorder caused by the development of one or more macroscopic fluid-filled cavities in the spinal cord. While the aetiology remains uncertain, hydrodynamics appear to play a role. This has led to the involvement of engineers, who have modelled the system in silico and on the bench. In the process, hypotheses from the neurosurgical literature have been tested, and others generated, while aspects of the system mechanics have been clarified. The spinal cord is surrounded by cerebrospinal fluid (CSF) which is subject both to the periodic excitation of CSF expelled from the head with each heartbeat, and to intermittent larger transients from cough, sneeze, etc., via vertebral veins. The resulting pulsatile flow and pressure wave propagation, and their possible effects on cord cavities and cord stresses, have been elucidated. These engineering contributions are here reviewed for the first time.

Subdural hematoma caused by intracranial hypotension after syringosubarachnoid shunting--case report.

PubMed

Yamazaki, Tomosato; Yanaka, Kiyoyuki; Uemura, Kazuya; Tsukada, Atsuro

2004-09-01

A 32-year-old man developed an extremely rare subdural hematoma after syringosubarachnoid shunting for syringomyelia. He presented with a 4-year history of neck pain and spastic paraparesis resulting from T-7 and T-8 vertebral body fracture suffered in a traffic accident at age 22 years. Magnetic resonance imaging revealed syringomyelia between the craniocervical junction and the T-10 level. The symptoms were slowly progressive, and a syringosubarachnoid shunting was performed. His spasticity improved after surgery, but he developed orthostatic headache 7 days after surgery. Magnetic resonance imaging of the brain demonstrated a thin subdural hematoma over the right cerebral convexity. The subdural hematoma resolved spontaneously within a week with conservative treatment. Vigorous cerebrospinal fluid outflow observed during surgery presumably lowered the pressure in the syrinx cavity, leading to significant but transient intracranial hypotension and consequently the formation of subdural hematoma.

Entire syringomyelia associated with Chiari II malformation and severe scoliosis and hydrocephalus.

PubMed

Liang, Jie; Wu, Weifei; Ru, Neng; Chen, Jianfeng

2017-01-01

The syrinx can occur in any region of the spinal cord and is common in cervical and/or thoracic region, and distributing along spinal cord is unusual, especially association with Chiari II malformation and scoliosis. To report a first case of entire syringomyelia associated with Chiari II malformation and severe scoliosis and hydrocephalus. The patient began to experience symptoms of bilateral hand weakness in adulthood. In this patient, MR imaging of the brain and spine showed syrinx along entire spine and hydrocephalus, cerebellar tonsillar herniation, and expansion of the fourth ventricle and posterior cranial fossa. The patient underwent operative treatment to prevent the progression of her neurological deficit. At 12 months' follow-up, the patient's neurological deficit remains stable with the scoliosis left untreated. Foramen magnum decompression, duraplasty and syrinx-shunting are effective methods even to CM II and entire syrinx.

Racial, Socioeconomic, and Gender Disparities in the Presentation, Treatment, and Outcomes of Adult Chiari I Malformations.

PubMed

Krucoff, Max O; Cook, Steven; Adogwa, Owoicho; Moreno, Jessica; Yang, Siyun; Xie, Jichun; Firempong, Alexander O; Lad, Nandan; Bagley, Carlos A

2017-01-01

To examine the influence of race, gender, and socioeconomic factors on presentations and outcomes of adult Chiari I malformations. The charts of 638 adult patients with Chiari I malformations were reviewed, and 287 patients were included in the study. Race, gender, insurance status, symptoms, depth of cerebellar tonsillar herniation, and presence of syringomyelia were examined as covariates in multivariate logistic regression models to identify independent predictors of presentation and outcome. Patients with public insurance had a longer stay in the hospital (P = 0.01). A higher proportion of male patients presented with upper extremity weakness (P = 0.01), lower extremity weakness (P = 0.040), and cranial nerve findings (P = 0.02). Men had shorter onset to diagnosis times (P = 0.02), worse tonsillar herniation (P = 0.03), and more severe symptoms (P = 0.05). White patients more frequently presented with back pain (P = 0.03), and African American patients more frequently presented with lower extremity weakness (P = 0.01). African Americans had worse tonsillar herniation (P < 0.01) and were more likely to present with syringomyelia (P = 0.01). Multivariate regression analysis revealed that back pain (P < 0.01), upper extremity weakness (P ≤ 0.01), upper extremity paresthesias (P < 0.01), and upper with lower extremity paresthesias (P = 0.04) were significant predictors of syringomyelia. The only independent predictor of outcome was size of tonsillar herniation (P = 0.03). Significant differences in presentation of Chiari I malformation resulting from gender, race, and insurance status were quantified for the first time. Copyright © 2016 Elsevier Inc. All rights reserved.

A Retrospective 2D Morphometric Analysis of Adult Female Chiari Type I Patients with Commonly Reported and Related Conditions

PubMed Central

Eppelheimer, Maggie S.; Houston, James R.; Bapuraj, Jayapalli R.; Labuda, Richard; Loth, Dorothy M.; Braun, Audrey M.; Allen, Natalie J.; Heidari Pahlavian, Soroush; Biswas, Dipankar; Urbizu, Aintzane; Martin, Bryn A.; Maher, Cormac O.; Allen, Philip A.; Loth, Francis

2018-01-01

Purpose: Researchers have sought to better understand Chiari type I malformation (CMI) through morphometric measurements beyond tonsillar position (TP). Soft tissue and bone structures within the brain and craniocervical junction have been shown to be different for CMI patients compared to healthy controls. Yet, several morphological characteristics have not been consistently associated with CMI. CMI is also associated with different prevalent conditions (PCs) such as syringomyelia, pseudotumor, Ehlers-Danlos syndrome (EDS), scoliosis, and craniocervical instability. The goal of this study was two-fold: (1) to identify unique morphological characteristics of PCs, and (2) to better explain inconsistent results from case-control comparisons of CMI. Methods: Image, demographic, and PC information was obtained through the Chiari1000, a self-report web-accessed database. Twenty-eight morphometric measurements (MMs) were performed on the cranial MR images of 236 pre-surgery adult female CMI participants and 140 female healthy control participants. Custom software was used to measure 28 structures within the posterior cranial fossa (PCF) compartment, craniocervical junction, oral cavity, and intracranial area on midsagittal MR images for each participant. Results: Morphometric analysis of adult females indicated a smaller McRae line length in CMI participants with syringomyelia compared to those without syringomyelia. TP was reduced in CMI participants with EDS than those without EDS. Basion to posterior axial line was significantly longer in CMI participants with scoliosis compared to those without scoliosis. No additional MMs were found to differ between CMI participants with and without a specific PC. Four morphometric differences were found to be consistently different between CMI participants and healthy controls regardless of PC: larger TP and a smaller clivus length, fastigium, and corpus callosum height in CMI participants. Conclusion: Syringomyelia, EDS, and scoliosis were the only PCs that showed significant morphometric differences between CMI participants. Additionally, four midsagittal MR-based MMs were found to be significantly different between healthy controls and CMI participants regardless of the presence of one or more PCs. This study suggests that the prevalence of comorbid conditions are not strongly related to CMI morphology, and that inconsistent findings in the radiographic literature cannot be explained by varying prevalence of comorbid conditions in CMI study samples. PMID:29403363

[Results of surgical treatment of syringomyelia associated with Chiari 1 malformation. An analysis of 125 cases].

PubMed

Zuev, A A; Pedyash, N V; Epifanov, D S; Kostenko, G V

2016-01-01

The rate of Chiari malformation (CM) in a population ranges from 3 to 8 per 100,000 population. In 62-80% of cases, CM is accompanied by the development of syringomyelia (SM) at various levels. The clinical picture in these patients is a combination of CM and SM manifestations; however, SM symptoms often prevail, which creates some problems in the diagnosis of the disease and in the choice of optimal treatment. On the basis of our own experience of surgical interventions, we aimed to clarify the indications for surgical treatment of SM associated with CM and to determine the optimal amount of surgery and the criteria for evaluation of treatment outcomes. Two hundred twenty five patients with a combination of syringomyelia and Chiari 1 malformation were examined in the period from 2011 to February 2015. Of them, 125 patients were operated on. The mean age of the operated patients was 56±8 years. The mean time from the appearance of the first signs of the disease to surgery was 75±82 months. All operations were performed by a single surgeon. The operations were carried out in the half-sitting (89.6%) or prone (10.4%) position. The operation included sparing suboccipital craniectomy, C1 arch resection, recovery of the cerebrospinal fluid (CSF) circulation along the posterior surface of the cerebellum, and reconstruction of the dura mater in the craniovertebral junction region. Exploration of the arachnoid mater of the cisterna magna after dura opening revealed no arachnopathy in 78 (62.4%) patients (Chiari 0 malformation according to Klekamp). The type 1 arachnopathy (by Klekamp) was detected in 31 patients (24.8%), and the type 2 arachnopathy was observed in 16 (12.8%). The condition of 109 (88%) patients was evaluated one year after the surgery. Sixty one (56%) patients had partial or complete regression of preoperative neurological symptoms. The disease stopped progressing in 44 patients (40%). The disease was progressing in 4 (3.7%) patients. No recurrence of a CSF circulation disturbance at the craniovertebral level was observed during follow-up. Early postoperative complications occurred in 4 (3.2%) patients: wound CSF leakage in 1 (0.8%) patient, acute epidural hematoma in 1 (0.8%) patient, and aseptic meningitis in 2 (1.6%) patients. Temporary deteriorations in the condition (headache worsening, meteosensitivity) were detected in 11 (8.9%) patients. These symptoms regressed by the end of the 1st postoperative month. There were no deaths. The indication for surgery in patients with a combination of CM and SM is the presence of neurological symptoms associated with syringomyelia and their progression as well as headache caused by herniation of the cerebellar tonsils, which significantly deteriorates the patient's quality of life. The main criteria for evaluating the efficacy of treatment include stabilization of the clinical symptoms and/or improvement in the patient condition. Suboccipital craniectomy followed by reconstruction of the dura mater and recovery of the CSF circulation in the craniovertebral region is an effective treatment of syringomyelia associated with Chiari 1 malformation.

Radiological Evaluation of 510 Cases of Basilar Invagination with Evidence of Atlantoaxial Instability (Group A Basilar Invagination).

PubMed

Goel, Atul; Jain, Sonal; Shah, Abhidha

2018-02-01

To evaluate the musculoskeletal and soft tissue neural alterations in cases with group A basilar invagination. Between January 2007 and August 2016, 510 patients with group A basilar invagination were surgically treated. The radiologic images of these patients were reviewed retrospectively. The patients were divided into 4 groups: group A1, comprising 60 patients with syringomyelia; group A2, comprising 354 patients with "external syrinx," marked by excessive cerebrospinal fluid (CSF) in the extramedullary space; group A3, comprising 51 patients with both syringomyelia and external syrinx; and group A4, comprising 45 patients with no abnormality of CSF cavitation in the spinal canal. A number of musculoskeletal and neural parameters, including the extent of basilar invagination, degree of angulation of the odontoid process, and facet malalignment, were evaluated in each of the 4 groups. The degree of basilar invagination was 6-27.4 mm (average, 15.85 mm) in group A1, 4.3-24.5 mm (average, 12.56 mm) in group A2, 5.6-17.6 mm (average 10.8 mm) in group A3, and 5.2-17.3 mm (average, 11.74 mm) in group A4. The angle of inclination of the odontoid process was 61.1-90.7 degrees (average, 71.4 degrees) in group A1, 30.5-79.8 degrees (average, 60.05 degrees) in group A2, 68.5-78.3 degrees (average, 73.4 degrees) in group A3, and 62.2-87.4 degrees (average, 71.2 degrees) in group A4. The nature of bone malformations directly influences the presence or absence of external syrinx and syringomyelia. Copyright © 2017 Elsevier Inc. All rights reserved.

Syringo-Subarachnoid Shunt for the Treatment of Persistent Syringomyelia Following Decompression for Chiari Type I Malformation: Surgical Results.

PubMed

Soleman, Jehuda; Roth, Jonathan; Bartoli, Andrea; Rosenthal, Daniel; Korn, Akiva; Constantini, Shlomi

2017-12-01

Approximately 30% of patients treated with foramen magnum decompression (FMD) for Chiari I-associated syringomyelia will show persistence, recurrence, or progression of the syrinx. This study evaluates the clinical and radiologic outcomes of syringo-subarachnoid shunt (SSS) as the treatment for persistent syringomyelia after FMD. Data were collected retrospectively. The primary outcome measurement was neurologic function (assessed with the Modified Japanese Orthopedic Association [mJOA] scale). Secondary outcome measurements were surgical complications, reoperation rate, and syrinx status on magnetic resonance imaging (MRI). Twenty-one patients (14 females [66.7%]) underwent SSS, either concurrent to the FMD or at a later stage. Two minor surgical complications were seen: a wound dehiscence and postoperative kyphosis, both requiring revision surgery. No major complication or mortality occurred. The median change in the mJOA score was an improvement of 3 out of a possible 17 points on the scale (mean follow-up, 24.9 months). Expressed as a percentage, overall improvement was 11.8% (95% confidence interval [CI], 5.9-17.6; P < 0.001). On postoperative MRI, shrinkage of the syrinx was seen in all but 1 patient in whom the syrinx remained unchanged. Expressed as percentage, the improvement of the syrinx surface was 76.3% (95% CI, 65.0-87.7; P < 0.001), and the improvement of syrinx span was 36.4% (95% CI, 21.8-50.9; P = 0.05). SSS for persistent, recurrent, or increasing syrinx following FMD for Chiari I malformation is a safe and effective surgical treatment when performed selectively by an experienced neurosurgeon. Copyright © 2017 Elsevier Inc. All rights reserved.

Chiari malformation may increase perivascular cerebrospinal fluid flow into the spinal cord: A subject-specific computational modelling study.

PubMed

Lloyd, Robert A; Fletcher, David F; Clarke, Elizabeth C; Bilston, Lynne E

2017-12-08

Syringomyelia is associated with Chiari I malformation, although the mechanistic link is unclear. Studies have suggested that cerebrospinal fluid enters the spinal cord via the perivascular spaces, and that changes in the timing of the subarachnoid pressures may increase flow into the spinal cord. This study aims to determine how Chiari malformation and syringomyelia alter the subarachnoid space pressures and hence perivascular flow. Subject-specific models of healthy controls (N = 9), Chiari patients with (N = 7) and without (N = 8) syringomyelia, were developed from magnetic resonance imaging (MRI), to simulate the subarachnoid pressures. These pressures were input to an idealised model of the perivascular space to evaluate potential differences in perivascular flow. Peak pressures in Chiari patients without a syrinx were higher than in controls (46% increase; p = .029) and arrived earlier in the cardiac cycle than both controls (2.58% earlier; p = .045) and syrinx patients (2.85% earlier; p = .045). The perivascular model predicted Chiari patients without a syrinx would have the greatest flow into the cord (p < .05) if the arterial pulse delay was between 4 and 10% of the cardiac cycle. Using phase-contrast MRI the mean arterial delay for all subjects was similar, and was estimated as 4.7 ± 0.2%. The perivascular pumping rate showed a strong positive correlation (R Adj 2 =0.85; p < .0001) with extended periods of high pressure that arrived earlier in the cardiac cycle, suggesting these pressure characteristics may play a role in syrinx development. Copyright © 2017 Elsevier Ltd. All rights reserved.

Inherited occipital hypoplasia/syringomyelia in the cavalier King Charles spaniel: experiences in setting up a worldwide DNA collection.

PubMed

Rusbridge, Clare; Knowler, Penny; Rouleau, Guy A; Minassian, Berge A; Rothuizen, Jan

2005-01-01

Inherited diseases commonly emerge within pedigree dog populations, often due to use of repeatedly bred carrier sire(s) within a small gene pool. Accurate family records are usually available making linkage analysis possible. However, there are many factors that are intrinsically difficult about collecting DNA and collating pedigree information from a large canine population. The keys to a successful DNA collection program include (1) the need to establish and maintain support from the pedigree breed clubs and pet owners; (2) committed individual(s) who can devote the considerable amount of time and energy to coordinating sample collection and communicating with breeders and clubs; and (3) providing means by which genotypic and phenotypic information can be easily collected and stored. In this article we described the clinical characteristics of inherited occipital hypoplasia/syringomyelia (Chiari type I malformation) in the cavalier King Charles spaniel and our experiences in establishing a pedigree and DNA database to study the disease.

Intracranial Hypertension in a Patient with a Chiari Malformation Accompanied by Hyperthyroidism.

PubMed

Pang, Chang Hwan; Lee, Soo Eon; Kim, Chi Heon; Chung, Chun Kee

2015-09-01

The Chiari malformation is an infrequently detected congenital anomaly characterized by the downward displacement of the cerebellum with a tonsillar herniation below the foramen magnum that may be accompanied by either syringomyelia or hydrocephalus. Surgery, such as foramen magnum decompression, is indicated for a symptomatic Chiari malformation, although an incidental lesion may be followed-up without further treatment. Infrequently, increased intracranial pressure emerges due to hyperthyroidism. A nineteen-year-old girl visited our outpatient clinic presented with a headache, nausea and vomiting. A brain and spinal magnetic resonance image study (MRI) indicated that the patient had a Chiari I malformation without syringomyelia or hydrocephalus. An enlarged thyroid gland was detected on a physical examination, and serum markers indicated Graves' disease. The patient started anti-hyperthyroid medical treatment. Subsequently, the headache disappeared after the medical treatment of hyperthyroidism without surgical intervention for the Chiari malformation. A symptomatic Chiari malformation is indicated for surgery, but a surgeon should investigate other potential causes of the symptoms of the Chiari malformation to avoid unnecessary surgery.

Dietzia papillomatosis bacteremia.

PubMed

Rammer, Paul; Calum, Henrik; Moser, Claus; Björnsdóttir, Maria K; Smedegaard, Heidi; Høiby, Niels; Bjarnsholt, Thomas

2013-06-01

The clinical significance of Dietzia papillomatosis is for the moment limited to the rare skin disease confluent and reticulated papillomatosis. We present a case of infection with D. papillomatosis in a 2-year-old boy with known syringomyelia. The microbiological diagnosis was done using 16S rRNA gene sequencing. This is the first report of bacteremia with D. papillomatosis.

Intracranial Hypertension in a Patient with a Chiari Malformation Accompanied by Hyperthyroidism

PubMed Central

Pang, Chang Hwan; Kim, Chi Heon; Chung, Chun Kee

2015-01-01

The Chiari malformation is an infrequently detected congenital anomaly characterized by the downward displacement of the cerebellum with a tonsillar herniation below the foramen magnum that may be accompanied by either syringomyelia or hydrocephalus. Surgery, such as foramen magnum decompression, is indicated for a symptomatic Chiari malformation, although an incidental lesion may be followed-up without further treatment. Infrequently, increased intracranial pressure emerges due to hyperthyroidism. A nineteen-year-old girl visited our outpatient clinic presented with a headache, nausea and vomiting. A brain and spinal magnetic resonance image study (MRI) indicated that the patient had a Chiari I malformation without syringomyelia or hydrocephalus. An enlarged thyroid gland was detected on a physical examination, and serum markers indicated Graves' disease. The patient started anti-hyperthyroid medical treatment. Subsequently, the headache disappeared after the medical treatment of hyperthyroidism without surgical intervention for the Chiari malformation. A symptomatic Chiari malformation is indicated for surgery, but a surgeon should investigate other potential causes of the symptoms of the Chiari malformation to avoid unnecessary surgery. PMID:26512271

Distribution of syringomyelia along the entire spinal cord in clinically affected Cavalier King Charles Spaniels.

PubMed

Loderstedt, Shenja; Benigni, Livia; Chandler, Kate; Cardwell, Jacqueline M; Rusbridge, Clare; Lamb, Christopher R; Volk, Holger A

2011-12-01

Chiari-like malformation (CM) and syringomyelia (SM) is an important disease complex in the Cavalier King Charles Spaniel (CKCS) but data about the anatomical distribution of SM along the spinal cord are lacking in veterinary medicine. The objective of this study was to define the anatomic distribution of SM in CKCS clinically affected by CM/SM. Magnetic resonance imaging (MRI) of the brain and the entire spinal cord of 49 dogs was performed and different morphological parameters compared. Syrinx formation was present in the C1-C4 region and in other parts of the spinal cord. The maximal dorsoventral syrinx size can occur in any region of the spinal cord and the total syrinx size was positively correlated with age. Seventy-six per cent of CKCS with a cranial cervical syrinx also have a syrinx affecting more caudal spinal cord regions. MRI restricted to the cervical region may underestimate the extent of SM and the severity of the disease process in the majority of dogs. Copyright © 2010 Elsevier Ltd. All rights reserved.

A challenging diagnosis of late-onset tumefactive multiple sclerosis associated to cervicodorsal syringomyelia: doubtful CT, MRI, and bioptic findings: Case report and literature review.

PubMed

Conforti, Renata; Capasso, Raffaella; Galasso, Rosario; Cirillo, Mario; Taglialatela, Gemma; Galasso, Luigi

2016-09-01

Tumefactive multiple sclerosis (MS) is an unusual variant of demyelinating disease characterized by lesions with pseudotumoral appearance on radiological imaging mimicking other space-occupying lesions, such as neoplasms, infections, and infarction. Especially when the patient's medical history is incompatible with MS, the differential diagnosis between these lesions constitutes a diagnostic challenge often requiring histological investigation. An older age at onset makes distinguishing tumefactive demyelinating lesion (TDL) from tumors even more challenging. We report a case of brain TDL as the initial manifestation of late-onset MS associated with cervico-dorsal syringomyelia. A 66-year-old Caucasian woman with a 15-day history headache was referred to our hospital because of the acute onset of paraphasia. She suffered from noncommunicating syringomyelia associated to basilar impression and she reported a 10-year history of burning dysesthesia of the left side of the chest extended to the internipple line level. Computed tomography (CT) and magnetic resonance imaging (MRI) examinations revealed a left frontal lesion with features suspicious for a tumor. Given the degree of overlap with other pathologic processes, CT and MRI findings failed to provide an unambiguous diagnosis; furthermore, because of the negative cerebrospinal fluid analysis for oligoclonal bands, the absence of other lesions, and the heightened suspicion of neoplasia, the clinicians opted to perform a stereotactic biopsy. Brain specimen analysis did not exclude the possibility of perilesional reactive gliosis and the patient, receiving anitiedemigen therapy, was monthly followed up. In the meanwhile, the second histological opinion of the brain specimen described the absence of pleomorphic glial cells indicating a tumor. These findings were interpreted as destructive inflammatory demyelinating disease and according to the evolution of MRI lesion burden, MS was diagnosed. TDL still remains a problematic entity clinically, radiologically, and sometimes even pathologically. A staged follow-up is necessary, and in our case, it revealed to be the most important attitude to define the nature of the lesion, confirming the classic MS diagnostic criteria of disseminate lesions in time and space. We discuss our findings according to the recent literature.

Comparison of gabapentin versus topiramate on clinically affected dogs with Chiari-like malformation and syringomyelia.

PubMed

Plessas, I N; Volk, H A; Rusbridge, C; Vanhaesebrouck, A E; Jeffery, N D

2015-09-19

To date there is no evidence-based data for efficacious treatment of neuropathic pain in dogs with Chiari-like malformation (CM) and syringomyelia (SM). The objective of this prospective cross-over study was to compare the effect of gabapentin versus topiramate, as an add-on treatment to carprofen, on quality of life (QoL) of dogs experiencing signs of neuropathic pain due to CM/SM. A visual analogue scale (VAS) was used to assess the QoL: (1) on day 0; (2) after 1 week of carprofen only; (3) after 2 weeks on carprofen and gabapentin; and (4) after 2 weeks on carprofen and topiramate. No significant difference was observed between VAS after gabapentin or topiramate (P=0.91). However, an improvement in QoL was observed when gabapentin was compared with baseline (P=0.009), but not for topiramate. In conclusion, the addition of gabapentin was more effective in improving QoL than carprofen alone, but the study failed to identify that gabapentin was more efficacious than topiramate. Perhaps the more favourable side effect profile of the former makes it more suitable for the treatment of neuropathic pain associated with CM/SM but further placebo-controlled trials are required to assess the efficacy of these drugs. British Veterinary Association.

Syringo-Subarachnoid-Peritoneal Shunt Using T-Tube for Treatment of Post-Traumatic Syringomyelia

PubMed Central

Kim, Seon-Hwan; Youm, Jin-Young; Kwon, Hyon-Jo

2012-01-01

Various surgical procedures for the treatment of post-traumatic syringomyelia have been introduced recently, but most surgical strategies have been unreliable. We introduce the concept and technique of a new shunting procedure, syringo-subarachnoid-peritoneal shunt. A 54-year-old patient presented to our hospital with a progressive impairment of motion and position sense on the right side. Sixteen years before this admission, he had been treated by decompressive laminectomy for a burst fracture of L1. On his recent admission, magnetic resonance (MR) imaging studies of the whole spine revealed the presence of a huge syrinx extending from the medulla to the L1 vertebral level. We performed a syringo-subarachnoid-peritoneal shunt, including insertion of a T-tube into the syrinx, subarachnoid space and peritoneal cavity. Clinical manifestations and radiological findings improved after the operation. The syringo-subarachnoid-peritoneal shunt has several advantages. First, fluid can communicate freely between the syrinx, the subarachnoid space, and the peritoneal cavity. Secondly, we can prevent shunt catheter from migrating because dural anchoring of the T-tube is easy. Finally, we can perform shunt revision easily, because only one arm of the T-tube is inserted into the intraspinal syringx cavity. We think that this procedure is the most beneficial method among the various shunting procedures. PMID:22993681

Dorsal arachnoid web.

PubMed

McCormick, Paul C

2014-09-01

Dorsal thoracic arachnoid web is a rare but often overlooked cause of progressive myelopathy. Syringomyelia, either above or below the compressive arachnoid band, may also be present. Dorsal arachnoid cyst and ventral spinal cord herniation may be mistaken for this condition. This video demonstrates the microsurgical identification and techniques of resection of a dorsal arachnoid band producing a progressive myelopathy in a 63-year-old man. The video can be found here: http://youtu.be/KDNTqiyW6yo.

Developments in the treatment of Chiari type 1 malformations over the past decade

PubMed Central

Pyne, Alexandra; Horn, Samantha R.; Poorman, Gregory W.; Janjua, Muhammad B.; Vasquez-Montes, Dennis; Bortz, Cole A.; Segreto, Frank A.; Frangella, Nicholas J.; Siow, Matthew Y.; Sure, Akhila; Zhou, Peter L.; Moon, John Y.; Diebo, Bassel G.; Vira, Shaleen N.

2018-01-01

Background Chiari malformations type 1 (CM-1), a developmental anomaly of the posterior fossa, usually presents in adolescence or early adulthood. There are few studies on the national incidence of CM-1, taking into account outcomes based on concurrent diagnoses. To quantify trends in treatment and associated diagnoses, as retrospective review of the Kid’s Inpatient Database (KID) from 2003-2012 was conducted. Methods Patients aged 0–20 with primary diagnosis of CM-1 in the KID database were identified. Demographics and concurrent diagnoses were analyzed using chi-squared and t-tests for categorical and numerical variables, respectively. Trends in diagnosis, treatments, and outcomes were analyzed using analysis of variance (ANOVA). Results Five thousand four hundred and thirty-eight patients were identified in the KID database with a primary diagnosis of CM-1 (10.5 years, 55% female). CM-1 primary diagnoses have increased over time (45 to 96 per 100,000). CM-1 patients had the following concurrent diagnoses: 23.8% syringomyelia/syringobulbia, 11.5% scoliosis, 5.9% hydrocephalus, 2.2% tethered cord syndrome. Eighty-three point four percent of CM-1 patients underwent surgical treatment, and rate of surgical treatment for CM-1 increased from 2003–2012 (66% to 72%, P<0.001) though complication rate decreased (7% to 3%, P<0.001) and mortality rates remained constant. Seventy percent of surgeries involved decompression-only, which increased neurologic complications compared to fusions (P=0.039). Cranial decompressions decreased from 2003–2012 (42.2–30.5%) while spinal decompressions increased (73.1–77.4%). Fusion rates have increased over time (0.45% to 1.8%) and are associated with higher complications than decompression-only (11.9% vs. 4.7%). Seven point four percent of patients experienced at least one peri-operative complication (nervous system, dysphagia, respiratory most common). Patients with concurrent hydrocephalus had increased; nervous system, respiratory and urinary complications (P<0.006) and syringomyelia increased the rate of respiratory complications (P=0.037). Conclusions CM-1 diagnoses have increased in the last decade. Despite the decrease in overall complication rates, fusions are becoming more common and are associated with higher peri-operative complication rates. Commonly associated diagnoses including syringomyelia and hydrocephalus, can dramatically increase complication rates. PMID:29732422

The prevalence of intraspinal anomalies in infantile and juvenile patients with "presumed idiopathic" scoliosis: a MRI-based analysis of 504 patients.

PubMed

Zhang, Wen; Sha, Shifu; Xu, Leilei; Liu, Zhen; Qiu, Yong; Zhu, Zezhang

2016-04-27

Though several studies have reported the incidence of intraspinal neural axis abnormalities in infantile and juvenile "presumed idiopathic" scoliosis, there has been a varying prevalence ranging from 11.1 to 26.0% based on a limited sample size. Therefore, such inconclusive findings have resulted in some questions on the MRI-associated role in the management of these patients. We aimed to investigate the prevalence and distribution of intraspinal anomalies in the infantile and juvenile patients with "presumed idiopathic" scoliosis and to explore the radiographic and clinical indicators with large sample size. A total of 504 infantile and juvenile patients diagnosed with "presumed idiopathic" scoliosis were examined for potentially-existing neural axis abnormalities by MRI. Patients were grouped into two cohorts according to the presence of neural axis abnormalities. Radiographic parameters including curve magnitude, curve pattern, location of apex, degree of thoracic kyphosis, and span of curve were recorded and compared between the two groups. The prevalence of the neural abnormalities between the infantile-age group and juvenile-age group was also compared. The student t test was used to evaluate the differences of continuous variables and the chi-square test was used to evaluate the difference of categorical variables. Fisher exact test was applied to detect the difference of the rate of intraspinal anomalies between the "infantile idiopathic scoliosis" and "juvenile idiopathic scoliosis" group. Involving the spinal cord, 94 patients (18.7%) were found to have a neural abnormality: Arnold-Chiari malformation alone in 43 patients, Arnold-Chiari malformation combined with syringomyelia in 18 patients, isolated syringomyelia in 13 patients, diastematomyelia in six patients, tethered cord combined with diastematomyelia in six patients, tethered cord alone in four patients, and other uncommon intraspinal abnormalities in the remaining four patients. Totally Arnold-Chiari malformation with or without syringomyelia accounted for 64.8% (61/94) among all these abnormalities. Male gender, left thoracic curve and right lumbar curve were found to be significantly associated with the presence of neural axis abnormalities on MRI. The incidence of neural axis abnormalities in the presumed IIS and JIS was 18.7%. Thus a routine MRI evaluation appears warranted for those "presumed idiopathic" scoliosis patients if aged less than 10 years, being male or having left thoracic or right lumbar curve.

The tragedy of syrinx.

PubMed

Demerdash, Amin; Loukas, Marios; Tubbs, R Shane

2014-03-01

Today, the term "syrinx" is used by many clinicians. Most users of the term, however, are not familiar with the origin of the word. The present paper reviews the history of the word syrinx, which is based in Greek mythology. Many modern terms have a derivation that is based on a rich history that has often been forgotten. The myth of Syrinx lives on in modern times by the continued use of syrinx in various words such as syringomyelia and syringe.

Effectiveness of breeding guidelines for reducing the prevalence of syringomyelia.

PubMed

Knowler, S P; McFadyen, A K; Rusbridge, C

Several toy breed dogs are predisposed to syringomyelia (SM), a spinal cord disorder, characterised by fluid-filled cavitation. SM is a complex trait with a moderately high heritability. Selective breeding against SM is confounded by its complex inheritance, its late onset nature and high prevalence in some breeds. This study investigated the early outcome of existing SM breeding guidelines. Six hundred and forty-three dogs, 550 Cavalier King Charles spaniels (CKCS) and 93 Griffon Bruxellois (GB), were identified as having either one (454 dogs) or both parents (189 dogs) with MRI-determined SM status. Offspring without SM were more common when the parents were both clear of SM (SM-free; CKCS 70 per cent, GB 73 per cent). Conversely, offspring with SM were more likely when both parents had SM (SM-affected; CKCS 92 per cent, GB 100 per cent). A mating of one SM-free parent with an SM-affected parent was risky for SM affectedness with 77 per cent of CKCS and 46 per cent of GB offspring being SM-affected. It is recommended that all breeding dogs from breeds susceptible to SM be MRI screened; that the SM status at five years old is established; and all results submitted to a central database that can be used by dog breeders to better enable mate selection based on estimated breeding values.

Atypical cerebellar slump syndrome and external hydrocephalus following craniocervical decompression for Chiari I malformation: case report.

PubMed

Thakar, Sumit; Dadlani, Ravi; Tawari, Manish; Hegde, Alangar S

2014-01-01

Symptomatic cerebellar slump (CS) and external hydrocephalus (EH) are amongst the rarer complications of foramen magnum decompression (FMD) for Chiari I malformation (CM). CS typically presents with delayed onset headache related to dural traction or with neurological deficit offsetting the benefit of FMD. EH, consisting of ventriculomegaly along with subdural fluid collection(s) (SFCs), has been related to cerebrospinal fluid egress from a tiny breach in an otherwise intact arachnoid. We describe the case of a 21-year-old man with CM and syringomyelia who presented with impaired gag, spastic quadriparesis, and raised intracranial pressure 1 week following an uneventful FMD during which the arachnoid had been widely fenestrated. Magnetic resonance imaging (MRI) showed an infratentorial SFC, dilated aqueduct and triventriculomegaly, features of CS, and a residual but resolving syrinx. His symptoms resolved following a high pressure ventriculo-peritoneal shunt. At a 6-month follow-up visit, he was asymptomatic and demonstrated partial resolution of the syrinx, with no recurrence of the SFC. The unusual features in the clinical course of this patient were an atypical CS syndrome presenting with concomitantly resolving syringomyelia, and the development of EH after a wide arachnoidal fenestration. This is the first case in indexed literature describing such a combination of unusual postoperative complications of a FMD. A hypothesis is presented to explain the clinico-radiological findings of the case.

Atypical Cerebellar Slump Syndrome and External Hydrocephalus following Craniocervical Decompression for Chiari I Malformation: Case Report

PubMed Central

THAKAR, Sumit; DADLANI, Ravi; TAWARI, Manish; HEGDE, Alangar S

2014-01-01

Symptomatic cerebellar slump (CS) and external hydrocephalus (EH) are amongst the rarer complications of foramen magnum decompression (FMD) for Chiari I malformation (CM). CS typically presents with delayed onset headache related to dural traction or with neurological deficit offsetting the benefit of FMD. EH, consisting of ventriculomegaly along with subdural fluid collection(s) (SFCs), has been related to cerebrospinal fluid egress from a tiny breach in an otherwise intact arachnoid. We describe the case of a 21-year-old man with CM and syringomyelia who presented with impaired gag, spastic quadriparesis, and raised intracranial pressure 1 week following an uneventful FMD during which the arachnoid had been widely fenestrated. Magnetic resonance imaging (MRI) showed an infratentorial SFC, dilated aqueduct and triventriculomegaly, features of CS, and a residual but resolving syrinx. His symptoms resolved following a high pressure ventriculo-peritoneal shunt. At a 6-month follow-up visit, he was asymptomatic and demonstrated partial resolution of the syrinx, with no recurrence of the SFC. The unusual features in the clinical course of this patient were an atypical CS syndrome presenting with concomitantly resolving syringomyelia, and the development of EH after a wide arachnoidal fenestration. This is the first case in indexed literature describing such a combination of unusual postoperative complications of a FMD. A hypothesis is presented to explain the clinico-radiological findings of the case. PMID:24257499

Charcot joint-like changes following ankle fracture in a patient with no underlying disease: report of a rare case.

PubMed

Kumagai, Masaru; Yokota, Kiyoshi; Endoh, Toshiya; Takemoto, Hitoshi; Nagata, Kensei

2002-01-01

Charcot joint is a disease that often occurs in patients with diabetes mellitus, tabes dorsalis, syringomyelia, chronic alcoholism, leprosy, trauma, or infection after fractures and dislocations. The treatment for Charcot joint has various complications, such as skin lesions, infections, and delayed union. We present our experience with a male patient who developed Charcot joint-like changes without diabetes mellitus or any other disease after an ankle fracture due to minor trauma.

A prospective controlled study of sleep respiratory events in patients with craniovertebral junction malformation.

PubMed

Botelho, Ricardo Vieira; Bittencourt, Lia Rita Azeredo; Rotta, José Marcos; Tufik, Sergio

2003-12-01

Craniovertebral junction malformation (CVJM) or Chiari malformation in adults, with or without syringomyelia and basilar invagination, produces neuronal dysfunction of the brainstem, cerebellum, cranial nerves, and upper spinal cord. The respiratory center and some of its afferent and efferent components can be altered in these diseases. The authors studied patients with and without CVJM to determine whether this physical feature contributed to sleep disturbances. Respiratory manifestations during sleep were studied prospectively, by using whole-night polysomnography, in 32 symptomatic patients (CVJM group) and 16 healthy volunteers (control group). Patients with CVJM presented with more sleep disturbances (reports of snoring and apnea) than those in the control group. The apnea/hypopnea index values were higher in patients with CVJMs than in the control group (13 +/- 15 compared with 3 +/- 6; p = 0.007) and the rate of central sleep apneas was higher in the CVJM than in the control group (22 +/- 30 compared with 4 +/- 8%; p = 0.009). The apnea/hypopnea index was highest in the subgroup with basilar invagination than in the other subgroups. The central apneic episodes were more frequent in the patients with basilar invagination (35 +/- 40%; p = 0.001) and in those with syringomyelia (17.6 +/- 24.6%; p = 0.003) than in the control group (4 +/- 8%). Patients with symptomatic CVJM, especially those with basilar invagination, presented with more sleep respiratory compromise than did those in the control group. The incidence of sleep apnea/hypopnea syndrome is significantly higher in patients with CVJM.

Malformations of the craniocervical junction (chiari type I and syringomyelia: classification, diagnosis and treatment)

PubMed Central

2009-01-01

Chiari disease (or malformation) is in general a congenital condition characterized by an anatomic defect of the base of the skull, in which the cerebellum and brain stem herniate through the foramen magnum into the cervical spinal canal. The onset of Chiari syndrome symptoms usually occurs in the second or third decade (age 25 to 45 years). Symptoms may vary between periods of exacerbation and remission. The diagnosis of Chiari type I malformation in patients with or without symptoms is established with neuroimaging techniques. The most effective therapy for patients with Chiari type I malformation/syringomyelia is surgical decompression of the foramen magnum, however there are non-surgical therapy to relieve neurophatic pain: either pharmacological and non-pharmacological. Pharmacological therapy use drugs that act on different components of pain. Non-pharmacological therapies are primarly based on spinal or peripheral electrical stimulation. It is important to determine the needs of the patients in terms of health-care, social, educational, occupational, and relationship issues, in addition to those derived from information aspects, particularly at onset of symptoms. Currently, there is no consensus among the specialists regarding the etiology of the disease or how to approach, monitor, follow-up, and treat the condition. It is necessary that the physicians involved in the care of people with this condition comprehensively approach the management and follow-up of the patients, and that they organize interdisciplinary teams including all the professionals that can help to increase the quality of life of patients. PMID:20018097

Acquired Chiari malformation secondary to atlantoaxial vertical subluxation in a patient with rheumatoid arthritis combined with atlanto-occipital assimilation.

PubMed

Kimura, Yuiko; Seichi, Atsushi; Gomi, Akira; Kojima, Masahiro; Inoue, Hirokazu; Kimura, Atsushi

2012-01-01

A 65-year-old woman with a history of rheumatoid arthritis presented with a rare case of acquired Chiari malformation secondary to atlantoaxial vertical subluxation, associated with congenital atlanto-occipital assimilation. Syringomyelia and tetraparesis improved immediately after posterior fossa decompression and simultaneous occipito-cervical junction fusion. The progression of acquired Chiari malformation is not well known. We concluded that coexisting assimilation accelerated crowded foramen magnum following atlantoaxial vertical subluxation and induced acquired Chiari malformation over the course of a few years.

A Poroelastic Fluid/Structure-Interaction Model of Cerebrospinal Fluid Dynamics in the Cord With Syringomyelia and Adjacent Subarachnoid-Space Stenosis.

PubMed

Bertram, C D; Heil, M

2017-01-01

An existing axisymmetric fluid/structure-interaction (FSI) model of the spinal cord, pia mater, subarachnoid space, and dura mater in the presence of syringomyelia and subarachnoid-space stenosis was modified to include porous solids. This allowed investigation of a hypothesis for syrinx fluid ingress from cerebrospinal fluid (CSF). Gross model deformation was unchanged by the addition of porosity, but pressure oscillated more in the syrinx and the subarachnoid space below the stenosis. The poroelastic model still exhibited elevated mean pressure in the subarachnoid space below the stenosis and in the syrinx. With realistic cord permeability, there was slight oscillatory shunt flow bypassing the stenosis via the porous tissue over the syrinx. Weak steady streaming flow occurred in a circuit involving craniocaudal flow through the stenosis and back via the syrinx. Mean syrinx volume was scarcely altered when the adjacent stenosis bisected the syrinx, but increased slightly when the syrinx was predominantly located caudal to the stenosis. The fluid content of the tissues over the syrinx oscillated, absorbing most of the radial flow seeping from the subarachnoid space so that it did not reach the syrinx. To a lesser extent, this cyclic swelling in a boundary layer of cord tissue just below the pia occurred all along the cord, representing a mechanism for exchange of interstitial fluid (ISF) and cerebrospinal fluid which could explain recent tracer findings without invoking perivascular conduits. The model demonstrates that syrinx volume increase is possible when there is subarachnoid-space stenosis and the cord and pia are permeable.

Spinal subarachnoid space pressure measurements in an in vitro spinal stenosis model: implications on syringomyelia theories.

PubMed

Martin, Bryn A; Labuda, Richard; Royston, Thomas J; Oshinski, John N; Iskandar, Bermans; Loth, Francis

2010-11-01

Full explanation for the pathogenesis of syringomyelia (SM), a neuropathology characterized by the formation of a cystic cavity (syrinx) in the spinal cord (SC), has not yet been provided. It has been hypothesized that abnormal cerebrospinal fluid (CSF) pressure, caused by subarachnoid space (SAS) flow blockage (stenosis), is an underlying cause of syrinx formation and subsequent pain in the patient. However, paucity in detailed in vivo pressure data has made theoretical explanations for the syrinx difficult to reconcile. In order to understand the complex pressure environment, four simplified in vitro models were constructed to have anatomical similarities with post-traumatic SM and Chiari malformation related SM. Experimental geometry and properties were based on in vivo data and incorporated pertinent elements such as a realistic CSF flow waveform, spinal stenosis, syrinx, flexible SC, and flexible spinal column. The presence of a spinal stenosis in the SAS caused peak-to-peak cerebrospinal fluid CSF pressure fluctuations to increase rostral to the stenosis. Pressure with both stenosis and syrinx present was complex. Overall, the interaction of the syrinx and stenosis resulted in a diastolic valve mechanism and rostral tensioning of the SC. In all experiments, the blockage was shown to increase and dissociate SAS pressure, while the axial pressure distribution in the syrinx remained uniform. These results highlight the importance of the properties of the SC and spinal SAS, such as compliance and permeability, and provide data for comparison with computational models. Further research examining the influence of stenosis size and location, and the importance of tissue properties, is warranted.

Chiari Malformation Type 1: A Systematic Review of Natural History and Conservative Management.

PubMed

Langridge, Benjamin; Phillips, Edward; Choi, David

2017-08-01

Chiari malformation type 1 (CM-1) is a variation of hindbrain development that can sometimes occur in asymptomatic individuals. Conventional treatment is surgical decompression, but little is known about the natural history of patients who do not undergo surgical management. This information is critical to determine how these patients should be managed. We conducted a systematic literature review to determine the natural history of CM-1, particularly in patients who did not undergo surgery and in asymptomatic individuals, to help patients and physicians determine when surgery is likely to be beneficial. The literature search was performed following Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines using the electronic databases PubMed, Scopus, Cochrane Library, and Web of Science. Inclusion and exclusion criteria were predefined. In symptomatic patients who did not undergo surgery, headaches and nausea often improved, whereas ataxia and sensory disturbance tended not to improve spontaneously. Of patients, 27%-47% had an improvement in symptoms after 15 months, and 37%-40% with cough headache and 89% with nausea who were managed nonoperatively improved at follow-up. Most asymptomatic individuals with CM-1 remained asymptomatic (93.3%) even in the presence of syringomyelia. The natural history of mild symptomatic and asymptomatic CM-1 in adults is relatively benign and nonprogressive; the decision to perform surgical decompression should be based on severity and duration of a patient's symptoms at presentation. It is reasonable to observe a patient with mild or asymptomatic symptoms even in the presence of significant tonsillar descent or syringomyelia. Copyright © 2017 Elsevier Inc. All rights reserved.

Technique for Mini-open Decompression of Chiari Type I Malformation in Adults.

PubMed

Pakzaban, Peyman

2017-08-01

The technique for decompression of Chiari type I malformation relies on open exposure of craniocervical junction for suboccipital craniectomy and upper cervical laminectomy with or without duraplasty. There is no detailed technical report of a minimally invasive approach for Chiari decompression in adults. To describe a mini-open technique for decompression of Chiari type I malformation (including duraplasty) in adults. Six consecutive adult patients with symptomatic Chiari type I malformation underwent decompression through a 3 to 4 cm midline incision via a speculum retractor. All patients underwent a limited suboccipital craniectomy and C1 laminectomy with an ultrasonic bone scalpel. All patients underwent duraplasty with a synthetic dural substitute. In the 2 patients with syringomyelia, the arachnoid was opened and intradural dissection was carried out. In the remaining 4 patients, the arachnoid was left intact. All operations were completed successfully through the mini-open exposure. Mean surgery time, blood loss, and length of stay were 114 min, 55 mL, and 1.3 days, respectively. Mean follow-up was 13.2 months (range 9-18). All patients had excellent clinical outcomes as defined by scores of 15 (3 patients) or 16 (3 patients) on Chicago Chiari Outcome Scale. There were no neurological complications or cerebrospinal fluid leaks. Postop computed tomography revealed good boney decompression. In the 2 patients with syringomyelia, MRI at 6 months revealed resolution of the syrinx. Decompression of Chiari type I malformation in adults can be performed safely and effectively through the mini-open exposure described in this report. Copyright © 2017 by the Congress of Neurological Surgeons

Efficacy of Posterior Fossa Decompression with Duraplasty for Patients with Chiari Malformation Type I: A Systematic Review and Meta-Analysis.

PubMed

Chai, Zhi; Xue, Xiaoming; Fan, Huijie; Sun, Lin; Cai, Hongyu; Ma, Yanmiao; Ma, Cungen; Zhou, Ran

2018-05-01

To quantitatively assess and compare the effectiveness and safety of posterior fossa decompression with duraplasty (PFDD) and posterior fossa decompression (PFD) in treating patients with Chiari malformation type I. PubMed, Embase, and Cochrane Library were searched through May 2017. Fourteen cohort studies comprising 3666 patients with Chiari malformation type I were included. Studies were pooled, and the relative risk (RR) and corresponding 95% confidence interval (CI) were calculated. The decrease in syringomyelia was better in patients in the PFDD group than in patients in the PFD group (RR = 1.57, 95% CI = 1.07-2.32, P heterogeneity  = 0.042, I 2  = 56.6%). The incidence of cerebrospinal fluid leak (RR = 5.23, 95% CI = 2.61-10.51, P heterogeneity  = 0.830, I 2  = 0%) and aseptic meningitis (RR = 4.02, 95% CI = 1.46-11.03, P heterogeneity  = 0.960, I 2  = 0%) significantly increased among patients in the PFDD group compared with patients in the PFD group. When stratifying by age, a significantly reduced risk in the reoperation rate was observed in the adult group. However, the clinical improvement and the incidence of wound infection were not significantly different between the 2 groups. This study confirmed that the decrease in syringomyelia was better for patients treated with PFDD than for patients treated with PFD alone. However, no significant difference was found in the clinical improvement and the reoperation rate between the 2 groups. Copyright © 2018 Elsevier Inc. All rights reserved.

Chiari-like Malformation.

PubMed

Loughin, Catherine A

2016-03-01

Chiari-like malformation is a condition of the craniocervical junction in which there is a mismatch of the structures of the caudal cranial fossa causing the cerebellum to herniate into the foramen magnum. This herniation can lead to fluid buildup in the spinal cord, also known as syringomyelia. Pain is the most common clinical sign followed by scratching. Other neurologic signs noted are facial nerve deficits, seizures, vestibular syndrome, ataxia, menace deficit, proprioceptive deficits, head tremor, temporal muscle atrophy, and multifocal central nervous system signs. MRI is the diagnostic of choice, but computed tomography can also be used. Copyright © 2016 Elsevier Inc. All rights reserved.

Long-term outcome of Cavalier King Charles spaniel dogs with clinical signs associated with Chiari-like malformation and syringomyelia.

PubMed

Plessas, I N; Rusbridge, C; Driver, C J; Chandler, K E; Craig, A; McGonnell, I M; Brodbelt, D C; Volk, H A

2012-11-17

The disease complex Chiari-like malformation (CM) and syringomyelia (SM) has been associated with the development of neuropathic pain (NeP), and commonly affects Cavalier King Charles spaniels (CKCS). This prospective cohort study followed 48 CKCSs with CM and/or SM and clinical signs suggestive of NeP for a period of 39 (±14.3) months from diagnosis. At the end of the study, 36 dogs were still alive; five dogs died of an unrelated or unknown cause, and seven were euthanased due to severe clinical signs suggestive of NeP. During the follow-up period, the clinical signs of scratching, facial rubbing behaviour, vocalisation and exercise ability were evaluated. Nine out of 48 dogs stopped scratching (P<0.001), but there was no statistically significant change in the number of dogs exhibiting exercise intolerance, vocalisation or facial rubbing behaviour. The overall severity of clinical signs based on a visual analogue scale (VAS) (0 mm: no clinical signs 100 mm: severe clinical signs) increased (from median 75 mm (interquartile ranges (IQR) 68-84) to 84 mm (IQR 71.5-91), P<0.001). A quarter of the dogs were static or improved. In general, the majority of the owners felt that the quality of life of their dogs was acceptable. Medical treatments received were gabapentin or pregabalin and/or intermittently, carprofen. The owner's perception of their animal's progress, and progress based on VAS, had strong positive correlation (Spearman's rank correlation (s(r)) 0.74, P<0.001). Overall, this study suggests that clinical signs suggestive of NeP progress in three-quarters of CKCSs with CM and/or SM.

A New Classification for Pathologies of Spinal Meninges-Part 2: Primary and Secondary Intradural Arachnoid Cysts.

PubMed

Klekamp, Jörg

2017-08-01

Spinal intradural arachnoid cysts are rare causes of radiculopathy or myelopathy. Treatment options include resection, fenestration, or cyst drainage. To classify intradural spinal arachnoid cysts and present results of their treatment. Among 1519 patients with spinal space occupying lesions, 130 patients demonstrated intradural arachnoid cysts. Neuroradiological and surgical features were reviewed and clinical data analyzed. Twenty-one patients presented arachnoid cysts as a result of an inflammatory leptomeningeal reaction related to meningitis, subarachnoid hemorrhage, intrathecal injections, intradural surgery, or trauma, ie, secondary cysts. For the remaining 109 patients, no such history could be elucidated, ie, primary cysts. Forty-six percent of primary and 86% of secondary cysts were associated with syringomyelia. Patients presented after an average history of 53 ± 88 months. There were 122 thoracic and 7 lumbar cysts plus 1 cervical cyst. Fifty-nine patients with primary and 15 patients with secondary cysts underwent laminotomies with complete or partial cyst resection and duraplasty. Mean follow-up was 57 ± 52 months. In the first postoperative year, profound improvements for primary cysts were noted, in contrast to marginal changes for secondary cysts. Progression-free survival for 10 years following surgery was determined as 83% for primary compared to 15% for secondary cysts. Despite differences in clinical presentation, progression-free survival was almost identical for patients with or without syringomyelia. Complete or partial resection leads to favorable short- and long-term results for primary arachnoid cysts. For secondary cysts, surgery can only provide clinical stabilization for a limited time due to the often extensive arachnoiditis. Copyright © 2017 by the Congress of Neurological Surgeons

Pediatric Chiari malformation type I: long-term outcomes following small-bone-window posterior fossa decompression with autologous-fascia duraplasty

PubMed Central

Liu, Hai; Yang, Chenlong; Yang, Jun; Xu, Yulun

2017-01-01

Chiari malformation type I (CM-I) is a common hindbrain disorder that is associated with deformity and elongation of the cerebellar tonsils. Although CM-I occurs in both pediatric and adult patients, its prevalence, clinical features and management in the pediatric population are not well defined. The current study evaluated a consecutive case series of 92 children (38 females and 54 males) who were diagnosed with congenital CM-I. All patients underwent small-bone-window posterior fossa decompression with autologous-fascia duraplasty. Clinical and radiological features were analyzed and long-term follow-up data were recorded. Risk factors associated with clinical outcomes were investigated using comprehensive statistical methods. Out of the 92 children, 11 (12.0%) were asymptomatic. Associated ventricular dilation was observed in 24 children (26.1%) and concomitant syringomyelia was observed in 72 children (78.3%). A total of 44 children (47.8%) showed scoliosis on plain films. Follow-up data (mean duration, 88.6 months) were available for all patients. Syringomyelia was absent or markedly reduced in 56 patients (77.8%). Symptoms were alleviated in 66 patients, remained unchanged in 12 patients and progressed in 3 patients. Statistical analysis indicated that the cerebellar tonsillar descent (CTD) grade, basilar invagination and platybasia influenced the clinical outcome (P<0.05). In conclusion, early recognition and surgical treatment of CM-I in pediatric patients can lead to good outcomes. The current results suggested that small-bone-window posterior fossa decompression with autologous-fascia duraplasty was an effective safe treatment option with a low complication rate. High CTD grade, basilar invagination and platybasia were indicated to be predictors of poor clinical prognosis. PMID:29285106

Dogs attending primary-care practice in England with clinical signs suggestive of Chiari-like malformation/syringomyelia.

PubMed

Sanchis-Mora, S; Pelligand, L; Thomas, C L; Volk, H A; Abeyesinghe, S M; Brodbelt, D C; Church, D B; Thomson, P C; McGreevy, P D; O'Neill, D G

2016-10-29

Chiari-like malformation/syringomyelia (CM/SM) in dogs describes a developmental disorder that can cause pain and reduced quality of life. This retrospective study aimed to report the period prevalence, clinical signs and risk factors for diagnosis of symptomatic CM/SM in the veterinary primary-care setting using a cross-sectional design. The study population included all dogs within the VetCompass Programme (September 1, 2009-June 13, 2014). Overall, the period prevalence of symptomatic CM/SM was 0.05 per cent (95 per cent CI 0.04 per cent to 0.06 per cent) for all breeds. The period prevalence in the Cavalier King Charles Spaniel (CKCS) was 1.6 per cent (95 per cent CI 1.2 to 2.06). Other breeds at increased odds included the King Charles Spaniel (KCS), Affenpinscher, Chihuahua and Pomeranian. Insured dogs had 4.6 times the odds (95 per cent CI 2.95 to 7.17) of having a diagnosis of CM/SM compared with uninsured dogs. Pain was the most commonly associated clinical sign (67 dogs, 72 per cent). Analgesics were prescribed to 72 (77.4 per cent) of the symptomatic dogs. Despite its low overall period prevalence, the high proportion of affected dogs identified with chronic pain suggests a significant welfare issue. Financial implications could impede the diagnostic process and lead to underestimation of the true prevalence. This study may help to inform clinicians about the clinical relevance and the need for improved awareness of clinical signs, particularly in high-risk breeds, to optimise the management of CM/SM in primary-care practice. British Veterinary Association.

Robinow syndrome: phenotypic variability in a family with a novel intragenic ROR2 mutation.

PubMed

Brunetti-Pierri, Nicola; Del Gaudio, Daniela; Peters, Hartmut; Justino, Henri; Ott, Claus-Eric; Mundlos, Stefan; Bacino, Carlos A

2008-11-01

Robinow syndrome comprises dysmorphic facial features, short stature, brachymesomelia, segmental spine defects, and genital hypoplasia. The range of severity in this disorder is broad. We report on the clinical and molecular findings of two sib pairs from the same extended family with Robinow syndrome due to a novel intragenic ROR2 deletion involving exons 6 and 7 that could not be detected by sequencing. The affected individuals exhibited variability with respect to the cleft lip, cleft palate, and cardiac findings and for the presence in one of the patients of syringomyelia, which has not been previously reported in Robinow syndrome. Copyright 2008 Wiley-Liss, Inc.

‘Serpent in the spine’: a case of giant spinal ependymoma of cervicothoracic spine

PubMed Central

Arrifin, Arlizan; Kaliaperumal, Chandrasekaran; Keohane, Catherine; O'Sullivan, Michael

2012-01-01

We describe a case of giant spinal ependymoma of cervicothoracic spine in a 30-year-old lady who presented with progressive spastic paraparesis and significant combined upper and lower motor neuron signs in her lower limbs over a 1-year period. She also had upper limb small muscle wasting with absent reflexes and diminished sensation. She was wheel chair bound with involvement of sphincters. Neuroimaging revealed a uniformly enhancing intramedullary lesion from C2–T3 level with associated syringomyelia. She underwent a complete excision of this World Health Organisation (WHO) II cellular ependymoma, resulting in significant clinical outcome and improvement in bladder and bowel function. PMID:22739334

Differentiation of idiopathic spinal cord herniation from dorsal arachnoid webs on MRI and CT myelography.

PubMed

Schultz, Randall; Steven, Andrew; Wessell, Aaron; Fischbein, Nancy; Sansur, Charles A; Gandhi, Dheeraj; Ibrahimi, David; Raghavan, Prashant

2017-06-01

OBJECTIVE Dorsal arachnoid webs (DAWs) and spinal cord herniation (SCH) are uncommon abnormalities affecting the thoracic spinal cord that can result in syringomyelia and significant neurological morbidity if left untreated. Differentiating these 2 entities on the basis of clinical presentation and radiological findings remains challenging but is of vital importance in planning a surgical approach. The authors examined the differences between DAWs and idiopathic SCH on MRI and CT myelography to improve diagnostic confidence prior to surgery. METHODS Review of the picture archiving and communication system (PACS) database between 2005 and 2015 identified 6 patients with DAW and 5 with SCH. Clinical data including demographic information, presenting symptoms and neurological signs, and surgical reports were collected from the electronic medical records. Ten of the 11 patients underwent MRI. CT myelography was performed in 3 patients with DAW and in 1 patient with SCH. Imaging studies were analyzed by 2 board-certified neuroradiologists for the following features: 1) location of the deformity; 2) presence or absence of cord signal abnormality or syringomyelia; 3) visible arachnoid web; 4) presence of a dural defect; 5) nature of dorsal cord indentation (abrupt "scalpel sign" vs "C"-shaped); 6) focal ventral cord kink; 7) presence of the nuclear trail sign (endplate irregularity, sclerosis, and/or disc-space calcification that could suggest a migratory path of a herniated disc); and 8) visualization of a complete plane of CSF ventral to the deformity. RESULTS The scalpel sign was positive in all patients with DAW. The dorsal indentation was C-shaped in 5 of 6 patients with SCH. The ventral subarachnoid space was preserved in all patients with DAW and interrupted in cases of SCH. In no patient was a web or a dural defect identified. CONCLUSIONS DAW and SCH can be reliably distinguished on imaging by scrutinizing the nature of the dorsal indentation and the integrity of the ventral subarachnoid space at the level of the cord deformity.

Clinical, radiological, and genetic similarities between patients with Chiari Type I and Type 0 malformations.

PubMed

Markunas, Christina A; Tubbs, R Shane; Moftakhar, Roham; Ashley-Koch, Allison E; Gregory, Simon G; Oakes, W Jerry; Speer, Marcy C; Iskandar, Bermans J

2012-04-01

Although Chiari Type I (CM-I) and Type 0 (CM-0) malformations have been previously characterized clinically and radiologically, there have been no studies focusing on the possible genetic link between these disorders. The goal of this study was to identify families in whom CM-0 and CM-I co-occurred and to further assess the similarities between these disorders. Families were ascertained through a proband with CM-I. Detailed family histories were obtained to identify first-degree relatives diagnosed with CM-0. Several criteria were used to exclude individuals with acquired forms of CM-I and/or syringomyelia. Individuals were excluded with syndromic, traumatic, infectious, or tumor-related syringomyelia, as well as CM-I due to a supratentorial mass, hydrocephalus, history of cervical or cranial surgery unrelated to CM-I, or development of symptoms following placement of a lumbar shunt. Medical records and MR images were used to characterize CM-I and CM-0 individuals clinically and radiologically. Five families were identified in which the CM-I proband had a first-degree relative with CM-0. Further assessment of affected individuals showed similar clinical and radiological features between CM-0 and CM-I individuals, although CM-I patients in general had more severe symptoms and skull base abnormalities than their CM-0 relatives. Overall, both groups showed improvement in symptoms and/or syrinx size following craniocervical decompression surgery. There is accumulating evidence suggesting that CM-0 and CM-I may be caused by a common underlying developmental mechanism. The data in this study are consistent with this hypothesis, showing similar clinical and radiological features between CM-0 and CM-I individuals, as well as the occurrence of both disorders within families. Familial clustering of CM-0 and CM-I suggests that these disorders may share an underlying genetic basis, although additional epigenetic and/or environmental factors are likely to play an important role in the development of CM-0 versus CM-I.

Craniometric Analysis of the Hindbrain and Craniocervical Junction of Chihuahua, Affenpinscher and Cavalier King Charles Spaniel Dogs With and Without Syringomyelia Secondary to Chiari-Like Malformation

PubMed Central

Kiviranta, Anna-Mariam; McFadyen, Angus K.; Jokinen, Tarja S.; La Ragione, Roberto M.; Rusbridge, Clare

2017-01-01

Objectives To characterize and compare the phenotypic variables of the hindbrain and craniocervical junction associated with syringomyelia (SM) in the Chihuahua, Affenpinscher and Cavalier King Charles Spaniel (CKCS). Method Analysis of 273 T1-weighted mid-sagittal DICOM sequences of the hindbrain and craniocervical junction from 99 Chihuahuas, 42 Affenpinschers and 132 CKCSs. The study compared 22 morphometric features (11 lines, eight angles and three ratios) of dogs with and without SM using refined techniques based on previous studies of the Griffon Bruxellois (GB) using Discriminant Function Analysis and ANOVA with post-hoc corrections. Results The analysis identified 14/22 significant traits for SM in the three dog breeds, five of which were identical to those reported for the GB and suggest inclusion of a common aetiology. One ratio, caudal fossa height to the length of the skull base extended to an imaginary point of alignment between the atlas and supraoccipital bones, was common to all three breeds (p values 0.029 to <0.001). Associated with SM were a reduced occipital crest and two acute changes in angulation i) ‘sphenoid flexure’ at the spheno-occipital synchondrosis ii) ‘cervical flexure’ at the foramen magnum allied with medulla oblongata elevation. Comparing dogs with and without SM, each breed had a unique trait: Chihuahua had a smaller angle between the dens, atlas and basioccipital bone (p value < 0.001); Affenpinschers had a smaller distance from atlas to dens (p value 0.009); CKCS had a shorter distance between the spheno-occipital synchondrosis and atlas (p value 0.007). Conclusion The selected morphometries successfully characterised conformational changes in the brain and craniocervical junction that might form the basis of a diagnostic tool for all breeds. The severity of SM involved a spectrum of abnormalities, incurred by changes in both angulation and size that could alter neural parenchyma compliance and/or impede cerebrospinal fluid channels. PMID:28121988

Craniometric Analysis of the Hindbrain and Craniocervical Junction of Chihuahua, Affenpinscher and Cavalier King Charles Spaniel Dogs With and Without Syringomyelia Secondary to Chiari-Like Malformation.

PubMed

Knowler, Susan P; Kiviranta, Anna-Mariam; McFadyen, Angus K; Jokinen, Tarja S; La Ragione, Roberto M; Rusbridge, Clare

2017-01-01

To characterize and compare the phenotypic variables of the hindbrain and craniocervical junction associated with syringomyelia (SM) in the Chihuahua, Affenpinscher and Cavalier King Charles Spaniel (CKCS). Analysis of 273 T1-weighted mid-sagittal DICOM sequences of the hindbrain and craniocervical junction from 99 Chihuahuas, 42 Affenpinschers and 132 CKCSs. The study compared 22 morphometric features (11 lines, eight angles and three ratios) of dogs with and without SM using refined techniques based on previous studies of the Griffon Bruxellois (GB) using Discriminant Function Analysis and ANOVA with post-hoc corrections. The analysis identified 14/22 significant traits for SM in the three dog breeds, five of which were identical to those reported for the GB and suggest inclusion of a common aetiology. One ratio, caudal fossa height to the length of the skull base extended to an imaginary point of alignment between the atlas and supraoccipital bones, was common to all three breeds (p values 0.029 to <0.001). Associated with SM were a reduced occipital crest and two acute changes in angulation i) 'sphenoid flexure' at the spheno-occipital synchondrosis ii) 'cervical flexure' at the foramen magnum allied with medulla oblongata elevation. Comparing dogs with and without SM, each breed had a unique trait: Chihuahua had a smaller angle between the dens, atlas and basioccipital bone (p value < 0.001); Affenpinschers had a smaller distance from atlas to dens (p value 0.009); CKCS had a shorter distance between the spheno-occipital synchondrosis and atlas (p value 0.007). The selected morphometries successfully characterised conformational changes in the brain and craniocervical junction that might form the basis of a diagnostic tool for all breeds. The severity of SM involved a spectrum of abnormalities, incurred by changes in both angulation and size that could alter neural parenchyma compliance and/or impede cerebrospinal fluid channels.

International survey on the management of Chiari 1 malformation and syringomyelia: evolving worldwide opinions.

PubMed

Singhal, Ash; Cheong, Alexander; Steinbok, Paul

2018-03-12

In 2003, pediatric neurosurgeons were surveyed under the auspices of the education committee of the International Society for Pediatric Neurosurgery (ISPN) to determine prevailing opinions regarding the management of Chiari I malformation (C1M) with and without associated syringomyelia. In the ensuing years, there has been further information from multiple C1M studies, with regards to indications, success rates of different surgical interventions, and complications. The purpose of this study was to re-evaluate current opinions and practices in pediatric C1M. Pediatric neurosurgeons worldwide were surveyed, using an e-mail list provided by the ISPN communication committee chairperson. Respondents were given scenarios similar to the 2003 C1M survey in order to determine opinions regarding whether to surgically intervene, and if so, with which operations. Of 300 surveys electronically distributed, 122 responses were received (40.6% response rate)-an improvement over the 30.8% response rate in 2003. Pediatric neurosurgeons from 34 different countries responded. There was broad consensus that non-operative management is appropriate in asymptomatic C1M (> 90%) as well as asymptomatic C1M with a small syrinx (> 65%). With a large syrinx, a majority (almost 80%) recommended surgical intervention. Scoliotic patients with CIM were generally offered surgery only when there was a large syrinx. There has been a shift in the surgical management over the past decade, with a bone-only decompression now being offered more commonly. There remains, however, great variability in the operation offered. This survey, with a relatively strong response rate, and with broad geographic representation, summarizes current worldwide expert opinion regarding management of pediatric C1M. Asymptomatic C1M and C1M with a small syrinx are generally managed non-operatively. When an operation is indicated, there has been a shift towards less invasive surgical approaches.

Early onset scoliosis with intraspinal anomalies: management with growing rod.

PubMed

Jayaswal, Arvind; Kandwal, Pankaj; Goswami, Ankur; Vijayaraghavan, G; Jariyal, Ashok; Upendra, B N; Gupta, Ankit

2016-10-01

To evaluate clinical and radiological outcomes of growing rod (GR) in the management of Early Onset Scoliosis (EOS) with intraspinal anomalies. The effect of repeated distractions following GR, in the presence of intraspinal anomalies has not been studied. During 2007-2012, 46 patients underwent fusionless surgery. Out of these 46 patients, 13 patients had one or more intraspinal anomalies. 11 patients had undergone prior neurosurgical procedure while 2 (filum terminale lipoma and syringomyelia) did not. A total of 88 procedures were conducted during the treatment period; 13 index surgeries, 74 distractions of GR and 1 unplanned surgery. The age at surgery was 6.8 ± 2.5 years (3.5-12 years). 11 patients had congenital scoliosis and 2 had idiopathic scoliosis. A total of 19 (41.30 %) intraspinal anomalies [Tethered Cord Syndrome (TCS) 08, Split Cord Malformation (SCM) 08, Syringomyelia 01, Meningomyelocele 01, Filum terminale Lipoma 01] were seen. The average lengthening procedures per patient were 5.7 (4-9) with distraction interval of 6.7 (6-7.25) months. Pre-operative Cobb angle was 78.50 ± 18.1 (54-114°) and improved to 53.10 ± 16.70 (36-84°) at final follow-up. A total of 15 complications related to implant (9), wound (2), anesthesia (2) and neurological (2) occurred in 7 patients. Among the two neurological complications, one patient sustained fall in the post-op period and reported to the emergency department with paraplegia and broken proximal screw. While other patient experienced MEP changes during index procedure. None of the patients had any neurological complications during repeated lengthening procedures. The most common cord anomalies associated with EOS in our study are TCS and SCM. Although presence of previous intraspinal anomaly does not seem to increase the incidence of neurological deficit, use of neuromonitoring is advisable for all index procedure and selected distractions. Level 4 (case series).

Chiari malformation Type I surgery in pediatric patients. Part 2: complications and the influence of comorbid disease in California, Florida, and New York.

PubMed

Greenberg, Jacob K; Olsen, Margaret A; Yarbrough, Chester K; Ladner, Travis R; Shannon, Chevis N; Piccirillo, Jay F; Anderson, Richard C E; Wellons, John C; Smyth, Matthew D; Park, Tae Sung; Limbrick, David D

2016-05-01

OBJECTIVE Chiari malformation Type I (CM-I) is a common and often debilitating pediatric neurological disease. However, efforts to guide preoperative counseling and improve outcomes research are impeded by reliance on small, single-center studies. Consequently, the objective of this study was to investigate CM-I surgical outcomes using population-level administrative billing data. METHODS The authors used Healthcare Cost and Utilization Project State Inpatient Databases (SID) to study pediatric patients undergoing surgical decompression for CM-I from 2004 to 2010 in California, Florida, and New York. They assessed the prevalence and influence of preoperative complex chronic conditions (CCC) among included patients. Outcomes included medical and surgical complications within 90 days of treatment. Multivariate logistic regression was used to identify risk factors for surgical complications. RESULTS A total of 936 pediatric CM-I surgeries were identified for the study period. Overall, 29.2% of patients were diagnosed with syringomyelia and 13.7% were diagnosed with scoliosis. Aside from syringomyelia and scoliosis, 30.3% of patients had at least 1 CCC, most commonly neuromuscular (15.2%) or congenital or genetic (8.4%) disease. Medical complications were uncommon, occurring in 2.6% of patients. By comparison, surgical complications were diagnosed in 12.7% of patients and typically included shunt-related complications (4.0%), meningitis (3.7%), and other neurosurgery-specific complications (7.4%). Major complications (e.g., stroke or myocardial infarction) occurred in 1.4% of patients. Among children with CCCs, only comorbid hydrocephalus was associated with a significantly increased risk of surgical complications (OR 4.5, 95% CI 2.5-8.1). CONCLUSIONS Approximately 1 in 8 pediatric CM-I patients experienced a surgical complication, whereas medical complications were rare. Although CCCs were common in pediatric CM-I patients, only hydrocephalus was independently associated with increased risk of surgical events. These results may inform patient counseling and guide future research efforts.

Two distinct populations of Chiari I malformation based on presence or absence of posterior fossa crowdedness on magnetic resonance imaging.

PubMed

Taylor, Davis G; Mastorakos, Panagiotis; Jane, John A; Oldfield, Edward H

2017-06-01

OBJECTIVE A subset of patients with Chiari I malformation demonstrate patent subarachnoid spaces around the cerebellum, indicating that reduced posterior fossa volume alone does not account for tonsillar descent. The authors distinguish two subsets of Chiari I malformation patients based on the degree of "posterior fossa crowdedness" on MRI. METHODS Two of the coauthors independently reviewed the preoperative MR images of 49 patients with Chiari I malformation and categorized the posterior fossa as "spacious" or "crowded." Volumetric analysis of posterior fossa structures was then performed using open-source DICOM software. The preoperative clinical and imaging features of the two groups were compared. RESULTS The posterior fossae of 25 patients were classified as spacious and 20 as crowded by both readers; 4 were incongruent. The volumes of the posterior fossa compartment, posterior fossa tissue, and hindbrain (posterior fossa tissue including herniated tonsils) were statistically similar between the patients with spacious and crowed subtypes (p = 0.33, p = 0.17, p = 0.20, respectively). However, patients in the spacious and crowded subtypes demonstrated significant differences in the ratios of posterior fossa tissue to compartment volumes as well as hindbrain to compartment volumes (p = 0.001 and p = 0.0004, respectively). The average age at surgery was 29.2 ± 19.3 years (mean ± SD) and 21.9 ± 14.9 years for spacious and crowded subtypes, respectively (p = 0.08). Syringomyelia was more prevalent in the crowded subtype (50% vs 28%, p = 0.11). CONCLUSIONS The authors' study identifies two subtypes of Chiari I malformation, crowded and spacious, that can be distinguished by MRI appearance without volumetric analysis. Earlier age at surgery and presence of syringomyelia are more common in the crowded subtype. The presence of the spacious subtype suggests that crowdedness alone cannot explain the pathogenesis of Chiari I malformation in many patients, supporting the need for further investigation.

“Stealth cranioplasty:” A novel endeavor for symptomatic adult Chiari I patients with syringomyelia: Technical note, appraisal, and philosophical considerations

PubMed Central

Rahman, Asifur; Rana, Md. Sumon; Bhandari, Paawan Bahadur; Asif, Dewan Shamsul; Uddin, Abu Naim Wakil; Obaida, Abu Saleh Mohammad Abu; Rahman, Md. Atikur; Alam, Md. Shamsul

2017-01-01

Aim and Objective: In this article, we describe a novel technique of reconstruction of posterior fossa by cranioplasty with use of preshaped titanium mesh following posterior fossa decompression (PFD) for Chiari malformation type I (CMI) with syringomyelia (SM) in symptomatic adults. Materials and Methods: Eleven patients underwent limited PFD and expansive cranioplasty with preshaped titanium mesh, what we term as “Stealth Cranioplasty” (SCP), following arachnoid preserving duraplasty (APD) and hexagonal tenting of the duraplasty with the cranioplasty (HTDC) for the management of symptomatic adult CMI with SM. All these patients had syringes extending from 3 to >10 vertebral levels. Results: Seven male and four female symptomatic CMI adult patients, between age ranges of 22 and 44 years (mean 29.45 years), presented with different neurological symptoms related to CMI and SM for 6–84 months (mean 37.09 months). All the patients underwent PFD, APD followed by SCP and HTDC and were followed up for 7–54 months (mean 35.90 months). Of 11 patients, 8 patients improved according to the Chicago Chiari Outcome Scale (CCOS) with score of 13–15 while 3 patients remained unchanged with CCOS of 12, and there was no worsening. There was no complication related to Chiari surgery in any of the patients. All the patients had good reestablishment of cisterna magna. Two patients had marked reduction of syrinx while eight patients had moderate-to-mild reduction and one patient had no change of syrinx. None of the patients needed redo surgery. Conclusion: SCP is an effective, fruitful, and cost-effective technique for the management of symptomatic adult CMI with SM. This technique has the advantages of preventing complications and recurrences in addition to the improvement of symptoms by addressing the basic pathology. PMID:29021676

Morphometric features of posterior cranial fossa are different between Chiari I malformation with and without syringomyelia.

PubMed

Yan, Huang; Han, Xiao; Jin, Mengran; Liu, Zhen; Xie, Dingding; Sha, Shifu; Qiu, Yong; Zhu, Zezhang

2016-07-01

To investigate whether the posterior cranial fossa (PCF) morphology in Chiari I malformation without syringomyelia (also called syrinx) (CMI-only) is different from that in Chiari I malformation with syrinx (CMI-S). Nineteen CMI patients without syrinx constituted the CMI-only group, whereas 48 CMI patients with syrinx were assigned to the CMI-S group. Another cohort of 40 age-matched asymptomatic adolescents was enrolled to serve as the control group. Six measurements were evaluated and compared between these three groups from T1-weighted magnetic resonance (MR) imaging, including the length of the clivus (AB), the anteroposterior diameter of the foramen magnum (BC), the length of the supraocciput (CD), the anteroposterior diameter of the posterior fossa (DA), the posterior fossa height (BE) and the clivus gradient ([Formula: see text]). The posterior cranial fossa morphology in relation to syrinx severity was also investigated. Compared to the normal controls, the AB, CD, DA, BE and [Formula: see text] were significantly larger in the CMI-S group. Similar changes in AB, CD, DA and BE were also demonstrated in the CMI-only group, while the clivus gradient ([Formula: see text]) was found to be normal when compared with the control group. A significantly decreased clivus gradient was observed in the CMI-S group as compared to CMI-only group. In addition, the clivus was significantly flattened in patients with a distended-syrinx in comparison to those with a non-distended syrinx. Small size of the posterior fossa was detected both in CMI cases with and without syrinx. The clivus gradient served as the only morphologic difference in the PCF between CMI-S and CMI-only patients and was correlated with the severity of the syrinx, may support the theory that the restricted circulation of cerebrospinal fluid at the anterior paramedial subarachnoid space contributes to the formation of a syrinx.

Use of Morphometric Mapping to Characterise Symptomatic Chiari-Like Malformation, Secondary Syringomyelia and Associated Brachycephaly in the Cavalier King Charles Spaniel

PubMed Central

Cross, Chloe; Griffiths, Sandra; McFadyen, Angus K.; Jovanovik, Jelena; Tauro, Anna; Kibar, Zoha; Driver, Colin J.; La Ragione, Roberto M.; Rusbridge, Clare

2017-01-01

Objectives To characterise the symptomatic phenotype of Chiari-like malformation (CM), secondary syringomyelia (SM) and brachycephaly in the Cavalier King Charles Spaniel using morphometric measurements on mid-sagittal Magnetic Resonance images (MRI) of the brain and craniocervical junction. Methods This retrospective study, based on a previous quantitative analysis in the Griffon Bruxellois (GB), used 24 measurements taken on 130 T1-weighted MRI of hindbrain and cervical region. Associated brachycephaly was estimated using 26 measurements, including rostral forebrain flattening and olfactory lobe rotation, on 72 T2-weighted MRI of the whole brain. Both study cohorts were divided into three groups; Control, CM pain and SM and their morphometries compared with each other. Results Fourteen significant traits were identified in the hindbrain study and nine traits in the whole brain study, six of which were similar to the GB and suggest a common aetiology. The Control cohort had the most elliptical brain (p = 0.010), least olfactory bulb rotation (p = 0.003) and a protective angle (p = 0.004) compared to the other groups. The CM pain cohort had the greatest rostral forebrain flattening (p = 0.007), shortest basioccipital (p = 0.019), but a greater distance between the atlas and basioccipital (p = 0.002) which was protective for SM. The SM cohort had two conformation anomalies depending on the severity of craniocervical junction incongruities; i) the proximity of the dens (p <0.001) ii) increased airorhynchy with a smaller, more ventrally rotated olfactory bulb (p <0.001). Both generated ‘concertina’ flexures of the brain and craniocervical junction. Conclusion Morphometric mapping provides a diagnostic tool for quantifying symptomatic CM, secondary SM and their relationship with brachycephaly. It is hypothesized that CM pain is associated with increased brachycephaly and SM can result from different combinations of abnormalities of the forebrain, caudal fossa and craniocervical junction which compromise the neural parenchyma and impede cerebrospinal fluid flow. PMID:28122014

Cerebrospinal Fluid Mechanics and Its Coupling to Cerebrovascular Dynamics

NASA Astrophysics Data System (ADS)

Linninger, Andreas A.; Tangen, Kevin; Hsu, Chih-Yang; Frim, David

2016-01-01

Cerebrospinal fluid (CSF) is not stagnant but displays fascinating oscillatory flow patterns inside the ventricular system and reversing fluid exchange between the cranial vault and spinal compartment. This review provides an overview of the current knowledge of pulsatile CSF motion. Observations contradicting classical views about its bulk production and clearance are highlighted. A clinical account of diseases of abnormal CSF flow dynamics, including hydrocephalus, syringomyelia, Chiari malformation type 1, and pseudotumor cerebri, is also given. We survey medical imaging modalities used to observe intracranial dynamics in vivo. Additionally, we assess the state of the art in predictive models of CSF dynamics. The discussion addresses open questions regarding CSF dynamics as they relate to the understanding and management of diseases.

[Cervical myelopathy from an aspect of a neurological surgeon].

PubMed

Koyama, T

1994-12-01

More than 65 per cent of intramedurally tumors and intradural extramedurally tumors were located in cervical region. They enact a cause of cervical myelopathy. A special attention should be paid to solitaly intramedurally vascular malformations, when the patients show an acute progressive myelopathy. In very rare cases of syringomyelia, intramedurally tumors such as hemangioblastoma or ependymoma could be found, so that enhanced MRI or angiography should be done if necessary. In cases of a herniated disc, cervical spondylosis and OPLL, the distance between articulo-pillar line and spinolaminal line must be measured. If the distance of both lines is near or the same, that means narrow cervical canal. In rare cases the form of the vertebral body is round. This is a rare cause of a type of narrow canal.

Clinical, radiological, and genetic similarities between patients with Chiari Type I and Type 0 malformations

PubMed Central

Markunas, Christina A.; Tubbs, R. Shane; Moftakhar, Roham; Ashley-Koch, Allison E.; Gregory, Simon G.; Oakes, W. Jerry; Speer, Marcy C.; Iskandar, Bermans J.

2012-01-01

Object Although Chiari Type I (CM-I) and Type 0 (CM-0) malformations have been previously characterized clinically and radiologically, there have been no studies focusing on the possible genetic link between these disorders. The goal of this study was to identify families in whom CM-0 and CM-I co-occurred and to further assess the similarities between these disorders. Methods Families were ascertained through a proband with CM-I. Detailed family histories were obtained to identify first-degree relatives diagnosed with CM-0. Several criteria were used to exclude individuals with acquired forms of CM-I and/or syringomyelia. Individuals were excluded with syndromic, traumatic, infectious, or tumor-related syringomyelia, as well as CM-I due to a supratentorial mass, hydrocephalus, history of cervical or cranial surgery unrelated to CM-I, or development of symptoms following placement of a lumbar shunt. Medical records and MR images were used to characterize CM-I and CM-0 individuals clinically and radiologically. Results Five families were identified in which the CM-I proband had a first-degree relative with CM-0. Further assessment of affected individuals showed similar clinical and radiological features between CM-0 and CM-I individuals, although CM-I patients in general had more severe symptoms and skull base abnormalities than their CM-0 relatives. Overall, both groups showed improvement in symptoms and/or syrinx size following craniocervical decompression surgery. Conclusions There is accumulating evidence suggesting that CM-0 and CM-I may be caused by a common underlying developmental mechanism. The data in this study are consistent with this hypothesis, showing similar clinical and radiological features between CM-0 and CM-I individuals, as well as the occurrence of both disorders within families. Familial clustering of CM-0 and CM-I suggests that these disorders may share an underlying genetic basis, although additional epigenetic and/or environmental factors are likely to play an important role in the development of CM-0 versus CM-I. PMID:22462700

Early analysis of operative management of Chiari I malformation in pediatric cystic fibrosis patients.

PubMed

Samples, Derek C; Thoms, Dewey J; Tarasiewicz, Izabela

2018-04-02

Chiari I malformation, defined as herniation of the cerebellar tonsils at least 5 mm below the foramen magnum, can result from congenital or acquired pathology. While the mechanism is not well understood, an association between Chiari I and cystic fibrosis has been described in the literature. The lifelong respiratory status management necessitated by cystic fibrosis creates a greater risk of Chiari symptomatology as well as post-operative CSF-related complications in the setting of duraplasty secondary to recurrent transient increases in intracranial pressure. We will review the literature, describe our experience with these patients, and propose bony decompression as an approach to treatment. A retrospective review of pediatric patients treated at our institution with both cystic fibrosis and Chiari I was performed. Since our first case in 2016, our department has evaluated four patients carrying that dual diagnosis. All four underwent posterior fossa decompression surgery. Two patients had incidental pathology. Two symptomatic patients exhibited headaches and/or coordination difficulty. Half of the patients had associated syringomyelia. All patients were offered posterior fossa decompression utilizing intraoperative ultrasound. All four patients underwent posterior fossa decompression without duraplasty. Average operative time was 128 min. There were no complications post-operatively. Average hospital stay was 3.8 days. Average surgical length of stay was 2.3 days. Morbidity and mortality were 0%. The longest follow-up to date is 20 months. The two asymptomatic patients remained so post-operatively. The child with headaches and imbalance had complete resolution of his symptoms after surgery, as did the toddler with headaches. Both patients with syringomyelia demonstrated significant decrease in the size of their syrinxes on imaging performed at least 3 months post-operatively. Based on the literature and our experience, we recommend considering posterior fossa decompression without duraplasty as treatment for pediatric cystic fibrosis patients with Chiari I malformation. This approach can be effective for symptomatic and prophylactic cases in this particular patient demographic because their comorbidities predispose them to Chiari pathology and symptomatology as well as certain post-operative complications.

Volume reduction of the jugular foramina in Cavalier King Charles Spaniels with syringomyelia.

PubMed

Schmidt, Martin Jürgen; Ondreka, Nele; Sauerbrey, Maren; Volk, Holger Andreas; Rummel, Christoph; Kramer, Martin

2012-09-06

Understanding the pathogenesis of the chiari-like malformation in the Cavalier King Charles Spaniel (CKCS) is incomplete, and current hypotheses do not fully explain the development of syringomyelia (SM) in the spinal cords of affected dogs. This study investigates an unconventional pathogenetic theory for the development of cerebrospinal fluid (CSF) pressure waves in the subarachnoid space in CKCS with SM, by analogy with human diseases. In children with achondroplasia the shortening of the skull base can lead to a narrowing of the jugular foramina (JF) between the cranial base synchondroses. This in turn has been reported to cause a congestion of the major venous outflow tracts of the skull and consequently to an increase in the intracranial pressure (ICP). Amongst brachycephalic dog breeds the CKCS has been identified as having an extremely short and wide braincase. A stenosis of the JF and a consequential vascular compromise in this opening could contribute to venous hypertension, raising ICP and causing CSF jets in the spinal subarachnoid space of the CKCS. In this study, JF volumes in CKCSs with and without SM were compared to assess a possible role of this pathologic mechanism in the development of SM in this breed. Computed tomography (CT) scans of 40 CKCSs > 4 years of age were used to create three-dimensional (3D) models of the skull and the JF. Weight matched groups (7-10 kg) of 20 CKCSs with SM and 20 CKCSs without SM were compared. CKCSs without SM presented significantly larger JF -volumes (median left JF: 0.0633 cm3; median right JF: 0.0703 cm3; p < 0.0001) when compared with CKCSs with SM (median left JF: 0.0382 cm3; median right JF: 0.0434 cm3; p < 0.0001). There was no significant difference between the left and right JF within each group. Bland-Altman analysis revealed excellent reproducibility of all volume measurements. A stenosis of the JF and consecutive venous congestion may explain the aetiology of CSF pressure waves in the subarachnoid space, independent of cerebellar herniation, as an additional pathogenetic factor for the development of SM in this breed.

Volume reduction of the jugular foramina in Cavalier King Charles Spaniels with syringomyelia

PubMed Central

2012-01-01

Background Understanding the pathogenesis of the chiari-like malformation in the Cavalier King Charles Spaniel (CKCS) is incomplete, and current hypotheses do not fully explain the development of syringomyelia (SM) in the spinal cords of affected dogs. This study investigates an unconventional pathogenetic theory for the development of cerebrospinal fluid (CSF) pressure waves in the subarachnoid space in CKCS with SM, by analogy with human diseases. In children with achondroplasia the shortening of the skull base can lead to a narrowing of the jugular foramina (JF) between the cranial base synchondroses. This in turn has been reported to cause a congestion of the major venous outflow tracts of the skull and consequently to an increase in the intracranial pressure (ICP). Amongst brachycephalic dog breeds the CKCS has been identified as having an extremely short and wide braincase. A stenosis of the JF and a consequential vascular compromise in this opening could contribute to venous hypertension, raising ICP and causing CSF jets in the spinal subarachnoid space of the CKCS. In this study, JF volumes in CKCSs with and without SM were compared to assess a possible role of this pathologic mechanism in the development of SM in this breed. Results Computed tomography (CT) scans of 40 CKCSs > 4 years of age were used to create three-dimensional (3D) models of the skull and the JF. Weight matched groups (7–10 kg) of 20 CKCSs with SM and 20 CKCSs without SM were compared. CKCSs without SM presented significantly larger JF -volumes (median left JF: 0.0633 cm3; median right JF: 0.0703 cm3; p < 0.0001) when compared with CKCSs with SM (median left JF: 0.0382 cm3; median right JF: 0.0434 cm3; p < 0.0001). There was no significant difference between the left and right JF within each group. Bland-Altman analysis revealed excellent reproducibility of all volume measurements. Conclusion A stenosis of the JF and consecutive venous congestion may explain the aetiology of CSF pressure waves in the subarachnoid space, independent of cerebellar herniation, as an additional pathogenetic factor for the development of SM in this breed. PMID:22954070

Posterior fossa reconstruction using titanium plate for the treatment of cerebellar ptosis after decompression for Chiari malformation.

PubMed

Udani, Vikram; Holly, Langston T; Chow, Daniel; Batzdorf, Ulrich

2014-01-01

We describe our use of a perforated titanium plate to perform a partial posterior fossa cranioplasty in the treatment of cerebellar ptosis and dural ectasia after posterior fossa decompression (PFD). Twelve patients who had undergone PFD underwent posterior fossa reconstruction using a titanium plate. Symptoms were related to either descent of the cerebellum into the decompression or to dural ectasia into the craniectomy defect. Twelve patients who had undergone large suboccipital craniectomies and who presented with persistent headaches and some with neurological symptoms related to syringomyelia, underwent reoperation with placement of a small titanium plate. Ten of 12 patients showed symptomatic improvement after reoperation. Placement of a titanium plate appears to be an effective method of treatment of cerebellar ptosis and dural ectasia after PFD for Chiari malformation. Copyright © 2014 Elsevier Inc. All rights reserved.

Neuropathic Arthropathy of the Glenohumeral Joint A Review of the Literature.

PubMed

Santiesteban, Lauren; Mollon, Brent; Zuckerman, Joseph D

2018-06-01

Neuropathic arthropathy, also known as Charcot arthropathy, is a degenerative disorder most commonly characterized by rapid destruction of the joint with extensive involvement of the bone and soft tissue. The underlying pathophysiology is thought to be due to loss of nociception (pain sensation), most frequently caused by diabetes mellitus, syphilitic myelopathy, or syringomyelia. A neuropathic shoulder is rare, with historic case series forming the bulk of the literature. The purpose of this review is to better understand the pathogenesis, clinical presentation, and management of neuropathic arthropathy of the glenohumeral joint. It should be stressed that the identification and management of the underlying etiology is paramount if the disease process is to be positively impacted. Although the mainstay of orthopedic management is non-surgical, little evidence exists to support the use of any specific therapeutic intervention. Recent literature suggests surgical reconstruction may be considered in very select patients.

Assessing the relative importance of health and conformation traits in the cavalier king Charles spaniel.

PubMed

Wijnrocx, Katrien; François, Liesbeth; Goos, Peter; Buys, Nadine; Janssens, Steven

2018-01-01

The selection of a future breeding dog is a complicated task, in which disease characteristics and different traits have to be combined and weighed against one another. Truncation selection, that is the exclusion of affected animals, may be very inefficient when selecting on a large number of traits, and may result in a reduction of the genetic diversity in a population or breed. Selection could be facilitated by the use of a selection index that combines multiple traits or breeding values into one score. This however requires a consideration of their relative value according to their economic weight, which is difficult to express in monetary units for health traits. The use of a choice experiment to derive non-market values might be a solution to this problem. This is a pilot study to assess the potential use of choice experiments to ascertain the public preference and relative importance attached to health- and conformation traits in the selection of a Cavalier King Charles spaniel. The focus was on two prevalent disorders, mitral valve disease and syringomyelia, and on several important conformation traits such as muzzle length and eye shape. Based on available prior information, a Bayesian D-optimal design approach was used to develop a choice experiment and the resulting choice sets. Every participant (breeder or owner) in the choice experiment was presented with a total of 17 choice sets, in which at most four traits could vary to reduce the cognitive burden. A total of 114 respondents participated in the choice experiment and results showed that respondents (breeders/owners) current attitudes were directed towards health (syringomyelia and mitral valve disease), followed by eye shape and level of inbreeding. This approach identifies the value breeders and owners attach to certain traits in the breeding objective. The resulting relative weights, represented as the logworths obtained from the choice experiment, could be an alternative to economic weights. They could be implemented as a weight when breeding values are available, but more study on this topic will be necessary. A challenge in this approach is to scale up the experiment with additional traits. Moreover, for other traits, the genetic parameters and correlations should be known first, in order to include them in the health selection index as well.

A genome-wide association study identifies candidate loci associated to syringomyelia secondary to Chiari-like malformation in Cavalier King Charles Spaniels.

PubMed

Ancot, Frédéric; Lemay, Philippe; Knowler, Susan P; Kennedy, Karen; Griffiths, Sandra; Cherubini, Giunio Bruto; Sykes, Jane; Mandigers, Paul J J; Rouleau, Guy A; Rusbridge, Clare; Kibar, Zoha

2018-03-22

Syringomyelia (SM) is a common condition affecting brachycephalic toy breed dogs and is characterized by the development of fluid-filled cavities within the spinal cord. It is often concurrent with a complex developmental malformation of the skull and craniocervical vertebrae called Chiari-like malformation (CM) characterized by a conformational change and overcrowding of the brain and cervical spinal cord particularly at the craniocervical junction. CM and SM have a polygenic mode of inheritance with variable penetrance. We identified six cranial T1-weighted sagittal MRI measurements that were associated to maximum transverse diameter of the syrinx cavity. Increased syrinx transverse diameter has been correlated previously with increased likelihood of behavioral signs of pain. We next conducted a whole genome association study of these traits in 65 Cavalier King Charles Spaniel (CKCS) dogs (33 controls, 32 with extreme phenotypes). Two loci on CFA22 and CFA26 were found to be significantly associated to two traits associated with a reduced volume and altered orientation of the caudal cranial fossa. Their reconstructed haplotypes defined two associated regions that harbor only two genes: PCDH17 on CFA22 and ZWINT on CFA26. PCDH17 codes for a cell adhesion molecule expressed specifically in the brain and spinal cord. ZWINT plays a role in chromosome segregation and its expression is increased with the onset of neuropathic pain. Targeted genomic sequencing of these regions identified respectively 37 and 339 SNPs with significantly associated P values. Genotyping of tagSNPs selected from these 2 candidate loci in an extended cohort of 461 CKCS (187 unaffected, 274 SM affected) identified 2 SNPs on CFA22 that were significantly associated to SM strengthening the candidacy of this locus in SM development. We identified 2 loci on CFA22 and CFA26 that contained only 2 genes, PCDH17 and ZWINT, significantly associated to two traits associated with syrinx transverse diameter. The locus on CFA22 was significantly associated to SM secondary to CM in the CKCS dog breed strengthening its candidacy for this disease. This study will provide an entry point for identification of the genetic factors predisposing to this condition and its underlying pathogenic mechanisms.

Twelve years of chiari-like malformation and syringomyelia scanning in Cavalier King Charles Spaniels in the Netherlands: Towards a more precise phenotype

PubMed Central

Van Bruggen, Leonie W. L.; Eggelmeijer, Wieteke; Noorman, Erik; Jacques, Arnold; Buys, Nadine; Janssens, Steven

2017-01-01

Chiari-like malformation (CM), syringomyelia (SM) and middle ear effusion (also called PSOM) are three conditions that frequently occur in Cavalier King Charles Spaniels (CKCS). Both CM and SM are currently screened in the Netherlands prior to breeding and are graded according to the British Veterinary Association’s Kennel Club (BVA/KC) scheme. This study evaluated the prevalence and estimated genetic parameter of CM, SM and middle ear effusion from 12 years of screening results. For SM, the classical method using the BVA/KC scheme, was compared with exact measuring of the central canal dilation. For CM, the BVA/KC scheme was compared with a more detailed scheme. Next to this the presence of microchip artifacts was assessed. 1249 screening of 1020 dogs were re-evaluated. Results indicated the presence of CM in all dogs, suggesting it has become a breed-specific characteristic. And although different grades of CM were observed, the condition did not deteriorate over time. SM was present in 39% of the dogs and a clear age effect was demonstrated, with SM increasing with age. This emphasizes the importance of screening at appropriate age, since SM can worsen with increasing age. One alternative is to promote repeated measures. The presence of middle ear effusion in this study was 19%–21% for dogs younger than 3 years, and 32%–38% for dogs older than 3 years. In as much as 60%, microchip artifacts were noticed, leading to the recommendation to place microchips in another location in breeds that are susceptible to developing SM. Finally, this study estimated the heritability of CM in this population, due to the lack of phenotypic variance, to be very low at 0.02–0.03. The heritability for SM central canal dilatation to be 0.30, compared to 0.13 for the classical BVA/KC method, using a model including the age effect and the combined effect of veterinary clinic and year of the evaluation. Genetic correlations were rather small, ranging from 0.16–0.33. As a conclusion, screening for SM and CM in the entire population should be maintained, and a selection scheme against SM should be based on estimated breeding values for the exact measurement of the central canal dilatation. PMID:28934242

Gastrointestinal problems in the handicapped child.

PubMed

Chong, S K

2001-10-01

Gastrointestinal issues are a major chronic problem in 80 to 90% of children with cerebral palsy and in children with neurodevelopmental disabilities who are at special risk of developing malnutrition because of uncoordinated swallowing, gastroesophageal reflux, and constipation. In addition to poor linear growth, there is a decrease in muscle strength and coordination, impaired cerebral function leading to decreased motivation and energy. Significant neurodevelopmental progress can be achieved with improved nutritional status. A multidisciplinary approach, with input from neurologists, gastroenterologists, nurses, occupational therapists, and dieticians, can make a major contribution to the medical wellbeing and quality of life of these children. Different neurological diseases ( eg, spinal dysraphism, syringomyelia, tethered cord syndromes) can give rise to gastrointestinal dysfunction and symptoms that may need different gastrointestinal or surgical management. The introduction of new drugs, including proton pump inhibitors and innovative endoscopic and surgical techniques in the management of gastroesophageal reflux disease and constipation also may have an impact on the treatment of neurologically handicapped children in the future.

A points-based algorithm for prognosticating clinical outcome of Chiari malformation Type I with syringomyelia: results from a predictive model analysis of 82 surgically managed adult patients.

PubMed

Thakar, Sumit; Sivaraju, Laxminadh; Jacob, Kuruthukulangara S; Arun, Aditya Atal; Aryan, Saritha; Mohan, Dilip; Sai Kiran, Narayanam Anantha; Hegde, Alangar S

2018-01-01

OBJECTIVE Although various predictors of postoperative outcome have been previously identified in patients with Chiari malformation Type I (CMI) with syringomyelia, there is no known algorithm for predicting a multifactorial outcome measure in this widely studied disorder. Using one of the largest preoperative variable arrays used so far in CMI research, the authors attempted to generate a formula for predicting postoperative outcome. METHODS Data from the clinical records of 82 symptomatic adult patients with CMI and altered hindbrain CSF flow who were managed with foramen magnum decompression, C-1 laminectomy, and duraplasty over an 8-year period were collected and analyzed. Various preoperative clinical and radiological variables in the 57 patients who formed the study cohort were assessed in a bivariate analysis to determine their ability to predict clinical outcome (as measured on the Chicago Chiari Outcome Scale [CCOS]) and the resolution of syrinx at the last follow-up. The variables that were significant in the bivariate analysis were further analyzed in a multiple linear regression analysis. Different regression models were tested, and the model with the best prediction of CCOS was identified and internally validated in a subcohort of 25 patients. RESULTS There was no correlation between CCOS score and syrinx resolution (p = 0.24) at a mean ± SD follow-up of 40.29 ± 10.36 months. Multiple linear regression analysis revealed that the presence of gait instability, obex position, and the M-line-fourth ventricle vertex (FVV) distance correlated with CCOS score, while the presence of motor deficits was associated with poor syrinx resolution (p ≤ 0.05). The algorithm generated from the regression model demonstrated good diagnostic accuracy (area under curve 0.81), with a score of more than 128 points demonstrating 100% specificity for clinical improvement (CCOS score of 11 or greater). The model had excellent reliability (κ = 0.85) and was validated with fair accuracy in the validation cohort (area under the curve 0.75). CONCLUSIONS The presence of gait imbalance and motor deficits independently predict worse clinical and radiological outcomes, respectively, after decompressive surgery for CMI with altered hindbrain CSF flow. Caudal displacement of the obex and a shorter M-line-FVV distance correlated with good CCOS scores, indicating that patients with a greater degree of hindbrain pathology respond better to surgery. The proposed points-based algorithm has good predictive value for postoperative multifactorial outcome in these patients.

Pressure wave propagation in fluid-filled co-axial elastic tubes. Part 1: Basic theory.

PubMed

Berkouk, K; Carpenter, P W; Lucey, A D

2003-12-01

Our work is motivated by ideas about the pathogenesis of syringomyelia. This is a serious disease characterized by the appearance of longitudinal cavities within the spinal cord. Its causes are unknown, but pressure propagation is probably implicated. We have developed an inviscid theory for the propagation of pressure waves in co-axial, fluid-filled, elastic tubes. This is intended as a simple model of the intraspinal cerebrospinal-fluid system. Our approach is based on the classic theory for the propagation of longitudinal waves in single, fluid-filled, elastic tubes. We show that for small-amplitude waves the governing equations reduce to the classic wave equation. The wave speed is found to be a strong function of the ratio of the tubes' cross-sectional areas. It is found that the leading edge of a transmural pressure pulse tends to generate compressive waves with converging wave fronts. Consequently, the leading edge of the pressure pulse steepens to form a shock-like elastic jump. A weakly nonlinear theory is developed for such an elastic jump.

Anophthalmia-plus syndrome: a clinical report and review of the literature.

PubMed

Makhoul, Imad R; Soudack, Michalle; Kochavi, Orna; Guilburd, Joseph N; Maimon, Shimon; Gershoni-Baruch, Ruth

2007-01-01

We describe a term male infant of healthy non-consanguineous parents, born with congenital malformations, including bilateral cleft palate and lip, mild microphthalmia with iris coloboma and glaucoma of the right eye, and blepharophimosis with severe microphthalmia of the left eye. Spine radiograph and MRI showed first sacral hemivertebra with spina bifida, and agenesis of the 2nd, 3rd, 4th, and 5th sacral vertebrae and coccyx. Spine MRI showed caudal tethering of spinal cord at L(3) level, filum terminalis lipoma and a syringomyelia. Brain ultrasound and MRI showed hypoplasia of corpus callosum with mild dilatation of the lateral ventricles. Orbital MRI showed bilateral microphthalmia-distorted small left eyeball with posteriorly located lens, and a split vitreous body in the right eye, suggestive of primary hyperplastic vitreous. The karyotype was normal. Summary of the findings in nine cases (our case and eight published cases) support the notion that anophthalmia-plus syndrome (APS) is a distinct syndrome. Gene locus of APS is yet to be identified. (c) 2006 Wiley-Liss, Inc.

Large Amplitude Cortical Evoked Potentials in Nonepileptic Patients. Reviving an Old Neurophysiologic Tool to Help Detect CNS Pathology.

PubMed

Martín-Palomeque, Guillermo; Castro-Ortiz, Antonio; Pamplona-Valenzuela, Pilar; Saiz-Sepúlveda, Miguel Á; Cabañes-Martínez, Lidia; López, Jaime R

2017-01-01

Although large amplitude evoked potentials (EPs) are typically associated with progressive myoclonic epilepsy patients, giant EPs imply central nervous system (CNS) hyperexcitability and can be seen in various nonepileptic disorders. We performed a retrospective chart review including history, physical examination, imaging and diagnostic studies of nonepileptic patients with large amplitude somatosensory evoked potentials (SSEPs) and visual evoked potentials (VEPs) during 2007 to 2013. Large amplitude EPs were defined as follows: VEPs (N75-P100) >18 μV; and SSEPs (N20-P25) >6.4 μV. Recording montage for VEPs was Oz-Cz and SSEPs C3'/C4'-Fz. Fifty-two patients (33 females, 19 males; age range, 9-90 years) were identified. No CNS pathology was detected in 7 patients. All remaining patients were diagnosed with new CNS disorders including: vascular (37%); myelopathies (13%); demyelinating (11%); space occupying lesions (8.7%); syringomyelia (8.7%); hydrocephalus (6.5%); Vitamin B-12 deficiency (4.3%); multiple system atrophy (4.3%); and toxins (2.2%). This study supports the notion that large amplitude EP implies CNS hyperexcitability and CNS disease. These results confirm the utility of EP studies in patients with suspected CNS pathology.

Contemporary occurrence of hydrocephalus and Chiari I malformation in sagittal craniosynostosis. Case report and review of the literature.

PubMed

Sgulò, Francesco Giovanni; Spennato, Pietro; Aliberti, Ferdinando; Di Martino, Giuliana; Cascone, Daniele; Cinalli, Giuseppe

2017-01-01

Chiari malformation type I (CM-I) and hydrocephalus are often associated with complex craniosynostosis. On the contrary, their simultaneous occurrence in monosutural synostosis is extremely rare. The pathophysiological hypothesis is that they may alter posterior fossa growth and lead to cerebellar tonsil herniation also without skull base primary involvement. Hydrocephalus is multifactorial and may be secondary to fourth ventricle outlet obstruction. The management of these cases is quite complex and not well defined. Cranial vault remodeling should be the only treatment when CM-I is asymptomatic and not related to syringomyelia. Suboccipital decompression should be reserved only in complicated CM-I, usually as a second surgical step following the correction of the supratentorial deformity. In our opinion, the associated hydrocephalus should be treated first in order to normalize intracranial hypertension before opening the cranial sutures. We report the case of a 26-month-old child that presented with sagittal craniosynostosis, hydrocephalus, and CM-I. He was managed by performing endoscopic third ventriculostomy (ETV) first and cranial vault remodeling thereafter. Clinico-radiological outcome was very satisfying. Concerning literature is reviewed; physiopathology and surgical management are discussed.

Automated single-trial assessment of laser-evoked potentials as an objective functional diagnostic tool for the nociceptive system.

PubMed

Hatem, S M; Hu, L; Ragé, M; Gierasimowicz, A; Plaghki, L; Bouhassira, D; Attal, N; Iannetti, G D; Mouraux, A

2012-12-01

To assess the clinical usefulness of an automated analysis of event-related potentials (ERPs). Nociceptive laser-evoked potentials (LEPs) and non-nociceptive somatosensory electrically-evoked potentials (SEPs) were recorded in 37 patients with syringomyelia and 21 controls. LEP and SEP peak amplitudes and latencies were estimated using a single-trial automated approach based on time-frequency wavelet filtering and multiple linear regression, as well as a conventional approach based on visual inspection. The amplitudes and latencies of normal and abnormal LEP and SEP peaks were identified reliably using both approaches, with similar sensitivity and specificity. Because the automated approach provided an unbiased solution to account for average waveforms where no ERP could be identified visually, it revealed significant differences between patients and controls that were not revealed using the visual approach. The automated analysis of ERPs characterized reliably and objectively LEP and SEP waveforms in patients. The automated single-trial analysis can be used to characterize normal and abnormal ERPs with a similar sensitivity and specificity as visual inspection. While this does not justify its use in a routine clinical setting, the technique could be useful to avoid observer-dependent biases in clinical research. Copyright © 2012 International Federation of Clinical Neurophysiology. Published by Elsevier Ireland Ltd. All rights reserved.

George Marinesco in the Constellation of Modern Neuroscience

PubMed Central

Opris, Ioan; Nestianu, Valeriu S.; Nestianu, Adrian; Bilteanu, Liviu; Ciurea, Jean

2017-01-01

George Marinesco is the founder of Romanian School of Neurology and one of the most remarkable neuroscientists of the last century. He was the pupil of Jean-Martin Charcot in Salpêtrière Hospital in Paris, France, but visited many other neurological centers where he met the entire constellation of neurologists of his time, including Camillo Golgi and Santiago Ramón y Cajal. The last made the preface of Nervous Cell, written in French by Marinesco. The original title was “La Cellule Nerveuse” and is considered even now a basic reference book for specialists in the field. He was a refined clinical observer with an integrative approach, as could be seen from the multitude of his discoveries. The descriptions of the succulent hand in syringomyelia, senile plaque in old subjects, palmar jaw reflex known as Marinesco-Radovici sign, or the application of cinematography in medicine are some of his important contributions. He was the first who described changes of locus niger in a patient affected by tuberculosis, as a possible cause in Parkinson disease. Before modern genetics, Marinesco and Sjögren described a rare and complex syndrome bearing their names. He was a hardworking man, focused on his scientific research, did not accepted flattering of others and was a great fighter against the injustice of the time. PMID:29317856

George Marinesco in the Constellation of Modern Neuroscience.

PubMed

Opris, Ioan; Nestianu, Valeriu S; Nestianu, Adrian; Bilteanu, Liviu; Ciurea, Jean

2017-01-01

George Marinesco is the founder of Romanian School of Neurology and one of the most remarkable neuroscientists of the last century. He was the pupil of Jean-Martin Charcot in Salpêtrière Hospital in Paris, France, but visited many other neurological centers where he met the entire constellation of neurologists of his time, including Camillo Golgi and Santiago Ramón y Cajal. The last made the preface of Nervous Cell, written in French by Marinesco. The original title was "La Cellule Nerveuse" and is considered even now a basic reference book for specialists in the field. He was a refined clinical observer with an integrative approach, as could be seen from the multitude of his discoveries. The descriptions of the succulent hand in syringomyelia, senile plaque in old subjects, palmar jaw reflex known as Marinesco-Radovici sign, or the application of cinematography in medicine are some of his important contributions. He was the first who described changes of locus niger in a patient affected by tuberculosis, as a possible cause in Parkinson disease. Before modern genetics, Marinesco and Sjögren described a rare and complex syndrome bearing their names. He was a hardworking man, focused on his scientific research, did not accepted flattering of others and was a great fighter against the injustice of the time.

Association of Chiari malformation and vitamin B12 deficit in a family.

PubMed

Welsch, Melanie; Antes, Sebastian; Kiefer, Michael; Meyer, Sascha; Eymann, Regina

2013-07-01

A clear etiology of Chiari malformation is still lacking. Some associations between this disorder and genetical variations have been reported. Documented cases of familial Chiari malformation in three consecutive generations are rare. Furthermore, an association of Chiari disorder and vitamin B12 deficit has rarely been described in literature. In this study, three generations of a family suffering from Chiari 1 or Chiari 0 malformation have been examined with MRI, clinically and laboratory (hemograms). Chiari malformation could be confirmed in all presented patients: While the F2 generation (children: 1 × ♀, 1 × ♂) and the female F1 generation (mother and sister of mother) suffered from Chiari type 1, the male F0 generation showed Chiari 0 malformation. F0 and F1 generation further presented with syringomyelia (F0: C4-D1; F1: C4-D2). All patients except the grandfather (F0) underwent surgical posterior fossa decompression to relive successfully cerebellar and hydrocephalus associated progressing clinical symptoms. The hemograms of generation 1 and 2 revealed familial vitamin B12 deficit. A hereditary component is discussed in Chiari malformation, yet proved etiology is still lacking. As folic acid plays an important role in the development of the neural tube, vitamin B12 deficit might have some impact on the development of Chiari malformations.

Two cases of sarcoidosis presenting as longitudinally extensive transverse myelitis.

PubMed

Scott, Amanda Mary; Yinh, Janeth; McAlindon, Timothy; Kalish, Robert

2018-05-17

Neurosarcoidosis is uncommon with an incidence of approximately 5 to 15%. Central nervous system involvement can be divided into brain and spinal cord neurosarcoidosis. Spinal cord sarcoidosis is extremely rare, occurring in less than 1% of all sarcoidosis cases. Its manifestations may include cauda equina syndrome, radiculopathy, syringomyelia, cord atrophy, arachnoiditis, and myelopathy or transverse myelitis. We highlight two cases of spinal cord sarcoidosis, each presenting with longitudinally extensive transverse myelitis, that demonstrate the dilemmas that physicians face with regard to diagnosis and treatment. Given its rarity and the diversity of possible manifestations, establishing the diagnosis of spinal cord sarcoidosis is often very difficult. Extensive evaluation must be conducted to rule out primary neurologic, primary rheumatologic, infectious, and neoplastic diseases. MRI often demonstrates hyperintensity on T2-weighted images and enhancement following gadolinium administration. CSF analysis most consistently shows a lymphocytic pleocytosis and elevated proteins. While these less invasive investigations may be helpful, the gold standard for diagnosis is biopsy of neurologic or non-neurologic tissue confirming the presence of non-caseating granulomas. Evidence-based guidelines for the treatment of transverse myelitis secondary to sarcoidosis are lacking due to its rarity; therefore, therapy is based on expert and anecdotal experience and usually consists of high doses of steroids in combination with various immunosuppressive agents. The use of infliximab in particular appears promising, but there is a need for further investigation into the ideal treatment regimen.

Peripheral neuropathy in Tangier disease: A literature review and assessment.

PubMed

Mercan, Metin; Yayla, Vildan; Altinay, Serdar; Seyhan, Serhat

2018-06-01

Tangier disease (TD) (OMIM#205400) is a rare cause of inherited metabolic neuropathies characterized by marked deficiency of high-density lipoproteins and accumulation of cholesterol esters in various tissue resulting from reverse cholesterol transport deficiency. We report a case of a patient with TD with multifocal demyelinating neuropathy with conduction block who presents with winging scapula, tongue, and asymmetric extremity weakness. We also present a review of all studies published from 1960 to 2017 regarding peripheral neuropathy in TD. Our search identified 54 patients with TD with peripheral neuropathy. Syringomyelia-like neuropathy subtype (52.4%) was more frequent than multifocal sensorial and motor neuropathy subtype (26.2%), focal neuropathy subtype (19.1%), and distal symmetric polyneuropathy subtype (2.4%). Splenomegaly was the most common (40.7%) clinical manifestation in these patients. The pattern of electrodiagnostic abnormalities are: (1) demyelinating abnormalities were more predominant in the upper extremities than in the lower extremities and (2) slowing of motor nerve conduction was more prominent in the intermediate segment than in distal nerve segments. The sural-sparing pattern was present in 34.6% and conduction block was present in 11.5% of the patients. Our literature review and our case showed the clinical spectrum of TD neuropathy is quite wide and that it should be considered in the differential diagnosis of non-uniform demyelinating neuropathies. © 2018 Peripheral Nerve Society.

Childhood angular kyphosis: a plea for involvement of the pediatric neurosurgeon.

PubMed

Cornips, E; Koudijs, S; Vles, J; van Rhijn, L

2017-06-01

Childhood angular kyphosis is rare, as most children are affected by a mixed kyphotic and scoliotic deformity. Published series involving a mix of kyphosis and kyphoscoliosis, pediatric and adult, congenital and acquired cases are almost exclusively authored by orthopedic surgeons, suggesting that (pediatric) neurosurgeons are not involved. We present five cases that illustrate the spectrum of angular kyphosis, and these were treated by a multidisciplinary team including child neurologist, orthopedic surgeon, and pediatric neurosurgeon as complementary partners. Angular kyphosis is a cosmetic problem but above all a serious threat to the spinal cord and as such to the child's ambulatory, sphincter, and genito-urinary functions. Spinal cord stretch over the internal kyphosis may cause pain and/or neurological deficit, often accompanied by myelomalacia or even segmental cord atrophy. Spinal cord function may be additionally affected by associated disorders such as syringomyelia or tethered cord, an orthopedic surgeon may be less familiar with. The decision when and how to proceed surgically should be made by a multidisciplinary team, including a pediatric neurosurgeon who actively participates in the operation and helps to safely achieve adequate spinal cord decompression and stabilization. Childhood angular kyphosis is a complex, heterogeneous disorder that should be managed by a multidisciplinary team in specialized pediatric spine centers. While every case is truly unique, the spinal cord is always at risk, especially during decompression, stabilization, and eventual correction of deformity. Pediatric neurosurgeons have an important role to play in preoperative work-up, actual operation, and follow-up.

Posterior fossa volume and response to suboccipital decompression in patients with Chiari I malformation.

PubMed

Badie, B; Mendoza, D; Batzdorf, U

1995-08-01

Smaller posterior fossa (PF) volume has been suggested to be one of the mechanisms responsible for tonsillar herniation through the foramen magnum in patients with Chiari I malformation (CM I). Although previous radiological analyses of the cranial anatomy have suggested a smaller PF volume in patients with CM I, the relationship of the PF volume to decompressive surgery has not been reported. We have measured the ratio of PF volume to supratentorial volume (PF ratio [PFR]) in 20 patients with CM I and 20 control patients by retrospectively studying their magnetic resonance images with a computerized image analyzer. The mean PFR in patients with CM I (with or without syringomyelia) was significantly smaller than for those in the control group (15.6 +/- 1.9 versus 17.5 +/- 1.2, P = 0.0008). Although PFR did not correlate with the extent of tonsillar herniation in patients with CM I, it did directly correlate with their age, i.e., younger patients with CM I (but not control patients) had smaller PFRs. All but three patients responded both clinically and radiographically to decompressive surgery. Those patients who did not benefit from surgical intervention had normal PFRs. We conclude that: 1) PFRs are smaller in most patients with CM I; 2) a smaller PF may be a primary cause of tonsillar herniation; 3) patients with CM I who have smaller PFRs tend to develop symptoms earlier than those with normal values; 4) patients with smaller PFRs tend to respond better to suboccipital decompression.

Georges Marinesco (1863-1938): neurologist, neurohistologist and neuropathologist.

PubMed

Catala, M; Poirier, J

2012-01-01

Gheorghe Marinescu (Georges Marinesco, in French) is a Romanian physician, founder of the School of Neurology in that country. He begins his medical studies in Bucharest, then has the opportunity to reach Paris and join the School of Neurology in La Salpêtrière Hospital, lead by Jean-Martin Charcot. This trip will forever imprint the mind of Marinescu, a great friend of France, a respectful student of Charcot and a friendly colleague of many Parisian neurologists. Marinescu's works are multiple and very important. He describes the succulent hand in syringomyelia and the palmar-jaw reflex. Marinescu is also one of the first to use the cinema for medical purposes. His work as an anatomo-clinician, a method developed by Charcot, is important. We denote the description of the locus niger affected by tuberculosis in a case of parkinsonism (this description paving Etienne Brissaud's way to highlight the anatomical origin of Parkinson's disease), the original clinical description of Marinesco-Sjögren syndrome, and that of medullomyoblastoma. Marinescu is also a famous neurocytologist as evidenced by his work, La Cellule Nerveuse, published in 1909. The first volume of the book is devoted to the aspects of the normal nervous tissue: the neurofibrillar network, the chromatophilic elements, and the paranucleolar corpuscles (now known as Marinescu's bodies). The second volume of the book is almost related to features revealed by experimental lesions: chromatolysis and neuronophagia. Furthermore, Marinescu describes with Oscar Blocq, small nodules than are now regarded as the first description of senile plaques.

Neurological Surgery at the National Institutes of Health

PubMed Central

Mehta, Gautam U.; Heiss, John D.; Park, John K.; Asthagiri, Ashok R.; Lonser, Russell R.

2010-01-01

The Surgical Neurology Branch (SNB) in the intramural program of the National Institute of Neurological Disorders and Stroke at the National Institutes of Health has been a unique setting for academic neurosurgery for nearly 60 years. Every patient evaluated and treated in the SNB is enrolled in a clinical research protocol, which underscores a singular focus on advancing neurosurgical research and patient care. Since the inception of the SNB, this research effort has been driven by dedicated clinician-investigators and basic scientists including Maitland Baldwin, Igor Klatzo, John M. Van Buren, Ayub K. Ommaya, Richard J. Youle and Edward H. Oldfield. These and other SNB investigators have studied and advanced treatment of a number of neuropathologic processes including delineation of differences between cytotoxic and vasogenic edema, head injury, Cushing’s disease, the effects of vascular endothelial growth factor in nervous system tissues, tumor suppressor syndromes, the pathophysiology of syringomyelia, mechanisms underlying cerebral vasospasm after subarachnoid hemorrhage, spinal arteriovenous malformations, mechanisms of cell death and drug delivery. Currently, SNB efforts are focused on central nervous system drug-delivery, the natural history of familial tumor syndromes, functional neurosurgery, epilepsy, vasospasm and development of chemotherapeutics for malignant glioma. Throughout its history, the SNB has also been dedicated to training neurosurgeon clinician-investigators; 22 previous fellows/staff have become Chairs of their respective neurosurgical departments. Recently, the commitment to training future neurosurgeon clinician-investigators has been further defined with the development of a residency-training program in neurological surgery approved in 2010. PMID:21278842

Subdural Fluid Collection and Hydrocephalus After Foramen Magnum Decompression for Chiari Malformation Type I: Management Algorithm of a Rare Complication.

PubMed

Rossini, Zefferino; Milani, Davide; Costa, Francesco; Castellani, Carlotta; Lasio, Giovanni; Fornari, Maurizio

2017-10-01

Chiari malformation type I is a hindbrain abnormality characterized by descent of the cerebellar tonsils beneath the foramen magnum, frequently associated with symptoms or brainstem compression, impaired cerebrospinal fluid circulation, and syringomyelia. Foramen magnum decompression represents the most common way of treatment. Rarely, subdural fluid collection and hydrocephalus represent postoperative adverse events. The treatment of this complication is still debated, and physicians are sometimes uncertain when to perform diversion surgery and when to perform more conservative management. We report an unusual occurrence of subdural fluid collection and hydrocephalus that developed in a 23-year-old patient after foramen magnum decompression for Chiari malformation type I. Following a management protocol, based on a step-by-step approach, from conservative therapy to diversion surgery, the patient was managed with urgent external ventricular drainage, and then with conservative management and wound revision. Because of the rarity of this adverse event, previous case reports differ about the form of treatment. In future cases, finding clinical and radiologic features to identify risk factors that are useful in predicting if the patient will benefit from conservative management or will need to undergo diversion surgery is only possible if a uniform form of treatment is used. Therefore, we believe that a management algorithm based on a step-by-step approach will reduce the use of invasive therapies and help to create a standard of care. Copyright © 2017 Elsevier Inc. All rights reserved.

[Papillary oedema revealing Arnold Chiari malformation type 1: about a case].

PubMed

Imane, Mouhoub; Asmae, Maadane; Toufik, Ramdani; Rachid, Sekhsoukh

2016-01-01

Arnold Chiari malformation type 1 is defined as a herniation of the cerebellar tonsils into the foramen magnum of more than 5 mm. Symptoms are most commonly dominated by occipital headache, torticollis and sometimes swallowing disorders. Ophthalmologically abnormal convergences, oculomotor palsy and diplopia are the main clinical signs. We report the case of a 9 year old child, who presented with visual loss evolving since 6 months. Ophthalmologic examination showed visual acuity of 4/10 in both eyes, retained ocular motility and rotational nystagmus. The examination of the anterior segment of the eye showed megalocornea with no evidence of goniodysgenesis, iridodonesis associated with atrophy of the dilator muscle and microcoria with lazy photomotor reflex. Normal intraocular pressure was 14 mmHg. Ocular fundus examination, despite difficulties in performing it, objectified bilateral papilledema (stage II). General physical examination showed torticollis, scoliosis and a tetra-pyramidal syndrome. MRI showed Chiari malformation type I associated with hydrocephalus and syringomyelia. Neurosurgical intervention based on internal CSF drainage with occipitocervical osteo-dural decompression was proposed. The evolution was favorable with regression of clinical signs. Ophthalmologically, there was a regression of papilledema but visual acuity remained stationary. The occurrence of papilledema associated with Chiari malformation type 1 is rare, it has been only reported in 2% of symptomatic patients. Its pathophysiology is still poorly understood. The originality of our study consists in the association of cerebellar malformations with ocular malformations including megalocornea and microcoria which make ophthalmologic examination more difficult to perform.

Spontaneous C1 anterior arch fracture as a postoperative complication of foramen magnum decompression for Chiari malformation type 1

PubMed Central

Hirano, Yoshitaka; Sugawara, Atsushi; Mizuno, Junichi; Takeda, Masaaki; Watanabe, Kazuo; Ogasawara, Kuniaki

2011-01-01

Background: C1 fracture accounts for 2% of all spinal column injuries and 10% of cervical spine fractures, and is most frequently caused by motor vehicle accidents and falls. We present a rare case of C1 anterior arch fracture following standard foramen magnum decompression for Chiari malformation type 1. Case Description: A 63-year-old man underwent standard foramen magnum decompression (suboccipital craniectomy and C1 laminectomy) under a diagnosis of Chiari malformation type 1 with syringomyelia in June 2009. The postoperative course was uneventful until the patient noticed progressive posterior cervical pain 5 months after the operation. Computed tomography of the upper cervical spine obtained 7 months after the operation revealed left C1 anterior arch fracture. The patient was referred to our hospital at the end of January 2010 and C1–C2 posterior fusion with C1 lateral mass screws and C2 laminar screws was carried out in March 2010. Complete pain relief was achieved immediately after the second operation, and the patient resumed his daily activities. Conclusion: Anterior atlas fracture following foramen magnum decompression for Chiari malformation type 1 is very rare, but C1 laminectomy carries the risk of anterior arch fracture. Neurosurgeons should recognize that fracture of the atlas, which commonly results from an axial loading force, can occur in the postoperative period in patients with Chiari malformation. PMID:22059133

Rostral cranial fossa as a site for cerebrospinal fluid drainage - volumetric studies in dog breeds of different size and morphotype.

PubMed

Sokołowski, Wojciech; Czubaj, Norbert; Skibniewski, Michał; Barszcz, Karolina; Kupczyńska, Marta; Kinda, Wojciech; Kiełbowicz, Zdzisław

2018-05-18

Hydrocephalus is a multifactorial condition, whose aetiology is not fully understood. Congenital hydrocephalus frequently occurs in small and brachycephalic dog breeds. Although it is widely accepted that the cribriform plate located in the rostral cranial fossa (RCF) is a site of cerebrospinal fluid (CSF) drainage, the RCF has not been studied extensively. Literature reports indicate that a decreased caudal cranial fossa (CCF) volume in the course of the Chiari-like malformation may obstruct CSF circulation. We hypothesised that morphological diversity among different breeds in the volume of the RCF may affect CSF circulation. The aim of the study was to carry out a volumetric analysis of the RCF and the cranial cavity and to determine the ratio between them in dog breeds of different size and morphotype. We performed computed tomography (CT) morphometric analysis of the RCF compartment by obtaining volume measurements from the transverse and reformatted sagittal and dorsal planes. The rostral cranial fossa percentage - volume of the rostral cranial fossa/volume of cranial cavity × 100 (volRCF/volCC × 100) was lower in small and brachycephalic dog breeds than in the other dogs. A reduced RCF volume was detected in small and brachycephalic dog breeds, some of which are predisposed to congenital hydrocephalus. This may lead to overcrowding of brain parenchyma in the RCF and may impede CSF circulation. Our observations may be useful for future studies focusing on the causes and new therapies to treat conditions such as hydrocephalus and syringomyelia.

Microsurgical Resection of Spinal Cord Hemangioblastoma: 2-Dimensional Operative Video.

PubMed

Pojskic, Mirza; Arnautovic, Kenan I

2018-05-18

This video demonstrates microsurgical resection of spinal cord hemangioblastoma. Hemangioblastomas are rare, benign, highly vascularized tumors classified as grade I according to World Health Organization classification systems. About 3% of all intramedullary tumors are hemangioblastomas.1,2 Spinal cord hemangioblastomas are either sporadic3,4 or manifestations of von Hippel-Lindau (VHL) disease in 20% to 45% of patients.5,6 A 30-year-old male presented with sudden onset urinary incontinence. Magnetic resonance imaging showed contrast enhancing intramedullary tumor with adjacent cyst in T11, and syringomyelia extending to C1. Surgical resection followed rules that apply to resection of arteriovascular malformations: coagulation of arterial feeders precedes the coagulation of the draining vein, which is preserved until the end of surgery.2,4,5,7,8 First, posterior midline myelotomy was performed and the tumor cyst was drained in order to develop a dissection plane. Following this, we continuously separated dorsal nerve roots from the tumor nodule using microsurgical technique. The key step in tumor resection is devascularization of the tumor, achievable in 2 ways.2,7,9-13 The circumferential detachment of the normal pia from the tumor pia is crucial in developing a plane of dissection. The coagulation and division of arterial feeders while preserving the drainage vein further devascularizes the tumor. Once the tumor mural nodule was detached from the spinal cord, the drainage vein was coagulated last and the tumor was removed. The patient fully recovered from his incontinence and was neurologically intact. Screening for VHL disease was negative. Written consent was obtained directly from the patient.

Endoscopic third ventriculostomy

PubMed Central

Yadav, Yad Ram; Parihar, Vijay; Pande, Sonjjay; Namdev, Hemant; Agarwal, Moneet

2012-01-01

Endoscopic third ventriculostomy (ETV) is considered as a treatment of choice for obstructive hydrocephalus. It is indicated in hydrocephalus secondary to congenital aqueductal stenosis, posterior third ventricle tumor, cerebellar infarct, Dandy-Walker malformation, vein of Galen aneurism, syringomyelia with or without Chiari malformation type I, intraventricular hematoma, post infective, normal pressure hydrocephalus, myelomeningocele, multiloculated hydrocephalus, encephalocele, posterior fossa tumor and craniosynostosis. It is also indicated in block shunt or slit ventricle syndrome. Proper Pre-operative imaging for detailed assessment of the posterior communicating arteries distance from mid line, presence or absence of Liliequist membrane or other membranes, located in the prepontine cistern is useful. Measurement of lumbar elastance and resistance can predict patency of cranial subarachnoid space and complex hydrocephalus, which decides an ultimate outcome. Water jet dissection is an effective technique of ETV in thick floor. Ultrasonic contact probe can be useful in selected patients. Intra-operative ventriculo-stomography could help in confirming the adequacy of endoscopic procedure, thereby facilitating the need for shunt. Intraoperative observations of the patent aqueduct and prepontine cistern scarring are predictors of the risk of ETV failure. Such patients may be considered for shunt surgery. Magnetic resonance ventriculography and cine phase contrast magnetic resonance imaging are effective in assessing subarachnoid space and stoma patency after ETV. Proper case selection, post-operative care including monitoring of ICP and need for external ventricular drain, repeated lumbar puncture and CSF drainage, Ommaya reservoir in selected patients could help to increase success rate and reduce complications. Most of the complications develop in an early post-operative, but fatal complications can develop late which indicate an importance of long term follow up. PMID:22865970

Pregnancy in spinal cord-injured women, a cohort study of 37 pregnancies in 25 women.

PubMed

Le Liepvre, H; Dinh, A; Idiard-Chamois, B; Chartier-Kastler, E; Phé, V; Even, A; Robain, G; Denys, P

2017-02-01

A retrospective observational study. To describe specificities of pregnancy in a traumatic spinal cord-injured (SCI) population managed by a coordinated medical care team involving physical medicine and rehabilitation (PMR) physicians, urologists, infectious diseases' physicians, obstetricians and anaesthesiologists. NeuroUrology Department in a University Hospital, France. All consecutive SCI pregnant women managed between 2001 and 2014 were included. A preconceptional consultation was proposed whenever possible. Obstetrical and urological outcomes, delivery mode and complications were reported. Overall, thirty-seven pregnancies in 25 women, of a mean age of 32±4 years, were included. Thirty-five children were born alive (three miscarriages, a twin pregnancy) without complications except for a case of neonatal respiratory distress in premature twins born at 33 weeks. The mean birth weight was 2979±599 g. Twenty-one (57%) pregnancies benefited from preconceptional care. A weekly oral cyclic antibiotic programme was prescribed in 28 (75%) pregnancies. The main complications during pregnancy included pyelonephritis (30%), lower urinary tract infections (UTI) (32%), pressure sores (8.8%) and prematurity (12% deliveries before 37 weeks, with only one delivery before 36 weeks). Two patients suffered from autonomic dysreflexia, one with serious complication (brain haematoma). Caesarean sections were performed for 68% of deliveries (23/34) to prevent syringomyelia deterioration (n=10), stress urinary incontinence aggravation (n=3) or for obstetrical reasons (n=7). Mothers' and infants' outcomes were satisfying after pregnancy in SCI women, but required many adjustments. Pregnancy must be prepared by a preconceptional consultation, and managed by a multidisciplinary team involving specialists of neurological disability and pregnancy.

Risk factors for pressure sores in adult patients with myelomeningocele – a questionnaire-based study

PubMed Central

Plaum, Pål-Erik; Riemer, Gunnar; Frøslie, Kathrine Frey

2006-01-01

Background Myelomeningocele (MMC) is a part of a complex neural tube defect and a disorder of the cerebrospinal fluid system. Pressure sores are a frequent complication for patients with MMC. Little is known about the risk factors for pressure sores in adults with MMC. The aim of this study was to investigate an association between the presence of pressure sores and other patient characteristics, in order to develop an improved strategy for the management of sores. Methods A structured questionnaire regarding sores, medical condition, function and living factors was designed and sent to the 193 patients with MMC registered in the year 2003 at TRS, a National Centre for Rare Disorders in Norway. Results Out of 193 total, 87 patients participated and 71 patients (82%) reported sores; 26 (30%) at the time of the interview and 45 (52%) during the last 5 years. Sores were mostly localized on toes and feet and occurred exclusively in regions with reduced or missing sensibility. A significant association was found between sores and memory deficit (p = 0.02), Arnold Chiari malformation (p = 0.02) and a record of previous sores (p = 0.004). Sores were not significantly associated with hydrocephalus, syringomyelia, nutrition, body mass index, smoking, physical activity, employment or living together with other persons. Some patients (18, 21%) reported skin inspection by others and the remainder relied on self-inspection. Conclusion Patients with sensory deficit, memory problems, and Arnold Chiari malformation had a higher risk of having pressure sores. This patient group needs improved skin inspection routines and sore treatment. PMID:17196099

Risk factors for pressure sores in adult patients with myelomeningocele--a questionnaire-based study.

PubMed

Plaum, Pål-Erik; Riemer, Gunnar; Frøslie, Kathrine Frey

2006-12-29

Myelomeningocele (MMC) is a part of a complex neural tube defect and a disorder of the cerebrospinal fluid system. Pressure sores are a frequent complication for patients with MMC. Little is known about the risk factors for pressure sores in adults with MMC. The aim of this study was to investigate an association between the presence of pressure sores and other patient characteristics, in order to develop an improved strategy for the management of sores. A structured questionnaire regarding sores, medical condition, function and living factors was designed and sent to the 193 patients with MMC registered in the year 2003 at TRS, a National Centre for Rare Disorders in Norway. Out of 193 total, 87 patients participated and 71 patients (82%) reported sores; 26 (30%) at the time of the interview and 45 (52%) during the last 5 years. Sores were mostly localized on toes and feet and occurred exclusively in regions with reduced or missing sensibility. A significant association was found between sores and memory deficit (p = 0.02), Arnold Chiari malformation (p = 0.02) and a record of previous sores (p = 0.004). Sores were not significantly associated with hydrocephalus, syringomyelia, nutrition, body mass index, smoking, physical activity, employment or living together with other persons. Some patients (18, 21%) reported skin inspection by others and the remainder relied on self-inspection. Patients with sensory deficit, memory problems, and Arnold Chiari malformation had a higher risk of having pressure sores. This patient group needs improved skin inspection routines and sore treatment.

Stereological and Morphometric Analysis of MRI Chiari Malformation Type-1

PubMed Central

Alkoç, Ozan Alper; Songur, Ahmet; Eser, Olcay; Toktas, Muhsin; Esi, Ertap; Haktanir, Alpay

2015-01-01

Objective In this study, we aimed to investigate the underlying ethiological factors in chiari malformation (CM) type-I (CMI) via performing volumetric and morphometric length-angle measurements. Methods A total of 66 individuals [33 patients (20-65 years) with CMI and 33 control subjects] were included in this study. In sagittal MR images, tonsillar herniation length and concurrent anomalies were evaluated. Supratentorial, infratentorial, and total intracranial volumes were measured using Cavalieri method. Various cranial distances and angles were used to evaluate the platybasia and posterior cranial fossa (PCF) development. Results Tonsillar herniation length was measured 9.09±3.39 mm below foramen magnum in CM group. Tonsillar herniation/concurrent syringomyelia, concavity/defect of clivus, herniation of bulbus and fourth ventricle, basilar invagination and craniovertebral junction abnormality rates were 30.3, 27, 18, 2, 3, and 3 percent, respectively. Absence of cisterna magna was encountered in 87.9% of the patients. Total, IT and ST volumes and distance between Chamberlain line and tip of dens axis, Klaus index, clivus length, distance between internal occipital protuberance and opisthion were significantly decreased in patient group. Also in patient group, it was found that Welcher basal angle/Boogard angle increased and tentorial slope angle decreased. Conclusion Mean cranial volume and length-angle measurement values significantly decreased and there was a congenital abnormality association in nearly 81.5 percent of the CM cases. As a result, it was concluded that CM ethiology can be attributed to multifactorial causes. Moreover, congenital defects can also give rise to this condition. PMID:26713146

Magnetic resonance imaging features of dogs with incomplete recovery after acute, severe spinal cord injury

PubMed Central

Lewis, Melissa J.; Cohen, Eli B.; Olby, Natasha J.

2017-01-01

Study Design Retrospective case series Objectives Describe the magnetic resonance imaging (MRI) features of dogs chronically impaired after severe spinal cord injury (SCI) and investigate associations between imaging variables and residual motor function. Setting United States of America Methods Thoracolumbar MRI from dogs with incomplete recovery months to years after clinically complete (paralysis with loss of pain perception) thoracolumbar SCI were reviewed. Lesion features were described and quantified. Gait was quantified using an ordinal, open field scale (OFS). Associations between imaging features and gait scores, duration of injury (DOI) or SCI treatment were determined. Results 35 dogs were included. Median OFS was 2 (0–6), median DOI was 13 months (3–83) and intervertebral disc herniation was the most common diagnosis (n=27). Myelomalacia was the most common qualitative feature followed by cystic change; syringomyelia and fibrosis were uncommon. Lesion length corrected to L2 length (LL:L2) was variable (median LL:L2=3.5 (1.34–11.54)). Twenty-nine dogs had 100% maximum cross-sectional spinal cord compromise (MSCC) at the lesion epicenter and the length of 100% compromised area varied widely (median length 100% MSCC:L2=1.29 (0.39–7.64). Length 100% MSCC:L2 was associated with OFS (p=0.012). OFS was not associated with any qualitative features. DOI or treatment type were not associated with imaging features or lesion quantification. Conclusions Lesion characteristics on MRI in dogs with incomplete recovery after severe SCI were established. Length of 100% MSCC was associated with hind limb motor function. Findings demonstrate a spectrum of injury severity on MRI amongst severely affected dogs which is related to functional status. PMID:29057987

Management of parturients in active labor with Arnold Chiari malformation, tonsillar herniation, and syringomyelia

PubMed Central

Ghaly, Ramsis F.; Tverdohleb, Tatiana; Candido, Kenneth D.; Knezevic, Nebojsa Nick

2017-01-01

Background: Arnold-Chiari malformation Type 1 (ACM-1) in parturients is a topic of ongoing discussion between obstetricians and anesthesiologists. The primary unanswered question remains; How should the anesthesia provider proceed with labor analgesia and anesthesia for cesarean section when confronted with an advanced, asymptomatic, or minimally symptomatic case of ACM-1 during labor? Case Description: A 24-year-old, ASA II, G1P0 full-term parturient presented to Labor and Delivery for vaginal delivery. A diagnosis of ACM-1 was made 12 years ago when a brain magnetic resonance imaging (MRI) was performed for right-sided numbness following a rear-end motor vehicle collision. The patient had been asymptomatic since then and had been seen by an outside neurologist frequently for the past 10 years. During the anesthesia evaluation, it was noted that she had an exaggerated patellar reflex, and a questionable left-sided Babinski; subsequently, an MRI study was requested. Review of a brain MRI demonstrated an advanced form of ACM with a 1.7 cm transtonsillar herniation and a large syrinx extending from C1 down to C5. Following a discussion with the patient, family, and primary OB team, a plan for elective cesarean section was made per neurosurgical recommendations. This was conducted uneventfully under general anesthesia. The patient had no complaints in the post-anesthesia care unit. Conclusion: Unfamiliarity of health care providers with regards to ACM-1 parturients can be countered by increasing awareness of this condition throughout medical specialties involved in their care. The Ghaly Obstetric Guide to Arnold-Chiari malformation Type 1, along with proper training of anesthesia care providers regarding the specificities of ACM-1 parturients aids in better management and understanding of this complex condition. PMID:28217389

Management of parturients in active labor with Arnold Chiari malformation, tonsillar herniation, and syringomyelia.

PubMed

Ghaly, Ramsis F; Tverdohleb, Tatiana; Candido, Kenneth D; Knezevic, Nebojsa Nick

2017-01-01

Arnold-Chiari malformation Type 1 (ACM-1) in parturients is a topic of ongoing discussion between obstetricians and anesthesiologists. The primary unanswered question remains; How should the anesthesia provider proceed with labor analgesia and anesthesia for cesarean section when confronted with an advanced, asymptomatic, or minimally symptomatic case of ACM-1 during labor? A 24-year-old, ASA II, G1P0 full-term parturient presented to Labor and Delivery for vaginal delivery. A diagnosis of ACM-1 was made 12 years ago when a brain magnetic resonance imaging (MRI) was performed for right-sided numbness following a rear-end motor vehicle collision. The patient had been asymptomatic since then and had been seen by an outside neurologist frequently for the past 10 years. During the anesthesia evaluation, it was noted that she had an exaggerated patellar reflex, and a questionable left-sided Babinski; subsequently, an MRI study was requested. Review of a brain MRI demonstrated an advanced form of ACM with a 1.7 cm transtonsillar herniation and a large syrinx extending from C1 down to C5. Following a discussion with the patient, family, and primary OB team, a plan for elective cesarean section was made per neurosurgical recommendations. This was conducted uneventfully under general anesthesia. The patient had no complaints in the post-anesthesia care unit. Unfamiliarity of health care providers with regards to ACM-1 parturients can be countered by increasing awareness of this condition throughout medical specialties involved in their care. The Ghaly Obstetric Guide to Arnold-Chiari malformation Type 1, along with proper training of anesthesia care providers regarding the specificities of ACM-1 parturients aids in better management and understanding of this complex condition.

Asymptomatic Chiari Type I malformation: should patients be advised against participation in contact sports?

PubMed

Spencer, Robert; Leach, Paul

2017-08-01

Chiari type I malformation (CM-I) is characterised by caudal displacement of the cerebellar tonsils through the foramen magnum, crowding the craniocervical junction. It is being increasingly diagnosed in asymptomatic patients due to the widespread availability of MRI, and there are case reports of these patients suffering sudden death or neurological injury following head or neck trauma, raising the issue of whether they should be prohibited from contact sport participation, given the likelihood of frequent trauma. General neurosurgical opinion is that patients who are symptomatic and those with an associated spinal cord syrinx should be offered foramen magnum decompression, however asymptomatic patients without syringomyelia are not offered this in the majority of cases. The authors performed a full review of the published literature, including all case reports, case series, studies and literature reviews regarding CM-I and either contact sports or trauma, excluding patients that had undergone surgical intervention and those becoming symptomatic in circumstances other than head or neck trauma. 21 case reports of CM-I patients deteriorating following trauma were identified, including four cases of sudden death following head or neck injury. However, studies of large samples of CM-I patients are yet to capture an incident of sudden death or acute neurological deterioration, suggesting that the risk is very low. CM-I patients may have an increased risk of concussion and post-concussion syndrome compared to the background population however. Overall, the authors feel that there should be no restriction of sports participation for CM-I patients, but a discussion to make them and their families aware of the possible increased risks is important.

Spinal ultrasound in patients with anorectal malformations: is this the end of an era?

PubMed

Scottoni, Federico; Iacobelli, Barbara Daniela; Zaccara, Antonio Maria; Totonelli, Giorgia; Schingo, Antonio Maria Salvatore; Bagolan, Pietro

2014-08-01

Even if lumbar magnetic resonance imaging (MRI) is considered the gold standard in the diagnosis of occult spinal dysraphism (SD) in patients with anorectal malformations (ARMs), spinal ultrasound (US) performed up to 5 months of life have been largely used as a screening test. The aim of the present study was to evaluate the accuracy in terms of sensibility and specificity of neonatal US to detect occult SD in patients with ARMs. Retrospective analysis of all patients treated for ARMs between 1999 and 2013 at our institution who underwent both spinal US (up to 5 months of life) and MRI. Sensibility and specificity have been calculated for US based on MRI results. Of 244 patients treated for ARMs at our institution, 82 (34 females, 48 males) underwent both the imaging studies and have been included in this study. ARMs types were: anal stenosis (7), recto-vestibular fistula (19), recto-perineal fistula (3) and cloaca (5) in female and imperforate anus (7) recto-perineal fistula (14), recto-urethral fistula (22), recto-vesical fistula (5) in males. Forty-seven patients (57, 3 % of total, 18 females, 29 males) had some occult SD (tethered spinal cord, spinal lipoma, syringomyelia) at MRI. Only 7 (14, 8 %) patients of those with spinal anomalies at MRI had pathological US studies. In our population, sensibility and specificity of US for diagnosis of occult SD were, respectively, 14, 8 and 100 %. Since it is well known that a screening test must have a high sensibility, our data suggest that spinal ultrasound is not suitable as a screening test for occult spinal dysraphism in patients with ARMs. Furthermore, we strongly advise against the use of US as a screening test for spinal dysraphism to prevent a false sense of security in physician and patients' families.

Acute Presentation of Chiari I Malformation with Hemiparesis in a Pediatric Patient.

PubMed

Miranda, Stephen P; Kimmell, Kristopher T; Silberstein, Howard J

2016-01-01

Chiari I malformation (CM-I) is defined by cerebellar tonsillar herniation through the foramen magnum. Patients typically present with chronic complaints, including headache, dizziness, and numbness, although there are few reports in the literature of pediatric patients presenting acutely with neurological deficit caused by CM-I. We report a child who presented acutely with hemiparesis and magnetic resonance imaging findings consistent with CM-I and spinal cord edema. A 3-year old boy with normal development presented with difficulty walking and increased drooling. His mother stated he was running into objects and had balance issues for several days. Neurological examination showed ataxia with falling to the right after a few steps and weakness of the right arm and leg. His medical history was remarkable only for mild asthma, although he had recently been treated for an upper respiratory viral infection. Computed tomography of the head demonstrated no brainstem mass. Magnetic resonance imaging of the head and cervical spine showed tonsillar ectopia approximately 2 cm below the craniocervical junction with increased T2 signal in the spinal cord from C1 to C3 consistent with syringomyelia and cord edema. The patient underwent suboccipital craniectomy with removal of the posterior arch of C1 and dural patch graft. His postoperative course was unremarkable, with complete resolution of his symptoms at his 1-month follow-up visit. This case highlights an unusual presentation of CM-I with neurological deficit related to spinal cord edema, possibly precipitated by the "water-hammer" effect of this patient's coughing fits. Providers should be aware of the acute presentations of CM-I. Copyright © 2016 Elsevier Inc. All rights reserved.

Sagittal MRI often overestimates the degree of cerebellar tonsillar ectopia: a potential for misdiagnosis of the Chiari I malformation.

PubMed

Tubbs, R Shane; Yan, Huang; Demerdash, Amin; Chern, Joshua J; Fries, Fabian N; Oskouian, Rod J; Oakes, W Jerry

2016-07-01

We hypothesized that by using coronal MRI, Chiari I malformation could be more precisely diagnosed, would provide simple anatomic landmarks, would provide information regarding asymmetry of hindbrain herniation, and would be a better method for analyzing the tonsillar herniation postoperatively when the opisthion has been removed. Fifty consecutive pediatric patients diagnosed with Chiari I malformation had comparison between the measurements of their caudally descended cerebellar tonsils on midsagittal and coronal MRI images. On MRI coronal imaging, tonsillar asymmetry was found in 48 patients. Maximal left tonsillar descent was 20.9 mm, and maximal right tonsillar descent was 17.4 mm. On MRI sagittal imaging, tonsillar descent ranged from 5 to 27.4 mm. Fifty-eight % of patients had syringomyelia. Five patients (10 %) on coronal MRI were found to have both cerebellar tonsils that were less than 3 mm below the foramen magnum. However, all of these patients had greater than 3 mm of tonsillar ectopia on sagittal imaging. Nineteen patients (38 %) on coronal MRI were found to have one of the cerebellar tonsils that were less than 3 mm below the foramen magnum. Similarly, each of these had greater than 3 mm of tonsillar ecotpia as measured on midsagittal MRI. Also, based on these findings, Chiari I malformation is almost always an asymmetrical tonsillar ectopia. Sagittal MRI overestimates the degree of tonsillar ectopia in patients with Chiari I malformation. Misdiagnosis may occur if sagittal imaging alone is used. The cerebellar tonsils are paramedian structures, and this should be kept in mind when interpreting midline sagittal MRI.

A Systematic Review of Chiari I Malformation: Techniques and Outcomes.

PubMed

Zhao, Jian-Lan; Li, Mei-Hua; Wang, Chun-Liang; Meng, Wei

2016-04-01

To elucidate the most efficacious treatments of Chiari I malformation (CIM). A literature search was performed using PubMed, CINAHL/Ovid, Cochrane library, and the Elsevier database. The key words "Chiari I malformation," "Chiari malformation type I," "surgery," and "treatment" were used for the search. Articles had to be peer reviewed and provide primary outcomes measured by clinical and radiographic outcomes after surgical treatments. Exclusion criteria included non-English-language articles, case reports, commentaries, information from textbooks and expert opinions, and articles that did not provide outcomes concerning specific surgical methods. Patients included were classified into 4 groups according the procedure: only bony decompression but not duroplasty (group I), bony decompression plus duroplasty (group II), bony decompression plus the resection of tonsils (group III), and shunt (group IV). Eighteen studies were identified. Groups II and III had a significantly higher improvement rate (82.25%, 86.10%, P < 0.05) of outcomes with regard to clinical signs and symptoms than the other groups. Group IV showed a statistically higher rate (30.49%, P < 0.05) of aggregating clinical signs and symptoms. In patients with syringomyelia, group III showed better clinical improvement (96.08%). Group II displayed a significantly higher rate of decrease in the size of cavities (83.33%, P < 0.05). Group IV had a statistically higher rate of increase in the size of cavities (33.87%, P < 0.05). Only bony decompression cannot achieve satisfactory outcomes. Bony decompression plus duroplasty showed the most favorable outcomes. Resection of tonsils was not recommended because of the high rate of side effects. Shunt may aggregate clinical signs and symptoms and increase the size of cavities. Copyright © 2016 Elsevier Inc. All rights reserved.

[Comparison of posterior fossa decompression with and without duraplasty for surgical management for adult Chiari malformation type Ⅰ].

PubMed

Li, H Y; Li, Y M; Chen, H; Li, Y; Shi, X W

2017-07-04

Objective: To evaluate and compare the efficacy between posterior fossa decompression without duraplasty (PFD) and posterior fossa decompression with duraplasty (PFDD) in the surgical management for adult Chiari Ⅰ malformation. Methods: Fifty-seven patients suffered from Chiari malformation type Ⅰ were treated in Department of Neurosurgery, Henan Provincial People's Hospital from August 2008 to October 2013. Twenty-three patients received posterior fossa decompression without duraplasty and the other 34 patients received posterior fossa decompression with duraplasty. The clinical results were retrospectively analyzed to compare the efficacy of two different surgical approaches. Results: There was no death or severe neurological dysfunction case in 57 patients of the two groups. Patients undergoing PFD had shorter length of hospital stay[(13.7±3.5) d vs (16.2±4.1) d, P <0.05]and surgical time[(98.7±22.1) min vs (132.3±39.6)min, P <0.05]. Cerebrospinal fluid-related complications and intracranial infection were more common in patients undergoing PFDD[(0/23, 0) vs (8/34, 23.5%), P <0.05]. Clinical improvement was comparable in two groups[(15/23, 65.2%) vs (26/34, 76.5%), P >0.05]at the one-year follow-up. The rate of syrinx regression in patients with Syringomyelia was higher in patients undergoing PFDD[(3/12, 25%) vs (17/22, 77.3%), P <0.05]. Conclusion: For adult patients with Chiari malformation type Ⅰ, PFD has the advantages of simple manipulation, short length of hospital stay and low incidence of cerebrospinal fluid-related complications and intracranial infection, compared with PFDD. It is comparable to PFDD in clinical improvement, but the effect of PFD is not as good as that of PFDD in the aspect of syrinx regression.

No significant difference between chiari malformation type 1.5 and type I.

PubMed

Liu, Wei; Wu, Hongxing; Aikebaier, Yalikun; Wulabieke, Maoliti; Paerhati, Rexiti; Yang, Xiaopeng

2017-06-01

Chiari malformation Type 1.5 (CM 1.5) was defined as the association of Chiari malformation Type I (CM I) and brainstem herniation. The objective was to demonstrate the difference of clinical features and surgical outcomes between CM 1.5 and CM I. All CM 1.5 and CM I adult patients who underwent posterior fossa decompression with duraplasty at our institution between 2006 and 2010 were retrospectively reviewed. Clinical characteristics, imaging features, and long-term outcomes were compared between CM 1.5 and CM I patients. A total of 142 adult patients were enrolled, including 27 CM 1.5 and 115 CM I patients. The average follow-up period was 102 months. Age at diagnosis was significantly younger in CM 1.5 group than CM I group (p=0.039). And the degree of tonsillar herniation was significantly more severe in CM 1.5 group than CM I group (p<0.001). There was no significant difference in other clinical and imaging characteristics. Moreover, improvement of symptoms was observed in 21 CM 1.5 patients (77.8%) and 94 CM I patients (81.7%), and no significant difference was detected (p=0.637). There was no significant difference in the resolution of syringomyelia between CM 1.5 (72.7%) and CM I (76.5%) patients, either (p=0. 710). Although CM 1.5 patients presented with brainstem herniation and more severe tonsillar herniation, other clinical and imaging features and surgical outcomes were similar with CM I patients. We think CM 1.5 is just a subtype of CM I, rather than a unique type of Chiari malformations. Copyright © 2017 Elsevier B.V. All rights reserved.

Interventional management of intractable sympathetically mediated pain by computed tomography-guided catheter implantation for block and neuroablation of the thoracic sympathetic chain: technical approach and review of 322 procedures.

PubMed

Agarwal-Kozlowski, K; Lorke, D E; Habermann, C R; Schulte am Esch, J; Beck, H

2011-08-01

We retrospectively evaluated the safety and efficacy of computed tomography-guided placement of percutaneous catheters in close proximity to the thoracic sympathetic chain by rating pain intensity and systematically reviewing charts and computed tomography scans. Interventions were performed 322 times in 293 patients of mean (SD) age 59.4 (17.0) years, and male to female ratio 105:188, with postherpetic neuralgia (n = 103, 35.1%), various neuralgias (n = 88, 30.0%), complex regional pain syndrome (n = 69, 23.6%), facial pain (n = 17, 5.8%), ischaemic limb pain (n = 7, 2.4%), phantom limb pain (n = 4, 1.4%), pain following cerebrovascular accident (n = 2, 0.7%), syringomyelia (n = 2, 0.7%) and palmar hyperhidrosis (n = 1, 0.3%). The interventions were associated with a total of 23 adverse events (7.1% of all procedures): catheter dislocation (n = 9, 2.8%); increase in pain intensity (n = 8, 2.5%); pneumothorax (n = 3, 0.9%); local infection (n = 2, 0.6%); and puncture of the spinal cord (n = 1, 0.3%). Continuous infusion of 10 ml.h(-1) ropivacaine 0.2% through the catheters decreased median (IQR [range]) pain scores from 8 (6-9 [2-10]) to 2 (1-3 [0-10]) (p < 0.0001). Chemical neuroablation was necessary in 137 patients (46.8%). We conclude that this procedure leads to a significant reduction of pain intensity in otherwise obstinate burning or stabbing pain and is associated with few hazards. © 2011 The Authors. Anaesthesia © 2011 The Association of Anaesthetists of Great Britain and Ireland.

Anterior only fusion for scoliosis in patients with myelomeningocele.

PubMed

Sponseller, P D; Young, A T; Sarwark, J F; Lim, R

1999-07-01

A series of patients with single major scoliosis curvatures attributable to spina bifida treated by anterior only spinal fusion was studied for 2 years to determine whether the infection rate could be decreased, adequate correction and pelvic balance could be provided, and posterior surgery could be avoided in these patients. Anterior surgery alone was performed for thoracolumbar scoliosis greater than 45 degrees if the compensatory thoracic curve was less than 40 degrees and there was no significant junctional kyphosis. Fourteen patients were treated at a mean age of 11.9 years (range, 7-16 years), with a mean curve of 64 degrees (range, 51 degrees-85 degrees), and motor levels distributed from T10-L4. Thirteen patients had prior neurosurgery for tether, syrinx, or Arnold-Chiari malformation. The spine was fused over a mean of seven vertebrae. A 3/16 inch Texas Scottish Rite Hospital rod was used most commonly (10 patients). Blood loss averaged 1100 cc. The mean curve correction was 57% at 40 months after surgery. Loss of correction occurred primarily by adding on outside the instrumented area. Mean pelvic obliquity was improved from 16 degrees to 9 degrees. There was one superficial infection. Results were good in five patients, fair in four, and poor in five. Failures were attributable to proximal decompensation in two patients who required revision surgery (two), neurologic deterioration in two, and screw pullout in one. Both patients with decompensation had syringomyelia. Both patients with neurologic deterioration had large curves (> 75 degrees). Both patients recovered after rod removal. Retrospectively, by eliminating patients with syrinx or with a curve greater than 75 degrees, all poor results would be eliminated. Anterior only fusion and instrumentation may have significant advantages, but only for selected patients with thoracolumbar curves less than 75 degrees, compensatory curves less than 40 degrees, no increased kyphosis, and no syrinx. Quadriceps function should be monitored. On the basis of this preliminary experience, continued use of this approach using stricter selection seems warranted.

Origins of the sensory examination in neurology.

PubMed

Freeman, Cassiopeia; Okun, Michael S

2002-12-01

Formal testing of sensation as part of the neurological examination followed the improvements in examination techniques as well as advances in neuroscience. By the 1890s, the observation that temperature sense was frequently impaired at the same time that pain was appreciated led to the supposition that the two paths traveled closely. Through the works of Brown-Séquard and Edinger the existence of a crossed afferent tract was verified. The distinction between two sensory pathways was clear by 1898, when van Gehuchten reported a case of syringomyelia and suggested that the pain and temperature fibers were carried anterolaterally and the position sense fibers carried posteriorly in the spinal cord. Many authors describing patients with tabes dorsalis suspected the posterior columns of the spinal cord played a key role in position sense. It is difficult to determine in the 19th century who first employed the use of movements of joints as a test for proprioceptive function; however, Bell in 1826 recognized what he termed a sixth sense, which later was characterized as proprioceptive function. Goldscheider went on to report the degrees of movement that were considered normal for each joint. Although vibratory sense had been described by Cardano and Ingrassia in the 16th century and tests had been developed by Rinne and Rumpf by the 19th century, it was not until 1903 that Rydel and Seiffer found that vibratory sense and proprioceptive sense were closely related and that both senses were carried in the posterior columns of the spinal cord. By 1955, the sensory examination included tests for light-touch, superficial pain, temperature, position sense, vibration, muscle (deep pain), and two-point discrimination. Tests for these sensibilities still remain in use. We will review the origins of the understanding of sensation, which ultimately led to the development of the sensory examination. We will highlight individuals who made important discoveries and observations, as well as review the history of each of the elements of the sensory examination.

Mutations in ACY1, the Gene Encoding Aminoacylase 1, Cause a Novel Inborn Error of Metabolism

PubMed Central

Sass, Jörn Oliver; Mohr, Verena; Olbrich, Heike; Engelke, Udo; Horvath, Judit; Fliegauf, Manfred; Loges, Niki Tomas; Schweitzer-Krantz, Susanne; Moebus, Ralf; Weiler, Polly; Kispert, Andreas; Superti-Furga, Andrea; Wevers, Ron A.; Omran, Heymut

2006-01-01

N-terminal acetylation of proteins is a widespread and highly conserved process. Aminoacylase 1 (ACY1; EC 3.5.14) is the most abundant of the aminoacylases, a class of enzymes involved in hydrolysis of N-acetylated proteins. Here, we present four children with genetic deficiency of ACY1. They were identified through organic acid analyses using gas chromatography–mass spectrometry, revealing increased urinary excretion of several N-acetylated amino acids, including the derivatives of methionine, glutamic acid, alanine, leucine, glycine, valine, and isoleucine. Nuclear magnetic resonance spectroscopy analysis of urine samples detected a distinct pattern of N-acetylated metabolites, consistent with ACY1 dysfunction. Functional analyses of patients’ lymphoblasts demonstrated ACY1 deficiency. Mutation analysis uncovered recessive loss-of-function or missense ACY1 mutations in all four individuals affected. We conclude that ACY1 mutations in these children led to functional ACY1 deficiency and excretion of N-acetylated amino acids. Questions remain, however, as to the clinical significance of ACY1 deficiency. The ACY1-deficient individuals were ascertained through urine metabolic screening because of unspecific psychomotor delay (one subject), psychomotor delay with atrophy of the vermis and syringomyelia (one subject), marked muscular hypotonia (one subject), and follow-up for early treated biotinidase deficiency and normal clinical findings (one subject). Because ACY1 is evolutionarily conserved in fish, frog, mouse, and human and is expressed in the central nervous system (CNS) in human, a role in CNS function or development is conceivable but has yet to be demonstrated. Thus, at this point, we cannot state whether ACY1 deficiency has pathogenic significance with pleiotropic clinical expression or is simply a biochemical variant. Awareness of this new genetic entity may help both in delineating its clinical significance and in avoiding erroneous diagnoses. PMID:16465618

Suitability of foramen magnum measurements in sex determination and their clinical significance.

PubMed

Tellioglu, A Metin; Durum, Y; Gok, M; Karakas, S; Polat, A G; Karaman, C Z

2018-01-01

The foramen magnum provides a transition between fossa cranii posterior and canalis vertebralis. Medulla oblongata, arteria vertebralis and nervus accessorius spinal part pass through the foramen magnum. In this study, we aimed to make the morphometric measurements of the foramen magnum on computed tomography (CT) and to determine the feasibility of sex determination based on these measurements. Besides sex determination, from a clinical aspect, it is important to know the measurements of the foramen magnum in the normal population in terms of diseases characterised by displacement of the posterior fossa structures through foramen magnum to upper cervical spinal canal such as Chiari malformations and syringomyelia. All the data for our study was obtained retrospectively from 100 patients (50 males, 50 females) who had a CT scan of the head and neck region in Adnan Menderes University Hospital, Department of Radiology. To examine the foramen magnum in each and every occipital bone, we measured the foramen magnum's anteroposterior diameter, transverse diameter, the area of the foramen magnum and its circumference. We found that men have a higher average value than women in our study. According to Student's t-test results; in all measured parameters, there is significant difference between the genders (p < 0.05). When multivariate discriminant function test is performed for all four measurements, the discrimination rate is 64% for all women, 70% for all men and 67% for both genders. As a result of our study, the metric data we obtained will be useful in cases where the skeletons' sex could not be determined by any other methods. We believe that, our study may be useful for other studies in determining of sex from foramen magnum. Our measurements could give some information of the normal ranges of the foramen magnum in normal population, so that this can contribute to the diagnosis process of some diseases by imaging. (Folia Morphol 2018; 77, 1: 99-104).

Stratified Whole Genome Linkage Analysis of Chiari Type I Malformation Implicates Known Klippel-Feil Syndrome Genes as Putative Disease Candidates

PubMed Central

Markunas, Christina A.; Soldano, Karen; Dunlap, Kaitlyn; Cope, Heidi; Asiimwe, Edgar; Stajich, Jeffrey; Enterline, David; Grant, Gerald; Fuchs, Herbert

2013-01-01

Chiari Type I Malformation (CMI) is characterized by displacement of the cerebellar tonsils below the base of the skull, resulting in significant neurologic morbidity. Although multiple lines of evidence support a genetic contribution to disease, no genes have been identified. We therefore conducted the largest whole genome linkage screen to date using 367 individuals from 66 families with at least two individuals presenting with nonsyndromic CMI with or without syringomyelia. Initial findings across all 66 families showed minimal evidence for linkage due to suspected genetic heterogeneity. In order to improve power to localize susceptibility genes, stratified linkage analyses were performed using clinical criteria to differentiate families based on etiologic factors. Families were stratified on the presence or absence of clinical features associated with connective tissue disorders (CTDs) since CMI and CTDs frequently co-occur and it has been proposed that CMI patients with CTDs represent a distinct class of patients with a different underlying disease mechanism. Stratified linkage analyses resulted in a marked increase in evidence of linkage to multiple genomic regions consistent with reduced genetic heterogeneity. Of particular interest were two regions (Chr8, Max LOD = 3.04; Chr12, Max LOD = 2.09) identified within the subset of “CTD-negative” families, both of which harbor growth differentiation factors (GDF6, GDF3) implicated in the development of Klippel-Feil syndrome (KFS). Interestingly, roughly 3–5% of CMI patients are diagnosed with KFS. In order to investigate the possibility that CMI and KFS are allelic, GDF3 and GDF6 were sequenced leading to the identification of a previously known KFS missense mutation and potential regulatory variants in GDF6. This study has demonstrated the value of reducing genetic heterogeneity by clinical stratification implicating several convincing biological candidates and further supporting the hypothesis that multiple, distinct mechanisms are responsible for CMI. PMID:23620759

Relationship between Cough-Associated Changes in CSF Flow and Disease Severity in Chiari I Malformation: An Exploratory Study Using Real-Time MRI.

PubMed

Bezuidenhout, A F; Khatami, D; Heilman, C B; Kasper, E M; Patz, S; Madan, N; Zhao, Y; Bhadelia, R A

2018-05-10

Currently no quantitative objective test exists to determine disease severity in a patient with Chiari I malformation. Our aim was to correlate disease severity in symptomatic patients with Chiari I malformation with cough-associated changes in CSF flow as measured with real-time MR imaging. Thirteen symptomatic patients with Chiari I malformation (tonsillar herniation of ≥5 mm) were prospectively studied. A real-time, flow-sensitized pencil-beam MR imaging scan was used to measure CSF stroke volume during rest and immediately following coughing and relaxation periods (total scan time, 90 seconds). Multiple posterior fossa and craniocervical anatomic measurements were also obtained. Patients were classified into 2 groups by neurosurgeons blinded to MR imaging measurements: 1) nonspecific Chiari I malformation (5/13)-Chiari I malformation with nonspecific symptoms like non-cough-related or mild occasional cough-related headache, neck pain, dizziness, paresthesias, and/or trouble swallowing; 2) specific Chiari I malformation (8/13)-patients with Chiari I malformation with specific symptoms and/or objective findings like severe cough-related headache, myelopathy, syringomyelia, and muscle atrophy. The Spearman correlation was used to determine correlations between MR imaging measurements and disease severity, and both groups were also compared using a Mann-Whitney U test. There was a significant negative correlation between the percentage change in CSF stroke volume (resting to postcoughing) and Chiari I malformation disease severity ( R = 0.59; P = .03). Mann-Whitney comparisons showed the percentage change in CSF stroke volume (resting to postcoughing) to be significantly different between patient groups ( P = .04). No other CSF flow measurement or anatomic measure was significantly different between the groups. Our exploratory study suggests that assessment of CSF flow response to a coughing challenge has the potential to become a valuable objective noninvasive test for clinical assessment of disease severity in patients with Chiari I malformation. © 2018 by American Journal of Neuroradiology.

Brain-stem hemangioblastomas: The seemingly innocuous lesion in a perilous location.

PubMed

Joseph, Jeena; Behari, Sanjay; Gupta, Shruti; Bhaisora, Kamlesh Singh; Gandhi, Anish; Srivastava, Arun; Jaiswal, Awadhesh K

2018-01-01

Hemangioblastomas [75% sporadic, 25% with Von Hippel Lindau (VHL) disease] are highly vascular, benign lesions. The surgical nuances, management, and complication avoidance in brain-stem hemangioblastomas (BHs) have been studied. Over 18 years, 27(mean age: 29 years; range 15-60 years) consecutive cases of BH underwent microsurgical excision. All patients were assessed clinico-radiologically for neurological deficits and screened for VHL disease. Outcome of the patients was based on Karnofsky Performance Status scale (KPS). 12 out of 19 (70.4%) patients with hydrocephalus underwent a cerebrospinal fluid (CSF) diversion procedure. Lower cranial nerve palsy was present in 10 (37%) patients and motor weakness in 13 (48%). The tumours [mean size 3.34 ± 1.06 cm, range: 1.4-5.5 cm; 11 solid, rest solid-cystic; 18 (66.7%) subpial and 9 (33.33%) intramedullary] were divided into four categories based on size: A: <2 cm (n = 5,18.5%); B: 2-3 cm (n = 10,37%); C: 3-4 cm (n = 6,22.2%); D: >4 cm (n = 6,22.2%). Their location was at posterior cervicomedullary junction (n = 12); pontomedullary junction (n = 7); pons (n = 3), medulla (n = 3) and ponto-mesencephalic region (n = 2). Multiple flow voids were seen in >50% patients with tumour >2 cm. 5 patients had syringomyelia; and, 8 had diffuse cervical cord expansion. Two patients with a large vascular tumour underwent preoperative embolization. Six patients had VHL disease; one underwent bilateral adrenalectomy for refractory hypertension; and, the another, nephrectomy for renal cell carcinoma. Twenty-six patients underwent a midline suboccipital craniectomy; and, 1 with a cerebellopontine angle tumour, a retromastoid craniectomy. 15 patients underwent total excision; 10 patients, near-total (<10% remaining) excision, and 2 patients, a subtotal (>10% remaining)) excision. Three patients (2 with VHL disease) expired due to exsanguinating hemorrhage, spreading venous thrombosis and aspiration pneumonitis, respectively. At follow-up visit (median: 25 ± interquartile range 2-56months), 17 patients had improved KPS, 4 remained in same status and 3 (recently operated, on tracheostomy) had worsened KPS. Significant improvement is achievable in neurological status in patients following successful extirpation of a brain-stem hemangioblastoma, despite a turbulent perioperative period. Leaving tumour capsule adherent to the brain-stem often helps in preserving brain-stem function. Postoperatively, the patients should be monitored for their respiratory and lower cranial nerve status to prevent aspiration pneumonitis.

Amelioration of motor/sensory dysfunction and spasticity in a rat model of acute lumbar spinal cord injury by human neural stem cell transplantation

PubMed Central

2013-01-01

Introduction Intraspinal grafting of human neural stem cells represents a promising approach to promote recovery of function after spinal trauma. Such a treatment may serve to: I) provide trophic support to improve survival of host neurons; II) improve the structural integrity of the spinal parenchyma by reducing syringomyelia and scarring in trauma-injured regions; and III) provide neuronal populations to potentially form relays with host axons, segmental interneurons, and/or α-motoneurons. Here we characterized the effect of intraspinal grafting of clinical grade human fetal spinal cord-derived neural stem cells (HSSC) on the recovery of neurological function in a rat model of acute lumbar (L3) compression injury. Methods Three-month-old female Sprague–Dawley rats received L3 spinal compression injury. Three days post-injury, animals were randomized and received intraspinal injections of either HSSC, media-only, or no injections. All animals were immunosuppressed with tacrolimus, mycophenolate mofetil, and methylprednisolone acetate from the day of cell grafting and survived for eight weeks. Motor and sensory dysfunction were periodically assessed using open field locomotion scoring, thermal/tactile pain/escape thresholds and myogenic motor evoked potentials. The presence of spasticity was measured by gastrocnemius muscle resistance and electromyography response during computer-controlled ankle rotation. At the end-point, gait (CatWalk), ladder climbing, and single frame analyses were also assessed. Syrinx size, spinal cord dimensions, and extent of scarring were measured by magnetic resonance imaging. Differentiation and integration of grafted cells in the host tissue were validated with immunofluorescence staining using human-specific antibodies. Results Intraspinal grafting of HSSC led to a progressive and significant improvement in lower extremity paw placement, amelioration of spasticity, and normalization in thermal and tactile pain/escape thresholds at eight weeks post-grafting. No significant differences were detected in other CatWalk parameters, motor evoked potentials, open field locomotor (Basso, Beattie, and Bresnahan locomotion score (BBB)) score or ladder climbing test. Magnetic resonance imaging volume reconstruction and immunofluorescence analysis of grafted cell survival showed near complete injury-cavity-filling by grafted cells and development of putative GABA-ergic synapses between grafted and host neurons. Conclusions Peri-acute intraspinal grafting of HSSC can represent an effective therapy which ameliorates motor and sensory deficits after traumatic spinal cord injury. PMID:23710605

Autologous olfactory ensheathing cell transplantation in human paraplegia: a 3-year clinical trial

PubMed Central

Féron, F.; Cochrane, J.; Bassingthwaighte, L.; Bayliss, C.; Davies, W.; Fronek, P.; Gray, C.; Kerr, G.; Licina, P.; Nowitzke, A.; Perry, C.; Silburn, P.A.S.; Urquhart, S.; Geraghty, T.

2008-01-01

Olfactory ensheathing cells show promise in preclinical animal models as a cell transplantation therapy for repair of the injured spinal cord. This is a report of a clinical trial of autologous transplantation of olfactory ensheathing cells into the spinal cord in six patients with complete, thoracic paraplegia. We previously reported on the methods of surgery and transplantation and the safety aspects of the trial 1 year after transplantation. Here we address the overall design of the trial and the safety of the procedure, assessed during a period of 3 years following the transplantation surgery. All patients were assessed at entry into the trial and regularly during the period of the trial. Clinical assessments included medical, psychosocial, radiological and neurological, as well as specialized tests of neurological and functional deficits (standard American Spinal Injury Association and Functional Independence Measure assessments). Quantitative test included neurophysiological tests of sensory and motor function below the level of injury. The trial was a Phase I/IIa design whose main aim was to test the feasibility and safety of transplantation of autologous olfactory ensheathing cells into the injured spinal cord in human paraplegia. The design included a control group who did not receive surgery, otherwise closely matched to the transplant recipient group. This group acted as a control for the assessors, who were blind to the treatment status of the patients. The control group also provided the opportunity for preliminary assessment of the efficacy of the transplantation. There were no adverse findings 3 years after autologous transplantation of olfactory ensheathing cells into spinal cords injured at least 2 years prior to transplantation. The magnetic resonance images (MRIs) at 3 years showed no change from preoperative MRIs or intervening MRIs at 1 and 2 years, with no evidence of any tumour of introduced cells and no development of post-traumatic syringomyelia or other adverse radiological findings. There were no significant functional changes in any patients and no neuropathic pain. In one transplant recipient, there was an improvement over 3 segments in light touch and pin prick sensitivity bilaterally, anteriorly and posteriorly. We conclude that transplantation of autologous olfactory ensheathing cells into the injured spinal cord is feasible and is safe up to 3 years of post-implantation, however, this conclusion should be considered preliminary because of the small number of trial patients. PMID:18689435

Principles of management of osteometabolic disorders affecting the aging spine.

PubMed

Hadjipavlou, Alexander G; Katonis, Paul G; Tzermiadianos, Michael N; Tsoukas, George M; Sapkas, George

2003-10-01

Osteoporosis is the most common contributing factor of spinal fractures, which characteristically are not generally known to produce spinal cord compression symptoms. Recently, an increasing number of medical reports have implicated osteoporotic fractures as a cause of serious neurological deficit and painful disabling spinal deformities. This has been corroborated by the present authors as well. These complications are only amenable to surgical management, requiring instrumentation. Instrumenting an osteoporotic spine, although a challenging task, can be accomplished if certain guidelines for surgical techniques are respected. Neurological deficits respond equally well to an anterior or posterior decompression, provided this is coupled with multisegmental fixation of the construct. With the steady increase in the elderly population, it is anticipated that the spine surgeon will face serious complications of osteoporotic spines more frequently. With regard to surgery, however, excellent correction of deformities can be achieved, by combining anterior and posterior approaches. Paget's disease of bone (PD) is a non-hormonal osteometabolic disorder and the spine is the second most commonly affected site. About one-third of patients with spinal involvement exhibit symptoms of clinical stenosis. In only 12-24% of patients with PD of the spine is back pain attributed solely to PD, while in the majority of patients, back pain is either arthritic in nature or a combination of a pagetic process and coexisting arthritis. In this context, one must be certain before attributing low back pain to PD exclusively, and antipagetic medical treatment alone may be ineffective. Neural element dysfunction may be attributed to compressive myelopathy by pagetic bone overgrowth, pagetic intraspinal soft tissue overgrowth, ossification of epidural fat, platybasia, spontaneous bleeding, sarcomatous degeneration and vertebral fracture or subluxation. Neural dysfunction can also result from spinal ischemia when blood is diverted by the so-called "arterial steal syndrome". Because the effectiveness of pharmacologic treatment for pagetic spinal stenosis has been clearly demonstrated, surgical decompression should only be instituted after failure of antipagetic medical treatment. Surgery is indicated as a primary treatment when neural compression is secondary to pathologic fractures, dislocations, spontaneous epidural hematoma, syringomyelia, platybasia, or sarcomatous transformation. Five classes of drugs are available for the treatment of PD. Bisphosphonates are the most popular antipagetic drug and several forms have been investigated.

The Clinical Features and Surgical Outcomes of Spinal Cord Tanycytic Ependymomas: A Report of 40 Cases.

PubMed

Tao, Xiaogang; Hou, Zonggang; Hao, Shuyu; Zhang, Qi; Wu, Zhen; Zhang, Junting; Liu, Baiyun

2017-10-01

Spinal cord tanycytic ependymomas (TEs) rarely are reported because of extremely low incidence. Understanding of this disease is therefore poor. The aim of this study was to analyze the incidence and clinical, radiologic, pathologic, and prognostic features of spinal cord TEs. Approximately 4000 spinal cord tumors were resected surgically in Beijing Tiantan Hospital between April 2009 and May 2016. We identified all cases of pathologically proved TEs among these patients. TEs accounted for approximately 1% of spinal cord tumors (40 of an estimated 4000). Patients with TE were a mean age of 40.0 years and had no significant sex preference (21 male and 19 female). The median diameter of the maximal tumor was 54.2 mm (range, 16-153 mm). The mean preoperative Japanese Orthopedic Association (JOA) score was 13.0. Radiologically, 47.5% (19/40) cases showed poorly defined tumor border, and 40% (16/40) of them showed preoperative syringomyelia. Gross total resection was achieved in 30 cases, subtotal resection in 8, and partial resection in 2. At the time of discharge, JOA score and neurologic function showed improvement in 26 cases (65%), no change in 12 cases (30%), and worsening in 2 cases (5%). At a median follow-up of 43 months (range, 7-101 months), 1- and 5-year progression-free survival rates were 100.0% and 97.5%, respectively. Only one patient had tumor recurrence which was found 30 months after surgery. No patient died of tumor recurrence. At the latest follow-up, JOA score showed improvement in 30 cases (75%), no change in 8 cases (20%), and worsening in 2 cases (5%). Univariate analysis revealed that tumor size and preoperative JOA score were significantly associated with the short-term outcomes. Meanwhile, age ≥40 years, tumor size ≥50 mm, non-gross total resection, and preoperative JOA score <14 were significantly associated with an increased risk of worsened long-term outcomes. Poorly defined tumor border and preoperative JOA score <14 significantly limited the extent of tumor resection. Spinal cord TE is a rare subtype of ependymomas with low recurrence. Long-term survival can be expected, although poorly defined tumor border is an independent predictor of long-term outcomes. Microsurgical treatment of spinal cord TEs remains a formidable challenge due to the poorly defined border and critical neurovascular structures encasement. It is unnecessary for radical tumor resection at the cost of severe neurologic deficits. Copyright © 2017 Elsevier Inc. All rights reserved.

Logistic regression analysis of risk factors for postoperative recurrence of spinal tumors and analysis of prognostic factors.

PubMed

Zhang, Shanyong; Yang, Lili; Peng, Chuangang; Wu, Minfei

2018-02-01

The aim of the present study was to investigate the risk factors for postoperative recurrence of spinal tumors by logistic regression analysis and analysis of prognostic factors. In total, 77 male and 48 female patients with spinal tumor were selected in our hospital from January, 2010 to December, 2015 and divided into the benign (n=76) and malignant groups (n=49). All the patients underwent microsurgical resection of spinal tumors and were reviewed regularly 3 months after operation. The McCormick grading system was used to evaluate the postoperative spinal cord function. Data were subjected to statistical analysis. Of the 125 cases, 63 cases showed improvement after operation, 50 cases were stable, and deterioration was found in 12 cases. The improvement rate of patients with cervical spine tumor, which reached 56.3%, was the highest. Fifty-two cases of sensory disturbance, 34 cases of pain, 30 cases of inability to exercise, 26 cases of ataxia, and 12 cases of sphincter disorders were found after operation. Seventy-two cases (57.6%) underwent total resection, 18 cases (14.4%) received subtotal resection, 23 cases (18.4%) received partial resection, and 12 cases (9.6%) were only treated with biopsy/decompression. Postoperative recurrence was found in 57 cases (45.6%). The mean recurrence time of patients in the malignant group was 27.49±6.09 months, and the mean recurrence time of patients in the benign group was 40.62±4.34. The results were significantly different (P<0.001). Recurrence was found in 18 cases of the benign group and 39 cases of the malignant group, and results were significantly different (P<0.001). Tumor recurrence was shorter in patients with a higher McCormick grade (P<0.001). Recurrence was found in 13 patients with resection and all the patients with partial resection or biopsy/decompression. The results were significantly different (P<0.001). Logistic regression analysis of total resection-related factors showed that total resection should be the preferred treatment for patients with benign tumors, thoracic and lumbosacral tumors, and lower McCormick grade, as well as patients without syringomyelia and intramedullary tumors. Logistic regression analysis of recurrence-related factors revealed that the recurrence rate was relatively higher in patients with malignant, cervical, thoracic and lumbosacral, intramedullary tumors, and higher McCormick grade and patient received partial resection or biopsy. Tumor property, tumor location, McCormick grade, tumor resection, and intramedullary tumors are risk factors for the recurrence of spinal tumors. Clinical assessment of these risk factors may be helpful in selecting appropriate treatment strategies.

Logistic regression analysis of risk factors for postoperative recurrence of spinal tumors and analysis of prognostic factors

PubMed Central

Zhang, Shanyong; Yang, Lili; Peng, Chuangang; Wu, Minfei

2018-01-01

The aim of the present study was to investigate the risk factors for postoperative recurrence of spinal tumors by logistic regression analysis and analysis of prognostic factors. In total, 77 male and 48 female patients with spinal tumor were selected in our hospital from January, 2010 to December, 2015 and divided into the benign (n=76) and malignant groups (n=49). All the patients underwent microsurgical resection of spinal tumors and were reviewed regularly 3 months after operation. The McCormick grading system was used to evaluate the postoperative spinal cord function. Data were subjected to statistical analysis. Of the 125 cases, 63 cases showed improvement after operation, 50 cases were stable, and deterioration was found in 12 cases. The improvement rate of patients with cervical spine tumor, which reached 56.3%, was the highest. Fifty-two cases of sensory disturbance, 34 cases of pain, 30 cases of inability to exercise, 26 cases of ataxia, and 12 cases of sphincter disorders were found after operation. Seventy-two cases (57.6%) underwent total resection, 18 cases (14.4%) received subtotal resection, 23 cases (18.4%) received partial resection, and 12 cases (9.6%) were only treated with biopsy/decompression. Postoperative recurrence was found in 57 cases (45.6%). The mean recurrence time of patients in the malignant group was 27.49±6.09 months, and the mean recurrence time of patients in the benign group was 40.62±4.34. The results were significantly different (P<0.001). Recurrence was found in 18 cases of the benign group and 39 cases of the malignant group, and results were significantly different (P<0.001). Tumor recurrence was shorter in patients with a higher McCormick grade (P<0.001). Recurrence was found in 13 patients with resection and all the patients with partial resection or biopsy/decompression. The results were significantly different (P<0.001). Logistic regression analysis of total resection-related factors showed that total resection should be the preferred treatment for patients with benign tumors, thoracic and lumbosacral tumors, and lower McCormick grade, as well as patients without syringomyelia and intramedullary tumors. Logistic regression analysis of recurrence-related factors revealed that the recurrence rate was relatively higher in patients with malignant, cervical, thoracic and lumbosacral, intramedullary tumors, and higher McCormick grade and patient received partial resection or biopsy. Tumor property, tumor location, McCormick grade, tumor resection, and intramedullary tumors are risk factors for the recurrence of spinal tumors. Clinical assessment of these risk factors may be helpful in selecting appropriate treatment strategies. PMID:29434866

Posterior odontoid process angulation in pediatric Chiari I malformation: an MRI morphometric external validation study.

PubMed

Ladner, Travis R; Dewan, Michael C; Day, Matthew A; Shannon, Chevis N; Tomycz, Luke; Tulipan, Noel; Wellons, John C

2015-08-01

OBJECT Osseous anomalies of the craniocervical junction are hypothesized to precipitate the hindbrain herniation observed in Chiari I malformation (CM-I). Previous work by Tubbs et al. showed that posterior angulation of the odontoid process is more prevalent in children with CM-I than in healthy controls. The present study is an external validation of that report. The goals of our study were 3-fold: 1) to externally validate the results of Tubbs et al. in a different patient population; 2) to compare how morphometric parameters vary with age, sex, and symptomatology; and 3) to develop a correlative model for tonsillar ectopia in CM-I based on these measurements. METHODS The authors performed a retrospective review of 119 patients who underwent posterior fossa decompression with duraplasty at the Monroe Carell Jr. Children's Hospital at Vanderbilt University; 78 of these patients had imaging available for review. Demographic and clinical variables were collected. A neuroradiologist retrospectively evaluated preoperative MRI examinations in these 78 patients and recorded the following measurements: McRae line length; obex displacement length; odontoid process parameters (height, angle of retroflexion, and angle of retroversion); perpendicular distance to the basion-C2 line (pB-C2 line); length of cerebellar tonsillar ectopia; caudal extent of the cerebellar tonsils; and presence, location, and size of syringomyelia. Odontoid retroflexion grade was classified as Grade 0, > 90°; Grade I,85°-89°; Grade II, 80°-84°; and Grade III, < 80°. Age groups were defined as 0-6 years, 7-12 years, and 13-17 years at the time of surgery. Univariate and multivariate linear regression analyses, Kruskal-Wallis 1-way ANOVA, and Fisher's exact test were performed to assess the relationship between age, sex, and symptomatology with these craniometric variables. RESULTS The prevalence of posterior odontoid angulation was 81%, which is almost identical to that in the previous report (84%). With increasing age, the odontoid height (p < 0.001) and pB-C2 length (p < 0.001) increased, while the odontoid process became more posteriorly inclined (p = 0.010). The pB-C2 line was significantly longer in girls (p = 0.006). These measurements did not significantly correlate with symptomatology. Length of tonsillar ectopia in pediatric CM-I correlated with an enlarged foramen magnum (p = 0.023), increasing obex displacement (p = 0.020), and increasing odontoid retroflexion (p < 0.001). CONCLUSIONS Anomalous bony development of the craniocervical junction is a consistent feature of CM-I in children. The authors found that the population at their center was characterized by posterior angulation of the odontoid process in 81% of cases, similar to findings by Tubbs et al. (84%). The odontoid process appeared to lengthen and become more posteriorly inclined with age. Increased tonsillar ectopia was associated with more posterior odontoid angulation, a widened foramen magnum, and an inferiorly displaced obex.

Association of Chiari malformation type I and tethered cord syndrome: preliminary results of sectioning filum terminale☆

PubMed Central

Milhorat, Thomas H.; Bolognese, Paolo A.; Nishikawa, Misao; Francomano, Clair A.; McDonnell, Nazli B.; Roonprapunt, Chan; Kula, Roger W.

2018-01-01

Objective The pathogenesis of CM-I is incompletely understood. We describe an association of CM-I and TCS that occurs in a subset of patients with normal size of the PCF. Methods The prevalence of TCS was determined in a consecutively accrued cohort of 2987 patients with CM-I and 289 patients with low-lying cerebellar tonsils (LLCT). Findings in 74 children and 244 adults undergoing SFT were reviewed retrospectively. Posterior cranial fossa size and volume were measured using reconstructed 2D computed tomographic scans and MR images. Results were compared to those in 155 age- and sex-matched healthy control individuals and 280 patients with generic CM-I. The relationships of neural and osseus structures at the CCJ and TLJ were investigated morphometrically on MR images. Intraoperative CDU was used to measure anatomical structures and CSF flow in the lumbar theca. Results Tethered cord syndrome was present in 408 patients with CM-I (14%) and 182 patients with LLCT (63%). In 318 patients undergoing SFT, there were no significant differences in the size or volume of the PCF as compared to healthy control individuals. Morphometric measurements demonstrated elongation of the brain stem (mean, 8.3 mm; P < .001), downward displacement of the medulla (mean, 4.6 mm; P < .001), and normal position of the CMD except in very young patients. Compared to patients with generic CM-I, the FM was significantly enlarged (P < .001). The FT was typically thin and taut (mean transverse diameter, 0.8 mm). After SFT, the cut ends of the FT distracted widely (mean, 41.7 mm) and CSF flow in the lumbar theca increased from a mean of 0.7 cm/s to a mean of 3.7 cm/s (P < .001). Symptoms were improved or resolved in 69 children (93%) and 203 adults (83%) and unchanged in 5 children (7%) and 39 adults (16%) and, worse, in 2 adults (1%) over a follow-up period of 6 to 27 months (mean, 16.1 months ± 4.6 SD). Magnetic resonance imaging 1 to 18 months after surgery (mean, 5.7 months ± 3.8 SD) revealed upward migration of the CMD (mean, 5.1 mm, P < .001), ascent of the cerebellar tonsils (mean, 3.8 mm, P < .001), reduction of brain stem length (mean, 3.9 mm, P < .001), and improvement of scoliosis or syringomyelia in some cases. Conclusions Chiari malformation type I/TCS appears to be a unique clinical entity that occurs as a continuum with LLCT/TCS and is distinguished from generic CM-I by enlargement of the FM and the absence of a small PCF. Distinctive features include elongation and downward displacement of the hindbrain, normal position of the CMD, tight FT, and reduced CSF flow in the lumbar theca. There is preliminary evidence that SFT can reverse moderate degrees of tonsillar ectopia and is appropriate treatment for cerebellar ptosis after Chiari surgery in this cohort. PMID:19559924