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Sample records for chiari malformation syringomyelia

  1. Syringomyelia associated with Chiari malformation in children

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    Sakamoto, Hiroaki; Kitano, Shouhei; Nishikawa, Misao; Yasui, Toshihiro [Osaka City General Hospital (Japan); Fujitani, Ken; Hakuba, Akira; Nakanishi, Naruhiko

    1997-02-01

    Among 28 patients with myelomeningocele (MMC group), the myelomeningocele in all patients was repaired shortly after birth, and a shunt was implanted for the associated hydrocephalus in 18 patients. MRI of the group of 28 indicated 20 were afflicted with Chiari II malformation, and the remaining 8 by Chiari I malformation. Among 8 patients lacking myelomeningocele (non-MMC group), seven demonstrated a large syrinx at the cervical and cervico-thoracic level; only one had a syrinx extending from the cervical level down to the lumbar level. None of these patients had hydrocephalus. Surgical decompression to improve cerebrospinal fluid (CSF) flow at the major cistern improved neurological signs in 7 patients. MRI indicated 4 patients were afflicted with Chiari I malformation, and the remaining 4 with Chiari II malformation. In the MMC group, the initial development of the syrinx at the lumbar level may be the result of a combination of occlusion of the caudal end of the central canal brought about by repair of the myelomeningocele and CSF flow into the hydromyelic cavity via the patent proximal portion of the central canal. In the non-MMC group, the syringomyelia may be considered an early onset type of syringomyelia associated with adult type Chiari malformation because the location of the syrinx was quite similar to that found in adult type Chiari malformation, and decompressive surgery was quite effective. In the non-MMC group, turbulence of the CSF now at the major cistern caused by the herniated cerebellum plays an important role in the enlargement of the syringomyelia. To offer greater appropriate management of pediatric Chiari malformation accompanied by syringomyelia, the malformation should be classified not by degree of the herniated brain tissue but by its association with neural tube defect (myelomeningocele). (K.H.)

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

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    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.

  3. Clinical Presentation of Chiari I Malformation and Syringomyelia in Children.

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    Pindrik, Jonathan; Johnston, James M

    2015-10-01

    Chiari I malformation and syringomyelia may be associated with a wide spectrum of symptoms and signs in children. Clinical presentations vary based on patient age and relative frequency; some diagnoses represent incidental radiographic findings. Occipitocervical pain, propagated or intensified by Valsalva maneuvers (or generalized irritability in younger patients unable to communicate verbally), and syringomyelia with or without scoliosis are the most common clinical presentations. Cranial nerve or brainstem dysfunction also may be observed in younger patients, and is associated with more complex deformity that includes ventral compression secondary to basilar invagination, retroflexion of the dens, and/or craniocervical instability.

  4. Prevalence of Chiari I Malformation and Syringomyelia.

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    Kahn, Elyne N; Muraszko, Karin M; Maher, Cormac O

    2015-10-01

    Chiari I malformation (CM) is a common neurosurgical diagnosis and spinal cord syrinx is frequently found in patients with CM. Asymptomatic CM is a common imaging finding. Symptomatic CM is less common. Variation in prevalence estimates may be attributed to differences in sensitivity of CM detection between studies as well as differences in the populations being analyzed. The prevalence of low tonsil position and CM on MRI is higher in children and young adults compared with older adults. Studies that include a large number of older adults find a lower prevalence compared with analyses of children.

  5. A rare course of scoliosis associated with Chiari malformation and syringomyelia.

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    Tanaka, Masato; Sugimoto, Yoshihisa; Arataki, Shinya; Takigawa, Tomoyuki; Ozaki, Toshifumi

    2014-01-01

    Spinal deformity is an important clinical manifestation of Chiari I malformation (CM-I) and syringomyelia. Here we report the result of an 8-year follow-up of a 13-year-old girl with severe scoliosis associated with Chiari malformation and a large syringomyelia. The patient presented at our hospital at the age of 13 with a 68° scoliosis. Magnetic resonance imaging showed Chiari malformation and a large syringomyelia. Neurosurgical treatment involved foramen magnum decompression and partial C1 laminectomy, but the scoliosis still progressed. We present the first case report of a rare course of scoliosis in a patient with CM-I and a large syringomyelia.

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

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    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.

  7. Chiari 0 malformation with syringomyelia syringobulbia and syrinx cavity in pons

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    Yuan Zhou; Handong Wang; Ning Li; Yixing Lin; Lin Zhu; Huilin Cheng

    2016-01-01

    Chiari 0 malformation (CM-0) with syringomyelia syringobulbia and syrinx cavity in pons simultaneously is rare. We present a case of 31-year-old man, review the available literature, and discuss the experience of therapy from the neurosurgical perspective.

  8. Chiari-like malformation and syringomyelia in cavalier King Charles spaniels

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    Rusbridge, C.

    2007-01-01

    This thesis is the cumulation of over a decade of study into the pathogenesis and treatment Chiari-like malformation and syringomyelia (CM/SM) in the cavalier King Charles spaniel (CKCS). Chiari-like malformation is a condition where the brain is too big for the skull and is crushed and pushed out t

  9. Difficult intubation in a parturient with syringomyelia and Arnold-Chiari malformation: Use of Airtraq™ laryngoscope

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    Bensghir Mustapha

    2011-01-01

    Full Text Available Anesthetic technique in parturient with syringomyelia and Arnold-Chiari malformation is variable depending on the teams. Difficult intubation is one of the risks when general anesthesia is opted. Different devices have been used to manage the difficult intubation in pregnant women. We report the use of Airtraq™ laryngoscope after failed standard laryngoscopy in a parturient with syringomyelia and Arnold-Chiari type I malformation.

  10. Surgical treatment of Chiari I malformation complicated with syringomyelia

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    BAO, CHANGSHUN; YANG, FUBING; LIU, LIANG; WANG, BING; LI, DINGJUN; GU, YINGJIANG; ZHANG, SHULING; CHEN, LIGANG

    2013-01-01

    The aim of this study was to evaluate the curative effects of various surgical procedures on Chiari I malformation (CMI) complicated with syringomyelia. A total of 185 patients with CMI complicated with syringomyelia who received treatment between January 1997 and December 2011 were recruited. All patients underwent posterior fossa decompression in which the lamina of the first cervical vertebra was removed, with the removal of the second or third depending on the severity of the cerebellar tonsil herniation. Of the patients, 76 underwent large-bone-window decompression and duraplasty, while 109 underwent small-bone-window decompression, displaced cerebellar tonsil resection and duraplasty. The curative effects of the different surgical procedures were analyzed retrospectively. Clinical symptoms were eliminated or improved in 156 patients (84.3%) by the time of discharge from hospital. A total of 148 patients were evaluated using magnetic resonance imaging (MRI) which revealed that the cisterna magna was reconstructed in 92 patients and spinal syrinx was reduced in 75. Follow-up was performed on 147 patients (79.5%) for between 3 months and 12 years. During the follow-up, symptoms were eliminated or improved in 110 patients (74.8%), not improved in 26 (17.7%) and deteriorated in 11 (7.5%). MRI was performed on 95 patients during follow-up examinations and the cisterna magna was reconstructed in 87 patients and spinal syrinx was reduced in 79. Small-bone-window decompression plus duraplasty is an effective surgical procedure for treating CMI complicated with syringomyelia and intraoperative cerebellar tonsillectomy significantly aids patient recovery. PMID:23251294

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

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    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.

  12. American Syringomyelia & Chiari Alliance Project

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    ... Friends: For those living with Chiari malformation and syringomyelia — and the families and friends who support them — ... need, organized for easy retrieval. Conditions • Chiari MalformationSyringomyelia • Newsletter • Medical Articles • Related Disorders • BJO Scholarships • Patient ...

  13. Clinical and neuroradiological features of syringomyelia associated with Chiari malformation

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    Isu, Toyohiko; Iwasaki, Yoshinobu; Akino, Minoru; Abe, Hiroshi; Tashiro, Kunio; Sudo, Kazumasa; Miyasaka, Kazuo (Hokkaido Univ., Sapporo (Japan). School of Medicine); Saito, Hisatoshi

    1990-01-01

    The clinical presentation and radiological features were analyzed in 30 cases of syringomyelia associated with Chiari malformation. None of the patients had spinal dysraphism. The age on admission ranged from 6 to 59 years with a mean of 27 years. Syringomyelia was diagnosed by CT myelography and or MRI from 1982 to 1988. The initial symptoms were skeletal abnormality (43%) such as scolisis (12 cases) or pescavus (one case), unilateral pain or numbness (40%) and unilateral motor weakness (17%). Frequently seen signs on admission were sensory deficit (100%), scoliosis (57%), muscle weakness (57%), muscle atrophy (37%) and lower cranial nerve palsy (40%). The neurological findings were asymmetrical in all patients. The characteristic neurological findings in the cases presenting under 20 years of age were unilateral sensory and motor deficits (61%) with decreased or absent deep tendon reflex on the same side. The localization of the syrinx in axial section varied according to the level even in the same case. In 15 cases with unilateral sensory disturbance or unilateral sensory and motor deficit, the syrinx was located in the region corresponding to the posterolateral portion on the same side as that of sensory disturbance in the cervical or thoracic level. On the other hand, in 15 cases with bilateral sensory and motor deficit, the syrinx was located in the central portion and extended into the posterolateral portion of the more affected side. The authors think that the syrinx which originates from the unilateral posterolateral portion, extends to involve the gray matter around the central canal and the posterolateral portion of the other side with progression of the syrinx to cause bilateral disturbance. As a result, unilateral neurological symptoms also become bilateral with progression of the syrinx. (author).

  14. Volumetric analysis of syringomyelia following hindbrain decompression for Chiari malformation Type I: syringomyelia resolution follows exponential kinetics

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    Coumans, Jean-Valery; Walcott, Brian P.; Butler, William E.; Nahed, Brian V.; Kahle, Kristopher T.

    2013-01-01

    Object Resolution of syringomyelia is common following hindbrain decompression for Chiari malformation, yet little is known about the kinetics governing this process. The authors sought to establish the volumetric rate of syringomyelia resolution. Methods A retrospective cohort of patients undergoing hindbrain decompression for a Chiari malformation Type I with preoperative cervical or thoracic syringomyelia was identified. Patients were included in the study if they had at least 3 neuroimaging studies that detailed the entirety of their preoperative syringomyelia over a minimum of 6 months postoperatively. The authors reconstructed the MR images in 3 dimensions and calculated the volume of the syringomyelia. They plotted the syringomyelia volume over time and constructed regression models using the method of least squares. The Akaike information criterion and Bayesian information criterion were used to calculate the relative goodness of fit. The coefficients of determination R2 (unadjusted and adjusted) were calculated to describe the proportion of variability in each individual data set accounted for by the statistical model. Results Two patients were identified as meeting inclusion criteria. Plots of the least-squares best fit were identified as 4.01459e−0.0180804x and 13.2556e−0.00615859x. Decay of the syringomyelia followed an exponential model in both patients (R2 = 0.989582 and 0.948864). Conclusions Three-dimensional analysis of syringomyelia resolution over time enables the kinetics to be estimated. This technique is yet to be validated in a large cohort. Because syringomyelia is the final common pathway for a number of different pathological processes, it is possible that this exponential only applies to syringomyelia related to treatment of Chiari malformation Type I. PMID:21882909

  15. Charcot arthropathy of the elbow joint as a presenting feature of Chiari malformation with syringomyelia.

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    Sahoo, Sushanta K; Salunke, Pravin

    2014-12-01

    Charcot arthropathy of the elbow joint is occasionally seen with Chiari malformation with syringomyelia, but rarely as a presenting feature as in the reported case. The treatment is directed toward its underlying cause to halt its progression. Thus, it is important to diagnose the cause as early as possible.

  16. Chiari 0 malformation with syringomyelia syringobulbia and syrinx cavity in pons

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    Yuan Zhou

    2016-12-01

    Full Text Available Chiari 0 malformation (CM-0 with syringomyelia syringobulbia and syrinx cavity in pons simultaneously is rare. We present a case of 31-year-old man, review the available literature, and discuss the experience of therapy from the neurosurgical perspective.

  17. Quantitative analysis of Chiari-like malformation and syringomyelia in the Griffon Bruxellois dog.

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    Susan P Knowler

    Full Text Available 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.

  18. Cerebrospinal fluid dynamics in Chiari malformation associated with syringomyelia

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    LIU Bin; WANG Zhen-yu; XIE Jing-cheng; HAN Hong-bin; PEI Xin-long

    2007-01-01

    Background About 50%-70% of patients with Chiari malformation I (CMI) presented with syringomyelia (SM), which is supposed to be related to abnormal cerebrospinal fluid (CSF) flow around the foramen magnum. The aim of this study was to investigate the cerebrospinal fluid dynamics at levels of the aqueduct and upper cervical spine in patients with CMI associated with SM, and to discuss the possible mechanism of formation of SM.Methods From January to April 2004, we examined 10 adult patients with symptomatic CMI associated with SM and 10 healthy volunteers by phase-contrast MRI. CSF flow patterns were evaluated at seven regions of interest (ROI): the aqueduct and ventral and dorsal subarachnoid spaces of the spine at levels of the cerebellar tonsil, C2-3, and C5-6. The CSF flow waveforms were analyzed by measuring CSF circulation time, durations and maximum velocities of cranial- and caudal-directed flows, and the ratio between the two maximum velocities. Data were analyzed by ttest using SPSS 11.5.Results We found no definite communication between the fourth ventricle and syringomyelia by MRI in the 10 patients.In both the groups, we observed cranial-directed flow of CSF in the early cardiac systolic phase, which changed the direction from cranial to caudal from the middle systolic phase to the early diastolic phase, and then turned back in cranial direction in the late diastolic phase. The CSF flow disappeared at the dorsal ROI at the level of C2-3 in 3 patients and 1 volunteer, and at the level of C5-6 in 6 patients and 3 volunteers. The durations of CSF circulation at all the ROIs were significantly shorter in the patients than those in the healthy volunteers (P=0.014 at the midbrain aqueduct, P=0.019 at the inferior margin of the cerebellar tonsil, P=0.014 at the level of C2-3, and P=0.022 at the level of C5-6). No significant difference existed between the two groups in the initial point and duration of the caudal-directed CSF flow during a cardiac cycle at

  19. Inheritance of Chiari-Like Malformation: Can a Mixed Breeding Reduce the Risk of Syringomyelia?

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    Susan P Knowler; Henny v/d Berg; Angus McFadyen; La Ragione, Roberto M; Clare Rusbridge

    2016-01-01

    Canine Chiari-like malformation (CM) is a complex abnormality of the skull and craniocervical junction associated with miniaturization and brachycephaly which can result in the spinal cord disease syringomyelia (SM). This study investigated the inheritance of CM in a Griffon Bruxellois (GB) family and feasibility of crossbreeding a brachycephalic CM affected GB with a mesaticephalic normal Australian terrier and then backcrossing to produce individuals free of the malformation and regain GB b...

  20. Symptomatic Chiari Malformation with Syringomyelia after Severe Traumatic Brain Injury: Case Report

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    Luis Rafael Moscote-Salazar; Nasly Zabaleta-Churio; Gabriel Alcala-Cerra; Andres M. Rubiano; Willem Guillermo Calderon-Miranda; Hernando Raphael Alvis-Miranda; Amit Agrawal

    2016-01-01

    Chiari malformation Type I (CM-I) is a congenital disorder, which is basically a tonsillar herniation (≥ 5 mm) below the foramen magnum with or without syringomyelia. The real cause behind this malformation is still unknown. Patients may remain asymptomatic until they engender a deteriorating situation, such as cervical trauma. The objective of this case report is to give a broad perspective on CM-I from the clinical findings obtained in a patient with asymptomatic non-communicating syringomy...

  1. Symptomatic Chiari Malformation with Syringomyelia after Severe Traumatic Brain Injury: Case Report

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    Luis Rafael Moscote-Salazar

    2016-01-01

    Full Text Available Chiari malformation Type I (CM-I is a congenital disorder, which is basically a tonsillar herniation (≥ 5 mm below the foramen magnum with or without syringomyelia. The real cause behind this malformation is still unknown. Patients may remain asymptomatic until they engender a deteriorating situation, such as cervical trauma. The objective of this case report is to give a broad perspective on CM-I from the clinical findings obtained in a patient with asymptomatic non-communicating syringomyelia associated with a CM-I exacerbated within 2 years of a TBI, and to discuss issues related to that condition.

  2. Symptomatic Chiari Malformation with Syringomyelia after Severe Traumatic Brain Injury: Case Report.

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    Moscote-Salazar, Luis Rafael; Zabaleta-Churio, Nasly; Alcala-Cerra, Gabriel; M Rubiano, Andres; Calderon-Miranda, Willem Guillermo; Alvis-Miranda, Hernando Raphael; Agrawal, Amit

    2016-01-01

    Chiari malformation Type I (CM-I) is a congenital disorder, which is basically a tonsillar herniation (≥ 5 mm) below the foramen magnum with or without syringomyelia. The real cause behind this malformation is still unknown. Patients may remain asymptomatic until they engender a deteriorating situation, such as cervical trauma. The objective of this case report is to give a broad perspective on CM-I from the clinical findings obtained in a patient with asymptomatic non-communicating syringomyelia associated with a CM-I exacerbated within 2 years of a TBI, and to discuss issues related to that condition.

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

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    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.

  4. Computational fluid dynamics modelling of cerebrospinal fluid pressure in Chiari malformation and syringomyelia.

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    Clarke, Elizabeth C; Fletcher, David F; Stoodley, Marcus A; Bilston, Lynne E

    2013-07-26

    The pathogenesis of syringomyelia in association with Chiari malformation (CM) is unclear. Studies of patients with CM have shown alterations in the CSF velocity profile and these could contribute to syrinx development or enlargement. Few studies have considered the fluid mechanics of CM patients with and without syringomyelia separately. Three subject-specific CFD models were developed for a normal participant, a CM patient with syringomyelia and a CM patient without syringomyelia. Model geometries, CSF flow rate data and CSF velocity validation data were collected from MRI scans of the 3 subjects. The predicted peak CSF pressure was compared for the 3 models. An extension of the study performed geometry and flow substitution to investigate the relative effects of anatomy and CSF flow profile on resulting spinal CSF pressure. Based on 50 monitoring locations for each of the models, the CM models had significantly higher magnitude (psyringomyelia mechanisms and relative effects of CSF velocity profile and spinal geometry on CSF pressure.

  5. Imaging Findings in Chiari I Malformation with Syringomyelia in a Case of Charcot Shoulder

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    Shantanu Kumar

    2011-01-01

    Full Text Available Neuropathic arthropathy of the shoulder is reported in only 5% of cases. Here, we report a rare case of neuropathic arthropathy of the shoulder, secondary to Chiari malformation Type I with associated syringomyelia, that remained undetected for four years. A 38-year-old female presented to our Department with a swelling over the right shoulder that had persisted for four years. X-ray of the joint showed destruction of the head of the right humerus, with typical blunt amputated appearance of the bone and increased joint space. Magnetic resonance imaging showed destruction and lateral dislocation of the head of the humerus. Large amount of fluid collection was seen in and around the right shoulder joint. Neuropathic osteoarthropathy can be defined as bone and joint changes that occurs secondary to loss of sensation. In our case, neuropathic shoulder joint was secondary to syringomyelia associated with Chiari I malformation.

  6. Clinical and neuroimaging features of Chiari type I malformations with and without associated syringomyelia

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    Yamazaki, Yoshinori; Tachibana, Shigekuni; Takano, Makoto; Fujii, Kiyotaka [Kitazato Univ., Sagamihara, Kanagawa (Japan)

    1998-09-01

    The clinical and neuroimaging characteristics of 22 consecutive patients with Chiari type I malformations were evaluated to investigate the pathogenesis of syrinx formation. All patients underwent magnetic resonance imaging and x-ray tomography before surgery. The electric manometric Queckenstedt test was performed on 16 patients pre- and postoperatively. Syringomyelia was present in 17 patients and absent in five patients. All patients without syringomyelia suffered from foramen magnum compression syndrome, with a wider basal angle, more acute clivo-axial angle, shorter clivus, and more prominent tonsillar ectopia than patients with syringomyelia. Low brain stem position, basilar impression, and beaking of the cervicomedullary junction were also more prominent in patients without syringomyelia. Marked to complete block with the neck in flexed position by Queckenstedt test was present in all patients except one. Patients with Chiari malformation not associated with syringomyelia have more pronounced compression of the brain stem at the foramen magnum. Therefore, despite a block of the cerebrospinal fluid pathway at the foramen magnum, syrinx formation may be prevented by severe compression. (author)

  7. Anesthesia for a patient of acromesomelic dysplasia with associated hydrocephalus, Arnold Chiari malformation and syringomyelia

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    Rudrashish Haldar

    2013-01-01

    Full Text Available Acromesomelic dysplasias are autosomal recessive osteochondrodysplasias. Acromesomelic dysplasia Maroteaux-type (AMDM, also known as St Helena dysplasia, is of two types: The classical and the mild variety. About 50 cases of AMDM have been reported till date, most of them being the classical variety. There is scarcity of literature on anesthesia for such patients. We are reporting a case of general anesthetic management of AMDM, associated with hydrocephalus, Arnold Chiari malformation type-1 and syringomyelia. The patient was a 10-year-old short-statured boy who presented with symptomatic thoracic kyphoscoliosis, gibbus deformity and back pain. On examination, there was no neurological deficit. Radiology revealed thoracic kyphoscoliosis, mild ventriculomegaly and upper cervical syringomyelia. The patient underwent posterior fossa decompression in the prone position under general anesthesia. We will discuss the anesthetic considerations for such patients and review the pertinent literature.

  8. A patient with optic pathway glioma, scoliosis, Chiari type I malformation and syringomyelia : is it Neurofibromatosis type 1?

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    Chakravarty A

    2002-10-01

    Full Text Available A 22 years old girl had features of optic pathway glioma, scoliosis, Chiari type 1 malformation and cervical syringomyelia. She had no cutaneous lesions. We considered this combination to be more than coincidental and argue in favour of considering the case as a variant form of Neurofibromatosis type 1. The relevent literature in favour of our contention has been reviewed.

  9. Factors for surgery indications in children with Chiari malformation Type I combined with scoliosis without syringomyelia

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    Glagolev N.V.

    2014-09-01

    Full Text Available Objective: Specification of indications for surgical treatment of Chiari malformations Type I (CMI in combination with scoliosis without syringomyelia. Material et Methods: The article describes the results of treatment of children with Chiari malformations Type I (CMI in combination with scoliosis without syringomyelia. The decompression surgery of the posterior fossa was made in 30 patients with CM I. All patients varied in the degrees of scoliosis. Results were evaluated including clinical and radiological survey, data of computer angiography and ultrasonic diagnostics of head and neck vessels in the preoperative and postoperative periods. The behavior of the angle of the spinal deformity in patients after decompression surgery of the posterior fossa was evaluated. Results: It has been found out that scoliosis convincingly regresses after this operation, if the angle of curvature of less than 30 degrees. Conclusion: The presence of vascular pathology at the level of the cranio-vertebral joints should be taken into account in the determination of indications for surgical treatment of patients with CMI in combination with scoliosis.

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

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    García-Ramos Rocío

    2009-12-01

    Full Text Available Abstract 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.

  11. An update on the pathogenesis of syringomyelia secondary to Chiari-like malformations in dogs.

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    Driver, C J; Volk, H A; Rusbridge, C; Van Ham, L M

    2013-12-01

    Syringomyelia (SM) is a spinal cord disease that can cause neuropathic pain in dogs. The pathogenesis of SM secondary to Chiari-like malformation (CM) has been the focus of intense research in recent years. The gulf in our understanding of CM/SM in dogs relative to the analogous human condition has progressively narrowed. CM is primarily a disease of abnormal geometric morphometry affecting the caudal cranial fossa and the brain parenchyma contained within it. This review describes how advanced imaging techniques have revealed a series of morphometric abnormalities associated with CM/SM. The series is presented in a logical order to help describe the pathogenesis of CM and the subsequent formation of syringes, with particular reference to the concepts of craniospinal compliance and cerebrospinal fluid pulse pressure timing.

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

    Science.gov (United States)

    Tieppo Francio, Vinicius

    2014-11-09

    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.

  13. High-field MR of syringomyelia associated with Chiari I malformation

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    Miyasaka, Kazuo; Terae, Satoshi; Takahashi, Chihiro; Abe, Satoru; Abe, Hiroshi; Tashiro, Kunio (Hokkaido Univ., Sapporo (Japan). School of Medicine)

    1992-12-01

    Forty-seven patients with syringomyelia associated with Chiari-type I malformation were studied by means of high field MRI. A swelling of the spinal cord was observed in 65% of them, with the frequency significantly increasing with the degree of foramen magnum stenosis. A spontaneous regression of the syrinx was recognized in 2 out of the 9 patients whose tonsils were elevated. These observations indicate that stenosis of the foramen magnum plays a key role in the progression of syringomyelia; thus, foramen-magnum decompression can reasonably be accepted as a surgical method of treatment of the disease. The syrinx extended above the C1 in 16% and below the lumbar enlargement in only 5%. Septum formation in the syrinx was found in 24%, while an eccentric location of the syrinx was very frequent (97%). A lack of spinal-cord swelling and septum formation and an eccentric location of the syrinx should be taken into consideration when a syringo-subarachnoid shunt is attempted. (author).

  14. Factors contributing to improvement of syringomyelia after foramen magnum decompression for Chiari type I malformation.

    Science.gov (United States)

    Nagoshi, Narihito; Iwanami, Akio; Toyama, Yoshiaki; Nakamura, Masaya

    2014-05-01

    Although various surgical approaches have been proposed for treating syringomyelia associated with Chiari type I malformation, a standard method has yet to be established. we prospectively investigated the results of our surgical method: foramen magnum decompression combined with C1 laminectomy and excision of the outer layer of the dura mater. Twenty patients underwent surgery between 2000 and 2010 at our hospital. After surgery, the size of the syrinx decreased in 11 patients (decreased group) but remained unchanged in nine patients (unchanged group). The following parameters were compared: age at the time of surgery, duration of morbidity, improvement of preoperative symptoms, morphological type and length of the syrinx, presence or absence of scoliosis, cervical alignment, basal and clivo-axial angles, and postoperative subarachnoid space at the foramen magnum level. Preoperative symptoms improved in all patients in the decreased group but in only one patient in the unchanged group. The average duration of morbidity was significantly shorter in the decreased group. Morphological examination revealed that the size of all central-type syrinxes decreased after surgery, whereas in all cases of deviated-type syrinx, size was unchanged. The average length of preoperative syrinx was significantly shorter in the decreased group. The postoperative subarachnoid space at the foramen magnum was enlarged in the entire decreased group, whereas residual narrowing of the space was observed in 44 % of patients in the unchanged group. No significant intergroup differences were observed in the other factors. In patients with syringomyelia, a longer and deviated type of syrinx, a longer duration of morbidity, and postoperative residual narrowing of the subarachnoid space are associated with a poor prognosis after the surgical procedure. The pathogenesis of syringomyelia is inconsistent, and the choice of surgical technique for each pathological condition is important.

  15. Magnetic resonance 4D flow analysis of cerebrospinal fluid dynamics in Chiari I malformation with and without syringomyelia

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    Bunck, Alexander C. [University Hospital of Muenster, Department of Clinical Radiology, Muenster (Germany); University of Cologne, Department of Radiology, Cologne (Germany); Kroeger, Jan Robert; Juettner, Alena; Heindel, Walter; Schwindt, Wolfram; Niederstadt, Thomas [University Hospital of Muenster, Department of Clinical Radiology, Muenster (Germany); Brentrup, Angela [University Hospital of Muenster, Department of Neurosurgery, Muenster (Germany); Fiedler, Barbara [University Hospital of Muenster, Department of General Pediatrics, Muenster (Germany); Crelier, Gerard R. [ETH and University of Zurich, Institute for Biomedical Engineering, Zurich (Switzerland); Martin, Bryn A. [Ecole Polytechnique Federale de Lausanne, Laboratory of Hemodynamics and Cardiovascular Technology, School of Engineering, Interfaculty Institute of Bioengineering, Lausanne (Switzerland); Maintz, David [University Hospital of Muenster, Department of Clinical Radiology, Muenster (Germany); University Hospital of Cologne, Department of Radiology, Cologne (Germany)

    2012-09-15

    To analyse cerebrospinal fluid (CSF) hydrodynamics in patients with Chiari type I malformation (CM) with and without syringomyelia using 4D magnetic resonance (MR) phase contrast (PC) flow imaging. 4D-PC CSF flow data were acquired in 20 patients with CM (12 patients with presyrinx/syrinx). Characteristic 4D-CSF flow patterns were identified. Quantitative CSF flow parameters were assessed at the craniocervical junction and the cervical spinal canal and compared with healthy volunteers and between patients with and without syringomyelia. Compared with healthy volunteers, 17 CM patients showed flow abnormalities at the craniocervical junction in the form of heterogeneous flow (n = 3), anterolateral flow jets (n = 14) and flow vortex formation (n = 5), most prevalent in patients with syringomyelia. Peak flow velocities at the craniocervical junction were significantly increased in patients (-15.5 {+-} 11.3 vs. -4.7 {+-} 0.7 cm/s in healthy volunteers, P < 0.001). At the level of C1, maximum systolic flow was found to be significantly later in the cardiac cycle in patients (30.8 {+-} 10.3 vs. 22.7 {+-} 4.1%, P < 0.05). 4D-PC flow imaging allowed comprehensive analysis of CSF flow in patients with Chiari I malformation. Alterations of CSF hydrodynamics were most pronounced in patients with syringomyelia. (orig.)

  16. A unifying hypothesis for hydrocephalus, Chiari malformation, syringomyelia, anencephaly and spina bifida

    Directory of Open Access Journals (Sweden)

    Williams Helen

    2008-04-01

    Full Text Available Abstract This work is a modified version of the Casey Holter Memorial prize essay presented to the Society for Research into Hydrocephalus and Spina Bifida, June 29th 2007, Heidelberg, Germany. It describes the origin and consequences of the Chiari malformation, and proposes that hydrocephalus is caused by inadequate central nervous system (CNS venous drainage. A new hypothesis regarding the pathogenesis, anencephaly and spina bifida is described. Any volume increase in the central nervous system can increase venous pressure. This occurs because veins are compressible and a CNS volume increase may result in reduced venous blood flow. This has the potential to cause progressive increase in cerebrospinal fluid (CSF volume. Venous insufficiency may be caused by any disease that reduces space for venous volume. The flow of CSF has a beneficial effect on venous drainage. In health it moderates central nervous system pressure by moving between the head and spine. Conversely, obstruction to CSF flow causes localised pressure increases, which have an adverse effect on venous drainage. The Chiari malformation is associated with hindbrain herniation, which may be caused by low spinal pressure relative to cranial pressure. In these instances, there are hindbrain-related symptoms caused by cerebellar and brainstem compression. When spinal injury occurs as a result of a Chiari malformation, the primary pathology is posterior fossa hypoplasia, resulting in raised spinal pressure. The small posterior fossa prevents the flow of CSF from the spine to the head as blood enters the central nervous system during movement. Consequently, intermittent increases in spinal pressure caused by movement, result in injury to the spinal cord. It is proposed that posterior fossa hypoplasia, which has origins in fetal life, causes syringomyelia after birth and leads to damage to the spinal cord in spina bifida. It is proposed that hydrocephalus may occur as a result of

  17. A unifying hypothesis for hydrocephalus, Chiari malformation, syringomyelia, anencephaly and spina bifida.

    Science.gov (United States)

    Williams, Helen

    2008-04-11

    This work is a modified version of the Casey Holter Memorial prize essay presented to the Society for Research into Hydrocephalus and Spina Bifida, June 29th 2007, Heidelberg, Germany. It describes the origin and consequences of the Chiari malformation, and proposes that hydrocephalus is caused by inadequate central nervous system (CNS) venous drainage. A new hypothesis regarding the pathogenesis, anencephaly and spina bifida is described.Any volume increase in the central nervous system can increase venous pressure. This occurs because veins are compressible and a CNS volume increase may result in reduced venous blood flow. This has the potential to cause progressive increase in cerebrospinal fluid (CSF) volume. Venous insufficiency may be caused by any disease that reduces space for venous volume. The flow of CSF has a beneficial effect on venous drainage. In health it moderates central nervous system pressure by moving between the head and spine. Conversely, obstruction to CSF flow causes localised pressure increases, which have an adverse effect on venous drainage.The Chiari malformation is associated with hindbrain herniation, which may be caused by low spinal pressure relative to cranial pressure. In these instances, there are hindbrain-related symptoms caused by cerebellar and brainstem compression. When spinal injury occurs as a result of a Chiari malformation, the primary pathology is posterior fossa hypoplasia, resulting in raised spinal pressure. The small posterior fossa prevents the flow of CSF from the spine to the head as blood enters the central nervous system during movement. Consequently, intermittent increases in spinal pressure caused by movement, result in injury to the spinal cord. It is proposed that posterior fossa hypoplasia, which has origins in fetal life, causes syringomyelia after birth and leads to damage to the spinal cord in spina bifida. It is proposed that hydrocephalus may occur as a result of posterior fossa hypoplasia, where

  18. Inheritance of Chiari-Like Malformation: Can a Mixed Breeding Reduce the Risk of Syringomyelia?

    Science.gov (United States)

    Knowler, Susan P; v/d Berg, Henny; McFadyen, Angus; La Ragione, Roberto M; Rusbridge, Clare

    2016-01-01

    Canine Chiari-like malformation (CM) is a complex abnormality of the skull and craniocervical junction associated with miniaturization and brachycephaly which can result in the spinal cord disease syringomyelia (SM). This study investigated the inheritance of CM in a Griffon Bruxellois (GB) family and feasibility of crossbreeding a brachycephalic CM affected GB with a mesaticephalic normal Australian terrier and then backcrossing to produce individuals free of the malformation and regain GB breed characteristics. The study family cohort (n = 27) included five founder dogs from a previous baseline study of 155 GB which defined CM as a global malformation of the cranium and craniocervical junction with a shortened skull base and increased proximity of the cervical vertebrae to the skull. T1-weighted sagittal DICOM images of the brain and craniocervical junction were analysed for five significant traits (two angles, three lines) identified from the previous study and subsequent Qualitative Trait Loci analysis. Mean measurements for mixed breed, pure-breed and baseline study groups were compared. Results indicated that mixed breed traits posed less risk for CM and SM and were useful to distinguish the phenotype. Moreover on the MR images, the filial relationships displayed by the traits exhibited segregation and those presenting the greatest risk for CM appeared additive towards the severity of the condition. The external phenotypes revealed that by outcrossing breed types and with careful selection of appropriate conformation characteristics in the first generation, it is possible to regain the GB breed standard and reduce the degree of CM. The four GB affected with SM in the study all exhibited reduced caudal skull development compared to their relatives. The craniocervical traits may be useful for quantifying CM and assessing the possibility of SM thus assisting breeders with mate selection. However, such a system requires validation to ensure appropriateness for

  19. Inheritance of Chiari-Like Malformation: Can a Mixed Breeding Reduce the Risk of Syringomyelia?

    Directory of Open Access Journals (Sweden)

    Susan P Knowler

    Full Text Available Canine Chiari-like malformation (CM is a complex abnormality of the skull and craniocervical junction associated with miniaturization and brachycephaly which can result in the spinal cord disease syringomyelia (SM. This study investigated the inheritance of CM in a Griffon Bruxellois (GB family and feasibility of crossbreeding a brachycephalic CM affected GB with a mesaticephalic normal Australian terrier and then backcrossing to produce individuals free of the malformation and regain GB breed characteristics. The study family cohort (n = 27 included five founder dogs from a previous baseline study of 155 GB which defined CM as a global malformation of the cranium and craniocervical junction with a shortened skull base and increased proximity of the cervical vertebrae to the skull. T1-weighted sagittal DICOM images of the brain and craniocervical junction were analysed for five significant traits (two angles, three lines identified from the previous study and subsequent Qualitative Trait Loci analysis. Mean measurements for mixed breed, pure-breed and baseline study groups were compared. Results indicated that mixed breed traits posed less risk for CM and SM and were useful to distinguish the phenotype. Moreover on the MR images, the filial relationships displayed by the traits exhibited segregation and those presenting the greatest risk for CM appeared additive towards the severity of the condition. The external phenotypes revealed that by outcrossing breed types and with careful selection of appropriate conformation characteristics in the first generation, it is possible to regain the GB breed standard and reduce the degree of CM. The four GB affected with SM in the study all exhibited reduced caudal skull development compared to their relatives. The craniocervical traits may be useful for quantifying CM and assessing the possibility of SM thus assisting breeders with mate selection. However, such a system requires validation to ensure

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

    Science.gov (United States)

    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.

  1. Changes over time in craniocerebral morphology and syringomyelia in cavalier King Charles spaniels with Chiari-like malformation

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    Driver Colin J

    2012-11-01

    Full Text Available Abstract Background Chiari-like malformation (CM and syringomyelia is a neurological disease complex with high prevalence in cavalier King Charles spaniels (CKCS. The natural progression of this disease with time has not been described. The objectives of this study were to i determine if syringomyelia progresses with time ii determine if features of craniocrebral morphology previously associated with CM are progressive (including caudal cranial fossa volume, caudal cranial fossa parenchymal volume, ventricular dimensions, height of the foramen magnum and degree of cerebellar herniation. A retrospective morphometric analysis was undertaken in 12 CKCS with CM for which repeat magnetic resonance images were available without surgical intervention. Results The maximal syrinx width, height of the foramen magnum, length of cerebellar herniation and caudal cranial fossa volume increased over time. Ventricular and caudal fossa parenchymal volumes were not significantly different between scans. Conclusions The results of this study suggest that syringomyelia progresses with time. Increased caudal cranial fossa volume may be associated with active resorption of the supraoccipital bone, which has previously been found in histology specimens from adult CKCS. We hypothesise that active resorption of the supraoccipital bone occurs due to pressure from the cerebellum. These findings have important implications for our understanding of the pathogenesis and variable natural clinical progression of CM and syringomyelia in CKCS.

  2. CSF dynamics in the patients with syringomyelia associated with Chiari's malformation; Quantitative analysis on cine MRI

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    Kuroda, Satoshi; Matsuzawa, Hitoshi; Iwasaki, Yoshinobu; Hida, Kazutoshi; Imamura, Hiroyuki; Abe, Hiroshi (Hokkaido Univ., Sapporo (Japan). School of Medicine); Saito, Hisatoshi

    1994-01-01

    In a series of 12 patients with syringomyelia associated with Chiari's malformation, the authors quantitatively analyzed cerebrospinal fluid (CSF) dynamics in the subarachnoid space of the cranio-spinal junction, using cine MRI combined with pre-saturation method. In most of subjects, cine MRI revealed (1) decreased or increased maximum velocity of CSF in the caudal direction and (2) disturbed CSF motion in the caudal direction (delayed % cardiac cycle) in the craniospinal junction, strongly suggesting disturbed CSF dynamics in the craniospinal junction because of the tonsilar herniation. Of 12 subjects, 8 patients underwent formen magnum decompression and 4 underwent syringo-subarachnoid shunt (SS shunt). In the patients who showed marked callapse of syrinx after foramen magnum decompression, follow-up cine MRI revealed the normalization of % cardiac cycle, representing postoperative improvement of CSF dynamics in the craniospinal function. On the other hand, % cardiac cycle did not improve significantly in the patients who did not show marked collapse of the syrinx or suffered from meningitis after surgery. Significant changes were not observed in the patients who underwent SS shunt. In summary, these results suggested that cine MRI combined with pre-saturation method could detect the pathophysiological changes and evaluate the efficacy of the surgery, especially foramen magnum decompression, in the patients with syringomyelia associated with Chiari's malformation. (author).

  3. The efficacy of intraoperative ultrasonography-assisted microinvasive cisterna magna reconstruction for Chiari malformation typeⅠwith syringomyelia

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    LI Peng-chao

    2012-08-01

    Full Text Available Objective To report the method and effect of intraoperative ultrasonography-assisted microinvasive cisterna magna reconstruction for Chiari malformation type Ⅰ with syringomyelia. Methods Ninty-three patients suffered from Chiari malformation typeⅠwith syringomyelia were treated by microinvasive cisterna magna reconstruction. The skin incision was 1.50-3.00 cm. The bone removal of foramen magnum was 1.50 cm × 2.00 cm with C1 reserved. Dura and arachnoid were incised and sutured linearly. All of the patients underwent cerebellar tonsillar resection and exploration of median aperture of fourth ventricle. Intraoperative ultrasonography was performed both before and after cerebellar tonsillar resection to judge the effect of cisterna magna reconstruction. According to Tator method, the curative effect was divided into 3 groups, improved, stable and worsen. MRI were reviewed at the same time, and the result was divided into syrinx disappeared, reduced, no change and expanded. Results The operation was successful in all patients. Postoperative complications included cerebellum hemorrhage (n = 1, cerebral infarction (n = 1, hydrocephalus (n = 1, subcutaneous dropsy (n = 2 and were recovered after specific treatment. All patients were followed up for 6 months to 12 months after operation. Thirty-six cases were improved, 55 cases were stable, and 2 cases got worse. The MRI showed that the syringomyelia shrinked or disappeared in 90 cases, no change in 3 cases and no expansion. Eighty cases were followed up for 30 months to 36 months after operation, 12 stable cases improved, 1 stable case got worse, while the others remained unchanged. The MRI showed no change was compared with previous follow-up imaging. Conclusion Microinvasive cisterna magna reconstruction is a surgical procedure with mininal injury, quick recovery, stable effect, fewer complications, and high security. Intraoperative ultrasonography provides reliable data and is easy to perform.

  4. Hemiplegia cruzada associada a impressão basilar, malformação de Arnold-Chiari e siringomielia: relato de caso Cruciate hemiplegia associated with basilar impression, Arnold-Chiari malformation and syringomyelia: case report

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    José Alberto Gonçalves da Silva

    1996-12-01

    Full Text Available Os autores apresentam um caso de hemiplegia cruzada associada a impressão basilar, malformação de Arnold-Chiari e siringomielia. Discutem as propostas anatômicas e a fiopatogenia desta síndrome de ocorrência bastante rara.The authors report a case of cruciate hemiplegia associated with basilar impression, Chiari malformation and syringomyelia. The neuroanatomical controversy, the surgical treatment and the good outcome of the patient are discussed.

  5. Posterior fossa decompression with tonsillectomy in 104 cases of basilar impression, Chiari malformation and/or syringomyelia

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    José Alberto Gonçalves da Silva

    2011-10-01

    Full Text Available The prime objective in the surgical treatment of basilar impression (BI, Chiari malformation (CM and/or syringomyelia (SM is based on the restoration of the normal cerebrospinal fluid (CSF dynamics at the craniovertebral junction through the creation of a large artificial cisterna magna. A small suboccipital craniectomy has been emphasized to avoid caudal migration of the hindbrain structures into the vertebral canal. Nevertheless, the results showed downward migration of the hindbrain related to that type of craniectomy. The authors present, otherwise, the results of 104 cases of BI, CM and/or SM, whose surgical treatment was characterized by a large craniectomy with the patient in the sitting position, tonsillectomy, large opening of the fourth ventricle and duraplasty with creation of a large artificial cisterna magna. A significant upward migration of the posterior fossa structures was detected by postoperative magnetic resonance imaging.

  6. Increased pulsatile movement of the hindbrain in syringomyelia associated with the Chiari malformation: cine-MRI with presaturation bolus tracking

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    Terae, S. [Hokkaido Univ. School of Medicine, Sapporo (Japan). Dept. of Radiology; Miyasaka, K. [Hokkaido Univ. School of Medicine, Sapporo (Japan). Dept. of Radiology; Abe, S. [Hokkaido Univ. School of Medicine, Sapporo (Japan). Dept. of Radiology; Abe, H.; Tashiro, K. [Hokkaido Univ. School of Medicine, Sapporo (Japan). Dept. of Neurology

    1994-02-01

    Cine-MRI with presaturation bolus tracking was used in patients with syringomyelia associated with a Chiari malformation to study pulsatile movement of the hindbrain, cervical spinal cord, cerebrospinal fluid and the fluid within the syrinx. Nine patients had 13 examinations, 6 preoperative, 3 after syringosubarachnoid shunting and 4 after posterior fossa decompression. Five controls were also examined. Dynamic display of the acquired images demonstrated downward displacement of the presaturation bolus on the cerebellar tonsils and medulla oblongata (or upper cervical cord) at the C1 level in all preoperative examinations and in two patients after syringo-subarachnoid shunting but with residual foramen magnum obstruction. Downward displacement of the bolus on the cervical spinal cord was also demonstrated in 7 examinations, but not observed in the controls. Thus, the hindbrain-spinal cord axis showed larger pulsatile movements in patients with foramen magnum obstruction. Based on these observations and a review of the literature, a new theory on the mode of extension of syringomyelia, emphasising the role of increased pulsatile movement of the hindbrain-spinal cord axis is proposed: that the pulsatile movements, together with a one-way valve mechanism in the syrinx cavity act as a ``vacuum-pump`` to enlarge the syrinx. (orig.)

  7. Craniocervical decompression with duraplasty and cerebellar tonsillectomy as treatment for Chiari malformation-I complicated with syringomyelia.

    Science.gov (United States)

    Bao, C S; Liu, L; Wang, B; Xia, X-G; Gu, Y J; Li, D J; Zhan, S L; Chen, G L; Yang, F B

    2015-02-03

    This study aimed to investigate the therapeutic effects of craniocervical decompression with duraplasty and cerebellar tonsillectomy for the treatment of Chiari malformation-I with syringomyelia (CM I-SM). From January 2005 to December 2011, 127 patients with CM I-SM underwent craniocervical decompression with duraplasty and cerebellar tonsillectomy and the therapeutic effects of these surgeries were evaluated using Tator scores. No patient in this study died or showed disease deterioration after the surgery. Re-examination by magnetic resonance imaging (MRI) showed that the cisterna magna was obviously larger after the operation in all but one patient. Moreover, syringomyelia (SM) was reduced in 76 patients. CM I-SM symptoms disappeared or decreased in 112 patients after following discharge. Follow-up was conducted in 84 of the patients and 79 of these patients exhibited improved symptoms. A second MRI re-examination showed that the cisterna magna was successfully constructed in 44 patients; 42 of these patients showed further eliminated or obviously reduced SM. Craniocervical decompression with duraplasty and cerebellar tonsillectomy achieved favorable therapeutic effects. Thus, craniocervical decompression with duraplasty and cerebellar tonsillectomy is a rational surgical approach with beneficial clinical effects. The proposed approach may have useful applications in the treatment of CM I-SM.

  8. Microsurgery Treatment of Chiari I Malformation Combination Syringomyelia%Chiari I畸形并脊髓空洞症的显微外科微创手术治疗

    Institute of Scientific and Technical Information of China (English)

    钟亮; 周均林; 张伟; 兰林; 何东; 刘峰

    2015-01-01

    目的:探讨Chiari I畸形并脊髓空洞症的显微外科微创手术治疗效果。方法回顾分析26例Chiari I畸形患者,采用显微外科微创手术治疗,评价手术效果。结果随访1~3年。术后未发生严重并发症及死亡,有效率100%。结论 Chiari I畸形并脊髓空洞症的显微外科微创手术治疗创伤小、并发症少、疗效确切,有积极的临床意义。%Objective To study the effect of Microsurgery in treatment of Chiari I malformation combination syringomyelia.Methods 26 cases of Chiari I malformation were retrospective analyzed, using minimally invasive surgical treatment, evaluating operation effect. Results followed up for 1 to 3 years. No postoperative serious complications and death, the effective rate was 100%.Conclusion Curative effect of microsurgery for Chiari I malformation and syringomyelia is small trauma, fewer complications, and have a positive clinical signiifcance.

  9. Posterior fossa reconstruction in the treatment of Chiari I malformation associated with syringomyelia%颅后窝重建术治疗Chiari I畸形合并脊髓空洞症

    Institute of Scientific and Technical Information of China (English)

    沈建; 方黎明; 濮宏健; 朱岁军; 唐超; 周永庆; 黄红光

    2011-01-01

    Objective To evaluate posterior fossa reconstruction (posterior cranial fossa decompression,duraplasty, resection of the cerebellar tonsil and separation of the arachnoid membrane from the dura) in the treatment of Chiarimalformation.Methods A total of 46 patients suffered from Chiarimalformation associated with syringomyelia received posterior fossa reconstruction in our department from November 2002 to January 2008.Results The rate of symptom improvement post - operation was 84.8%.The length of syringomyelia was decreased significantly and KPS got remarkable improvement.Conclusion Posterior fossa reconstruction is a rational option for the treatment of Chiarimalformation associated with syringomyelia.%目的 探讨后颅窝重建术(后颅窝减压+硬脑膜成形+小脑扁桃体切除+蛛网膜粘连分解)治疗Chiari I畸形合并脊髓空洞症的方法及疗效.方法 2002年11月至2008年1月对收治的46例Chiari I畸形合并脊髓空洞症患者行后颅窝重建术.结果 后颅窝重建术症状改善率84.8%,脊髓空洞长度明显减小,KPS评分显著改善.结论后颅窝重建术是治疗Chiari I畸形合并脊髓空洞症较为合理的术式.

  10. Chiari Malformation: Symptoms

    Science.gov (United States)

    ... straining (known as a Valsalva maneuver), coughing, sneezing, posture, singing, laughing, etc. Although most Chiari experts agree ... by herniated tonsils unusual anatomy in the upper spine nerve damage due to syrinx (Syringomyelia) Once a ...

  11. Syringomyelia with Chiari I Malformation Presenting as Hip Charcot Arthropathy: A Case Report and Literature Review

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    Roya Memarpour

    2015-01-01

    Full Text Available Neuroarthropathy (neuropathic osteoarthropathy, also known as Charcot joint, is a condition characterized by a progressive articular surface destruction in the setting of impaired nociceptive and proprioceptive innervation of the involved joint. It is seen most commonly in the foot and ankle secondary to peripheral neuropathy associated with diabetes mellitus. Cases of hip (Charcot neuroarthropathy are rare and almost exclusively reported in patients with neurosyphilis (tabes dorsalis. We report a case of a 36-year-old man who presented to the emergency department complaining of right hip pain. On physical examination, pain and thermal sensory deficits were noted in the upper torso with a cape-like distribution, as well as signs of an upper motor neuron lesion in the left upper and lower extremities. A magnetic resonance imaging study (MRI of the right hip showed evidence of early articular surface destruction and periarticular edema consistent with hip Charcot arthropathy. An MRI of the spine revealed an Arnold-Chiari type I malformation with extensive syringohydromyelia of the cervical and thoracic spine.

  12. Chiari Malformation

    Science.gov (United States)

    ... more accurate recommendations to individuals with syringomyelia regarding optimal surgical or non-surgical treatments. More information about ... Through Research Know Your Brain Preventing Stroke Understanding Sleep The Life and Death of a Neuron Genes ...

  13. Posterior reduction and internal fixation with posterior cranial fossa cranioectomy decompression for Chiari malformation type Ⅰ with basilar invagination, atlantoaxial subluxation, and syringomyelia

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    HU Peng

    2012-08-01

    Full Text Available Background Chiari malformation type Ⅰ(CM-Ⅰ is one of the soft tissue anomalies in craniovertebral junction (CVJ. This kind of soft tissue anomaly usually develops with bone anomaly, such as atlantoaxial subluxation, basilar invagination, platybasia, C1 assimilation, etc. For these complex combined anomalies, the treatment remains unaddressed. This study was performed to evaluate the effect of posterior reduction and internal fixation with posterior cranial fossa cranioectomy decompression for Chiari malformation type Ⅰ with basilar invagination, atlantoaxial subluxation, and syringomyelia. Methods Patients with basilar invagination and atlantoaxial subluxation treated from July 2004 to September 2011 were reviewed. Including criterions were made to screen matching patients. Including patients were retrospectively analyzed on both clinical outcomes and radiographical results. Japanese Orthopaedic Association (JOA score was used to evaluate the clinical outcomes, while the syrinx maximum size was measured on transverse view of MRI T2 image. The results were analyzed by SPSS 17.0 using t -text. Significant difference was considered when P ≤ 0.05. Results Fourteen patients met the including criterions, including 4 male patients and 10 female patients, with a mean age of 31.86 ± 11.36 (standard deviation, range: 17-51 years. Mean JOA score preoperatively of 14 patients was 13.07 ± 1.59 (standard deviation, while that was 15.57 ± 1.02 (standard deviation postoperatively (t = 9.946, P = 0.000. The mean syrinx size was (7.05 ± 1.98 mm (standard deviation, while that was (2.21 ± 1.91 mm (standard deviation postoperatively (t = 7.271, P = 0.000. There were no procedure-related morbidity or mortality happened. Conclusion Direct posterior reduction and internal fixation with posterior cranial fossa cranioectomy decompression can obviously improve the clinical outcomes and shrink syrinx for patients suffered from Chiari malformation typeⅠ with

  14. Advances in Diagnosis and Treatment of Chiari Malformation Associated with Syringomyelia%Chiari畸形合并脊髓空洞症的诊疗进展

    Institute of Scientific and Technical Information of China (English)

    张雷; 刘庆鹏; 姚猛; 孙崇毅; 周昌伟

    2015-01-01

    Chiari malformation is a congenital malformation characterized with hindbrain hernia,syringomyelia is the most common complications of the disease.It is generally accepted that it was caused by underdevelopment of the occipital somite originating from the paraxial mesoderm.The diagnosis of Chiari malformation is mainly based on MRI.The experts have not been able to reach a consensus on the cause of the disease,monitoring means,follow-up conditions or treatments.Chiari malformation can be classified into four types according to anatomic features.The various clinical classification can guide the selection of surgical treatment.The clinical manifestations varied,according to the common symptoms and signs can be roughly divided into four syndrome group.Surgery is the only cure-intended therapy to Chiari malformation.Majority of the patients experience improvement in their symptoms and signs after accordingly treatment,such as posterior fossa decompression,cerebellar tonsillectomy,cistern magna reconstruction and shunt.With the continuous improvement of surgical techniques,the treatment of Chiari malformation with syringomyelia will get more satisfactory effect.%Chiari畸形(Chiari malformation)是一种以小脑扁桃体下疝入枕骨大孔为特征的先天性畸形,脊髓空洞症(syringomyelia)是该病最常见的并发症,其诊断主要依赖于MRI.目前,对于该病的病因、监测手段、随访条件以及治疗方法等,专家们还未能达成共识.根据Chiari畸形解剖上的异常将其分为四型,分型有利于术式的选择,尽管关于分型的争议较多,但Hans Chiari分型仍最普遍被临床广为接受和应用.Chiari畸形临床表现多变,根据常见主要症状及体征大体可分为四种类型.手术是治疗Chiari畸形合并脊髓空洞症的唯一有效方法,针对不同病情采用后颅窝减压术、小脑扁桃体切除及枕大池重建术、脊髓空洞分流术等,大部分患者的症状和体征可以得到改善.

  15. [Monitoring of Somatosensory Evoked Potentials during Foramen Magnum Decompression of Chiari Malformation Type I Complicated with Syringomyelia: A Report of Two Cases].

    Science.gov (United States)

    Kadoya, Tatsuo; Takenaka, Ichiro; Kinoshita, Yuki; Shiraishi, Munehiro; Uehara, Hirofumi; Yamamoto, Toshinori; Joyashiki, Takeshi

    2015-03-01

    Few reports exist on anesthetic management for foramen magnum decompression (FMD) of Chiari malformation type I (CM I) complicated with syringomyelia. In two such cases we monitored somatosensory evoked potentials (SEP). Case 1 : A 40-year-old woman presented with occipital headache and nuchal pain for 2 months; numbness and muscular weakness of bilateral upper limbs for a month. Magnetic resonance imaging (MRI) scan showed CM I complicated with syringomyelia. Case 2 : A 32-year-old man presented with numbness and muscular weakness of bilateral upper limbs for 5 months; numbness and muscular weakness of lower limbs for 2 months. MRI scan showed CM I complicated with syringomyelia. They underwent FMD. In both cases, general anesthesia was induced with remifentanil, propofol and rocuronium, and was maintained with oxygen, air, remifentanil and propofol. Moreover, we monitored SEP. Their operative courses were uneventful. In case 1, SEP latency became shorter after FMD. Her preoperative neurologic symptoms disappeared on first postoperative day. In contrast there was no change of SEP latency after FMD in case 2. His preoperative neurologic symptoms showed no change on fifth postoperative day. SEP monitoring may be a useful index for prediction of early recovery of neurologic symptoms after FMD.

  16. Assessment of cerebellar pulsation in dogs with and without Chiari-like malformation and syringomyelia using cardiac-gated cine magnetic resonance imaging.

    Science.gov (United States)

    Driver, C J; Watts, V; Bunck, A C; Van Ham, L M; Volk, H A

    2013-10-01

    Canine Chiari-like malformation (CM) is characterised by herniation of part of the cerebellum through the foramen magnum. In humans with Chiari type I malformation (CM-I), abnormal pulsation of the cerebellum during the cardiac cycle has been documented and is pivotal to theories for the pathogenesis of syringomyelia (SM). In this retrospective study, cardiac-gated cine balanced fast field echo (bFEE) magnetic resonance imaging (MRI) was used to assess pulsation of the brain in dogs and to objectively measure the degree of cerebellar pulsation with the neck in a flexed position. Overall, 17 Cavalier King Charles Spaniels (CKCS) with CM, including eight with SM and nine without SM, were compared with six small breed control dogs. Linear regions of interest were generated for the length of cerebellar herniation from each phase of the cardiac cycle and the degree of cerebellar pulsation was subsequently calculated. Age, bodyweight and angle of neck flexion were also compared. CKCS with CM and SM had significantly greater pulsation of the cerebellum than control dogs (P=0.003) and CKCS with CM only (P=0.031). There was no significant difference in age, bodyweight and angle of neck flexion between the three groups. Cardiac-gated cine bFEE MRI permitted the dynamic visualisation of cerebellar pulsation in dogs. These findings support the current theories regarding the pathogenesis of SM secondary to CM and further highlight the similarities between canine CM and human CM-I.

  17. Correlation with the MR T[sub 1]-WI appearances and surgical outcomes in cases of syringomyelia associated with Chiari type 1 malformation

    Energy Technology Data Exchange (ETDEWEB)

    Imae, Shinji; Koyama, Tsunemaro (Ohtsu Municipal Hospital, Shiga (Japan))

    1992-08-01

    Twenty-two patients with syringomyelia caused by a Chiari type 1 malformation underwent posterior fossa decompression. The male to female ratio was 10:12, with the patients ranging in age from 6 to 72 years (mean age: 33 years). Twelve patients were operated on by means of suboccipital craniectomy (SOC), patcy-graft dural plasty using lyophilized dura mater, and tonsilectomy, while 10 patients underwent only SOC and dural plasty. The mean follow-up period was 2.3 years. All the patients underwent a magnetic resonance imaging scan (T[sub 1]-weighted image) before and after the operation. The width of the syrinx cavity was measured on an axial T[sub 1]-weighted image, and the length and the position of the cerebellar tonsils were measured on a sagittal T[sub 1]-weighted image; the results were then classified into two or three types according to the appearance of the syrinx and the cerebellar tonsils. The after-effects of surgical treatment were evaluated on each type. A postoperative reduction of the syrinx was observed in 18 patients (83%). There was no significant difference between the surgical outcome and the size of any one syrinx. However, a postoperative MRI scan showed a reduction of the syrinx in 15 among 16 patients (94%) when the tonsillar tip fell below the C1 level. These results indicate that posterior fossa decompression is effective in patients with a syrinx showing Chiari type 1 malformation, especially when the position of the cerebellar tonsils is below the C1 level. (author).

  18. Familial Chiari malformation: case series.

    Science.gov (United States)

    Schanker, Benjamin D; Walcott, Brian P; Nahed, Brian V; Kahle, Kristopher T; Li, Yan Michael; Coumans, Jean-Valery C E

    2011-09-01

    Chiari malformations (Types I-IV) are abnormalities of the posterior fossa that affect the cerebellum, brainstem, and the spinal cord with prevalence rates of 0.1%-0.5%. Case reports of familial aggregation of Chiari malformation, twin studies, cosegregation of Chiari malformation with known genetic conditions, and recent gene and genome-wide association studies provide strong evidence of the genetic underpinnings of familial Chiari malformation. The authors report on a series of 3 family pairs with Chiari malformation Type I: 2 mother-daughter pairs and 1 father-daughter pair. The specific genetic causes of familial Chiari malformation have yet to be fully elucidated. The authors review the literature and discuss several candidate genes. Recent advances in the understanding of the genetic influences and pathogenesis of familial Chiari malformation are expected to improve management of affected patients and monitoring of at-risk family members.

  19. Foramen Magnum Decompression and Duraplasty is Superior to Only Foramen Magnum Decompression in Chiari Malformation Type 1 Associated with Syringomyelia in Adults.

    Science.gov (United States)

    Gürbüz, Mehmet Sabri; Berkman, Mehmet Zafer; Ünal, Emre; Akpınar, Elif; Gök, Şevki; Orakdöğen, Metin; Aydın, Salih

    2015-10-01

    Retrospective cohort study. To compare surgical results of foramen magnum decompression with and without duraplasty in Chiari malformation type 1 (CM-1) associated syringomyelia (SM). The optimal surgical treatment of CM-1 associated with SM is unclear. Twenty-five cases of CM-1 with SM were included. There were 12 patients (48%) in the non-duraplasty group and 13 patients (52%) in the duraplasty group. The rate of improvement, state of postoperative SM size, amount of tonsillar herniation, preoperative symptom duration, complications and reoperation rates were analysed. The rate of clinical improvement was significantly higher with duraplasty (84.6%) than without (33.3%, p <0.05). The rate of postoperative syrinx regression was significantly higher in the duraplasty group (84.6%) than in the non-duraplasty group (33.3%, p <0.05). One case in the duraplasty group needed a reoperation compared with five cases in the non-duraplasty group (p =0.059). Duraplasty is superior to non-duraplasty in CM-1 associated with SM despite a slightly higher complication rate.

  20. [Isolated respiratory insufficiency in Arnold-Chiari malformation].

    Science.gov (United States)

    Noseda, A; Devriendt, J; Hoffmann, G; Schmerber, J

    1996-01-01

    We report on a 45-year old woman with daytime sleepiness, polycythemia, hypoxemia and hypercapnia, admitted to hospital on three occasions in a 10 month period for acute respiratory failure. Polysomnography demonstrated apneas of central type, testing of the respiratory drive suggested central alveolar hypoventilation and magnetic resonance imaging showed an Arnold-Chiari malformation with syringomyelia. The originality of this case is the absence of any neurologic sign, respiratory failure being the sole manifestation of the Arnold-Chiari malformation.

  1. Evaluation of magnetic resonance imaging of Arnold-Chiari malformation

    Energy Technology Data Exchange (ETDEWEB)

    Yoshino, Kimihiro; Suga, Masakazu; Takemoto, Motohisa

    1987-06-01

    We evaluated the usefulness of magnetic resonance imaging (MRI) in the diagnosis of the Arnold-Chiari malformation. The patient, a 52-year-old man, complained of dizziness on walking. He initially refused to undergone myelography, but 4 months later, underwent MRI test, which lead to the diagnosis of Arnold-Chiari malformation (I type). Not all patients with symptoms of Arnold-Chiari malformation, syringomyelia, syringobulbia and cervical spinal tumor, undergone myelography, which is an invasive technique, therefore MRI should be the first examination for the patients with disorders involving the craniocervical junction.

  2. 青少年伴/不伴Arnold-Chiari畸形的脊髓空洞的影像学特征比较%Comparison of radiological features between Arnold-Chiari malformation-associated syringomyelia and idiopathic syringomyelia in adolescent

    Institute of Scientific and Technical Information of China (English)

    谢丁丁; 朱泽章; 邱勇; 沙士甫; 刘臻; 江龙; 闫煌; 陈玲

    2015-01-01

    目的 探讨继发于Arnold-Chiari畸形的脊髓空洞(Arnold-Chiari malformation-associated syringomyelia,ACMS)与特发性脊髓空洞(idiopathic syringomyelia,IS)之间影像学特征的差异及其临床意义.方法 对2006年6月~2013年6月在本院接受治疗并符合入选标准的132例(男70例,女62例)ACMS患儿及46例(男24例,女22例)IS患儿进行回顾性分析.在全脊髓MRI图像上评估2组患儿空洞的形态学特征,包括空洞与脊髓最大比值、空洞长度、空洞位置及形态.结果 ACMS组空洞与脊髓最大比值为0.61±0.18,空洞长度为9.01±4.88(2 ~20)个节段,均显著大于IS组(0.43 ±0.14,4.09±2.07),差异有统计学意义(P<0.05).ACMS组中82.57%空洞累及颈胸段,而IS组中50.00%空洞仅累及颈段.ACMS组中43.94%为膨胀型,而IS组中78.26%为局限型,差异有统计学意义(P<0.05).结论 与IS相比,ACMS的空洞与脊髓最大比值更大,累及节段更长.

  3. Comparison between curative effects of two different microsurgical methods on Chiari I malformation accompanied with syringomyelia%两种不同手术方法治疗合并脊髓空洞的Chiari Ⅰ畸形的效果比较

    Institute of Scientific and Technical Information of China (English)

    朱青峰; 王千; 王国芳; 周志国

    2011-01-01

    Objective To study the curative effect of different microsurgical methods on chiarimalformation accompanied with syringomyelia. Methods Nineteen patients with Chiarimalformation accompanied with syringomyelia serving as observed group were treated by the cerebellar tonsillectomy, central canal opening and extensive posterior fossa decompression with duraplasty from January,2007 to December, 2009. Twenty-one patients with Chiarimalformation accompanied with syringomyelia serving as control group were treated by posterior fossa decompression with duraplasty from January, 2002 to December, 2006. The curative effect in both the groups were analyzed and compared. Results The rate of the symptoms improvement (100%, 19/19) was significantly higher in the treatment than that (52.4%, 11/21) in the control group (P<0.05). Conclusion The cerebellar tonsillectomy, central canal opening and extensive posterior fossa decompression with duraplasty can improve the prognosis in the patients with Chiarimalformation accompanied with syringomyelia.%目的 探讨合并脊髓空洞的Chiari Ⅰ畸形的手术治疗方法.方法 2007年1月至2009年12月,收治的Chiari Ⅰ畸形合并脊髓空洞的19例患者作为观察组,在显微镜下进行小脑扁桃体下疝切除+脊髓中央管口松解+枕下减压扩大硬脑膜修补术治疗.同时搜集2002年1月至2006年12月收治的Chiari I畸形合并脊髓空洞的21例患者作为埘照组,单纯采用枕下减压扩大硬脑膜修补术治疗,分析两组的临床效果.结果 观察组症状缓解优良率为100.0%(19/19),对照组为52.4%(11/21),两组相较,差异显著(P<0.01).结论 应用显微手术进行小脑扁桃体下疝切除+脊髓中央管口松解+枕下减压扩大硬脑膜修补术治疗Chiari I畸形合并脊髓空洞临床效果好,值得推广.

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

    Science.gov (United States)

    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 (Pchange 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), Pgeneral, 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.

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

    Science.gov (United States)

    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

  6. Increase in cerebellar volume in Cavalier King Charles Spaniels with Chiari-like malformation and its role in the development of syringomyelia.

    Directory of Open Access Journals (Sweden)

    Thomas A Shaw

    Full Text Available Previous research in Cavalier King Charles Spaniels (CKCS has found that Chiari-like malformation and syringomyelia (CM/SM are associated with a volume mismatch between the caudal cranial fossa (CCF and the brain parenchyma contained within. The objectives of this study were to i compare cerebellar volume in CKCS (a "high risk' group which frequently develops CM/SM, small breed dogs (medium risk--occasionally develop CM/SM, and Labradors (low risk--CM/SM not reported; ii evaluate a possible association between increased cerebellar volume and CM/SM in CKCS; iii investigate the relationship between increased cerebellar volume and crowding of the cerebellum in the caudal part of the CCF (i.e. the region of the foramen magnum. Volumes of three-dimensional, magnetic resonance imaging derived models of the CCF and cerebellum were obtained from 75 CKCS, 44 small breed dogs, and 31 Labradors. As SM is thought to be a late onset disease process, two subgroups were formed for comparison: 18 CKCS younger than 2 years with SM (CM/SM group and 13 CKCS older than 5 years without SM (CM group. Relative cerebellar volume was defined as the volume of the cerebellum divided by the total volume of brain parenchyma. Our results show that the CKCS has a relatively larger cerebellum than small breed dogs and Labradors and provide evidence that increased cerebellar volume in CKCS is associated with crowding of cerebellum in the caudal part of the CCF. In CKCS there is an association between increased cerebellar volume and SM. These findings have implications for the understanding of the pathological mechanisms of CM/SM, and support the hypothesis that it is a multifactorial disease process governed by increased cerebellar volume and failure of the CCF to reach a commensurate size.

  7. Questionnaire-based behaviour analysis of Cavalier King Charles spaniels with neuropathic pain due to Chiari-like malformation and syringomyelia.

    Science.gov (United States)

    Rutherford, Lynda; Wessmann, Annette; Rusbridge, Clare; McGonnell, Imelda M; Abeyesinghe, Siobhan; Burn, Charlotte; Volk, Holger A

    2012-12-01

    Chiari-like malformation (CM)/syringomyelia (SM) is a disease complex recognised in Cavalier King Charles spaniels (CKCSs) that can lead to neuropathic pain (NeP). In humans, NeP is associated with anxiety, depression and reduced quality of life (QoL). In this study, databases of three specialist veterinary centres were searched and CKCS breed societies and health forums were contacted to identify CKCS with an imaging diagnosis of CM/SM. Owners completed questionnaires on behaviour, signalment, general health status, NeP and QoL. Data were analysed from 122 dogs out of 564 questionnaires completed, after incomplete questionnaires and data from dogs that had other potentially debilitating disease processes were excluded. NeP severity score was significantly and positively correlated with 'stranger-directed' fear (r(S)=0.28), non-social fear (r(S)=0.34), 'separation-related' behaviour (r(S)=0.38), attachment behaviour (r(S)=0.24), excitability (r(S)=0.21) and proxy for pain sensation (r(S)=0.29). Increased NeP was also significantly associated with decreased QoL (r(S)=0.47), ability to settle (r(S)=0.26) and willingness to exercise (r(S)=0.50). Severity of NeP was positively associated with certain fear-associated behaviour and with decreased owner-perceived QoL. Thus, neurobehavioural changes should be considered in the management of NeP in CKCS with CM/SM.

  8. Plastic repairing of the cistern magna to treat the Chiari malformation complicated with syringomyelia%枕大池成形术治疗Chiari畸形合并脊髓空洞症

    Institute of Scientific and Technical Information of China (English)

    刘永生; 申明峰

    2010-01-01

    目的 回顾性分析枕大池成形术治疗Chiari畸形合并脊髓空洞症的疗效.方法 采用枕大池成形术(后颅凹减压+硬脑膜成形+小脑扁桃体切除+蛛网膜粘连松解)治疗17例Chiari畸形合并脊髓空洞症患者.对其近期疗效和远期疗效进行总结.结果 近期疗效:17例患者术后临床症状消失或改善15例(有效率为90%),MRI检查提示下疝扁桃体消失,脊髓空洞缩小.远期疗效:MRI检查提示枕大池成形,脊髓空洞消失或明显变细,脊髓蛛网膜下腔增宽.治疗有效12例(有效率70%).结论 枕大池成形术治疗Chiari畸形合并脊髓空洞症效果满意,是一种较为合理的治疗方法.%Objective To evaluate retrospectively the efficacy of cistema magna plastic repair in the treatment of Chiari malformation complicated with syringomyelia Methods Fifty-one patients suffered from Chiari malformation complicated with syringomyelia were treated in our department from May 2004 to October 2009. Seventeen patients received cistema magna plastic repairment (the decompression of posterior cranial fossa, duraplastic repair,resection of hernia below cerebellar tonsil and separation of adherence of arachnoid membrane). Results Clinical symptoms disappeared or improved in 15 cases (effective rate of 90%). MRI showed tonsillar herniation disappeared,syringomyelia narrow. MRI showed cistema magna plastic repair, syringomyelia disappeared or significantly thinned and spinal cord subarachnoid space widened. Effective treatment obtained in 12 cases (effective rate 70%). Conclusions The efficiency of Cistern magna plastic repair treating Chiari malformation with syringomyelia is satisfied.

  9. [Treatment of syringomyelia in patients with Chiari malformation and craniosynostosis. A case report and review of the literature].

    Science.gov (United States)

    Aransay-Garcia, A; Villarejo-Ortega, F J

    2016-11-16

    Introduccion. Los pacientes con craneosinostosis complejas o unisuturales presentan frecuentemente malformacion de Chiari y siringomielia. El tratamiento quirurgico de la siringomielia en estos pacientes es controvertido. Caso clinico. Niña de 3 años con craneosinostosis compleja no corregida quirurgicamente. Permanecio asintomatica a pesar de que en la resonancia magnetica craneal se evidencio una malformacion de Chiari y un año despues desarrollo una siringomielia cervicodorsolumbar. Se le realizo una craniectomia suboccipital descompresiva, pero posteriormente sufrio un empeoramiento de la siringomielia. El registro de presion intracraneal resulto patologico, por lo que se decidio realizar una craneotomia descompresiva frontoparietotemporal bilateral y remodelacion de la boveda craneal, con lo que se consiguio una disminucion significativa de la siringomielia. Conclusiones. Tras la revision de la bibliografia, se observa que actualmente no existe un consenso sobre el tratamiento de la siringomielia en los pacientes con craneosinostosis y malformacion de Chiari. Algunos autores recomiendan la simultanea descompresion quirurgica suboccipital y de la boveda craneal, otros solo la descompresion de la boveda craneal, y otros la ampliacion de la fosa posterior con distractores. En los casos en los que se realizo primero la descompresion suboccipital no se consiguio resolver ni estabilizar la siringomielia. Concluimos que el tratamiento mas eficaz para los pacientes con siringomielia y craneosinostosis es la remodelacion descompresiva de la boveda craneal, ya que el principal factor causante de la siringomielia es la hipertension intracraneal y la falta de distensibilidad del craneo.

  10. 两种不同术式治疗Chiari畸形合并脊髓空洞症的疗效分析%Two different surgical therapies in the treatment of Chiari malformation complicated with syringomyelia

    Institute of Scientific and Technical Information of China (English)

    沈建; 徐庆生; 叶科; 钱晓波; 沈剑峰; 周永庆; 詹仁雅; 黄红光

    2008-01-01

    Objective To evaluate and compare retrospectively the efficacy between posterior cranial fossa plastic repair and cisterna magna plastic repair in the treatment of Chiari malformation complicated with syringomyelia. Methods 85 patients suffered from Chiari malformation complicated with syringomyelia were treated in our department from November 2000 to January 2006. 39 patents received posterior cranial fossa plastic repair ( the decompression of posterior cranial fessa and dura plastic repair), the other 46 patients with cistema magna plastic repair ( the decompression of posterior cranial fossa, duraplastic repair, resection of hernia below cerebellar tonsil and separation of adherence of arachnoid membrane). Results There were significant differences between two groups in postoperative syringomyelia length and long-term effects, with the rate of improvement of 64% and 90%, and the rate of deterioration 19% and 3%, respectively. Conclusion Cisterna magna plastic repair is a reasonable option for the treatment of Chiari malformation with syringomyelia, compared with posterior cranial fossa plastic repair.%目的 通过回顾性比较后颅窝成形术及枕大池成形术对Chiari畸形合并脊髓空洞症的治疗,明确两种不同术式治疗Chiari畸形合并脊髓空洞的疗效.方法 对收治的85例Chiari畸形合并空洞患者,其中39例患者行后颅窝成形术(后颅窝减压+硬脑膜成形);46例患者行枕大池成形术(后颅窝减压+硬脑膜成形+小脑扁桃体切除+蛛网膜粘连分解).结果 两组远期疗效相比有统计学意义,症状改善率分别为64%和90%,恶化19%和3%;术后脊髓空洞长度改变,两组比较有统计学意义.结论 枕大池成形术是治疗Chiari畸形合并脊髓空洞症较为合理的术式,疗效优于后颅窝成形术.

  11. Reconstruction of the cisterna magna in treatment of Chiari malformation associated with syringomyelia%枕大池重建术治疗Chiari畸形合并脊髓空洞

    Institute of Scientific and Technical Information of China (English)

    罗文伟; 林荫; 张远征; 张军; 王晓亮; 陈文培; 陈西亚

    2008-01-01

    Objective To investigate the efficacy of reconstruction of the cisterna magna in the surgical treatment of Chiari malformation associated with syringomyelia. Methods The patients with Chiari malformation associated with syringomyelia were performed the surgery of reconstruction of cisterna magna after they were diagnosed by MRI.All patients were followed up post-operatively for more than two years.Results All of 21 patients have got improvement in different degree after surgery, and post-operative MRI showed that cavity in the spinal cord was decreased.Conclusion This surgical approach can be achieved todecompress craniocervical junction, improve the CSF circulation, alleviate the clinical symptom efficiently, and be considered an effective treatment for syringomyelia.%目的 研究探讨枕大池重建术治疗Chiari畸形合并脊髓空洞症的手术治疗方法 .方法 Chiari畸形合并脊髓空洞患者经MRI确诊后,应用枕大池重建术显微外科技术治疗,并随访2年以上.结果 21例患者术后症状均有不同程度改善,复查头颅MRI见脊髓空洞缩小.结论 该手术可使颅颈交界区充分减压,改善脑脊液循环,有效缓解临床症状,并对脊髓空洞症有明显的治疗作用.

  12. Symptomatic Chiari malformation in infancy and adolescence; Cine-MRI and clinical evaluation

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    Fukushima, Takeo; Matsuda, Toshihiro; Tsugu, Hitoshi; Sakamoto, Seisaburou; Tomonaga, Masamichi; Asakawa, Koji; Mitsudome, Akihisa (Fukuoka Univ. (Japan). School of Medicine); Hashimoto, Takahisa

    1994-04-01

    Of the 7 children and adolescents with Chiari malformation reported here 5 had Chiari type 1 and 2 had Chiari type 2 disease. All 5 Chiari type 1 patients had syringomyelia and scoliosis, but the Chiari type 2 patients did not. All the patients except one with localized syringomyelia (case 3) underwent posterior decompression with suboccipital craniectomy, upper cervical laminectomy and duraplasty, and their clinical signs and symptoms improved. Case 3 received a syringo-subarachnoid shunt and improved clinically. Motor function was restored better than sensory function. Cranial nerve signs were cleared up in the Chiari type 2 patients. In the Chiari type 1 patients postoperative neurological improvement correlated well with the collapse of syringomyelia. Cine MR imaging was useful in the evaluation of CSF dynamics at the cranio-vertebral junction and in the syringomyelic cavity before and after surgery. (author).

  13. Results of the treatment of syringomyelia associated with Chiari malformation: analysis of 60 cases Resultados do tratamento da siringomielia associada com malformação de Chiari: análise de 60 casos

    Directory of Open Access Journals (Sweden)

    José Arnaldo Motta de Arruda

    2004-06-01

    Full Text Available We analyze the results of surgical treatment of 60 patients presenting syringomyelia (SM associated with Chiari malformation (CM who were operated in the period 1982-2000. For each case, analysis covered 15 signs and 16 symptoms included in a protocol that separated SM signs and symptoms from those of CM. A score system was established in parallel with the protocol to make the evaluation of treatment results easier. All cases were submitted to craniovertebral decompression by C1 and eventually C2 laminectomy and cerebellar tonsillectomy with duramater graft. To evaluate the results, statistical proportion difference tests and variance analyses were made to a reliability index of 95% (p=0.05. We conclude that the statistical improvement of CM signs and symptoms was very significant (p=0. Syringomyelia signs and symptoms also improved significantly, except for "upper limb hyporeflexia" , which did not improve. No statistical difference in the improvement of SM symptoms as compared to CM symptom was found. Syringomyelia signs improved statistically more than CM signs. In half of patients, the percent improvement of signs and symptoms ranged between 40% and 60%.RESUMO Analisamos os resultados do tratamento cirúrgico de 60 pacientes com a associação de siringomielia (SM e malformação de Chiari (MC operados no período de 1982 a 2000. Em cada caso foram analisados 15 sinais e 16 sintomas, incluídos em um protocolo que separou os sinais e sintomas decorrentes da SM dos sinais e sintomas decorrentes da MC. Paralelamente ao protocolo, foi elaborado um sistema de pontuação que facilitou a avaliação dos resultados do tratamento. Todos os casos foram submetidos à descompressão crânio vertebral com laminectomia de C1 e eventualmente de C2, e tonsilectomia cerebelar com plástica de dura-máter. Para avaliação dos resultados, foram aplicados testes estatísticos de diferença de proporções e análise de variância com confiabilidade de 95% (p

  14. Modified surgical treatment for Arnold-Chiarimalformation complicated with syringomyelia%改良手术治疗Arnold-Chiari畸形Ⅰ型合并脊髓空洞

    Institute of Scientific and Technical Information of China (English)

    王治国; 包志军; 贺世明

    2013-01-01

    Objective To evaluate the methods and effects of modified surgical treatment for Amold-Chiarimalformation complicated with syringomyelia.Methods Twelve patients with ArnoldChiarimalformation complicated with syringomyelia,underwent a modified surgical treatment,effects were observed.Results During the first follow-up,sensation and motion function were significantly improved in 10 cases,unchanged in 2 cases.During the second follow-up,2 cases previously unchanged were improved,while the other were with no change.During the first follow-up,lesion showed on MRI disappeared in 5 cases,reduced in 4 cases,no significant change in 3 cases,no case extend,3 cases with hydrocephalus ventricle was significantly reduced.The second follow-up,MRI showed that there was no change compared with the first follow-up.Conclusion The remove of the pia mater of tonsillar hernia + reconstruction the cisterna magna have good effect on surgical therapies for Arnold-Chiari I malformation complicated with syringomyelia.%目的 探讨Arnold-Chiari畸形I型合并脊髓空洞改良手术治疗方法及效果.方法 选择12例Arnold-Chiari畸形Ⅰ型合并脊髓空洞患者,行小脑扁桃体下疝软脑膜下切除并蛛网膜修补枕大池重建改良手术治疗,观察疗效.结果 术后第一次随访10例感觉、运动功能明显改善,2例稳定,无加重患者.第二次随访原稳定者好转2例,余患者症状无明显改变.第一次随访MRI检查显示脊髓空洞消失5例、缩小4例、无变化3例,无一例脊髓空洞扩大,3例合并脑积水者脑室明显缩小.第二次随访MRI检查与第一次随访相比无明显变化.结论 小脑扁桃体下疝软脑膜下切除并蛛网膜修补枕大池重建改良手术对Arnold-Chiari畸形Ⅰ型合并脊髓空洞治疗具有良好效果.

  15. Arnold-Chiari畸形Ⅰ型并脊髓空洞症的显微外科治疗%Microsurgical treatment of Arnold-Chiarimalformation combined with syringomyelia

    Institute of Scientific and Technical Information of China (English)

    包长顺; 杨福兵; 刘亮; 王斌; 詹书良; 陈礼刚

    2011-01-01

    目的 探讨Arnold-Chiari畸形Ⅰ型并脊髓空洞症的手术方式及术后疗效. 方法 泸州医学院附属医院神经外科自1997年1月至2010年6月采用不同手术方式治疗Arnold-Chiari畸形Ⅰ型并脊髓空洞症患者185例,回顾性分析其临床资料,总结手术方式及疗效. 结果 出院时患者症状消失或改善156例,无变化29例,无症状恶化和死亡病例.随访147例患者,症状消失或改善110例,症状无变化26例,症状恶化11例;95例行MRI复查,其中枕大池解剖成形者82例,脊髓空洞明显缩小79例. 结论 后颅窝减压+硬膜成形术、小脑扁桃体切除+枕大池成形术是治疗Arnold-Chiari畸形Ⅰ型并脊髓空洞症有效、合理的手术方式.%Objective To investigate the methods ofmicrosurgical treatment of Arnold-Chiarimalformation combined with syringomyelia and their curative effects.Methods The clinical data of 185 patients with Arnold-Chiarimalformation combined with syringomyelia,admitted to our hospital from January 1997 to June 2010 and received different surgical treatments,were summarized.The relationship between curative effects and operative methods were analyzed.Results When these 185 patients were discharged from our hospital,the clinical signs and symptoms eliminated in 156 (84.3%)and not changed in 29; no symptomatic deterioration and death were noted.One hundred and forty-seven patients were followed up for 3 months-12 years; symptoms disappeared or alleviated in 110 (74.8%),not changed in 26 and deteriorated in 11.The MR findings in 95 patients demonstrated that the cisternals reconstructed and expanded in 82 and the cavities in spinal cords reduced in 79.Conclusion Both posterior fossa decompression + duraplasty and removal of cerebella tonsils + plastic operation of cistern magna are effective and advisable methods in the treatment of Arnold-Chiarimalformation combined with syringomyelia.

  16. Operative strategy of congenital atlantoaxial dislocation-induced Chiari malformation and (or)syringomyelia%先天性寰枢椎脱位导致Chiari畸形和(或)脊髓空洞的手术策略

    Institute of Scientific and Technical Information of China (English)

    段光明; 周定标; 余新光

    2008-01-01

    Objective To study the operative strategy of congenital atlantoaxial dislocation(CAAD)-induced Chiari malformation and (or) syringomyelia. Methods The operation in reported 23cases of CAAD-induced Chiari malformation and (or) syringomyelia was composed with the transoral resection of odontoid process to achieve anterior decompression at first stage and occipito-cervical bone grafting fusion at second stage. Results MRI examination revealed the tonsils ascent and (or) syrinx reduction in 19 cases after first-stage operation. Compared with their preoperative manifestations, 14cases were obviously improved and 5 improved to some extent after operation, while 4 were unchanged. Conclusions CAAD is the main cause of tonsils descent and (or) syringomyelia in the series of patients. After anterior decompression by transoral resection of odontoid process, most patients will get recovered in tonsils descent and (or) syringomyelia reduction. The main aim of posterior operation is to reconstruct the stability of craniovertebral junction.%目的 探讨先天性寰枢椎脱位(CAAD)导致Chiari畸形和(或)脊髓空洞的手术策略.方法 收集我科23例CAAD导致Chiari畸形和f或1脊髓空洞患者的手术情况.一期经口齿状突切除前路减压,二期主要行后路枕颈植骨融合术. 结果 一期手术后复查MRI,小脑扁桃体下疝还纳、脊髓空洞缩小19例,无变化4例.术后临床表现明显改善14例,改善5例,无变化4例. 结论 在该类患者中,CAAD是造成小脑扁桃体下疝和(或)脊髓空洞的主要原因.单纯经口齿状突切除前路减压,多可使扁桃体下疝还纳,空洞缩小.后路手术的主要目的是重建颅颈交界处的稳定.

  17. Hypopituitarism induced by syringomyelia associated with Chiari malformation and review of literatures%Chiari畸形并脊髓空洞症致腺垂体功能减退1例并文献复习

    Institute of Scientific and Technical Information of China (English)

    宋素彩; 栗夏莲; 王丹萍; 邵明伟

    2011-01-01

    Objective To analyze the characteristics of a patient with hypopituitarism and reviewed related literatures to investigate the pathogenesis of hypopituitarism induced by syringomyelia associated with Chiari malformation. Methods Detailed physical examination was performed in a male whose complaint was retardation of growth. Then the serum gonadal hormones, thyroid hormones and adrenal cortical hormones level were assayed. The GnRH stimulating test,X -ray of wrist joint and pituitary MRI were also performed. Results Patients has a history of perinatal injury. The body height is 143cm and no secondary sexual characters were developed. The LH, T,TSH, FT3 and 24 -hous uretic free cortisol is 0. 00mIU/L, 0. 10ng/ml, 1.23mlU/L, 3.47pmoL/L and 46. 0nmoL/L respectively. The GnRH stimulating test shows that the peak of LH which appeared at 120 - min is 0. 29mIU/L. The bone age is 13 and the MRI of pituitary shows: the shape is abnormal,the image of stalk is dimness, and cerebellar tonsil hereniation. Conclusions Chiari malformation is the basis of the hypopituitarism induced by syringomyelia. Perinatal injury is an inducement. Hypopituitarism happened along the development of syringomyelia. So patients with syringomyelia, especially syringomyelia associated with Chiari malformation should be examined the pituitery function.%目的 Chiari畸形并脊髓空洞是腺垂体功能减退症罕见的病因,本文通过分析1例Chiari畸形并脊髓空洞导致腺垂体功能减退患者的临床资料和文献复习,探讨Chiari畸形并脊髓空洞引起腺垂体功能减退的发病机制.方法 对一"生长发育迟缓9年"的23岁男性患者行详细体检,测定性腺激素、甲状腺激素、肾上腺皮质激素水平,并行促性腺激素释放素刺激试验及骨龄和垂体MRI检查.结果 患者有围产期损伤史,身材矮小,第二性征未发育;LH 0.00mIU/L,T 0.10ng/ml,FSH 1.23mlU/L,m 3.47pmol/L,24h游离皮质醇46.0nmol/L,促性腺激素释放素

  18. Clinical study of surgical treatment for Arnold Chiari malformation type Ⅰ with syringomyelia%Arnold ChiariⅠ畸形合并脊髓空洞的手术分析

    Institute of Scientific and Technical Information of China (English)

    苏亦明; 陈飞; 孙亚邓

    2007-01-01

    目的 探讨Arnold Chiari Ⅰ畸形(Arnold Chiari Malformation type Ⅰ,ACM)合并脊髓空洞(Syringomyelia,SM)的外科治疗方法.方法 对2000~2006年36例ACM合并SM的患者采用后颅窝减压、成形术,并根据不同的临床特点予以空洞切开引流、下疝小脑扁桃体切除、颈枕交界区粘连分离以及脊髓中央管口松解术.结果 术后随访1年以上,症状明显缓解28例,稳定无变化7例,1例疼痛加重并出现对侧上肢麻木;MRI检查示2例脊髓空洞消失,26例空洞明显缩小,8例空洞无明显变化.结论 缓解颅颈交界区的压迫和疏通脑脊液循环是手术成功的根本.

  19. The curative effect of revision operation for Chiari I malformation complicated with syringomyelia followed surgical treatment%Chiari畸形Ⅰ型并脊髓空洞症术后翻修的疗效观察

    Institute of Scientific and Technical Information of China (English)

    王曲; 高方友; 刘窗溪; 韩国强; 熊云彪; 王俊; 姚倍金; 尹浩; 廖昆

    2014-01-01

    目的 探讨Chiari畸形Ⅰ型并脊髓空洞症患者术后翻修的方法及疗效.方法 2004年5月至2013年4月收治Chiari畸形Ⅰ型并脊髓空洞症术后患者15例,患者均存在原有症状体征无改善或(和)加重,或(和)出现新的神经功能缺失,其中初次手术采用后颅窝大骨窗减压术8例,后颅窝骨窗减压+硬膜外层切开术3例,后颅窝成形术+硬膜扩大修补术2例,下疝小脑扁桃体切除术+枕大池成形术2例.翻修手术均采用下疝小脑扁桃体切除+第四脑室流出道探查术,3例行疏通术.结果 翻修术后随访4 ~ 47个月平均24.1个月,症状基本消失者8例,明显改善者5例,无改善者2例.MRI示小脑扁桃体下缘均达枕骨大孔上方,脊髓空洞均缩小.结论 对Chiari畸形Ⅰ型并脊髓空洞症术后疗效不佳者采用下疝小脑扁桃体切除+第四脑室流出道疏通术进行翻修是安全、可靠、有效方法.%Objective To study the methods and the curative effect of revision operation for Chiari I malformation complicated with syringomyelia followed surgical treatment.Methods The clinical data of 15 patients underwent revision operation for Chiarimalformation complicated with syringomyelia followed surgical treatment from May 2004 to April 2013 were retrospectively reviewed,they still had symptoms and signs or/and suffered from symptoms aggravated.In the prior operation there were the enlargement decompression posterior cranial fossa in 8 cases,the decompression with resection of outer layer of the dura in 3 cases,the small bone window craniotomy decompression of posterior cranial fossa and resection of the hernia of cerebella tonsil in 2 cases,the decompression of posterior cranial fossa and dura plastic repair in 2 cases.The resection of the hernia of cerebella tonsil and exploration the fourth ventricle outlet were performed in the revision operation of 15 cases,reconstruction of the fourth ventricle outlet in 3 cases.Results The

  20. Basilar impression, Chiari malformation and syringomyelia: a retrospective study of 53 surgically treated patients Impressão basilar, malformação de Chiari e siringomielia: estudo retrospecivo de 53 casos operados

    Directory of Open Access Journals (Sweden)

    José Alberto Gonçalves da Silva

    2003-06-01

    Full Text Available The present study shows the results of 53 patients who have been treated surgically for basilar impression (BI, Chiari malformation (CM, and syringomyelia (SM. The patients were divided into two groups. Group I (24 patients underwent osteodural decompression with large inferior occipital craniectomy, laminectomy from C 1 to C 3, dural opening in Y format, dissection of arachnoid adhesion between the cerebellar tonsils, medulla oblongata and spinal cord, large opening of the fourth ventricle and dural grafting with the use of bovine pericardium. Group II patients (29 patients underwent osteodural-neural decompression with the same procedures described above plus dissection of the arachnoid adherences of the vessels of the region of the cerebellar tonsils, and tonsillectomy (amputation in 10 cases, and as for the remainning 19 cases, intrapial aspiration of the cerebellar tonsils was performed. The residual pial sac was sutured to the dura in craniolateral position. After completion of the suture of the dural grafting, a thread was run through the graft at the level of the created cisterna magna and fixed to the cervical aponeurosis so as to move the dural graft on a posterior- caudal direction, avoiding, in this way, its adherence to the cerebellum.São analisados os resultados obtidos com o tratamento cirúrgico de 53 casos de impressão basilar (IB, malformação de Chiari (MC e siringomielia (SM. Os pacientes foram divididos em dois grupos: no grupo I (24 casos foi realizada a descompressão osteodural, caracterizada por craniectomia ampla occipital inferior, laminectomia variável de C 1 a C 3, abertura da dura-máter em forma de Y, dissecção das aderências aracnóideas das tonsilas cerebelares com o bulbo e medula cervical, abertura ampla do quarto ventrículo e enxerto dural; no grupo II (29 casos foi utilizada a descompressão osteodural-neural, caracterizada pelos mesmos detalhes técnicos empregados no grupo I, acrescidos da

  1. Chiari Malformation: Diagnosis

    Science.gov (United States)

    ... that provide feedback on muscle movement and position, balance, and cerebellar function. Tests for coordination include: Rapid ... forth flow of CSF between the brain and spine and by how much. In Chiari cases with ...

  2. Magnetic resonance imaging features of complex Chiari malformation variant of Chiari 1 malformation

    Energy Technology Data Exchange (ETDEWEB)

    Moore, Hannah E. [Primary Children' s Medical Center, Department of Medical Imaging, Salt Lake City, UT (United States); Moore, Kevin R. [University of Utah School of Medicine, Department of Radiology, Salt Lake City, UT (United States); Primary Children' s Medical Center, Department of Medical Imaging, Salt Lake City, UT (United States)

    2014-11-15

    Complex Chiari malformation is a subgroup of Chiari 1 malformation with distinct imaging features. Children with complex Chiari malformation are reported to have a more severe clinical phenotype and sometimes require more extensive surgical treatment than those with uncomplicated Chiari 1 malformation. We describe reported MR imaging features of complex Chiari malformation and evaluate the utility of craniometric parameters and qualitative anatomical observations for distinguishing complex Chiari malformation from uncomplicated Chiari 1 malformation. We conducted a retrospective search of the institutional imaging database using the keywords ''Chiari'' and ''Chiari 1'' to identify children imaged during the 2006-2011 time period. Children with Chiari 2 malformation were excluded after imaging review. We used the first available diagnostic brain or cervical spine MR study for data measurement. Standard measurements and observations were made of obex level (mm), cerebellar tonsillar descent (mm), perpendicular distance to basion-C2 line (pB-C2, mm), craniocervical angle (degrees), clivus length, and presence or absence of syringohydromyelia, basilar invagination and congenital craniovertebral junction osseous anomalies. After imaging review, we accessed the institutional health care clinical database to determine whether each subject clinically met criteria for Chiari 1 malformation or complex Chiari malformation. Obex level and craniocervical angle measurements showed statistically significant differences between the populations with complex Chiari malformation and uncomplicated Chiari 1 malformation. Cerebellar tonsillar descent and perpendicular distance to basion-C2 line measurements trended toward but did not meet statistical significance. Odontoid retroflexion, craniovertebral junction osseous anomalies, and syringohydromyelia were all observed proportionally more often in children with complex Chiari malformation than in

  3. Headache in children with Chiari I malformation.

    Science.gov (United States)

    Toldo, Irene; Tangari, Marta; Mardari, Rodica; Perissinotto, Egle; Sartori, Stefano; Gatta, Michela; Calderone, Milena; Battistella, Pier Antonio

    2014-05-01

    Headache is the most common symptom of Chiari 1 malformation, a condition characterized by the herniation of cerebellar tonsils through the foramen magnum. However, the headache pattern of cases with Chiari 1 malformations is not well defined in the literature, especially in children. The aim of this retrospective chart review was to evaluate the frequency and the characteristics of headache in children with Chiari 1 malformation at initial evaluation and during follow up. Forty-five cases with tonsillar ectopia were selected among 9947 cases under 18 years of age who underwent neuroimaging between 2002 and 2010. A semistructured clinical interview (mean follow-up: 5.2 years) was conducted. Headache was classified according to the second edition of the International Classification of Headache Disorders. Possible associations between clinical picture, in particular headache pattern, but also other signs and symptoms attributable to Chiari 1 malformation, and the extent of tonsillar ectopia were found for 3 different groups: those with borderline (headache, and 9/33 (27%) of those patients (5 with mild and 4 with severe tonsillar ectopia) reported headache attributed to Chiari 1 malformation. In our studied pediatric population, the most common symptom for cases diagnosed with Chiari 1 malformation was headache, and headache attributed to Chiari 1 malformation was the most common headache pattern in patients with Chiari 1 malformation. The presence of headache attributed to Chiari 1 malformation along with 3 other signs or symptoms of Chiari 1 malformation were highly predictive of severe tonsillar ectopia. © 2014 American Headache Society.

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

    Science.gov (United States)

    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.

  5. Comparison study of two surgical treatments for Arnold Chiari I malformation with syringomyelia%两种手术方式治疗Arnold Chiari Ⅰ畸形合并脊髓空洞的临床疗效比较

    Institute of Scientific and Technical Information of China (English)

    罗飚

    2012-01-01

    Objective To observe the clinical effect of treating Arnold Chiari I malformation with syringornyelia patients by two kinds of surgical treatment and explore a more effective surgical method. Method 42 Arnold Chiari I malformation with syringomyelia patients were enrolled in this study, they were divided into the A group and B group, then the complications after treatment and the clinical effect after 3 months' treatment in two groups were compared. Result There was no statistical significant (P>0.05 ) in the complications after treatment, there was a statistical significant (P0.05);治疗3个月后临床疗效为优的病例及术后脊髓空洞缩小的病例比较,差异有统计学意义(P<0.05),B组优于A组.结论 手术治疗ACM Ⅰ合并SM患者时,应首选后颅窝减压+脊髓空洞切开分流+小脑扁桃体切除治疗的方式,可提高临床疗效,且不会增加术后并发症.

  6. 后颅窝内减压治疗Chiari 畸形Ⅰ型并脊髓空洞中期疗效评价%Mid-term outcomes of posterior fossa decompression and cranioplasty for Chiari malformation and syringomyelia type Ⅰ

    Institute of Scientific and Technical Information of China (English)

    吕学明; 袁绍纪; 张荣伟; 卢培刚; 孙希炎; 朱伟杰; 刘子生; 陈援朝; 吕福林

    2011-01-01

    Objective To evaluate the mid-term outcomes of posterior fossa decompression and cranioplasty for Chiari malformation and syringomyelia type Ⅰ. Methods Posterior fossa decompression and cranioplasty were performed in a consecutive series of 40 patients. Clinical outcomes were assessed by Karnofsky Performance Scale (KPS) scores and activies of daily living (ADL) questionnaires before and after operation. Operation time, intraoperative blood loss, incision status and complications were recorded. Radiological examination was obtained for each patient to assess the situation of changes in syringomyelia, postoperative cerebrospinal fluid flow rates and the stability of the craniocervical junction. Results The KPS scores increased significantly from (70 ± 10) preoperatively to (80 ± 10) postoperatively(P < 0.01 ). The ADL scores were decreased remarkably from (20.6 ±2.2) preoperatively to (15.8 ±0.5) postoperatively (P <0.01 ) and the proportion with optimal effect was 86%. Syringomyelia narrowing and disappearance rate was 90.5% (19/21). Cerebrospinal fluid flow speed improved compared with that before operation.Conclusion Posterior fossa decompression and cranioplasty is an effective and convenient method for Chiari malformation and syringomyelia type Ⅰ .%目的 评价后颅窝内减压(后颅窝减压+后颅窝颅骨成形术)治疗Chiari 畸形Ⅰ型并脊髓空洞疾病的中期临床疗效.方法 采用后颅窝内减压术治疗40 例符合标准的Chiari 畸形Ⅰ型并脊髓空洞.观察手术时间、切口愈合情况,分析相关并发症.采用远期生活质量评估(KPS)和美国Lawton 和Brody1996 年制定的日常生活能力量表评分(ADL)评价疗效,并且通过影像学检查对脊髓空洞的变化、脑脊液流速情况以及颅颈交界区稳定性情况进行评价.结果 随访KPS 分值由术前的(70 ±10)分提高到到末次随访时(80 ± 10)分(P <0.01),ADL 分值由术前的(20.6 ±2.2)分降至末次随访时(15

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

    Science.gov (United States)

    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.

  8. Posterior cranial fossa dimensions in Chiarimalformation relates to the degree of cerebellar tonsillar descent and syringomyelia%Chiari Ⅰ畸形后颅窝线性容积与小脑扁桃体下疝及脊髓空洞的相关性研究

    Institute of Scientific and Technical Information of China (English)

    金铂; 苏亦兵; 王汉斌; 王科大

    2013-01-01

    目的 对Chiari Ⅰ畸形患者后颅窝的各线性结构进行测量,探讨不同下疝程度以及是否并发脊髓空洞的Chiari Ⅰ畸形患者的发病机制以及利用MRI在后颅窝结构上的测量意义.方法 回顾分析2004年8月至2012年6月北京积水潭医院神经外科住院治疗的Chiari Ⅰ畸形患者97例,在MRI上测量其斜坡的长度、枕骨基底部的长度、蝶骨底长度、枕骨鳞部、脑干的长度、小脑半球的长度、枕骨大孔径、Twining's线、小脑幕与Twining's线的角度.结果 根据MRI影像学的表现,将Chiari Ⅰ畸形患者小脑扁桃体下缘尾向移位程度分为3度,其中Ⅰ度48例(49.5%),Ⅱ度36例(37.1%),Ⅲ度13例(13.4%);合并脊髓空洞69例(71.1%),无脊髓空洞28例(28.9%).Chiari Ⅰ畸形小脑扁桃体下疝Ⅰ度的患者斜坡的长度(d+e)、枕骨基底部的长度(e)、脑干长度(b)和小脑半球的长度(c)显著大于Ⅱ度和Ⅲ度的患者(P<0.05).合并脊髓空洞患者枕骨大孔径(B to OP)显著小于无脊髓空洞患者(P<0.05),脑干的长度(b)显著长于无脊髓空洞组患者(P<0.05).结论 在Chiari Ⅰ畸形患者中,后颅窝骨性结构及内在神经结构的发育异常,是引起小脑扁桃体下疝及脊髓空洞等病理生理改变的重要原因,在手术治疗中要对此区域的病理改变进行有针对性的治疗,为患者制定合适的手术策略.%Objective To measure the linear structures of posterior cranial fossa in Chiarimalformation (CMI) patients base on fMRI techniques and to investigate the causes associated with different degrees of cerebellar tonsillar descent and syringomyelia. Methods A retrospective radiographic study was performed for 97 CMI patients who were treated in Jishuitan hospital during 2004-2012. According to the degree of cerebellar tonsillar descent, CMI patients were divided into three categories: CMI Ⅰ in 48 cases (49.5%), CMI Ⅱ in 36 cases (37.1%), and CMI Ⅲ in 13

  9. Chiari II malformation. Pt. 4

    Energy Technology Data Exchange (ETDEWEB)

    Naidich, T.P.; McLone, D.G.; Fulling, K.H.

    1983-08-01

    Computed tomography successfully delineates the multiple components of the Chiari II malformation at the craniocervical junction, the hindbrain, and the cervical spinal cord. These include wide foramen magnum and upper cervical spinal canal; incomplete fusions of the posterior arches of C1 and lower cervical vertebrae; cascading protrusions of vermis, fourth ventricle, medulla, and cervical cord into the spinal canal; cervicomedullary ''kinking''; anterior displacement and sequential sagittal compression of each protrusion by the protrusions posterior to it; compression of all protrusions by the posterior lip of foramen magnum and the posterior arch of C1; and associated cervical hydromyelia, cervical diastematomyelia, and cervical arachnoid cysts.

  10. Papilloedema due to Chiari I malformation.

    Science.gov (United States)

    Zhang, Jason Chao; Bakir, Belal; Lee, Andrew; Yalamanchili, Sushma S

    2011-10-16

    The Chiari I malformation is a congenital abnormality characterised by downward displacement of the cerebellar tonsils through the foramen magnum into the cervical spine. It presents clinically most often in young adult women. Known ocular manifestations linked to Chiari I consist primarily of oculomotor paresis with cranial nerve VI palsy and convergence/divergence abnormalities. Papilloedema is a rare manifestation of Chiari I with a clinical presentation often similar to that of idiopathic intracranial hypertension. To highlight this unusual complication, the authors report a 64-year-old female who developed papilloedema as the only presenting neurological symptom resulting from a Chiari I malformation.

  11. Clinical Outcome Measures in Chiari I Malformation.

    Science.gov (United States)

    Yarbrough, Chester K; Greenberg, Jacob K; Park, Tae Sung

    2015-10-01

    Chiari malformation type 1 (CM-I) is a common and often debilitating neurologic disease. Reliable evaluation of treatments has been hampered by inconsistent use of clinical outcome measures. A variety of outcome measurement tools are available, although few have been validated in CM-I. The recent development of the Chicago Chiari Outcome Scale and the Chiari Symptom Profile provides CM-I-specific instruments to measure outcomes in adults and children, although validation and refinement may be necessary.

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

    Science.gov (United States)

    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.

  13. Presenting Symptoms of Chiari Type I Malformation

    Directory of Open Access Journals (Sweden)

    J Gordon Millichap

    2009-06-01

    Full Text Available Clinical and radiographic predictors of neurologic symptoms were investigated in a population-based retrospective study of 51 children identified with Chiari I malformation at the University of California, and Kaiser Department of Radiology, San Francisco.

  14. Terminal hemimyelocystocele associated with Chiari II malformation

    Directory of Open Access Journals (Sweden)

    Umamaheswara Reddy V.

    2014-06-01

    Full Text Available Terminal myelocystocele (TMC results from failure of embryonic CSF to drain outside the neural tube creating CSF reservoir within a dorsal meningocele. Association of Chiari II malformation with diastematomyelia and myelocystocele is extremely rare. Myelocystoceles do not have neural tissue so they have good prognosis after treatment, however when associated with hydromelia and Chiari malformation they present with neurological deficits. We present details of a 2 year old female who presented to us with this rare anomaly.

  15. Congenital Chiari malformations: A review

    Directory of Open Access Journals (Sweden)

    Vannemreddy Prasad

    2010-01-01

    Full Text Available Chiari malformation is the commonest anomaly of the craniovertebral junction involving both the skeletal as well as the neural structures. This entity has rapidly evolved over the past decade with newer visualization techniques, thus posing new challenges to diagnosis and management. This review includes the developmental theories, the latest nomenclature and existing treatment modalities of this interesting anomaly. Five theories tried to explain the malformation of the hindbrain and the neuraxis but no single theory completes the development of embryonic defects. Several atypical presentations have been reported with either incidental/asymptomatic features resulting in further classifications. The new magnetic resonance imaging flow techniques attempt to substantiate the clinical presentations and correlate with the abnormality which can be subtle in correlation. Surgical correction to improve the cranial volume, decrease the hydrocephalus and improve flow across the foramen magnum is the mainstay but needs to be tailored to a given type of malformation. Further clinical and imageological studies, especially longitudinal natural history, might improve our understanding of the atypical/asymptomatic presentations and the management that is currently available.

  16. Electrical Cauterization of Hernia Below Cerebellar Tonsil Combined with the Decompression of Posterior Cranial Fossa for the Treatment of ChiariMalformation Complicated with Syringomyelia%小脑扁桃体热灼加后颅窝减压治疗ChiariⅠ型畸形合并脊髓空洞症

    Institute of Scientific and Technical Information of China (English)

    邹叔骋; 黄红星; 刘博; 张卫民

    2013-01-01

    [目的]探讨小脑扁桃体热灼加后颅窝减压治疗ChiariⅠ型畸形合并脊髓空洞症的手术方式及疗效.[方法]对18岁以上成年患者及健康对照者行MRI检测后颅窝容积相关骨性标志间线性距离并进行比较,对72例MRI证实的Chiari Ⅰ型畸形合并脊髓空洞症患者采用后颅窝正中入路,显微镜下行小脑扁桃体熟灼,松解粘连,使之回缩到枕骨大孔水平以上;后颅窝减压,硬膜扩大缝合.[结果]成年患者后颅窝容积相关骨性标志间线性距离均显著小于健康对照组(P<0.05).72例患者手术顺利,65例术后获得6个月至5年随访,按Tator标准,有效58例,有效率89.2%,无严重并发症.65例患者均复查MRI,63例示下疝之扁桃体均回缩到枕骨大孔水平以上,19例脊髓空洞消失,脊髓形态接近正常,38例脊髓空洞缩小.[结论]采用小脑扁桃体热灼加后颅窝减压是治疗Chiari Ⅰ型畸形合并脊髓空洞症的有效方法.%[Objective]To explore the method and efficacy of electrical cauterization of hernia below cerebellar tonsil combined with the decompression of posterior cranial fossa for the treatment of Chiarimalformation complicated with syringomyelia. [Methods]After MRI was performed in patients over 18 yeas old and healthy controls, the linearity distance among boney landmarks related to posterior cranial fossa volume was measured and compared. Totally 72 patients with Chiarimalformation complicated with syringomyelia confirmed by MRI underwent electrical cauterization of hernia below cerebellar tonsil, separation of the adhesions retracted above foramen magnum, decompression of posterior cranial fossa and extensive suture of dura under microscope through middle posterior cranial fossa approach. [Results]The linearity distance among boney landmarks related to posterior cranial fossa volume of adult patients was significantly shorter than that in control group( P<0. 05). The 72 patients were operated

  17. Surgical treatment of Chiari malformation associated with syringomyelia%Chiari畸形并脊髓空洞症的外科治疗

    Institute of Scientific and Technical Information of China (English)

    孙学东; 李壮志; 张学军

    2004-01-01

    Chiari畸形即慢性小脑扁桃体下疝畸形(Chiari Malformarion,CM)是一种先天性后颅窝容积过小,导致后颅窝的中线脑组织挤压到颈椎管内的颅底凹陷畸形。这种先天性畸形最早由John Cleland医师在1883年首先在解剖上观察到,然而直到1891年才由Chiari医师详细描述。常合并有脊髓空洞症(SM)。其临床表现多以后枕颈部疼痛为主诉,也可以有运动或感觉神

  18. ANAESTHETIC MANAGEMENT IN A PATIENT WITH ARNOLD-CHI ARI MALFORMATION TYPE I AND SYRINGOMYELIA

    Directory of Open Access Journals (Sweden)

    Kartika

    2013-04-01

    Full Text Available ABSTRACT: Syringomyelia is an unusual neurological condition characterised by the the presence of cystic cavity in the spinal cord resultin g in neurological manifestations. Here, we report a safe anesthetic management of patient with Arnold-Ch iari malformation type I and syringomyelia posted for foramen magnum decompression . INTRODUCTION: Arnold-Chiari malformation (ACM is a developmental malformation characterised by downward displacement of cerebellar tonsils into spinal canal due to reduced capacity of the posterior fossa. ACM may be complicate d by other malformations like Platybasia, basilar invagination and occipitalization although S yringomyelia (SM is most commonly seen. [1] There are four types of ACM; types I – IV. Type I ACM manifests with headaches, neck pain, and mild co-ordination problems mostly asymptomatic a nd discovered on brain or cervical spine MRI scans. It has adult onset characterised by downward displacement of cerebellar tonsils and medulla through the foramen magnum. [2] Syringomyelia is an unusual neurological condition characterised by the presence of fluid filled cystic cavity or syrinx within the spin al cord. Ollivies d’ Angers (1827 coined the term syringomyelia from two greek words meaning “ch annel” and “marroin”. [3] It has a prevalence of 8.4 per 100,000 and occurs more frequen tly in men than in women in the third or fourth decade of life. Rarely, it may develop in chi ldhood or late adulthood. [4

  19. 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

    Science.gov (United States)

    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

  20. Spinal Deformity Associated with Chiari Malformation.

    Science.gov (United States)

    Kelly, Michael P; Guillaume, Tenner J; Lenke, Lawrence G

    2015-10-01

    Despite the frequency of Chiari-associated spinal deformities, this disease process remains poorly understood. Syringomyelia is often present; however, this is not necessary and scoliosis has been described in the absence of a syrinx. Decompression of the hindbrain is often recommended. In young patients (<10 years old) and/or those with small coronal Cobb measurements (<40°), decompression of the hindbrain may lead to resolution of the spinal deformity. Spinal fusion is reserved for those curves that progress to deformities greater than 50°. Further research is needed to understand the underlying pathophysiology to improve prognostication and treatment of this patient population.

  1. Abrupt clinical onset of Chiari type I/syringomyelia complex: clinical and physiopathological implications.

    Science.gov (United States)

    Massimi, Luca; Della Pepa, Giuseppe M; Caldarelli, Massimo; Di Rocco, Concezio

    2012-07-01

    Chiari I malformation (CI) continues to raise great interest among physicians due to the larger and larger number of newly diagnosed cases. The clinical and radiological picture and the management options of such a chronic disease are well acknowledged as well as those of the associated syringomyelia. Little is known, on the other hand, about abrupt clinical onset following decompensation of CI/syringomyelia complex. This review on the sudden onset of these two conditions shows that this is a very rare phenomenon; only 41 cases are being reported in the last three decades. In all these cases, acute onset was referable to CI/syringomyelia and the clinical course quickly precipitated. Motor deficits (36.5 %), respiratory failure (29 %), cranial nerve palsy (17 %), and cardiac arrest (14.5 %) were the most common findings, thus confirming that abrupt onset may have severe and life-threatening consequences. Indeed, sudden or early mortality accounted for 19.5 % of cases. In spite of that, most of the surviving subjects had an excellent outcome following either surgical or medical/rehabilitation treatment. Physiopathology of abrupt onset is attributed to the acute compression of the brainstem/upper cervical spinal cord by ectopic tonsils and syringobulbia/syringomyelia, frequently precipitated by a minor injury, followed by impairment of medullary baroreceptors and midbrain reticular substance (cardiac arrest, syncope), medullary chemoreceptors and phrenic nerve nuclei (respiratory failure), lower cranial nerve nuclei (cardiac arrest, cranial nerve palsy), and pyramidal tracts (motor deficits). About 87 % of patients of this review were asymptomatic prior to their acute onset. The problem of the management of asymptomatic subjects is still open.

  2. Fetal syringomyelia.

    Science.gov (United States)

    Guo, Anne; Chitayat, David; Blaser, Susan; Keating, Sarah; Shannon, Patrick

    2014-08-06

    We explored the prevalence of syringomyelia in a series of 113 cases of fetal dysraphism and hindbrain crowding, of gestational age ranging from 17.5 to 34 weeks with the vast majority less than 26 weeks gestational age. We found syringomyelia in 13 cases of Chiari II malformations, 5 cases of Omphalocele/Exostrophy/Imperforate anus/Spinal abnormality (OEIS), 2 cases of Meckel Gruber syndrome and in a single pair of pyopagus conjoined twins. Secondary injury was not uncommon, with vernicomyelia in Chiari malformations, infarct like histology, or old hemorrhage in 8 cases of syringomyelia. Vernicomyelia did not occur in the absence of syrinx formation. The syringes extended from the sites of dysraphism, in ascending or descending patterns. The syringes were usually in a major proportion anatomically distinct from a dilated or denuded central canal and tended to be dorsal and paramedian or median. We suggest that fetal syringomyelia in Chiari II malformation and other dysraphic states is often established prior to midgestation, has contributions from the primary malformation as well as from secondary in utero injury and is anatomically and pathophysiologically distinct from post natal syringomyelia secondary to hindbrain crowding.

  3. Three kinds of operation method in the treatment of Chiarimalformation with syringomyelia curative effect contrast%三种手术方法治疗Chiari Ⅰ畸形并脊髓空洞的疗效对比

    Institute of Scientific and Technical Information of China (English)

    艾克拜尔·亚里坤; 乌拉别克·毛力提; 刘宇利; 杨小朋

    2014-01-01

    Objective To investigate effects of three operation methods in treatment of Chiari Ⅰ with syringomyelia.Methods Retrospective study of patients betwenJune 2005 and June 2012 in The department of Neurosurgery,People's Hospital of Xinjiang Uygur Autonomous Region for a total of 133 cases patient of Chiarimalformation with syrigomyelia.Group A was performed in 20 cases for simple posterior bony decompression; group B was performed in 65 cases for posterior bony decompression + dural enlargement repair;group C was performed in 48 cases for posterior bony decompression + dural enlargement repair + resection of the cerebellar tonsils.To comparison the effect of the operation,syringomyelia resolution and the rate of postoperative complications.Results Outpatient follow up for 6 months-9 years,a total of 118 patients were followed up for.According to the criteria of Tator,A,B,C three groups in the superior,good,poor rate was significant difference (P < 0.05).Promote syringomyelia regression had significant difference(P <0.05).Group B and C have the same effect of operation,better than group A.A,B,C three groups of infection were 1 case,2 cases,1 case; postoperative transient headache were 6 cases,30 cases,25 cases;early postoperative cerebrospinal fliud leakage and subcutaneous effusion were 0 case,8 cases,6 cases ; 1 case of death in group C.The total complication rate had no significant difference (P > 0.05).Conclusion Posterior bony decompression plus dural enlargement repair,posterior bony decompression withdural enlargement repair andResection of the cerebellar tonsils were the same effect,arereliable methods for Chiari Ⅰ with syringomyelia,better than soly posterior bony decompression.%目的 观察三种手术方法治疗ChiariⅠ畸形并脊髓空洞疗效.方法 总结新疆维吾尔自治区人民医院神经外科2005年6月至2012年6月133例ChiariⅠ畸形并脊髓空洞患者,A组行单纯骨性减压20例;B组行

  4. Neural endoscopic assisted micro-invasive management of Chiari I malformation

    Institute of Scientific and Technical Information of China (English)

    DENG Kan; LI Yong-ning; LI Gui-lin; GAO Jun; YANG Zhong; DI Xiao; WANG Ren-zhi

    2010-01-01

    Background In order to make posterior fossa decompression for the management of Chiari I malformation simple and less invasive while using direct visualization, a novel solely endoscopic procedure has been employed for the decompression of Chiari malformation type I. The objective of this study was to present neural endoscopic posterior fossa decompression and atlas laminectomy for Chiari type I patients.Methods Twenty-one patients with Chiari type I underwent neural endoscopic posterior fossa decompression and atlas laminectomy. We described the procedure for neural endoscopic posterior fossa decompression and atlas laminectomy. All patients in this series demonstrated cerebellar tonsil herniation below the foramen magnum in addition to syringomyelia. All patients in the reviewed study underwent preoperative MRI as well as 3-month postoperative MRI. Additional follow-up MRI varied but was usually repeated 12 months to 18 months after surgery. Postoperative MRI studies were retrospectively reviewed and compared with preoperative studies.Results All patients showed clinical improvements, and none had any complications. Patients with syringomyelia had symptoms entirely disappear. Eleven patients (52.4%) experienced radiographic improvement in syringomyelia (decreased size or resolution) during the follow-up period. Nine patients (42.8%) demonstrated decreased syrinx size and four (19%) demonstrated resolved syrinx. Of the 15 patients with symptomatic syringomyelia, 11 (73.3%)experienced symptomatic improvement. The median time to symptom improvement was four months after surgery. Post surgical MRI examinations indicated complete and sufficient decompression of foramen magnum region.Conclusions Endoscope atlanto-occipital decompression surgery is an innovative, safe and effective surgical procedure. It has similar results compared to traditional surgery, however with the added advantages of being minimal invasive, having fewer complications, decreased influence on

  5. Basilar invagination, Chiari malformation, syringomyelia: A review

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    Goel Atul

    2009-01-01

    Full Text Available Institute and personal experience (over 25 years of basilar invagination was reviewed. The database of the department included 3300 patients with craniovertebral junction pathology from the year 1951 till date. Patients with basilar invagination were categorized into two groups based on the presence (Group A or absence (Group B of clinical and radiological evidence of instability of the craniovertebral junction. Standard radiological parameters described by Chamberlain were used to assess the instability of the craniovertebral junction. The pathogenesis and clinical features in patients with Group A basilar invagination appeared to be related to mechanical instability, whereas it appeared to be secondary to embryonic dysgenesis in patients with Group B basilar invagination. Treatment by facetal distraction and direct lateral mass fixation can result in restoration of craniovertebral and cervical alignment in patients with Group A basilar invagination. Such a treatment can circumvent the need for transoral or posterior fossa decompression surgery. Foramen magnum bone decompression appears to be a rational surgical treatment for patients having Group B basilar invagination. The division of patients with basilar invagination on the basis of presence or absence of instability provides insight into the pathogenesis of the anomaly and a basis for rational surgical treatment.

  6. Cerebellar tonsilletomy and reconstruction of the cistern magna for treatment of syringomyelia with Chiari malformation%小脑扁桃体切除并枕大池重建术治疗Arnold-Chiari畸形并脊髓空洞症

    Institute of Scientific and Technical Information of China (English)

    包长顺; 杨福兵; 刘亮; 王斌; 夏祥国; 詹书良; 陈礼刚

    2011-01-01

    目的:探讨小脑扁桃体切除并枕大池重建术治疗Arnold-Chiari畸形并脊髓空洞症(ACM-SM)的手术疗效.方法:对2005年1月至2010年12月收治的109例ACM-SM患者,采用下疝小脑扁桃体切除并行枕大池重建术,按照Tator标准评价手术效果.结果:所有患者无一例死亡或恶化,随访69例患者,65例症状显著改善;核磁共振复查34例,结果显示枕大池均解剖成形,32例脊髓空洞消失或明显缩小.结论:小脑扁桃体切除并枕大池重建术是治疗合并脊髓空洞症的Arnold-Chiari畸形有效、合理的手术方式.%Objective To explore the therapeutic effect of cerebellar tonsillectomy and cistern magna reconstrction in the treatment of Amold-Chiari malformation associated with syringomyelia. Methods A retrospective analysis was conducted among 109 patients undergoing cerebellar tonsilloctomy and cistern magna reconstruction from Jan 2005 to Dec 2010. The outcomes of the surgeries were evaluated using Tator criterion. Results No patient died or clinically aggravated when they discharged from hospital. During the follow-up of 69 patients for a mean of 2 years,65 patients showed obvious clinical improvement. Magetic resonance imaging employed in 34 patients demonstrated that all the cistern magna had been reconstructed and the syringomyelia disappeared or significant reduced in 32 cases. Conclusions Cerebellar tonsillectomy and cistern magna reconstrction should be an effective and advisable method for treament of Arnold-Chiari malformation associated with syringomyelia.

  7. Chiari Ⅰ畸形的发病机制及诊疗进展%Pathogenesis and treatment of Chiarimalformation

    Institute of Scientific and Technical Information of China (English)

    马志明; 王义荣

    2013-01-01

    ChiariⅠ畸形是后脑发育异常的先大性畸形,有关其发病机制有很多学说,如容积学说、遗传学说等.而近几年学者们又根据Chiari Ⅰ畸形伴发脊髓空洞症总结了一些新的学说,但具体机制至今未明确.本文从Chiari Ⅰ畸形的定义、发病机制、临床表现、诊断及治疗等角度对该疾病进行综述.%Chiarimalformation is a congenital anomaly of posterior brain.There has been many theories for its pathogenesis.Recent years,researchers have sunmarized new theories about the pathogenesis of syringomyelia associated Chiarimalformation.However,the exact pathogenesis of Chiarimalformation has not been clarified.There are a lot of surgical treatments of Chiarimalformation.Importantly,there remains no consensus about the best and generally applicable treatment.

  8. Large tentorium meningioma causing chiari malformation type-1 with syringomalia with complete resolution of syrinx and chiari after surgical excision: rare case report with review of literature

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    Kankane Vivek Kumar

    2016-03-01

    Full Text Available A 35-year-old female was admitted with a 3-year history of headache, gait disturbance and vertigo on & off and one year history of nasal regurgitation. Magnetic resonance imaging demonstrated a large tentorium meningioma left sided and syringomyelia in the upper cervical cord associated with caudal displacement of the cerebellar tonsil (chiari type -1 malformation. Herniation of the cerebellar tonsil and distortion of the brain stem had probably caused disturbance of cerebrospinal fluid flow which combined with obstruction of the spinal canal, caused the syrinx. Complete excision of the tumor resulted in symptomatic improvement of these symptoms with complete resolution of syrinx & chiari.

  9. Pathogenesis and Cerebrospinal Fluid Hydrodynamics of the Chiari I Malformation.

    Science.gov (United States)

    Buell, Thomas J; Heiss, John D; Oldfield, Edward H

    2015-10-01

    This article summarizes the current understanding of the pathophysiology of the Chiari I malformation that is based on observations of the anatomy visualized by modern imaging with MRI and prospective studies of the physiology of patients before and after surgery. The pathogenesis of a Chiari I malformation of the cerebellar tonsils is grouped into 4 general mechanisms.

  10. Craniovertebral Junction Instability in the Setting of Chiari I Malformation.

    Science.gov (United States)

    Goldstein, Hannah E; Anderson, Richard C E

    2015-10-01

    This article addresses the key features, clinical presentation, and radiographic findings associated with craniovertebral junction instability in the setting of Chiari I malformation. It further discusses surgical technique for treating patients with Chiari I malformation with concomitant craniovertebral junction instability, focusing on modern posterior rigid instrumentation and fusion techniques.

  11. 后颅窝扩大重建术促进Chiari畸形合并脊髓空洞症患者上下肢运动及感觉功能恢复%Restoration of motor and sensory functions of upper and lower extremities by posterior fossa reconstruction in patients with Chiari malformation combined with syringomyelia

    Institute of Scientific and Technical Information of China (English)

    赵丽萍; 王丹玲; 石英

    2003-01-01

    目的观察后颅窝扩大重建术后 Chiari畸形合并脊髓空洞症患者上下肢运动及感觉功能的恢复情况,探讨此术式对 Chiari畸形合并脊髓空洞症脊髓功能恢复的作用.方法所有患者被分为两组:后颅窝扩大重建术组 (posterior fossa reconstruction,PFR组 )共 56例患者;后颅窝减压术组 (posterior fossa decompression,PFD组 )共 62例患者.定期随访.按 NCSS (Neurosurgical Cervical Spine Scale)定量分析上下肢运动及感觉障碍恢复情况,其结果作统计学分析.结果 PFR组的上、下肢和感觉功能的有效率分别为 76% ,71% ,70%; PFD组的上、下肢和感觉功能的有效率分别为 58% ,60% ,66%.两组上下肢及感觉障碍的有效率经χ 2检验差异无统计学意义.结论 PFR 能明显促进 Chiari畸形合并脊髓空洞症患者上下肢运动及感觉障碍的功能恢复.%Aim To explore effect of posterior fossa reconstruction on restoration of spinal cord function by observing the effect of posterior fossa reconstruction(PFR) on functional restoration of spine in patients with Chiari malformation and syringomyelia.Methods 118 patients with Chiari malformation and syringomyelia were divided into 2 groups: Posterior fossa reconstruction(PFR)group(56 patients) and Posterior fossa decompression(PFD)group(62 patients).All the patients were followed up at regular time.Quantitative analysis of the recovery of motor and sensory function in upper and lower extremities according to NCSS (Neurosurgical Cervical Spine Scale) in the two groups had been done. The results were statistically analysed.Results The efficiency rates of the motor function recovery in the upper and lower extremities and the recovery of the sensory disturbance were 76% ,71% ,70% respectively in PFR group and 58% ,60% ,66% in PFD group.The difference of the results in the two groups was not significant according to χ 2 test.Conclusion PFR can promote the recovery of motor and sensory functions of upper

  12. Chiari type I malformation in children.

    Science.gov (United States)

    Massimi, L; Novegno, F; di Rocco, C

    2011-01-01

    The diagnosis of Chiari type I malformation (CIM) is more and more frequent in clinical practice due to the wide diffusion of magnetic resonance imaging. In many cases, such a diagnosis is made incidentally in asymptomatic patients, as including children investigated for different reasons such as mental development delay or sequelae of brain injury. The large number of affected patients, the presence of asymptomatic subjects, the uncertainties surrounding the pathogenesis of the malformation, and the different options for its surgical treatment make the management of CIM particularly controversial.This paper reports on the state of the art and the recent achievements about CIM aiming at providing further information especially on the pathogenesis, the natural history, and the management of the malformation, which are the most controversial aspects. A historial review introduces and explains the current classification. Furthermore, the main clinical, radiological, and neurophysiological findings of CIM are described to complete the picture of this heterogeneous and complex disease.

  13. Co-existence of Chiari malformation type I and Epstein-Barr virus meningoencephalitis in a 3-year-old child: case report and review of the literature

    Energy Technology Data Exchange (ETDEWEB)

    Solomou, E.K.; Kotsarini, C.; Badra, F.A. [University Hospital of Patras, Department of Radiology, Rio (Greece); Krepis, A.; Papanastasiou, D. [University Hospital of Patras, Pediatric Clinic, Patras (Greece); Patriarcheas, G. [Diagnostic Centre B Diagnosis, Patras (Greece)

    2005-01-01

    In the present report we describe an unusual case of a 3-year-old girl who was admitted to our hospital with Epstein-Barr virus meningoencephalitis. Brain magnetic resonance imaging revealed diffuse abnormalities in white matter and Chiari I malformation with cervical and thoracic hydro-syringomyelia. (orig.)

  14. 原位硬膜条返转引流治疗Arnold-Chiari畸形合并脊髓空洞症%The orthotopic drainage with displaying strip of dura in syrinx cavity for treatment of syringomyelia combined with Arnold-Chiari malformation

    Institute of Scientific and Technical Information of China (English)

    秦家振; 赵国良; 戴宜武; 赵春平; 罗永春; 李运军

    2009-01-01

    @@ Arnold-Chiari畸形(Amold-Chiari malfomation,ACM)是一种以小脑扁桃体下疝为特征的先天性畸形,脊髓空洞(syringomyelia,SM)为其常见的合并症,在治疗方法上争论较多,对手术效果亦评说不定.

  15. Chiari-I malformation in two fighter pilots.

    Science.gov (United States)

    Akin, Ahmet; Canakci, Zafer; Sen, Ahmet; Tore, Hasan F

    2003-07-01

    This report describes two cases of Chiari Malformation Type I (Chiari-I) in fighter pilots of the Turkish Air Force. Chiari-I is a congenital malformation characterized by herniation of cerebellar tonsils through the foramen magnum. Patients have symptoms and signs related to dysfunction of the brainstem, spinal cord, and cerebellum. They generally are symptomatic in the earlier years of life. However, asymptomatic cases can eventually become symptomatic in later years. Symptoms can be provoked by increasing intracranial pressure (Valsalva or straining). We report on two pilots with Chiari-I malformation who had no symptoms or signs in their daily activities. Furthermore, these pilots had successfully completed physiological training, including centrifuge training, without any symptoms. However, they suffered from headache, neck spasms, and/or disequilibrium under +Gz during flight training sorties. The clinical presentation, diagnosis, treatment, possibility of acquired cases, and aeromedical disposition of Chiari malformations are discussed.

  16. Acquired Chiari malformation type I associated with a supratentorial fistulous arteriovenous malformation: a case report.

    Science.gov (United States)

    Chen, Kuo-Wei; Kuo, Meng-Fai; Lee, Chung-Wei; Tu, Yong-Kwang

    2015-03-01

    A case of acquired Chiari malformation type I with frontal fistulous arteriovenous malformation (AVM) is presented, and the pathophysiology is discussed. The tonsillar herniation and hydrocephalus both resolved after AVM was excised. This case provides some insight into the complex hemodynamic change exerted by the fistulous AVM and the mechanism of the development of acquired Chiari malformation type I.

  17. Stenogyria - not only in Chiari II malformation.

    Science.gov (United States)

    Bekiesinska-Figatowska, Monika; Duczkowska, Agnieszka; Brągoszewska, Hanna; Duczkowski, Marek; Mierzewska, Hanna

    2014-12-15

    Stenogyria, meaning multiple small compacted gyri separated by shallow sulci, is reported in the literature in association with Chiari II malformation (CM II) which in turn is reported in association with myelomeningocele (MMC). The authors present five cases of stenogyria (and other abnormalities found in CM II, like callosal hypoplasia/dysplasia, agenesis of the anterior commissure, hypoplasia of the falx cerebri) in children without the history of MMC or any other form of open spinal dysraphism. In these cases stenogyria was associated with Chiari I malformation, rhombencephalosynapsis and spina bifida. Stenogyria, which is not a true neuronal migration disorder, should not be mistaken for polymicrogyria which is also present in CM II. It is histologically different from polymicrogyria because the cortex is normally organized. Also on MRI, the general sulcal pattern is preserved in stenogyria, while it is completely distorted in polymicrogyria. The authors conclude that features traditionally attributed to CM II, like stenogyria, occur not only in the population of patients with MMC as opposed to the widely accepted theory.

  18. Optical Coherence Tomography in Patients with Chiari I Malformation

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    Michele Figus

    2015-01-01

    Full Text Available Background/Aims. To evaluate optic nerve head with spectral domain optical coherence tomography (OCT in patients with Chiari I malformation (CMI compared to healthy controls. Methods. Cross-sectional study. OCT of the optic nerve head of 22 patients with CMI and 22 healthy controls was quantitatively analyzed. The healthy controls were matched for age and sex with the study population. Mean retinal nerve fiber layer (RNFL thickness was calculated for both eyes; the mean thickness value was also registered for each quadrant and for each subfield of the four quadrants. Results. CMI patients showed a reduction of the RNFL thickness in both eyes. This reduction was more statistically significant (P<0.05 for the inferior quadrant in the right eye and in each quadrant than nasal one in the left eye. Conclusion. A distress of the retinal nerve fibers could explain the observed reduction of the RNFL thickness in patients with CMI; in our series the reduction of the RNFL thickness seems lower when CMI is associated with syringomyelia.

  19. Effect of decompression window area of posterior fossa on short-term surgical outcomes in Chiari malformation patients with syringomyelia%Chiari畸形合并脊髓空洞症术中不同大小后颅窝减压窗近期疗效的比较

    Institute of Scientific and Technical Information of China (English)

    刘彬; 王振宇; 谢京诚; 李振东; 马长城; 陈晓东

    2016-01-01

    Objective To explore the effect of decompression skull window area on short-term surgical outcomes in the Chiari malformation (CM) patients with syringomyelia (SM). Methods The clinical data of 117 patients with CM and SM treated in our hospital from 2007 to 2010 were analyzed retrospectively. The decompression of posterior cranial fossa and duraplasty was performed in all the patients. The decompression of syringomyelias with puncture was performed in the patients in whom the horizontal diameters of the syringomyelias were more than 50%of the horizontal diameters of the spinal cords. The patients were divided into 2 groups according to the decompressed area of the posterior cranial fossa, i.e. group A (n=87) where the decompression skull window area was about 12 cm2 and group B (n=30) where the skull window area was less than 6 cm2. The therapeutic effects were evaluated with Tater scale 2 weeks after the operation in all the patients. Results Of 117 patients, 97 were improved in the postoperative symptoms and 20 not. The rate (87.4%, 76/87) of improved symptoms was significantly higher in group A than that (70.0%, 21/30) in group B (P脊髓50%者行空洞穿刺减压术.根据后颅窝减压窗面积分为2组:Ⅰ组,87例,减压面积为12 cm2(3 cm×4 cm);Ⅱ组,30例,减压面积<6 cm2.术后2周采用Tater评分评定疗效.结果 117例患者手术后症状均无加重,症状改善97例(82.9%),无变化20例.Ⅰ组症状改善76例(87.4%),Ⅱ组为21例(70%);两组症状改善率差异显著(P<0.05).结论 Chiari畸形合并脊髓空洞症患者的后颅窝减压面积以不小于12 cm2的近期疗效较好.

  20. 枕大池成形术对Chiari畸形Ⅰ型伴脊髓空洞症患者疗效及脑脊液动力学的影响%Effect of cisterna magna plastic repairing on cerebrospinal fluid dynamics in Chiari malformation type Ⅰ with syringomyelia

    Institute of Scientific and Technical Information of China (English)

    包长顺; 陈明生; 杨福兵; 张苓; 刘亮; 王斌; 陈礼刚

    2015-01-01

    Objective To explore the effect of cisterna magna plastic repairing (posterior vertebral column resection and cerebella tonsillectomy and reconstruction of the cisterna magna by artificial dura) on cerebrospinal fluid dynamics in Chiari malformation type Ⅰ with syringomyelia and evaluate the efficacy of this surgical method.Methods Forty patients with Chiari malformation type Ⅰ with syringomyelia (CMI-SM),admitted to and diagnosed by MRIin our hospital from January 2011 to December 2013,were selected.All patients were treated with cistema magna plastic repairing.Phase-contrast MR imaging (PC-MRI) was performed,changes of cerebrospinal fluid dynamics indicators of cerebral aqueduct,pons ventral side and C3 ventral side were detected and compared and Japanese Orthopaedic Association (JOA) scale scores were evaluated 24 h before operation and 12 months after operation.Results No neurological deterioration or death was noted;JOA scores in all patients were increased from 7.89±3.52 to 12.53±4.32,with significant difference (P<0.05);post-operated PC-MRI indicated that the cistema magna formed well,spinal subarachnoid space was clear,syringomyelia in 30 patients was significantly reduced;as compared with those before operation,the stroke volume and mean flux were significantly increased,but maximum peak velocity was significantly reduced (P<0.05).Conclusion Cisterna magna plastic repairing can reduce syringomyelia,increase cerebrospinal fluid flow,and reduce peak flow of cerebrospinal fluid,which is an effective surgical procedure for Chiari malformation type I with syringomyelia.%目的 探讨枕大池成形术(后颅窝减压+小脑扁桃体部分切除+硬膜成形)对Chiari畸形Ⅰ型伴脊髓空洞症(CMI-SM)患者疗效及脑脊液动力学的影响. 方法 泸州医学院附属医院神经外科自2011年1月至2013年12月应用枕大池成形手术治疗CMI-SM患者40例,术前24 h及术后12个月患者均行核磁共振相位对比序列(PC-MRI)检

  1. Type I Chiari malformation presenting central sleep apnea.

    Science.gov (United States)

    Kitamura, Takuro; Miyazaki, Soichiro; Kadotani, Hiroshi; Kanemura, Takashi; Okawa, Masako; Tanaka, Toshihiko; Komada, Ichiro; Hatano, Taketo; Suzuki, Hideaki

    2014-04-01

    Sleep apnea is a rare but a well-known clinical feature of type I Chiari malformation. It may be obstructive or central in nature. Sleep apnea in patients with type I Chiari malformation rarely presents without accompanying neurological signs or symptoms. We here report a case of a 10-year-old girl who presented with central sleep apnea without any other neurological signs but was ultimately diagnosed with type I Chiari malformation. The patient initially showed mild improvement in symptoms after administration of an acetazolamide. Finally, posterior fossa decompression dramatically improved her respiratory status during sleep, both clinically and on polysomnography. This case suggests that type I Chiari malformation should be considered in the differential diagnoses of central apneas in children, even if there are no other neurological signs and symptoms. Furthermore, sagittal craniocervical magnetic resonance imaging may be necessary for a definitive diagnosis.

  2. Arnold-Chiari Malformation Type I and II in Iranian Adults: Clinical and Radiologic Features

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    M. Masuomian

    2008-01-01

    Full Text Available Background/Objective: To evaluate clinical and ra-diological presentations of Arnold chiari malforma-tion in Iranian adults."nPatients and Methods: eighty patients with the clini-cal and radiological features of the chiari malforma-tions were evaluated by using computed tomography scan (CT scan or MRI, from 2001 to 2005 in our uni-versity-affiliated hospital. Surgical confirmation of the diagnosis was obtained in all patients."nResults: The mean age of our cases was 26.7 years (18-58 years.Our patients consisted of 14 (77.7% male and four (22.3% female. The most common presenting symptom was sensory complaints in eight patients (44.5%. Headache, gait disturbances, vertigo and cerebellar dysfunction (vertigo and nystagmus, were seen in 6 (33.3%, 4 (22.2%, 3 (16.6% and 2 (11.1% patients respectively. According to surgery, 15 (83.3% were classified as chiari I malformation, while three patients (16.7% fulfilled the anatomic criteria of chiari II. Imaging showed that all patients had cerebellar herniation. Hydrocephaly, Meningo-myelocele, syringomyelia, cerebellar atrophy and corpus callosum agenesis were seen in five (27.7%, 3 (16.7%, 6 (33.3%, 2 (11.1% and one (5.1% patients respectively. "nConclusion: Chiari type I was more common than type II in our patients such as others and syringemye-lia was the most common imaging abnormality after cerebellar herniation.

  3. A rare case of Chiari type-1 malformation accompanied by symptomatic cerebrospinal fluid hypovolemia: comparison of congenital Chiari type-1 malformation and acquired Chiari malformation secondary to cerebrospinal fluid hypovolemia: case report.

    Science.gov (United States)

    Hatae, Ryusuke; Kohri, Ryusuke; Maeda, Kazushi; Miyazono, Masayuki

    2014-01-01

    A 23-year-old woman was injured in a rear-end collision. She had general malaise and posterior neck pain, which were more severe when she was in an upright position. Magnetic resonance imaging (MRI) revealed the presence of cerebellar tonsil descensus and syringomyelia in the spinal cord. Radioisotope (RI) cisternography showed signs of an early accumulation of RI in the bladder, and a delayed accumulation of RI in the cerebral fornix. We considered the possibilities of cerebrospinal fluid (CSF) hypovolemia and congenital Chiari type-1 malformation as being responsible for her headache. To obtain a definitive diagnosis, we performed gadolinium (Gd)-enhanced MR cisternography and found evidence of CSF leakage. We performed an epidural blood patch (EBP), and her symptoms resolved. In 2 years since the episode, her symptoms have not recurred, and additional treatment has not been required. In addition, MRI performed 2 years after the EBP did not reveal any changes. There seems no previous report which described successful differentiation of pre-existing congenital Chiari type-1 malformation from the acquired one caused by symptomatic CSF hypovolemia. Because treatment protocols differ between these two conditions, the establishment of a correct diagnosis is important.

  4. Changes in cerebrospinal fluid flow assessed using intraoperative MRI during posterior fossa decompression for Chiari malformation.

    Science.gov (United States)

    Bond, Aaron E; Jane, John A; Liu, Kenneth C; Oldfield, Edward H

    2015-05-01

    OBJECT The authors completed a prospective, institutional review board-approved study using intraoperative MRI (iMRI) in patients undergoing posterior fossa decompression (PFD) for Chiari I malformation. The purpose of the study was to examine the utility of iMRI in determining when an adequate decompression had been performed. METHODS Patients with symptomatic Chiari I malformations with imaging findings of obstruction of the CSF space at the foramen magnum, with or without syringomyelia, were considered candidates for surgery. All patients underwent complete T1, T2, and cine MRI studies in the supine position preoperatively as a baseline. After the patient was placed prone with the neck flexed in position for surgery, iMRI was performed. The patient then underwent a bone decompression of the foramen magnum and arch of C-1, and the MRI was repeated. If obstruction was still present, then in a stepwise fashion the patient underwent dural splitting, duraplasty, and coagulation of the tonsils, with an iMRI study performed after each step guiding the decision to proceed further. RESULTS Eighteen patients underwent PFD for Chiari I malformations between November 2011 and February 2013; 15 prone preincision iMRIs were performed. Fourteen of these patients (93%) demonstrated significant improvement of CSF flow through the foramen magnum dorsal to the tonsils with positioning only. This improvement was so notable that changes in CSF flow as a result of the bone decompression were difficult to discern. CONCLUSIONS The authors observed significant CSF flow changes when simply positioning the patient for surgery. These results put into question intraoperative flow assessments that suggest adequate decompression by PFD, whether by iMRI or intraoperative ultrasound. The use of intraoperative imaging during PFD for Chiari I malformation, whether by ultrasound or iMRI, is limited by CSF flow dynamics across the foramen magnum that change significantly when the patient is

  5. Surgical Outcome of Adult Idiopathic Chiari Malformation Type 1

    Science.gov (United States)

    Yuh, Woon Tak; Kim, Chi Heon; Kim, Hyun-Jib; Jahng, Tae-Ahn; Park, Sung Bae

    2016-01-01

    Objective The pathophysiology of idiopathic Chiari malformation (CM) type 1 is disturbance of free cerebrospinal fluid (CSF) flow and restoration of normal CSF flow is the mainstay of treatment. Additional migration of the medulla oblongata in pediatric patients is referred to as CM type 1.5, but its significance in adult patients is unknown. This study is to compare surgical outcomes of adult idiopathic CM type 1.5 with that of type 1. Methods Thirty-eight consecutive adult patients (M : F=11 : 27; median, 33.5; range, 18–63) with syringomyelia due to idiopathic CM type 1 were reviewed. Migration of the medulla oblongata was noted in 13 patients. The modified McCormick scale (MMS) was used to evaluate functional status before and one year after surgery. All patients underwent foramen magnum decompression and duroplasty. Factors related to radiological success (≥50% decrease in the diameter of the syrinx) were investigated. The follow-up period was 72.7±55.6 months. Results Preoperative functional status were MMS I in 11 patients and MMS II in 14 of CM type 1 and MMS I in 8 and II in 5 of CM type 1.5. Of patients with MMS II, 5/14 patients in group A and 3/5 patients in group B showed improvement and there was no case of deterioration. Radiological success was achieved in 32 (84%) patients and restoration of the cisterna magna (p=0.01; OR, 46.5) was the only significant factor. Conclusion Migration of the medulla oblongata did not make a difference in the surgical outcome when the cisterna magna was restored. PMID:27651871

  6. Delamination technique together with longitudinal incisions for treatment of Chiari I/syringomyelia complex: a prospective clinical study

    Directory of Open Access Journals (Sweden)

    Savas Yildiray

    2009-06-01

    Full Text Available Abstract Background Treatment modalities in Chiari malformation type 1(CMI accompanied by syringomyelia have not yet been standardized. Pathologies such as a small posterior fossa and thickened dura mater have been discussed previously. Various techniques have been explored to enlarge the foramen magnum and to expand the dura. The aim of this clinical study was to explore a new technique of excision of the external dura accompanied by widening the cisterna magna and making longitudinal incisions in the internal dura, without disturbing the arachnoid. Methods Ten patients with CMI and syringomyelia, operated between 2004 and 2006, formed this prospective series. All cases underwent foramen magnum decompression of 3 × 3 cm area with C1–C2 (partial laminectomy, resection of foramen magnum fibrous band, excision of external dura, delamination and widening of internal dura with longitudinal incisions. Results Patients were aged between 25 and 58 years and occipital headache was the most common complaint. The mean duration of preoperative symptoms was 4 years and the follow-up time was 25 months. Clinical progression was halted for all patients; eight patients completely recovered and two reported no change. In one patient, there was a transient cerebrospinal fluid (CSF fistula that was treated with tissue adhesive. While syringomyelia persisted radiologically with radiological stability in five patients; for three patients the syringomyelic cavity decreased in size, and for the remaining two it regressed completely. Conclusion Removal of the fibrous band and the outer dural layer, at level of foramen magnum, together with the incision of inner dural layer appears to be good technique in adult CMI patients. The advantages are short operation time, no need for duraplasty, sufficient posterior fossa decompression, absence of CSF fistulas as a result of extra arachnoidal surgery, and short duration of hospitalization. Hence this surgical technique has

  7. Large occipito-cervical encephalocele with Chiari III malformation

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    S Ambekar

    2011-01-01

    Full Text Available Chiari type III is the rarest of the Chiari malformations and is usually associated with high morbidity and mortality. Treatment consists of primary closure of the encephalocele with or without cerebro-spinal fluid (CSF shunting. In our case, the patient was treated with ventriculoperitoneal shunt followed by excision of the encephalocele. We propose that large encephaloceles should be treated with CSF shunting prior to repair of the sac so as to achieve optimal result.

  8. Sports participation with Chiari I malformation.

    Science.gov (United States)

    Strahle, Jennifer; Geh, Ndi; Selzer, Béla J; Bower, Regina; Himedan, Mai; Strahle, MaryKathryn; Wetjen, Nicholas M; Muraszko, Karin M; Garton, Hugh J L; Maher, Cormac O

    2016-04-01

    OBJECT There is currently no consensus on the safety of sports participation for patients with Chiari I malformation (CM-I). The authors' goal was to define the risk of sports participation for children with the imaging finding of CM-I. METHODS A prospective survey was administered to 503 CM-I patients at 2 sites over a 46-month period. Data were gathered on imaging characteristics, treatment, sports participation, and any sport-related injuries. Additionally, 81 patients completed at least 1 subsequent survey following their initial entry into the registry and were included in a prospective group, with a mean prospective follow-up period of 11 months. RESULTS Of the 503 CM-I patients, 328 participated in sports for a cumulative duration of 4641 seasons; 205 of these patients participated in contact sports. There were no serious or catastrophic neurological injuries. One patient had temporary extremity paresthesias that resolved within hours, and this was not definitely considered to be related to the CM-I. In the prospective cohort, there were no permanent neurological injuries. CONCLUSIONS No permanent or catastrophic neurological injuries were observed in CM-I patients participating in athletic activities. The authors believe that the risk of such injuries is low and that, in most cases, sports participation by children with CM-I is safe.

  9. MR imaging of syringomyelia

    Energy Technology Data Exchange (ETDEWEB)

    Itabashi, Takashi; Yuyama, Takuo; Arimizu, Noboru and others

    1988-09-01

    Magnetic resonance imaging examinations were reviewed on a total of 31 patients with syringomyelia. Magnetic resonance imaging gave a diagnostic accuracy comparable to delayed computed tomography-myelography (delayed-CTM) for syringomyelia. It is superior to delayed-CTM in diagnosing it at a higher level of the spinal cord. When Chiari malformation was coexistent with syringomyelia, lesions were occasionally seen over the total spinal cord. Medullary syringomyelia was seen in three of the five patients with spinal tumor. Liquid within the cavity was depicted as hypointensity area on T2-weighted images in most of the patients with either idiopathic syringomyelia or the association of Chiari malformation. Similar finding was seen in two patients with traumatic syringomyelia, in whom the cavity spread upward and downward from the traumatic site. In cases of syringomyelia associated with spinal tumor, however, the inside of the cavity, as well as tumor tissues, was depicted as hyperintensity area on T2-weighted images, which has important implications for the association of spinal tumor. (Namekawa, K.).

  10. Chiari畸形1型临床和MRI表现与脊髓空洞症的关系%Correlation between the clinical and MRI findings of Chiari malformation and that of cyringomyelia

    Institute of Scientific and Technical Information of China (English)

    张丕逊; 徐建明; 杜崇禧; 赵建伟; 赵玉林

    2003-01-01

    @@小脑扁桃体下疝畸形(Chiari malformation,CM),系先天性脑发育异常,以Ⅰ型(CMI)最常见.本文通过分析经手术的116例CMI临床和MBI所见探讨其与脊髓空洞症(syringomyelia,SM)之关系.……

  11. Progress on Arnold-Chiari Malformation%Arnold-Chiari畸形的研究进展

    Institute of Scientific and Technical Information of China (English)

    周冲; 刘亮

    2015-01-01

    Arnold - Chiari malformation is one kind of congenital dysplastic diseases, characterized by tonsil of cerebellum bulging into foramen magnum plane or canalis vertebralis,mainly caused by structural abnormality of skeleton and nervous tissue of cranio-cervical junction region,always complicated with syringomyelia. The pathogenesis of Arnold-Chiari malformation is not completely clear so far,may be correlated with gene mutation and decrease of posterior cranial fossa volume. The classification criterion of Arnold-Chiari malformation is not unified yet,can be classified as 0 -,Ⅰ-,1. 5 -,Ⅱ-,Ⅲ-,Ⅳ-type at present. Posterior cranial fossa decompression is the main operation of Arnold-Chiari malformation,and various modified operations appeared based on posterior cranial fossa decompression,but the operating steps and methods are still disputable. This paper reviewed the etiology,pathogenesis,classification criterion,clinical manifestation,diagnostic criteria, related examinations and operations of Arnold-Chiari malformation.%Arnold-Chiari畸形是一种先天发育异常性疾病,以颅颈交界区骨架和神经组织结构异常导致小脑扁桃体疝至枕骨大孔平面以下或进入椎管中为特征,常伴有脊髓空洞症。Arnold-Chiari畸形的发病机制至今尚未完全明确,可能与基因突变或后颅窝容积减小等有关。目前Arnold-Chiari畸形尚未统一分型,一般分为0、Ⅰ、1.5、Ⅱ、Ⅲ、Ⅳ型。后颅窝减压术是目前治疗Arnold-Chiari畸形的主要术式,在此基础上有许多改良术式产生,但手术过程中的操作步骤及具体方法仍存在争议。本文就Arnold-Chiari畸形的病因和发病机制、分型命名、临床表现、诊断标准及相关检查、手术治疗进行综述。

  12. Two Cases of Arnold-Chiari Malformation with Respiratory Failure

    Directory of Open Access Journals (Sweden)

    Sinem Iliaz

    2014-03-01

    Full Text Available Arnold–Chiari malformation is defined as downward displacement of the brainstem and cerebellum through the foramen magnum. It has different clinical presentations and four subtypes. It is known that downward migration of posterior fossa components through the foramen magnum and associated lower cranial nerve palsy and brainstem compression can cause respiratory failure. Acute respiratory failure could mark the onset of the disease. Posterior fossa decompression performed to treat primary disease can improve the central sleep abnormalities. As respiratory failure is rarely seen, this paper presents two cases of Arnold–Chiari malformation with respiratory failure.

  13. 小脑扁桃体下疝并脊髓空洞症的诊治进展%Advances in diagnosis and treatment of Chiari malformation associated with syringomyelia

    Institute of Scientific and Technical Information of China (English)

    苏雨行; 李刚

    2006-01-01

    小脑扁桃体下疝畸形也称Chiari畸形(Chiari malformation,CM),是一种以小脑扁桃体向下疝人枕骨大孔为特征的先天性畸形,脊髓空洞症(syringomyelia,SM)为其常见的合并症。本文就CM并SM的诊治进展作一综述。

  14. Natural history of scoliosis after posterior fossa decompression in patients with Chiari malformation/syringomyelia%Chiari畸形合并脊髓空洞患者后颅窝减压术后脊柱侧凸的自然转归分析

    Institute of Scientific and Technical Information of China (English)

    沙士甫; 朱泽章; 邱勇; 孙旭; 钱邦平; 刘臻; 闫煌; 江龙

    2014-01-01

    目的 评估Chiari畸形/脊髓空洞患者后颅窝减压术(PFD)后脊柱侧凸的自然进展并分析影响侧凸转归的相关因素.方法 对2002年1月至2007年12月期间南京大学医学院附属鼓楼医院脊柱外科PFD治疗的26例Chiari畸形/脊髓空洞伴脊柱侧凸患者的影像学资料进行回顾性分析,记录PFD术前及末次随访时主弯Cobb角、弯型、矢状面参数(胸椎后凸、胸腰段交界后凸、腰椎前凸)、小脑扁桃体下疝程度、空洞大小及长度.定义末次随访Cobb角小于或等于行后颅窝减压术时5°为侧凸未进展(未进展组),大于行后颅窝减压术时5°以上为侧凸进展(进展组).运用成组设计资料t检验和Fisher精确检验比较两组PFD术前各项参数间的差异.结果 26例患者接受PFD手术时平均年龄6.5 ~14.7(10.3±2.4)岁,主弯Cobb角20 ~45(33.5±7.7)°.术后24~118(57.5±26.9)个月随访时,11例(42%)患者脊柱侧凸获得稳定或改善.进展组平均初始年龄显著大于非进展组(11.1±2.2)比(9.2±2.2)岁,P=0.041,此外进展组中胸腰双弯比例显著高于非进展组(26.7%比9.1%,P=0.037).而Cobb角、Risser征、矢状面参数、小脑扁桃体下疝程度、空洞大小及长度、术前伴神经症状/体征比例在两组间差异均无统计学意义(均P >0.05).结论 后颅窝减压术后42%的Chiari畸形/脊髓空洞患者可获得脊柱侧凸的稳定或改善.PFD手术时年龄较大、胸腰双弯弯型可能是术后脊柱侧凸进展的危险因素.%Objective To explore the natural history of scoliosis after posterior fossa decompression (PFD) in patients with Chiari malformation/syringomyelia and examine the risk factors associated with curve progression.Methods A retrospective radiographic study was performed at our scoliosis center for 26patients undergoing PFD for Chiari malformation between January 2002 and December 2007.Their clinical and radiological parameters,including age

  15. Chiari type 1 malformation in a pseudotumour cerebri patient: is it an acquired or congenital Chiari malformation?

    Science.gov (United States)

    Istek, Seref

    2014-06-04

    Chiari malformation type 1 (CM1) is a developmental abnormality of the cerebellar tonsils. Patients with CM1 commonly present with headache. Papilloedema is rarely seen in CM1. However, a 52-year-old woman presented to the hospital with a headache and her ophthalmological examination revealed bilateral papilloedema. Her cranial MRI was compatible with borderline CM1. Bilateral papilloedema and headache suggested idiopathic intracranial hypertension (IIH) as the preliminary diagnosis. IIH is a rare case in CM1. This article argues about this association and discusses as to whether it is an acquired or congenital Chiari malformation.

  16. Nuclear magnetic resonance (NMR) imaging of Arnold-Chiari type I malformation with hydromyelia

    Energy Technology Data Exchange (ETDEWEB)

    DeLaPaz, R.L.; Brady, T.J.; Buonanno, F.S.; New, P.F.; Kistler, J.P.; McGinnis, B.D.; Pykett, I.L.; Taveras, J.M.

    1983-02-01

    Saturation recovery nuclear magnetic resonance (NMR) images and metrizamide computed tomography (CT) scans were obtained in an adult patient with a clinical history suggestive of syringomyelia. Both NMR and CT studies showed low lying cerebellar tonsils. The CT study demonstrated central cavitation of the spinal cord from the midthoracic to midcervical levels but could not exclude an intramedullary soft tissue mass at the cervico-medullary junction. The NMR images in transverse, coronal, and sagittal planes demonstrated extension of an enlarged central spinal cord cerebrospinal fluid space to the cervico-medullary junction. This was felt to be strong evidence for exclusion of an intramedullary soft tissue mass and in favor of a diagnosis of Arnold-Chiari Type I malformation with hydromyelia. The noninvasive nature of spinal cord and cervico-medullary junction evaluation with NMR is emphasized.

  17. Persistent primitive hypoglossal artery associated with Chiari II malformation: Diagnosis and clinical implications

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    Gupta Mudit

    2010-01-01

    Full Text Available We present a case of persistent primitive hypoglossal artery (PPHA associated with Chiari II malformation and discuss the clinical implications. There has been one reported case of PPHA associated with Chiari 1 malformation, but none in association with Chiari II. Our patient also had a widened hypoglossal canal, with cerebrospinal fluid (CSF sac herniation through it.

  18. ChiariⅠ型畸形及伴随病变的MRI分析%MRI Study of ChiariMalformation and its Complication

    Institute of Scientific and Technical Information of China (English)

    朱斌; 范海建

    2011-01-01

    目的 探讨Chiari Ⅰ畸形及其合并症的MRI表现.方法 收集2009-01-01至2011-03-01鼓楼医院268例Chiari Ⅰ畸形患者作为A组;随即选取鼓楼医院无颅颈交界畸形的患者37例作为B组,全部行MRI检查,对各项数据进行统计学分析.结果 Chiari Ⅰ畸形患者后颅窝体积及颅底角大小明显小于对照组;单纯Chiari Ⅰ畸形患者与合并脊柱侧弯的Chiari Ⅰ畸形患者脊髓空洞的检出率无显著差异,但明显高于无Chiari Ⅰ畸形脊柱侧弯患者;单纯Chiari Ⅰ畸形患者与合并脊柱侧弯的Chiari Ⅰ畸形患者小脑扁桃体下疝程度的差异无统计学意义.结论 通过比较chiari Ⅰ畸形与对照组之间MRI图像的差异,对其诊断和并发症的发现有重要的作用,有利于全面评估、指导临床治疗方案的选择.%Objective This research was aimed to study MRI performances of the Chiari I malformation and its complications. Methods 268 patients from 01.01.2009 to 01.03.2011 that diagnosed with Chiari I malformation in Nanjing drum tower hospital were selected and classified as group A; 37 cases without craniocervical junction deformity were classified as group B. Both groups were examined by MRI and data were analyzed. Results the volumes of the posterior fossa (check 单复数形式) and the sizes of the posterior fossa horn in Chiari I malformation patients are smaller than the control group. The detection rate of the secondary syringomyelia showed no significant between patients with chiari I malformation only and chiari I malformation patients that combined with scoliosis, but presented a higher tendency than non-Chiari I malformation scoliosis patients. There is no statistic significant between Single(?) Chiari I malformation patients and merger scoliosis patients with Chiari I malformation cerebellar tonsillar next hernia degree. (wtf=what the fuck is this? Hahaha :D). Conclusion By comparing differences of the MRI performances between chiari I

  19. 微创枕大池重建术辅助术中B 超评价ChiariⅠ型畸形合并脊髓空洞症临床疗效%The efficacy of intraoperative ultrasonography-assisted microinvasive cisterna magna reconstruction for Chiari malformation typeⅠwith syringomyelia

    Institute of Scientific and Technical Information of China (English)

    李鹏超; 刘勇; 邱险峻; 靳玉强; 李玥堃; 杨淑琴; 邢志霞

    2012-01-01

    目的 探讨微创枕大池重建术治疗ChiariⅠ型畸形术中辅助B 超判断枕大池重建效果的可行性.方法 选择2008 年1-12 月诊断明确的ChiariⅠ型畸形合并脊髓空洞症患者共93 例,施行微创枕大池重建术.术中保留寰椎后弓,并于切开硬膜前通过B 超实时观察小脑扁桃体下疝形态、大小、"活塞"样往复运动状态,以及枕大池形态和大小;并于切除部分小脑扁桃体后对枕大池重建效果进行判断.分别于术后6 和30 个月时通过Tator法进行临床疗效评价.结果 所有患者均顺利完成手术,术后并发症包括小脑出血(1 例)、小脑梗死(1 例)、脑积水(1 例)、皮下积液(2 例),均经对症治疗痊愈出院.术后6 个月随访93 例患者,临床症状好转36 例、稳定55 例、加重2 例,MRI 显示脊髓空洞缩小或完全消失90 例、无变化3 例;术后30 个月随访80 例患者,原症状稳定者中12 例好转、1 例加重,MRI显示脊髓空洞无进一步变化.结论 微创枕大池重建术治疗ChiariⅠ型畸形损伤小、术后恢复迅速、效果稳定、并发症少、安全性高.术中通过B 超判断枕大池重建效果简单易行,数据可靠.%Objective To report the method and effect of intraoperative ultrasonography-assisted microinvasive cisterna magna reconstruction for Chiari malformation type I with syringomyelia. Methods Ninty-three patients suffered from Chiari malformation type I with syringomyelia were treated by microinvasive cisterna magna reconstruction. The skin incision was 1.50-3.00 cm. The bone removal of foramen magnum was 1.50 cm X 2.00 cm with C1 reserved. Dura and arachnoid were incised and sutured linearly. All of the patients underwent cerebellar tonsillar resection and exploration of median aperture of fourth ventricle. Intraoperative ultrasonography was performed both before and after cerebellar tonsillar resection to judge the effect of cisterna magna reconstruction. According to Tator

  20. Familial Aggregation of Chiari Malformation: Presentation, Pedigree, and Review of the Literature.

    Science.gov (United States)

    Nagy, Laszlo; Mobley, James; Ray, Coby

    2016-01-01

    This article reports the largest familial aggregation of Chiari malformation in a single family to date as reported in the literature. This study is a retrospective case series of a family of whom five individuals have a confirmed case of Chiari malformation and three additional individuals have Chiari signs and symptoms. This contribution further supports the implication of genetics in the transmission of Chiari malformation. The family reported in this study also has a significant incidence of Ehlers-Danlos. Three sisters, including a set of twins, presented with confirmed cases of Chiari malformation and four of the five children of the twin sisters presented with confirmed or suspected Chiari malformation. Of note, the non-twin sister has three children who are unaffected. This report provides further evidence for a shared loci between the Chiari malformation and Ehlers-Danlos.

  1. Morning glory disc anomaly with Chiari type I malformation.

    Science.gov (United States)

    Arlow, Tim; Arepalli, Sruthi; Flanders, Adam E; Shields, Carol L

    2014-04-30

    Morning glory disc anomaly is a rare optic nerve dysplasia associated with various neovascular abnormalities. Due to these associations, children with morning glory disc anomaly have brain imaging and angiography to detect other congenital defects. The authors report the case of an infant with morning glory disc anomaly and coexisting Chiari type I malformation.

  2. The relationship between obesity and symptomatic Chiari I malformation in the pediatric population

    Directory of Open Access Journals (Sweden)

    Sandi Lam

    2015-01-01

    Full Text Available Background: Concomitant with the rise in childhood obesity in the United States is an increase in the diagnosis of Chiari I malformation (CM1. Objective: To discern a correlation between obesity and CM1, defined as >5 mm of cerebellar tonsillar descent on sagittal magnetic resonance imaging. Methods: Charts of CM1 patients aged 2–20 years were retrospectively reviewed. Chiari size, age, body mass index (BMI, and CM1 signs/symptoms were recorded. Patients were stratified by age: 2–9, 10–14, and 15–20 years. Mixed-effect linear models and linear regression analysis were applied to investigate the relationship between BMI-for-age percentiles and CM1 signs/symptoms. Results: One hundred sixty-seven patients were included (mean age 14.5 ± 2.97 years, BMI 22.98 ± 6.5, and Chiari size 12.27 mm ± 5.91. When adjusted for age, 42% were overweight or obese–higher than normative BMI for children in the studied area (29.6%. When stratified by age, patients between 2 and 9 years were most commonly obese and presented the highest mean BMI (25.66, largest Chiari size (13.58, and highest incidence of headache (75% and syringomyelia (66.67%. Patients between 15 and 20 years were most commonly overweight and presented the smallest Chiari size (11.76 mm, but the highest incidence of cerebellar (50% and brainstem (8.55% compression symptoms. A significant positive correlation existed between BMI and headache in the first two age groups: (R2: 0.36, P = 0.03; R2: 0.39, P = 0.01, respectively. Obese patients had higher incidence of headache in the 10–14 group (R2: 0.37, P = 0.02 and the largest Chiari size in the 15–20 group (R2: 0.40, P = 0.03. Conclusions: The pediatric CM1 population is more likely to be overweight or obese. Younger obese patients presented the highest incidence of Chiari-related headache symptoms, and older obese patients, the highest incidence of findings other than headache. Thus, body weight and age should be considered when

  3. Suboccipital craniectomy with or without duraplasty: what is the best choice in patients with Chiari type 1 malformation? Craniectomia suboccipital com ou sem duroplastia: qual a melhor escolha em pacientes com malformação de Chiari tipo 1?

    Directory of Open Access Journals (Sweden)

    Flávio Ramalho Romero

    2010-08-01

    Full Text Available The best surgical treatment for Chiari malformation is unclear, especially in patients with syringomyelia. We reviewed the records of 16 patients who underwent suboccipital craniectomy at our institution between 2005 and 2008. Of the six patients who did not undergo duraplasty, four showed improvement postoperatively. Two patients without syringomyelia showed improvement postoperatively. Of the four patients with syringomyelia, three showed improvement, including two with a decrease in the cavity size. One patient showed improvement in symptoms but the syringomyelia was unchanged. The cavity size increased in the one patient who did not show improvement. Among the 10 patients who underwent duraplasty, improvements were noted in four of the five patients without syringomyelia and in all of the five with syringomyelia. There is a suggestion that patients with syringomyelia may have a higher likelihood of improvement after undergoing duraplasty.A melhor opção de tratamento cirúrgico na malformação de Chiari é desconhecido, especialmente em paciente com siringomielia. Nós revisamos casos de 16 pacientes submetidos à craniectomia suboccipital em nossa instituição de 2005 à 2008. Dos 6 pacientes que não foram submetidos à duroplastia, quatro tiveram melhora pós operatória. Dois pacientes sem siringomielia tiveram melhora pós operatória. Dos quatro pacientes com siringomielia, três tiveram melhora, incluindo dois com diminuição do tamanho da cavidade. Um paciente teve melhora da sintomatologia mas a siringomielia não se modificou. Dez pacientes foram submetidos à duroplastia. Melhora foi observada em 4 de 5 pacientes sem siringomielia e 5 de 5 casos com siringomielia. Há uma sugestão de que pacientes com siringomielia possam ter uma maior taxa de melhora após serem submetidos à duroplastia.

  4. Clinical Study of Foramen Magnum Decompression with Removal of the Outer Layer of the Dura for Chiari Malformation Associated with Syringomyelia%枕骨大孔减压伴硬膜外层切开术治疗Chiari畸形合并脊髓空洞症的临床研究

    Institute of Scientific and Technical Information of China (English)

    于明军; 洪洋; 刘云会

    2012-01-01

    Objective To investigate the outcomes of foramen magnum decompression with removal of the outer layer of the dura on Chiari malformation with syringomyelia ( ACM-SM) compared with foramen magnum decompression. Methods Thirty patients were performed ( Group A) foramen magnum decompression and thirty-eight patients were performed foramen magnum decompression with removal of the outer layer of the dura( Group B) . In the end, the outcomes were documented and analyzed. Results Short-term effects; at the time of discharge, in group A, 21(70% ) patients achieved an improvement in their clinical symptoms. In Group B, 35(92.1% ) patients achieved an improvement in their clinical symptoms. The outcomes of group B was better than that of group A (P =0.02). Long-term effects:28 patients were followed up in group A, among which 19(67.9% ) patients had an improvement in their clinical symptoms. 33 patients were followed in group B, among which 31 (93. 9% ) patients had an improvement in their clinical symptoms. The outcomes of group B was better than that of group A (P <0.05). MRI showed that in group A, syringomyelia decreased in 20 cases. In group B, syringomyelia decreased in 31 cases. By chi-square test(x2 test) , the syringomyelia got more narrow in group B than that of group A (P < 0.05). Conclusions Operation is an effective treatment for ACM-SM. Different methods should be chosen according to the patients'unique feature. Foramen magnum decompression with removal of the outer layer of the dura is better than foramen magnum decompression in either long-term effects or short-term effects.%目的 探讨枕骨大孔减压伴硬膜外层切开术治疗Chiari畸形合并脊髓空洞症(ACM-SM)的手术疗效.方法 按患者手术方式不同分为两组:A组30例,枕骨大孔减压组;B组38例,枕骨大孔减压伴硬膜外层切开组,回顾性分析两组患者预后情况.结果 近期疗效:出院时,A组有效率70% (21/30),B组有效率92.1%(35/38),二者比

  5. Interobserver reliability and diagnostic performance of Chiari II malformation measures in MR imaging-part 2

    NARCIS (Netherlands)

    Geerdink, Niels; van der Vliet, Ton; Rotteveel, Jan J.; Feuth, Ton; Roeleveld, Nel; Mullaart, Reinier A.

    2012-01-01

    Brain MR imaging is essential in the assessment of Chiari II malformation in clinical and research settings concerning spina bifida. However, the interpretation of MR images of the malformation is not always straightforward. Morphometric analyses of the extent of Chiari II malformation may improve t

  6. Interobserver reliability and diagnostic performance of Chiari II malformation measures in MR imaging--part 2.

    NARCIS (Netherlands)

    Geerdink, N.; Vliet, T. van der; Rotteveel, J.J.; Feuth, T.; Roeleveld, N.; Mullaart, R.A.

    2012-01-01

    PURPOSE: Brain MR imaging is essential in the assessment of Chiari II malformation in clinical and research settings concerning spina bifida. However, the interpretation of MR images of the malformation is not always straightforward. Morphometric analyses of the extent of Chiari II malformation may

  7. [Diagnosis of syringomyelia and its classification on the basis of symptoms, radiological appearance, and causative disorders].

    Science.gov (United States)

    Terae, Satoshi; Hida, Kazutoshi; Sasaki, Hidenao

    2011-09-01

    Although it is easier to accurately diagnose syringomyelia with the advent of magnetic resonance (MR) imaging, syringomyelia still poses challenges to clinicians because of its complex symptomatology, uncertain pathogenesis and multiple treatment options. Here, we propose criteria for classification of syringomyelia not related to those associated with spinal intramedullary tumors. The classification aims to distinguish between the presyrinx state and syringomyelia, between asymptomatic and symptomatic syringomyelia and to clarify the associated disorders such as Chiari malformations, spinal arachnoiditis and spinal cord trauma. Diagnostic criteria for Chiari I and II malformations with MR imaging were also defined. Several hypotheses proposed to explain the pathogenesis of syringomyelia associated with Chiari I malformation were reviewed. A questionnaire survey on syringomyelia based on the proposed criteria conducted between November 2009 and April 2010 in Japan revealed that 160 (22.6%) of the 708 patients were asymptomatic. Chiari I malformation was the most frequent causative disorder (48.3%), followed by spinal arachnoiditis (15.8%). The proposed criteria for classification of syringomyelia will facilitate a nationwide survey of syringomyelia in Japan. Such a survey will inform us of its prevalence and prognosis more precisely, and enable us to build a reliable database that may help determine the optimal treatment for the disease in the future.

  8. Chiari I malformation as part of the Floating-Harbor syndrome?

    Science.gov (United States)

    Kurzbuch, Arthur R; Magdum, Shailendra

    2016-12-01

    We report the first case of a patient diagnosed with Floating-Harbor syndrome (FHS) and Chiari I malformation. The 3-year-old girl was of proportional short stature, had delay of language development, conductive hearing loss and a high threshold of pain. Diagnosis of Chiari I malformation may be difficult in FHS patients who present with communication problems. Clinicians following patients with FHS should be aware of a possible relation between FHS and Chiari I malformation.

  9. Diagnostic value of low field MRI in Chiari malformation%Chiari畸形的低场MRI诊断价值

    Institute of Scientific and Technical Information of China (English)

    高保军; 于雪萍; 王秀云; 杨法祖

    2015-01-01

    目的:探讨Chiari畸形的低场MRI表现,评价低场MRI对Chiari畸形的诊断价值。方法回顾性分析封丘县人民医院诊断为Chiari畸形的18例患者的MRI 影像学资料及临床资料。结果小脑扁桃体下疝至枕大孔平面下5~19 mm,其中伴脊髓空洞症11例,伴脑积水5例,二者并存1例,合并颅底凹陷症1例。结论低场MRI可清晰显示后颅凹、脊髓的解剖结构,并可显示颅底及其他发育异常,是Chiari畸形的最佳影像学诊断方法,对其临床治疗及预后判断具有重要参考价值。%Objective To investigate the performance of low field MRI in Chiari malformation,and evaluate the diagnostic val-ue of low field MRI in Chiari malformation. Methods The clinical data of 18 patients who were diagnosed with Chiari malformation were retrospectively analyzed. Results The cerebellar tonsillar hernia to magnum 5-19 mm,of which 11 cases associated with sy-ringomyelia,5 cases with hydrocephalus,1 case with both of the above,1 case was complicated with basilar invagination. Conclu-sion The low field MRI can clearly show the anatomy of posterior fossa,spinal cord,and can display the skull base and other de-velopmental abnormalities,is the best method for diagnosis of Chiari malformation,has important reference value for the clinical treatment and prognosis.

  10. Headache and Chiari I Malformation in Children and Adolescents.

    Science.gov (United States)

    Victorio, M Cristina; Khoury, Chaouki K

    2016-02-01

    Headache is a common problem in children and adolescents. Its recurrent and disabling nature may lead to use of neuroimaging to exclude secondary causes of headache such as Chiari I malformation (CM I). CM I has a variety of presentation with headache being the most common symptom. CM I can be asymptomatic and is also often found incidentally in neuroimaging done for conditions other than headache. This article reviews the spectrum of headache in patients with CM I.

  11. Association of HTLV-I with Arnold Chiari syndrome and syringomyelia

    Directory of Open Access Journals (Sweden)

    Graça Maria de Castro Viana

    2008-12-01

    Full Text Available HTLV-I is associated with a broad spectrum of manifestations, including tropical spastic paraparesis and adult T-cell leukemia/lymphoma. Arnold Chiari syndrome is a condition characterized by herniation of the cerebellar tonsils through the foramen magnum. This condition should be suspected in all patients with headache and impaired motor coordination. Syringomyelia is a developmental anomaly that leads to the formation of an intramedullary cavity. Its clinical presentation is classically characterized by syringomyelic dissociation of sensation, with suspended distribution in the proximal portion of the trunk and upper limbs and preservation in other regions. We report here a case of association of the three diseases, which is rare in clinical practice, illustrating the difficulty in the diagnosis and therapeutic management of these conditions.

  12. Syringomyelia

    Science.gov (United States)

    Syringomyelia is a rare disorder that causes a cyst to form in your spinal cord. This cyst, ... the syrinx. They often begin in early adulthood. Syringomyelia usually results from a skull abnormality called a ...

  13. Arnold-Chiari Type II Malformation: A Case Report and Review of Prenatal Sonographic Findings

    Directory of Open Access Journals (Sweden)

    Maryam Nik Nejadi

    2008-01-01

    Full Text Available The Arnold-Chiari malformation is a congenital abnormality of CNS, characterized by downwarddisplacement the parts of the cerebellum, fourth ventricle, pons and medulla oblongata into thespinal canal. This malformation is one of causative factor of death in neonates and infants. Athorough understanding of the direct and indirect sonographic findings is necessary for diagnosis ofChiari II malformation in the developing fetus.In this case report, we present a Chiari malformation II detected at 23 weeks of gestation by routinelysonographic screening. The Role of prenatal sonography in recognition of the malformation andprognostic value of these features are discussed.

  14. Chiari I malformation with underlying pseudotumor cerebri: Poor symptom relief following posterior decompression surgery

    Directory of Open Access Journals (Sweden)

    Ahmed Alnemari

    2017-01-01

    Conclusion: This study suggests that the presence of Chiari I malformation in a patient conceals the symptoms of PTC which may become apparent following posterior decompression surgery. Other possibilities could be that the patients are misdiagnosed for Chiari I malformation when they are in fact suffering from PTC, or that PTC is a complication of surgery.

  15. Surgical treatment of Chiari malformation complicated with basilar impression

    Directory of Open Access Journals (Sweden)

    Yuan MA

    2011-02-01

    Full Text Available Objective To evaluate the therapeutic effect of small craniotomic posterior fossa decompression combined with occipital-cervical bone graft fusion and internal fixation on Chiari malformation complicated with basilar impression.Methods The clinical data of 16 cases(7 males and 9 females,aged 17 to 65 years,mean 36.4 of Chiari malformation complicated with basilar impression from 2006 to 2010 were retrospectively analyzed.The diagnoses for all the patients were confirmed by radiology.Small craniotomic posterior fossa decompression was performed in all patients,cerebellar tonsils were resected,and then one-stage occipital-cervical bone graft fusion using autogenous iliac bone and internal wiring fixation were performed.Neck support was used for 3 months after surgery.Results Symptoms were significantly improved in all cases after surgical operation.No patient died or infected.Cerebrospinal fluid leakage was found at draining site in one case.Transient pain of scapular and chest was found in one case and disappeared spontaneously.A 6-months follow-up showed that 6 patients were cured,9 improved and 1 unchanged according to Symon and Lavender standard.Postoperative MRI showed the reconstructed cisterna magna was clear in all patients,no cerebellar ptosis was found,and the occipital-cervical graft bone was fused.Conclusion In patients with Chiari malformation complicated with basilar impression,small craniotomic posterior fossa decompression combined with one-stage occipital-cervical bone graft fusion and internal wiring fixation has a clear and definite effect,it can increase the volume of posterior fossa and alleviate the ventral brain stem compression simultaneously,and reconstruct the stability of cranio-cervical junction.

  16. Presentation of occult Chiari I malformation following spinal anesthesia

    Directory of Open Access Journals (Sweden)

    Saravanan P Ankichetty

    2012-01-01

    Full Text Available Chiari I malformation (CM-I manifests with tonsillar herniation below foramen magnum. These patients are at high risk of respiratory depression and bulbar dysfunction in the perioperative period with underlying obstructive sleep apnea. However, the safe use of both general and regional anaesthesia has been documented in a known CM-I parturients. We describe the successful management of a patient who had hypercapnic respiratory failure in the post-anaesthetic care unit following an uneventful subarachnoid block for left knee replacement surgery. This patient was retrospectively diagnosed with occult CM-I and moderate to severe obstructive sleep apnea in the postoperative period.

  17. Isolated unilateral trismus as a presentation of Chiari malformation: case report.

    Science.gov (United States)

    Feinberg, Michelle; Babington, Parker; Sood, Shawn; Keating, Robert

    2016-05-01

    The authors present a case of Chiari malformation manifesting as isolated trismus, describe the typical symptoms associated with Chiari malformation, and discuss the potential anatomical causes for this unique presentation. A 3-year-old boy presented with inability to open his jaw for 6 weeks with associated significant weight loss. The results of medical and radiological evaluation were negative except for Type I Chiari malformation with cerebellar tonsils 12 mm below the level of the foramen magnum. The patient underwent Chiari decompression surgery. Postoperatively, his ability to open his mouth was significantly improved, allowing resumption of a regular diet. Postoperative MRI revealed almost complete resolution of the syringobulbia. To the best of the authors' knowledge, this is the first reported case of isolated trismus from Chiari malformation with syringobulbia.

  18. A extremely rare case of cervical intramedullary granuloma due to Brucella accompanied by Chiari Type-1 malformation.

    Science.gov (United States)

    Tufan, Kadir; Aydemir, Fatih; Sarica, Feyzi Birol; Kursun, Ebru; Kardes, Özgür; Cekinmez, Melih; Caner, Hakan

    2014-01-01

    Chiari Type-1 malformation is displacement of the cerebellar tonsils through the foramen magnum into the cervical spine and usually does not exceed the level of C2. It is 50-70% associated with syringomyelia. Nervous system involvement due to brucellosis is called neurobrucellosis, and neurological involvement rate has been reported an average of 3-5%, ranging between 3% and 25% at different series. Intramedullary abscess or granuloma due to Brucella is extremely rare. Hence far, six cases have been reported in the literature and only two of these cases were reported as intramedullary granuloma. This case is presented in order to remind the importance of the cervical cord granuloma which was presented once before in the literature and to emphasize the importance of evaluation of patient history, clinical and radiological findings together in the evaluation of a patient.

  19. Non-operative outcomes in Chiari I malformation patients.

    Science.gov (United States)

    Killeen, Amy; Roguski, Marie; Chavez, Alexis; Heilman, Carl; Hwang, Steven

    2015-01-01

    While postoperative outcomes of Chiari I malformation patients have been well-reported, there is a paucity of literature concerning non-operative management in these patients. We retrospectively identified patients with Chiari I malformation who were not recommended for surgery based on lack of clinical objective findings or inconsistent cough headaches and conducted patient follow-up with a prospective telephone survey. Of the 68 patients (mean age at diagnosis 30.1 ± 17.4 years), 72% were female and 31% were pediatric patients (age at diagnosis ⩽ 18 years). Average follow up was 4.9 ± 2.9 years. Typical presenting symptoms included cough headache, non-specific headache, nausea, ataxia, dysphagia and paresthesias. Overall, 40% of patients who had cough headaches and 61.5% of patients with non-specific headaches reported improvement. The presence of subjective sensory symptoms was significantly associated with less likelihood of cough headache improvement while the presence of a cough headache was also associated with a lower likelihood of improvement in all non-cough symptoms. The pediatric subgroup had a greater rate of improvement with all cases of nausea/emesis and paresthesias improved or resolved at follow-up. Overall 67% of pediatric patients had improved cough headache and 71% had improvement of migraines/diffuse headaches. We found that many symptoms of Chiari I patients from our conservatively managed cohort either improved or remained unchanged over time. However, the presence of cough headaches was a significant negative predictor of concomitant symptom improvement. This further validates the view that patients with cough headaches should be considered for surgical intervention and provides useful information to counsel patients.

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

    Science.gov (United States)

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

    2017-07-25

    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.

  1. Syringomyelia

    DEFF Research Database (Denmark)

    Blegvad, C; Grotenhuis, J A; Juhler, M

    2014-01-01

    extends to the ICD-10 classification of diseases in regards to syringomyelia (G95.0) and hydromyelia (Q06.4). We propose a new unifying concept for classification that also incorporates diagnostics and treatment. METHODS: The PubMed online database was used to gain a general overview of the existing...... pathogenetic theories in relation to syringomyelia. Illustrative cases at our department were included and similar cases of the literature were found using the PubMed database. All material was reviewed with main focus on the classification and terminology used. RESULTS: Despite syringomyelia (G95...

  2. Scoliosis Associated With Syringomyelia

    Directory of Open Access Journals (Sweden)

    Gh Fathi

    2007-01-01

    Full Text Available Introduction: The differential diagnosis of idiopathic and syringomyelia associated scoliosis is important because corrective surgery for scoliosis associated with syringomyelia prior to management of syringomyelia can be dangerous. There are important imaging indicators for diagnosis of syringomyelia associated with scoliosis. A few of these indicators have been assessed in our study. Methods: A retrospective descriptive study including 38 patients with both scoliosis and syringomyelia was performed at the Shafa Yahyaeian center. Standard scoliosis series radiographs and MRI of all patients were studied. The type of scoliosis, location and magnitude of deformity, kyphosis or lordosis in the sagittal plane, location and size of syrinx were assessed. Results: Thoracic kyphosis was present in 94.7% of patients. 37% of patients had scoliosis with convexity to left. Arnold –chiari malformation was present in 36% and cord tethering in 21% of patients. The locations of syrinx were as follows: 47.4%cervical, 44.6 thoracic, 2.7% lumbar and 5.3% were holocord. Conclusion: Kyphosis, abscence of lordosis in sagittal plane, progressive scoliosis and scoliosis with convexity to left are atypical findings and could be indicators of the presence of syringomyelia. If these indicators are present, a diagnosis of idiopathic scoliosis should be made with caution.

  3. Natural and surgical history of Chiari malformation Type I in the pediatric population.

    Science.gov (United States)

    Pomeraniec, I Jonathan; Ksendzovsky, Alexander; Awad, Ahmed J; Fezeu, Francis; Jane, John A

    2016-03-01

    OBJECT The natural and surgical history of Chiari malformation Type I (CM-I) in pediatric patients is currently not well described. In this study the authors discuss the clinical and radiological presentation and outcomes in a large cohort of pediatric CM-I patients treated with either conservative or surgical management. METHODS The authors retrospectively reviewed 95 cases involving pediatric patients with CM-I who presented between 2004 and 2013. The patients ranged in age from 9 months to 18 years (mean 8 years) at presentation. The cohort was evenly split between the sexes. Twenty-five patients underwent posterior fossa decompression (PFD) with either dural splitting or duraplasty. Seventy patients were managed without surgery. Patients were followed radiologically (mean 44.8 months, range 1.2-196.6 months) and clinically (mean 66.3 months, range 1.2-106.5 months). RESULTS Seventy patients were treated conservatively and followed with serial outpatient neurological and radiological examinations, whereas 25 patients were treated with PFD. Of these 25 surgical patients, 11 were treated with duraplasty (complete dural opening) and 14 were treated with a dura-splitting technique (incomplete dural opening). Surgical intervention was associated with better clinical resolution of symptoms and radiological resolution of tonsillar ectopia and syringomyelia (p = 0.0392). Over the course of follow-up, 20 (41.7%) of 48 nonsurgical patients who were symptomatic at presentation experienced improvement in symptoms and 18 (75%) of 24 symptomatic surgical patients showed clinical improvement (p = 0.0117). There was no statistically significant difference in resolution of symptoms between duraplasty and dura-splitting techniques (p = 0.3572) or between patients who underwent tonsillectomy and tonsillopexy (p = 0.1667). Neither of the 2 patients in the conservative group with syrinx at presentation showed radiological evidence of resolution of the syrinx, whereas 14 (87.5%) of

  4. Syringomyelia

    Science.gov (United States)

    ... accurate recommendations to future patients with syringomyelia regarding optimal surgical or non-surgical treatments. Also, NIH-funded ... Through Research Know Your Brain Preventing Stroke Understanding Sleep The Life and Death of a Neuron Genes ...

  5. Orbital Emphysema Causing SyncopeChiari Malformation with Thick Occipital Bone

    Directory of Open Access Journals (Sweden)

    Yasuhara,Takao

    2011-02-01

    Full Text Available A case of a Chiari malformation with an extraordinarily thick occipital bone is described. The thick occipital bone might make the posterior fossa narrow with consequent herniation of the cerebellar tonsils to the foramen magnum and formation of a syrinx. At dural plasty, well-developed marginal and occipital sinuses should be deliberately handled with the preservation of normal venous drainage. This case gives us the essence of the occurrence mechanisms of Chiari malformation and foramen magnum decompression.

  6. Cutis Marmorata Telangiectatica and Chiari Type I

    Directory of Open Access Journals (Sweden)

    J Gordon Millichap

    2003-12-01

    Full Text Available A 10-year-old girl born with telangiectasias of the lip, trunk, extremities, palms, and soles was found to have an asymptomatic Chiari I malformation without syringomyelia on MRI, and is reported from Children’s Hospital, Alabama.

  7. Resolution of syringomyelia in ten cases of "up-and-down Chiari malformation" after posterior fossa decompression Resolução de siringomielia em dez casos de malformação de Chiari observada apenas com o paciente em posição sentada durante a descompressão da fossa posterior

    Directory of Open Access Journals (Sweden)

    José Alberto Gonçalves da Silva

    2010-10-01

    Full Text Available The authors describe ten cases of syringomyelia without hindbrain herniation depicted by preoperative magnetic resonance imaging (MRI in supine position. However, the herniation was observed in all cases during the operation with the patient in sitting position. The postoperative MRI revealed an intense reduction of the syrinx in all patients, as well as it was also observed a clinical amelioration in all cases. The surgical treatment was based on a large craniectomy with the patient in sitting position, tonsillectomy, large opening of the fourth ventricle and duraplasty with creation of a large cisterna magna.Os autores descrevem 10 casos de siringomielia sem herniação do rombencéfalo, observada na ressonância magnética realizada em decúbito dorsal. Por outro lado, a herniação foi observada em todos os pacientes durante a operação com o paciente em posição sentada. A ressonância magnética pós-operatória evidenciou redução da cavidade siringomiélica nos dez pacientes, bem como foi observada melhora clínica em todos os casos. O tratamento cirúrgico consistiu de craniectomia ampla da fossa posterior, tonsilectomia, abertura ampla do quarto ventrículo e duroplastia com a criação de ampla cisterna magna.

  8. Are evoked potentials clinically useful in the study of patients with Chiari malformation Type 1?

    Science.gov (United States)

    Moncho, Dulce; Poca, Maria A; Minoves, Teresa; Ferré, Alejandro; Cañas, Victoria; Sahuquillo, Juan

    2017-02-01

    OBJECTIVE In this study, the authors describe the brainstem auditory evoked potential (BAEP) and somatosensory evoked potential (SSEP) alterations found in a large cohort of patients with Chiari malformation Type 1 (CM-1), the relationship between the BAEPs/SSEPs and the clinical findings, the abnormalities in patients with associated syringomyelia, and the clinical and neuroradiological risk factors that are associated with abnormal evoked potentials (EPs). METHODS A prospectively collected database containing 545 patients with CM-1 was queried to search for patients satisfying the following criteria: 1) an age of at least 14 years, 2) neuroradiological criteria of CM-1, 3) no prior Chiari-related surgeries, and 4) preoperative EP studies conducted at the authors' institution. The 200 patients included in this cohort were classified into CM-0, CM-1, and CM-1.5 subtypes. Linear, planimetric, and angular measurements of the posterior fossa were conducted, as well as syringomyelia measurements. Two separate multiple logistic regression models were used, one to predict the covariates associated with abnormal BAEPs, and a second model to explore the variables associated with an abnormal SSEP. In these models, the BAEPs and SSEPs were dichotomized as being normal or abnormal. RESULTS Headaches were the main symptom in 70.5% of the patients, and Valsalva-induced headaches were most frequent in patients with CM-1 and CM-1.5 compared with patients with CM-0 (p = 0.031). BAEPs were abnormal in 38.5% of patients, and abnormal SSEPs were found in 43.5% of the entire cohort. Syringomyelia was most frequent in patients with CM-0 (64.3%) and CM-1 (51.1%) compared with those with CM-1.5 (34.7%; p = 0.03). Age (OR 1.03, 95% CI 1.00-1.06), the degree of tonsillar herniation (OR 1.08, 95% CI 1.01-1.16), and lower cranial nerve dysfunction (OR 3.99, 95% CI 1.29-14.01) had a statistically significant correlation with abnormal BAEPs. Only age (OR 1.07, 95% CI 1.04-1.10) and the degree

  9. Study of Chiari malformation associated with scoliosis as the first symptom%脊柱侧凸为首发症状的Chiari畸形临床研究

    Institute of Scientific and Technical Information of China (English)

    王嵘; 邱勇; 蒋健; 王斌; 朱泽章; 赵寅涛; 刘志坚

    2008-01-01

    目的 研究以脊柱侧凸为首发症状的Chiari畸形的临床特征和治疗策略.方法 收集南京鼓楼陕院住院治疗的Chiari畸形患者60例,35例以脊柱侧凸为首发症状;25例末合并脊柱侧凸作为对照组,病例对照研究.结果 35例Chiari畸形合并脊柱侧凸患者,年龄3~33岁(平均13.75岁),97%合并脊髓空洞,不具有典型的Chiari畸形的临床症状和体征,以腹壁反射异常多见.两组之间手术方式和手术并发症无统计学意义.结论 Chiari畸形以脊柱侧凸为首发症状,多合并脊髓空洞,发病年龄轻,其临床症状和神经损害体征不明显,治疗策略上主张早期进行枕颈部枕骨大孔区减压和硬脑膜重建成形手术.%Objective To study the clinical manifestation and intervention strategy for Chiari malformation associated with seoliosis as the first symptom.Methods 60 patients with Chiari malformation were included in this control study.Among which 35 patients were Confirmed to be Chiari malformation associmed with scoliosis as the first symptom.These 35 patients were included in test group and the other 25 patients were included in control group.Results 35 patients in test group were younger(3~33 yrs old,average 13.75 yrs).The abnormal neurological manifestation of typical Chiari malfi,rmation was slight in test group.Most of these patients in test group had syringomyelia(97%)and ahnormal upper abdominal reflex.There was no diflhrence between the two groups in surgical intervention and postoperative complication.Conclusions In order to decrease the risk of neurological complication,foramen magnum decompression (include C.posterior arch)is necessary before instrumentation and correction of scoliosis in Chiari malformation patients with syringomyelia and scoliosis as the first symptom.

  10. Surgical Management of Patients with Chiari I Malformation

    Directory of Open Access Journals (Sweden)

    John Siasios

    2012-01-01

    Full Text Available Chiari malformations (CMs constitute a variety of four mainly syndromes (I, II, III, and IV, which describe the protrusion of brain tissue into the spinal canal through the foramen magnum. These malformations frequently occur in combination with other pathological entities such as myelomeningocele, hydrocephalus, and/or hydrosyringomyelia. The recent improvement of imaging techniques has increased not only the rate of CM diagnosis but also the necessity for its early treatment. Several different surgical techniques have been employed in the treatment of patients with symptomatic CM-I. In our current study, a systematic and critical review of the pertinent literature was made for identifying the most commonly employed surgical procedures in the management of these patients. Emphasis was given in outlining the advantages and disadvantages of each surgical approach. Moreover, an attempt was made for defining those parameters that may be prognostic factors for their surgical outcome. There is a consensus that surgical treatment is reserved only for symptomatic patients with CM-I. It has also been postulated that early surgically intervention is usually associated with better outcome. Despite the large number of previously published clinical series, further clinical research with large-scale studies is necessary for defining surgical treatment guidelines in these patients.

  11. Smooth ocular pursuit in Chiari type II malformation.

    Science.gov (United States)

    Salman, Michael S; Sharpe, James A; Lillakas, Linda; Steinbach, Martin J; Dennis, Maureen

    2007-04-01

    Chiari type II malformation (CII) is a congenital anomaly of the cerebellum and brainstem, both important structures for processing smooth ocular pursuit. CII is associated with myelomeningocele and hydrocephalus. We investigated the effects of CII on smooth pursuit (SP) eye movements, and determined the effects of spinal lesion level, number of shunt revisions, nystagmus, and brain dysmorphology on SP. SP was recorded using an infrared eye tracker in 21 participants with CII (11 males, 10 females; age range 8-19y, mean 14y 3mo [SD 3y 2mo]). Thirty-eight healthy children (21 males, 17 females) constituted the comparison group. Participants followed a visual target moving sinusoidally at +/- 10 degrees amplitude, horizontally and vertically at 0.25 or 0.5Hz. SP gains, the ratio of eye to target velocities, were abnormal in the CII group with nystagmus (n= 8). The number of shunt revisions (range 0-10), brain dysmorphology, or spinal lesion level (n= 15 for lower and n= 6 for upper spinal lesion level) did not correlate with SP gains. SP is impaired in children with CII and nystagmus. Abnormal pursuit might be related to the CII dysgenesis or to effects of hydrocephalus. The lack of effect of shunt revisions and abnormal tracking in participants with nystagmus provide evidence that it is related primarily to the cerebellar and brainstem malformation.

  12. Fetal diffusion tensor quantification of brainstem pathology in Chiari II malformation.

    Science.gov (United States)

    Woitek, Ramona; Prayer, Daniela; Weber, Michael; Amann, Gabriele; Seidl, Rainer; Bettelheim, Dieter; Schöpf, Veronika; Brugger, Peter C; Furtner, Julia; Asenbaum, Ulrika; Kasprian, Gregor

    2016-05-01

    This prenatal MRI study evaluated the potential of diffusion tensor imaging (DTI) metrics to identify changes in the midbrain of fetuses with Chiari II malformations compared to fetuses with mild ventriculomegaly, hydrocephalus and normal CNS development. Fractional anisotropy (FA) and apparent diffusion coefficient (ADC) were calculated from a region of interest (ROI) in the midbrain of 46 fetuses with normal CNS, 15 with Chiari II malformations, eight with hydrocephalus and 12 with mild ventriculomegaly. Fetuses with different diagnoses were compared group-wise after age-matching. Axial T2W-FSE sequences and single-shot echo planar DTI sequences (16 non-collinear diffusion gradient-encoding directions, b-values of 0 and 700 s/mm(2), 1.5 Tesla) were evaluated retrospectively. In Chiari II malformations, FA was significantly higher than in age-matched fetuses with a normal CNS (p = .003), while ADC was not significantly different. No differences in DTI metrics between normal controls and fetuses with hydrocephalus or vetriculomegaly were detected. DTI can detect and quantify parenchymal alterations of the fetal midbrain in Chiari II malformations. Therefore, in cases of enlarged fetal ventricles, FA of the fetal midbrain may contribute to the differentiation between Chiari II malformation and other entities. • FA in the fetal midbrain is elevated in Chiari II malformations. • FA is not elevated in hydrocephalus and mild ventriculomegaly without Chiari II. • Measuring FA may help distinguish different causes for enlarged ventricles prenatally. • Elevated FA may aid in the diagnosis of open neural tube defects. • Elevated FA might contribute to stratification for prenatal surgery in Chiari II.

  13. Magnetic resonance imaging of syringomyelia

    Energy Technology Data Exchange (ETDEWEB)

    Itabashi, Takashi; Shimizu, Kou; Yuyama, Takuo and others

    1988-02-01

    Forty-five patients with syringomyelia were studied with MR imaging. They were classified into four groups. Twenty-one patients were associated with Chiari malformation, and eight with intramedullary tumors. Nine patients had idiopathic syringomyelia, and seven had posttraumatic syringomyelia. Thirty-three of them were also studied with delayed CT myelography (DCTM). The results of DCTM had a good correlation with those of MR imaging except for five cases who did not show positive contrast accumulation in the cord. Fluid pulsation in the syrinx was shown with long-TR, long-TE pulse sequences as an area of signal void within the cord. These signs of signal void were visualized on sixteen out of twenty-one Chiari malformation associated cases, on four of nine idiopathic cases, on two of seven posttraumatic cases, and on none of eight tumor-associated cases. Cardiac gated multi-phase imaging was applied and reviewed on the cinemode display, and effects of CSF pulsatile motion were discussed. This technique clearly showed the fine changes of signal intensities from systolic phase to diastolic phase, and was expected to play a significant role on the investigation of CSF motion in syringomyelia.

  14. Essential features of Chiari II malformation in MR imaging : an interobserver reliability study-part 1

    NARCIS (Netherlands)

    Geerdink, Niels; van der Vliet, Ton; Rotteveel, Jan J.; Feuth, Ton; Roeleveld, Nel; Mullaart, Reinier A.

    2012-01-01

    Brain MR imaging is essential in the assessment of Chiari II malformation in clinical and research settings concerning spina bifida. However, the interpretation of morphological features of the malformation on MR images may not always be straightforward. In an attempt to select those features that u

  15. Essential features of Chiari II malformation in MR imaging: an interobserver reliability study--part 1.

    NARCIS (Netherlands)

    Geerdink, N.; Vliet, T. van der; Rotteveel, J.J.; Feuth, T.; Roeleveld, N.; Mullaart, R.A.

    2012-01-01

    PURPOSE: Brain MR imaging is essential in the assessment of Chiari II malformation in clinical and research settings concerning spina bifida. However, the interpretation of morphological features of the malformation on MR images may not always be straightforward. In an attempt to select those featur

  16. Arnold Chiari Malformation With Sponastrime (Spondylar and Nasal Changes, With Striations of the Metaphyses) Dysplasia

    Science.gov (United States)

    Jeong, Je Hoon; Lee, A Leum; Cho, Sung Yoon; Jin, Dong Kyu; Im, Soo-Bin

    2016-01-01

    Abstract SPOndylar and NAsal changes, with STRIations of the Metaphyses (SPONASTRIME) dysplasia (SD) is a dwarfing autosomal recessive syndrome, characterized by a variety of clinical and radiographic features, which form the basis for diagnosis. We describe the presentation of an Arnold Chiari malformation in a patient with a clinical diagnosis of SD. The malformation was successfully treated by decompression of the foramen magnum and elevation of the cerebellum, with complete resolution of pain. We report a rare case of Arnold Chiari malformation in a patient presenting with clinical and radiographic features strongly suggestive of SD and be successfully treated. PMID:27149441

  17. Clinical characteristics of neurogenic dysphagia in adult patients with Chiari malformation type I.

    Science.gov (United States)

    Yu, T; Li, J; Wang, K; Ge, Y; Jiang, A C; Duan, L P; Wang, Z Y

    2017-04-18

    To investigate changes of swallowing function and associated symptoms in Chiari malformation typeI (CMI) patients with and without dysphagia by the analysis of their clinical and high-resolution manometry (HRM) parameters. A total of 42 patients diagnosed with symptomatic CMI without atlantoaxial dislocations which were confirmed by clinical manifestations and magnetic resonance imaging (MRI) findings between January 2010 and July 2015 at Peking University Third Hospital were included in this study. Twenty patients had a history of various dysphagia symptoms, or reported symptoms of choking, coughing after eating or drinking, while the other 22 patients denied symptoms of dysphagia. The data collected from the medical records of these patients included the patient's age, sex, date of diagnosis, duration of illness, symptoms, results of MRI and HRM, and date of surgery. (1) Dysphagia group had 14 female patients, and no-dysphagia group had 8 female patients. Dysphagia usually occurred in female patients, and in addition to dysphagia, we recorded other symptoms and signs in the CMI patients, including numbness, hypoesthesia, limb weakness, neck pain, muscle atrophy, ataxia, hoarseness, symptoms caused by posterior cranial nerve damage, pharyngeal reflex, uvula deviation, and pyramidal signs. A higher percentage of the CMI patients with dysphagia (15/20) had symptoms of posterior cranial nerve damage compared with the control group (5/22; P=0.01). (2)HRM showed a significant difference in upper esophageal sphincter (UES) relax ratio measurement (75.3% vs. 63.1%, P=0.023) and UES proximal margin (17.2 cm vs. 15.7 cm, P=0.005) between the two groups. (3) The percentage of syringomyelia affecting the bulbar or upper cervical region on MRI was significantly higher in the dysphagia group (17/20 vs. 7/22, P=0.001). CMI was usually accompanied by symptoms caused by posterior cranial nerve damage, ataxia, and positive pyramidal signs. Location of the syringomyelia affecting

  18. Neurofibromatosis type 1 and Chiari type 1 malformation: A case report and literature review of a rare association

    Science.gov (United States)

    Pozetti, Marianne; Belsuzarri, Telmo Augusto Barba; Belsuzarri, Natalia C. B.; Seixas, Naira B.; Araujo, João F. M.

    2016-01-01

    Background: The association between neurofibromatosis type 1 (NF-I) and Chiari I malformation (CMI) is rare, and not many studies are reported in the literature. Performing magnetic resonance imaging (MRI) in patients with NF-1 is essential because several cases of Chiari type I are completely asymptomatic. We emphasize the need for inclusion of Chiari I as diagnosis in association with NF-1. Case Description: The patient was a 51-year-old black man who presented with complaints of pain and decreased motion and sensibility of his hands, wrists, and forearms, along with progressive dysarthria. Even though the computed tomography (CT) scan of the skull did not show changes, the MRI showed hydro/syringomyelia in the cervical spine area. Midline suboccipital craniectomy with total laminectomy of c1 and partial laminectomy of c2 was performed; tonsillectomy was also performed for cistern expansion because of intense thickening and obliteration of the obex by the cerebellar tonsils. Following treatment, the patient showed remission of symptoms. Conclusion: NF-1 in association with CMI is rare, and early diagnosis and surgical treatment are essential to slow down the myelopathy; although they prevent neurological damages, patients with NF-1 must remain under doctor's attention in case of association with CMI. Our literature review showed that symptoms can vary and include headache, gait disturbance, and sensory/motor diminution, until asymptomatic patients. Moreover, the incidence of NF-1 is considerably higher in CMI patients in comparison to the global incidence (8.6–11.8% and 0.775%, respectively). The surgical technique must be evaluated case by case according to the degree of cerebrospinal fluid obstruction. PMID:27500008

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

    Science.gov (United States)

    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.

  20. MRI findings and sleep apnea in children with Chiari I malformation.

    Science.gov (United States)

    Khatwa, Umakanth; Ramgopal, Sriram; Mylavarapu, Alexander; Prabhu, Sanjay P; Smith, Edward; Proctor, Mark; Scott, Michael; Pai, Vidya; Zarowski, Marcin; Kothare, Sanjeev V

    2013-04-01

    Chiari I malformation is characterized by downward herniation of the cerebellar tonsils through the foramen magnum. Scant data are available on the clinical course, relationship to the extent of herniation on magnetic resonance imaging in Chiari I malformation and the presence of sleep-disordered breathing on polysomnography. Retrospective analysis was performed looking at polysomnographic findings of children diagnosed with Chiari I malformation. Details on how Chiari I malformation was diagnosed, brainstem magnetic resonance imaging findings, and indications for obtaining the polysomnogram in these patients were reviewed. We also reviewed available data on children who had decompression surgery followed by postoperative polysomnography findings. Twenty-two children were identified in our study (11 males, median age 10 years, range 1 to 18). Three had central sleep apnea, five had obstructive sleep apnea, and one had both obstructive and central sleep apnea. Children with sleep-disordered breathing had excessive crowding of the brainstem structures at the foramen magnum and were more likely to have a greater length of herniation compared with those children without sleep-disordered breathing (P = 0.046). Patients with central sleep apneas received surgical decompression, and their conditions were significantly improved on follow-up polysomnography. These data suggest that imaging parameters may correlate with the presence of sleep-disordered breathing in children with Chiari I malformation.

  1. Fetal diffusion tensor quantification of brainstem pathology in Chiari II malformation

    Energy Technology Data Exchange (ETDEWEB)

    Woitek, Ramona; Prayer, Daniela; Weber, Michael; Schoepf, Veronika; Furtner, Julia; Asenbaum, Ulrika; Kasprian, Gregor [Medical University of Vienna, Department of Biomedical Imaging and Image-guided Therapy, Vienna (Austria); Amann, Gabriele [Medical University of Vienna, Department of Clinical Pathology, Vienna (Austria); Seidl, Rainer [Medical University of Vienna, Department of Paediatrics and Adolescent Medicine, Vienna (Austria); Bettelheim, Dieter [Medical University of Vienna, Department of Obstetrics and Gynecology, Vienna (Austria); Brugger, Peter C. [Medical University of Vienna, Center for Anatomy and Cell Biology, Vienna (Austria)

    2016-05-15

    This prenatal MRI study evaluated the potential of diffusion tensor imaging (DTI) metrics to identify changes in the midbrain of fetuses with Chiari II malformations compared to fetuses with mild ventriculomegaly, hydrocephalus and normal CNS development. Fractional anisotropy (FA) and apparent diffusion coefficient (ADC) were calculated from a region of interest (ROI) in the midbrain of 46 fetuses with normal CNS, 15 with Chiari II malformations, eight with hydrocephalus and 12 with mild ventriculomegaly. Fetuses with different diagnoses were compared group-wise after age-matching. Axial T2W-FSE sequences and single-shot echo planar DTI sequences (16 non-collinear diffusion gradient-encoding directions, b-values of 0 and 700 s/mm{sup 2}, 1.5 Tesla) were evaluated retrospectively. In Chiari II malformations, FA was significantly higher than in age-matched fetuses with a normal CNS (p =.003), while ADC was not significantly different. No differences in DTI metrics between normal controls and fetuses with hydrocephalus or vetriculomegaly were detected. DTI can detect and quantify parenchymal alterations of the fetal midbrain in Chiari II malformations. Therefore, in cases of enlarged fetal ventricles, FA of the fetal midbrain may contribute to the differentiation between Chiari II malformation and other entities. (orig.)

  2. Foramen magnum decompression for Chiari I malformation: a procedure not to be underestimated.

    Science.gov (United States)

    Duddy, John Charles; Allcutt, David; Crimmins, Darach; O'Brien, David; O'Brien, Donncha Finbarr; Rawluk, Daniel; Sattar, Mohammed Taufiq; Young, Steven; Caird, John

    2014-06-01

    Chiari I malformation may be treated with foramen magnum decompression (FMD). We aim to describe the symptoms with which patients initially present, and to determine the number and type of complications occurring after FMD for Chiari I malformation. Retrospective review of medical records for patients who had FMD performed for Chiari I malformation between January 2009 and December 2011. Post-operative outcomes were recorded and analysed. Patient demographic details and other relevant medical conditions were also noted. Between January 2009 and December 2011, 54 FMDs were performed for Chiari I malformation. Among them, 40(74%) patients were female and 14 patients (26%) were male. The majority of patients (42.6%) were aged 16-39 years and 24.07% of patients were children aged < 16 years. A total of 30(55.6%) patients had documented evidence of a syrinx pre-operatively. 18(33.3%) patients developed complications. Nine of these developed multiple complications while nine had a single problem. One mortality was reported. Ten (18.5%) patients developed hydrocephalus requiring shunting. Two patients developed subdural collections requiring evacuation associated with hydrocephalus. Six (11.1%) patients developed post-operative infections: two CNS infections; one wound infection; and three other infections. FMD for Chiari I malformation is a procedure which carries risk. In particular, the risk of developing post-operative hydrocephalus requiring permanent shunting is relatively high. ICP monitoring prior to FMD may be required to definitively rule out raised intracranial pressure.

  3. Fractal dimension analysis of cerebellum in Chiari Malformation type I.

    Science.gov (United States)

    Akar, Engin; Kara, Sadık; Akdemir, Hidayet; Kırış, Adem

    2015-09-01

    Chiari Malformation type I (CM-I) is a serious neurological disorder that is characterized by hindbrain herniation. Our aim was to evaluate the usefulness of fractal analysis in CM-I patients. To examine the morphological complexity features of this disorder, fractal dimension (FD) of cerebellar regions were estimated from magnetic resonance images (MRI) of 17 patients with CM-I and 16 healthy control subjects in this study. The areas of white matter (WM), gray matter (GM) and cerebrospinal fluid (CSF) were calculated and the corresponding FD values were computed using a 2D box-counting method in both groups. The results indicated that CM-I patients had significantly higher (p<0.05) FD values of GM, WM and CSF tissues compared to control group. According to the results of correlation analysis between FD values and the corresponding area values, FD and area values of GM tissues in the patients group were found to be correlated. The results of the present study suggest that FD values of cerebellar regions may be a discriminative feature and a useful marker for investigation of abnormalities in the cerebellum of CM-I patients. Further studies to explore the changes in cerebellar regions with the help of 3D FD analysis and volumetric calculations should be performed as a future work.

  4. Closing the dura: dural hitching versus surgicel and tisseel overlay graft in craniocervicaldecompression for Chiari 1 malformation.

    Science.gov (United States)

    Tonkins, Michael; Farooqi, Naeem; Ahmed, Rohan; Sinha, Saurabh; Bhattacharyya, Debapriya

    2017-03-02

    This study compares dural hitching to surgicel and tisseel overlay graft following craniocervical decompression and C1 laminectomy with simple durotomy for Chiari I malformation. Outcome measures were syrinx decompression, headache resolution and complication rates. A retrospective analysis of case notes was conducted. Patients who had undergone craniocervical decompression (CCD) were grouped by method of dural closure. Outcomes compared were rates of syrinx decompression, headache resolution, and post-operative complications. Statistical analysis was conducted using SPSS v20. We identified 32 adult patients for inclusion in this study. 53.1% (n = 17) had asyrinx, and 78.1% (n = 25) had a pre-operative headache. All were treated with suboccipital craniectomy, C1 laminectomy (with or without C2 laminectomy), and durotomy. The dura was either left open by dural hitching (n = 23) or closed with surgicel and tisseel overlay graft (n = 9). We found a statistically significant association between the method of dural closure and the rate of syrinx resolution. Resolution occurred in 91.7% (n = 11) of the hitching group, compared to 20.0% (n = 1) of the overlay graft group: Χ(2)(1) = 5.6, p = .018. There were no statistically significant differences between the two groups in the rates of headache resolution or other complications. In patients with symptomatic Chiari I malformation and associated syringomyelia, syrinx resolution is more likely if the dura is hitched open rather than closed bysurgicel and tisseel overlay graft after durotomy.

  5. Quantitative trait loci (QTL study identifies novel genomic regions associated to Chiari-like malformation in Griffon Bruxellois dogs.

    Directory of Open Access Journals (Sweden)

    Philippe Lemay

    Full Text Available Chiari-like malformation (CM is a developmental abnormality of the craniocervical junction that is common in the Griffon Bruxellois (GB breed with an estimated prevalence of 65%. This disease is characterized by overcrowding of the neural parenchyma at the craniocervical junction and disturbance of cerebrospinal fluid (CSF flow. The most common clinical sign is pain either as a direct consequence of CM or neuropathic pain as a consequence of secondary syringomyelia. The etiology of CM remains unknown but genetic factors play an important role. To investigate the genetic complexity of the disease, a quantitative trait locus (QTL approach was adopted. A total of 14 quantitative skull and atlas measurements were taken and were tested for association to CM. Six traits were found to be associated to CM and were subjected to a whole-genome association study using the Illumina canine high density bead chip in 74 GB dogs (50 affected and 24 controls. Linear and mixed regression analyses identified associated single nucleotide polymorphisms (SNPs on 5 Canis Familiaris Autosomes (CFAs: CFA2, CFA9, CFA12, CFA14 and CFA24. A reconstructed haplotype of 0.53 Mb on CFA2 strongly associated to the height of the cranial fossa (diameter F and an haplotype of 2.5 Mb on CFA14 associated to both the height of the rostral part of the caudal cranial fossa (AE and the height of the brain (FG were significantly associated to CM after 10 000 permutations strengthening their candidacy for this disease (P = 0.0421, P = 0.0094 respectively. The CFA2 QTL harbours the Sall-1 gene which is an excellent candidate since its orthologue in humans is mutated in Townes-Brocks syndrome which has previously been associated to Chiari malformation I. Our study demonstrates the implication of multiple traits in the etiology of CM and has successfully identified two new QTL associated to CM and a potential candidate gene.

  6. Intradiploic occipital pseudomeningocele in a patient with remote history of surgical treatment of Chiari malformation.

    Science.gov (United States)

    Mahaney, Kelly B; Menezes, Arnold H

    2014-11-01

    An intradiploic CSF pseudocyst is a rare entity that has been described in association with trauma, as a sequela of untreated hydrocephalus, or occasionally as a congenital finding in older adults. The authors present the case of a woman with a remote history of a posterior fossa intradural procedure, in which she underwent Chiari malformation decompression, Silastic substitute-assisted duraplasty, and occipitocervical fusion; she presented 19 years later with recurrent symptoms of Chiari malformation. She was found to have an occipital intradiploic pseudomeningocele, arising within her dorsal occipitocervical fusion mass and resulting in dorsal hindbrain compression. She underwent a posterior fossa decompression and revision of her failed duraplasty, and she had a good recovery. This case demonstrates intradiploic CSF pseudomeningocele as a rare potential delayed complication of an intradural procedure for the treatment of Chiari malformation with occipitocervical fusion.

  7. The Role of Cine Flow Magnetic Resonance Imaging in Patients with Chiari 0 Malformation.

    Science.gov (United States)

    Ozsoy, Kerem Mazhar; Oktay, Kadir; Cetinalp, Nuri Eralp; Gezercan, Yurdal; Erman, Tahsin

    2016-12-14

    The aim of this study was to define the role of phase-contrast cine magnetic resonance imaging in deciding the therapeutic strategy and underlying pathophysiology resulting in syrinx formation in patients with Chiari type 0 malformation. Seven patients admitted to our clinic with diagnosis of Chiari 0 malformations during the period January 2005 to July 2016 were enrolled in the study. All patients underwent a detailed preoperative neurological examination. The entire neuroaxis magnetic resonance imaging and phase-contrast cine magnetic resonance imaging was obtained preoperatively and postoperatively. Seven patients (5 female and 2 male) cover the inclusion criteria of the Chiari type 0. All of the patients with Chiari type 0 malformation had absent cine flow at the craniovertebral junction except two patients. All these five patients underwent surgical interventions; suboccipital decompression and duraplasty. All of them showed both clinical and imaging improvements postoperatively. Cine flow magnetic resonance imaging appears to be a useful tool in the management of patients with Chiari 0 malformations. There was a good correlation between the clinical presentation and cine flow preoperatively, and between clinical improvement and cine flow postoperatively.

  8. Surgical History of Sleep Apnea in Pediatric Patients with Chiari Type 1 Malformation.

    Science.gov (United States)

    Pomeraniec, Isaac Jonathan; Ksendzovsky, Alexander; Yu, Pearl L; Jane, John A

    2015-10-01

    Sleep apnea represents a relative indication for posterior fossa decompression in pediatric patients with Chiari malformation type 1. Duraplasty was associated with improvement of sleep apnea in 100% of patients and dural splitting with improvement in 50% of patients. Duraplasty and dural splitting were associated with a similar reduction in tonsillar herniation on radiographic imaging of 58% (37% excluding tonsillectomy) and 35%, respectively. Longitudinal follow-up studies of patients with either neurologic deficits or severe symptoms will further elucidate the natural history of Chiari malformation type 1 and more appropriately gauge the risk-benefit tradeoff of surgical intervention.

  9. Simulating Cerebrospinal Fluid Flow and Spinal Cord Movement Associated with Syringomyelia

    OpenAIRE

    Vinje, Vegard

    2016-01-01

    Syringomyelia is a progressive disease where fluid filled cavities develop inside the spinal cord, and is frequently seen together with Chiari Malformation I (CMI). CMI is characterized by downwards displacements of the Cerebellar Tonsils obstructing flow in the Subarachnoid space, (SAS) which causes abnormal Cerebrospinal fluid (CSF) flow. Many theories on the pathogenesis of syringomyelia have been proposed, many related to abnormal CSF flow, but a full explanation has not yet been given. I...

  10. Surgical treatment of Chiari malformation and discussion of related questions%Chiari畸形的外科治疗及相关问题探讨

    Institute of Scientific and Technical Information of China (English)

    刘翔; 刘宏毅; 章文斌; 邹元杰

    2011-01-01

    Objective To study the surgical treatment of the Chiari malformation and relevant clinical problems. Methods The clinical data of 35 patients with Chiari malformation ,in whom 26 with syringomyelia were analyzed retrospectively. All patients were operated after posterior fossa decompression, C1 bow decompression, pillow big pool expand forming, cerebellar tonsillar next hernia dissection or electricity coagulation resection. Results All patients were followed up from 1to 4 years. The clinical symptoms were ameliorated and dynamic review images were obtained a good result. Conclusion According to the clinical symptoms and radiologic features, personalized choices in the posterior fossa decompression of big or small bone window, pillow big pool expand forming and and resection of downward hernia of cerebellar tonilar were an effective method in the surgical treatment of Chiari malformation.%目的 探讨Chiari 畸形的外科手术治疗和相关临床问题.方法 回顾分析35 例Chiari 畸形(26 例合并脊髓空洞)的外科治疗经验.全部病例均行后颅窝减压,C1 后弓减压,枕大池扩大成形,小脑扁桃体下疝切除术或电灼术.结果 全部病例随访6 个月~ 4 年,获得不同程度的临床症状改善,动态复查影像均获得良好的效果.结论 Chiari 畸形的外科治疗中根据临床症状和影像特点,个性化的选择行后颅窝大或小的骨窗减压,枕大池扩大成形和小脑扁桃体下疝切除手术治疗,是处理该种畸形获得临床疗效的好方法.

  11. Management of Chiari I malformation in children: effectiveness of intra-operative ultrasound for tailoring foramen magnum decompression.

    Science.gov (United States)

    Narenthiran, Ganesalingam; Parks, Christopher; Pettorini, Benedetta

    2015-08-01

    Patients with Chiari I malformation (CM-1) commonly undergo foramen magnum decompression (FMD). However, there is no consensus on how this should be achieved. An approach would be to rationalize surgical steps based on pre-operative imaging and intra-operative findings. The aim of this study is to assess the usefulness of intra-operative ultrasound scanning (IOUS) in tailoring foramen magnum decompression in children with CM-1 and whether the use of IOUS is effective in reducing the risk of complications without increasing the need for re-operation. We performed a retrospective study. IOUS was utilized during FMD: a subjective assessment was made on whether there was adequate cerebellar tonsillar and/or CSF pulsation following suboccipital craniectomy. If there was adequate pulsation, the dura was not opened. Additional data were collected including age and gender of patients, presence of pre-operative syringomyelia, intra-operative ultrasound findings, length of follow-up, complications and radiological and clinical outcome. The statistical analysis was performed with XLStat®(Addinsoft SARL™, France). Nineteen patients underwent FMD from June 2011 to December 2012. The mean age was 10.5 years; there were nine females and ten males. Eleven patients had syringomyelia at diagnosis. Based on IOUS, eight patients underwent dural decompression and 11 patients bony decompression only. One patient had a post-operative pseudomeningocoele and two patients required re-operation. There was no significant statistical difference between the two groups regarding post-operative improvement in the syrinx. We found that tailoring FMD for patients with CM-1 using intra-operative findings using ultrasound scan was useful in avoiding unnecessary manoeuvres, while not compromising on the outcome.

  12. Association of Chiari I malformation and cerebellar ectopia with sensorineural hearing loss.

    Science.gov (United States)

    Haktanir, Alpay; Yücedağ, Fatih; Kaçar, Emre; Ulu, Sahin; Gültekin, Mehmet Ali; Ünlü, Ebru; Bucak, Abdülkadir; Ayçiçek, Abdullah

    2013-07-01

    We aimed to examine the prevalence of cerebellar tonsil ectopia and Chiari 1 malformation in sensorineural hearing loss (SHL) that has, to the best of our knowledge, not been studied previously. Magnetic resonance imaging records of 166 subjects with SHL and 50 controls without known otologic disturbances were included in the study. A tonsils descent more than 2 mm was assumed as cerebellar ectopia, and a descent equal to or more than 5 mm was assumed as Chiari 1 malformation. A tonsil descent group was also formed by summation of both groups. Transverse diameters of bilateral intracranial vertebral arteries and transverse sinuses were also measured, and all parameters were analyzed using appropriate statistics. A significant difference of frequencies of Chiari 1, ectopia, and tonsil descent was detected between patients and controls. In comparison of cerebellar ectopia and Chiari 1 groups, SHL did not show any significant difference. The left lateral sinus diameter showed positive correlation with tonsil descent. There was no significant correlation for the diameters of other vessels. A powerful correlation was detected between SHL and age. In addition, right and vertebral artery diameters showed positive correlations with age. Chiari 1 malformation and cerebellar ectopia showed an association with SHL. These patients should also be evaluated for otologic disturbances. Further high-resolution magnetic resonance imaging studies to explain the exact cause of this currently unknown association seems required.

  13. A novel technique to treat acquired Chiari I malformation after supratentorial shunting

    NARCIS (Netherlands)

    Potgieser, Adriaan R E; Hoving, Eelco W

    2016-01-01

    PURPOSE: The acquired Chiari I malformation with abnormal cranial vault thickening is a rare late complication of supratentorial shunting. It poses a difficult clinical problem, and there is debate about the optimal surgical strategy. Some authors advocate supratentorial skull enlarging procedures w

  14. Chiari Type I Malformations in Young Adults: Implications for the College Health Practitioner

    Science.gov (United States)

    Elam, Mary Jane; Vaughn, John A.

    2011-01-01

    In this article, the authors describe 2 cases of Chiari type I malformation (CM-I) in students presenting to a college health center within a 6-month period. A review of CM-I, including epidemiology, typical presentation, evaluation, and management, is followed by a discussion of the clinical and functional implications of the disorder in an…

  15. Anesthetic management in a child with Arnold-Chiari malformation and bilateral vocal cord paralysis.

    NARCIS (Netherlands)

    Setz, A.C.W.; Boer, H.D. de; Driessen, J.J.; Scheffer, G.J.

    2005-01-01

    We report a case of a child who was scheduled for an emergency ventriculoperitoneal shunt procedure. The patient had a type II Arnold-Chiari malformation (ACM) and associated hydrocephalus and presented with near complete respiratory obstruction from bilateral abductor vocal cord palsy. Early diagno

  16. The Arnold-Chiari Malformation and Its Implications for Individuals with Spina Bifida and Hydrocephalus.

    Science.gov (United States)

    Mittler, Joel E.

    1986-01-01

    The Arnold-Chiari malformation is present in most infants born with myelomeningocele (a form of spina bifida) and hydrocephalus. The syndrome is responsible for structural abnormalities in the brain, and peripheral nervous system. Etiology, symptoms, impact on central nervous system structures, surgical treatment, and implications for education…

  17. Recurrent subdural hygromas after foramen magnum decompression for Chiari Type I malformation.

    Science.gov (United States)

    Pereira, Erlick A C; Steele, Louise F; Magdum, Shailendra A

    2014-06-01

    A paediatric case of foramen magnum decompression for Chiari Type I malformation complicated by recurrent subdural hygromas (SH) and raised intracranial pressure without ventriculomegaly is described. SH pathogenesis is discussed, with consideration given to arachnoid fenestration. We summarise possibilities for treatment and avoidance of this unusual consequence of foramen magnum decompression.

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

    Science.gov (United States)

    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.

  19. Myelopathy-mimicking symptoms of epidural venous engorgement and syringomyelia due to inferior vena cava stenosis at the thoracolumbar junction in a patient with Budd-Chiari syndrome.

    Science.gov (United States)

    Lee, Jung-Hee; Song, Wook-Jae; Kang, Kyung-Chung

    2015-10-01

    Epidural venous engorgement can result from various lesions, such as arteriovenous malformation, thrombosis or occlusion of the inferior vena cava (IVC), or an abdominal masslike lesion. Most patients with these problems complain of low-back pain, radicular pain, or neurogenic claudication, which are symptoms suggestive of disc herniation or spinal stenosis. However, these patients rarely exhibit neurological deficits or cauda equina syndrome. The authors encountered a case of a 60-year-old man presenting with lower-extremity weakness and voiding difficulty for a period of 1 year. To investigate the patient's myelopathy-mimicking symptoms, a lumbar spine MRI scan was performed. The MR images exhibited tortuous and dilated spinal vessels compressing the spinal cord and thecal sac at the T11-L3 level, which were concurrent with syringomyelia evidenced by a 22 × 2.5-mm cyst at the T11-12 level. 3D CT scanning of the whole aorta revealed total occlusion and regression of the IVC in the intrahepatic region 3 cm inferior to the right atrium and dilation of multiple collateral veins. The patient was diagnosed with chronic Budd-Chiari syndrome Type I. The authors performed venography, followed by intrahepatic IVC recanalization via stent placement under fluoroscopic and ultra sonographic guidance and without surgical exploration. After this treatment, there was a marked decrease in epidural venous engorgement and the patient's symptoms resolved almost completely. This case indicates that epidural venous engorgement at thoracolumbar levels may cause symptoms suggestive of myelopathy and can be successfully treated by minimally invasive procedures to eliminate the underlying causes.

  20. Retrospective study of patients with Chiari: malformation submitted to surgical treatment Estudo retrospectivo de pacientes com malformação de Chiari tratados cirurgicamente

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    Mário Augusto Taricco

    2008-06-01

    Full Text Available The Chiari malformation (CM is characterized by variable herniation of one or both cerebellar tonsils, associated or not with displacement of the hindbrain structures into the vertebral canal. This is a retrospective study of 29 patients with CM submitted to surgical procedure between 1990 and 2003. There were 14 females and 15 males whose ages ranged from 16 to 65 years. There were seven patients with isolated CM, 12 associated with syringomyelia (SM, three associated with basilar impression (BI and seven associated with SM and BI. The surgery was based on posterior fossa decompression. In seven patients a catheter was introduced from the subarachnoid space into the III ventricle and five were submitted to tonsillectomy. Twenty-one patients improved, one worsened, one remained unchanged, four missed follow up and two died. We conclude that the best results with CM surgery are obtained by an effective posterior fossa decompression. Those CM cases associated with other abnormalities, such as SM and BI, probably need complementary techniques which will be the theme for new prospective studies.A malformação de Chiari (MC é o deslocamento variável de uma ou ambas as tonsilas cerebelares para o canal vertebral. Este trabalho é um estudo retrospectivo de pacientes com MC tratados com cirurgia de 1990 até 2003. Foram analisados 29 prontuários, sendo 15 pacientes do sexo masculino e 14 do feminino, com idade variando entre 16 a 65 anos. Destes, sete só apresentavam MC, 12 tinham MC associada com siringomielia (SM, três associada com impressão basilar (IB e sete associada com SM e IB. Foi realizada descompressão da fossa posterior e plástica da dura-máter em todos. Em sete pacientes, foi acrescida a colocação de catéter do espaço subaracnóideo para o interior do IV ventrículo e em cinco a tonsilectomia. Ocorreu melhora dos sintomas em 21 pacientes, um permaneceu inalterado, houve piora em um caso, quatro não tiveram seguimento e dois

  1. Hemifacial spasm in a patient with neurofibromatosis and Arnold-Chiari malformation: a unique case association Espasmo hemifacial em paciente com neurofibromatose e malformação de Arnold-Chiari: uma associação rara

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    Andre Carvalho Felício

    2007-09-01

    Full Text Available BACKGROUND: The association of hemifacial spasm (HFS, Chiari type I malformation (CIM and neurofibromatosis type 1 (NF1 has not been described yet. CASE REPORT: We report the case of a 31-year-old woman with NF1 who developed a right-sided HFS. On magnetic resonance imaging (MRI a CIM was seen without syringomyelia. The patient has been successfully treated with botulinum toxin type A injections for 5 years without major side effects. CONCLUSION:Clinical features of HFS, CMI and NF1 are highlighted together with their possible relationship. Also, therapeutic strategies are also discussed.INTRODUÇÃO: A associação entre espasmo hemifacial (EHF, malformação de Chiari tipo I (MCI e neurofibromatose tipo I (NFI ainda não foi descrita. RELATO DO CASO: Relatamos o caso de mulher com 31 anos com NFI que desenvolveu EHF à direita. Na ressonância magnética (RM foi observada MCI sem seringomielia associada. A paciente foi tratada com sucesso com toxina botulínica tipo A por 5 anos sem efeitos colaterais. CONCLUSÃO: Ressaltamos as características clínicas do EHF, MCI e NFI assim como uma possível relação entre elas. Além disto, discutimos também estratégias terapêuticas.

  2. Anesthesia management for pregnant patient with Arnold-Chiari malformation type I: A case report

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    Şaban Yalçın

    2012-06-01

    Full Text Available The Arnold-Chiari I malformation (ACM is a congenitalanomaly characterized by downward herniation of thecerebellar tonsils into the spinal canal. The anomaly maypresent in a variety of ways with vague symptoms suchas head and limb pains, vertigo, hypoesthesia, weaknessin the extremities. Diagnosis is often difficult and thereforedelayed. Measurement of intracranial pressure in thesepatients is an important measure thus precautions shoulbe taken for avoidance of increased intracranial pressure.In this case report, we discussed the anesthetic managementof a pregnant patient with Arnold Chiari I malformationwho underwent an emergency caesarean section.

  3. Diagnosis of Chiari III malformation by second trimester fetal MRI with postnatal MRI and CT correlation

    Energy Technology Data Exchange (ETDEWEB)

    Smith, Alice B.; Glenn, Orit A. [University of California, San Francisco, Department of Radiology, San Francisco, CA (United States); Gupta, Nalin [University of California, San Francisco, Department of Neurosurgery, San Francisco, CA (United States); Otto, Carl [California Pacific Medical Center, Department of Perinatology, San Francisco, CA (United States)

    2007-10-15

    We report a case of Chiari III malformation diagnosed by fetal MRI. Ultrasound (US) performed at a gestational age of 18 weeks demonstrated a posterior skull base cyst. Repeat US at 19 weeks demonstrated neural tissue in the cyst, consistent with an encephalocele. MR imaging at 23 weeks confirmed the presence of an occipital encephalocele, demonstrated additional bony defect in the upper cervical spine, and identified abnormal morphology and position of the brainstem consistent with the diagnosis of Chiari III. Postnatal MRI and CT confirmed the fetal MRI findings and demonstrate the utility of fetal MRI in the early evaluation of songraphically detected posterior fossa abnormalities. (orig.)

  4. [Brainstem auditory evoked potentials and somatosensory evoked potentials in Chiari malformation].

    Science.gov (United States)

    Moncho, Dulce; Poca, María A; Minoves, Teresa; Ferré, Alejandro; Rahnama, Kimia; Sahuquillo, Juan

    2013-06-16

    Introduccion. La malformacion de Chiari (MC) incluye una serie de anomalias congenitas que tienen como comun denominador la ectopia de las amigdalas del cerebelo por debajo del foramen magno, lo que puede condicionar fenomenos compresivos del troncoencefalo, la medula espinal alta y los nervios craneales, alterando las respuestas de los potenciales evocados auditivos del tronco cerebral (PEATC) y de los potenciales evocados somatosensoriales (PESS). Sin embargo, las indicaciones de ambas exploraciones en las MC han sido motivo de estudio en un numero limitado de publicaciones, centradas en series cortas y heterogeneas de pacientes. Objetivo. Revisar los hallazgos de los PEATC y los PESS en los estudios publicados en pacientes con MC tipo 1 (MC-1) o tipo 2 (MC-2), y su indicacion en el diagnostico, tratamiento y seguimiento, especialmente en la MC-1. Desarrollo. Es un estudio de revision realizado mediante analisis de los estudios publicados en Medline desde 1966, localizados mediante PubMed, utilizando combinaciones de las palabras clave 'Chiari malformation', 'Arnold-Chiari malformation', 'Chiari type 1 malformation', 'Arnold-Chiari type 1 malformation', 'evoked potentials', 'brainstem auditory evoked potentials' y 'somatosensory evoked potentials', asi como informacion de pacientes con MC-1 valorados en los servicios de neurocirugia y neurofisiologia clinica del Hospital Universitari Vall d'Hebron. Conclusiones. Los hallazgos mas comunes de los PESS son la reduccion en la amplitud cortical para el nervio tibial posterior, la reduccion o ausencia del potencial cervical del nervio mediano y el aumento del intervalo N13-N20. En el caso de los PEATC, los hallazgos mas frecuentes descritos son el aumento del intervalo I-V y la alteracion periferica o coclear.

  5. 分期前后方减压治疗合并颅底陷入的Chiari畸形%Treatment of Chiari malformation combined with basilar invagination by staged anterior and posterior decompression

    Institute of Scientific and Technical Information of China (English)

    佟怀宇; 余新光; 张远征; 乔广宇; 王鹏; 尚爱加

    2012-01-01

    Objective There is usually anterior compression of the brain stem in Chiari malformation patients combined with basilar invagination.After anterior decompression,many patients will present elevation of the cerebellar tonsil and resolution of syringomyelia.However,still in some patients,the Chiari malformation and syringomyelia remain unchanged after anterior decompression.This article is to discuss a staged anterior and posterior decompression strategy for the treatment of these patients.Methods 9 cases of Chiari malformation combined with basilar invagination were operated by transoral transpharyngeal odontoidectomy first,observed for 1 -2 months,the Chiari malformation and syringomyelia did not relieve,then posterior foramen magnum decompression,tonsillectomy and expansion of posterior fossa dura combined with occipito - cervical fusion or C1-2 fusion were performed.The patients were followed up for 24 - 60 months ( mean 32 months).Results There was no mortality or severe complication.The clinical symptoms were improved in all patients.The Chiari malformation and syringomyelia were relieved in 8 patients,while unchange in 1 case.Conclusions For Chiari malformation patients combine with basilar invagination,anterior decompression with odontoidectomy should be performed first. In many patients,increase ofposterior fossa volume,improved CSF dynamic may be achieved,posterior fusion will be enough.While in some other patients,only symptoms of basilar invagination may be improved after anterior decompression.So posterior decompression is still important.%目的 合并颅底陷入的Chiari畸形患者常有前方脑干压迫,多数经前路减压即可使扁桃体上移,空洞缩小,但少数患者前路减压后Chiari畸形和脊髓空洞并不减轻,本文旨在探讨采用分期前后方减压治疗该类患者.方法 9例患者经口咽入路齿状突磨除,观察1-2个月后发现Chiari畸形和脊髓空洞未减轻,遂行后方枕大孔减压,小脑扁桃体切

  6. Morphometric analysis of foramen magnum dimensions and intracranial volume in pediatric Chiari I malformation.

    Science.gov (United States)

    Furtado, Sunil V; Thakre, Darpan J; Venkatesh, Prasanna K; Reddy, Kalyan; Hegde, A S

    2010-02-01

    Foramen magnum dimensions and intracranial volume in Chiari I malformations in children were studied, and the statistical relationship between patient demographics, radiological features and foramen magnum morphometry was investigated. Linear measurements were used to calculate the intracranial volume using preoperative magnetic resonance images and computed tomogram images. The area of the foramen magnum was obtained independently using computer imaging software and a regression formula. The result of 21 pediatric patients was compared with a matched control group. The area of the foramen magnum was within the range of the expected value deduced using a formula based on the intracranial volume. There was no statistical difference in the area and linear dimensions of the foramen magnum in the study and control groups. Six patients (28%) had a foramen magnum in close proximity to a spherical shape. The authors provide a simple, accurate and reproducible method of estimating foramen magnum area in the pediatric Chiari I group. The irregular shape of the foramen magnum is accentuated by developmental bony and soft tissue anomalies at the cranio-vertebral junction in Chiari malformation. Consequently, an individualized cross-sectional assessment of the foramen magnum in relation to the hindbrain tissue in the same plane is required to study the initiation and propagation of the Chiari I symptomatology.

  7. Clinical course of incidental syringomyelia without predisposing pathologies.

    Science.gov (United States)

    Kim, Jiha; Kim, Chi Heon; Jahng, Tae-Ahn; Chung, Chun Kee

    2012-05-01

    Although the widespread use of MRI has facilitated the diagnosis of subclinical syringomyelia, little information has been established regarding its natural course. To elucidate the clinical course and treatment strategy of incidental syringomyelia without predisposing pathologies, we retrospectively reviewed the clinical course of 12 adult patients with incidental syringomyelia. No patients had any predisposing pathology, including Chiari malformation or spinal cord tumor. Using the medical records and MRI, we analyzed the neurological and radiological features of each patient. After a mean of 39.9 months follow-up, no patient developed neurological deterioration. Although one patient had radiological progression without neurological deterioration, 11 patients (91.7%) had no change on MRI. Additionally, all patients experienced a favorable clinical course without surgery. These results indicate that, for patients with incidental syringomyelia without predisposing pathology, close observation rather than surgery can be recommended.

  8. Arnold—chiari畸形并脊髓空洞症显微手术治疗(附19例临床分析)%Microsurgery treatment for Arnold- chiari malformation associated with syrigomyelia: technique in 19 oprations

    Institute of Scientific and Technical Information of China (English)

    陈彬; 高志波; 梁卫东; 高心保

    2011-01-01

    Objective To study the method of operative treatment for Arnold-chiari malformation associated with syringomyelia. Methods 19 cases of Arnold-chiari malformation associated with syringomyelia were performed microsurgery treatment According to different conditions, different operations were given,including posterior cranial fossa decompression, cerebellar tonsillectomy, syringomyelia section and so on, whose purposes were to remove the deformity of great occipital foramen region, relieve the compression on the cranial nerves of posterior group and recover the normal circulation of cerebraospinal fluid in vertebral canal. Results The clinical symptoms in all cases were improved in different degress after operation. The inferior hernia of cerebellar tonsil had recovered after follow - up by MRI examination. The cavity of spinal cord reduced. Conclusion The different operative styles in patients with arnold-chiari malformation associated with syringomyelia should be adopted according to the patient's conditions and imaging examinations, which can attain a satisfactory effect.%目的 观察Arnold-chiari畸形并脊髓空洞症的手术治疗及效果.方法 对19例Arnold-chiari畸形并脊髓空洞症的患者进行显微手术,根据不同情况,予后颅窝减压、小脑扁桃体切除、脊髓空洞切开分流术等手术,以解除枕骨大孔区畸形,缓解对后组颅神经压迫,并恢复椎管内脑脊液正常循环状态.结果 19例患者术后临床症状均有不同程度改善.术后随访复查MRI可见小脑扁桃体下疝回纳,脊髓空洞缩小.结论 对Arnold-chiari畸形并脊髓空洞症患者,根据其MRI采取不同手术方法,可以达到满意疗效.

  9. An association of 19p13.2 microdeletions with Malan syndrome and Chiari malformation.

    Science.gov (United States)

    Shimojima, Keiko; Okamoto, Nobuhiko; Tamasaki, Akiko; Sangu, Noriko; Shimada, Shino; Yamamoto, Toshiyuki

    2015-04-01

    Patients with microdeletions in the 19p13.2 chromosomal region show developmental delays, overgrowth, and distinctive features with big head appearances. These manifestations are now recognized as Sotos syndrome-like features (Sotos syndrome 2) or Malan syndrome. We identified three female patients with 19p13.2 deletions involving NFIX, a gene responsible for Malan syndrome. We compared the genotypic and phenotypic data of these patients with those of the patients previously reported. The most of the clinical features were found to overlap; however, Chiari malformation type I was observed in two of the three patients evaluated in this study. Because Chiari malformation type I has never been reported in the patients with NSD1-related Sotos syndrome, this finding indicates the possible role of 19p13.2 deletion in patients with mimicking features of Sotos syndrome but have negative NSD1 testing results.

  10. Chiari I malformation associated with atlanto-occipital assimilation presenting as orthopnea and cough syncope.

    Science.gov (United States)

    Mangubat, Erwin Zeta; Wilson, Tom; Mitchell, Brian A; Byrne, Richard W

    2014-02-01

    Although it is not uncommon for patients with Chiari I malformations to present with respiratory complaints, cough syncope is a rare presenting symptom. We report an adult patient who had both a Chiari I malformation and atlanto-occipital assimilation, and complained of cough syncope, orthopnea, and central sleep apnea. The patient underwent decompressive craniectomy of the posterior fossa and a cervical level 2 laminectomy. However, due to an initial under-appreciation of the profound narrowing of the foramen magnum as a result of these concomitant pathologies, the patient had continued impaired cerebrospinal fluid flow, leading to a symptomatic pseudomeningocele and required a more extensive decompression that included a cervical level 3 laminectomy as well as a temporary lumbar drain. On 2 year follow-up, he remained asymptomatic.

  11. Bilateral Vocal Cord Palsy with Arnold Chiari Malformation: A Rare Case Series

    Science.gov (United States)

    Arora, Nikhil; Meher, Ravi; Bhargava, Eishaan K.

    2016-01-01

    Stridor in paediatric age group is not an uncommon presentation to the ENT emergency. The range of differential diagnosis is vast. The presentation may vary from noisy breathing to severe respiratory distress and apnea. Early and meticulous diagnosis is crucial for the management as the condition may be life threatening. We report a rare case series of 3 infants with Arnold Chiari Malformation who presented to the hospital with stridor and were diagnosed with bilateral vocal cord palsy. These 3 infants had similar underlying neurological condition with hydrocephalus and raised intracranial pressure. Chiari malformation is the one of the most common congenital central nervous system anomaly associated with bilateral vocal cord paralysis. However, the presentation is rare. This article, thus, emphasizes the significance of early diagnosis and immediate management of this condition. PMID:27790480

  12. Arnold Chiari malformation with spina bifida: a lost opportunity of folic Acid supplementation.

    Science.gov (United States)

    Ganesh, Deepa; Sagayaraj, Benjamin M; Barua, Ravi Kumar; Sharma, Nidhi; Ranga, Upasana

    2014-12-01

    In Arnold Chiari (kee-AHR-ee) II malformation elongated cerebellar tonsils are displaced inferiorly through the Foramen Magnum into the upper cervical spinal canal. It is a complex anomaly with skull, dura, brain, spine and cord manifestations. Meningomyelocele is seen in all cases. We present a case of type II Arnold Chiari Malformation diagnosed in utero in a pregnant lady .There was no periconceptional folic acid supplementation. As the role of the Methylene Tetra Hydro Folate Reductase gene polymorphism in neural tube defects is becoming evident, a simple opportunity as folic acid supplementation should not be missed. Folate supplementation as fortification of cereal grains will also prevent other conditions like congenital heart defects, urinary tract anomalies, orofacial defects, limb defects and pyloric stenosis.

  13. Trans-amniotic stem cell therapy (TRASCET) minimizes Chiari-II malformation in experimental spina bifida.

    Science.gov (United States)

    Dionigi, Beatrice; Brazzo, Joseph A; Ahmed, Azra; Feng, Christina; Wu, Yaotang; Zurakowski, David; Fauza, Dario O

    2015-06-01

    We sought to study the impact of trans-amniotic stem cell therapy (TRASCET) in the Chiari-II malformation in experimental spina bifida. Sprague-Dawley fetuses (n=62) exposed to retinoic acid were divided into three groups at term (21-22 days gestation): untreated isolated spina bifida (n=21), isolated spina bifida treated with intra-amniotic injection of concentrated, syngeneic, labeled amniotic fluid mesenchymal stem cells (afMSCs) on gestational day 17 (n=28), and normal controls (n=13). Analyses included measurements of brainstem and cerebellar placement on high resolution MRI and histology. Statistical comparisons included ANOVA. In parallel to the expected induced coverage of the spina bifida in the afMSC-treated group (Pspina bifida by TRASCET minimizes the Chiari-II malformation in the retinoic acid rodent model, further suggesting it as a practical alternative for the prenatal management of spina bifida. Copyright © 2015 Elsevier Inc. All rights reserved.

  14. CT in three cases of syringomyelia

    Energy Technology Data Exchange (ETDEWEB)

    Ugawa, Yoshikazu; Sakuta, Manabu (Japan Red Cross Medical Center, Tokyo (Japan)); Yagishita, Akira; Inoue, Kiyoharu

    1983-08-01

    We presented the results of our experience with metrizamide computed tomographic myelography (MCTM) and myelography in three cases of syringomyelia. In case 1, MCTM revealed Chiari malformation, even though this malformation was not clear in the myelography. In case 3, Chiari malformation was apparent in MCTM and in the myelography. In case 1, CT of the spinal cord without enhancement (plain CT) showed a syrinx in the cervical spinal cord, and MCTM made the syrinx clearer with enhancement. MCTM also demonstrated a cavity at the levels of Th/sub 6/ and L/sub 1/ of the vertebra. In case 2, a syrinx was disclosed in MCTM, in spite of normal myelography. In case 3, myelography demonstrated a spinal cord swelling. MCTM made a syrinx less clear than plain CT. Not only MCTM but also plain CT is an excellent method for the recognition of syringomyelia. The non-invasive plain CT is the first choice of the examinations in cases with syrinogomyelia, because MCTM may make a syrinx obscure. CT and MCTM have to be performed at many levels of the spinal cord in case with syringomyelia. CT or MCTM may demonstrate a syrinx or a cavity at the levels of the spinal cord, where no syrinx is suspected to exist by clinical features, as well as at the levels appearing normal in the myelography.

  15. Intraoperative somatosensory evoked potential recovery following opening of the fourth ventricle during posterior fossa decompression in Chiari malformation: case report.

    Science.gov (United States)

    Grossauer, Stefan; Koeck, Katharina; Vince, Giles H

    2015-03-01

    The most appropriate surgical technique for posterior fossa decompression in Chiari malformation (CM) remains a matter of debate. Intraoperative electrophysiological studies during posterior fossa decompression of Type I CM (CM-I) aim to shed light on the entity's pathomechanism as well as on the ideal extent of decompression. The existing reports on this issue state that significant improvement in conduction occurs after craniotomy in all cases, but additional durotomy contributes a further improvement in only a minority of cases. This implies that craniotomy alone might suffice for clinical improvement without the need of duraplasty or even subarachnoid manipulation at the level of the craniocervical junction. In contrast to published data, the authors describe the case of a 32-year-old woman who underwent surgery for CM associated with extensive cervicothoracic syringomyelia and whose intraoperative somatosensory evoked potentials (SSEPs) did not notably improve after craniotomy or following durotomy; rather, they only improved after opening of the fourth ventricle and restoration of CSF flow through the foramen of Magendie. Postoperatively, the patient recovered completely from her preoperative neurological deficits. To the authors' knowledge, this is the first report of significant SSEP recovery after opening the fourth ventricle in the decompression of a CM-I. The electrophysiological and operative techniques are described in detail and the findings are discussed in the light of available literature. The authors conclude that there might be a subset of CM-I patients who require subarachnoid dissection at the level of the craniocervical junction to benefit clinically. Prospective studies with detailed electrophysiological analyses seem warranted to answer the question regarding the best surgical approach in CM-I decompression.

  16. Malformação de Chiari do tipo II sintomática Symptomatic Chiari type II malformation

    Directory of Open Access Journals (Sweden)

    J. FRANCISCO SALOMÃO

    1998-03-01

    Full Text Available Os autores analisam uma série de 17 crianças portadoras de mielodisplasias que desenvolveram sinais e sintomas da malformação de Chiari do tipo II. De acordo com a idade, dois grupos ficaram bem definidos: Grupo I, crianças no primeiro ano de vida, em que predominaram sinais e sintomas de comprometimento do tronco encefálico e nervos cranianos bulbares (n=13; Grupo II, composto por crianças com idade superior a um ano, em que as principais manifestações foram dor cervical e sinais cerebelares (n=4. O resultado do tratamento cirúrgico nos dois grupos foi distinto: enquanto a mortalidade no Grupo I atingiu 46,1%, nenhum paciente do Grupo II veio a falecer. O tratamento inicial consistiu na instalação ou revisão de derivação ventricular, sendo a descompressão crânio-vertebral reservada àqueles que não se beneficiaram com esses procedimentos. Os autores enfatizam a necessidade do imediato reconhecimento e tratamento do quadro, de modo a se obter resultados satisfatórios.The Chiari type II malformation is the leading cause of death in infants with myelomeningocele. The authors report 17 cases of symptomatic Chiari type II malformation occurring in two distinct age dependent population. In Group I, 13 neonates and infants in the first year of life presented with cranial nerve and brain stem dysfunction characterized by vocal cord paralysis, apnea, dysphagia and laryngeal stridor. In Group II, 4 patients developed signs and symptoms after the first year of life. In this group, the presentation was more insidious and included neck pain and cerebellar manifestations. The surgical treatment consisted initially in shunt implantation or revision and when there was no improvement, posterior fossa decompression was performed. The response to the surgical treatment differed considerably between the two groups: older patients improved promptly after surgery and there was no casualties; in newborn and infants, especially those under 6 months

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

    Science.gov (United States)

    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

  18. Diagnosis and microsurgical treatment of Chiari malformation%Chiari畸形的临床诊断和显微手术治疗体会

    Institute of Scientific and Technical Information of China (English)

    丁晓东; 张远征

    2011-01-01

    目的 探讨Chiari畸形的诊断及显微外科处理方法.方法 回顾性分析50例Chiari畸形的临床资料,其中合并脊髓空洞30例,齿状突型颅底陷入3例.结果 50例术后平均随访35个月,按Tator疗效标准:优36例(72%);良12例(24%);差2例(4%).有效率96%.空洞缩小21例,消失7例.结论 有限的后颅窝减压及枕大池重建是治疗Chiari畸形的有效方法;合并齿状突型颅底陷入应先行口咽入路齿状突磨除术或许为较好的选择.%Objective To study the diagnosis and the microsurgical treatment for Chiari malformation( CM ).Methods The clinical data of 50 cases of Chiari malformation were analyzed retrospectively,30 cases were combinated with syringomyelia,3 with cranial basal invagination referred to dens of axis.Results 50 cases were followed up for an average 35 months.According to Tator's standard,the curative effect was excellent in 36,good in 12 and bad in 2 patients.The effective rate was 96%.The cavities in spinal cords reduced in 21 cases,disappeared in 7.Conclusion Limited posterior fossa decompression with reconstruction of cistern magna is a effective method for treatment of CM ; CM with cranial basilar invagination referred to dens of axis treated firstly by anterior decompression of odontoidectomy via oral approach may be an optimal selection.

  19. ChiariⅠ型畸形合并脊髓空洞症的外科治疗探讨%Craniovertebral decompression and posterior fossa reconstruction treatment of Chiari syringomyelia complex

    Institute of Scientific and Technical Information of China (English)

    汪阳; 洪涛

    2006-01-01

    目的 探讨颈枕区减压和后颅窝重建术治疗ChiariⅠ型(chiari malformation typeⅠ,CMⅠ)畸形合并脊髓空洞症(syringomyelia,SM)的疗效.方法 对1998-2004年收治的69例患者,采取颈枕区减压和后颅窝重建术治疗.结果 术后58例获得随访,症状改善47例(81%),稳定7例(12%),恶化4例(7%),无手术死亡;MRI复查脊髓空洞有不同程度缩小,部分枕大池解剖恢复.结论 对CMⅠ合并SM患者,行颈枕区减压和后颅窝重建术,可以阻止病情发展,手术效果肯定,但要长期随访.

  20. Treatment of syringomyelia with a syringosubarachnoid shunt.

    Science.gov (United States)

    Tator, C H; Briceno, C

    1988-02-01

    The surgical results in 40 patients with syringomyelia, treated with a syringosubarachnoid shunt or other procedures are reviewed. The principal indication for surgery was that of significant neurological deterioration. There were 12 patients with idiopathic syringomyelia without tonsillar ectopia, 12 with an associated Chiari malformation, 11 with post-traumatic syringomyelia and five patients with spinal arachnoiditis. There were 38 syringosubarachnoid shunts performed in 35 patients, and an excellent or good result was achieved in 26 patients (74.3%). In terms of the type of syringomyelia, the best results were obtained in the idiopathic group without tonsillar ectopia and in the post-traumatic group. A short duration of pre-operative symptoms favoured a better outcome, and in our opinion, early surgical treatment is indicated for all patients with neurological deterioration. All eight patients in whom a posterior fossa decompression was performed as the initial surgical procedure required a second operation, either a syringosubarachnoid or syringoperitoneal shunt to achieve neurological improvement or stabilization. Thus, the syringosubarachnoid shunt is an effective therapeutic modality for patients with syringomyelia, particularly for the idiopathic and post-traumatic groups. More than one surgical procedure may be required to achieve cessation of deterioration. Overall, excellent or good results were achieved in 29 (72.5%) of the 40 patients.

  1. Mechanisms of cerebellar tonsil herniation in patients with Chiari malformations as guide to clinical management

    Science.gov (United States)

    Nishikawa, Misao; Kula, Roger W.; Dlugacz, Yosef D.

    2010-01-01

    Background The pathogenesis of Chiari malformations is incompletely understood. We tested the hypothesis that different etiologies have different mechanisms of cerebellar tonsil herniation (CTH), as revealed by posterior cranial fossa (PCF) morphology. Methods In 741 patients with Chiari malformation type I (CM-I) and 11 patients with Chiari malformation type II (CM-II), the size of the occipital enchondrium and volume of the PCF (PCFV) were measured on reconstructed 2D-CT and MR images of the skull. Measurements were compared with those in 80 age- and sex-matched healthy control individuals, and the results were correlated with clinical findings. Results Significant reductions of PCF size and volume were present in 388 patients with classical CM-I, 11 patients with CM-II, and five patients with CM-I and craniosynostosis. Occipital bone size and PCFV were normal in 225 patients with CM-I and occipitoatlantoaxial joint instability, 55 patients with CM-I and tethered cord syndrome (TCS), 30 patients with CM-I and intracranial mass lesions, and 28 patients with CM-I and lumboperitoneal shunts. Ten patients had miscellaneous etiologies. The size and area of the foramen magnum were significantly smaller in patients with classical CM-I and CM-I occurring with craniosynostosis and significantly larger in patients with CM-II and CM-I occurring with TCS. Conclusions Important clues concerning the pathogenesis of CTH were provided by morphometric measurements of the PCF. When these assessments were correlated with etiological factors, the following causal mechanisms were suggested: (1) cranial constriction; (2) cranial settling; (3) spinal cord tethering; (4) intracranial hypertension; and (5) intraspinal hypotension. PMID:20440631

  2. Yawning as a presenting symptom of Chiari malformation Type I: report of 2 cases.

    Science.gov (United States)

    Zebian, Bassel; Hogg, Florence Rosie Avila; Fu, Richard Zhiming; Sivakumaran, Ramanan; Stapleton, Simon

    2015-06-01

    Yawning is thought to be a behavior regulated by the brainstem. Although excessive yawning has been reported in brainstem strokes, demyelination, and tumors, the cases presented here are the first reports of excessive yawning in patients with Chiari malformation Type I (CM-I). The authors believe that brainstem compression at the craniocervical junction and ensuing edema were implicated in this curious symptomatology. They describe excessive yawning as a presenting feature of CM-I in 2 adolescent females. The presentation was acute in the first case and more chronic in the second. Both patients underwent foramen magnum decompression, which resulted in complete cessation of the excessive yawning.

  3. Syringomyelia associated with arachnoid septum at the craniovertebral junction, contradicting the currently prevailing theory of syringomyelia formation.

    Science.gov (United States)

    Chang, Han Soo; Tsuchiya, Tsukasa; Fujisawa, Naoaki; Oya, Soichi; Matsui, Toru

    2012-01-01

    Despite a number of various hypotheses in the literature, the pathophysiology of syringomyelia is still not well understood. In this article, we report two cases of cervical syringomyelia not associated with Chiari I malformation. Both cases had a septum-like structure in the subarachnoid space on the dorsal side of the cord at the craniovertebral junction. Cardiac-gated phase-contrast cine-mode magnetic resonance imaging (MRI) demonstrated decreased cerebrospinal fluid (CSF) flow on the dorsal side of the spinal cord. Surgical excision of this septum, restoring the CSF flow, resulted in a prompt reduction of the syrinx size in both cases. Findings in these cases contradict the currently prevailing hypothesis of syrinx formation that postulate that the piston-like movement of the cerebellar tonsils enhance the pulsatile CSF flow in the spinal subarachnoid space, driving the CSF into the syrinx through the perivascular space of Virchow and Robin. The authors propose that a mechanism based on the decreased pulsatile CSF flow in the spinal subarachnoid space will be more suitable as a hypothesis in studying the pathophyisiology of syringomyelia. These cases also provide an important lesson in managing the patients with syringomyelia not associated with Chiari I malformation.

  4. Postoperative epidural hematoma contributes to delayed upper cord tethering after decompression of Chiari malformation type I

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    Antonio Lopez-Gonzalez

    2014-01-01

    Full Text Available Background: Symptomatic arachnoiditis after posterior fossa surgical procedures such as decompression of Chiari malformation is a possible complication. Clinical presentation is generally insidious and delayed by months or years. It causes disturbances in the normal flow of cerebrospinal fluid and enlargement of a syrinx cavity in the upper spinal cord. Surgical de-tethering has favorable results with progressive collapse of the syrinx and relief of the associated symptoms. Case Description: A 30-year-old male with Chiari malformation type I was treated by performing posterior fossa bone decompression, dura opening and closure with a suturable bovine pericardium dural graft. Postoperative period was uneventful until the fifth day in which the patient suffered intense headache and progressive loose of consciousness caused by an acute posterior fossa epidural hematoma. It was quickly removed with complete clinical recovering. One year later, the patient experienced progressive worsened of his symptoms. Upper spinal cord tethering was diagnosed and a new surgery for debridement was required. Conclusions: The epidural hematoma compressing the dural graft against the neural structures contributes to the upper spinal cord tethering and represents a nondescribed cause of postoperative fibrosis, adhesion formation, and subsequent recurrent hindbrain compression.

  5. Type I Chiari malformation presenting orthostatic syncope who treated with decompressive surgery

    Science.gov (United States)

    Shin, Hyun-Seung; Kim, Jeong A; Kim, Dong-Seok

    2016-01-01

    Chiari malformations are a congenital anomaly of the hindbrain. The most common, Chiari malformation type I (CM-I), is characterized by herniation of the cerebellar tonsils extending at least 3 mm below the plane of the foramen magnum. Consequently, CM-I is associated with hydrocephalus and symptoms involving compression of the cervicomedullary junction by ectopic tonsils. Several studies have reported the clinical symptoms associated with CM-I, including suboccipital headache, weakness in the upper extremities, facial numbness, loss of temperature sensation, ataxia, diplopia, dysarthria, dysphagia, vomiting, vertigo, nystagmus, and tinnitus. Syncope is one of the rarest presentations in patients with CM-I. There are many hypotheses regarding the causes of syncope in patients with CM-I; however, the mechanisms are not clearly understood. Although surgical decompression for CM-I in patients with syncope has yielded good clinical results in some studies, such cases are rarely reported. We report a case of orthostatic syncope in a patient with CM-I who was treated with surgical intervention. PMID:28018469

  6. The Association between Sleep-Disordered Breathing and Magnetic Resonance Imaging Findings in a Pediatric Cohort with Chiari 1 Malformation

    Directory of Open Access Journals (Sweden)

    Reshma Amin

    2015-01-01

    Full Text Available BACKGROUND: The prevalence of sleep-disordered breathing (SDB reported in the literature for Chiari malformation type 1 (CM1 is uniformly high (24% to 70%. In Canada, there is limited access to pediatric polysomnography (PSG. Therefore, the identification of clinical features would be invaluable for triaging these children.

  7. Health-related quality of life in pediatric Chiari Type I malformation: the Chiari Health Index for Pediatrics.

    Science.gov (United States)

    Ladner, Travis R; Westrick, Ashly C; Wellons, John C; Shannon, Chevis N

    2016-01-01

    OBJECT The purpose of this study was to design and validate a patient-reported health-related quality of life (HRQOL) instrument for pediatric Chiari Type I malformation (CM-I), the Chiari Health Index for Pediatrics (CHIP). METHODS The CHIP has 45 items with 4 components making up 2 domain scores, physical (pain frequency, pain severity, nonpain symptoms) and psychosocial; physical and psychosocial scores are combined to create an overall HRQOL score. Increasing scores (0 to 1) represent increasing HRQOL. Fifty-five patients with CM-I (mean age 12 ± 4 years, 53% male) were enrolled and completed the CHIP and Health Utilities Index Mark 3 (HUI3). Twenty-five healthy controls (mean age 11.9 ± 4 years, 40% male) also completed the CHIP. CHIP scores were compared between these groups via the Mann-Whitney U-test. For CHIP discriminative function, subscore versus presence of CM-I was compared via receiver operating characteristic curve analysis. CHIP scores in the CM-I group were stratified by symptomatology (asymptomatic, headaches, and paresthesias) and compared via Kruskal-Wallis test with Mann-Whitney U-test with Bonferroni correction (p Health Utilities Index Mark 3) via univariate and multivariate linear regression. RESULTS CHIP physical and psychosocial subscores were, respectively, 24% and 18% lower in CM-I patients than in controls (p HRQOL score was 23% lower as well (p related quality of life (R(2) = 0.311, p related (R(2) = 0.155, p = 0.003) quality of life. The AUC for CHIP HRQOL versus presence of CM-I was 0.820. Overall CHIP HRQOL score varied significantly with symptomatology (p = 0.001) and HUI3 multiattribute composite HRQOL score (R(2) = 0.440, p HRQOL instrument, with construct validity in assessing pain-, cognitive-, and emotion-related quality of life, as well as symptomatic features unique to CM-I. It holds promise as a discriminative HRQOL index in CM-I outcomes assessment.

  8. MRI diagnosis of syringomyelia. Including discussions on pathogenesis

    Energy Technology Data Exchange (ETDEWEB)

    Takahashi, Mutsumasa; Sakamoto, Yuji; Kojima, Ryutaro; Matsuno, Yasuji (Kumamoto Univ. (Japan). School of Medicine)

    1989-07-01

    Eighteen cases of syringomyelia were classified into communicating type (8 cases), traumatic type (5 cases) and tumor type (5 cases). Although there was similarity in the appearance of MRI regardless of the causes, there were some characteristics which were found more prevalently in one type of syringomyelia than the other. Traumatic syringomyelia involved longer segments, frequently entire length of the spinal cord. Chiari I malformation was more often associated with the communicating type, while the cystic appearance of syringomyelia was more frequently associated with the tumor type. Enlargement of the spinal cord was often seen with the tumor type, followed by traumatic type. T2-weighted images showed increased intensity in the area of the spinal cord trauma in all trauma cases, while tumor types also showed increased signal intensity within the tumor on T2-weighted images. Flow void sign of the syrinx was observed frequently in the communicating type. Although there was overlapping of the findings among syringomyelias of different causes, it was often possible to make differentiation among 3 types. When clinical and MR findings were considered together, MRI was one of the most important radiologic examinations in the evaluation of syringomyelia. (author).

  9. Chiari I畸形并脊髓空洞症86例外科治疗分析

    Institute of Scientific and Technical Information of China (English)

    寿记新; 王新军; 吴建珩

    2006-01-01

    Chiari I畸形(Chiari I malformation,CM I)是一种以小脑扁桃体向下疝入枕骨大孔为特征的先天性畸形,脊髓空洞症(syringomyelia,SM)为其最常见的合并症.我院1995-02~2004-05共收治86例Chiari I畸形合并脊髓空洞症,以不同方式进行手术,效果满意,现分析如下。

  10. Coordination function disorders and their evaluation in patients with Chiari malformation type 1

    Directory of Open Access Journals (Sweden)

    D N Dunin

    2012-01-01

    Full Text Available To study a range of coordination disorders in Chiari malformation type 1 (CM1, a clinical examination and magnetic resonance imaging (MRI were made in 73 patients (52 women and 21 men aged 17 to 71 years with this disorder. Special tests were used to identify cerebellar and vestibular disorders. Vertigo was noted in 76.7% of the patients with CM1. The special tests revealed impaired coordination in the majority (93.1% of the patients. Babinsky’s asynergy was the most common (52% of the patients symptom of coordination disorders. The findings confirmed the high rate of coordination disorders in CM1 and the informative value of supplementary studies of cerebellar and vestibular functions along with routine evaluation of the neurological status.

  11. Chiari Type I malformation yielded to the diagnosis of Crouzon syndrome

    Directory of Open Access Journals (Sweden)

    Aydin Canpolat

    2014-01-01

    Full Text Available Chiari malformation Type I (CM-I related to syndromic craniosynostosis in pediatric patients has been well-studied. The surgical management consists of cranial vault remodeling with or without posterior fossa decompression. There were also cases, in whom CM-I was diagnosed prior to the craniosynostosis in early childhood. We present a 16-year-old boy who admitted with symptoms related to CM-I. With careful examination and further genetic investigations, a diagnosis of Crouzon syndrome was made, of which the patient and his family was unaware before. The patient underwent surgery for posterior fossa decompression and followed-up for Crouzon′s syndrome. To our knowledge, this is the only case report indicating a late adolescent diagnosis of Crouzon syndrome through clinical symptoms of an associated CM-I.

  12. Chiari malformation and central sleep apnea syndrome: efficacy of treatment with adaptive servo-ventilation

    Directory of Open Access Journals (Sweden)

    Jorge Marques do Vale

    2014-10-01

    Full Text Available The Chiari malformation type I (CM-I has been associated with sleep-disordered breathing, especially central sleep apnea syndrome. We report the case of a 44-year-old female with CM-I who was referred to our sleep laboratory for suspected sleep apnea. The patient had undergone decompressive surgery 3 years prior. An arterial blood gas analysis showed hypercapnia. Polysomnography showed a respiratory disturbance index of 108 events/h, and all were central apnea events. Treatment with adaptive servo-ventilation was initiated, and central apnea was resolved. This report demonstrates the efficacy of servo-ventilation in the treatment of central sleep apnea syndrome associated with alveolar hypoventilation in a CM-I patient with a history of decompressive surgery.

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

    Science.gov (United States)

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

    2016-01-01

    Частота аномалии Киари (АК) в популяции составляет от 3 до 8 на 100 000 населения. В 62—80% наблюдений она сопровождается развитием сирингомиелии (СМ) на разных уровнях. Клиническая картина у этих пациентов складывается из проявлений АК и СМ, однако нередко на первый план выходят симптомы СМ, что создает определенные трудности в диагностике патологии и определении оптимальных методов лечения. Цель исследования — уточнить показания к хирургическому лечению СМ, ассоциированной с АК, определить оптимальный объем операции и критерии оценки ее результатов на основании собственного опыта хирургических вмешательств. Материал и методы. За период с 2011г. по февраль 2015 г. проведено обследование 225 пациентов с сочетанием СМ и АК 1-го типа, из них прооперированы 125 человек. Средний возраст оперированных составил 56±8 лет, средний срок от появления первых признаков заболевания до операции — 75±82 мес. Все операции выполнены одним хирургом. Операции проводились в положении пациента полусидя (89,6%) и лежа на животе (10,4%), заключались в экономной субокципитальной краниоэктомии, резекции дужки СI позвонка, восстановлении ликвородинамики по задней поверхности мозжечка, пластике твердой мозговой оболочки (ТМО) в области краниовертебрального перехода. Результаты. При ревизии арахноидальной оболочки большой затылочной цистерны после вскрытия ТМО у 78 (62,4%) больных арахнопатия отсутствовала (0-й тип, по Klekamp). Арахнопатия 1-го типа по Klekamp выявлена у 31 (24,8%) пациента, арахнопатия 2-го типа — у 16 (12,8%). Через 1 год после операции произведена оценка состояния 109 (88%) пациентов. У 61 (56%) пациента отмечен частичный или полный регресс предоперационной неврологической симптоматики, у 44 (40%) — заболевание перестало прогрессировать. У 4 (3,7%) пациентов заболевание обострилось. За время наблюдения рецидива нарушений ликвородинамики на краниовертебральном уровне не выявлено. Ранние послеоперационные осложнения развились у 4 (3,2%) пациентов: у 1 (0,8%) — раневая ликворея, у 1 (0,8%) — острая эпидуральная гематома, у 2 (1,6%) — асептический менингит. Преходящие ухудшения состояния (усиление головной боли, метеочувствительность) были выявлены у 11 (8,9%) пациентов. К концу 1-го месяца после операции данная симптоматика регрессировала. Летальных исходов не было. Выводы. Показанием к операции у больных с сочетанием АК и СМ служит наличие неврологической симптоматики, связанной с СМ, и ее прогрессирование, а также головная боль, обусловленная вклинением миндаликов мозжечка и существенно нарушающая качество жизни пациента. Основными критериями оценки эффективности проведенного лечения признаются стабилизация клинических симптомов и/или улучшение состояния больного. Субокципитальная краниоэктомия с последующей пластикой ТМО и восстановлением ликвородинамики в краниовертебральной области является эффективным методом лечения СМ, ассоциированной с АК 1-го типа.

  14. Chiari Malformation

    Science.gov (United States)

    ... Child What Kids Say About: Handling Stress Anxiety, Fears, and Phobias Community Service: A Family's Guide to ... neck, and spine) down through a funnel-like hole below the skull. This hole is called the ...

  15. Chiari Malformation

    Science.gov (United States)

    ... children who have neural tube defects. Some types cause no symptoms and don't need treatment. If you have symptoms, they may include Neck pain Balance problems Numbness or other abnormal feelings in ...

  16. 后颅窝小骨窗减压及硬脑膜成形术治疗Chiari畸形Ⅰ型45例疗效分析%Therapeutic effect analysis of small bone flap craniotomy decompression of posterior cranial fossa and duraplasty for 45 patients with Chiari malformation type Ⅰ

    Institute of Scientific and Technical Information of China (English)

    凌国源; 玉石; 黄锦丰; 陈文斗

    2014-01-01

    目的 探讨采用后颅窝小骨窗减压及硬脑膜成形术治疗Chiari畸形Ⅰ型的疗效.方法 回顾性分析45例Chiari畸形Ⅰ型患者行后颅窝小骨窗减压及硬脑膜成形术临床资料,其中合并脊髓空洞症31例.结果 按Tator等标准,术后1个月优30例,良15例.随访6个月至6年,其中优37例,良8例.31例合并脊髓空洞症患者中有26例脊髓空洞消退,5例脊髓空洞无明显变化.结论 后颅窝小骨窗减压及硬脑膜成形术可使颅颈交界区充分减压,并对脊髓空洞症有明显的治疗作用,是治疗Chiari畸形Ⅰ型安全有效的治疗方法.%Objective To evaluate the therapeutic effect of small bone flap craniotomy decompression of posterior cranial fossa and duraplasty in the treatment of Chiari malformation type Ⅰ.Methods The clinical data of 45 Chiari malformation type Ⅰ patients who were treated with small bone flap craniotomy decompression of posterior cranial fossa and duraplasty were retrospectively analyzed,31 cases among them with syringomyelia.Results According to Tator etc.standard,1 month after surgery,the excellent in 30 cases,good in 15 cases.Follow up from 6 months to 6 years,the excellent in 37 cases,good in 8 cases.Among 31 patients with syringomyelia,26 cases were syringomyelia subsided,5 cases were not obvious change.Conclusion The small bone flap craniotomy decompression of posterior cranial fossa and duraplasty can make the craniocervical decompression,and has obvious effect of treating syringomyelia,is safe and effective in treatment of Chiari malformation type Ⅰ.

  17. PROGRESSIVE SYRINGOHYDROMYELIA AND DEGENERATIVE AXONOPATHY IN A BOBCAT (LYNX RUFUS) FOLLOWING SURGICAL CORRECTION OF A CHIARI-LIKE MALFORMATION.

    Science.gov (United States)

    Sadler, Ryan; Schumacher, Juergen; Ramsay, Edward; McCleery, Brynn; Baine, Katherine; Thomas, William; Nobrega-Lee, Michelle; Henry, George A; Newman, Shelley J

    2016-03-01

    A 3-yr-old male captive bobcat (Lynx rufus) presented with chronic ataxia and right-sided head tilt. Magnetic resonance imaging (MRI) revealed cerebellar crowding and compression consistent with Chiari-like malformation. The clinical signs did not improve after surgical occipital craniectomy, and 2 mo postoperatively a second MRI showed hydromyelia and continued cerebellar compression. The bobcat was euthanized, and necropsy showed chronic focal cerebellar herniation and chronic multifocal atlanto-occipital joint osteophyte proliferation. Histology confirmed the presence of a thick fibrous membrane along the caudal aspect of the cerebellar vermis, suggestive of postoperative adhesions, and axonal degeneration of the cervical spinal cord, even in sections without a central canal lesion. These lesions appear to have been complications associated with surgical correction of the Chiari-like malformation.

  18. MRI tight posterior fossa sign for prenatal diagnosis of Chiari type II malformation

    Energy Technology Data Exchange (ETDEWEB)

    Ando, Kumiko; Ishikura, Reiichi; Ogawa, Masayo; Takada, Yoshihiro; Yamamoto, Satoshi; Fujiwara, Masayuki; Hirota, Shozo [Hyogo College of Medicine, Department of Radiology, Nishinomiya, Hyogo (Japan); Shakudo, Miyuki [Osaka City General Hospital, Department of Radiology, Osaka (Japan); Tanaka, Hiroyuki [Hyogo College of Medicine, Department of Gynecology, Nishinomiya (Japan); Minagawa, Kyoko [Hyogo College of Medicine, Department of Pediatrics, Nishinomiya (Japan)

    2007-12-15

    Chiari type II malformation (CMII) is one of three hindbrain malformations that display hydrocephalus. We have observed that cerebrospinal fluid (CSF) signal in the posterior fossa, which is always apparent on normal fetal MR images, is not visible in a fetus with CMII. We use the term 'tight posterior fossa' for this MR imaging finding, and evaluate the diagnostic value of this finding on fetal MR images. Included in the study were 21 fetuses which underwent brain MR imaging at 1.5 T using two-dimensional balanced turbo-field-echo (2-D balanced-TFE) in the axial and sagittal planes. Postnatal diagnoses were CMII (n=5), CNS abnormalities other than CMII (n=8), and no abnormality (n=8). A tight posterior fossa was defined as an absent or slit-like water signal space around the hindbrain in the posterior fossa on both sagittal and axial MR images. All CMII fetuses displayed a tight posterior fossa on MR images. Hydrocephalus was visualized in all CMII fetuses and myelomeningocele in four fetuses, but hindbrain herniation was visualized only in two of five fetuses. The CSF signal surrounding the hindbrain was clearly visible in all the other 16 fetuses, including five with hydrocephalus not associated with CMII, although it was slightly narrower in a fetus with a cloverleaf skull than in the normal fetuses. Tight posterior fossa in the presence of hydrocephalus is a useful and characteristic finding of CMII on fetal MRI. (orig.)

  19. Spontaneous resolution of syringomyelia in an adult patient with tight cisterna magna.

    Science.gov (United States)

    Perrini, Paolo

    2012-12-01

    Spontaneous resolution of syringomyelia in adult patients with Chiari malformation is exceptionally rare, with only 10 cases having been reported. A 21-year-old man working as a carpenter presented with a 1-year history of paresthesias in his right arm. A magnetic resonance imaging scan disclosed a cervicothoracic syrinx associated with tight tonsillar impaction of the cisterna magna without herniation. The patient left the carpentry job and underwent close monitoring with serial clinical and neuroradiological controls. The patient's symptoms gradually disappeared and magnetic resonance imaging studies revealed progressive shrinkage of the syrinx despite persistence of crowding of posterior fossa structures at the level of the foramen magnum. This case suggests that spontaneous resolution of syringomyelia can occasionally be triggered by the cessation of daily physical strain in patients with tight cisterna magna. Health care professionals should be aware that strenuous physical activities could affect the natural history of syringomyelia.

  20. Chiari畸形诊治的研究进展%Advancement of Chiari Malformation

    Institute of Scientific and Technical Information of China (English)

    彭逸龙; 伍益; 李智斌; 董家军; 钟鸣谷

    2013-01-01

    Chiari畸形又称小脑扁桃体下疝畸形,是一种先天性颅颈交界区畸形疾病,是以颅后窝容积缩小、小脑扁桃体向下进入椎管腔为主要病理学特征的先天性发育畸形.1891年奥地利病理学家Chiari首次详细介绍了菱脑畸形,定义脑积水、小脑扁桃体锥形楔状下疝,1896年补充报告14例并分为l、2型,认为是先天发育异常所致.1907年Julius Arnold补充为3、4型,并被命名为Arnold-Chiari malformation.本文对近年来Chiari畸形的相关研究综述如下.

  1. Infantile osteopetrosis, craniosynostosis, and Chiari malformation type I with novel OSTEM1 mutation

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    A H Mahmoud Adel

    2013-01-01

    Full Text Available We report a case of a 9-month-old Arab infant, with novel OSTEM mutation and unpublished triad of osteopetrosis (OP, craniosynostosis (CS, and Chiari malformation type I (CM1. The index presented with progressive irritability, abnormal movements, following an accidental fall. The history revealed early onset of irritability, progressive visual loss, and global developmental delay, more prominent at the gross motor level and a suspected congenital cytomegalovirus infection. The pregnancy was uneventful with subsequent unremarkable delivery. The parents are Arabs′first cousins with no apparent symptoms or signs of bone disease. Three dimensional brain computed tomography (CT showed ventriculomegaly, thick calvaria, and CS of the coronal and sagittal sutures. Patient had signs of left lower motor neuron facial palsy, and CT of petrous bones confirms the presence of osteopetrotic petrous with slim mastoid portions of the facial nerve canals both sides. Brain magnetic resonance imaging showed CM1. Skeletal survey showed sclerotic skeleton. He needed ventriculoperitoneal shunt and died at 18 months of age. Molecular testing for OSTEM1 gene revealed novel homozygous mutation that segregated from his parents. This novel OSTEM1 gene novel mutation and the combination of OP, infantile CS, and CM1 is to our knowledge never been reported.

  2. Suboccipital bony decompression combined with removal of the dural band as treatment for Chiari I malformation

    Institute of Scientific and Technical Information of China (English)

    周大彪; 赵继宗; 张东; 赵元立

    2004-01-01

    @@ Chiari I malformation (CMI) is a disorder involving hindbrain maldevelopments characterized by herniation of the cerebellar tonsils through the foramen magnum. The clinical presentations of CMI are related to the direct compression of the brainstem and cerebellum and to disturbances to cerebral spinal fluid (CSF) circulation. Surgical intervention is indicated in symptomatic patients with neuroradiological abnormalities. Though many different surgical options are available, suboccipital decompression has been widely accepted as a preferred procedure with or without additional manipulations.1-11 Nevertheless, any intradural procedures will risk related complications, including meningitis or arachnoiditis, CSF leakage, pseudomeningoceles, vascular injuries, brainstem dysfunction, as well as delayed neurological deterioration. Since the main benefit of surgery for CMI is to arrest the progression of the disease and stabilize the patient's neurological state,2,12 additional procedures seem to be unnecessary. To achieve acceptable therapeutic results for CMI, it is beneficial to perform a simple and effective procedure involving a minimal extent of surgery. The purpose of this article is to present an extradural technique for the modification of suboccipital decompression and to evaluate its efficacy based on clinical and neuroradiological results.

  3. Digenic mutations of human OCRL paralogs in Dent’s disease type 2 associated with Chiari I malformation

    Science.gov (United States)

    Duran, Daniel; Jin, Sheng Chih; DeSpenza, Tyrone; Nelson-Williams, Carol; Cogal, Andrea G; Abrash, Elizabeth W; Harris, Peter C; Lieske, John C; Shimshak, Serena JE; Mane, Shrikant; Bilguvar, Kaya; DiLuna, Michael L; Günel, Murat; Lifton, Richard P; Kahle, Kristopher T

    2016-01-01

    OCRL1 and its paralog INPP5B encode phosphatidylinositol 5-phosphatases that localize to the primary cilium and have roles in ciliogenesis. Mutations in OCRL1 cause the X-linked Dent disease type 2 (DD2; OMIM# 300555), characterized by low-molecular weight proteinuria, hypercalciuria, and the variable presence of cataracts, glaucoma and intellectual disability without structural brain anomalies. Disease-causing mutations in INPP5B have not been described in humans. Here, we report the case of an 11-year-old boy with short stature and an above-average IQ; severe proteinuria, hypercalciuria and osteopenia resulting in a vertebral compression fracture; and Chiari I malformation with cervico-thoracic syringohydromyelia requiring suboccipital decompression. Sequencing revealed a novel, de novo DD2-causing 462 bp deletion disrupting exon 3 of OCRL1 and a maternally inherited, extremely rare (ExAC allele frequency 8.4×10−6) damaging missense mutation in INPP5B (p.A51V). This mutation substitutes an evolutionarily conserved amino acid in the protein’s critical PH domain. In silico analyses of mutation impact predicted by SIFT, PolyPhen2, MetaSVM and CADD algorithms were all highly deleterious. Together, our findings report a novel association of DD2 with Chiari I malformation and syringohydromyelia, and document the effects of digenic mutation of human OCRL paralogs. These findings lend genetic support to the hypothesis that impaired ciliogenesis may contribute to the development of Chiari I malformation, and implicates OCRL-dependent PIP3 metabolism in this mechanism. PMID:28018608

  4. 枕骨大孔减压伴硬膜外层切开术在治疗Chiari畸形Ⅰ型中的应用%Application of Foramen Magnum Decompression with Removal of the Outer Layer of the Dura in Chiari Type I Malformation Treatment

    Institute of Scientific and Technical Information of China (English)

    马维宁; 李春; 张明杰; 李少一

    2012-01-01

    were researched of 45 patients diagnosed with Chiari I malformation, and then the patients were divided into two groups: group I of Chiari I malformation without syringomyeha (n - 17), and group II of Chiari I malformation with syringomyelia (n = 28). Further,clinical and MRI changes pre and post operation were compared. Results Main clinical symptoms of the 17 patients were obviously improved after the surgery, no obvious MRI changes after operation were found except for elevated tonsil of cerehellum and deepened cisterna magna, and no syringomyelia was developed. The other 28 patients with syringomyelia, whose clinical symptoms included limb numbness and anesthesia,also gained ideal improvement. Their syringomyelia was alleviated,and even disappeared during the follow-up, .meanwhile, discomforts like numbness,pain and sensation loss were relieved. However,symptoms such as myatrophy and sphincter disfunction were found no obvious change. Conclusion Foramen magnum decompression with removal of the outer layer of the dura for treatment of Chiari I malformation is effective. Chiari I malformation patients without syringomyelia can get greater improvement,and early operational treatment may reduce the incidence rate of syringomyelia in them.

  5. 婴儿Chiari畸形II型1例报告%Chiari II malformation in infant:a case report

    Institute of Scientific and Technical Information of China (English)

    胡凤娥

    2014-01-01

    目的:分析婴儿Chiari畸形Ⅱ型的临床资料,加深儿科医师的认识。方法回顾性分析1例确诊为Chiari畸形Ⅱ型患儿的临床、实验室、影像学资料,并复习相关文献。结果患儿,男,2个月,因颈部肿物2个月入院。颅脑MRI示小脑扁桃体部分疝入枕骨大孔。予手术治疗后一直间断发热,不能竖头、独坐,前囟门进行性增大,CT诊断脑积水。结论 Chi-ari畸形Ⅱ型会导致神经功能异常,预后差,病死率高;早期诊断,积极手术,可改善预后。%Objective To analyze the clinical data of an infant with Chiari II malformation so as to deepen understanding of this disease in pediatrics. Methods The clinical, laboratory and imaging data of an infant with Chiari II malformation were retrospectively analyzed and a literature review was performed. Results A 2-month-old male infant was admitted to our hospital due to a neck tumor for two months. Brain magnetic resonance imaging (MRI) showed herniation of the cerebellar tonsils into the foramen magnum. The infant was finally diagnosed with Chiari II malformation based on the result of MRI and the presence of myelomeningocele. After repair of spinal cord and meninges and decompression of pillow macroporous by surgery, the infant presented with intermittent fever and progressively enlarged anterior fontanel, with culture-positive cerebrospinal fluid for bacte-ria and hydrocephalus diagnosed by CT, and intracranial infection was considered. Ventriculoperitoneal shunt was not performed due to uncontrolled intracranial infection after treatment, and motor retardation was found during the follow-up. Conclusions Chiari II malformation may results in nervous system dysfunction with poor diagnosis and high case fatality rate. Early diagnosis and operation may be helpful to the improvement of prognosis.

  6. Chiari malformation type I: a case-control association study of 58 developmental genes.

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    Aintzane Urbizu

    Full Text Available Chiari malformation type I (CMI is a disorder characterized by hindbrain overcrowding into an underdeveloped posterior cranial fossa (PCF, often causing progressive neurological symptoms. The etiology of CMI remains unclear and is most likely multifactorial. A putative genetic contribution to CMI is suggested by familial aggregation and twin studies. Experimental models and human morphometric studies have suggested an underlying paraxial mesoderm insufficiency. We performed a case-control association study of 303 tag single nucleotide polymorphisms (SNP across 58 candidate genes involved in early paraxial mesoderm development in a sample of 415 CMI patients and 524 sex-matched controls. A subgroup of patients diagnosed with classical, small-PCF CMI by means of MRI-based PCF morphometry (n = 186, underwent additional analysis. The genes selected are involved in signalling gradients occurring during segmental patterning of the occipital somites (FGF8, Wnt, and retinoic acid pathways and from bone morphogenetic proteins or BMP, Notch, Cdx and Hox pathways or in placental angiogenesis, sclerotome development or CMI-associated syndromes. Single-marker analysis identified nominal associations with 18 SNPs in 14 genes (CDX1, FLT1, RARG, NKD2, MSGN1, RBPJ1, FGFR1, RDH10, NOG, RARA, LFNG, KDR, ALDH1A2, BMPR1A considering the whole CMI sample. None of these overcame corrections for multiple comparisons, in contrast with four SNPs in CDX1, FLT1 and ALDH1A2 in the classical CMI group. Multiple marker analysis identified a risk haplotype for classical CMI in ALDH1A2 and CDX1. Furthermore, we analyzed the possible contributions of the most significantly associated SNPs to different PCF morphometric traits. These findings suggest that common variants in genes involved in somitogenesis and fetal vascular development may confer susceptibility to CMI.

  7. Task-specific and general cognitive effects in Chiari malformation type I.

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    Philip A Allen

    Full Text Available OBJECTIVE: Our objective was to use episodic memory and executive function tests to determine whether or not Chiari Malformation Type I (CM patients experience cognitive dysfunction. BACKGROUND: CM is a neurological syndrome in which the cerebellum descends into the cervical spine causing neural compression, severe headaches, neck pain, and number of other physical symptoms. While primarily a disorder of the cervico-medullary junction, both clinicians and researchers have suspected deficits in higher-level cognitive function. DESIGN AND METHODS: We tested 24 CM patients who had undergone decompression neurosurgery and 24 age- and education-matched controls on measures of immediate and delayed episodic memory, as well as three measures of executive function. RESULTS: The CM group showed performance decrements relative to the controls in response inhibition (Stroop interference, working memory computational speed (Ospan, and processing speed (automated digit symbol substitution task, but group differences in recall did not reach statistical significance. After statistical control for depression and anxiety scores, the group effects for working memory and processing speed were eliminated, but not for response inhibition. This response inhibition difference was not due to overall general slowing for the CM group, either, because when controls' data were transformed using the linear function fit to all of the reaction time tasks, the interaction with group remained statistically significant. Furthermore, there was a multivariate group effect for all of the response time measures and immediate and delayed recall after statistical control of depression and anxiety scores. CONCLUSION: These results suggest that CM patients with decompression surgery exhibit cognitive dysfunction compared to age- and education-matched controls. While some of these results may be related to anxiety and depression (likely proxies for chronic pain, response inhibition

  8. Cerebrospinal fluid flow impedance is elevated in Type I Chiari malformation.

    Science.gov (United States)

    Shaffer, Nicholas; Martin, Bryn A; Rocque, Brandon; Madura, Casey; Wieben, Oliver; Iskandar, Bermans J; Dombrowski, Stephen; Luciano, Mark; Oshinski, John N; Loth, Francis

    2014-02-01

    Diagnosis of Type I Chiari malformation (CMI) is difficult because the most commonly used diagnostic criterion, cerebellar tonsillar herniation (CTH) greater than 3-5 mm past the foramen magnum, has been found to have little correlation with patient symptom severity. Thus, there is a need to identify new objective measurement(s) to help quantify CMI severity. This study investigated longitudinal impedance (LI) as a parameter to assess CMI in terms of impedance to cerebrospinal fluid motion near the craniovertebral junction. LI was assessed in CMI patients (N = 15) and age-matched healthy controls (N = 8) using computational fluid dynamics based on subject-specific magnetic resonance imaging (MRI) measurements of the cervical spinal subarachnoid space. In addition, CTH was measured for each subject. Mean LI in the CMI group (551 ± 66 dyn/cm5) was significantly higher than in controls (220 ± 17 dyn/cm5, p < 0.001). Mean CTH in the CMI group was 9.0 ± 1.1 mm compared to -0.4 ± 0.5 mm in controls. Regression analysis of LI versus CTH found a weak relationship (R2 = 0.46, p < 0.001), demonstrating that CTH was not a good indicator of the impedance to CSF motion caused by cerebellar herniation. These results showed that CSF flow impedance was elevated in CMI patients and that LI provides different information than a standard CTH measurement. Further research is necessary to determine if LI can be useful in CMI patient diagnosis.

  9. Task-Specific and General Cognitive Effects in Chiari Malformation Type I

    Science.gov (United States)

    Allen, Philip A.; Houston, James R.; Pollock, Joshua W.; Buzzelli, Christopher; Li, Xuan; Harrington, A. Katherine; Martin, Bryn A.; Loth, Francis; Lien, Mei-Ching; Maleki, Jahangir; Luciano, Mark G.

    2014-01-01

    Objective Our objective was to use episodic memory and executive function tests to determine whether or not Chiari Malformation Type I (CM) patients experience cognitive dysfunction. Background CM is a neurological syndrome in which the cerebellum descends into the cervical spine causing neural compression, severe headaches, neck pain, and number of other physical symptoms. While primarily a disorder of the cervico-medullary junction, both clinicians and researchers have suspected deficits in higher-level cognitive function. Design and Methods We tested 24 CM patients who had undergone decompression neurosurgery and 24 age- and education-matched controls on measures of immediate and delayed episodic memory, as well as three measures of executive function. Results The CM group showed performance decrements relative to the controls in response inhibition (Stroop interference), working memory computational speed (Ospan), and processing speed (automated digit symbol substitution task), but group differences in recall did not reach statistical significance. After statistical control for depression and anxiety scores, the group effects for working memory and processing speed were eliminated, but not for response inhibition. This response inhibition difference was not due to overall general slowing for the CM group, either, because when controls' data were transformed using the linear function fit to all of the reaction time tasks, the interaction with group remained statistically significant. Furthermore, there was a multivariate group effect for all of the response time measures and immediate and delayed recall after statistical control of depression and anxiety scores. Conclusion These results suggest that CM patients with decompression surgery exhibit cognitive dysfunction compared to age- and education-matched controls. While some of these results may be related to anxiety and depression (likely proxies for chronic pain), response inhibition effects, in particular

  10. The association between Chiari malformation Type I, spinal syrinx, and scoliosis.

    Science.gov (United States)

    Strahle, Jennifer; Smith, Brandon W; Martinez, Melaine; Bapuraj, J Rajiv; Muraszko, Karin M; Garton, Hugh J L; Maher, Cormac O

    2015-06-01

    OBJECT Chiari malformation Type I (CM-I) is often found in patients with scoliosis. Most previous reports of CM-I and scoliosis have focused on patients with CM-I and a spinal syrinx. The relationship between CM-I and scoliosis in the absence of a syrinx has never been defined clearly. The authors sought to determine if there is an independent association between CM-I and scoliosis when controlling for syrinx status. METHODS The medical records of 14,118 consecutive patients aged ≤ 18 years who underwent brain or cervical spine MRI at a single institution in an 11-year span were reviewed to identify patients with CM-I, scoliosis, and/or syrinx. The relationship between CM-I and scoliosis was analyzed by using multivariate regression analysis and controlling for age, sex, CM-I status, and syrinx status. RESULTS In this cohort, 509 patients had CM-I, 1740 patients had scoliosis, and 243 patients had a spinal syrinx. The presence of CM-I, the presence of syrinx, older age, and female sex were each significantly associated with scoliosis in the univariate analysis. In the multivariate regression analysis, older age (OR 1.02 [95% CI 1.01-1.03]; p scoliosis. CM-I was not independently associated with scoliosis when controlling for these other variables (OR 0.99 [95% CI 0.79-1.29]; p = 0.9). CONCLUSIONS A syrinx was independently associated with scoliosis in a large pediatric population undergoing MRI. CM-I was not independently associated with scoliosis when controlling for age, sex, and syrinx status. Because CM-I is not independently associated with scoliosis, scoliosis should not necessarily be considered a symptom of low cerebellar tonsil position in patients without a syrinx.

  11. Sudden unexpected nocturnal death in Chiari type 1 malformation and potential role of opioid analgesics

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    Fereydoon Roohi

    2014-01-01

    Full Text Available Background: Chiari malformation type 1 (CM1 is a common congenital anomaly of the craniocervical junction. CM1 is reported to run a usually benign course and patients typically experience no symptoms or chronic, slowly progressive symptoms. However, recent reports indicate that a subset of patients with CM1 may present with acute deterioration and sudden unexpected death (SUD. We report a case of SUD during sleep in a young man with CM1, which we believe was related to the administration of common and therapeutic doses of narcotic analgesics for the management of pain. We will clarify the pathophysiology of acute deterioration and SUD in CM1 and the possibility that the adverse effects of opiate analgesics likely were the leading cause of death in our patient. Case Description: In this review, we present a 29-year-old male with worsening headache secondary to previously diagnosed CM1. The patient died suddenly and unexpectedly after administration of common and therapeutic doses of narcotic analgesics for the management of pain. Conclusion: The mechanism(s of acute neurological deterioration and sudden death in patients with CM1 remains poorly understood. We believe the rapid fatal deterioration in our patient following administration of opioids suggests that this category of medication may cause sudden unexpected "neurogenic" cardiac death in CM1 patients by inducing sleep-related breathing difficulties and associated hypercapnia. Hypercapnia by further increasing intracranial pressure can result in a sudden pressure-induced decompensation of the cardiopulmonary control centers in the brain stem and cause instantaneous cardiorespiratory arrest.

  12. Sudden unexpected nocturnal death in Chiari type 1 malformation and potential role of opioid analgesics

    Science.gov (United States)

    Roohi, Fereydoon; Gropen, Toby; Kula, Roger W.

    2014-01-01

    Background: Chiari malformation type 1 (CM1) is a common congenital anomaly of the craniocervical junction. CM1 is reported to run a usually benign course and patients typically experience no symptoms or chronic, slowly progressive symptoms. However, recent reports indicate that a subset of patients with CM1 may present with acute deterioration and sudden unexpected death (SUD). We report a case of SUD during sleep in a young man with CM1, which we believe was related to the administration of common and therapeutic doses of narcotic analgesics for the management of pain. We will clarify the pathophysiology of acute deterioration and SUD in CM1 and the possibility that the adverse effects of opiate analgesics likely were the leading cause of death in our patient. Case Description: In this review, we present a 29-year-old male with worsening headache secondary to previously diagnosed CM1. The patient died suddenly and unexpectedly after administration of common and therapeutic doses of narcotic analgesics for the management of pain. Conclusion: The mechanism(s) of acute neurological deterioration and sudden death in patients with CM1 remains poorly understood. We believe the rapid fatal deterioration in our patient following administration of opioids suggests that this category of medication may cause sudden unexpected “neurogenic” cardiac death in CM1 patients by inducing sleep-related breathing difficulties and associated hypercapnia. Hypercapnia by further increasing intracranial pressure can result in a sudden pressure-induced decompensation of the cardiopulmonary control centers in the brain stem and cause instantaneous cardiorespiratory arrest. PMID:24778905

  13. C1 arch regeneration, tight cisterna magna, and cervical syringomyelia following foramen magnum surgery.

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    Rahme, Ralph; Koussa, Salam; Samaha, Elie

    2009-07-01

    Although bone regrowth following craniocervical decompression has been rarely reported to cause late recurrence of Chiari symptoms, syringomyelia has not been observed in such cases. We report a unique case of cervical syringomyelia resulting from spontaneous regeneration of the posterior C1 arch after foramen magnum decompression. A 38-year-old male patient underwent resection of a symptomatic foramen magnum meningioma. Three years later, he developed neuropathic pain in his left upper extremity with worsening dysphagia and dysphonia. MRI revealed regeneration of the posterior arch of C1 with tight tonsillar impaction of the foramen magnum and extensive cervical syringomyelia. Surgical exploration was undertaken. Neo-ossification of the posterior arch of C1 and thick arachnoid adhesions were found to obstruct cerebrospinal fluid flow through the foramen of Magendie. Foramen magnum decompression, arachnoid dissection, and duraplasty were thus performed and cerebrospinal fluid flow was reestablished through the foramen of Magendie. Postoperatively, patient's symptoms improved dramatically and repeat MRI showed complete resolution of the syrinx cavity. Spontaneous bone regrowth and arachnoid scarring may lead to the development of cervical syringomyelia several years after foramen magnum surgery. Neurosurgeons should be aware of this rare complication whose management is similar to that of Chiari malformations, namely craniocervical decompression and establishment of a patent foramen of Magendie.

  14. Combined Spinal-Epidural Analgesia for Laboring Parturient with Arnold-Chiari Type I Malformation: A Case Report and a Review of the Literature

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    Clark K. Choi

    2013-01-01

    Full Text Available Anesthetic management of laboring parturients with Arnold-Chiari type I malformation poses a difficult challenge for the anesthesiologist. The increase in intracranial pressure during uterine contractions, coughing, valsalva maneuvers, and expulsion of the fetus can be detrimental to the mother during the process of labor and delivery. No concrete evidence has implicated high cerebral spinal fluid pressure on maternal and fetal complications. The literature on the use of neuraxial techniques for managing parturients with Arnold-Chiari is extremely scarce. While most anesthesiologists advocate epidural analgesia for management of labor pain and spinal anesthesia for cesarean section, we are the first to report the use of combined spinal-epidural analgesia for managing labor pain in a pregnant woman with Arnold-Chiari type I malformation. Also, we have reviewed the literature and presented information from case reports and case series to support the safe usage of neuraxial techniques in these patients.

  15. Patient-reported Chiari malformation type I symptoms and diagnostic experiences: a report from the national Conquer Chiari Patient Registry database.

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    Fischbein, Rebecca; Saling, Julia R; Marty, Paige; Kropp, Denise; Meeker, James; Amerine, Jenna; Chyatte, Michelle Renee

    2015-09-01

    Chiari malformation (CM) is a condition in which cerebellar tonsillar ectopia may manifest with various clinical presentations. This study reports from the only national, online patient registry available, the symptoms, comorbid neurocognitive and psychological conditions, and diagnostic experiences of patients living with CM type I (CM I). The current research is one component of a large investigation designed to collect information from individuals with CM through the online Conquer Chiari Patient Registry questionnaire. Analyses included descriptive statistics to study body system impact and patient diagnostic experiences. Participants were 768 individuals with CM I and were predominantly female (86.8 %) and Caucasian (93.8 %) with an average age of 35 years. Pain was the most frequently reported symptom (76.69 %) experienced prior to diagnosis with headaches implicated most often (73.44 %). Neurocognitive comorbidities included memory difficulties (43.88 %) and aphasia (43.75 %) and psychological disorders such as depression (31.77 %) and anxiety disorders (19.92 %) were reported. Average time to diagnosis from first physician visit to diagnosis was 3.43 years, and only 8.46 % of patients had previous awareness of CM. CM I diagnosis was found incidentally for 24.87 % of participants. Common misdiagnoses were classified as psychological (19.26 %) and neurological (19.26 %). Fear was the most frequent emotion elicited at the time of correct diagnosis (42.19 %). CM I can be a challenging condition for patients and physicians, during both the search for diagnosis and management of symptoms. Patient and physician education about CM I may permit early intervention and the prevention of further deterioration and patient suffering.

  16. Unusual Ventilatory Response to Exercise in Patient with Arnold-Chiari Type 1 Malformation after Posterior Fossa Decompression

    Science.gov (United States)

    Smith, Keely; Gomez-Rubio, Ana M.; Harris, Tomika S.; Brooks, Lauren E.

    2016-01-01

    We present a case of a 17-year-old Hispanic male with Arnold-Chiari Type 1 [AC-Type 1] with syringomyelia, status post decompression, who complains of exercise intolerance, headaches, and fatigue with exertion. The patient was found to have diurnal hypercapnia and nocturnal alveolar hypoventilation. Cardiopulmonary testing revealed blunting of the ventilatory response to the rise in carbon dioxide (CO2) resulting in failure of the parallel correlation between increased CO2 levels and ventilation; the expected vertical relationship between PETCO2 and minute ventilation during exercise was replaced with an almost horizontal relationship. No new pathology of the brainstem was discovered by MRI or neurological evaluation to explain this phenomenon. The patient was placed on continuous noninvasive open ventilation (NIOV) during the day and CPAP at night for a period of 6 months. His pCO2 level decreased to normal limits and his symptoms improved; specifically, he experienced less headaches and fatigue during exercise. In this report, we describe the abnormal response to exercise that patients with AC-Type 1 could potentially experience, even after decompression, characterized by the impairment of ventilator response to hypercapnia during exertion, reflecting a complete loss of chemical influence on breathing with no evidence of abnormality in the corticospinal pathway. PMID:27418995

  17. Unusual Ventilatory Response to Exercise in Patient with Arnold-Chiari Type 1 Malformation after Posterior Fossa Decompression

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    Keely Smith

    2016-01-01

    Full Text Available We present a case of a 17-year-old Hispanic male with Arnold-Chiari Type 1 [AC-Type 1] with syringomyelia, status post decompression, who complains of exercise intolerance, headaches, and fatigue with exertion. The patient was found to have diurnal hypercapnia and nocturnal alveolar hypoventilation. Cardiopulmonary testing revealed blunting of the ventilatory response to the rise in carbon dioxide (CO2 resulting in failure of the parallel correlation between increased CO2 levels and ventilation; the expected vertical relationship between PETCO2 and minute ventilation during exercise was replaced with an almost horizontal relationship. No new pathology of the brainstem was discovered by MRI or neurological evaluation to explain this phenomenon. The patient was placed on continuous noninvasive open ventilation (NIOV during the day and CPAP at night for a period of 6 months. His pCO2 level decreased to normal limits and his symptoms improved; specifically, he experienced less headaches and fatigue during exercise. In this report, we describe the abnormal response to exercise that patients with AC-Type 1 could potentially experience, even after decompression, characterized by the impairment of ventilator response to hypercapnia during exertion, reflecting a complete loss of chemical influence on breathing with no evidence of abnormality in the corticospinal pathway.

  18. Multimodal evaluation of CSF dynamics following extradural decompression for Chiari malformation Type I.

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    Quon, Jennifer L; Grant, Ryan A; DiLuna, Michael L

    2015-06-01

    OBJECT Extradural decompression is a minimally invasive technique for treating Chiari malformation Type I (CM-I) that avoids the complications of dural opening. While there is no agreement on which surgical method is optimal, mounting evidence demonstrates that extradural decompression effectively treats clinical symptoms, with a minimal reoperation rate. Neurological symptoms such as headache may be related to obstructed flow of CSF, and one aspect of successful extradural decompression is improved CSF dynamics. In this series, the authors report on their use of phase-contrast cine flow MRI to assess CSF flow as well as satisfactory decompression. METHODS The authors describe their first surgical series of 18 patients with CM-I undergoing extradural decompression and correlate clinical improvement with radiological changes. Patients were categorized as having complete, partial, or no resolution of their symptoms. Posterior fossa area, cisterna magna area, and tonsillar herniation were assessed on T2-weighted MRI, whereas improvement of CSF flow was evaluated with phase-contrast cine flow MRI. All patients received standard pre- and postoperative MRI studies; 8 (44.4%) patients had pre- and postoperative phase-contrast cine, while the rest underwent cine studies only postoperatively. RESULTS All 18 patients presented with symptomatic CM-I, with imaging studies demonstrating tonsillar herniation ≥ 5 mm, and 2 patients had associated syringomelia. All patients underwent suboccipital decompression and C-1 laminectomy with splitting of the dura. Patients with complete resolution of their symptoms had a greater relative increase in cisterna magna area compared with those with only partial improvement (p = 0.022). In addition, in those with complete improvement the preoperative cisterna magna area was smaller than in those who had either partial (0.020) or no (0.025) improvement. Ten (91%) of the 11 patients with improved flow also had improvement in their symptoms

  19. Computed tomographic study of posterior cranial fossa, foramen magnum, and its surgical implications in Chiari malformations.

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    Iqbal, Showkathali; Robert, Ambooken P; Mathew, Dominic

    2017-01-01

    The aim of this study is to measure the height and volume of the bony part of the posterior cranial fossa (PCF) and the surface area of the foramen magnum (FM) using computed tomography (CT) scans and to correlate our clinical findings with the available current literature. This cross-sectional study was conducted in a tertiary care referral hospital in the Southern part of India during the period from January 2015 to August 2015. A total of 100 CT scans of the suspected head injury patients were collected retrospectively form the basis for this study. The height, volume of PCF and the anteroposterior (AP), transverse diameter, and surface area of the FM were measured. The values of all parameters were subjected to statistical analysis using SPSS version 16. The age of the patients were ranged between 18 and 70 years with the mean age of 41.22 ± 13.93 years. The dimensions of the posterior fossa and FM were larger in males compared to females. The mean height of the posterior fossa was 38.08 (±4.718) mm (P = 0.0001), and the mean volume of the posterior fossa was 157.23 (±6.700) mm(3) (P = 0.0001). The mean AP, transverse diameter, and the surface area of the FM were 33.13 (± 3.286) mm, 29.01 (± 3.081) mm, and 763.803 (±138.276) mm(2), respectively. The normal dimensions of the posterior fossa and FM were less in females than males and were useful to radiologists and neurosurgeons to better their diagnostic inferences, as well as to determine the proper treatment options in Chiari malformation type I (CMI) and other posterior fossa anomalies. The posterior fossa tissue volume can be reliably measured in patients with CMI using our method. More studies were required because there were variations in dimensions among individuals of different races in different regions of the world.

  20. A prospective natural history study of nonoperatively managed Chiari I malformation: does follow-up MRI surveillance alter surgical decision making?

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    Whitson, Wesley J; Lane, Jessica R; Bauer, David F; Durham, Susan R

    2015-08-01

    OBJECT Chiari malformation Type I (CM-I) in children is a common incidental finding. Resolution of cerebellar tonsil ectopia has been reported, but no studies have followed tonsil position over regular intervals throughout childhood. To better elucidate the clinical and radiological natural history of CM-I in children, the authors prospectively followed up children with nonoperatively managed CM-I for up to 7 years. METHODS The study included all children evaluated for CM-I over a period of 12 years for whom surgery was not initially recommended. The study excluded patients with associated conditions, including syringomyelia and hydrocephalus. For all patients, initial management was nonoperative, and follow-up management consisted of annual cervical spine or brain MRI and clinical examination. At each follow-up examination, the neurological examination findings, subjective symptoms, and the position of the cerebellar tonsils on MR images were recorded. An alteration in tonsillar descent of 2 mm or greater was considered a change. RESULTS Neurological examination findings did not change over the course of the study in the 52 children who met the inclusion criteria. Although radiological changes were common, no surgeries were performed solely because of radiological change. Overall, tonsil position on radiological images remained stable in 50% of patients, was reduced in 38%, and increased in 12%. Resolution was seen in 12% of patients. Radiological changes in tonsil position were seen during every year of follow-up. On average, in any given year, 24% of images showed some form of change in tonsil position. A total of 3 patients, for whom no changes were seen on MR images, ultimately underwent surgery for subjective clinical reasons. CONCLUSIONS CM-I in children is not a radiologically static entity but rather is a dynamic one. Radiological changes were seen throughout the 7 years of follow-up. A reduction in tonsillar descent was substantially more common than an

  1. Syringomyelia secondary to "occult" dorsal arachnoid webs: Report of two cases with review of literature

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    Parag P Sayal

    2016-01-01

    Full Text Available In a certain group of patients with syringomyelia, even with the advent of sophisticated magnetic resonance imaging (MRI, no associated abnormality or cerebrospinal fluid (CSF block is easily identified. This type of syringomyelia is often termed idiopathic. Current literature has less than 10 reports of arachnoid webs to be the causative factor. We present our experience in the management of two cases of syringomyelia secondary to arachnoid webs. Both our patients presented with progressive neurological deterioration with MRI scans demonstrating cervical/thoracic syrinx without Chiari malformation or low-lying cord. There was no history of previous meningitis or trauma. Both patients underwent myelography that demonstrated dorsal flow block implying CSF obstruction. Cord displacement/change in caliber was also noted and this was not evident on MRI scans. Both patients underwent thoracic laminectomy. After opening the dura, thickened/abnormal arachnoid tissue was found that was resected thus widely communicating the dorsal subarachnoid space. Postoperatively at 6 months, both patients had significant symptomatic improvement with follow-up MRI scans demonstrating significant resolution of the syrinx. In patients with presumed idiopathic syringomyelia, imaging studies should be closely inspected for the presence of a transverse arachnoid web. We believe that all patients with idiopathic symptomatic syringomyelia should have MRI CSF flow studies and/or computed tomography (CT myelography to identify such arachnoid abnormalities that are often underdiagnosed. Subsequent surgery should be directed at the establishment of normal CSF flow by laminectomy and excision of the offending arachnoid tissue.

  2. Syringomyelia secondary to "occult" dorsal arachnoid webs: Report of two cases with review of literature.

    Science.gov (United States)

    Sayal, Parag P; Zafar, Arif; Carroll, Thomas A

    2016-01-01

    In a certain group of patients with syringomyelia, even with the advent of sophisticated magnetic resonance imaging (MRI), no associated abnormality or cerebrospinal fluid (CSF) block is easily identified. This type of syringomyelia is often termed idiopathic. Current literature has less than 10 reports of arachnoid webs to be the causative factor. We present our experience in the management of two cases of syringomyelia secondary to arachnoid webs. Both our patients presented with progressive neurological deterioration with MRI scans demonstrating cervical/thoracic syrinx without Chiari malformation or low-lying cord. There was no history of previous meningitis or trauma. Both patients underwent myelography that demonstrated dorsal flow block implying CSF obstruction. Cord displacement/change in caliber was also noted and this was not evident on MRI scans. Both patients underwent thoracic laminectomy. After opening the dura, thickened/abnormal arachnoid tissue was found that was resected thus widely communicating the dorsal subarachnoid space. Postoperatively at 6 months, both patients had significant symptomatic improvement with follow-up MRI scans demonstrating significant resolution of the syrinx. In patients with presumed idiopathic syringomyelia, imaging studies should be closely inspected for the presence of a transverse arachnoid web. We believe that all patients with idiopathic symptomatic syringomyelia should have MRI CSF flow studies and/or computed tomography (CT) myelography to identify such arachnoid abnormalities that are often underdiagnosed. Subsequent surgery should be directed at the establishment of normal CSF flow by laminectomy and excision of the offending arachnoid tissue.

  3. 弥漫性颅骨肥厚致获得性Chiari Ⅰ畸形一例并文献复习%Acquired Chiarimalformation secondary to generalized hyperostotic skull: a case report and literature review

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    尹绍雅; 陈祎阳; 杨国军; 王宏

    2011-01-01

    目的 探讨弥漫性颅骨肥厚致获得性Chiari Ⅰ畸形个体化治疗方案及机制.方法 报告1例弥漫性颅骨增厚致颅内压增高和小脑扁桃体下疝,并复习相关文献.结果 颅骨肥厚造成颅内容积减小,部分患者可合并小脑扁桃体下疝,可继发于Camurati - Engelmann病、颅骨-骨干发育不良、Worth - type骨内膜肥厚和硬化性骨化病等.作者所报告病例即属于常染色体显性遗传性骨硬化症.治疗上宜采用幕上开颅减压术,而禁忌采用单纯枕下减压术.结论 弥漫性颅骨肥厚引起获得性ChiariⅠ畸形很少见,治疗上应行幕上开颅减压术.%Objective Acquired Chiarimalformation associated with generalized hyperostotic skull is a rare condition.The management of this condition is far more different from congenital and other kind of acquired Chiarimalformation.This study tried to establish an individual management plan for these patients.Method A 22 - year old female patient was admitted for headache and papilledema.Generalized hyperostotic skull and secondary Chiarimalformation were found after examination.This case was reported and related literatures were reviewed.Results Most cases of generalized hyperostosis of skull are associated with Camurati - Engelmann disease,craniodiaphyseal dysplasia,Worth - type endosteal hyperostosis,sclerosteosis,or other conditions.Infrequently,a Chiari malformation may also be described.This patient was a Worth - type endosteal hyperostosis and the surgical approach of subtemporal decompression was taken.Conclusions Acquired Chiarimalformation is rarely associated with hyperostosis of skull.For a patient with symptom,a procedure of subtemporal decompression should be taken and suboccipital approach should be prohibitive.

  4. Factors associated with spinal fusion after posterior fossa decompression in pediatric patients with Chiari I malformation and scoliosis.

    Science.gov (United States)

    Mackel, Charles E; Cahill, Patrick J; Roguski, Marie; Samdani, Amer F; Sugrue, Patrick A; Kawakami, Noriaki; Sturm, Peter F; Pahys, Joshua M; Betz, Randal R; El-Hawary, Ron; Hwang, Steven W

    2016-12-01

    OBJECTIVE The authors performed a study to identify clinical characteristics of pediatric patients diagnosed with Chiari I malformation and scoliosis associated with a need for spinal fusion after posterior fossa decompression when managing the scoliotic curve. METHODS The authors conducted a multicenter retrospective review of 44 patients, aged 18 years or younger, diagnosed with Chiari I malformation and scoliosis who underwent posterior fossa decompression from 2000 to 2010. The outcome of interest was the need for spinal fusion after decompression. RESULTS Overall, 18 patients (40%) underwent posterior fossa decompression alone, and 26 patients (60%) required a spinal fusion after the decompression. The mean Cobb angle at presentation and the proportion of patients with curves > 35° differed between the decompression-only and fusion cohorts (30.7° ± 11.8° vs 52.1° ± 26.3°, p = 0.002; 5 of 18 vs 17 of 26, p = 0.031). An odds ratio of 1.0625 favoring a need for fusion was established for each 1° of increase in Cobb angle (p = 0.012, OR 1.0625, 95% CI 1.0135-1.1138). Among the 14 patients older than 10 years of age with a primary Cobb angle exceeding 35°, 13 (93%) ultimately required fusion. Patients with at least 1 year of follow-up whose curves progressed more 10° after decompression were younger than those without curve progression (6.1 ± 3.0 years vs 13.7 ± 3.2 years, p = 0.001, Mann-Whitney U-test). Left apical thoracic curves constituted a higher proportion of curves in the decompression-only group (8 of 16 vs 1 of 21, p = 0.002). CONCLUSIONS The need for fusion after posterior fossa decompression reflected the curve severity at clinical presentation. Patients presenting with curves measuring > 35°, as well as those greater than 10 years of age, may be at greater risk for requiring fusion after posterior fossa decompression, while patients less than 10 years of age may require routine monitoring for curve progression. Left apical thoracic curves

  5. Factors of Dystrophia in Patients with Chiari Malformations Type I%小脑扁桃体下疝畸形患者营养障碍相关因素分析

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    苏丞; 更·党木仁加甫; 朱国华; 麦麦提力

    2016-01-01

    Objective To discuss the situation of dystrophia in patients with Chiari Malformation Type I. Methods A total of 93 patients diagnosed with Chiari Malformation Type I(observational group)with or without syringomyelia and without basilar invagination by a combined head and cervical spine MRI were selected,and divided into syringomyelia group(with syringomyelia,n=62)and single group(without syringomyelia,n=31);100 healthy people with same age and gender were selected as control group and the age,height,weight,biceps circumference, waist circumference,hip circumference and hemoglobin of observational group and control group were recorded and compared respectively,and the nutritional status were compared after grouped by gender,and finally a comparison between syringomyelia group and single group was conducted.Results The BMI,biceps circumference,waist circumference,hip circumference,hemoglobin of patients in observational group were markedly lower than those of control group,the difference of which was statistically significant(P <0.05).And significant differences in BMI, biceps circumference,waist circumference,hip circumference,hemoglobin were found between single group and syringomyelia group(P < 0.05 ).The BMI,biceps circumference,waist circumference,hip circumference in male CMI patients were lower than those in female CMI patients(P < 0.05 ).And all of the indexes of both male and female CMI patients were lower than those of the healthy patients of the same age and gender(P <0.05).Conclusion Dystrophia is apparent in CMI patients and there are differences among patients of different genders and patients with or without syringomyelia.The improvement in dystrophia may benefit the recovery of CMI.%目的:探讨小脑扁桃体下疝畸形患者营养障碍的情况。方法选取经头颈部 MRI 确诊为小脑扁桃体下疝畸形,伴或不伴脊髓空洞症状且排除颅底凹陷症的患者93例(观察组),其中伴发脊髓空洞的小

  6. Dimensions of the posterior fossa in patients symptomatic for Chiari I malformation but without cerebellar tonsillar descent

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    Sekula L Kathleen

    2005-12-01

    Full Text Available Abstract Background Chiari I malformation (CMI is diagnosed by rigid radiographic criteria along with appropriate clinical symptomatology. The aim of this study was to investigate the dimensions of the posterior cranial fossa in patients without significant tonsillar descent but with symptoms comparable to CMI. Methods Twenty-two patients with signs and symptoms comparable to CMI but without accepted radiographic criteria of tonsillar descent > 3–5 mm were referred to our clinic for evaluation. A history and physical examination were performed on all patients. In reviewing their MRI scans, nine morphometric measurements were recorded. The measurements were compared to measurements from a cohort of twenty-five individuals with cranial neuralgias from our practice. Results For patients with Chiari-like symptomatology, the following statistically significant abnormalities were identified: reduced length of the clivus, reduced length of basisphenoid, reduced length of basiocciput, and increased angle of the tentorium. Multiple morphometric studies have demonstrated similar findings in CMI. Conclusion The current classification of CMI is likely too restrictive. Preliminary morphologic data suggests that a subgroup of patients exists with tonsillar descent less than 3 mm below the foramen magnum but with congenitally hypoplastic posterior fossa causing symptomatology consistent with CMI.

  7. Treatment of Chiari malformation with craniocervical junction malformation%Chiari畸形合并颅颈交界畸形的处理策略

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    陈利锋; 余新光; 周定标; 卜博; 张远征; 许百男

    2011-01-01

    目的 探讨Chiari畸形合并颅颈交界畸形的临床特点及治疗方法.方法 回顾性分析56例Chiari畸形合并颅颈交界畸形的临床资料,其中合并颅底陷入35例,寰枕融合25例,颈椎分节不全6例.病人均行颅后窝减压硬脑膜扩大修补术,行小脑扁桃体部分切除术21例.固定方式采用钛缆固定和自体骨移植41例,C侧块~C椎弓根螺钉固定和自体骨移植15例.结果 术后发生颅内感染2例,脑脊液切口漏1例.55例随访6~60个月,平均42个月;植骨融合满意52例,钛缆固定和自体骨移植植骨未能融合3例;症状改善51例(92.7%),无变化4例(7.3%);随访期间无死亡病例.结论 Chiari畸形合并颅颈交界畸形应行颅颈交界关节稳定性检查及评估,对有潜在颅颈交界不稳定病人应行后路减压并枕颈植骨固定融合术,单纯后路减压可能加重颅颈交界不稳定,症状难以缓解甚至加重.%Objective To investigate the clinical features and treatment of Chiari malformation complicated by craniocervical junction malformation. Methods Clinical data of 56 patients with Chiari malformation complicated by craniocervical junction malformation were retrospectively analyzed, including by basilar invagination in 35, by atlas assimilation in 25, by Klippel-Feil anomaly in 6. Posterior fossa decompression with expanded dural repair was performed in all the patients, including additional partial resection of the cerebellar tonsil in 21. Fixation with titanium cables and autologous bone transplantation were performed in 41 cases, fixation with C1 lateral mass and C2 vertebral pedicle screw fixation and autologous bone transplantation in 15. Results Postoperatively intracranial infection occurred in 2 cases and cerebrospinal fluid leakage from the incision in 1. Fifty-five patients were followed up for mean time of 42 months, ranged from 6 to 60 months. Complete fusion was achieved in 52 cases and incomplete fusion in 3 receiving fixation

  8. Neurological deterioration after foramen magnum decompression for Chiari malformation type I: old or new pathology?

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    Klekamp, Jörg

    2012-12-01

    Decompression of the foramen magnum is widely accepted as the procedure of choice for patients with Chiari malformation Type I (CM-I). This study was undertaken to determine the mechanisms responsible for neurological deterioration after foramen magnum decompression and the results of secondary interventions. Between 1987 and 2010, 559 patients with CM-I presented, 107 of whom had already undergone a foramen magnum decompression, which included a syrinx shunt in 27 patients. Forty patients who were neurologically stable did not undergo another operation. Sixty-seven patients with progressive symptoms received a recommendation for surgery, which was refused by 16 patients, while 51 patients underwent a total of 61 secondary operations. Hospital and outpatient records, radiographic studies, and intraoperative images were analyzed. Additional follow-up information was obtained by telephone calls and questionnaires. Short-term results were determined after 3 and 12 months, and long-term outcomes were evaluated using Kaplan-Meier statistics. Sixty-one secondary operations were performed after a foramen magnum decompression. Of these 61 operations, 15 involved spinal pathologies not related to the foramen magnum (spinal group), while 46 operations were required for a foramen magnum issue (foramen magnum group). Except for occipital pain and swallowing disturbances, the clinical course was comparable in both groups. In the spinal group, 5 syrinx shunt catheters were removed because of nerve root irritations or spinal cord tethering. Eight patients underwent a total of 10 operations on their cervical spine for radiculopathies or a myelopathy. No permanent surgical morbidity occurred in this group. In the foramen magnum group, 1 patient required a ventriculoperitoneal shunt for hydrocephalus 7 months after decompression. The remaining 45 secondary interventions were foramen magnum revisions, of which 10 were combined with craniocervical fusion. Intraoperatively, arachnoid

  9. Rare association between cystic fibrosis, Chiari I malformation, and hydrocephalus in a baby: a case report and review of the literature

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    Jea Andrew

    2011-08-01

    Full Text Available Abstract Introduction Cystic fibrosis, an epithelial cell transport disorder caused by mutations of the cystic fibrosis transmembrane conductance regulator gene, is not generally associated with malformations of the central nervous system. We review eight previously published reports detailing an infrequent association between cystic fibrosis and Chiari I malformation. Case presentation To the best of our knowledge, our report describes only the ninth case of a baby presenting with a new diagnosis of cystic fibrosis and Chiari I malformation, in this case in a 10-month-old, full-term Caucasian baby boy from the United States of America. Neurosurgical consultation was obtained for associated developmental delay, macrocephaly, bulging anterior fontanel, and papilledema. An MRI scan demonstrated an extensive Chiari I malformation with effacement of the fourth ventricle, obliteration of the outlets of the fourth ventricle and triventricular hydrocephalus without aqueductal stenosis. Our patient was taken to the operating room for ventriculoperitoneal shunt placement. Conclusions It is possible that the cystic fibrosis transmembrane conductance regulator gene may play a previously unrecognized role in central nervous system development; alternatively, this central nervous system abnormality may have been acquired due to constant valsalva from recurrent coughing or wheezing or metabolic and electrolyte imbalances that occur characteristically in cystic fibrosis.

  10. Computational Investigation of Cerebrospinal Fluid Dynamics in the Posterior Cranial Fossa and Cervical Subarachnoid Space in Patients with Chiari I Malformation

    Science.gov (United States)

    Støverud, Karen-Helene; Langtangen, Hans Petter; Ringstad, Geir Andre; Eide, Per Kristian; Mardal, Kent-Andre

    2016-01-01

    Purpose Previous computational fluid dynamics (CFD) studies have demonstrated that the Chiari malformation is associated with abnormal cerebrospinal fluid (CSF) flow in the cervical part of the subarachnoid space (SAS), but the flow in the SAS of the posterior cranial fossa has received little attention. This study extends previous modelling efforts by including the cerebellomedullary cistern, pontine cistern, and 4th ventricle in addition to the cervical subarachnoid space. Methods The study included one healthy control, Con1, and two patients with Chiari I malformation, P1 and P2. Meshes were constructed by segmenting images obtained from T2-weighted turbo spin-echo sequences. CFD simulations were performed with a previously verified and validated code. Patient-specific flow conditions in the aqueduct and the cervical SAS were used. Two patients with the Chiari malformation and one control were modelled. Results The results demonstrated increased maximal flow velocities in the Chiari patients, ranging from factor 5 in P1 to 14.8 in P2, when compared to Con1 at the level of Foramen Magnum (FM). Maximal velocities in the cervical SAS varied by a factor 2.3, while the maximal flow in the aqueduct varied by a factor 3.5. The pressure drop from the pontine cistern to the cervical SAS was similar in Con1 and P1, but a factor two higher in P2. The pressure drop between the aqueduct and the cervical SAS varied by a factor 9.4 where P1 was the one with the lowest pressure jump and P2 and Con1 differed only by a factor 1.6. Conclusion This pilot study demonstrates that including the posterior cranial fossa is feasible and suggests that previously found flow differences between Chiari I patients and healthy individuals in the cervical SAS may be present also in the SAS of the posterior cranial fossa. PMID:27727298

  11. Craniospinal Polyostotic Fibrous Dysplasia, Aneurysmal Bone Cysts, and Chiari Type 1 Malformation Coexistence in a Patient with McCune-Albright Syndrome.

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    Urgun, Kamran; Yılmaz, Baran; Toktaş, Zafer Orkun; Akakın, Akın; Konya, Deniz; Demir, Mustafa Kemal; Kılıç, Türker

    2016-01-01

    Aneurysmal bone cysts (ABCs) are defined as benign cystic lesions of bone composed of blood-filled spaces. ABCs may be a secondary pathology superimposed on fibrous dysplasia (FD). Concomitant FD and ABC in relation with McCune-Albright syndrome is an extremely rare condition. Here, we report concomitant, double ABCs in bilateral occipital regions and FD from the skull base to the C2 vertebra with Chiari type 1 malformation. A 14-year-old female with a diagnosis of McCune-Albright syndrome presented with swellings at the back of her head. The lesions were consistent with ABCs and were totally resected with reconstruction of the calvarial defects. The coexistence of FD, bilateral occipital ABCs, and Chiari malformation type 1 in a McCune-Albright patient is an extremely rare condition and, to our knowledge, has not been reported to date. Exact diagnosis and appropriate surgical treatment usually lead to a good outcome.

  12. Abnormalities in auditory evoked potentials of 75 patients with Arnold-Chiari malformations types I and II Anormalidades nos potenciais evocados auditivos de 75 pacientes com os tipos I e II das malformações de Arnold-Chiari

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    Paulo Sergio A. Henriques Filho

    2006-09-01

    Full Text Available OBJECTIVE: To evaluate the frequency and degree of severity of abnormalities in the auditory pathways in patients with Chiari malformations type I and II. METHOD: This is a series-of-case descriptive study in which the possible presence of auditory pathways abnormalities in 75 patients (48 children and 27 adults with Chiari malformation types I and II were analyzed by means of auditory evoked potentials evaluation. The analysis was based on the determination of intervals among potentials peak values, absolute latency and amplitude ratio among potentials V and I. RESULTS: Among the 75 patients studied, 27 (36% disclosed Arnold-Chiari malformations type I and 48 (64% showed Arnold-Chiari malformations type II. Fifty-three (71% of these patients showed some degree of auditory evoked potential abnormalities. Tests were normal in the remaining 22 (29% patients. CONCLUSION: Auditory evoked potentials testing can be considered a valuable instrument for diagnosis and evaluation of brain stem functional abnormalities in patients with Arnold-Chiari malformations type I and II. The determination of the presence and degree of severity of these abnormalities can be contributory to the prevention of further handicaps in these patients either through physical therapy or by means of precocious corrective surgical intervention.OBJETIVO: Avaliar a freqüência e grau de comprometimento das vias auditivas em tronco cerebral por meio de potencial evocado auditivo, em pacientes afetados por malformações de Arnold-Chiari de tipos I e II. MÉTODO: Foi efetuado um estudo descritivo de tipo série de casos, sendo selecionados 75 pacientes (48 crianças e 27 adultos nos quais foi realizada avaliação dos potenciais evocados das vias auditivas, com base à determinação dos valores dos intervalos entre picos de potenciais, da latência absoluta e da razão entre as amplitudes dos potenciais V e I. RESULTADOS: Entre os 75 pacientes avaliados, 27 (36% apresentavam

  13. Cefalea recurrente pospunción dural en una paciente con síndrome de Chiari tipo I no conocido Recurrent postdural puncture headache in a woman with undiagnosed Chiari I malformation

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    M.A. Rodríguez-Navarro

    2009-03-01

    en los enfermos con malformaciones cerebelosas tipo Chiari no diagnosticadas.Objective: To identify the risk of neurological complications of spinal anesthesia in patients with preexisting Chiari I malformation and to differentiate this entity from "acquired Chiari I malformation", caused by intracranial hypotension. These two entities can be difficult to distinguish radiologically. Case report: After undergoing dural puncture for epidural analgesia during delivery, a 37-year-old woman developed recurrent postdural puncture headache (PDPH. Cerebral magnetic resonance imaging (MRI revealed Chiari I malformation (displacement of the cerebellar tonsils into the foramen magnum and diffuse dural gadolinium enhancement. The patient had experienced chronic headaches exacerbated by Valsalva's maneuver before receiving epidural analgesia but had not reported these symptoms. She was treated with analgesics, caffeine and corticosteroids, which resolved the symptoms. After 6 months of follow-up, a second MRI scan revealed the persistence of the anatomical alterations characteristic of Chiari I malformation but without meningeal alterations. Discussion: After dural puncture, "intracranial hypotension syndrome" can occur, caused by alteration of cerebrospinal fluid (CSF flow and pressure between the cranial and spinal compartments. Continuous CSF leak through the puncture site would cause a negative pressure gradient that would "push" the cerebellar tonsils toward the foramen magnum. Is the pressure change more marked in patients with prior alterations, such as Chiari malformation? Is the previous malformation the cause of the recurrent headache, despite treatment? Conclusion: Thorough clinical evaluation is required before spinal anesthesia is administered since severe neurological complications can occur in patients with undiagnosed Chiari malformations.

  14. Expanding the clinical spectrum of the 16p11.2 chromosomal rearrangements: three patients with syringomyelia.

    Science.gov (United States)

    Schaaf, Christian P; Goin-Kochel, Robin P; Nowell, Kerri P; Hunter, Jill V; Aleck, Kirk A; Cox, Sarah; Patel, Ankita; Bacino, Carlos A; Shinawi, Marwan

    2011-02-01

    16p11.2 rearrangements are associated with developmental delay, cognitive impairment, autism spectrum disorder, behavioral problems (especially attention-deficit hyperactivity disorder), seizures, obesity, dysmorphic features, and abnormal head size. In addition, congenital anomalies and abnormal brain findings were frequently observed in patients with these rearrangements. We identified and performed a detailed microarray, phenotypic, and radiological characterization of three new patients with 16p11.2 rearrangements: two deletion patients and one patient with the reciprocal duplication. All patients have a heterozygous loss (deletion) or gain (duplication) corresponding to chromosomal coordinates (chr16: 29 528 190-30 107 184) with a minimal size of 579 kb. The deletion patients had language delay and learning disabilities and one met criteria for pervasive developmental disorder not otherwise specified. The duplication patient received a diagnosis of autism and had academic deficits and behavioral problems. The patients with deletion had long cervicothoracic syringomyelia and the duplication patient had long thoracolumbar syringomyelia. The syringomyelia in one patient with deletion was associated with Chiari malformation. Our findings highlight the broad spectrum of clinical and neurological manifestations in patients with 16p11.2 rearrangements. Our observation suggests that genes (or a single gene) within the implicated interval have significant roles in the pathogenesis of syringomyelia. A more comprehensive and systematic research is warranted to study the frequency and spectrum of malformations in the central nervous system in these patients.

  15. Bilateral vocal cord palsy causing stridor as the only symptom of syringomyelia and Chiari I malformation, a case report

    Directory of Open Access Journals (Sweden)

    Saif Yousif

    2016-01-01

    Conclusion: Although uncommon, formation of a syrinx should be considered for patients who present with stridor and reiterates the importance of MRI as an important investigative tool of bilateral vocal cord palsy.

  16. Diagnostic value of magnetic resonance imaging (MRI) in 13 cases of Arnold-Chiarimalformation%MRI对13例Arnold-Chiari Ⅰ型畸形的诊断价值

    Institute of Scientific and Technical Information of China (English)

    蔡萍; 丁仕义

    2001-01-01

    @@ 阿-希二氏Ⅰ型畸形(Arnold-Chiarimalformation,ACM-Ⅰ)是最常见的后脑畸形,磁共振成像(Magnetic resonance imaging,MRI)检查对ACM-Ⅰ的诊断明显优于其它传统方法,为其正确诊断提供了新的手段.本研究分析我院1996年开始MRI检出的ACM-Ⅰ 13例,旨在评价MRI对ACM-Ⅰ的诊断价值.

  17. Chiari畸形患者的术后护理%Postopertive nursing of patients with Chiari malformation

    Institute of Scientific and Technical Information of China (English)

    叶永琍; 缪建平; 朱利芳; 郁秀静

    2010-01-01

    @@ Chiari畸形(Chiari malformation,CM)是后颅凹正中线结构在胚胎期的异常发育致使小脑扁桃体疝入枕大孔内而引起延髓、上颈髓受压及颅内压增高等表现的一组综合征~([1]),又称小脑扁桃体畸形.CM属于先天性疾病,常伴有脊髓空洞症~([2]).目前对于本病的发病机制尚不十分清楚,手术是治疗CM最主要的手段~([3]),其目的是解除对神经组织的压迫,重建腩脊液循环通路~([4]).2003年2月至2009年2月我院手术治疗CM患者49例,术后疗效满意,现报道如下.

  18. Surgical treatment of Chiari malformation: review and progress%Chiari畸形外科治疗回顾与进展

    Institute of Scientific and Technical Information of China (English)

    李鹏超; 刘勇; 菅凤增; 张远征

    2012-01-01

    The surgical treatment of Chiari malformation (CM) began in 1932. With the advance of medical technology, the surgical technique of CM is also in constant improvement. But due to its pathogenesis has not yet clear, there is no accepted optimal method, and different levels of the operation is still controversial. The author reviewed the concept, pathogenesis, diagnosis and surgical treatment of CM. The hot topics and new technological application were also reviewed in this article.%Chiari畸形的外科治疗始于1932 年,70 余年来随着医学技术的不断进步,其手术方式不断改进.然而,由于发病机制至今尚未明确,致使不能形成一种临床公认的最佳外科手术方法,手术过程中不同层次的操作步骤或具体方法仍存有争议.本文拟就Chiari畸形的概念、发病机制、诊断与神经外科手术治疗方法进行回顾,并对目前的热点问题和新技术应用进行概述.

  19. The Association of Pseudohypoparathyroidism Type Ia with Chiari Malformation Type I: A Coincidence or a Common Link?

    Science.gov (United States)

    2016-01-01

    A 19-month-old boy was referred for progressive weight gain. His past medical history included congenital hypothyroidism and developmental delay. Physical examination revealed characteristics of Albright Hereditary Osteodystrophy, macrocephaly, and calcinosis cutis. He had hypocalcemia, hyperphosphatemia, and elevated Parathyroid Hormone levels. Genetic testing revealed a known mutation of GNAS gene, confirming the diagnosis of Pseudohypoparathyroidism Type Ia (PHP-Ia) (c.34C>T (p.G1n12X)). He had a normal brain MRI at three months, but developmental delay prompted a repeat MRI that revealed Chiari Malformation Type I (CM-I) with hydrocephalus requiring neurosurgical intervention. This was followed by improvement in attaining developmental milestones. Recently, he was diagnosed with growth hormone deficiency. This case suggests the potential association of CM-I with PHP-Ia. Larger studies are needed to assess whether CM-I with hydrocephalus are common associations with PHP-Ia and to define potential genetic links between these conditions. We propose a low threshold in performing brain MRI on PHP-1a patients, especially those with persistent developmental delay to rule out CM-I. Early intervention may improve neurodevelopmental outcomes and prevent neurosurgical emergencies. PMID:27703483

  20. Perioperative monitoring of pulsatile and static intracranial pressure in patients with Chiari malformation type 1 undergoing foramen magnum decompression.

    Science.gov (United States)

    Frič, Radek; Eide, Per Kristian

    2016-02-01

    Patients with Chiari malformation type 1 (CMI) often present with elevated pulsatile and static intracranial pressure (ICP). The preferred treatment of CMI, foramen magnum decompression (FMD), is assumed to normalise ICP and craniospinal pressure dissociation. In order to further explore the mechanisms behind FMD, the present study investigated whether or not pulsatile and static ICP normalises immediately after FMD. The study included CMI patients undergoing FMD with perioperative ICP monitoring as a part of clinical management. The pulsatile and static ICP scores were retrieved from the department's ICP database, and the clinical and radiological data from the patient records. Eleven patients were included in the study. During the first 3 days following FMD, mixed model analysis revealed no significant time-dependent differences of preoperatively elevated either pulsatile (mean wave amplitude, MWA; p = 0.85) and/or static (mean ICP, p = 0.90) ICP. Percentage of mean ICP >15 mmHg increased during days 2 and 3 after FMD. Two patients from the present series had to receive ventriculoperitoneal shunt after FMD in the early postoperative period. The present observations suggest that anatomical restoration of cerebrospinal fluid pathways by FMD does not lead to immediate normalisation of preoperatively altered pulsatile and static ICP in patients with CMI. This finding may explain persistent symptoms during the early period after FMD.

  1. Surgical treatment of type I Chiari malformation: the role of Magendie’s foramen opening e tonsils manipulation

    Directory of Open Access Journals (Sweden)

    Claudio Henrique Fernandes Vidal

    2015-02-01

    Full Text Available The treatment for type 1 Chiari malformation (CM 1 is one of the most controversial topics in the neurosurgical field. The present study evaluated two of the most applied surgical techniques to treat CM 1. Method 32 patients were evaluated and divided in two groups: group 1 had 16 patients that were submitted to decompression of occipital bone and dura mater of the craniovertebral junction (CVJ; group 2 also had 16 patients and in addition to the previous procedure, they were submitted to Magendie’s foramen opening e tonsils manipulation. The comparison between the groups included neurological exam and cerebrospinal fluid flow imaging during pre and postoperative periods. Results Both techniques were equivalents in terms of neurological improvement of the patients (p > 0.05, but the group 2 had more surgical complications, with relative risk of 2.45 (CI 1.55-3.86 for adverse events. Whatever the cerebrospinal fluid flow at CVJ, the patients of the group 1 achieved greater amount of flow than the group 2 (p < 0.05 during the postoperative period. Conclusion The cranial and dural decompression of the CVJ without arachnoidal violation was the best surgical intervention for treatment of CM 1, between these two compared techniques.

  2. Treatment for Chiari 1 malformation (CIM): analysis of a pediatric surgical series.

    Science.gov (United States)

    Valentini, Laura; Visintini, Sergio; Saletti, Veronica; Chiapparini, Luisa; Estienne, Margherita; Solero, Carlo Lazzaro

    2011-12-01

    Children may present a peculiar picture of CIM, as syncopes and acute paraparesis. In a series of 99 children operated for CIM at FINCB there were no major surgical morbidity nor mortality. The preoperative symptoms improved more in this pediatric series than in the adult cases treated at the same institution in the same period; a reason could be the shorter duration of symptoms and another the children plasticity. An untreated nonsyndromic craniosynostosis was present in 10 cases. In our hands, the results of the limited extradural decompression were poor. In some CIM associated with psychiatric symptoms an unexpected improvement was observed after tonsilar resection. The associated Syringomyelia reduced in more than 80% of children and disappeared in a significant number. The rare associated tethered cord (5%) needed a double treatment, detethering by itself being insufficient to treat also tonsillar descent. The clinical symptoms are often more serious in children than in the adults, but the results of surgery, especially on the syrinx, are better.

  3. Valoración de la capacidad laboral de la malformación de Arnold Chiari tipo I Valuation of work disability of the Arnold Chiari malformation

    Directory of Open Access Journals (Sweden)

    Raúl Jesús Regal Ramos

    2011-03-01

    ándolas con los requerimientos del puesto de trabajo, no obstante existen una serie de aspectos comunes relevantes en la valoración laboral de estos pacientes.Introduction: The recent publication of a consensus document by the Research Institute of Rare Diseases, the increased incidence of Arnold-Chiari malformation (by the increased demand of cervical MRI and, simultaneously, the increased presence of these patients in hospitals where doctors can assess their functional capacity, invites us to reflect on the assessment of the working disability of these patients. Objectives: This review pretends to make an approach to various relevant aspects on the assessment of these patients' working disability. Material and methods: The following bibliographical database have been reviewed until May 2010: Medline, Embase, Cochrane. Results: The intensity of the symptoms (mostly pain is not directly related to the severity of the lesions observed in the imaging tests. The clinical symptoms may fluctuate, with periods of exacerbation and remission. The symptoms that best respond to surgery are those due to compression of the brainstem (especially cervical headache, disappearing in some cases. The presence of atrophy, ataxia, scoliosis in the pre surgery tests, and more than two years between the beginning of the symptoms and the surgery are poor post surgery evolution factors. Those clinical manifestations that do not disappear in the post surgery period or in the follow-up period could be related to permanent damage of the neural pathways or their nuclei. Despite being a congenital disease, it could be treated as a working accident, if symptoms appear after trauma. Evidence of the effectiveness of pain treatment is low. Conclusions: These patients' evaluation should always be individualized, considering the organic and or functional limitations and relating them to the requests of their job. Nevertheless, there exists a series of common relevant aspects in these patients' working

  4. Precocious puberty in two girls with Chiari I malformation: a contribution to a larger use of brain MRI in the diagnosis of central precocious puberty.

    Science.gov (United States)

    Pucarelli, I; Accardo, F; Tarani, L; Demiraj, V; Segni, M; Pasquino, A M

    2010-06-01

    Up to now Chiari malformation has been reported only in four subjects with precocious puberty, with a prevalence among boys. This article describes the case of two female children affected by progressive precocious puberty detected through brain magnetic resonance imaging (MRI). Brain imaging, even without neurological signs, can identify patients at risk of developing subsequently severe neurological symptoms. Our observation supports the usefulness of brain MRI both in males and females, even when no symptoms are present, to identify and detect high risk cases. However, there is no consensus in Literature in performing MRI in all the patients of both sexes with central precocious puberty, due to its high costs.

  5. [Arnold-Chiari malformation in Noonan syndrome and other syndromes of the RAS/MAPK pathway].

    Science.gov (United States)

    Ejarque, Ismael; Millán-Salvador, José M; Oltra, Silvestre; Pesudo-Martínez, José V; Beneyto, Magdalena; Pérez-Aytés, Antonio

    2015-05-01

    Introduccion. El sindrome de Noonan (SN) y otros sindromes con fenotipo similar, como LEOPARD, cardiofaciocutaneo, Costello y Legius, estan asociados a mutaciones en genes incluidos en la via RAS/MAPK (rasopatias), una importante via de señalizacion relacionada con la proliferacion celular. El descenso de las amigdalas cerebelares dentro del canal medular cervical, conocido como malformacion de Arnold-Chiari (MAC), se ha descrito en pacientes afectos de SN, lo que ha llevado a sugerir que la MAC podria formar parte del espectro fenotipico del SN. Presentamos dos casos con SN y MAC. Casos clinicos. Caso 1: mujer de 29 años con fenotipo de Noonan. Fue intervenida a los 9 años de estenosis valvular pulmonar. A los 27 años, presento MAC sintomatica que preciso descompresion quirurgica. Presentaba mutacion c.922A>G (N308D) en el gen PTPN perteneciente a la via RAS/MAPK. Caso 2: niña de 10 años con fenotipo de Noonan y MAC asintomatica detectada en resonancia magnetica cerebral. Era portadora de la mutacion c.923A>G (N308S) en el gen PTPN11. Conclusiones. Hemos encontrado en la bibliografia seis pacientes con esta asociacion, cuatro con fenotipo Noonan y dos con LEOPARD. Nuestros dos pacientes aportan evidencia suplementaria a la hipotesis de que la MAC formaria parte del espectro fenotipico del SN. El escaso numero de pacientes publicados con esta asociacion no permite extraer recomendaciones sobre el momento y la frecuencia de estudio de neuroimagen; no obstante, una exploracion neurologica cuidadosa deberia incluirse en la guia anticipatoria de salud en los sindromes de la via RAS/MAPK.

  6. Chiari 1 malformation presenting as central sleep apnea during pregnancy: a case report, treatment considerations, and review of the literature

    Directory of Open Access Journals (Sweden)

    Erik K. St. Louis

    2014-10-01

    Full Text Available Purpose: Chiari malformation (CM type 1 frequently causes obstructive or central sleep-disordered breathing (SDB in both adults and children, although SDB is relatively rare as a presenting manifestation in the absence of other neurological symptoms. The definitive treatment of symptomatic CM is surgical decompression. We report a case that is, to our knowledge, a novel manifestation of central sleep apnea (CSA due to CM type 1 with severe exacerbation and initial clinical presentation during pregnancy.Methods: Case report from tertiary care comprehensive sleep medicine center with literature review of sleep-disordered breathing manifestations associated with CM type 1. PubMed search was conducted between January 1982 and October 2013. Results: We report a 25-year-old woman with severe central sleep apnea initially presenting during her first pregnancy that eventually proved to be caused by CM type 1. The patient was successfully treated preoperatively by adaptive servoventilation (ASV, with effective resolution of sleep-disordered breathing following surgical decompression, and without recurrence in a subsequent pregnancy.Our literature review found that 58% of CM patients with SDB had OSA alone, 28% had CSA alone, 8 (10% had mixed OSA/CSA, and 6 (8% had hypoventilation. Of CM patients presenting with SDB, 50% had OSA, 42% had CSA, 8% had mixed OSA/CSA, and 10.4% had hypoventilation. We speculate that CSA may develop in CM patients in whom brainstem compression results in excessive central chemoreflex sensitivity with consequent hypocapnic CSA.Conclusions: CM type 1 may present with a diversity of SDB manifestations, and timely recognition and surgical referral are necessary to prevent further neurological deficits. ASV therapy can effectively manage CSA caused by CM type 1, which may initially present during pregnancy.

  7. Chiari malformation Type I surgery in pediatric patients. Part 1: validation of an ICD-9-CM code search algorithm.

    Science.gov (United States)

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

    2016-05-01

    OBJECTIVE Administrative billing data may facilitate large-scale assessments of treatment outcomes for pediatric Chiari malformation Type I (CM-I). Validated International Classification of Diseases, Ninth Revision, Clinical Modification (ICD-9-CM) code algorithms for identifying CM-I surgery are critical prerequisites for such studies but are currently only available for adults. The objective of this study was to validate two ICD-9-CM code algorithms using hospital billing data to identify pediatric patients undergoing CM-I decompression surgery. METHODS The authors retrospectively analyzed the validity of two ICD-9-CM code algorithms for identifying pediatric CM-I decompression surgery performed at 3 academic medical centers between 2001 and 2013. Algorithm 1 included any discharge diagnosis code of 348.4 (CM-I), as well as a procedure code of 01.24 (cranial decompression) or 03.09 (spinal decompression or laminectomy). Algorithm 2 restricted this group to the subset of patients with a primary discharge diagnosis of 348.4. The positive predictive value (PPV) and sensitivity of each algorithm were calculated. RESULTS Among 625 first-time admissions identified by Algorithm 1, the overall PPV for CM-I decompression was 92%. Among the 581 admissions identified by Algorithm 2, the PPV was 97%. The PPV for Algorithm 1 was lower in one center (84%) compared with the other centers (93%-94%), whereas the PPV of Algorithm 2 remained high (96%-98%) across all subgroups. The sensitivity of Algorithms 1 (91%) and 2 (89%) was very good and remained so across subgroups (82%-97%). CONCLUSIONS An ICD-9-CM algorithm requiring a primary diagnosis of CM-I has excellent PPV and very good sensitivity for identifying CM-I decompression surgery in pediatric patients. These results establish a basis for utilizing administrative billing data to assess pediatric CM-I treatment outcomes.

  8. Hydrodynamic and longitudinal impedance analysis of cerebrospinal fluid dynamics at the craniovertebral junction in type I Chiari malformation.

    Directory of Open Access Journals (Sweden)

    Bryn A Martin

    Full Text Available Elevated or reduced velocity of cerebrospinal fluid (CSF at the craniovertebral junction (CVJ has been associated with type I Chiari malformation (CMI. Thus, quantification of hydrodynamic parameters that describe the CSF dynamics could help assess disease severity and surgical outcome. In this study, we describe the methodology to quantify CSF hydrodynamic parameters near the CVJ and upper cervical spine utilizing subject-specific computational fluid dynamics (CFD simulations based on in vivo MRI measurements of flow and geometry. Hydrodynamic parameters were computed for a healthy subject and two CMI patients both pre- and post-decompression surgery to determine the differences between cases. For the first time, we present the methods to quantify longitudinal impedance (LI to CSF motion, a subject-specific hydrodynamic parameter that may have value to help quantify the CSF flow blockage severity in CMI. In addition, the following hydrodynamic parameters were quantified for each case: maximum velocity in systole and diastole, Reynolds and Womersley number, and peak pressure drop during the CSF cardiac flow cycle. The following geometric parameters were quantified: cross-sectional area and hydraulic diameter of the spinal subarachnoid space (SAS. The mean values of the geometric parameters increased post-surgically for the CMI models, but remained smaller than the healthy volunteer. All hydrodynamic parameters, except pressure drop, decreased post-surgically for the CMI patients, but remained greater than in the healthy case. Peak pressure drop alterations were mixed. To our knowledge this study represents the first subject-specific CFD simulation of CMI decompression surgery and quantification of LI in the CSF space. Further study in a larger patient and control group is needed to determine if the presented geometric and/or hydrodynamic parameters are helpful for surgical planning.

  9. Syndrome of occipitoatlantoaxial hypermobility, cranial settling, and chiari malformation type I in patients with hereditary disorders of connective tissue.

    Science.gov (United States)

    Milhorat, Thomas H; Bolognese, Paolo A; Nishikawa, Misao; McDonnell, Nazli B; Francomano, Clair A

    2007-12-01

    Chiari malformation Type I (CM-I) is generally regarded as a disorder of the paraxial mesoderm. The authors report an association between CM-I and hereditary disorders of connective tissue (HDCT) that can present with lower brainstem symptoms attributable to occipitoatlantoaxial hypermobility and cranial settling. The prevalence of HDCT was determined in a prospectively accrued cohort of 2813 patients with CM-I. All patients underwent a detailed medical and neuroradiological workup that included an assessment of articular mobility. Osseous structures composing the craniocervical junction were investigated morphometrically using reconstructed 3D computed tomography and plain x-ray images in 114 patients with HDCT/CM-I, and the results were compared with those obtained in patients with CM-I (55 cases) and healthy control individuals (55 cases). The diagnostic criteria for Ehlers-Danlos syndrome and related HDCT were met in 357 (12.7%) of the 2813 cases. Hereditability was generally compatible with a pattern of autosomal dominant transmission with variable expressivity. The diagnostic features of HDCT/CM-I were distinguished from those of CM-I by clinical stigmata of connective tissue disease, a greater female preponderance (8:1 compared with 3:1, p supine and upright positions in healthy control individuals and patients with CM-I. In patients with HDCT/CM-I, there was a reduction of the basion-dens interval (3.6 mm, p cervical traction or returning to the supine position. The identification of HDCT in 357 patients with CM-I establishes an association between two presumably unrelated mesodermal disorders. Morphometric evidence in this cohort-cranial settling, posterior gliding of the occipital condyles, and reduction of the clivus-axis angle, clivus-atlas angle, and atlas-axis angle in the upright position-suggests that hypermobility of the occipitoatlantal and atlantoaxial joints contributes to retroodontoid pannus formation and symptoms referable to basilar

  10. Clinical Character and Misdiagnosis of Arnold-ChiariMalformation%Arnold-Chiari Ⅰ型畸形的临床及误诊分析

    Institute of Scientific and Technical Information of China (English)

    杨杰; 李蜀渝

    2002-01-01

    目的:研究21例Arnold-Chiari Ⅰ型畸形患者的临床及误诊原因.方法:回顾性分析21例Arnold-Chiari Ⅰ型畸形患者的临床资料.结果:本病大多合并脊髓空洞症,临床主要为枕骨大孔区、脊髓中央管及小脑受损征侯群.临床表现多样,缺乏特异性是误诊的主要原因.结论:本病的诊断依靠其临床特点及磁共振检查.

  11. MRI Analysis and Pathogenesis Exploration in Chiari I Malformation%Arnold-Chiari Ⅰ畸形MRI分析及发病机制探讨

    Institute of Scientific and Technical Information of China (English)

    姜新雅; 李文政; 仇斌

    2001-01-01

    目的:分析后颅窝长、宽、深及小脑扁桃体垂直径,枕骨大孔前后径同该病的关系。方法:对57例Chiari Ⅰ畸形病例及58例正常对照MRI进行后颅窝大小及小脑扁桃体垂直径,枕骨大孔前后径测量,采用u检验进行统计学分析。结果:病理组后颅窝长、宽、深明显小于对照组,小脑扁桃体垂直径明显大于对照组,两组之间枕骨大孔前后径无显著性差异。结论:小脑扁桃体垂直径增大对Chiari Ⅰ畸形诊断有重要价值。轴旁中胚层枕节发育不良致骨性后颅窝狭小是Chiari Ⅰ畸形可能的发病机制。

  12. 家族性Chiari畸形临床特点及影像学分析%Clinical and Imaging Analysis of Familial Chiari Malformation

    Institute of Scientific and Technical Information of China (English)

    齐巍; 邓晓峰; 杨辰龙; 李安琪; 徐宇伦

    2015-01-01

    Objective To analyze the clinical and imaging characteristics of familial Chiari malformations.Methods Clinical and imaging studies of 3 families with 6 Chiari malformation patients were retrospectively studied, 4 of which underwent posterior fossa decompression and the other 2 patients were not surgically treated.Results Patients of the first family were twin brothers, both with Chiari malformation type 1.5 (CM 1.5) and syrinx. The elder brother had scoliosis and the younger brother had hydrocephalus. Patients of the second family are mother and son, both with Chiari malformation type I (CMI) and syrinx. Besides, the mother had scoliosis and the son had hydrocephalus. Patients of the third family are non-twin sisters, and the elder sister had CM I and syrinx, but the younger sister had CM 1.5 without syrinx. The 4 patients who underwent posterior fossa decompression had good outcome.Conclusions Familial Chiari malformations may occur in many kinds of relationships, such as twins, mother and son, and non-twin sisters, and clinical and imaging manifestations maybe various.%目的:探讨家族性Chiari畸形的临床及影像学特点。方法回顾性分析三个Chiari畸形家族共6例患者的临床及影像学表现。其中4例患者行后颅窝减压术,另外2例患者未手术。结果第一个家族为男性双胞胎患者,均患有Chiari畸形1.5型(CM 1.5)及脊髓空洞,其中兄长合并脊柱侧弯而无脑积水,弟弟合并脑积水但无脊柱侧弯。第二个家族两位患者为母子关系,均患有Chiari畸形I型(CM I)及脊髓空洞,此外,母亲合并脊柱侧弯而无脑积水,儿子合并脑积水但无脊柱侧弯。第三个家族两位患者为非双胞胎姐妹关系,其中姐姐为CM I合并脊髓空洞,而妹妹为CM 1.5且无脊髓空洞,二者均无脊柱侧弯及脑积水。4例患者行后颅窝减压术后恢复良好。结论家族性Chiari畸形可发生于多种家族关系中,如双胞胎兄弟、

  13. Malformação de Chiari tipo I: relato de dois casos com apresentações clínicas pouco usuais Type I Chiari malformation: report of two cases with unusual clinical presentation

    Directory of Open Access Journals (Sweden)

    EDUARDO R. PUPPI MORO

    1999-09-01

    Full Text Available Relatamos dois casos de malformação de Chiari do tipo 1, com apresentações clínicas pouco usuais. O primeiro caso refere-se a uma paciente de 17 anos, com quadro agudo de insuficiência respiratória e o segundo caso a uma paciente com síndrome vestibular associada a síndrome cerebelar leve e cefaléia. Em ambos os casos o exame neurológico demonstrou a presença de nistagmo do tipo "downbeating". Enfatizamos a valorização da semiologia neurológica, determinando a investigação complementar adequada e o tratamento efetivo.We describe two patients with Chiari type I malformation with unusual clinical presentation. The first one with clinical picture of acute respiratory insuficiency and the second one with vestibular and mild cerebellar syndrome and headache. In both cases the neurological examination demonstrated the presence of "downbeating nystagmus". We emphasize the value of neurological semiology, determining a correct complementary evaluation and effective treatment.

  14. Syringomyelia associated with adhesive spinal arachnoiditis: MRI

    Energy Technology Data Exchange (ETDEWEB)

    Inoue, Y.; Nemoto, Y.; Daikokuya, H.; Tashiro, T.; Shakudo, M.; Nakayama, K.; Yamada, R. [Dept. of Radiology, Osaka City University Medical School (Japan); Ohata, K.; Hakuba, A.; Nagai, K. [Dept. of Neurosurgery, Osaka City University Medical School (Japan)

    2001-04-01

    We reviewed the MRI of seven patients with syringomyelia associated with surgically proven adhesive spinal arachnoiditis to describe clinical and MRI findings in this condition. Using 0.5, 1.0 or 1.5 tesla units, we obtained sagittal T1- and T2-weighted spin-echo and axial T1-weighted images. Additional sagittal T2-weighted images were obtained without using motion-artefact suppression. Contrast medium was given intravenously to five patients. The syrinx cavities were thoracic in five cases, cervicothoracic in one, and extended from C4 to L1 in one. No Chiari malformation or craniovertebral junction anomaly was demonstrated. Meningeal thickening was seen on T2-weighted sagittal images only in one case. Cord deformity due to adhesion or displacement due to an associated arachnoid cyst was seen in all cases best demonstrated on axial images. Focal blurring of the syrinx wall on axial images was seen in six patients. Flow voids were seen in the syrinx fluid in all cases on T2-weighted images obtained without motion-artefact suppression. No abnormal contrast enhancement was demonstrated. (orig.)

  15. Valoración de la capacidad laboral de la malformación de Arnold Chiari tipo I Valuation of work disability of the Arnold Chiari malformation

    OpenAIRE

    Raúl Jesús Regal Ramos

    2011-01-01

    Introducción: La reciente publicación de un documento de consenso por el Instituto de Investigación de Enfermedades Raras, el aumento de la incidencia de malformación de Arnold - Chiari (por la cada vez más frecuente solicitud de RMN cervicales) y, paralelamente, el aumento de la presencia de estos pacientes en las consultas de los médicos encargados de valorar su capacidad funcional, nos invita a hacer una reflexión sobre la valoración laboral de estos pacientes. Objetivos: Esta revisión pre...

  16. Chiari 畸形合并脊髓空洞症45例外科治疗分析

    Institute of Scientific and Technical Information of China (English)

    张国栋; 裴傲

    2010-01-01

    @@ Chiari 畸形(Chiari malformation,CM)是一种少见的枕大孔区先天性畸形,又称小脑扁桃体下疝畸形,常合并脊髓空洞(syringomyelia,SM).由于发病机制尚不明确,虽然手术方法多样,但文献报道的结果不尽相同[1].我们在2001年1月至2007年12月对45例CM合并SM的患者进行了手术治疗,现通过对本组病例的回顾性分析,并结合文献探讨CM合并SM的临床特点和手术治疗方法.

  17. Magnetic resonance imaging in syringomyelia; Ressonancia magnetica na siringomielia

    Energy Technology Data Exchange (ETDEWEB)

    Minguetti, Guilberto [Parana Univ., Curitiba, PR (Brazil). Dept. de Clinica Medica; Tamanini, Alexandro; Nogara, Heloisa

    1998-10-01

    The authors present 13 cases of idiopathic syringomyelia, not associated to trauma, tumors, arachnoiditis or vascular disease. They show the site and extension of the lesions as well as the association with Chiari I (7 cases), enlargement of the spinal canal (2 cases), Klippel-Feil (1 case), basilar impression (1 case) and platibasia (1 case). They concluded that MRI is the diagnostic modality of choice in the diagnosis and evaluation of treatment of syringomyelia. (author) 11 refs., 9 figs., 2 tabs.

  18. Contiguous triple spinal dysraphism associated with Chiari malformation Type II and hydrocephalus: an embryological conundrum between the unified theory of Pang and the unified theory of McLone.

    Science.gov (United States)

    Dhandapani, Sivashanmugam; Srinivasan, Anirudh

    2016-01-01

    Triple spinal dysraphism is extremely rare. There are published reports of multiple discrete neural tube defects with intervening normal segments that are explained by the multisite closure theory of primary neurulation, having an association with Chiari malformation Type II consistent with the unified theory of McLone. The authors report on a 1-year-old child with contiguous myelomeningocele and lipomyelomeningocele centered on Type I split cord malformation with Chiari malformation Type II and hydrocephalus. This composite anomaly is probably due to select abnormalities of the neurenteric canal during gastrulation, with a contiguous cascading impact on both dysjunction of the neural tube and closure of the neuropore, resulting in a small posterior fossa, probably bringing the unified theory of McLone closer to the unified theory of Pang.

  19. A rat model of chronic syringomyelia induced by epidural compression of the lumbar spinal cord.

    Science.gov (United States)

    Lee, Ji Yeoun; Kim, Shin Won; Kim, Saet Pyoul; Kim, Hyeonjin; Cheon, Jung-Eun; Kim, Seung-Ki; Paek, Sun Ha; Pang, Dachling; Wang, Kyu-Chang

    2017-02-17

    OBJECTIVE There has been no established animal model of syringomyelia associated with lumbosacral spinal lipoma. The research on the pathophysiology of syringomyelia has been focused on Chiari malformation, trauma, and inflammation. To understand the pathophysiology of syringomyelia associated with occult spinal dysraphism, a novel animal model of syringomyelia induced by chronic mechanical compression of the lumbar spinal cord was created. METHODS The model was made by epidural injection of highly concentrated paste-like kaolin solution through windows created by partial laminectomy of L-1 and L-5 vertebrae. Behavioral outcome in terms of motor (Basso-Beattie-Bresnahan score) and urinary function was assessed serially for 12 weeks. Magnetic resonance images were obtained in some animals to confirm the formation of a syrinx and to monitor changes in its size. Immunohistochemical studies, including analysis for glial fibrillary acidic protein, NeuN, CC1, ED-1, and caspase-3, were done. RESULTS By 12 weeks after the epidural compression procedure, syringomyelia formation was confirmed in 85% of the rats (34 of 40) on histology and/or MRI. The syrinx cavities were found rostral to the epidural compression. Motor deficit of varying degrees was seen immediately after the procedure in 28% of the rats (11 of 40). In 13 rats (33%), lower urinary tract dysfunction was seen. Motor deficit improved by 5 weeks after the procedure, whereas urinary dysfunction mostly improved by 2 weeks. Five rats (13%, 5 of 40) died 1 month postoperatively or later, and 3 of the 5 had developed urinary tract infection. At 12 weeks after the operation, IHC showed no inflammatory process, demyelination, or accelerated apoptosis in the spinal cords surrounding the syrinx cavities, similar to sham-operated animals. CONCLUSIONS A novel experimental model for syringomyelia by epidural compression of the lumbar spinal cord has been created. The authors hope that it will serve as an important research

  20. Case Report: Acute obstructive hydrocephalus associated with infratentorial extra-axial fluid collection following foramen magnum decompression and durotomy for Chiari malformation type I.

    Science.gov (United States)

    Munakomi, Sunil; Bhattarai, Binod; Chaudhary, Pramod

    2016-01-01

    Acute obstructive hydrocephalus due to infratentorial extra-axial fluid collection (EAFC) is an extremely rare complication of foramen magnum decompression (FMD) and durotomy for Chiari malformation type I. Presence of infratentorial  EAFC invariably causes obstruction at the level of the fourth ventricle or aqueduct of Silvius, thereby indicating its definitive role in hydrocephalus. Pathogenesis of EAFC is said to be a local arachnoid tear as a result of durotomy, as this complication is not described in FMD without durotomy. Controversy exists in management. Usually EAFC is said to resolve with conservative management; so hydrocephalus doesn't require treatment. However, in this case EAFC was progressive and ventriculo-peritoneal shunting (VPS) was needed for managing progressive and symptomatic hydrocephalus.

  1. Assessment of 3D T2-weighted high-sampling-efficiency technique (SPACE) for detection of cerebellar tonsillar motion: new useful sign for Chiari I malformation.

    Science.gov (United States)

    Ucar, Murat; Tokgoz, Nil; Koc, Ali Murat; Kilic, Koray; Borcek, Alp Ozgun; Oner, Ali Yusuf; Kalkan, Gokalp; Akkan, Koray

    2015-01-01

    To describe tonsillar blackout sign (TBS) on three-dimensional (3D)-SPACE, evaluate its performance in identifying Chiari malformation (CM1) as diagnostic marker, and investigate its role in differentiation of symptomatic and asymptomatic CM1. One-hundred fifty-six patients were divided into two groups based on caudal displacement of cerebellar tonsils: CM1 (Group I) and non-CM1 (Group II). Group I was subclassified as symptomatic and asymptomatic by a neurosurgeon. Two radiologists evaluated TBS and cerebrospinal fluid flow abnormality. All subjects presenting TBS had CM1. Difference in presence of TBS between Group I and Group II was highly significant (P<.001).Grading of TBS in symptomatic patients was significantly higher than that in asymptomatic patients (P<.001). TBS is highly suggestive of CM1 and potentially useful in differentiation of symptomatic and asymptomatic CM1. Copyright © 2015 Elsevier Inc. All rights reserved.

  2. Focal dermal hypoplasia: report of a case with myelomeningocele, Arnold-Chiari malformation and hydrocephalus with a review of neurologic manifestations of Goltz syndrome.

    Science.gov (United States)

    Peters, Tess; Perrier, Renee; Haber, Richard M

    2014-01-01

    Focal dermal hypoplasia (Goltz syndrome, Online Mendelian Inheritance in Man [OMIM] 305600) is a rare X-linked dominant congenital disorder involving defects of mesodermal- and ectodermal-derived structures. It is associated with mutations in the PORCN gene, a regulator of Wnt signaling proteins. The phenotype is highly variable, although all describe characteristic skin findings as a primary diagnostic feature. To date there are few case reports of focal dermal hypoplasia associated with central nervous system abnormalities. We report the second case of focal dermal hypoplasia associated with myelomenigocele, Arnold-Chiari malformation and hydrocephalus and the first in a male. Genetic testing identified a novel mosaic three base pair deletion within the PORCN gene (c.853_855delACG). This case highlights the importance of neurological evaluation in focal dermal hypoplasia and consideration of other syndromes more commonly associated with central nervous system abnormalities. In this report we summarize the literature on neurological manifestations in Goltz syndrome.

  3. Neuropathic arthropathy caused by Chiari malformation%Chiari畸形合并神经性关节病38例临床分析

    Institute of Scientific and Technical Information of China (English)

    谷重山; 王振宇; 马长城

    2010-01-01

    Objective To discuss the relative pathogenic factors and mechanisms of neuropathic arthropathy caused by Chiari malformation. Method 309 cases with Chiari malformation in the third hospital of Peking University from 1998 to 2008 were reviewed, and there were 38 cases with neuropathic arthropathy. The general conditions, affected joints, clinical manifestation, radiology and operations were summarized. Results There were remarkable relationship between course of disease, upper limb muscle atrophy and neuropathic arthropathy. After the operations of occipital - cervical decompression or syrinx shunting,18 patients improved in arthropathy. Conclusions Patients with atrophy of upper limb muscles may be prone to neuropathic arthropathy. At the same time, the course of disease is another risk factor. Treatment includes operations on primary diseases,functional exercise of affected joints and drug therapy.%目的 探讨Chiari畸形合并神经性关节病的相关因素和发病机制.方法 回顾性分析38例Chiari畸形合并神经性关节病患者的临床资料,总结其发病特点、发病部位、临床表现、影像学表现和手术治疗等情况.结果 病程长短、上肢肌肉是否萎缩与神经性关节病的发生有关.经枕颈减压术或空洞分流术治疗后,18例患者关节症状改善.结论 上肢肌肉萎缩和病程较长是神经性关节病的危险因素.治疗上以手术治疗原发病为主,辅以关节局部功能锻炼和药物治疗.

  4. Headache precipitated by Valsalva maneuvers in patients with congenital Chiari I malformation Cefaléia precipitada por manobras de Valsalva em pacientes com malformação congênita de Chiari tipo I

    Directory of Open Access Journals (Sweden)

    Hugo André de Lima Martins

    2010-06-01

    Full Text Available The objective of this study was to characterize the headache precipitated by Valsalva maneuvers associated with Chiari type I malformation (CM-1. Nineteen patients were evaluated, with ages ranging from 30 to 75 years. Ten of them presented headache. Pain was more prevalent in the occipital (80% and frontal region (60%. The headaches were of significantly shorter duration in the women compared with the men. The frequency of headache crises was relatively high. All patients with Valsalva-related headache suffered from at least one episode per month. The most prevalent precipitating factors were coughing, which is well described in the literature, and sexual activity, which only now is recognized as an event associated with CM-1.Neste estudo, objetivou-se caracterizar a cefaléia desencadeada por manobra de Valsalva, em portadores de malformação de Chiari tipo 1 (MC-1. Foram avaliados 19 pacientes com idades variando entre 30 e 75 anos. Dentre estes, 10 apresentaram cefaléia. A região mais acometida foi a occipital (80% e frontal (60%. A cefaléia foi de duração bem menor nas mulheres em relação aos homens. A freqüência da cefaléia foi relativamente alta, com todos os pacientes apresentando pelo menos um episódio por mês. Os principais fatores desencadeantes foram a tosse, que já é bem descrita pela literatura, e a atividade sexual, que apenas recentemente foi associada a MC-1.

  5. Chiari Malformation: Treatment

    Science.gov (United States)

    ... and loss of sensation; patients will adapt their lifestyle accordingly. Symptoms may fluctuate over time; patients report that trauma can cause symptoms to come back. Patients in the poor range (10-20%) may end up getting revisions ...

  6. The analysis of the pathophysiology of syringomyelia by means of magnetic resonance imaging

    Energy Technology Data Exchange (ETDEWEB)

    Abe, Toshiaki (Jikei Univ., Tokyo (Japan). School of Medicine)

    1992-12-01

    The pathophysiology of syringomyelia was examined with magnetic resonance imaging (MRI) in 70 patients. According to complications, syringomyelia was classified as being associated with (1) Chiari malformation, (2) both Chiari malformation and basal arachnoiditis, (3) basal arachnoiditis, (4) an obstruction of the foramen Magendie, and (5) spinal arachnoiditis. MRI features in Type 1 included: wedge-shaped herniated tonsils with the cisterna magna, pencil-shaped rostral end of the intraspinal syrinx, and obliterated caudal part of the fourth ventricle. These findings suggested that the herniated tonsils might result in the disturbance of cerebro-spinal fluid (CSF) circulation. In Type 2, the tip of the herniated tonsils was round; a triangular space was noted between the herniated tonsils and the upper spinal cord; the rostral end of the intraspinal syrinx was round; and the caudal part of the fourth ventricle was well visualized. These findings revealed that the brain stem and spinal cord were less pressed, suggesting that CSF circulatory disturbance resulted from basal arachnoiditis around the foramen magnum. In Type 3, the cisterna magna was narrowed by a thickened arachnoid membrane. In type 4, the intraspinal syrinx was directly connected with the fourth ventricle via the patent central canal associated with hydrocephalus. The position of the cerebellar tonsils and the size of the cisterna magna were both normal. In Type 5, a partial adhesion of the arachnoid membrane around the spinal cord was shown, and the syrinx was found extended toward the rostral as well as the caudal direction of the arachnoid adhesion. The rostral end of the syrinx was sometimes changed in size during MRI examinatin. This phenomenon sustained William's theory of 'suck and slosh mechanism' of intraspinal syrinx extension. (N.K.).

  7. Endoscopic foramen magnum decompression of Chiari malformation%神经内镜下寰枕部减压手术治疗Chiari畸形

    Institute of Scientific and Technical Information of China (English)

    张世渊; 郭建忠; 姚晓辉; 胡涛; 沈波; 吉宏明; 张汉伟

    2011-01-01

    Objective To investigate the methods and effectiveness to treat Chiari malformation with the surgical decompression of the foramen magnum under endoscope. Method 23 cases with Chiari malformation.(without atlanto- axialdislocation and basilar invagination) diagnosed by magnetic resonance imaging( MRI)were operated with the surgical decompression of the foramen magnum under the endoscope. Results 23 cases were followed- up 0. 5 -3. 0, years after operation, all the symptoms and signs were remarkably relieved after the operation. No complications was found. In 16 cases with spinal cavities,7 cases spinal cavities disappeared and scases deflated,l case no evident change. Conclusions The endoscope -assisted decompression of the foramen magnum is a safe and effective surgical method to treat Chairi malformation.%目的 探讨神经内镜下寰枕减压手术治疗Chiari畸形的手术方法和临床疗效.方法 对23例经MRI确诊为Chiari畸形但不伴有寰枢锥脱位和齿突凹陷的患者在神经内镜下进行了寰枕减压手术,并进行疗效观察.结果 23例术后随访0.5-3.0年,原有症状均有不同程度改善,感觉障碍程度减轻,肢体肌力有所增强.无手术后并发症.术前合并脊髓空洞症的16例患者中,术后7例空洞消失,8例范围缩小,1例范围无明显变化.结论 神经内镜下寰枕减压手术治疗Chiari畸形方法可行,手术安全、有效、创伤小、疗效满意.

  8. 多层螺旋CT三维重建在Chiari I型畸形骨性后颅窝测量中的应用%Multislice spiral CT three-dimensional reconstruction of the posterior cranial fossa in Chiari I malformation

    Institute of Scientific and Technical Information of China (English)

    田超; 阎世鑫; 靳松; 谢中福

    2011-01-01

    目的 评价多层螺旋CT(MSCT)三维重建诊断Chiari I型畸形患者骨性后颅窝发育不全的临床意义.方法 以52例Chiari I型畸形患者骨性后颅窝为研究对象并以100例正常成人后颅窝为参照.测量项目包括:后颅窝前后径(TL)、后颅窝高度(PCFH)、枕大孔前后径(FMD)、斜坡长度(CL)、后枕长度(SL)、小脑幕倾斜角(ACTS)和后颅窝容积(PCFV).采用独立样本资料t检验比较Chiari I型畸形组及对照组后颅窝形态测量结果的差异,计算Pearson相关系数(r)评价TL、PCFH、FMD、CL、SL和ACTS与PCFV间的相关性.结果 与对照组比较,Chiari I型畸形组的TL、CL、PCFH和PCFV减小而FMD和ACTS增加,差异有统计学意义,SL虽然减小但差异无统计学意义.PCFH、CL和SL与PCFV呈正性相关,FMD和ACTS与PCFV呈负性相关,其中PCFH(r=0.813,P<0.001)和CL(r=0.752,P<0.001)与PCFV的相关性较高.Chiari I型畸形组与对照组各测量结果间的95%置信区间均无重叠.结论 MSCT三维重建技术能够对骨性后颅窝形态进行准确测量,可以作为评价骨性后颅窝发育不全的影像学方法之一.MSCT的测量结果可以进一步完善对Chiari I型畸形患者后颅窝狭窄的诊断.%Objective To assess the clinical usefulness of MSCT three-dimensional reconstruction in diagnosis of osseous posterior cranial fossa(PCF)hypoplasia in patients with Chiari I malformation. Methods Osseous PCF of 52 adult patients with Chiari I malformation and 100 healthy adults control subjects involved in this study. Variable measurements includeing Twining's line (TL) , foramen magnum diameter (FMD) , posterior cranial fossa height (PCFH) , clivus length (CL) , supraocciput length (SL) , angle of cerebellar tentorium steepness (ACTS) and posterior cranial fossa volume (PFCV). Independent-sample Student's t test was used as a statistical method. Pearson correlation coefficient (r) was calculated to summarize the relationship between parameters and volume

  9. 枕下减压和硬膜成形术治疗Chiari畸形I型%Surgery of the ChiariMalformation with Suboccipital Decompression and Duraplasty

    Institute of Scientific and Technical Information of China (English)

    储呈春; 卞留贯; 孙青芳; 李云峰; 郑励力; 沈建康

    2011-01-01

    Objective:To explore the efficacy and surgical technique of suboccipital decompression with duroplasty for treating the Chiarimalformation. Methods:44 patients with Chiarimalformation were retrospectively analyzed. All the patients underwent posterior fossa decompression and dura enlargement with duroplasty. The extent of the region of magnum foramen, including the posterior arch of Cl, was 4 cm × 3 cm. The evaluation of the outcome was based on the improvement of clinical feature at the early stage ( post - operative one week), and Karnofysky scale at the late stage (6 months after operation). The mean follow-up period was 17 months in 38 cases. Results:At early stage, the symptoms and signs improved in 30 cases (68%). 38 cases were followed up ( from 6 months to 5 years, average of 17 months). During the follow-up period, postoperative neurological improvement in 34 cases ( 89%, 34/38 ), Karnofyski scale improved 5 scores on average. The other 4 cases were stable. The MRI findings demonstrated that the cavities in spinal cords disappeared in 8 cascs, reduced in 16 cases. Conclusions:The effectiveness of posterior fossa decompression with duroplasty as a procedure benefits most patients with Chiarimalformations.%目的:探讨枕下减压和硬膜成形术治疗Chiari畸形I型的疗效以及手术技巧.方法:采用常规后正中入路、枕骨游离骨瓣、枕大孔区减压(包括C1后弓)、硬膜扩大成形术,治疗Chiari畸形I型患者44例;术后通过临床表现改善情况评价早期(术后1周)疗效,依据Karnofysky评分标准评价后期(术后6个月)疗效.结果:术后早期神经功能改善30例(68%).38例获得随访,平均随访17个月,随访期间神经功能改善率为89%(34/38),Karnofysky评分平均提高5分;稳定4例(11%);无神经功能恶化者.32例出现空洞的患者中,28例得到MRI随访,其中空洞消失8例(均为颈髓空洞)、较术前缩小的16例、无变化4例.结论

  10. Chiari畸形合并颅底凹陷的外科手术治疗%Surgical treatment of Chiari malformation complicated with basilar impression

    Institute of Scientific and Technical Information of China (English)

    马原; 贺伟旗; 程林; 卢敏; 顾建文; 匡永勤; 夏勋; 杨立斌; 张俊海; 程敬民; 杨涛; 黄海东

    2011-01-01

    目的 探讨小骨窗后颅窝减压加枕颈植骨融合内固定术治疗Chiari畸形合并颅底凹陷的手术效果.方法 回顾性分析2006-2010年收治的16例Chiari畸形合并颅底凹陷患者,其中男7例,女9例,年龄17~65岁,平均36.4岁.所有患者均经影像学确诊,手术采取小骨窗后颅窝减压,小脑扁桃体切除,一期取自体髂骨行颈枕植骨融合钢丝内固定,术后行颈托固定3个月.结果 所有患者术后症状均明显改善,无感染或死亡,1例出现引流口脑脊液漏,1例出现一过性肩胛区和胸胁部疼痛,未经治疗自行消失.术后随访6个月,按Symon和Lavender临床标准,治愈6例,好转9例,无效1例.复查MRI显示重建枕大池清晰,无小脑下垂发生,植骨已融合. 结论小骨窗后颅窝减压加枕颈植骨融合钢丝内固定术治疗Chiari畸形合并颅底凹陷效果确切,在增加后颅窝容积同时可减轻脑干腹侧受压,并能重建颅颈交界区稳定性.%Objective To evaluate the therapeutic effect of small craniotomic posterior fossa decompression combined with occipitalcervical bone graft fusion and internal fixation on Chiari malformation complicated with basilar impression. Methods The clinical data of 16 cases (7 males and 9 females, aged 17 to 65 years, mean 36. 4) of Chiari malformation complicated with basilar impression from 2006 to 2010 were retrospectively analyzed. The diagnoses for all the patients were confirmed by radiology. Small craniotomic posterior fossa decompression was performed in all patients, cerebellar tonsils were resected, and then one-stage occipital-cervical bone graft fusion using autogenous iliac bone and internal wiring fixation were performed. Neck support was used for 3 months after surgery. Results Symptoms were significantly improved in all cases after surgical operation. No patient died or infected. Cerebrospinal fluid leakage was found at draining site in one case. Transient pain of scapular and chest was found

  11. The diagnosis and treatment of chiari malformation%小脑扁桃体下疝畸形的临床诊断及治疗

    Institute of Scientific and Technical Information of China (English)

    乌拉别克·毛力提; 艾克拜尔·亚里坤

    2016-01-01

    Objective To study the diagnosis and treatment of chiari malformation so as to provide references for clinical practices.Methods Forty-one patients meeting diagnostic criteria of chiari malformation who received posterior fossa decom-pression and partial tonsillectomy from January 2012 to January 2014 were selected.The prognosis was evaluated by using Glasgow scale in one month after operation and the improvement of symptoms and complications were observed in the 6-12 months follow-up period.Results One month after operation for all 41 cases,there were 33 cases with good recovery (75.61%),10 cases with weak efficacy(24.39%).Of the 39 survivals,symptoms improved well occurred in 22 cases,good in 13 cases,with the total effective rate of 89.74%.Postoperative fever ranging from 38 ℃ to 39 ℃ happened to 10 cases,with fe-ver time less than one week.During the follow-up,there was no one presenting cerebrospinal fluid leakage,intracranial infec-tion,cerebral spinal-arachnoiditis and other severe complications.Conclusion We should combine clinical symptoms with ima-ging exanimation to diagnosis chiari malformation.The Application of posterior fossa decompression and partial tonsillectomy as well as expanding repair of dura mater with artificial mesh may play clinical roles in improving the clinical symptoms,reduc-ing postoperative complications and improving progress.%目的:研究小脑扁桃体下疝畸形的临床诊断及治疗方法,以期为临床诊治提供一定的依据。方法选取2012-01—2014-01收治的符合小脑扁桃体下疝畸形诊断标准的患者41例,所有患者均行后颅窝减压术及部分扁桃体切除术。术后1个月复查,根据格拉斯哥预后评分评价治疗效果。术后随访6~12个月,观察患者的症状改善及并发症情况。结果41例患者中术后1个月复查,恢复良好33例(75.61%),疗效较差10例(24.39%)。39例生存患者中,症状改善优22例,良13例,总有效率为89.74%

  12. Sleep apnea and REM sleep behavior disorder in patients with Chiari malformations Apnéia do sono e distúrbio do comportamento da fase do sono com REM em pacientes com malformações de Chiari

    Directory of Open Access Journals (Sweden)

    Paulo Sérgio A. Henriques-Filho

    2008-06-01

    Full Text Available BACKGROUND: Chiari malformations (CM may result in the appearance of REM sleep behavior disorder (RBD and sleep apnea syndrome (SAS that can be considered markers of brain stem dysfunction. PURPOSE: To evaluate the frequency of RBD and SAS in patients with CM type I and II. METHOD: Were evaluated 103 patients with CM by means of full night polysomnography. Were scoring different sleep stages, frequency of abnormal movements (through video monitoring and abnormal respiratory events. RESULTS: Of the 103 patients, 36 showed CM type I and 67 CM type II. Episodes of RBD were observed in 23 patients. Abnormal apnea-hypopnea index (AHI was observed in 65 patients. CONCLUSION: The high rate of RBD suggests that this parassomnia and the increased frequency of central sleep apnea episodes, may be considered as a marker of progressive brain stem dysfunction.INTRODUÇÃO: Malformações de Chiari (MC podem gerar o aparecimento de distúrbio comportamental da fase do sono com REM (DCR e síndrome da apnéia do sono (SAS, sugerindo a ocorrência de disfunção do tronco cerebral. OBJETIVO: Avaliar a freqüência de DCR e SAS em pacientes com MC I ou II. MÉTODO: Utilizou-se a polissonografia de noite inteira para a avaliação de 103 pacientes. Classificaram-se as diferentes fases do sono e analisou-se a freqüência de movimentos anormais (monitorada por vídeo e de eventos respiratórios anormais. RESULTADOS: Dos 103 pacientes analisados, 36 eram portadores de MC I e 67 de MC II. Episódios de DCR foram observados em 23 pacientes. O índice de apnéia/hipopnéia foi considerado anormal em 65 pacientes. CONCLUSÃO: A alta freqüência de DCR e o aumento da freqüência de episódios de apnéia central do sono podem ser considerados manifestação de disfunção progressiva do tronco cerebral.

  13. Hydrodynamic study of syringomyelia by MRI and intraoperative ultrasonography

    Energy Technology Data Exchange (ETDEWEB)

    Moritake, Kouzo; Takaya, Mikio; Minamikawa, Jun; Ishikawa, Masatsune; Kikuchi, Haruhiko; Minami, Shunsuke (Kyoto Univ. (Japan). Faculty of Medicine)

    1989-08-01

    Syringomyelic cavities were studied with magnetic resonance imaging (MRI) in eleven patients with special reference to the hemodynamic contribution to the pathogenesis of syringomyelia. They were also studied intraoperatively with ultrasonography (USG) in five patients. Syrinx cavities combined with lumbosacral lipomyelomeningocele or with spinal stenosis did not present a flow-void phenomenon reflecting pulsatile movements of syrinx fluid. On serial MRI study in those the patients, enlargement of the syrinx cavity was not observed. Syrinx cavities occupying the caudal part of the spinal cord did not develop either. These cases were not treated surgically but followed conservatively. On the other hand, the flow-void sign in the syrinx cavities was present on MRI in patients who also had Chiari type I or type II malformations. They were treated with a syringo-subarachnoid shunt. In these cases, intraoperative USG disclosed marked fluctuation of syrinx cavity size synchronous with the motions of pulmonary ventilation. In all of them, clinical signs and symptoms improved postoperatively to various degrees. These results suggest that both the flow-void sign in the syrinx cavity on MRI and marked fluctaution of cavity size on intraoperative USG are indications for the shunt operation and support William's revised theory (1987). Fluctuation of cystic cavity size synchronous with ventilation suggests that venous pressure in the spinal subarachnoid space contributes to the pathogenesis of syringomyelic cavities. Further analysis of the fluctuation of cystic cavities by video monitoring will provide further information on the etiology and other clinical problems of syringomyelia. (author).

  14. Low field MRI in diagnosis of Arnold-Chiari malformation%Arnold-Arnold-Chiari畸形的MRI诊断

    Institute of Scientific and Technical Information of China (English)

    李仲华; 张华山; 黄明亮

    2010-01-01

    目的 探讨Arnold-Chiari畸形的MRI影像表现,评价MRI对Arnold-Chiari畸形的诊断价值.方法 回顾性分析MRI及手术证实的21例Arnold-Chiari畸形的MRI表现.结果 Arnold-Chiari畸形Ⅰ型16例,Ⅱ型4例,Ⅲ型1例,Ⅳ型病例未发现,17例伴有脊髓空洞.结论 MRI能清楚显示Arnold-Chiari畸形病灶的部位、形态及准确分型,是最好的影像学检查方法;脊髓空洞是Arnold-Chiari 畸形的主要合并症.

  15. Chiari畸形合并脊髓空洞症的手术治疗%Surgical treatment of Chiari malformation complicated by syringomyelia

    Institute of Scientific and Technical Information of China (English)

    李中林; 郭克勤; 于如同; 倪鸣山

    2004-01-01

    目的探讨治疗Chiari畸形合并脊髓空洞症的手术方法及其效果.方法对41例Chiari畸形合并脊髓空洞症病例采用不同手术方式进行治疗,31例行颅后窝减压术,10例行显微颅后窝减压+硬膜扩大成形术.结果采用颅后窝减压术的31例中有28例(占90.3%)症状好转,采用显微颅后窝减压+硬膜扩大成形术的10例症状均较术前明显好转.结论对Chiari畸形合并脊髓空洞症病例采用颅后窝减压术,疗效较满意;采用显微颅后窝减压+硬膜扩大成形术可提高疗效,减少并发症.

  16. Surgical treatment of 31 cases of chiari I malformation complicated with syringomyelia%Chiari畸形合并脊髓空洞症的手术治疗

    Institute of Scientific and Technical Information of China (English)

    李其林

    2008-01-01

    目的 探讨chiari畸形合并脊髓空洞症的手术方法及疗效.方法 对31例手术患者进行回顾分析,其中28例经MRI证实的chiari畸形合并脊髓空洞症患者行后路枕下减压及小脑扁桃体切除术,3例经MRI与颅骨X线证实的chiari畸形合并颅底凹陷患者行后路枕下减压及寰枕融合术并评定其疗效.结果 出院时患者症状明显改善或改善者27例,无改善但病情稳定者4例.随访26例患者,复查MRI脊髓空洞消失或基本消失20例,缩小3例,无变化3例.结论 后颅凹减压术,小脑扁桃体切除术,寰枕融合术及其不同组合是治疗chiari畸形合并脊髓空洞症的有效方法.

  17. 小脑扁桃体切除术治疗Chiari畸形合并脊髓空洞%Cerebellar Tonsil Resection Treating Chiari Malformation with Syringomyelia

    Institute of Scientific and Technical Information of China (English)

    罗学忠; 龙霄翱; 方琦; 陈兵

    2005-01-01

    目的探讨切除小脑扁桃体治疗Chiari畸形合并脊髓空洞的疗效.方法对21例Chiari畸形合并脊髓空洞的病人采用切除下疝的小脑扁桃体.结果随访复查17例病人中, MRI均显示枕大池重新形成,脊髓空洞明显缩小,其中3例消失.结论小脑扁桃体切除是治疗Chiari畸形合并脊髓空洞症的有效方法.

  18. Microsurgical Treatment of Arnold-chiari Malformation with Syringomyelia%Arnold-Chiari畸形并脊髓空洞症的显微外科手术治疗

    Institute of Scientific and Technical Information of China (English)

    马骏; 高方友; 刘窗溪; 韩国强; 熊云彪; 王俊

    2010-01-01

    目的 探讨Arnold-chiari畸形并脊髓空洞症的显微外科手术治疗方法.方法 对36例Arnold-chiari畸形并脊髓空洞症采取后颅窝减压术加下疝小脑扁桃体切除及枕大池成形术治疗,并对手术效果进行回顾性分析.结果 随访3~6个月,症状好转者34例,2例无明显加重;所有病例脊髓空洞均有明显缩小.结论 后颅窝减压术加下疝小脑扁桃体切除及枕大池成形术是治疗Arnold-chiari畸形有效的手术方式.

  19. Chiari畸形合并脊髓空洞症的改良锁孔手术%Micro-invasive and microsurgical treatment of Chiari malformation with syringomyelia

    Institute of Scientific and Technical Information of China (English)

    陈剑舞; 薛毅辉; 康德智; 王晨阳; 江常震

    2009-01-01

    @@ Chiari畸形是一组以后颅窝狭小伴小脑扁桃体下疝畸形为特点的先天发育异常性疾病,常合并脊髓空洞症.目前手术治疗方法较多,疗效差异较大[1].2005年1月-2008年12月,笔者科室收治Chiari畸形合并脊髓空洞症56例,采用后颅窝改良锁孔手术,行小骨窗减压及枕大池成形,治疗Chiari畸形合并脊髓空洞症18例,效果良好,现报道如下.

  20. Surgical treatment of Chiari malformation combined with syringomyelia%Chiari畸形合并脊髓空洞症的外科治疗

    Institute of Scientific and Technical Information of China (English)

    卞留贯

    2011-01-01

    Chiari畸形又称小脑扁桃体下疝畸形.John Cleland于1883年首次描述了脑干、小脑移位变形和延髓空洞,Chiari分别于1891年、1895年将颅-椎移行区神经轴下移的程度进行了分类,1894年德国病理学家Arnold又作了更为详尽的描述,因此,1907年Schwalb和Gredig将此命名为ArnoldChiari畸形,而Sarnat和Williams则称其为Chiari畸形或描述表达为小脑扁桃体下疝畸形[1].

  1. Study on motor evoked potential of Arnoid-Chiari malformation%运动诱发电位在 Arnoid-Chiari畸形诊治中的应用价值

    Institute of Scientific and Technical Information of China (English)

    徐伟; 徐启武; 李盛昌

    2001-01-01

    Objective To study the electrical stimulation motor evokedpotentials in Arnoid-Chiari malformation.Methods Electrical stimulation motor evoked potentials were recorded in 30 cases with Arnoid-Chiari malformation proved by MRI.Results Abnormality rate of MEP was 96.7%,and the abnormal patterns mainly consisted of the delay of latency and the delay of central motor conduction time(CMCT). The change of MEP was connected with myodynamia and the type of Arnoid-Chiari malformation .Conclusions The change of MEP is more sensitive than clinical change, and can indicate the damage of the motor function objectively and quantitatively, and MEP can be used as an objective criterion for surgical effect, and can predict the recovery of motor function.%目的 探讨Arnoid-Chiari畸形(ACM)患者运动诱发电位(MEP)的变化规律。方法 经MRI证实的30例Arnoid-Chiari畸形患者接受电刺激运动诱发电位测试,对其中20例进行术后复查。结果 Arnoid-Chiari畸形患者MEP的中枢运动传导时间(CMCT)延长,异常率为96.7%(29/30),肌力减退愈重,MEP异常愈明显,Arnoid-Chiari畸形Ⅱ型的MEP异常较Ⅰ型更显著,MEP的改变与肌力的改变明显相关。结论 MEP改变比临床评定更为敏感,且能反映运动功能的损害程度;MEP可预测预后,能作为评定手术效果的客观指标。

  2. Control study of early and late post-operative functional exercise in Arnold-Chiari malformation patients%Arnold-Chiari畸形患者术后早期与晚期功能锻炼的对照研究

    Institute of Scientific and Technical Information of China (English)

    殷淑珍; 梁艳

    2009-01-01

    目的 探讨Arnold-Chiari畸形患者术后早期或晚期功能锻炼对术后症状缓解和并发症发生的影响.方法 按术后是否早期下床活动,将74例Arnold-Chiari畸形患者分为早期活动组41例和晚期活动组33例.观察两组术后自觉症状、客观体征和护理指标.结果 2组间疼痛缓解、肌力改善、温度觉改善、肢体麻木缓解、压疮、头晕、泌尿系感染等差异有统计学意义,早期活动组优于晚期活动组.结论 Arnold-Chiari畸形患者术后早期进行功能锻炼,对缓解术后症状、减少患者并发症的发生有积极的影响.%Objective To study influence of early and late functional exercise on post-operative symptomatic relief and complication in Arnold-Chiari malformation patients. Methods A total of 74 Arnold-Chiari malformation patients were divided into two groups: early functional exercise(n=41) and late functional exercise(n=33). The result of the two groups were compared. Results Age and sex of the two groups had no significant difference. Pain relief, muscle force improvement, thermesthesia improvement, numbness relief and decubitus in the two groups had significantly different. Symptoms of patients who did early functional exercise relieved better. Conclusions Early post-operative functional exercise had beneficial effect on symptom relief and complication prevention in Arnold-Chiari malformation patients.

  3. Spontaneous drainage in syringomyelia: magnetic resonance imaging findings

    Energy Technology Data Exchange (ETDEWEB)

    Bogdanov, E.I.; Mendelevich, E.G. [Kazan State Medical Univ. (Russian Federation). Dept. of Neurology and Rehabilitation; Ibatullin, M.M. [Republic Medical Diagnostic Centre of Tatarstan, Kazan (Russian Federation). Department of Radiology

    2000-09-01

    We present five cases of syringomyelia associated with Chiari I or other causes of partial obstructions at the cervicomedullary junction, with spontaneous disruption of the wall of a cervical syrinx and formation of a communication between the cavity and the subarachnoid space, shown on axial MRI. MRI can be used to investigate the hydrodynamics, showing the liquid inside the disrupted syrinx wall and the pathway of drainage. The finding of spontaneous drainage may be important for understanding the pathogenesis of syringomyelia and may be helpful for choosing a surgical approach. (orig.)

  4. Clinical significance of changes in pB-C2 distance in patients with Chiari Type I malformations following posterior fossa decompression: a single-institution experience.

    Science.gov (United States)

    Bonney, Phillip A; Maurer, Adrian J; Cheema, Ahmed A; Duong, Quyen; Glenn, Chad A; Safavi-Abbasi, Sam; Stoner, Julie A; Mapstone, Timothy B

    2016-03-01

    OBJECT The coexistence of Chiari malformation Type I (CM-I) and ventral brainstem compression (VBSC) has been well documented, but the change in VBSC after posterior fossa decompression (PFD) has undergone little investigation. In this study the authors evaluated VBSC in patients with CM-I and determined the change in VBSC after PFD, correlating changes in VBSC with clinical status and the need for further intervention. METHODS Patients who underwent PFD for CM-I by the senior author from November 2005 to January 2013 with complete radiological records were included in the analysis. The following data were obtained: objective measure of VBSC (pB-C2 distance); relationship of odontoid to Chamberlain's, McGregor's, McRae's, and Wackenheim's lines; clival length; foramen magnum diameter; and basal angle. Statistical analyses were performed using paired t-tests and a mixed-effects ANOVA model. RESULTS Thirty-one patients were included in the analysis. The mean age of the cohort was 10.0 years. There was a small but statistically significant increase in pB-C2 postoperatively (0.5 mm, p < 0.0001, mixed-effects ANOVA). Eleven patients had postoperative pB-C2 values greater than 9 mm. The mean distance from the odontoid tip to Wackenheim's line did not change after PFD, signifying postoperative occipitocervical stability. No patients underwent transoral odontoidectomy or occipitocervical fusion. No patients experienced clinical deterioration after PFD. CONCLUSIONS The increase in pB-C2 in patients undergoing PFD may occur as a result of releasing the posterior vector on the ventral dura, allowing it to relax posteriorly. This increase appears to be well-tolerated, and a postoperative pB-C2 measurement of more than 9 mm in light of stable craniocervical metrics and a nonworsened clinical examination does not warrant further intervention.

  5. The formation of the foramen magnum and its role in developing ventriculomegaly and Chiari I malformation in children with craniosynostosis syndromes.

    Science.gov (United States)

    Rijken, Bianca F M; Lequin, Maarten H; Van Veelen, Marie-Lise C; de Rooi, Johan; Mathijssen, Irene M J

    2015-09-01

    Craniosynostosis syndromes are characterized by prematurely fused skull sutures, however, less is known about skull base synchondroses. This study evaluates how foramen magnum (FM) size, and closure of its intra-occipital synchondroses (IOS) differ between patients with different craniosynostosis syndromes and control subjects; and whether this correlates to ventriculomegaly and/or Chiari malformation type I (CMI), intracranial disturbances often described in these patients. Surface area and anterior-posterior (A-P) diameter were measured in 175 3D-CT scans of 113 craniosynostosis patients, and in 53 controls (0-10 years old). Scans were aligned in a 3D multiplane-platform. The frontal and occipital horn ratio was used as an indicator of ventricular volume, and the occurrence of CMI was recorded. Synchondroses were studied in scans with a slice thickness ≤1.25 mm. A generalized linear mixed model and a repeated measures ordinal logistic regression model were used to study differences. At birth, patients with craniosynostosis syndromes have a smaller FM than controls (p < 0.05). This is not related to the presence of CMI (p = 0.36). In Crouzon-Pfeiffer patients the anterior and posterior IOS fused prematurely (p < 0.01), and in Apert patients only the posterior IOS fused prematurely (p = 0.028). The FM is smaller in patients with craniosynostosis syndromes than in controls, and is already smaller at birth. In addition to the timing of IOS closure, other factors may influence FM size. Copyright © 2015 European Association for Cranio-Maxillo-Facial Surgery. Published by Elsevier Ltd. All rights reserved.

  6. Exome sequencing of two Italian pedigrees with non-isolated Chiari malformation type I reveals candidate genes for cranio-facial development.

    Science.gov (United States)

    Merello, Elisa; Tattini, Lorenzo; Magi, Alberto; Accogli, Andrea; Piatelli, Gianluca; Pavanello, Marco; Tortora, Domenico; Cama, Armando; Kibar, Zoha; Capra, Valeria; De Marco, Patrizia

    2017-08-01

    Chiari malformation type I (CMI) is a congenital abnormality of the cranio-cerebral junction with an estimated incidence of 1 in 1280. CMI is characterized by underdevelopment of the occipital bone and posterior fossa (PF) and consequent cerebellar tonsil herniation. The presence for a genetic basis to CMI is supported by many lines of evidence. The cellular and molecular mechanisms leading to CM1 are poorly understood. The occipital bone formation is dependent on complex interactions between genes and molecules with pathologies resulting from disruption of this delicate process. Whole-exome sequencing of affected and not affected individuals from two Italian families with non-isolated CMI was undertaken. Single-nucleotide and short insertion-deletion variants were prioritized using KGGSeq knowledge-based platform. We identified three heterozygous missense variants: DKK1 c.121G>A (p.(A41T)) in the first family, and the LRP4 c.2552C>G (p.(T851R)) and BMP1 c.941G>A (p.(R314H)) in the second family. The variants were located at highly conserved residues, segregated with the disease, but they were not observed in 100 unaffected in-house controls. DKK1 encodes for a potent soluble WNT inhibitor that binds to LRP5 and LRP6, and is itself regulated by bone morphogenetic proteins (BMPs). DKK1 is required for embryonic head development and patterning. LRP4 is a novel osteoblast expressed receptor for DKK1 and a WNT and BMP 4 pathways integrator. Screening of DKK1 in a cohort of 65 CMI sporadic patients identified another missense variant, the c.359G>T (p.(R120L)), in two unrelated patients. These findings implicated the WNT signaling in the correct development of the cranial mesenchyme originating the PF.

  7. Tonsillar pulsatility before and after surgical decompression for children with Chiari malformation type 1: an application for true fast imaging with steady state precession

    Energy Technology Data Exchange (ETDEWEB)

    Radmanesh, Alireza [University of California, San Francisco, Department of Radiology and Biomedical Imaging, San Francisco, CA (United States); Washington University School of Medicine, Mallinckrodt Institute of Radiology, St Louis, MO (United States); Greenberg, Jacob K.; Smyth, Matthew D.; Limbrick, David D. [Washington University School of Medicine, Department of Neurosurgery, St Louis, MO (United States); Chatterjee, Arindam; Sharma, Aseem [Washington University School of Medicine, Mallinckrodt Institute of Radiology, St Louis, MO (United States)

    2015-04-01

    We hypothesize that surgical decompression for Chiari malformation type 1 (CM-1) is associated with statistically significant decrease in tonsillar pulsatility and that the degree of pulsatility can be reliably assessed regardless of the experience level of the reader. An Institutional Review Board (IRB)-approved Health Insurance Portability and Accountability Act (HIPAA)-compliant retrospective study was performed on 22 children with CM-1 (8 males; mean age 11.4 years) who had cardiac-gated true-FISP sequence and phase-contrast cerebrospinal fluid (CSF) flow imaging as parts of routine magnetic resonance (MR) imaging before and after surgical decompression. The surgical technique (decompression with or without duraplasty) was recorded for each patient. Three independent radiologists with different experience levels assessed tonsillar pulsatility qualitatively and quantitatively and assessed peritonsillar CSF flow qualitatively. Results were analyzed. To evaluate reliability, Fleiss kappa for multiple raters on categorical variables and intra-class correlation for agreement in pulsatility ratings were calculated. After surgical decompression, the degree of tonsillar pulsatility appreciably decreased, confirmed by t test, both qualitatively (p values <0.001, <0.001, and 0.045 for three readers) and quantitatively (amount of decrease/p value for three readers 0.7 mm/<0.001, 0.7 mm/<0.001, and 0.5 mm/0.022). There was a better agreement among the readers in quantitative assessment of tonsillar pulsatility (kappa 0.753-0.834), compared to qualitative assessment of pulsatility (kappa 0.472-0.496) and qualitative assessment of flow (kappa 0.056 to 0.203). Posterior fossa decompression with duraplasty led to a larger decrease in tonsillar pulsatility, compared to posterior fossa decompression alone. Tonsillar pulsatility in CM-1 is significantly reduced after surgical decompression. Quantitative assessment of tonsillar pulsatility was more reliable across readers than

  8. Cervical spinal canal narrowing in idiopathic syringomyelia

    Energy Technology Data Exchange (ETDEWEB)

    Struck, Aaron F. [Massachusetts General Hospital, Department of Neurology, Boston, MA (United States); Carr, Carrie M. [Mayo Clinic, Department of Radiology, Rochester, MN (United States); Shah, Vinil [University of California San Francisco, Department of Radiology, San Francisco, CA (United States); Hesselink, John R. [University of California San Diego, Department of Radiology, San Diego, CA (United States); Haughton, Victor M. [University of Wisconsin, Department of Radiology, Madison, WI (United States)

    2016-08-15

    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. (orig.)

  9. Poro-elastic modeling of Syringomyelia - a systematic study of the effects of pia mater, central canal, median fissure, white and gray matter on pressure wave propagation and fluid movement within the cervical spinal cord.

    Science.gov (United States)

    Støverud, Karen H; Alnæs, Martin; Langtangen, Hans Petter; Haughton, Victor; Mardal, Kent-André

    2016-01-01

    Syringomyelia, fluid-filled cavities within the spinal cord, occurs frequently in association with a Chiari I malformation and produces some of its most severe neurological symptoms. The exact mechanism causing syringomyelia remains unknown. Since syringomyelia occurs frequently in association with obstructed cerebrospinal fluid (CSF) flow, it has been hypothesized that syrinx formation is mechanically driven. In this study we model the spinal cord tissue either as a poro-elastic medium or as a solid linear elastic medium, and simulate the propagation of pressure waves through an anatomically plausible 3D geometry, with boundary conditions based on in vivo CSF pressure measurements. Then various anatomic and tissue properties are modified, resulting in a total of 11 variations of the model that are compared. The results show that an open segment of the central canal and a stiff pia (relative to the cord) both increase the radial pressure gradients and enhance interstitial fluid flow in the central canal. The anterior median fissure, anisotropic permeability of the white matter, and Poisson ratio play minor roles.

  10. Posterion fossa decompression for Chiari I malformation with hydrocephalus:a meta-analysis%后颅窝减压术治疗Chiari畸形Ⅰ型合并脑积水疗效的Meta分析

    Institute of Scientific and Technical Information of China (English)

    张家康; 彭飞; 陈鑫; 侯旭; 张大明; 刘震东; 赵世光

    2016-01-01

    Objective To evaluate the effect of posterior fossa decompression (PFD) on ChiariⅠmalformation associated with hydrocephalus. Methods The data which accorded with the demands of the present study were individually extracted the Chinese and English literature obtained by searching electronic database including EBSCO, PUBMED, EMBASE, CNKI, VIP, Wanfang and OVID from the day when the library was built to 31 May 2015. All the statistical analyses were performed by the STATA software version 13. Results The rate of improvement of Chiari I malformation associated with hydrocephalus was 82.3% (95% CI 74.2%~90.4%). The female patients account for 62.9%(95%CI 52.6%~73.1%)of all the patients with Chiari I malformation associated with hydrocephalus. Conclusion The female patients account for the majority of all the patients with Chiari malformation associated with hydrocephalus, and PFD is an effective method to treat this disease.%目的:评价后颅窝减压术(PFD)治疗合并脑积水的Chiari畸形Ⅰ型的有效性。方法计算机检索EBSCO、PubMed、EmBase、OVID等英文数据库以及中国知网、维普、万方等中文数据库,检索从建库到2015年5月31日发表的文献。采用STATA 13.0软件进行Meta分析。结果共纳入3篇文献,86例,PFD治疗83例;PFD术后好转率为82.3%(95%CI 74.2%~90.4%);合并脑积水的Chiari畸形Ⅰ型中,女性占62.9%(95%CI 52.6%~73.1%)。结论合并脑积水的Chiari畸形Ⅰ型中,女性占大多数;PFD治疗合并脑积水的Chiari畸形Ⅰ型具有较高的有效率。

  11. The Reinvestigation of Diagnostic Standard of Chiari Type Ⅰ Malformation%ChiariⅠ型畸形的诊断标准再探讨

    Institute of Scientific and Technical Information of China (English)

    王梅云; 戴建平; 高培毅; 程敬亮; 李树新

    2005-01-01

    目的采用新方法再探讨Chiari Ⅰ型畸形的诊断标准.方法利用医用计算机图像处理软件OSIRIS,对27例小脑扁桃体下疝超过5 mm的Chiari Ⅰ型畸形病例、25例小脑扁桃体下疝<3 mm的可疑Chiari Ⅰ型畸形病例以及40例不伴中枢神经系统疾病的患者的头颅MRI图像分别进行颅腔和脑实质的径线与面积的测量,并对测量结果作统计学分析.所选病人均在18岁以上,MRI资料完整,不伴颅内高压、环枕融合和颅底凹陷.结果Chiari Ⅰ型畸形可疑组的枕底长与斜坡长的比值明显大于Chiari Ⅰ型畸形组(P<0.05),后脑面积与后颅窝面积的比值明显小于Chiari Ⅰ型畸形组(P<0.05),而这2项测量结果与对照组之间差异无显著性(P>0.05);此外,可疑组的后脑面积与大脑面积及大脑面积与幕上颅腔面积的比值与对照组和Chiari Ⅰ型畸形组间差异均无显著性(P>0.05).结论Chiari Ⅰ型畸形以小脑扁桃体下疝超过5 mm为诊断标准较为合理,下疝<3 mm应视为正常.该研究结果也支持后颅窝骨发育不良导致后颅窝过度拥挤是Chiari Ⅰ型畸形发病机制的学说.

  12. Chiari Ⅰ型畸形的发病机制探讨及测量方法比较%Investigation of the Pathogenesis and Craniometry of Chiari Type Ⅰ Malformation

    Institute of Scientific and Technical Information of China (English)

    王梅云; 李树新; 戴建平; 程敬亮; 任翠萍; 张焱

    2003-01-01

    目的探讨Chiari Ⅰ型畸形的发病机制并对测量方法进行比较.资料与方法利用医用计算机图像处理软件OSIRIS对年龄在18岁以上、MRI资料完整、不伴颅内高压、环枕融合和颅底凹陷的27例Chiari Ⅰ型畸形病例及年龄在18岁以上、不伴中枢神经系统疾病的40例(对照组)的头颅MR图像分别进行颅腔和脑实质的径线、角度与面积的测量,并对测量结果作统计学分析.结果 Chiari Ⅰ型畸形组的枕底长与斜坡长的比值明显小于对照组,后脑面积与后颅窝面积的比值明显大于对照组,而后颅面积与全颅面积的比值明显小于对照组(P<0.05).结论 Chiari Ⅰ型畸形的最可能的发病机制为胚胎枕节发育不良导致后颅窝狭小,难以完全容纳正常发育的后脑,使小脑扁桃体向下疝入椎管.采用长径和宽径的乘积代表不规则颅腔面积的研究方法具有可参照性.

  13. Expansive posterior fossa eranioplnsty with occipital flip for arnold-chiari Ⅰ malforma-tion%自体骨瓣后颅窝扩大成形治疗Chiari Ⅰ型畸形(附9例临床分析)

    Institute of Scientific and Technical Information of China (English)

    张亚召; 蔡恩源; 李盛善; 唐兆伟; 李海校

    2013-01-01

    Objective To explore the treatment of expansive posterior fossa cranioplasty with occipital flips for Arnold-chiari I malformation. Methods Methods 9 cases of the patients with Arnold-chiari I malformation all use surgical treatment, do the decompression of posterior cranial fossa,duraplastic repair, resection of hernia below cerebellar tonsil and separation of adherence of arachnoid membrane. Results Postoperative MRI studies revealed that cerebellar ton-sillar lower edge of the cases are rising to the foramen magnum level above, the posterior fossa volume increase,8 cases symptoms improved significantly, 1 cases no improvement. Follow-up of 3-18 months, all patients got good clinical outcomes with symptoms relief. MRI showed sy-ringomyelia disappeared in 2cases, significantly thinned in 5 cases, no improvem. Conclusion Conclusion Expansive posterior fossa cranioplasty using occipital flip is effective for arnold-chiari I malformation.%目的 探讨利用自体骨瓣进行后颅窝扩大成形术治疗Chiari Ⅰ型畸形的临床效果.方法 本组9例Chiari Ⅰ型患者均采用手术治疗,行后颅窝自体骨骨瓣扩大成形、硬脑膜成形、小脑扁桃体切除及粘连蛛网膜分解术.结果 9例患者术后均复查MRI示小脑扁桃体下缘均上升到枕骨大孔水平以上,后颅窝容积增大.8例症状改善明显,1例改善不明显.随访3~18个月,所有病例症状体征较术前改善.MRI示7例合并脊髓空洞患者中,2例空洞消失,5例脊髓空洞明显缩小.结论 自体骨瓣后颅窝扩大成形治疗Chiari Ⅰ畸形患者,临床症状改善,效果满意.

  14. Case Report: Acute obstructive hydrocephalus associated with infratentorial extra-axial fluid collection following foramen magnum decompression and durotomy for Chiari malformation type I [version 1; referees: 2 approved

    Directory of Open Access Journals (Sweden)

    Sunil Munakomi

    2016-01-01

    Full Text Available Acute obstructive hydrocephalus due to infratentorial extra-axial fluid collection (EAFC is an extremely rare complication of foramen magnum decompression (FMD and durotomy for Chiari malformation type I. Presence of infratentorial  EAFC invariably causes obstruction at the level of the fourth ventricle or aqueduct of Silvius, thereby indicating its definitive role in hydrocephalus. Pathogenesis of EAFC is said to be a local arachnoid tear as a result of durotomy, as this complication is not described in FMD without durotomy. Controversy exists in management. Usually EAFC is said to resolve with conservative management; so hydrocephalus doesn’t require treatment. However, in this case EAFC was progressive and ventriculo-peritoneal shunting (VPS was needed for managing progressive and symptomatic hydrocephalus.

  15. Chiari Ⅰ型畸形并发左肩Charcot关节1例并文献复习%Chiari I type malformation complicated by Charcot joint of the left shoulder

    Institute of Scientific and Technical Information of China (English)

    何丽; 任庆云; 王欣; 刘斋

    2012-01-01

    @@ Charcot关节又称神经性关节病,是一种罕见的神经感觉和神经营养障碍的破坏性关节病.笔者报道本院收治的Chiari Ⅰ型畸形伴有脊髓空洞症继发左肩Charcot关节1例,并结合文献进行分析,以提高对Charcot关节的认识.

  16. Chiari I型畸形合并脊髓空洞症的术后护理

    Institute of Scientific and Technical Information of China (English)

    张霞

    2013-01-01

    Arnold--chiari畸形(Arnold-chiari malformation;ACM)也称小脑扁桃体下疝畸形,是一种以小脑扁桃体下疝入枕骨大孔为主要特征的先天性疾病,主要表现延髓、上颈髓受压,颅神经、颈神经以及小脑受累和高颅压的症状和体征.脊髓空洞症(Syringomyelia;SM)是其最常见的并发症[1].我科自2003年6月~2011年6月共收治经MRI证实的15例Chiari I型畸形合并脊髓空洞症患者采用后颅窝减压、小脑延髓池扩大成形加空洞-蛛网膜下隙分流术,通过加强术后护理,疗效满意,现将护理体会报告如下

  17. Suboccipital craniectomy with opening of the fourth ventricle and duraplasty: study of 192 cases of craniovertebral malformations

    Directory of Open Access Journals (Sweden)

    Jose Alberto Goncalves da Silva

    2013-09-01

    Full Text Available The prime objective in the surgical treatment of basilar impression (BI, Chiari malformation (CM, and/or syringomyelia (SM is based on restoration of the normal cerebrospinal fluid (CSF dynamics at the craniovertebral junction and creation of a large artificial cisterna magna, avoiding the caudal migration of the hindbrain. It is observed that a large craniectomy might facilitate an upward migration of the posterior fossa structures. There are many surgical techniques to decompress the posterior fossa; however, a gold standard approach remains unclear. The authors present the results of 192 cases of BI, CM, and SM treated between 1975 and 2008 and whose surgical treatment was characterized by a large craniectomy without tonsillectomy with the patient in the sitting position, large opening of the fourth ventricle, and duraplasty.

  18. CT myelography in communicating syringomyelia

    Energy Technology Data Exchange (ETDEWEB)

    Pamir, M.N.; Ozer, A.F.; Zirh, T.A. (Marmara University Faculty of Medicine, Istanbul (Turkey). Department of Neurosurgery); Gurmen, N.; Erzen, C. (Marmara University, Istanbul (Turkey). Department of Radiology)

    Although the etiology of syringomyelia is not clearly understood, many surgical methods have been proposed for its treatment. One widely used technique in cases of communicating syringomyelia is that of posterior fossa decompression and plugging of the obex (Gardner's Operation). In this paper 5 cases of syringomyelia are presented which were investigated using detailed myelo-computerized tomographic techniques, of which 2 appeared to be communicating syringomyelia and which were treated by posterior fossa decompression and obex plugging. The authors also discuss the place of computed tomography in the differential diagnosis of communicating syringomyelia. (author). 42 refs.; 3 figs.; 2 tabs.

  19. 首诊睡眠呼吸暂停综合征的Chiari畸形1例%Chiari malformation presenting as sleep apnea syndrome:case report

    Institute of Scientific and Technical Information of China (English)

    袁伟锋; 徐虹; 黄文杰

    2009-01-01

    Chiari畸形(Chiari malformation,CM)是一种先天性小脑扁桃体延髓联合畸形,表现为小脑扁桃体楔形延长,伸入枕骨大孔而达颈椎管内,后组颅神经、上部颈神经根受牵拉下移而产生相应症状,主要表现为:(1)延髓及上颈段症状;(2)后组神经损害症状;(3)小脑症状;(4)高颅压症状。CM尚有其他临床表现,如小脑扁桃体疝出压迫延髓所致的睡眠呼吸暂停。

  20. Sleep disorder: a possible cause of attention deficit in children and adolescents with Chiari malformation type II Distúrbios do sono: possível causa de déficit de atenção em crianças e adolescentes com malformação de Chiari tipo II

    Directory of Open Access Journals (Sweden)

    Paulo Sérgio Azeredo Henriques Filho

    2009-03-01

    Full Text Available BACKGROUND: Attention deficit may be related to sleep disorders in Chiari malformation type II (CMII. Our aim is identify sleep disorders and their specific contribution in attention deficit. METHOD: We selected 24 patients with CM II and 24 without CM II. DSM-IV criteria and a neuropsychological analysis were applied in all. All patients underwent full night polysomnography. RESULTS: 14 CM II patients presented sleep apnea syndrome, REM sleep behavior disorder and periodic limb movement in sleep; six patients without CM II presented sleep apnea syndrome. Among these patients, 12 (six with CM II and six without CM II presented attention deficit related to the sleep disorders. CONCLUSION: Sleep disorders may impair cognitive functions, as attention, and contribute to poor quality of learning also in patients with CM II.INTRODUÇÃO: Déficits de atenção podem estar relacionados a distúrbios do sono em indivíduos com malformação de Chiari tipo II (CM II. Nosso objetivo é identificar distúrbios do sono e sua contribuição para a ocorrência de déficit de atenção. MÉTODO: Foram selecionados 24 pacientes com CM II e 24 sem CM II. Todos foram submetidos à avaliação neuropsicológica, aos critérios do DSM-IV e a polissonografia. RESULTADOS: 14 pacientes com CM II apresentaram síndrome da apnéia do sono, distúrbio do comportamento da fase do sono REM e movimentos periódicos dos membros em sono; seis pacientes sem CM II apresentaram síndrome da apnéia do sono. Entre estes pacientes, 12 (seis com CM II e seis sem CM II apresentaram déficit de atenção relacionado a distúrbios do sono. CONCLUSÃO: Distúrbios do sono podem prejudicar funções cognitivas, como a atenção, contribuindo para a piora da qualidade de aprendizado também em pacientes com CM II.

  1. 后颅窝减压及硬膜成形术与单纯后颅窝减压术比较治疗Ⅰ型Chiari畸形效果的Meta分析%Comparison of Posterior Fossa Decompression with and without Duraplasty for Surgical Treatment of Chiari Malformation Type Ⅰ: A Meta-Analysis

    Institute of Scientific and Technical Information of China (English)

    张豆豆; 陈海锋; 黄思庆; 郝德; 任海波; 汤俊佳

    2012-01-01

    Objective To systematically evaluate posterior fossa decompression with duraplasty (PFDD) and posterior fossa decompression without duraplasty (PFD) for Chiari malformation type I (CM-I). Methods A meta-analysis was performed according to the guideline of the MOOSE statement. Relevant literature and references were electronically searched in CENTRAL, Science Citation Index Expanded, MEDLINE (Ovid), PubMed, CBM, CNKI and WanFang Data from 1993 to September 2011. Two reviewers independently identified literature according to inclusion and exclusion criteria. The included studies were evaluated using the Newcastle-Ottawa Scale. Original data were extracted and analyzed using RevMan 5.1 software. Besides, the level of evidence was assessed using the GRADE system. Results Ten studies involving 829 patients were included. The results of meta-analyses showed that: a) compared with patients undergoing PFD, patients undergoing PFDD had a significantly lower reoperation rate (RR=0.41, 95%CI 0.23 to 0.74, P=0.003), and a higher rate of syringomyelia decrease (RR=1.27, 95%CI 1.03 to 1.56, P=0.02). But there was no significant difference in clinical improvement (RR=1.11, 95%CI 0.95 to 1.28, P=0.18). b) compared with PFD, patients undergoing PFDD had a higher rate of cerebrospinal fluid-related complications (RR=6.3, 95%CI 2.71 to 14.67, P0.05). Based on GRADE system, the evidence was at Level C and we made a weak recommendation. Conclusion Posterior fossa decompression with duraplasty is associated with a lower risk of reoperation, a better effect of syringomyelia decrease and a greater risk for cerebrospinal fluid-related complications, compared with PFD. Due to the influencing factors of lower-quality included studies, a prudent choice is suggested, and also more high-quality, large-sample studies are need.%目的 系统评价后颅窝减压及硬膜成形术(PFDD)与单纯后颅窝减压(PFD)两种术式比较治疗Ⅰ型Chiari畸形的有效性和安全性.方法

  2. 青少年Chiari畸形伴胸椎侧凸远端融合范围的选择及疗效比较%Selection of the lower instrumented vertebra in adolescents with thoracic scoliosis secondary to Chiari malformation

    Institute of Scientific and Technical Information of China (English)

    朱泽章; 江龙; 邱勇; 刘臻; 钱邦平; 吴涛; 伍伟飞; 闫煌

    2013-01-01

    目的 探讨青少年Chiari畸形伴胸椎侧凸不同远端固定节段的选择对手术疗效的影响.方法 后路选择性胸椎融合术且随访超过2年的青少年共27例,男11例,女16例;年龄为12~18岁,平均15.2岁.记录术前、术后以及末次随访时的临床资料及影像学指标:冠状面侧凸Cobb角、侧凸柔韧性、顶椎偏移(apical vertebral translation,AVT)、顶椎旋转(apical vertebral rotation,AVR)及躯干偏移;矢状面胸椎后凸角(thoracic kyphosis,TK)、胸腰段Cobb角(thoracolumbar kyphosis,TLK)、腰椎前凸角(lumbar lordosis,LL)及躯干偏移(sagittal vertical axis,SVA),并进行统计学分析.结果 随访时间2~7年,平均3.4年.末次随访时的胸弯矫正率为55.9%,矫正丢失率2.3%,腰弯自发矫正率为59.2%.术前腰弯修正为A型与B型两组患者末次随访时各项影像学指标的差异均无统计学意义.除腰弯修正为B型且远端固定椎为L1的病例末次随访时的平均LL(59.8°)明显大于L2者(40.8°)外,A型及B型病例中远端固定椎位于L1或L2者其他各项影像学指标的差异均无统计学意义.结论 术前腰弯修正为A型及B型的青少年Chiari畸形伴胸椎侧凸患者采用后路选择性胸椎融合术治疗,可获得满意的胸弯矫正及腰弯的自发性矫正.%Objective To investigate the effect of lowest instrumented vertebrae (LIV) selection on clinical outcome of posterior thoracic fusion in adolescents with thoracic scoliosis secondary to Chiari malformation.Methods A total of 27 adolescents with Chiari malformation-associated scoliosis,who had undergone posterior thoracic fusion and been followed up for at least 2 years,were enrolled in this study.There were 11 males and 16 females,with an average age of 15.2 years (range,12 to 18 years).The following radiographic parameters:coronal Cobb angle,curve flexibility,apical vertebral translation (AVT),apical vertebral rotation (AVR),trunk shift (C7PL-CSVL distance

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

    Directory of Open Access Journals (Sweden)

    Schmidt Martin

    2012-09-01

    Full Text Available Abstract 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 3; median right JF: 0.0434 cm3; p 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.

  4. Spinal syringomyelia following subarachnoid hemorrhage.

    Science.gov (United States)

    Nakanishi, Kinya; Uchiyama, Takuya; Nakano, Naoki; Fukawa, Norihito; Yamada, Kimito; Yabuuchi, Tomonari; Kato, Amami

    2012-04-01

    Subarachnoid blood has been reported as a cause of chronic spinal arachnoiditis. Although syringomyelia has been thought to be caused by spinal arachnoiditis, reports of syringomyelia following aneurysmal subarachnoid hemorrhage (SAH) are very rare. We describe two patients with syringomyelia associated with chronic spinal arachnoiditis following SAH. From January 2001 to December 2010, 198 patients with aneurysmal SAH were treated at Kinki University School of Medicine. Two of the 198 patients had syringomyelia following aneurysmal SAH; thus the rate of syringomyelia associated with aneurysmal SAH was 1.0%. Patient 1 was a 54-year-old woman who presented with back pain, back numbness and gait disturbance 20 months after SAH. Her MRI revealed syringomyelia of the spinal cord from C2 to T10. She underwent shunting of the syrinx to the subarachnoid space. Patient 2 was a 49-year-old man, who was admitted to the hospital with headache, diplopia, hoarseness, dysphagia and ataxia five months after SAH. MRI revealed syringomyelia from the medulla oblongata to C6, and an enlargement of the lateral and fourth ventricles. After foramen magnum decompression and C1 laminectomy, a fourth ventricle-subarachnoid shunt was placed by insertion of a catheter. Spinal arachnoiditis and spinal syringomyelia are rare but important chronic complications after SAH.

  5. Metrizamide computed tomography in syringomyelia

    Energy Technology Data Exchange (ETDEWEB)

    Ogawa, T.; Tamakawa, Y.; Arii, H. (Akita Univ. (Japan). Faculty of Medicine); Takahashi, M.; Hirota, K.

    1981-11-01

    Serial computed tomography of the cervical cord was performed following metrizamide myelography in five cases of clinically suspected syringomyelia. The syrinx filled with refluxed metrizamide was demonstrated in all of the cases. The reflux of metrizamide into the syrinx was most marked several hours following intrathecal injection of metrizamide. Computed tomography combined with metrizamide myelography is essential in the diagnosis of communicating syringomyelia.

  6. 枕颈减压、脊髓空洞穿刺抽液治疗Chiari畸形合并脊髓空洞症%Occipitocervical Decompression Combined with Drainage for Treatment of Chiari Malformation Accompanied by Syringomyelia

    Institute of Scientific and Technical Information of China (English)

    李元光; 刘炳存; 李红喜

    2001-01-01

    目的探讨一种治疗Chiari畸形合并脊髓空洞症的新手术方法.方法 61例经MRI诊断为Chiari畸形合并脊髓空洞症患者,均采用枕颈减压加脊髓空洞穿刺抽液术治疗.结果全部病例术后临床症状、体征均有不同程度改善;随访48例(术后1~5年),明显好转45例,好转3例;其中8例术后2~4年经MRI复查,脊髓空洞消失2例,空洞明显缩小5例,轻微缩小1例;手术无并发症,无死亡.结论枕颈减压加脊髓空洞穿刺抽液术治疗Chiari畸形合并脊髓空洞症是一种有效的方法.

  7. 不同术式对Arnold-Chiari畸形并脊髓空洞的疗效分析%Effects of two different operations on Arnold-Chiari malformation I and syringomyelia

    Institute of Scientific and Technical Information of China (English)

    张斌; 沈冰

    2009-01-01

    目的 比较分析两种手术方式治疗Chiari畸形并脊髓空洞的疗效.方法 25例Chiari畸形I型并脊髓空洞症患者均行后颅窝减压术,其中未开放硬膜12例,开放硬膜13例.结果 25例随访时间6个月-6年,平均3.6年,症状均有不同程度改善,两组比较差异无统计学意义(Z=-0.653,P>0.05).结论 不开放硬膜与开放硬膜手术均可达到后颅窝减压目的.

  8. Arnoid-Chiari畸形合并脊髓空洞症外科治疗(附27例)%Surgical Treatment of Arnoid-Chiari Malformation Combined with Syringomyelia

    Institute of Scientific and Technical Information of China (English)

    李均辉; 杨遇春; 叶大公; 青松文

    2002-01-01

    目的:探讨Arnoid-Chiari畸形合并脊髓空洞症(ACM/SM)的外科治疗方法.方法:在27例患者中,对单纯性ACM者,行枕下颅骨切除及硬膜扩大修补术;ACM/SM者,行以上手术同时行空洞与蛛网膜下腔分流术.结果:大部分患者症状消除或缓解,手术有效率为92.6%.结论:外科治疗对ACM及ACM/SM不失为一种有效的治疗方法.

  9. Radiotherapeutic problems in syringomyelia

    Energy Technology Data Exchange (ETDEWEB)

    Haas, J.P.

    1982-10-11

    The paper deals first with questions about the aetiology and pathogenesis, symptomatology and course of the syringomyelia. The discordant results of the radiation therapy as published since 1905 are discussed (basing on a comprehensive literature review). Today only the radiation quality of high-energy can be considered. The calculation of dose distribution must be done with a therapy treatment computer and on the basis of a CT-Scan. A survey is given about late radiation damages of the skin, the bones, the spinal cord and other neighbouring organs. The present knowledge of the radiation tolerance of the spinal cord is discussed in respect with this point of interest.

  10. Post-traumatic syringomyelia

    Directory of Open Access Journals (Sweden)

    Agrawal Amit

    2007-01-01

    Full Text Available Progressive post-traumatic cystic syringomyelia is an uncommon and increasingly recognized cause of morbidity following spinal cord injury. We hereby report a 35-year-old gentleman who sustained wedge compression fracture of L-1 vertebral body 15 years back and had complete paraplegia with bowel/bladder involvement. The neurological deficit recovered with minimal residual motor deficits and erectile dysfunction. He presented now with increasing neurological deficits associated with pain and paresthesia. The MRI spine showed a syrinx extending from the site of injury up to the medulla. He underwent a syringo-peritoneal shunt and at follow-up his pain and motor functions had improved but erectile dysfunction was persisting.

  11. 脊髓空洞症合并Charcot关节病1例并文献复习%Syringomyelia combined with Charcot joint disease( a report of 1 case and review of literature)

    Institute of Scientific and Technical Information of China (English)

    王胥; 阴鲁鑫; 高文昌; 周秀萍; 于如同

    2016-01-01

    目的 报告1例脊髓空洞症(SM)合并Charcot关节病(CJ),通过文献复习的方法提高对该病在诊断及治疗上的认识.方法 通过回顾该疾病的临床表现及典型的影像学特点,结合相关文献以求提高诊断正确率.结果 该病例左肘部为典型的Charcot关节病表现,颈椎MRI确诊为Chiari畸形合并脊髓空洞症.结论 临床表现及影像学特点是诊断该病的主要依据,目前尚无有效治疗手段,手术治疗有一定疗效.%Objective To report one case of syringomyelia ( SM ) combined with Charcot joint disease ( CJ ) , further improving diagnosis and treatment of the disease by the method of literature review.Method By reviewing the clinical features of the disease and the typical imaging features, in combination with related literature in order to improve diagnostic accuracy.Results The clinical diagnosis of the patient's left elbow sickness is the typical Charcot joint disease,the case is diagnosed as syringomyelia caused Chiari malformation by cervical spine MRI .Conclusion Clinical and radiological features is a major basis for diagnosis of the disease.There is currently no effective treatment but surgery has certain curative effect.

  12. Ⅰ型 Chiari 畸形和(或)脊髓空洞相关脊柱侧凸术前评估和治疗选择%Scoliosis associated with Chiari type Ⅰ malformation with or without syringomyelia:preop-erative assessment and therapy

    Institute of Scientific and Technical Information of China (English)

    王琨(综述); 杨操(审校)

    2015-01-01

    Ⅰ型 Chiari 畸形是小脑扁桃体下疝畸形,为后脑的先天性畸形,其病理特征是小脑扁桃体疝入椎管内,小脑低于枕骨大孔5mm 以上。虽然非特发性脊柱侧凸中较少见的为伴发 Chiari 畸形和(或)脊髓空洞者,但随着诊断技术的提高,该类患者已变得不那么少见。检索 Chiari 畸形合并脊柱侧凸,得到相关中英文文献100篇,作者对相关文献进行综述,以了解Ⅰ型 Chiari 畸形合并脊柱侧凸研究进展。

  13. Nocturnal apnea in Chiari type I malformation

    NARCIS (Netherlands)

    Aarts, L.A.; Willemsen, M.A.A.P.; Vandenbussche, N.L.; Gent, R. van

    2011-01-01

    A 4-year-old girl presented with sleep-disordered breathing. Her parents described breathing pauses of up to 20 s and progressive tiredness during the day. Obstructive apneas from an enlarged adenoid were thought to be the most probable cause. However, an adenotomy did not resolve the problem. Polys

  14. Obstetric ultrasonographic findings of Chiari type II: Case report

    Directory of Open Access Journals (Sweden)

    Alptekin Tosun

    2009-01-01

    Full Text Available Chiari malformations divided into 3 groups. Chiari type I malformation is caudal protrusion of cerebellar tonsils. Type II malformation is the most common and associate with meningomyelocele. Type III is a high cervical men-ingoencephalocele and uncommon.Spina bifida, has classified into open and closed forms as skin covered spine lesions. Cranial signs are not ac-companiment on closed type. Open type usually diag-nosed on prenatal period. Typical findings are ventricu-lomegaly, lemon sign (bifrontal indentation, banana sign (Chiari II malformation, obliteration of cisterna magna and small BPD and body measurements according to gestation age. Occipital horns are higher than 10 mm in ventriculomegaly. Choroid plexus are small and looking like tear. Limon sign defines biconcave frontal bones as looking like a lemon. Banana sign and obliteration of cis-terna magna resulted cause of hypoplasia of posterior fossa. Compression of cerebellum causing abnormal lo-calization, although cerebellar tonsils and vermis herni-ated to foramen magnum. Hemispheres are wrapping brain stem and looking like ‘‘C’’ (banana sign. Spinal longitudinal sonogram reveals open spine and skin de-fect, although dilatation on spine canal and increased in-terpedincular distance.

  15. Syringomyelia in an older patient.

    Science.gov (United States)

    Gill, Rupinder; Birns, Jonathan

    2014-11-01

    We describe the case of an 80-year-old man who presented with lower limb upper motor neurone weakness and spinothalamic tract sensory deficit secondary to previously undiagnosed syringomyelia. The case highlights the need for methodical history, examination and investigation in elderly patients to achieve diagnostic accuracy.

  16. Evaluation of surgical therapy for syringomyelia by means of magnetic resonance imaging

    Energy Technology Data Exchange (ETDEWEB)

    Abe, Toshiaki; Yatsuzuka, Hitoshi; Nishida, Shin; Suzuki, Takashi; Nakamura, Norio; Ida, Masahiro

    1988-12-01

    Magnetic resonance imaging (MRI) was performed to evaluate surgical therapy in 18 patients with syringomyelia. Fourteen patients had a Chiari I-syringomyelia complex, ten of whom underwent Gardner's operation - suboccipital craniectomy, upper cervical laminectomy, arachnoid dissection around the foramen Magendie, dural plasty, and plugging of the obex. Postoperative MRI studies revealed a collapse of the syringomyelic cavity in the 10 patients; and an enlargement of the cisterna magna, with an upward displacement of the cerebellar tonsiles, an increase in the size of the anterior-posterior diameter of the medulla, and a widering of the lower part of the fourth ventricle in 9 patients. These MR findings were comparable with clinical improvement and the cessation of neurologic deterioration in patients with syringomyelia. Using cine MR images, the flow of cerebral spinal fluid (CSF) was analyzed in 6 patients. The turbulent flow of CSF in the cisterna magna was not detected before surgery. There was an active flow inside the syrinx in all the preoperative patients, but it was much less active in the patients who had undergone Gardner's operation, although they had a residual syrinx inside the spinal cord. This suggested that the pulsatile flow inside the syrinx might be transmitted from the fourth ventricle through the patent central canal. (Namekawa, K).

  17. Arachnoiditis ossificans and syringomyelia: A unique presentation

    Science.gov (United States)

    Opalak, Charles F.; Opalak, Michael E.

    2015-01-01

    Background: Arachnoiditis ossificans (AO) is a rare disorder that was differentiated from leptomeningeal calcification by Kaufman and Dunsmore in 1971. It generally presents with progressive lower extremity myelopathy. Though the underlying etiology has yet to be fully described, it has been associated with various predisposing factors including vascular malformations, previous intradural surgery, myelograms, and adhesive arachnoiditis. Associated conditions include syringomyelia and arachnoid cyst. The preferred diagnostic method is noncontrast computed tomography (CT). Surgical intervention is still controversial and can include decompression and duroplasty or durotomy. Case Description: The authors report the case of a 62-year-old male with a history of paraplegia who presented with a urinary tract infection and dysautonomia. His past surgical history was notable for a C4–C6 anterior fusion and an intrathecal phenol injection for spasticity. A magnetic resonance image (MR) also demonstrated a T6-conus syringx. At surgery, there was significant ossification of the arachnoid/dura, which was removed. After a drain was placed in the syrinx, there was a significant neurologic improvement. Conclusion: This case demonstrates a unique presentation of AO and highlights the need for CT imaging when a noncommunicating syringx is identified. In addition, surgical decompression can achieve good results when AO is associated with concurrent compressive lesions. PMID:26693389

  18. Treatment of scoliosis associated with untreated syringomyelia using segmental pedicle screw system%节段性椎弓根钉系统治疗伴发脊髓空洞的脊柱侧凸

    Institute of Scientific and Technical Information of China (English)

    刘一; 张绍昆; 闫明; 付长峰; 赵松; 牛丰; 张琪

    2009-01-01

    Objective To evaluate the surgical results of scoliosis associated with untreated syringomyelia with segmental pedicle screw system. Methods Thirty-five patients of scoliosis associated with untreated syringomyelia were treated with segmental pedicle screw system. Twelve cases were associated with type I Chiari malformation. The curve patterns covered atypical curve in 18 cases and atypical curve in 17 cases. There were two groups of operations: 1) Thirty cases, older than 10 years, underwent single-stage posterior correction, pedicle screw system internal fixation and bone-graft fusion; 2) Five cases, younger than 10 year, firstly underwent posterior correction and internal fixation with growth rod, and then posterior correction, internal fixation and bone-graft fusion 4-6 years later. Results The average coronal Cobb angle measured 66.5° (range, 32°-121°) preoperatively and 22.6° (range, 0°-78°) postoperatively. The average correction rate was 69.4% (range, 31%-100%). All the 35 cases were followed up 58.4 months in average (range, 13-113 months). The average coronal Cobb angle measured 25.9° (range, 3°-78°) at the latest follow-up. Six cases experienced deformity progressive after surgery. The caudal fusion vertebra was not fixed down to the stable vertebra in five of the six cases. It was shown absence of superficial abdominal reflex in one case, and expansion of sensory deficit in another one cases. Neither of two cases needed further management. Conclusion Segmental pedicle screw instrumentation is a safe procedure in treatment of scoliosis associated with untreated syringomyelia except Chiari malformation with distended type syringomyelia. Arthrodesis should be ended at the stable vertebra in the treatment of scoliosis associated with syringomyelia.%目的 评价节段性椎弓根钉系统治疗伴发脊髓空洞的脊柱侧凸的手术疗效.方法 应用节段性椎弓根钉系统治疗伴发脊髓空洞的脊柱侧凸35例.合并Chiari

  19. Diagnosis of posttraumatic syringomyelia with MRI

    Energy Technology Data Exchange (ETDEWEB)

    Takahashi, Mutsumasa; Sakamoto, Yuji; Kojima, Ryutaro; Matsuno, Taiji

    1988-11-01

    Five cases with development of syringomyelia, following long stable period of paraplegia after spinal trauma, have been studied with a resistive MRI unit. In all five cases there was spinal cord damage at the lower dorsal or dorsolumbar vertebral levels. Symptoms and signs of syringomyelia developed after long steady period with increased paresthesia, muscle weakness and pain of the shoulders and upper extremities. MRI revealed syringomyelia involving the dorsal and cervical cord up to the C1 or C2 level. The syringomyelia cavity was hypointense on T1 weighted images, but T2 weighted images showed isointensity in 2 of 4 cases showing no flow void. At the site of the cord injury there was diffuse increased signal intensity within the cord, probably indicating myelomalacia or gliosis. Pathogenesis of post-traumatic syringomyelia was discussed from the standpoint of diagnosis and treatment.

  20. Syringomyelia as a presenting feature of shunt dysfunction: Implications for the pathogenesis of syringomyelia

    Directory of Open Access Journals (Sweden)

    Natarajan Muthukumar

    2012-01-01

    Full Text Available The pathogenesis of syringomyelia continues to be an enigma. The patency of the central canal and its role in the pathogenesis of communicating syringomyelia continues to elicit controversy. The case reported here provides an opportunity to retest some of the hypotheses of syringomyelia. A 33 year old female presented with sensory disturbances over the left upper extremity and trunk and was diagnosed to have panventriculomegaly with communicating syringomyelia. She was initially treated with ventriculoperitoneal shunting. As there was no change in her neurological status following shunt, this was followed by foramen magnum decompression with excision of an arachnoid veil covering the fourth ventricular outlet. She had clinical and radiological improvement after foramen magnum decompression. Five months later she had reappearance of the symptoms of syringomyelia and was found to have shunt dysfunction and holocord syrinx. She improved following shunt revision. This case is being reported to highlight the following points: 1. In patients with communicating syringomyelia and hydrocephalus, shunt dysfunction can present with symptoms of syringomyelia without the classical clinical features of shunt dysfunction, 2. In patients with communicating syringomyelia, the central canal of the spinal cord acts as an "exhaust valve" for the ventricular system, and, 3. studies about the patency of the central canal are reviewed in the context of this case and the role of the central canal in the pathogenesis of communicating syringomyelia is reviewed.

  1. Magnetic resonance tomography in syringomyelia

    Energy Technology Data Exchange (ETDEWEB)

    Koehler, D.; Treisch, J.; Hertel, G.; Schoerner, W.; Fiegler, W.

    1985-12-01

    Thirteen patients with a clinical diagnosis of syringomyelia were examined by nuclear tomography (0.35 T magnet) in the spin-echo mode. In all thirteen patients, the T1 images (Se 400/35) showed a longitudinal cavity with a signal intensity of CSF. The shape and extent of the syrinx could be adequately demonstrated in 12 of the 13 examinations. Downward displacement of the cerebellar tonsils was seen in eight cases. The examination took between half and one hour. Advantages of magnetic resonance tomography (nuclear tomography) include the absence of artifacts, images in the line of the lesion and its non-invasiveness.

  2. Valproic Acid-Induced Syringomyelia in Rat Fetuses

    Directory of Open Access Journals (Sweden)

    M. Jalali

    2005-01-01

    Full Text Available Among antiepileptic drugs, valproic acid (VA is a well known teratogenic agent. Although axial skeletal malformations (vertebral column and limb defects have been described, its main target organ is neuroepithelium of neural tube. Therefore it seems that administration of VA during early pregnancy may affect on neural tube and adjacent tissues. The goal of present study was to determine whether there is a relationship between maternal valproic acid exposure and developmental changes during neural tube and notochord and their interactions.For this reason, on 9th day of gestation, wistar rats were treated with double dose of 600 mg/kg VA given once in the morning and another in the evening (in experimental group. The controls were received the same volume of normal saline by animal feeding. For teratological studies, fetuses were examined on 20th day of gestation and histological study were carried out.Our findings showed that in addition to some well known congenital malformations (such as axial skeletal defects and spina bifida there was an abnormal cavitation in cervical and thoracic segments of spinal cord (syringomyelia which was accompanied with a delay in determination of notochord at these levels. At these area, the syrinx (cyst is lined by compact glial tissue. In this kind of abnormality there is an atrophy of gray and white matter in the neighboring of syrinx in the spinal cord.These data revealed that, there is a strong association between maternal VA administration and risk for severe spinal cord defect such as syringomyelia and the same pathological changes might occur in human .

  3. The influence of coughing on cerebrospinal fluid pressure in an in vitro syringomyelia model with spinal subarachnoid space stenosis

    Directory of Open Access Journals (Sweden)

    Martin Bryn A

    2009-12-01

    Full Text Available Abstract Background The influence of coughing, on the biomechanical environment in the spinal subarachnoid space (SAS in the presence of a cerebrospinal fluid flow stenosis, is thought to be an important etiological factor in craniospinal disorders, including syringomyelia (SM, Chiari I malformation, and hydrocephalus. The aim of this study was to investigate SAS and syrinx pressures during simulated coughing using in vitro models and to provide information for the understanding of the craniospinal fluid system dynamics to help develop better computational models. Methods Four in vitro models were constructed to be simplified representations of: 1 non-communicating SM with spinal SAS stenosis; 2 non-communicating SM due to spinal SAS stenosis with a distensible spinal column; 3 non-communicating SM post surgical removal of a spinal SAS stenosis; and 4 a spinal SAS stenosis due to spinal trauma. All of the models had a flexible spinal cord. To simulate coughing conditions, an abrupt CSF pressure pulse (~ 5 ms was imposed at the caudal end of the spinal SAS by a computer-controlled pump. Pressure measurements were obtained at 4 cm intervals along the spinal SAS and syrinx using catheter tip transducers. Results Pressure measurements during a simulated cough, showed that removal of the stenosis was a key factor in reducing pressure gradients in the spinal SAS. The presence of a stenosis resulted in a caudocranial pressure drop in the SAS, whereas pressure within the syrinx cavity varied little caudocranially. A stenosis in the SAS caused the syrinx to balloon outward at the rostral end and be compressed at the caudal end. A >90% SAS stenosis did not result in a significant Venturi effect. Increasing compliance of the spinal column reduced forces acting on the spinal cord. The presence of a syrinx in the cord when there was a stenosis in the SAS, reduced pressure forces in the SAS. Longitudinal pressure dissociation acted to suck fluid and tissue

  4. The safety and efficacy of posterior vertebral column resection for severe and rigid kyphoscoliosis assiociated with syringomyelia%后路全脊椎截骨术治疗合并脊髓空洞重度僵硬性脊柱侧后凸畸形的安全性及疗效分析

    Institute of Scientific and Technical Information of China (English)

    陶有平; 司泽兵; 吴继功; 马华松; 邵水霖; 张乐乐; 姬勇; 高博; 李海侠; 陶飞飞

    2015-01-01

    Objective To investigate the safety and efficacy of of posterior vertebral column resection (PVCR) for severe and rigid kyphoscoliosis assiociated with syringomyelia. Methods A total of 16 patients,diagnosed as severe and rigid kyphoscoliosis assiociated with syringomyelia, from Oct 2009 to Mar 2013 in our hospital, the patients had undergone PVCR. Patients who had combined with Chiar-I malformation, had underwent posterior fossa decompression before the kyphoscoliosis correction. The operation time ,intraoperative blood loss and complications were recorded. Results There were significant differences between pre-and post-operative coronal Cobb angle,thoracic kyphosis Cobb angle (P0.05). Conclusion PVCR for severe and rigid kyphoscoliosis assiociated with syringomyelia is an ef-fective and safe method. However,additional large multicenter studies are necessary to further as-sess the safety and efficacy.%目的:探讨后路全脊椎截骨术(Posterior Vertebral Column Resection,PVCR)治疗合并脊髓空洞的重度僵硬性脊柱侧后凸的安全性及临床疗效。方法回顾分析2009-10-2013-03在我院接受PVCR手术治疗的合并脊髓空洞的重度僵硬性脊柱侧后凸畸形患者16例;其中同时伴发Chiari畸形I型患者在脊柱畸形截骨矫行前均先于神经外科进行后颅凹减压术;对于单纯合并脊髓空洞患者,直接进行脊柱截骨畸形矫治;记录手术时间、出血量及围手术期并发症情况。结果手术前、后冠状面及矢状面主弯Cobb角比较,差异具有统计学意义(P<0.05);手术后与末次随访时冠状面及矢状面主弯Cobb角比较,差异无统计学意义(P>0.05)。结论对于合并脊髓空洞的重度僵硬性脊柱侧后凸畸形,采用PVCR进行治疗,可获得安全、有效的临床结果。

  5. Exercise Concepts for Individuals with Syringomyelia

    Science.gov (United States)

    ... Center Research Great Expectations Post navigation ← Previous Next → Exercise Concepts for Individuals with Syringomyelia Posted on November ... related duties? 3. Do you have questions about exercise? Do you exercise regularly? Are you involved in ...

  6. Association of idiopathic hypopituitarism and syringomyelia

    Energy Technology Data Exchange (ETDEWEB)

    Aihara, T. [Dept. of Radiology, Saitama Children`s Medical Center, Saitama (Japan); Nishikawa, M. [Dept. of Radiology, Saitama Children`s Medical Center, Saitama (Japan); Koda, N. [Div. of Endocrinology and Metabolism, Dept. of Pediatrics, Saitama Children`s Medical Center (Japan); Abe, T. [Dept. of Neurosurgery, Jikei Univ. School of Medicine, Tokyo (Japan)

    1995-06-01

    The magnetic resonance in an 8-year-old boy with hypopituitarism and cervical syringomyelia are presented. Magnetic resonance imaging of the brain clearly demonstrated syringomyelia in the cervical spinal cord and transection of the pituitary stalk, findings identical to those reported in the literature. Both disorders have been seen in association with a difficult labor, so we suggest that this is not a chance finding. (orig.)

  7. Das Budd-Chiari-Syndrom

    Directory of Open Access Journals (Sweden)

    Angermayr B

    2008-01-01

    Full Text Available Der Artikel gibt einen aktuellen Überblick über Definition, Pathophysiologie, Klinik undTherapie des Budd-Chiari-Syndroms, welches eineseltene und potenziell schwer verlaufende vaskuläre Lebererkankung ist.

  8. Pathogenesis of cerebral malformations in human fetuses with meningomyelocele

    NARCIS (Netherlands)

    de Wit, Olga A; den Dunnen, Wilfred Fa; Sollie, Krystyne M; Muñoz, Rosa Iris; Meiners, Linda C; Brouwer, Oebele F; Rodríguez, Esteban M; Sival, Deborah A

    2008-01-01

    BACKGROUND: Fetal spina bifida aperta (SBA) is characterized by a spinal meningomyelocele (MMC) and associated with cerebral pathology, such as hydrocephalus and Chiari II malformation. In various animal models, it has been suggested that a loss of ventricular lining (neuroepithelial/ependymal denud

  9. Pathogenesis of cerebral malformations in human fetuses with meningomyelocele

    NARCIS (Netherlands)

    de Wit, Olga A; den Dunnen, Wilfred Fa; Sollie, Krystyne M; Muñoz, Rosa Iris; Meiners, Linda C; Brouwer, Oebele F; Rodríguez, Esteban M; Sival, Deborah A

    2008-01-01

    BACKGROUND: Fetal spina bifida aperta (SBA) is characterized by a spinal meningomyelocele (MMC) and associated with cerebral pathology, such as hydrocephalus and Chiari II malformation. In various animal models, it has been suggested that a loss of ventricular lining (neuroepithelial/ependymal denud

  10. Syringomyelia

    Science.gov (United States)

    ... skin is being touched; occurs in the neck, shoulders, upper arms, and trunk in a cape-like pattern; and slowly gets worse over time Pain down the arms, neck, or into the middle back or legs Weakness (decreased muscle strength) in the arms or legs

  11. Pathogenesis of cerebral malformations in human fetuses with meningomyelocele

    Directory of Open Access Journals (Sweden)

    Brouwer Oebele F

    2008-03-01

    Full Text Available Abstract Background Fetal spina bifida aperta (SBA is characterized by a spinal meningomyelocele (MMC and associated with cerebral pathology, such as hydrocephalus and Chiari II malformation. In various animal models, it has been suggested that a loss of ventricular lining (neuroepithelial/ependymal denudation may trigger cerebral pathology. In fetuses with MMC, little is known about neuroepithelial/ependymal denudation and the initiating pathological events. The objective of this study was to investigate whether neuroepithelial/ependymal denudation occurs in human fetuses and neonates with MMC, and if so, whether it is associated with the onset of hydrocephalus. Methods Seven fetuses and 1 neonate (16–40 week gestational age, GA with MMC and 6 fetuses with normal cerebral development (22–41 week GA were included in the study. Identification of fetal MMC and clinical surveillance of fetal head circumference and ventricular width was performed by ultrasound (US. After birth, MMC was confirmed by histology. We characterized hydrocephalus by increased head circumference in association with ventriculomegaly. The median time interval between fetal cerebral ultrasound and fixing tissue for histology was four days. Results At 16 weeks GA, we observed neuroepithelial/ependymal denudation in the aqueduct and telencephalon together with sub-cortical heterotopias in absence of hydrocephalus and/or Chiari II malformation. At 21–34 weeks GA, we observed concurrence of aqueductal neuroepithelial/ependymal denudation and progenitor cell loss with the Chiari II malformation, whereas hydrocephalus was absent. At 37–40 weeks GA, neuroepithelial/ependymal denudation coincided with Chiari II malformation and hydrocephalus. Sub-arachnoidal fibrosis at the convexity was absent in all fetuses but present in the neonate. Conclusion In fetal SBA, neuroepithelial/ependymal denudation in the telencephalon and the aqueduct can occur before Chiari II malformation

  12. Comparison of dural grafts in Chiari decompression surgery: Review of the literature

    Directory of Open Access Journals (Sweden)

    A A Abla

    2010-01-01

    Full Text Available Background: Decompression of Chiari malformation is a common procedure in both pediatric and adult neurosurgery. Although the necessity for some bony removal is universally accepted, other aspects of Chiari surgery are the subject of debate. The most controversial points include the optimal amount of bony removal, the use of duraplasty (and the type of material, the need for subarachnoid dissection, and the need for tonsillar shrinkage. Material and Methods: We critically reviewed the literature to elucidate the risks and benefits of different graft types and to clarify optimal treatment options therein. Based on our search results, 108 relevant articles were identified. With specific inclusion and exclusion criteria, we noted three studies that directly compared two tlpes of dural substitutes in Chiari malformation surgery. Results: Our review did not support the superiority of either autologous or nonautologous grafts when duraplasty is employed. Our institutional experience, however, dictates that when the pericranium is available and of good quality, it should be utilized for duraplasty. It is non-immunogenic, inexpensive, and capable of creating a watertight closure with the dura. Conclusions: Discrepancies between the three comparative studies analyzed are likely attributable to increases in pericranial quality and thickness with maturity. Future randomized studies with large numbers and the power to resolve differences in the relatively low rates of complications in Chiari surgery are warranted.

  13. Lhermitte-Duclos disease associated with syringomyelia

    Energy Technology Data Exchange (ETDEWEB)

    Marcus, C.D. [Hopital Robert Debre, CHU, 51 - Reims (France). Service de Radiologie et d`Imagerie Medicale; Galeon, M. [Hopital Robert Debre, CHU, 51 - Reims (France). Service de Radiologie et d`Imagerie Medicale; Peruzzi, P. [Hopital Maison Blanche, 51 - Reims (France). Service de Neuro-Chirurgie; Bazin, A. [Hopital Maison Blanche, 51 - Reims (France). Service de Neuro-Chirurgie; Bernard, M.H. [Hopital Maison Blanche, 51 - Reims (France). Service de Neuro-Chirurgie; Pluot, M. [Hopital Robert Debre, 51 - Reims (France). Service d`Anatomo-Pathologie; Menanteau, B. [Hopital Robert Debre, CHU, 51 - Reims (France). Service de Radiologie et d`Imagerie Medicale

    1996-08-01

    A 23-year-old man presented with a 2-week history of intracranial hypertension. CT showed a large, nonenhancing cerebellar mass with surrounding calcification and displacement of the fourth ventricle. MRI revealed a septate lesion, with low signal on T1-weighted and high signal on T2-weighted images. The cerebellar tonsils were displaced below the foramen magnum and there was associated syringomyelia. The MRI features were characteristic of Lhermitte-Duclos (LD) disease (dysplastic gangliocytoma) and the diagnosis was confirmed following surgery. In this case, we emphasise the usefulness of MRI in the diagnosis of LD disease and consider the possible pathogenesis of the associated syringomyelia. (orig.)

  14. Outcomes of patients with syringomyelia undergoing spine deformity surgery: do large syrinxes behave differently from small?

    Science.gov (United States)

    Samdani, Amer F; Hwang, Steven W; Singla, Anuj; Bennett, James T; Ames, Robert J; Kimball, Jeff S

    2017-04-13

    A paucity of data exist studying outcomes of patients with syringomyelia undergoing spinal deformity correction. The literature does not stratify patients by syrinx size, which is likely a major contributor to outcomes. The study aimed to compare differences in outcomes between patients with large (≥4 mm) and small syrinxes (4 mm] and 17 with small syrinx [SS, <4 mm]). The outcome measures were radiographic, operative, and neurophysiological measures. We retrospectively reviewed 28 patients with syringomyelia who underwent spine deformity surgery with 2-year follow-up. Demographic, surgical, and radiographic data were collected and compared preoperatively and at 2 years. The LS group (11 patients) trended toward more left-sided thoracic curves (36% vs. 18%, p=.38) and was more likely to have had a Chiari decompression (45% vs. 12%, p=.08). The LS patients had larger preoperative major curves (LS=66° vs. SS=57°, p=.05), more thoracic kyphosis (LS=42°, SS=24°, p<.01), and greater rib prominences (LS=16°, SS=13°, p=.04). The LS patients had more levels fused (LS=12.2, SS=11.2, p=.05), higher estimated blood loss (EBL) (LS=1068 cc, SS=832 cc, p=.04), and a trend toward less percent correction of the major curve (LS=57%, SS=65%, p=.18). Four of 11 LS patients (36%) did not have somatosensory evoked potentials, and one of these also did not have motor evoked potentials. Neuromonitoring changes occurred in 3 of 11 (27%) LS patients and in none of the SS patients, with no postoperative deficits. Outcomes of patients with syringomyelia undergoing spine deformity surgery are dependent on the size of the syrinx. Those with large syringomyelia are fused longer with more EBL and less correction. Spine surgeons should be aware that these patients are more likely to have less reliable neuromonitoring, with a higher chance of experiencing a change. Copyright © 2017 Elsevier Inc. All rights reserved.

  15. 特发性青少年脊髓空洞症患儿后颅窝线性容积的改变及临床意义%Morphometric analysis of the posterior fossa in adolescents with idiopathic syringomyelia

    Institute of Scientific and Technical Information of China (English)

    闫煌; 朱泽章; 邱勇; 沙士甫; 江龙; 伍伟飞; 杨宗

    2013-01-01

    目的 评估青少年特发性脊髓空洞(idiopathic syringomyelia,IS)患儿后颅窝线性容积,并探讨其临床意义.方法 从2008年1月至2011年10月我院诊断为IS的病例中选择影像学资料完整的青少年患儿25例(IS组).同时选取性别、年龄匹配的继发于Chiari畸形Ⅰ型的脊髓空洞(Chiari malformation type Ⅰ& syringomyelia,CMI-S)患儿(CMI-S组)及正常青少年作为对照组.在正中矢状面MR影像上分别测量三组患儿后颅窝线性容积的距离指标(后颅窝斜坡长度、枕骨大孔径、枕上长度、后颅窝矢状径、后颅窝高径)和角度指标(后颅窝斜坡倾斜角),并运用单向方差分析统计三组患儿后颅窝各测量指标间的差异.结果 IS组后颅窝斜坡长度25.4~48.7 mm(平均38.3 mm);枕骨大孔前后径27.2~44.2 mm(平均33.6 mm);枕骨鳞部长度23.6~48.2 mm(平均36.7 mm);后颅窝矢状62.9~88.4 mm(平均74.3 mm);后颅窝高径18.5~43.9 mm(平均31.3 mm)及斜坡倾斜角30.0°~77.0°(平均56.7°).IS组与CMI-S组患儿的后颅窝线性容积各测量指标均显著小于正常青少年,差异有统计学意义(P<0.05).IS组患儿后颅窝斜坡倾斜角为56.7°±10.6°,显著大于CMI-S组患儿50.8°±11.7°,差异有统计学意义(P<0.05),其余指标差异没有统计学意义(P>0.05).结论 IS患儿与CMI-S患儿均存在后颅窝骨性结构的发育异常.与CMI-S患儿相比,IS患儿具有较为陡直的斜坡结构.%Objective To investigate the morphometric characteristics of the posterior fossa in adolescents with idiopathic syringomyelia (IS) and to elucidate the clinical significances.Methods A retrospective radiographic review was performed on 25 adolescents who were diagnosed as IS and treated at our clinic between January 2008 and October 2011.Another cohort of normal adolescents and chiari malformation type Ⅰ & syringomyelia(CMI-S) patients,matched with the IS patients in age and gender,were recruited as the control

  16. Syringomyelia: a practical, clinical concept for classification

    NARCIS (Netherlands)

    Blegvad, C.; Grotenhuis, J.A.; Juhler, M.

    2014-01-01

    BACKGROUND: The term syringomyelia describes many pathogenetically different disorders, and a variety of attempts to group these based on different criteria have been proposed in the literature. As a consequence a lack of consensus regarding classification and terminology exists. This inconsistency

  17. Magnetic resonance imaging (MRI) in syringomyelia

    NARCIS (Netherlands)

    H.L.J. Tanghe (Hervé)

    1995-01-01

    textabstractBased on an own material of 19 patients with syringomyelia and on the related literature a survey is given on the diagnosis, differential diagnosis, postoperative evaluation and the dynamics of CSF and cyst fluids, using magnetic resonance imaging (MRI). The following conclusions can be

  18. Syringomyelia regression after shunting of a trapped fourth ventricle

    Directory of Open Access Journals (Sweden)

    Dukagjin Morina

    2013-01-01

    Full Text Available We describe a case of progressive syringomyelia following post-infectious trapped fourth ventricle (TFV, which resolved after shunting of the fourth ventricle. A 28-year-old female who had previously undergone treatment of intracerebral hemorrhage and meningitis developed a hydrocephalus with TFV. After 3 years she developed disturbance of walking and coordination. Cranial-CT revealed an enlargement of the shunted fourth ventricle as a result of shunt dysfunction. Furthermore a cervical syringomyelia developed. The patient underwent a revision of a failed fourth ventriculo- peritoneal shunt. Postoperatively, syringomyelia resolved within 6 months and the associated neurological deficits improved significantly. An insufficiency of cerebrospinal fluid draining among patients with TFV can be associated with communicating syringomyelia. An early detection and treatment seems important on resolving syringomyelia and avoiding permanent neurological deficits. Ventriculo-peritoneal shunt in trapped fourth ventricles can resolve a secondary syringomyelia.

  19. A STUDY OF POSTERIOR FOSSA MALFORMATIONS: MR IMAGING

    Directory of Open Access Journals (Sweden)

    Ravi

    2015-02-01

    Full Text Available AIMS AND OBJECTIVES: The aim of our study is to describe the imaging findings of various posterior fossa malformations and to evaluate the supratentorial abnormalities associated with posterior fossa malformations. MATERIALS AND METHODS: MR images of 30 patients wi th posterior fossa malformations detected in the department of Radiodiagnosis, BMCRI over a period of two years, from December 2012 to December 2014 were evaluated retrospectively. The various posterior fossa malformations were evaluated. Associated suprat entorial abnormalities were noted. RESULTS: 30 patients with posterior fossa malformations were included in the study. The age group of patients ranged from 1year to 53years. There were 18 males and 12 females. The various posterior fossa malformations det ected were Dandy Walker malformation (1 case, Dandy Walker variant (2 cases, mega cisterna magna (8 cases, arachnoid cysts (5 cases, Chiari 1 malformation (5 cases, Chairi 2 malformation (2 cases, Joubert malformation (1 case, lipoma (2 cases, verm ian and/or cerebellar hypoplasia without posterior fossa CSF collection or cyst (4 cases. Associated supratentorial abnormalities were seen in 8 cases . CONCLUSION: MRI is the imaging modality of choice in the evaluation of posterior fossa malformations. I t is very important to know the imaging findings of these malformations and to have knowledge about the various supratentorial and spinal abnormalities associated with them so as to provide an accurate diagnosis which is very essential for predicting the p rognosis and planning further management.

  20. Retrocerebellar arachnoid cyst with syringomyelia : a case report.

    Directory of Open Access Journals (Sweden)

    Jain R

    2000-01-01

    Full Text Available Association of syringomyelia with retrocerebellar arachnoid cysts is rare. A case of 14 year old female is being reported, who presented with hydrocephalus caused by a large midline retrocerebellar infravermal arachnoid cyst leading to obstruction of the outlet foramina of the fourth ventricle. There was associated syringomyelia. The pathogenesis of syringomyelia is discussed. The need to evaluate cervical spinal cord by taking T1 weighted sagittal sections in all the patients of large posterior fossa mass lesions causing obstruction to the outlet foramina of the fourth ventricle has been stressed, in order to detect associated syringomyelia.

  1. Syringoperitoneal shunt - a promising treatment in syringomyelia

    Energy Technology Data Exchange (ETDEWEB)

    Wester, K.; Midgard, R.; Kjosavik, I.F.

    1988-09-01

    Five patients with radiologically relatively pronounced multicystic syringomyelia were operated with an open, non-valved syringoperitoneal shunt. In the postoperative MRI scans there was an immediate and pronounced reduction of all cystic compartments, and within a few months the cysts had virtually disappeared. Two of the patients experienced marked neurological improvement. In one patient the shunt procedure led to additional moderate neurological deficits caused by postoperative rotation of the intrameduallary T-drain. For this reason straight intramedullary drains are now used.

  2. Syringomyelia and cervical spondylosis: A clinicoradiological investigation

    Energy Technology Data Exchange (ETDEWEB)

    Yu, Y.L.; Moseley, I.F.

    1987-03-01

    Eight eight patients with proven syringomyelia have been studied retrospectively to explore the relationship between syringomyelia and spondylotic degeneration of the cervical spine. When compared with age and sex matched control subjects, they showed no significant increase in incidence or severity of spondylosis or of vertebral subluxation. Duration of symptoms, degree of disablity, and the pattern of analgesia or lower motor neurone lesions in the arms did not appear to be related to the development of spondylosis. The onset of moderate spondylosis, was however, earlier than in the controls, and individual cases with a remarkably early onset or unusual site and/or severity of spondylosis and subluxation were encountered, particularly following laminectomy. It was also noted that in a small number of patients the concomitant development of spondylosis contributed to clinical deterioration. It is concluded that while syringomyelia does not have a consistent, major role in the development of cervical spondylosis or vertebral luxation, it may contribute to progression of spinal degenerative disease in some patients.

  3. The role of lumboperitoneal shunts in the treatment of syringomyelia.

    Science.gov (United States)

    Oluigbo, Chima O; Thacker, Karen; Flint, Graham

    2010-07-01

    OBJECT The role of thecoperitoneal shunts in the management of syringomyelia is not well defined. In this study, the authors analyze the outcome of lumboperitoneal shunt procedures carried out to treat syringomyelia in their institution. METHODS The authors retrospectively reviewed the medical records of 19 patients who underwent lumboperitoneal shunt procedures for syringomyelia. RESULTS The mean follow-up duration was 25 months (range 3-51 months). Of 16 cases followed up, only 5 patients reported clinical improvement in their preoperative symptoms, but of these, 2 had clear radiological evidence of improvement. Three of 6 patients with syringomyelia due to spinal arachnoiditis improved. CONCLUSIONS Lumboperitoneal shunts may lead to useful improvement in the symptoms of a patient with syringomyelia while avoiding the risk of neurological deterioration inherent in myelotomies required for syrinx shunting procedures.

  4. Arnold-Chiari畸形并脊髓空洞症125例手术治疗疗效评价%The surgical treatments and curative effects evaluation of Arnold-Chiari malformation complicated with syringomye-lia: a study of 125 patients

    Institute of Scientific and Technical Information of China (English)

    赵红宇; 于宏伟; 王成林

    2006-01-01

    目的探讨Arnold-Chiari畸形并脊髓空洞症手术方式及术后疗效.方法对125例MR证实的Arnold-Chiari畸形并脊髓空洞症患者的手术治疗情况进行回顾性分析.患者分别行后颅窝减压及枕大池扩大重建.结果出院时患者症状消失和改善共110例,有效率88.0%.随访114例患者,98例复查MR,枕大池解剖成形者87例,脊髓空洞缩小68例,远期有效率77.2%.结论后颅窝减压及枕大池扩大重建是治疗Arnold-Chiari畸形并脊髓空洞症的有效方法.

  5. 小脑扁桃体切除并脊髓中央管口松解术治疗合并脊髓空洞的Chiari畸形%Chiari malformation with syringomyelia:surgical treatment with tonsillectomy plus central canal opening

    Institute of Scientific and Technical Information of China (English)

    张玉琪; 王忠诚; 马振宇; 李志红

    2004-01-01

    目的探索外科治疗合并脊髓空洞的Chiari畸形的新方法.方法采用切除下疝小脑扁桃体,并脊髓中央管口假膜切开术,原位硬膜缝合法.结果18例手术治疗,15岁以下儿童12例,术中发现均有脊髓中央管口(闩部)假膜;成人6例,3例有假膜形成.全部病例术后脊髓空洞消失或明显缩小.结论脊髓中央管口假膜是造成Chiari畸形脊髓空洞的原因之一,切除下疝小脑扁桃体并脊髓中央管口假膜切开术,是手术治疗合并脊髓空洞的Chiari畸形最根本的方法.

  6. NREM sleep parasomnia associated with Chiari I malformation.

    Science.gov (United States)

    Daftary, Ameet S; Walker, James M; Farney, Robert J

    2011-10-15

    Parasomnias are common sleep disorders in children, and most cases resolve naturally by adolescence.(1) They represent arousal disorders beginning in NREM sleep and are generally non-concerning in children. The diagnosis can usually be made by clinical assessment, and testing with polysomnography is not routinely indicated.(2) However, in certain cases with atypical features, polysomnography and more extensive neurologic evaluation are medically indicated.

  7. Surgery for posttraumatic syringomyelia: a retrospective study of seven patients

    Institute of Scientific and Technical Information of China (English)

    CAO Fei; YANG Xiao-feng; LIU Wei-guo; LI Gu; ZHENG Xue-sheng; WEN Liang

    2007-01-01

    Objective: To analyze retrospectively the clinical symptoms, signs, radiological findings and results of treatment of posttraumatic syringomyelia.Methods: The data of 7 patients with posttraumatic syringomyelia confirmed by computerized tomography(CT) and magnetic resonance imaging (MRI) in our hospital between 1999 and 2004 were reviewed retrospectively. The patients underwent decompressive laminectomy or syringo-subarachnoid (S-S) shunting with microsurgery. Long-term follow-up was available (range:13-65 months).Results: The major clinical manifestations of posttraumatic syringomyelia usually included the onset of increasing signs and the development of new symptoms after an apparently stable period. The clinical symptoms included pain, sensory disturbance, weakness, and problems in autonomic nerves. Syrinx existed merely at the cervical level in 4 cases and extended downward to the thoracic levels in the other 3 cases. One case underwent decompressive laminectomy, 6 cases were treated by S-S shunting. During the early postoperative period, all the patients showed an improvement of symptoms of syrinx without major complication or death. The decreased size or collapse of the syrinx was demonstrated by postoperative MRI.Conclusions: Posttraumatic syringomyelia is a disabling sequela of spinal cord injury, developing months to years after spinal injury. MRI is the standard diagnostic technique for syringomyelia. The patients with posttraumatic syringomyelia combined with progressive neurological deterioration should be treated with operations. S-S shunting procedure is effective in some patients with posttraumatic syringomyelia. Decompressive procedure may be an alternative primary surgical treatment for patients with kyphosis and cord compression.

  8. Imaging of Budd-Chiari syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Buckley, O.; O' Brien, J.; Snow, A.; Stunell, H.; Torreggiani, W.C. [Adelaide and Meath Hospital, Incorporating the National Children' s Hospital (AMNCH), Department of Radiology, Dublin 24 (Ireland); Lyburn, I. [Cheltenham Hospital, Department of Radiology, Cheltenham (United Kingdom); Munk, P.L. [Vancouver General Hospital, Department of Radiology, Vancouver (Canada)

    2007-08-15

    Budd-Chiari syndrome occurs when venous outflow from the liver is obstructed. The obstruction may occur at any point from the hepatic venules to the left atrium. The syndrome most often occurs in patients with underlying thrombotic disorders such as polycythemia rubra vera, paroxysmal nocturnal hemoglobinuria and pregnancy. It may also occur secondary to a variety of tumours, chronic inflammatory diseases and infections. Imaging plays an important role both in establishing the diagnosis of Budd-Chiari syndrome as well as evaluating for underlying causes and complications such as portal hypertension. In this review article, we discuss the role of modern imaging in the evaluation of Budd-Chiari syndrome. (orig.)

  9. Brain Malformations

    Science.gov (United States)

    Most brain malformations begin long before a baby is born. Something damages the developing nervous system or causes it ... medicines, infections, or radiation during pregnancy interferes with brain development. Parts of the brain may be missing, ...

  10. [Intradural arachnoid cyst associated with syringomyelia: a case report].

    Science.gov (United States)

    Ishi, Yukitomo; Aoyama, Takeshi; Kurisu, Kota; Hida, Kazutoshi; Houkin, Kiyohiro

    2014-05-01

    An intradural arachnoid cyst is a relatively rare condition, occurring within the spinal subarachnoid space. We present the even-more rare case of an intradural arachnoid cyst associated with syringomyelia at the same spinal level. The patient was a 66-year-old man who presented with bilateral leg numbness and gait disturbance. Magnetic resonance imaging (MRI) revealed an intradural arachnoid cyst located dorsal to, and compressing, the thoracic spinal cord at the level of the 7th thoracic vertebra (Th 7). In addition, syringomyelia existed at the level of Th 8, slightly caudal to the intradural arachnoid cyst. We dissected the cyst but did not perform any surgical procedures for the syringomyelia. Post-operative MRI showed that the cyst had disappeared and the syringomyelia had spontaneously shrunk. The patient was discharged with improvement in his numbness and gait disturbance. There are a few case reports of intradural arachnoid cysts associated with syringomyelia, but recent evidence suggests that its occurrence is more common than previously thought. A combination of these two diseases is thought to be caused by blockage of cerebrospinal fluid (CSF) flow, which is also thought to cause adhesive arachnoiditis. For this reason, resection of the arachnoid cyst could improve the CSF flow and contribute to the shrinkage of syringomyelia. Furthermore, early treatment may correlate with improvement in radiological findings and neurological symptoms.

  11. 经口寰枢椎复位内固定术治疗颅底凹陷症合并脊髓空洞症的疗效%Clinical efficacy of TARP for treatment of basilar invagination with syringomyelia: a report of 32 cases

    Institute of Scientific and Technical Information of China (English)

    石亮; 夏虹; 尹庆水; 艾福志; 王建华; 潘刚明; 王新宇; 马丽敏; 陈育岳

    2012-01-01

    Objectives: To investigate the effect of transoral atlantoaxial reduction and plate internal fixation for the treatment of basilar invagination with syringomyelia. Methods: From March 2005 to July 2011, 75 cases with basilar invagination were treated surgically. Of them, 35 patients were combined with syringomyelia, 32 cases of whom were followed up. There were 9 males and 23 females, aged from 23 to 74 years, with the course of disease of 6 to 372 months. 12 cases presented with Arnold-Chiari malformation, 7 cases with atlantoaxial dislocation, 1 patient with atlantooccipital fusion, 2 patients with cervical spondylosis and 1 patient with Klippel-Feil syndrome. All patients had signs and symptoms of spinal cord dysfunction, and the preoper-ative JOA score was 3-15 points. According to ASIA scale, there were 4 cases with grade B, 8 grade C, 20 grade D. Preoperative brainstem spinal cord angle was 123.3°±6.4° and syringomyelia size was 0.10-15.10cm3. All cases underwent TARP instrumentation, the postoperative improvement of neurofunction, brain stem spinal cord angle were recorded. The postoperative complications, follow-up changes in syringomyelia and bone graft fusion were also recorded. Results: All patients had successful surgery, with no neurovascular injury. After operation, 1 patient complained of neck discomfort, 2 cases complained of throat discomfort, which wereresolved after corresponsive treatment; 1 case had incision infection, which healed by removal of anterior instrument and posterior instrumentation; the remaining patients presented with no complication. The postoperative brainstem spinal cord angle recovered to 145.6°-165.3°, with an average of 161.3°±5.1°; JOA score improved to 10 to 16 points (average, 13.8±1.8), with an average improvement rate of 58.1%. 15 cases had ASIA scale improved. After 1 year, syringomyelia size of 30 cases shrank or disappeared, syringomyelia size of 1 case remained unchange, 1 case had syringomyelia size

  12. Myelopathy with syringomyelia following thoracic epidural anaesthesia.

    Science.gov (United States)

    Aldrete, J A; Ferrari, H

    2004-02-01

    Under general anaesthesia and muscle relaxation, a thoracic epidural catheter was inserted at the T8-T9 level in a 7-year-old boy scheduled to have a Nissen fundoplication to provide postoperative analgesia. After 4 ml of lignocaine 1.5% was injected through the catheter, hypotension resulted. Fifty-five minutes later 5 ml of bupivacaine 0.25% produced the same effect. In the recovery room a similar injection resulted in lower blood pressure and temporary apnoea. Sensory and motor deficits were noted the next day and four days later magnetic resonance imaging demonstrated spinal cord syringomyelia extending from T5 to T10. Four years later, dysaesthesia from T6 to T10 weakness of the left lower extremity and bladder and bowel dysfunction persist. The risks of inserting thoracic epidural catheters in patients under general anaesthesia and muscle relaxation are discussed, emphasising the possibility of spinal cord injury with disastrous consequences.

  13. Syringomyelia: A review of the biomechanics

    Science.gov (United States)

    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.

  14. Radiographic studies of the ventricles in syringomyelia

    Energy Technology Data Exchange (ETDEWEB)

    West, R.J.; Williams, B.

    1980-08-01

    Radiographic investigations of 171 patients with communicating syringomyelia have been reviewed. Hydrocephalus was found in one third of the cases and has occasionally progressed after operation on the posterior fossa, sometimes with accompanying clinical deterioration. The outlets of the fourth ventricle were usually abnormal; tonsillar descent, arachnoiditis and both together were seen. Arachnoiditis correlated strongly with a history of difficult birth. The foramen of Magendie was sometimes patent and sometimes blocked. There was no consistent level of occlusion corresponding to a persistent roof of the fourth ventricle. The cisterna magna was usually small or obliterated but some examples of large cisterns or subarachnoid pouches were found. Radiological demonstration of a communication from the fourth ventricle to the syrinx occurred in only seven patients by positive contrast material and not by air. It is suggested that a sizable communication is rare at the time when patients seek treatment.

  15. Favorable results with syringosubarachnoid shunts for treatment of syringomyelia.

    Science.gov (United States)

    Tator, C H; Meguro, K; Rowed, D W

    1982-04-01

    From 1969 to 1979, 20 patients with syringomyelia were treated with a syringosubarachnoid shunt. The principal indications for this procedure were: significant progressive neurological deterioration and absent or minimal tonsillar ectopia. There were 15 patients with idiopathic syringomyelia, four with posttraumatic syringomyelia, and one with syringomyelia secondary to spinal arachnoiditis. The operations were performed with an operating microscope, and attention was directed to preserving thearachnoid membrane to ensure proper placement of the distal end of the shunt in an intact subarachnoid space. In all cases, a silicone rubber ventricular catheter was inserted into the syrinx through a posterior midline myelotomy. The average follow-up period was 5 years. A favorable result was obtained in 15 of the 20 patients (75%), including an excellent result with improvement of neurological deficit in 11 patients and a good result with cessation of progression in four patients. In the remaining five patients the result was poor with further progression of neurological deficit. A short duration of preoperative symptoms was usually a favorable prognostic feature. Four patients with a history of less than 6 months all had excellent results. Thirteen patients had a syringosubarachnoid shunt only, and all had good or excellent results. Seven patients had other surgical procedures, before, accompanying, or after shunt placement, and two had favorable results. Thus, the syringosubarachnoid shunt is an effective therapeutic modality for many patients with syringomyelia, particularly if there is little or no tonsillar herniation.

  16. Gastrointestinal malformations

    DEFF Research Database (Denmark)

    Garne, Ester; Loane, Maria; Dolk, Helen;

    2007-01-01

    of mortality for the prenatally diagnosed infants. Clinicians need to balance the risk of early delivery against the benefits of clinical convenience when making case management decisions after prenatal diagnosis. Very few studies have been able to show benefits of prenatal diagnosis of congenital...... malformations for liveborn infants. This may be because the benefits of prenatal diagnosis are outweighed by the problems arising from a lower GA at birth....

  17. A quantitative analysis of cerebrospinal fluid flow in posttraumatic syringomyelia

    Energy Technology Data Exchange (ETDEWEB)

    Tobimatsu, Yoshiko; Nihei, Ryuuichi; Kimura, Tetsuhiko; Suyama, Tetsuo; Tobimatsu, Haruki (National Rehabilitation Center for the Disabled Hospital, Tokorozawa, Saitama (Japan))

    1991-08-01

    Cerebrospinal fluid (CSF) flow within the spinal canal and syrinx in posttraumatic syringomyelia were studied by cardiac-gated phase images of magnetic resonance imaging in 12 normal volunteers and 8 patients with syringomyelia. The cardiac-gated phase method was simple and useful for detection of CSF flow. Phase modulation was in direct proportion to flow velocity. Phase modulation was not affected by the T1 or T2 relaxation time. In normal volunteers, CSF flows caudally during systole and cranially during diastole. The maximum caudal CSF flow velocity at C2 level was from 0.45 cm/sec to 1.71 cm/sec, average; 1.27 cm/sec. All of symptomatic posttraumatic syringomyelia patients had the flow in the syrinx. (author).

  18. Acute respiratory failure as a first manifestation of syringomyelia

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    Al Bashapshe Ali

    2010-01-01

    Full Text Available A 40 year old woman presented with a short history of acute onset of breathlessness to the ER of our hospital and after initial evaluation for acute pulmonary embolism which was ruled out after carrying out the appropriate investigations, she was diagnosed to be afflicted with syringomyelia based on her neurological symptoms and clinical findings, which was confirmed by doing an MRI scan, which was her basic diagnosis that was complicated by acute hypercapnic respiratory failure. This case is being reported to highlight syringomyelia as an unusual cause of acute respiratory failure, which manifested clinically in this patient as its first presentation and the underlying neurological diagnosis has been found to be present in very few reported cases (less than 0.01% of case reports in the available literature as the basic disease in the absence of its classical presenting features. Problems associated with acute respiratory failure in the setting of syringomyelia are discussed.

  19. Small hepatic veins Budd-Chiari syndrome.

    Science.gov (United States)

    Riggio, Oliviero; Marzano, Chiara; Papa, Alessia; Pasquale, Chiara; Gasperini, Maria Ludovica; Gigante, Antonietta; Valla, Dominique Charles; Plessier, Aurélie; Amoroso, Antonio

    2014-05-01

    Budd-Chiari syndrome is a rare disorder characterized by hepatic venous outflow obstruction at any level from the small hepatic veins to the atrio-caval junction, in the absence of heart failure or constrictive pericarditis. Various imaging modalities are available for investigating the gross hepatic vascular anatomy but there are rare forms of this disease where the obstruction is limited to the small intrahepatic veins, with normal appearance of the large hepatic veins at imaging. In this cases only a liver biopsy can demonstrate the presence of a small vessels outflow block. We report two cases of small hepatic veins Budd-Chiari syndrome.

  20. Syringomyelia after operation: diagnosis and its formational mechanism

    Institute of Scientific and Technical Information of China (English)

    Shi Jiangang; Xu Guohua; Jia Lianshun; Yuan Wen; Shi Guodong; Wu Jianfeng; Ye XiaoJian; Ni Bin; Xiao Jianru; Tan Junming

    2008-01-01

    Objective: To describe the MRI findings and discuss the pathogenesis formation mechanism of syringomyelia in the patients after spine surgical operation. Methods: Totally 13 patients were been enrolled in the study. Before operation, none of our patients presented with spinal cord syringomyelia. The mean follow-up duration was 6 years (range 2-10 months) by MRI. Four of the 13 patients after spinal cord tumor removed operation, 3 patients after spinal trauma operation, 2 cases had scoliosis before, 2 cases were cervical spondylotic myelopathy and 2 cases had tethered cord syndrome before operation. MR features of pre- and post-operation on all patients were been studied in contrast with surgical results and clinical symptoms. The radiology diagnostic was made by 3 different radiologists respectively. The characteristics of length, width, signals, shape of cavity and spinal cord position as well as subarachnoid shape were focused on. Results: All the patients had no syringomyelia on MRI before operation. The spinal cord of 7 patients showed persist compression and 2 patients had tethered cord before operation. MRI features of syringomyelia after operation in our cases showed longitudinal cavity with syrinx fluid had T1 and T2 relation characteristics of cerebrospinal fluid (CSF). The mean length was 5.5 spinal segment, 4 cord of 13 patients presented cord compressed from anterior materials, 4 occurred postoperative adhesion to the back of lumbar spinal canal and spinal cord of 1 cases clung to the front wall of cervical spinal canal. Conclusion: The mechanism of syringomyelia in the patients after spinal surgical intervention may be the persisting compression or intention of the spinal cord in the period of pre- or post-operation. The edema, cyst, malacia of spinal cord are the most important lesions and risk factors resulted in the syringomyelia.

  1. Syringomyelia presenting with unilateral optic neuropathy: a case report

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    Ngoo QZ

    2017-03-01

    Full Text Available Qi Zhe Ngoo, Evelyn Li Min Tai, Wan Hazabbah Wan Hitam Department of Ophthalmology, School of Medical Sciences, Universiti Sains Malaysia, Kubang Kerian, Malaysia Purpose: In this case report, we present two cases of syringomyelia with optic neuropathy.Findings: In Case 1, a 36-year-old Malay lady presented to our clinic with acute onset of blurring of vision in her left eye that she experienced since past 1 month. She was diagnosed with syringomyelia 12 years ago and was on conservative management. Her visual acuity was 6/6 in the right eye and counting fingers at 1 m in the left. There was a positive relative afferent pupillary defect in her left eye. Optic nerve functions of her left eye were reduced. Visual field showed a left inferior field defect. Her extraocular muscle movements were full. Magnetic resonance imaging of the brain and spine showed syringomyelia at the level of C2–C6 and T2–T9. Both of her optic nerves were normal. Her condition improved with intravenous and oral corticosteroids. In Case 2, a 44-year-old Malay lady presented to our clinic with a progressive central scotoma in her right eye that she experienced since past 1 month. She had previous history of recurrent episodes of weakness in both of her lower limbs from past 8 months. Visual acuity in her right and left eye was 6/9 and 6/6, respectively. The relative afferent pupillary defect in her right eye was positive. Optic nerve functions of her right eye were affected. Visual field showed a central scotoma in her right eye. Her extraocular muscle movements were full. Fundoscopy of her right eye showed a pale optic disc. Her left eye fundus was normal. Magnetic resonance imaging of the brain and spine showed syringomyelia at T3–T6. Both of her optic nerves were normal. A diagnosis of syringomyelia with right optic atrophy was performed. Her condition improved with intravenous and oral corticosteroids.Conclusion: Optic neuropathy is a rare neuro

  2. Surgical treatment of idiopathic syringomyelia: Silastic wedge syringosubarachnoid shunting technique

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    Teck M Soo

    2014-01-01

    Conclusions: Shunting procedures for the syringomyelia disease spectrum have been criticized due to the inconsistent long-term outcomes. This surgical technique used to treat symptomatic idiopathic syringomyelia has been devised based on our intraoperative experience, surgical outcomes, and evaluation of the literature. The purpose of the wedges is to preserve patency of the communication between the syrinx cavity and the expanded subarachnoid space by preventing healing of the myelotomy edges and by maintaining an artificial conduit between the syrinx cavity and the subarachnoid space. Although short-term results are promising, continued long-term follow up is needed to determine the ultimate success of the silastic wedge shunting procedure.

  3. Clinical value of extension-flexion MRI in evaluating the indication of internal fixation for patients of craniovertebral junction malformation%前屈-后伸位MRI对判断颅脊交界区畸形内固定指征的临床价值

    Institute of Scientific and Technical Information of China (English)

    陈赞; 孙永华; 吴浩; 王兴文; 菅凤增

    2011-01-01

    malformation patients without instability of CVJ.For patients who were diagnosed instability of CVJ, occipitocervical internal fixation and fusion was indicated besides decompression.Results Twelve patients were diagnosed as instability of CVJ, and occipitocervical internal fixation and fusion, foramen magnum decompression and cisterna magna plasty were performed.In the 40 patients, the syringomyelia of 10 patients shrinked on the MRI on the third day postoperatively.There was no movement of the instruments in 12 patients with internal fixation, and the spinal cord was well decompressed.Three months after surgery, the syringomyelia of 17 patients obviously shrinked in MRI.The bony bridge was demonstrated in CT in 12 cases.Japanese Orthopedic Association (JOA) score at 3 months after surgery was 13.08 ± 1.40, and was higher than that of preoperation significantly (t = 5.928, P = 0.000).Conclusion Cervical EFMRI has significant value in the evaluation of possible AAD in patients of Arnold-Chiari malformation, and can help to determine the surgical strategy.

  4. Clinical manifestations and significance of post-traumatic thoracolumbar syringomyelia

    Institute of Scientific and Technical Information of China (English)

    邱勇; 朱泽章; 吕锦瑜; 王斌; 李卫国; 朱丽华

    2004-01-01

    Objective: To analyze the pathogenic mechanism and the clinical significance of post-traumatic thoracolumbar syringomyelia through reviewing the clinical manifestations. Methods: The data of 15 patients (14 males and 1 female, aged from 28 to 56 years, with an average of 36 years) with post-traumatic syringomyelia treated in our hospital from December 1997 to February 2002 were studied retrospectively. Two patients suffered from T11 fractures, 7 from T12 fractures and 6 from L1 fractures. There were 12 patients with burst fractures and 3 with fracture dislocations. Anterior decompression, bone graft, bone fusion and internal fixation were made on 6 patients, posterior decompression, bone graft, bone fusion and internal fixation on 1 patient, and non-surgical treatment on 8 patients. Results: Syringomyelia of the patients was diagnosed accurately with magnetic resonance imaging at 0.5-4 years after the original thoracolumbar fracture. The cavern was round in 6 cases, elliptic in 6 cases, and irregular in 3 cases. The patients also suffered from pain (80%), myodynamia attenuation in lower extremities (66.7%), aggravated spasm (46.7%), sensation loss or hypesthesia (46.7%), decreased coordinate function of lower extremities (20%) and autonomic nerve symptom (6.7%).Conclusions: Post-traumatic thoracolumbar syringomyelia should be suspected if the patient has new neurological symptoms, such as myodynamia attenuation in lower extremities, after the neural function becomes stable for certain time.

  5. THE PATHOGENESIS OF SYRINGOMYELIA IN SPINAL-CORD EPENDYMOMA

    NARCIS (Netherlands)

    LOHLE, PNM; WURZER, HAL; HOOGLAND, PH; SEELEN, PJ; GO, KG

    1994-01-01

    A spinal cord ependymoma with syringomyelia is presented. The pathogenesis of syrinx formation, associated with intramedullary tumors is not fully understood. In order to examine the mechanism of formation of the tumor-associated syrinx, syrinx fluid was obtained during surgery and concentrations of

  6. Magnetic resonance imaging in craniovertebral junction anomaly

    Energy Technology Data Exchange (ETDEWEB)

    Tada, Shimpei; Hata, Yuichi; Miyamoto, Yukio

    1985-03-01

    Materials consisted of 6 cases with occipitalization of the atlas, (4 of them complicated by basilar impression), 7 with basilar impression, one with hypoplasia of the atlas and C2-3 fusion, and one with os odontoideum. Basal angles after Welcker were all more than 130 in contrast to 118-138 (127 an average) in control group. Basal angle more than 140 denoted platybasia. Syringomyelia was seen in 7 of all 15 cases and 4 of 5 cases with platybasia. Chiari malformation was seen in 9 of all 15 cases and 4 of 5 with platybasia. Basal angles were closely related to craniovertebral junction bone anomaly, syringomyelia, and Chiari malformation. (author).

  7. Post-traumatic syringomyelia; Die posttraumatische Syringomyelie

    Energy Technology Data Exchange (ETDEWEB)

    Freund, M.; Sartor, K. [Universitaetsklinikum Heidelberg (Germany). Abt. Neuroradiologie; Aschoff, A. [Universitaetsklinikum Heidelberg (Germany). Neurochirurgische Klinik; Spahn, B. [Universitaetsklinikum Heidelberg (Germany). Abt. Orthopaedie 2

    1999-12-01

    The improvement of preclinical emergency medicine, better surgical and conservative therapies, and the development of intensive care units and specialized centers have improved the survival rate for patients with serious spinal cord injuries. Therefore, more sequelae of chronic spinal cord injuries such as post-traumatic spinal cord cavitations also occur. The first such case was described by Bastian in 1867. Generally, these cavitations were diagnosed from 2 months up to 32 years after the trauma. The overall prevalence of post-traumatic syringomyelia (PTS) is not known; however, with the increasing use of magnetic resonance imaging (MRI), its diagnosis has increased, ranging from 2.3% of paraplegic and tetraplegic patients in 1976 and 3.2% in 1985, to nearly 50% in a selected group of patients in 1991 and 1993. In 1995, a 4.45% incidence was reported. In our clinic we are currently treating 440 cases of syringomyelia, 140 of which are PTS. Several observations suggest more than one potential mechanism for the evolution of a post-traumatic cyst or PTS. Various factors, such as hemorrhage or, in particular, ischemia within the spinal cord, blockage of the cerebrosinal fluid (CSF) pathways around the cord or localized meningeal fibrosis either alone or in combination with other factors, may be involved. Clinically, sensory disturbances, loss of motor function, pain, and modification of the deep tendon reflexes are observed in most patients. On MRI, PTS is seen as a longitudinal, cystic cavity within the spinal cord, giving a hypointense signal on T{sub 1}-weighted images and a hyperintense signal on T{sub 2}-weighted images. For treatment planning it is mandatory to identify the lower and upper end of the PTS on the MRI. (orig.) [German] Aufgrund der verbesserten medizinischen Versorgung schwer Rueckenmarkverletzter hat die Zahl der (Langzeit-) Ueberlebenden erheblich zugenommen. Allerdings koennen Spaetkomplikationen auftreten, die zu einer deutlichen Zunahme der

  8. Syringomyelia associated with cervical spondylosis: A rare condition.

    Science.gov (United States)

    Landi, Alessandro; Nigro, Lorenzo; Marotta, Nicola; Mancarella, Cristina; Donnarumma, Pasquale; Delfini, Roberto

    2013-06-16

    Spinal spondylosis is an extremely common condition that has only rarely been described as a cause of syringomyelia. We describe a case of syringomyelia associated with cervical spondylosis admitted at our division and treated by our institute. It is the case of a 66-year-old woman. At our observation she was affected by moderate-severe spastic tetraparesis. T2-weighted magnetic resonance imaging (MRI) showed an hyperintense signal within spinal cord from C3 to T1 with a more sharply defined process in the inferior cervical spinal cord. At the same level bulging discs, facets and ligamenta flava hypertrophy determined a compression towards subarachnoid space and spinal cord. Spinal cord compression was more evident in hyperextension rather than flexion. A 4-level laminectomy and subsequent posterior stabilization with intra-articular screws was executed. At 3-mo follow up there was a regression of tetraparesis but motor deficits of the lower limbs residuated. At the same follow up postoperative MRI was executed. It suggested enlargement of the syrinx. Perhaps hyperintensity within spinal cord appeared "bounded" from C3 to C7 with clearer margins. At the level of surgical decompression, subarachnoid space and spinal cord enlargement were also evident. A review of the literature was executed using PubMed database. The objective of the research was to find an etiopathological theory able to relate syringomyelia with cervical spondylosis. Only 6 articles have been found. At the origin of syringomyelia the mechanisms of compression and instability are proposed. Perhaps other studies assert the importance of subarachnoid space regard cerebrospinal fluid (CSF) dynamic. We postulate that cervical spine instability may be the cause of multiple microtrauma towards spinal cord and consequently may damage spinal cord parenchyma generating myelomalacia and consequently syrinx. Otherwise the hemorrhage within spinal cord central canal can cause an obstruction of CSF outflow

  9. Anorectal malformations

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    Peña Alberto

    2007-07-01

    Full Text Available Abstract Anorectal malformations comprise a wide spectrum of diseases, which can affect boys and girls, and involve the distal anus and rectum as well as the urinary and genital tracts. They occur in approximately 1 in 5000 live births. Defects range from the very minor and easily treated with an excellent functional prognosis, to those that are complex, difficult to manage, are often associated with other anomalies, and have a poor functional prognosis. The surgical approach to repairing these defects changed dramatically in 1980 with the introduction of the posterior sagittal approach, which allowed surgeons to view the anatomy of these defects clearly, to repair them under direct vision, and to learn about the complex anatomic arrangement of the junction of rectum and genitourinary tract. Better imaging techniques, and a better knowledge of the anatomy and physiology of the pelvic structures at birth have refined diagnosis and initial management, and the analysis of large series of patients allows better prediction of associated anomalies and functional prognosis. The main concerns for the surgeon in correcting these anomalies are bowel control, urinary control, and sexual function. With early diagnosis, management of associated anomalies and efficient meticulous surgical repair, patients have the best chance for a good functional outcome. Fecal and urinary incontinence can occur even with an excellent anatomic repair, due mainly to associated problems such as a poorly developed sacrum, deficient nerve supply, and spinal cord anomalies. For these patients, an effective bowel management program, including enema and dietary restrictions has been devised to improve their quality of life.

  10. Allergy to Prolene Sutures in a Dural Graft for Chiari Decompression

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    Iahn Cajigas

    2015-01-01

    Full Text Available Allergy to Prolene suture is exceedingly rare with only 5 cases reported in the literature. There have been no such cases associated with neurosurgical procedures. Diagnosis is nearly always delayed in spite of persistent symptomatology. A 27-year-old girl with suspected Ehlers-Danlos, connective tissue disorder, underwent posterior fossa decompression for Chiari Type 1 malformation. One year later, the patient presented with urticarial rash from the neck to chest. Cerebrospinal fluid and blood testing, magnetic resonance imaging, and intraoperative exploration did not suggest allergic reaction. Eventually skin testing proved specific Prolene allergy. After suture material was removed, the patient no longer complained of pruritus or rash. This single case highlights the important entity of allergic reaction to suture material, namely, Prolene, which can present in a delayed basis. Symptomatology can be vague but has typical allergic characteristics. Multidisciplinary approach is helpful with confirmatory skin testing as a vital part of the workup.

  11. Aquaporin-4 expression in post-traumatic syringomyelia.

    Science.gov (United States)

    Hemley, Sarah J; Bilston, Lynne E; Cheng, Shaokoon; Chan, Jing Ning; Stoodley, Marcus A

    2013-08-15

    Aquaporin-4 (AQP4) is an astroglial water channel protein that plays an important role in the transmembrane movement of water within the central nervous system. AQP4 has been implicated in numerous pathological conditions involving abnormal fluid accumulation, including spinal cord edema following traumatic injury. AQP4 has not been studied in post-traumatic syringomyelia, a condition that cannot be completely explained by current theories of cerebrospinal fluid dynamics. Alterations of AQP4 expression or function may contribute to the fluid imbalance leading to syrinx formation or enlargement. The aim of this study was to examine AQP4 expression levels and distribution in an animal model of post-traumatic syringomyelia. Immunofluorescence and western blotting were used to assess AQP4 and glial fibrillary acidic protein (GFAP) expression in an excitotoxic amino acid/arachnoiditis model of post-traumatic syringomyelia in Sprague-Dawley rats. At all time-points, GFAP-positive astrocytes were observed in tissue surrounding syrinx cavities, although western blot analysis demonstrated an overall decrease in GFAP expression, except at the latest stage investigated. AQP4 expression was significantly higher at the level of syrinx at three and six weeks following the initial syrinx induction surgery. Significant increases in AQP4 expression also were observed in the upper cervical cord, rostral to the syrinx except in the acute stage of the condition at the three-day time-point. Immunostaining showed that AQP4 was expressed around all syrinx cavities, most notably adjacent to a mature syrinx (six- and 12-week time-point). This suggests a relationship between AQP4 and fluid accumulation in post-traumatic syringomyelia. However, whether this is a causal relationship or occurs in response to an increase in fluid needs to be established.

  12. [Post-traumatic syringomyelia: What should know the urologist?].

    Science.gov (United States)

    Caremel, R; Hamel, O; Gerardin, E; Lenormand, L; Parker, F; Lefort, M; Grise, P; Perrouin-Verbe, B

    2013-01-01

    The incidence of post-traumatic syringomyelia (PTS) is estimated according to recent studies at 25 to 30% of patients with traumatic spinal cord injuries in magnetic resonance imaging (MRI), which remains the gold standard exam for syringomyelia diagnosis and monitoring. Syringomyelia is translated by an increased cord signal (similar to CSF) with low-density T1-weighted image and high-density T2-weighted image, which extends beyond site of initial lesion at least to two vertebral segments. Two conditions are required for development of PTS: traumatic spinal cord injury and blocked the flow of CSF epidural. The mean interval from spinal cord injury to diagnosis SPT was 2.8years (range, 3months to 34years). The commonest symptoms are pain and sensory loss. PTS should be suspected if the patient has new neurological symptoms above level of injury, such as dissociated sensory injuries, reflexes abolition, and motor deficit, after the neural function becomes stable for certain time. In urologic practice, new neurological symptoms could be bladder and/or erectile dysfunction. The medical management based on prevention efforts with closed-glottis pushing, which could aggravate the syrinx cavity. In urology, extracorporeal shockwave lithotripsy, and laparoscopic or robotic surgery could extend the syrinx cavity for the same reason (increase abdominal pressure). The indications for surgical intervention and optimal surgical treatment technique for patients with PTS are not consensual. The literature demonstrated that surgery PTS is effective at arresting or improving motor deterioration, but not sensory dysfunction or pain syndromes.

  13. Secondary holocord syringomyelia with spinal hemangioblastoma: A report of two cases

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    Pai S

    2003-01-01

    Full Text Available Intramedullary spinal hemangioblastoma is well known to be accompanied by syringomyelia. However, holocord secondary syringomyelia is uncommon. We present 2 cases of spinal hemangioblastoma, one in the conus medullaris and the other in midthoracic region, accompanied by holocord syrinx. In both the cases the secondary syrinx resolved following successful total tumor excision with good neurological recovery.

  14. Syringomyelia in demyelinating disease of the central nervous system: Report of two cases

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    Savić Dejan

    2011-01-01

    Full Text Available Introduction. Syringomyelia is a cavitary extension inside the spinal cord which can be either symptomatic or congenitally-idiopathic. Syringomyelia during the course of the disease in patients presenting with clinically definite multiple sclerosis was described earlier. Syringomyelia in patients presenting with a clinically isolated syndrome suggestive of multiple sclerosis is unusual. Case Outline. We present two patients presenting with demy-elinating disease of the central nervous system with syringomyelia in the cervical and thoracic spinal cord. We did not find classical clinical signs of syringomyelia in our patients, but we disclosed syringomyelia incidentally during magnetic resonance exploration. Magnetic resonance exploration using the gadolinium contrast revealed the signs of active demyelinating lesions in the spinal cord in one patient but not in the other. Conclusion. Syringomyelia in demyelinating disease of the central nervous system opens the question whether it is a coincidental finding or a part of clinical features of the disease. Differentiation of the significance of syringomyelia finding in these patients plays a role in the choice of treatment concept in such patients.

  15. Spinal arachnoid cysts associated with syringomyelia: a review of the literature and report of a case.

    Science.gov (United States)

    Tucer, Bulent; Yilmaz, Muhammet Bahadır; Ekici, Mehmet Ali; Menku, Ahmet; Koc, Kemal

    2014-01-01

    Syringomyelia is a not infrequent pathology that develops related to changes in cerebrospinal fluid dynamics due to many etiological factors. The development of syringomyelia through a spinal arachnoid cyst is quite rare and has been defined in only 31 cases in the literature. A case due to thoracic epidural lipomatosis has also been reported. There have been no previous reports of thoracic region epidural lipomatosis with underlying arachnoid cyst and syringomyelia as in our case. We present a 27-year-old patient who developed thoracic arachnoid cyst and underlying syringomyelia because of the pressure of the thoracic epidural fat tissue and also evaluate the characteristics of patients with syringomyelia by virtue of an arachnoid cyst previously reported in the literature.

  16. External validity of the chiari severity index and outcomes among pediatric chiari I patients treated with intra- or extra-Dural decompression.

    Science.gov (United States)

    Pisapia, Jared M; Merkow, Maxwell B; Brewington, Danielle; Henn, Rosemary E; Sutton, Leslie N; Storm, Phillip B; Heuer, Gregory G

    2017-02-01

    Chiari malformation type-1 (CM-1) may be treated by intradural (ID) or extradural (ED) posterior fossa decompression, although the optimal approach is debated. The Chiari Severity Index (CSI) is a pre-operative metric to predict patient-defined improvement after CM-1 surgery. In this study, we evaluate the results of ID versus ED decompression and assess the external validity of the CSI. We performed a retrospective cohort study of pediatric CM-1 patients undergoing decompression at a single academic children's hospital. Characteristics of headache, syrinx, and myelopathy were collected to derive CSI grade. The primary outcome measure was pre-operative symptom resolution. The proportion of patients with favorable outcome was tabulated for each of the three CSI grades and compared to previously published results. From 2004 to 2014, 189 patients underwent ID (48%) or ED (52%) decompression at the Children's Hospital of Philadelphia (CHOP). Follow-up ranged from 1 to 75 months. Rates of symptom resolution (58-64%) and reoperation (8%) were similar regardless of surgical approach. Although proportions of favorable outcomes differed between the CHOP and Washington University (WU) cohorts, the difference was not related to CSI grade (p = 0.63). Furthermore, there was no difference in the proportion of favorable outcomes between the two cohorts regardless of ID (p = 0.26) or ED approach (p = 0.11). Equivalent rates of symptom resolution and reoperation following ID and ED decompression support the ED approach as a first-line surgical option for pediatric CM-1 patients. In addition, our findings provide preliminary evidence supporting the generalizability of the CSI and its use in future comparative trials.

  17. [Syringosubarachnoid shunt for noncommunicating syringomyelia associated with spinal lipoma: a case report].

    Science.gov (United States)

    Yamamuro, Shun; Morishita, Takashi; Watanabe, Takao; Maejima, Sadahiro; Katayama, Yoichi

    2012-06-01

    Noncommunicating syringomyelia may complicate repeated untethering procedures in patients with spinal lipomas. Surgical intervention is indicated when syringomyelia is symptomatic. However, the preferred surgical procedure for noncommunicating syringomyelia associated with spinal lipomas has not been established. The authors report a case of noncommunicating syringomyelia associated with a spinal lipoma which was successfully treated with a syringosubarachnoid (S-S) shunt. The patient was a 9-year-old girl with a past history of an excision of a dermal sinus at the age of three months and an untethering of the spinal cord at the age of five. She developed a gait disturbance in the year prior to surgery, and a magnetic resonance imaging (MRI) study demonstrated syringomyelia from the second thoracic vertebra to the fourth lumbar vertebra. S-S shunt surgery successfully addressed the syringomyelia without adverse events, and a follow-up MRI study showed reduction of the size of the lesion. S-S shunt placement was therefore considered to be an effective treatment option for noncommunicating syringomyelia associated with a spinal lipoma.

  18. Endovascular treatment of Budd-Chiari syndrome

    Institute of Scientific and Technical Information of China (English)

    MENG Qing-yi; SUN Nian-feng; WANG Jia-xiang; WANG Rui-hua; LIU Zhao-xuan

    2011-01-01

    Background Budd-Chiari syndrome (BCS) is a posthepatic portal hypertension caused by the obstruction of the lumen of the hepatic veins or the proximal inferior vena cava (IVC).This study aimed to evaluate the clinical experience of interventional therapy for Budd-Chiari syndrome.Methods IVC venography was carried out first,the obliteration or stenosis in the IVC was opened or dilated with the hard guided wire or Rups100 puncture needle and balloon,then a stent was routinely implanted for the type of obliteration or stenosis.Results The procedure was successful in 821 out of 903 cases including IVC intervention in 760 cases,and hepatic vein intervention in 61 cases.An IVC stent was used in 517 cases and hepatic vein stent in 19 cases.There were no pulmonary embolisms,but acute renal failure occurred in eight cases,hepatic coma in two cases and acute heart failure in 43 cases.Two patients died in this group and five cases were complicated with acute IVC thrombosis.Follow up of 7 to 124 months was made in 679 cases with recurrence found in 59 cases.Conclusions Interventional therapy is safe and effective with a fast recovery for most types of BCS.It is gradually becoming the first therapeutic choice.

  19. Radical correction of Budd-Chiari syndrome

    Institute of Scientific and Technical Information of China (English)

    LI Xiao-qiang; WANG Zhong-gao; MENG Qing-you; SANG Hong-fei; QIAN Ai-min; DUAN Peng-fei; RONG Jian-jie

    2007-01-01

    Background Interventional therapy is widely accepted as the first choice for the treatment of the Budd-Chiari syndrome,but the use of radical correctional therapy should not be discarded. This study describes radical correction by controlling bleeding from distal end of pathological segment of the inferior vena cava (IVC) and discusses potential surgical errors and postoperative complications.Methods Of the 216 patients in the study, 78 were treated with simple membranectomy, 64 with dissection of the pathological segment of the IVC and vascular prosthesis or pericardial patch plasty, 60 with resection of the pathological segment of the IVC and orthotopic graft transplantation with vascular prosthesis, and 14 with resection of the occlusive main hepatic vein and its upper IVC, hepatic venous outflow plasty and vascular prosthesis orthotopic graft transplantation from the hepatic venous entrance to the IVC of right atrial ostium.Results Except 14 cases who were discharged after hepatic vein outflow plasty, four cases died postoperatively, and 198 patients were discharged without complications. The symptoms of 15 patients were relieved partially and 2 without any change. There were no deaths intraoperatively. Of the 112 cases who were followed up for 72 months, 13 suffered from a relapse.Conclusions Radical correction is a beneficial therapy in the treatment of Budd-Chiari syndrome.

  20. Volume change theory for syringomyelia: A new perspective.

    Science.gov (United States)

    Rai, Survendra Kumar Rajdeo; Rai, Pooja Survendra Kumar

    2015-01-01

    The etiopathogenesis of syringomyelia is still an enigma. The authors present a novel theory based on fluid dynamics at the craniovertebral (CV) junction to explain the genesis of syringomyelia (SM). The changes in volume of spinal canal, spinal cord, central canal and spinal subarachnoid space (SSS) in relation to the posterior fossa have been analysed, specifically during postural movements of flexion and extension. The effect of fluctuations in volume of spinal canal and its contents associated with cerebrospinal fluid (CSF) flow dynamics at the CV junction have been postulated to cause the origin and propagation of the syringomyelia. The relevant literature on the subject has been reviewed and the author's theory has been discussed. Volume of spinal canal in flexion is always greater than that in extension. Flexion of spine causes narrowing of the ventral subarachnoid space (SAS) and widening of dorsal SAS while extension causes reverse changes leading to fluid movement in dorsal spinal SAS in flexion and ventral spinal SAS in extension. Cervical and lumbar spinal region with maximum bulk hence maximum area and volume undergo maximum deformation during postural changes. SSS CSF is the difference between the volume of spinal canal and spinal cord, varies in flexion and extension which is compensated by changes in posterior fossa (CSF) volume in normal circumstances. Blocked SAS at foramen magnum donot permit spinal SAS CSF exchange which during postural changes is compensated by cavitatory/cystic (syrinx) change at locations in cervical and lumbar spine with propensity for maximum deformation. Augmentation of posterior fossa volume by decompression helps by normalization of this CSF exchange dynamics but immobilizing the spinal movement theoretically will cease any dynamic volume changes thereby minimizing the destructive influence of the fluid exchange on the cord. Thus, this theory strengthens the rational of treating patients by either methodology.

  1. The roads to mitochondrial dysfunction in a rat model of posttraumatic syringomyelia.

    Science.gov (United States)

    Hu, Zhiqiang; Tu, Jian

    2015-01-01

    The pathophysiology of posttraumatic syringomyelia is incompletely understood. We examined whether local ischemia occurs after spinal cord injury. If so, whether it causes neuronal mitochondrial dysfunction and depletion, and subsequent energy metabolism impairment results in cell starvation of energy and even cell death, contributing to the enlargement of the cavity. Local blood flow was measured in a rat model of posttraumatic syringomyelia that had received injections of quisqualic acid and kaolin. We found an 86 ± 11% reduction of local blood flow at C8 where a cyst formed at 6 weeks after syrinx induction procedure (P syringomyelia.

  2. [Case of rapidly progressive syringomyelia due to a spinal hemangioblastoma].

    Science.gov (United States)

    Sakakibara, Satoko; Tamura, Takuya; Katayama, Taiji; Saito, Yufuko; Aiba, Ikuko; Inukai, Akira

    2014-01-01

    A 35-year-old man came to the hospital showing signs of worsening dysesthesia on his right hand. The dysesthesia started on his right hand and then spread to his forearm in two months. It also appeared on his left hand transiently. Initial MR imaging revealed a high signal intensity lesion at Th1-Th10 with an irregular margin (presyrinx state) below C3 on T2WI. The legion extended up to the medulla oblongata rapidly. Corticosteroid therapy lead to a slight improvement in dysesthesia symptoms but did not last. Immunosuppressant was also ineffective. Further examination using Gd enhanced MR imaging in a neurosurgery clinic in a university hospital revealed a spinal tumor at the Th10 level. A tumor resection was performed and dysesthesia improved. Pathological analysis showed hemangioblastoma. Presyrinx and syrinx above Th1 disappeared after the operation. It is necessary to search the whole spine carefully for the possibility of a tumor in the case of steroid resistant progressive spinal lesions with an unknown origin. And we stress the importance of timely surgical intervention regardless of idiopathic or secondary syringomyelia. We would like to report this clinical course presenting MR imaging and discuss the mechanism of forming syringomyelia based on the hypothesis of the alteration of CSF flow.

  3. Giant posterior fossa arachnoid cyst causing tonsillar herniation and cervical syringomyelia

    Directory of Open Access Journals (Sweden)

    Vijay P Joshi

    2013-01-01

    Full Text Available Acquired cerebellar tonsillar herniation and syringomyelia associated with posterior fossa mass lesions is an exception rather than the rule. In the present article, we describe the neuroimaging findings in a case of 28-year-old female patient presented with a history of paraesthesia involving right upper limb of 8-month duration. Magnetic resonance imaging showed a giant retrocerebellar arachnoid causing tonsillar herniation with cervical syringomyelia. The findings in the present case supports that the one of the primary mechanism for the development of syringomyelia may be the obstruction to the flow of cerebrospinal fluid causing alterations in the passage of extracellular fluid in the spinal cord and leading to syringomyelia.

  4. Chiari-related hydrocephalus: assessment of clinical risk factors in a cohort of 297 consecutive patients.

    Science.gov (United States)

    Guan, Jian; Riva-Cambrin, Jay; Brockmeyer, Douglas L

    2016-11-01

    OBJECTIVE Patients treated for Chiari I malformation (CM-I) with posterior fossa decompression (PFD) may occasionally and unpredictably develop postoperative hydrocephalus. The clinical risk factors predictive of this type of Chiari-related hydrocephalus (CRH) are unknown. The authors' objective was to evaluate their experience to identify risk factors that may predict which of these patients undergoing PFD will develop CRH after surgery. METHODS The authors performed a retrospective clinical chart review of all patients who underwent PFD surgery and duraplasty for CM-I at the Primary Children's Hospital in Utah from June 1, 2005, through May 31, 2015. Patients were dichotomized based on the need for long-term CSF diversion after PFD. Analysis included both univariate and multivariable logistic regression analyses. RESULTS The authors identified 297 decompressive surgeries over the period of the study, 22 of which required long-term postoperative CSF diversion. On multivariable analysis, age < 6 years old (OR 3.342, 95% CI 1.282-8.713), higher intraoperative blood loss (OR 1.003, 95% CI 1.001-1.006), and the presence of a fourth ventricular web (OR 3.752, 95% CI 1.306-10.783) were significantly associated with the need for long-term CSF diversion after decompressive surgery. CONCLUSIONS Younger patients, those with extensive intraoperative blood loss, and those found during surgery to have a fourth ventricular web were at higher risk for the development of CRH. Clinicians should be alert to evidence of CRH in this patient population after PFD surgery.

  5. Diagnosis and surgical treatment of terminal syringomyelia within spinal cord combined with tethered cord syndrome

    OpenAIRE

    Jing-cheng XIE; Wang, Zhen-Yu; Chen, Xiao-Dong

    2016-01-01

    Objective To summarize the clinical manifestations, imaging characteristics and experience of surgical treatment of spinal cord terminal syringomyelia with tethered cord syndrome (TCS).  Methods and Results Clinical data of 10 patients with spinal cord syringomyelia combined with TCS surgically treated under microscope from January 1999 to March 2014 in our hospital were retrospectively analyzed. There were 3 males and 7 females with average age of 15.06 years old (ranged from 2 to 35 y...

  6. Split Cord Malformations

    Directory of Open Access Journals (Sweden)

    Yurdal Gezercan

    2015-06-01

    Full Text Available Split cord malformations are rare form of occult spinal dysraphism in children. Split cord malformations are characterized by septum that cleaves the spinal canal in sagittal plane within the single or duplicated thecal sac. Although their precise incidence is unknown, split cord malformations are exceedingly rare and represent %3.8-5 of all congenital spinal anomalies. Characteristic neurological, urological, orthopedic clinical manifestations are variable and asymptomatic course is possible. Earlier diagnosis and surgical intervention for split cord malformations is associated with better long-term fuctional outcome. For this reason, diagnostic imaging is indicated for children with associated cutaneous and orthopedic signs. Additional congenital anomalies usually to accompany the split cord malformations. Earlier diagnosis, meticuolus surgical therapy and interdisciplinary careful evaluation and follow-up should be made for good prognosis. [Cukurova Med J 2015; 40(2.000: 199-207

  7. Budd-Chiari Syndrome in a Patient with Hepatitis C

    Directory of Open Access Journals (Sweden)

    Joseph Frankl

    2016-01-01

    Full Text Available Chronic Budd-Chiari syndrome can present with cirrhosis and signs and symptoms similar to those of other chronic liver diseases. We present a case of Budd-Chiari syndrome discovered during attempted transjugular intrahepatic portosystemic shunting in a patient with decompensated cirrhosis believed to be secondary to hepatitis C. Although the patient had hepatocellular carcinoma, the Budd-Chiari syndrome was a primary disease due to hepatic venous webs. Angioplasty was performed in this case, which resolved the patient’s symptoms related to portal hypertension. Follow-up venography 5 months after angioplasty demonstrated continued patency of the hepatic veins. A biopsy was obtained in the same setting, which showed centrilobular fibrosis indicating that venous occlusion was indeed the cause of cirrhosis. It is important to consider a second disease when treating a patient with difficult to manage portal hypertension.

  8. Cerebral palsy and congenital malformations

    DEFF Research Database (Denmark)

    Garne, Ester; Dolk, Helen; Krägeloh-Mann, Inge

    2007-01-01

    AIM: To determine the proportion of children with cerebral palsy (CP) who have cerebral and non-cerebral congenital malformations. METHODS: Data from 11 CP registries contributing to the European Cerebral Palsy Database (SCPE), for children born in the period 1976-1996. The malformations were...... classified as recognized syndromes, chromosomal anomalies, cerebral malformations or non-cerebral malformations. Prevalence of malformations was compared to published data on livebirths from a European database of congenital malformations (EUROCAT). RESULTS: Overall 547 out of 4584 children (11.9%) with CP...... were reported to have a congenital malformation. The majority (8.6% of all children) were diagnosed with a cerebral malformation. The most frequent types of cerebral malformations were microcephaly and hydrocephaly. Non-cerebral malformations were present in 97 CP children and in further 14 CP children...

  9. MR imaging of the spine in children

    Energy Technology Data Exchange (ETDEWEB)

    Duthoy, M.J.; Lund, G.

    1988-08-01

    MR imaging was reviewed in 66 pediatric spinal cord patients with diagnoses of posterior fossa tumor, primary spinal cord tumor, metastatic disease, neuroectodermal disorder, congenital malformation, trauma, and demyelinating, neurodegenerative, or metabolic disorders. MR proved to be useful in delineating the extent of posterior fossa and cord tumor including metastasis to the cord. MR was of limited value in demyelinating and metabolic disorders. Arnold Chiari malformations, syringomyelia, tethered cord and meningoceles were all easily evaluated using MR.

  10. Congenital Vascular Malformation

    Science.gov (United States)

    ... also be effective for small, localized birthmarks (port wine stains). Patients with a rare venous malformation (Kleppel– ... 3) non-profit organization focused on providing public education and improving awareness about vascular diseases. For more ...

  11. Pulmonary Arteriovenous Malformations

    DEFF Research Database (Denmark)

    Kjeldsen, A D; Oxhøj, H; Andersen, P E

    1999-01-01

    Hereditary hemorrhagic telangiectasia (HHT) is a dominantly inherited disease with a high prevalence of pulmonary arteriovenous malformations (PAVMs). The first symptom of HHT may be stroke or fatal hemoptysis associated with the presence of PAVM....

  12. Resolution of life-threatening dysphagia caused by caudal occipital malformation syndrome following foramen magnum decompressive surgery.

    Science.gov (United States)

    Graham, K J; Black, A P; Brain, P H

    2012-08-01

    A Cavalier King Charles Spaniel was presented with acute onset, life-threatening dysphagia suspected to be secondary to medulla oblongata compression caused by caudal occipital malformation syndrome. The patient required urgent tracheostomy tube placement to remain stable and was subsequently cured of the presenting neurological deficits by foramen magnum decompressive surgery. Neurogenic dysphagia is a relatively common presenting sign in human Chiari malformation syndromes, but has not been described as a major clinical sign in veterinary patients. Caudal occipital malformation syndrome should be included in the differential diagnosis list for susceptible breeds presenting with dysphagia. Early recognition favours expeditious surgical intervention and a positive outcome in human patients, and this may also be the case in veterinary patients. © 2012 The Authors. Australian Veterinary Journal © 2012 Australian Veterinary Association.

  13. Myelomeningocele associated with split cord malformation type I -three case reports-.

    Science.gov (United States)

    Higashida, Tetsuhiro; Sasano, Mari; Sato, Hironobu; Sekido, Ken'ichi; Ito, Susumu

    2010-01-01

    Three neonates presented with split cord malformation (SCM) associated with myelomeningocele (MMC), complicated with various coexisting anomalies. All patients were female and classified as SCM type I. All patients had a syrinx located rostral to the SCM. One patient had hydrocephalus and Chiari malformation causing serious respiratory problems. Two patients had partial hypertrichosis located close to the MMC, suggesting association with SCM. One patient had sacral hypoplasty and right kidney agenesis, suggesting that some embryologic errors may affect not only neural but also mesodermal development. All patients underwent surgical treatment for SCM after detailed evaluation and management of concomitant anomalies, and developed no new neurological deficits. Delayed surgery is an alternative treatment strategy for SCM in patients with both SCM and MMC with similar complications.

  14. Surgical treatment of the Amold-Chiari Malformation%Arnold-Chiari畸形手术治疗

    Institute of Scientific and Technical Information of China (English)

    王敬典; 谭涛; 刘宏建

    2006-01-01

    目的 总结Arnold-Chiari畸形临床表现、两种手术方法和治疗效果,以提高对Arnold-Chiari畸形诊断,改善手术效果.方法 回顾性分析一组66例病人,采用两种手术方法,包括颅后窝减压术和减压+空洞一蛛网膜下腔分流术.结果 后颅窝减压组,术后恢复良好者46例(88.5%),后颅窝减压+空洞蛛网膜下腔分流组术后恢复良好者11例(78.6%).结论 Arnold-Chiari畸形是一种常见病,手术是主要手段,后颅窝减压术或合并应用空洞-蛛网膜下腔分流术具有手术简单、有效、并发症少和安全等特点.

  15. MRI diagnosis of Chiari malformation%Chiari畸形的MRI表现

    Institute of Scientific and Technical Information of China (English)

    王振福; 吴伟; 李振之; 王晓华

    2001-01-01

    目的:探讨Chiari畸形的MRI表现,并评价其临床应用价值.方法:回顾性分析31例Chiari畸形患者的MRI资料与临床资料.结果:31例Chiari畸形患者小脑扁桃体疝至枕大孔平面下方3~25mn.20例合并脊髓空洞,枕大孔层面延前池前后径宽度为0~5mm,11例无脊髓空洞,延前池宽度为4.5~6mm,6例合并颅颈交界区畸形,4例合并脑积水.结论:小脑扁桃体位于枕大孔下方≥5mm即可诊断为Chiari畸形;延前池前后径宽度变窄(≤4mm)均出现脊髓空洞,而延前池宽度>4mm则很少出现脊髓空洞,临床可根据延前池的宽度来评价预后及的手术效果.

  16. MRI diagnosis of Chiari's malformation%Chiari畸形的MRI诊断

    Institute of Scientific and Technical Information of China (English)

    季洪兵; 郑春雨

    2007-01-01

    目的:探讨Chiari畸形的MRI影像表现,评价MRI对Chiari畸形的诊断价值.方法:回顾性分析MRI及手术证实的15例Chiari畸形的MRI表现.结果:小脑扁桃体下缘变尖12例.下疝至枕骨大孔平面下3.5~21 mm,12例伴有脊髓空洞.结论:MRI能清楚显示Chiari畸形病灶的部位及形态,是其最好的影像学检查方法;脊髓空洞是Chiari畸形主要合并症.

  17. Arnold-Chiari-II malformation and cognitive functioning in spina bifida.

    NARCIS (Netherlands)

    Vinck, A.; Maassen, B.A.M.; Mullaart, R.A.; Rotteveel, J.J.

    2006-01-01

    Spina bifida is a multifaceted neurological condition with complex neuropsychological sequelae. The cognitive outcome in spina bifida has frequently been attributed to the severity of the hydrocephalus. However, because of complex neuropathology, the influence of hydrocephalus alone does not suffici

  18. [Cerebrovenous orthostatic reactivity in pathology of the craniovertebral junction (Chiari malformation)].

    Science.gov (United States)

    Shakhnovich, V A; Mitrofanova, E V; Shimanskiy, V N; Konovalov, N A; Shkarubo, A N

    2015-01-01

    Мальформация Киари характеризуется вклинением миндалин мозжечка в большое затылочное отверстие, что приводит к нарушению циркуляции ликвора через краниовертебральный переход. Адекватным методом для выявления этих нарушений являются ортостатические нагрузки, которые приводят к перемещению ликвора через краниовертебральный переход. Возникающие при этом изменения внутричерепного давления оказывают влияние на церебровенозную ортостатическую реактивность (ЦВОР), которая оценивается неинвазивно при мальформации Киари. Материал и методы. Исследование проведено у 35 больных с мальформацией Киари (Киари I — 26 больных, Киари II — 9) в возрасте от 4 до 58 лет. Среди обследованных больных у 4 была выявлена гидроцефалия, у 6 — сирингомиелия. Методом транскраниальной допплерографии регистрировали венозный кровоток в прямом синусе мозга при изменении положения тела на ортостоле от +90° до –30°. Результаты. У больных с мальформацией Киари ЦВОР существенно отличается от нормы (более чем в 90% случаев) как ее увеличением (иногда в 5—6 раз по сравнению с верхней границей нормы — значительная гиперреактивность), так и полным отсутствием каких-либо изменений при ортостатической нагрузке (ареактивность). У больных c мальформацией Киари до хирургического лечения ЦВОР чаще всего характеризуется ареактивностью, а также умеренной или значительной гиперреактивностью. После хирургического лечения (декомпрессия большого затылочного отверстия) у больных с мальформацией Киари отмечается существенная нормализация краниовертебральных объемных соотношений, а ЦВОР чаще всего характеризуется нормореактивностью (63%), реже — умеренной гиперреактивностью. Скорость венозного кровотока в прямом синусе мозга при мальформации Киари до операции бывает повышена, а после хирургического лечения нормализуется. Выводы. Выявлена высокая частота нарушений ЦВОР (более 90% случаев) у больных с мальформацией Киари. После хирургического лечения этих больных больше чем в половине наблюдений (63%) отмечается полная нормализация ЦВОР.

  19. Arnold-Chiari畸形的临床分析%Clinical analysis of Arnold-Chiari malformation

    Institute of Scientific and Technical Information of China (English)

    王世清; 杨练球; 胡友权

    2008-01-01

    目的 探讨Arnold-Chiari畸形的临床特点及外科治疗效果.方法 回顾性分析1999年10月至2007年10月收治的15例Arnold-Chiari畸形患者的临床资料.结果 15例患者术前均经MRI确诊,行后颅窝减压及枕大池成形术,术后6个月至8年随访10例,MRI复查见空洞腔均明显缩小,其中3例空洞消失.按Tator标准,本组治疗效果优良率达90.0%.结论 MRI是Arnold-Chiari畸形合并脊髓空洞的最佳诊断手段;后颅窝减压及枕大池成形术治疗Amold-Chiari疗效满意.

  20. Surgical treatment of Arnold-Chiari malformation%Chiari畸形的手术治疗

    Institute of Scientific and Technical Information of China (English)

    王卿峰; 崔彦魁; 夏国庆; 宋伟; 韩东

    2012-01-01

    目的 探讨Chiari畸形的外科手术治疗.方法 回顾分析21例Chiari畸形(18例合并脊髓空洞)的外科治疗经验,全部病例均行后颅窝减压术,小脑扁桃体下疝切除术或电灼术.结果 全部病例随访6个月~2年,术后症状与体征均有不同程度好转.结论 后颅窝内减压术治疗Chiari畸形有一定疗效.

  1. A unifying hypothesis for hydrocephalus and the Chiari malformations part two: The hydrocephalus filling mechanism.

    Science.gov (United States)

    Williams, Helen

    2016-09-01

    It is proposed that negative central nervous system (CNS) pressure is one of the filling mechanisms of the fluid spaces of the CNS. Negative CNS pressure is caused by the combination of gravitational force and body movement. The venous system imposes pressure fluctuations on the CNS due to changes in posture and body cavity pressure. It is proposed here that filling of veins, arteries and cerebrospinal (CSF) spaces are all assisted by negative CNS pressure. Hyperemia in the CNS in response to pressure changes with movement was described in the first part of this hypothesis. By this means parenchyma water levels may increase (Williams, 2008). In the developmental forms of hydrocephalus expansion of CSF spaces is a more prominent feature than parenchyma water changes. This feature is explained by this second part of the hypothesis where the negative pulsatility of pressure that can accompany positive pressure pulsatility, which occurs with body movement, is described as the pathological force that leads to cavity filling. When CNS compliance is lost there is overrepresentation of low and well as high pressure pulsations in response to body movements. Pressure that leads to the development of hydrocephalus can be described as being abnormally labile. Negative CNS pressure causes cavity filling in an analogous way to pleural cavity filling, with water passing from parenchyma tissue. Positive pressure within the pressure profile may cause expansion of regions of the CNS skeletal system that are able to grow such as the cranial vault so that large head size is a frequent accompaniment to hydrocephalus that is caused by this mechanism. Hydrocephalic disorders that are characterised by negative pressure filling mechanism often have a skeletal anatomical abnormality that causes reduced CNS compliance and adversely affect neural development. This is often in the form of obstruction to CSF flow around the base of the brain that then leads to vault expansion by means of high pressure pulsation and ventricle enlargement by means of low pressure pulsation. In health pressure pulsatility does not lead to enduring changes in water distribution within the CNS compartments but it assists physiological water balance. Copyright © 2016 The Author. Published by Elsevier Ltd.. All rights reserved.

  2. Syringomyelia: Correlation between MRI and clinical findings before and after surgery

    Energy Technology Data Exchange (ETDEWEB)

    Schroth, G.; Palmbach, M.

    1988-12-01

    A comparison between clinical and MRI findings in 20 patients with syringomyelia produced the following results: 1. The position and extent of the syrinx do not correspond with the clinical findings. 2. Successfully operated syringomyelia cavities collapse in their sagittal diameters. The resulting oval shape of the syrinx can be used to evaluate shunt insufficiency. 3. In almost half of the patients the diameter of the spinal cord is within the 95% of normal controls and would therefore not have been diagnosed by myelography. 4. Anomalies of the cranio-cervical junction were present in only one-third of the patients. This finding does not support the hypothesis that syringomyelia is caused by hydrodynamic factors only.

  3. Shunting of recurrent post-traumatic syringomyelia into the fourth ventricle: a case report

    Directory of Open Access Journals (Sweden)

    Lin Chih-Lung

    2010-07-01

    Full Text Available Abstract Introduction Post-traumatic syringomyelia is a progressive degenerative disorder that is a well-recognized sequela of spinal cord injury. There is currently no optimal intervention capable of producing satisfactory long-term clinical results. Case presentation In this report, we present a 55-year-old Asian man with recurrent syringomyelia after shunt treatment. The syrinx extended from the thoracic cord into the medulla. We used a silicone tube to create a channel connecting the syrinx cavity directly to the fourth ventricle. The patient made a good recovery and follow-up magnetic resonance imaging revealed a considerable diminution in the size of the syrinx. Conclusions We present a new approach that has the potential to improve the outcome of patients with recurrent post-traumatic syringomyelia, who cannot be treated by conventional methods.

  4. Magnetic resonance imaging of the cervical spine: technical and clinical observations

    Energy Technology Data Exchange (ETDEWEB)

    Modic, M.T.; Weinstein, M.A.; Pavlicek, W.; Boumphrey, F.; Starnes, D.; Duchesneau, P.M.

    1983-12-01

    Seventy-two patients were examined to determine the clinical potential for magnetic resonance imaging (MRI) of the spine. MRI using different pulse sequences was compared with plain radiography, high-resolution computed tomography, and myelography. There were 35 normal patients; pathologic conditions studied included canal stenosis, herniated disk, metastatic tumor, neurofibroma, trauma, Chiari malformation, syringomyelia, arteriovenous malformation, and rheumatoid arthritis. MRI provided sharply defined anatomic delineation and tissue characterization. It was diagnostic in syringomyelia and Chiari malformation and was useful in the evaluation of trauma and spinal canal block from any cause. MRI was sensitive to degenerative disk disease and infection. The spin-echo technique, with three pulse sequence variations, seems very promising. A short echo time (TE) produces the best signal-to-noise ratio and spatial resolution. Lengthening the TE enhances differentiation of various tissues by their signal intensity, whil the combined increase of TE and recovery time (TR) produces selective enhancement of the cerebrospinal fluid signal intensity.

  5. An Unusual Presentation of Charcot Arthropathy Caused by Syringomyelia Mimicking a Soft Tissue Tumor

    Directory of Open Access Journals (Sweden)

    Cuneyd Gunay

    2014-01-01

    Full Text Available Charcot arthropathy is a chronic, degenerative condition and is associated with decreased sensorial innervation. Numerous causes of this arthropathy have been described. Here we report a case of neuropathic arthropathy secondary to syringomyelia which was misdiagnosed as a soft tissue tumor and treated surgically and additionally with radiotherapy at another institution. The patient had clinical and radiological signs of syringomyelia, associated with a limited range of motion, swelling, and pain in the affected joint. Neuropathic arthropathy, although less common, should be considered in cases of unexplained joint swelling, pain, and limited range of motion of the affected joint.

  6. Rare malformation of glans penis: arteriovenous malformation.

    Science.gov (United States)

    Akin, Y; Sarac, M; Yucel, S

    2013-01-01

    Pediatric glans penis malformations, especially arteriovenous malformations (AVM), are very rare. Herein, we report two rare cases. A 14-year-old boy attended our outpatient clinic with chief complaints of purple swelling and rapidly growing lesion on the glans penis. The lesion was excised surgically after physical and radiological evaluations. Pathology reported AVM and the patient is being followed up. The second case is a 2-year-old boy who was admitted with a big lesion involving glans penis and genital area that has been present since birth. In physical and radiological evaluations, lesion on the glans penis was pulsatile. Parents of the patient did not want any surgery and patient has been in follow-up. Diagnosis of the vascular lesions on glans penis is very easy by physical and radiological examinations today. Long-term follow-up is very important for AVM. Clinicians must make a careful effort to document new glans lesions in the pediatric population and decrease anxiety in the parents of affected children.

  7. Familial Brain Arteriovenous Malformations

    Directory of Open Access Journals (Sweden)

    J Gordon Millichap

    2007-11-01

    Full Text Available Researchers at University Medical Centre Utrecht, the Netherlands, reviewed the literature on patients with familial brain arteriovenous malformations (BAVMs, and their age, sex, and clinical presentation were compared with those in population-based patients with sporadic BAVMs.

  8. Increased detection rate of syringomyelia by whole spine sagittal magnetic resonance images: Based on the data from military conscription of Korean young males

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Myoung Seok; Oh, Chang Hyun [Seoul Regional Military Manpower Administration, Seoul (Korea, Republic of); Yoon, Seung Hwan; Park, Hyeong Chun; Park, Chong Oon; Kim, Yeo Ju [Inha Univ. Hospital/College of Medicine/Inha Univ., Incheon (Korea, Republic of)

    2012-09-15

    We compared the detection rate of syringomyelia according to the type of magnetic resonance (MR) images among the Korean military conscription. Among the total of 238910 examinees (males aged 18 to 32 years old) from January 2008 to December 2011, the examinees with conventional single lesion MR images (cervical, thoracic, and lumbar) with and without whole spine sagittal T2 weighted MR images (WSST2I) totaled 1206 cases, and syringomyelia was observed in 24 cases. The detection rate of syringomyelia according to the MR protocol (the presence of WSST2I or not) was done through analysis by annually and the clinical characters of syringomyelia was reviewed. The estimated prevalence of syringomyelia was approximately 10.0 cases per 100000 people. The detection rate was increased annually when the WSST2I proportion was increased (from 3.4 to 14.9 cases per 100000 persons, r = 0.939, p = 0.018). Clinical character of syringomyelia was ambiguous with other spinal diseases. The most affected spinal level was C5 to C7 (83%), and most cases were non communicating syringomyelia with benign central canal widening (79%). Whole spine sagittal MR image is useful to detect coexisting spinal diseases such as syringomyelia, and most syringomyelia in young males was benign hydromyelia. A whole spine sagittal MR image is recommended to increase the detection of syringomyelia.

  9. Proteus Syndrome with Arteriovenous Malformation

    Directory of Open Access Journals (Sweden)

    Ali Asilian

    2017-01-01

    Full Text Available Proteus syndrome is a rare sporadic disorder that appears with localized macrosomia, congenital lipomatosis, and slow flow vascular malformations, connective tissue nevus, and epidermal nevus. There are usually some manifestations at birth. The vascular abnormalities that have been reported in Proteus syndrome are capillary and slow flow venous malformation. We report a case of a 10-year-old boy with confirmed Proteus syndrome characterized by high flow vascular malformation (arteriovenous [AV] malformation unlike the usual vascular malformations seen in this syndrome. This case adds a new perspective to the established clinical findings of the Proteus syndrome.

  10. Syringomyelia following tuberculous meningitis. Report of three cases diagnosed by MR imaging

    Energy Technology Data Exchange (ETDEWEB)

    Tsuchiya, Kazuhiro; Takeshita, Koji; Makita, Kozo; Furui, Shigeru; Takenaka, Eiichi

    1988-11-01

    We present three cases with syringomyelia after tuberculous meningitis. The MR findings suggested the syrinx was formed by blockage of the CSF flow at the outlets of the fourth ventricle. We consider this complication is not a rare condition following tuberculous meningitis.

  11. Magnetic resonance imaging of post-traumatic syringomyelia and its surgical treatment

    Energy Technology Data Exchange (ETDEWEB)

    Isu, Toyohiko; Iwasaki, Yoshinobu; Nunomura, Mitsuru; Akino, Minoru; Koyanagi, Izumi; Abe, Hiroshi (Hokkaido Univ., Sapporo (Japan). School of Medicine); Saito, Hisatoshi

    1991-01-01

    The purpose of this study was to review magnetic resonance imaging (MRI) scans of post-traumatic syringomyelia and to assess the outcome of surgical treatment. The subjects were 16 patients (13 men and 3 women) whose ages ranged from 22 to 69 years, with a mean of 42 years. Nine patients had delayed neurologic symptoms 2 years and 2 months through 32 years after spinal injuries. The site of initial spinal cord injury was the lower cervical region in 4 patients, the thoracic region in 8, and the upper lumbar region in 4. In all patients, post-traumatic syringomyelia was easy to diagnose on MRI. MRI showed the syrinx extending superiorly and/or inferiorly from the area of old trauma, sometimes extending to the medulla oblongata. In the cervical cord and the upper thoracic cord, the syrinx was unilaterally or bilaterally situated in the postero-lateral portion. Below the middle thoracic cord, the syrinx was centrally located. Surgery was performed in 6 patients. At an average follow up of 2 years and 9 months, both pain and numbness were relieved in all patients (100%) and neurologic symptoms improved in 5 patients (83%). Post-traumatic syringomyelia should be considered in all patients having delayed onset or aggravation of neurologic symptoms after spinal injury. MRI appears promising for the early diagnosis of post-traumatic syringomyelia that can be treated favorably by surgical procedures. (N.K.).

  12. History of the Emergence and Recognition of Syringomyelia in the 19th Century.

    Science.gov (United States)

    Walusinski, Olivier

    2012-01-01

    Charles-Prosper Ollivier d'Angers coined the term "syringomyelia" in 1827 to describe the presence of a cavity in the spinal cord, which he considered pathological in all cases. In 1882 in Germany, Otto von Kahler and Friedrich Schultze defined the clinical syndrome, which associated Duchenne-Aran muscular dystrophy of a limb with sensory dissociation. They explained the syndrome by the presence of an abnormal cavity, distinct from that found in the spinal cord of healthy adults. Although Guillaume Duchenne de Boulogne and Jean-Martin Charcot had observed cases in France, both failed to identify syringomyelia, whereas a family physician in Brittany, Augustin Morvan, described the clinical symptomatology using the term "analgesic whitlow" in 1883. Based on several dozen observations that they collated in their remarkable theses,Anna Bumler in Zurich in 1887 and Isidore Bruhl in Paris in 1890 established the complete clinical picture of syringomyelia, covering anatomic functional and pathological aspects. Whereas Charcot isolated pathologies by correlating clinical signs with anatomo-pathological lesions, the isolation of syringomyelia initially involved an anatomo-pathological concept before the semiology was defined. In addition, this work would later enhance physiological understanding of sensory spinal pathways.

  13. Spinal cord stimulation combined with microsurgical DREZotomy for pain due to syringomyelia.

    Science.gov (United States)

    Shu, Wei; Li, Yongjie; Tao, Wei; Hu, Yongsheng

    2016-10-01

    A 49-year-old man with lower extremity neuropathic pain thought to be caused by spinal cord syringomyelia was treated with a combination of using spinal cord stimulation, decompression of the syrinx, relief of arachnoid adhesions and microsurgical DREZotomy. The patient had significant pain relief and has stable neurological status at follow-up.

  14. Posttraumatic syringomyelia: volumetric phantom and patient studies using MR imaging

    Energy Technology Data Exchange (ETDEWEB)

    Freund, Michael; Habicht, Dirk; Kalvine, Kira; Sartor, Klaus [Department of Neuroradiology, Medical School, University of Heidelberg (Germany); Aschoff, Alfred [Department of Neurosurgery, Medical School, University of Heidelberg (Germany)

    2002-12-01

    The purpose of this study was to determine the volume of posttraumatic syringomyelia (PTS) based on standard MRI data acquisitions, and to compare the volumes with the neurological deficits of the patients. Firstly, phantom studies were performed using routine T1- (T1W; TR 500 ms, TE 13 ms) spin-echo (SE) images, 3D gradient-echo (GE) images and T2-weighted (T2W) turbo spin-echo (TSE) images (TR 3000 ms, TE 130 ms), in the sagittal plane. The slices were interleaved so that there was no gap. Twelve phantoms simulating a PTS were constructed and filled with fluid. Each volume was exactly measured immediately prior to filling (volumes: 3600-74,000 mm{sup 3}, mean 27,500 mm{sup 3}). In the clinical study 32 patients with PTS were examined using the same protocol. Patients were supine and a phased-array coil was used. The phantom studies revealed measurement errors of within 35%. There were problems defining the boundaries in the small and irregular phantoms as well as in small and irregular PTS, and due to the partial-volume averaging effect. The two small irregular phantoms could only be measured on the axial images. The T2W images in the axial plane showed the best results: measurement accuracy 92%. In the clinical study all examinations were technically successful. The volumes of the PTS ranged between 200 and 19,800 mm{sup 3}; the mean volume was 4075 mm{sup 3}. Our initial results show that the volume measurement of a PTS using standard MRI sequences can help generate more objective and accurate measures of spinal cord lesions, and this may enhance the sensitivity of MRI in detecting disease progression or regression after treatment. (orig.)

  15. Longitudinal measurements of syrinx size in a rat model of posttraumatic syringomyelia.

    Science.gov (United States)

    Najafi, Elmira; Bilston, Lynne E; Song, Xin; Bongers, Andre; Stoodley, Marcus A; Cheng, Shaokoon; Hemley, Sarah J

    2016-06-01

    OBJECTIVE Syringomyelia pathophysiology is commonly studied using rodent models. However, in vivo studies of posttraumatic syringomyelia have been limited by the size of animals and lack of reliable noninvasive evaluation techniques. Imaging the rat spinal cord is particularly challenging because the spinal cord diameter is approximately 1-3 mm, and pathological lesions within the spinal cord parenchyma are even smaller. The standard technique has been histological evaluation, but this has its limitations. The aim of the present study was to determine whether syrinx size could be reliably measured using a preclinical high-field MRI animal system in a rat model of posttraumatic syringomyelia. METHODS The authors used an existing rat model of posttraumatic syringomyelia, which was created using a controlled pneumatic compression device to produce the initial spinal cord injury, followed by a subarachnoid injection of kaolin to produce arachnoiditis. T2-weighted MRI was performed on each animal using a 9.4-T scanner at 7, 10, and 13 weeks after injury. Animals were killed and syrinx sizes were calculated from in vivo MRI and histological studies. RESULTS MRI measurements of syrinx volume and length were closely correlated to histological measurements across all time points (Pearson product moment correlation coefficient r = ± 0.93 and 0.79, respectively). CONCLUSIONS This study demonstrates that high-field T2-weighted MRI can be used to measure syrinx size, and data correlate well with syrinx size measured using histological methods. Preclinical MRI may be a valuable noninvasive technique for tracking syrinx formation and enlargement in animal models of syringomyelia.

  16. Budd-Chiari like syndrome in decompensated alcoholic steatohepatitis and liver cirrhosis

    Institute of Scientific and Technical Information of China (English)

    Carlos Robles-Medranda; Hannah Lukashok; Beatriz Biccas; Vera L Pannain; Homero S Fogaca

    2006-01-01

    A rare case of pseudo-Budd-Chiari Syndrome in a patient with decompensated alcoholic liver disease is reported.Although clinical and radiological findings suggested Budd-Chiari Syndrome, the liver biopsy revealed micronodular cirrhosis and absence of histological signs of hepatic outflow obstruction.

  17. [Hepatic retransplant and acute Budd Chiari syndrome. Case report].

    Science.gov (United States)

    Savio-López, Andrés M; Lara-Molina, Evelin E; Soliva-Domínguez, Ramón; Capo-Jorge, José A; Gala-López, Boris; González-Castillo, Fernando

    2005-01-01

    Hepatic retransplant constitutes 10-20% of all orthotopic hepatic transplants. The piggy-back technique was used in hepatectomy with conservation of the retrohepatic vena cava. A side-to-side cavo-cavostomy technique is described in the case of hepatic congestion or acute Budd Chiari syndrome post-transplant. This is an extremely serious condition and can result in death. We present the first case of hepatic retransplant performed in Hospital Hermanos Ameijeiras in a patient who received who received his first transplant due to non-resectable hepatocarcinoma and who required retransplant due to acute rejection and graft dysfunction. During retransplant, the Belghiti side-to-side anastomosis technique was used to resolve the acute Budd Chiari syndrome that presented itself. Post-surgical evolution at 18 months was satisfactory without evidence of complications of the graft. Acute Budd Chiari syndrome post-transplant can satisfactorily be resolved with the Belghiti technique, although it is preferable to take prophylactic measures to avoid it.

  18. Syringomyelia and spinal arachnoiditis resulting from aneurysmal subarachnoid hemorrhage: Report of two cases and review of the literature

    Directory of Open Access Journals (Sweden)

    Taylor J Abel

    2014-01-01

    Full Text Available Syringomyelia resulting from arachnoiditis secondary to aneurysmal subarachnoid hemorrhage (SAH is an extremely rare clinical entity with few cases reported in the literature. The presentation, management, and pathogenesis of syringomyelia in this setting is poorly understood. We describe the presentation, radiology, management, and outcomes in two patients with syringomyelia resulting from arachnoiditis secondary to aneurysmal SAH and review the literature on this rare condition. Case number 1 was treated successfully with syrinx-subarachnoid shunt after extensive lysis of adhesions. Case number 2 was treated with syringoperitoneal shunt. Both patients had radiographic decreased syrinx size postoperatively. These patients add to the small literature on syringomyelia occurring secondary to SAH-associated arachnoiditis. The radiographic outcomes demonstrate that in the appropriately selected patient, syrinx-subarachnoid or syringoperitoneal shunting are viable options.

  19. The utility of superficial abdominal reflex in the initial diagnosis of scoliosis: a retrospective review of clinical characteristics of scoliosis with syringomyelia

    Directory of Open Access Journals (Sweden)

    Fujimori Takahito

    2010-08-01

    Full Text Available Abstract Background With increasing use of magnetic resonance imaging (MRI, underlying syringomyelia is increasingly found in patients with presumed idiopathic scoliosis. To determine the indications for MRI in the differential diagnosis of scoliosis, several clinical characteristics of syringomyelia have been reported. Neurological signs, particularly abnormal superficial abdominal reflex (SAR, are important in establishing the initial diagnosis of scoliosis. However, the prevalence of abnormal SAR in patients with scoliosis and the sensitivity of this sign in predicting syringomyelia are not well known. We aimed to determine the diagnostic utility of SAR and other characteristics of syringomyelia in patients with scoliosis. Methods We reviewed the medical records of 93 patients with scoliosis, 90 of whom underwent corrective surgery. All patients underwent MRI to determine the presence of syringomyelia. Mean age at surgery was 12.5 years. Abnormal SAR was defined as unilateral or bilateral absence or hyporeflexia of SAR. We calculated indices of diagnostic utility of abnormal SAR for non-idiopathic scoliosis and for syringomyelia. Abnormal SAR, left thoracic curve pattern, gender, and curve flexibility were compared between scoliosis with syringomyelia and idiopathic scoliosis. Logistic regression analysis was performed with the existence of syringomyelia as the dependent variable and curve flexibility as the independent variable. Results Abnormal SAR was observed in 20 patients (prevalence 22%. All 6 patients with myopathic scoliosis displayed bilateral absence of SAR. The sensitivity of abnormal SAR for non-idiopathic scoliosis was 38%, with 96% specificity, 90% PPV (positive predictive value, and 60% NPV (negative predictive value. Syringomyelia was identified in 9 of the 93 patients (9.7%; 8 of these had abnormal SAR. The sensitivity of abnormal SAR for syringomyelia in presumed idiopathic scoliosis was 89%, with 95% specificity, 80% PPV

  20. Longitudinal Study of the Relationship among Craniocervical Morphology, Clinical Progression, and Syringomyelia in a Cohort of Cavalier King Charles Spaniels

    OpenAIRE

    Cerda‐Gonzalez, S.; Olby, N.J.; Griffith, E.H.

    2016-01-01

    Background Craniocervical junction (CCJ) anomalies and secondary syringomyelia are commonly diagnosed in Cavalier King Charles spaniel (CKCS). Familiarity with the natural history of these abnormalities is vital to understanding the disease syndrome. Objective To evaluate magnetic resonance imaging (MRI) predictors of worsening clinical signs, syringomyelia, and morphology in CKCS longitudinally. Animals Fifty‐four client‐owned CKCS, 5–13 years old; 50% currently symptomatic. Methods Longitud...

  1. Cerebral malformations without antenatal diagnosis

    Energy Technology Data Exchange (ETDEWEB)

    Girard, Nadine J. [Diagnostic Neuroradiology, Hopital Timone, Marseille (France)

    2010-06-15

    Cerebral malformations are usually described following the different steps in development. Disorders of neurulation (dysraphisms), or diverticulation (holoprosencephalies and posterior fossa cysts), and total commissural agenesis are usually diagnosed in utero. In contrast, disorders of histogenesis (proliferation-differentiation, migration, organization) are usually discovered in infants and children. The principal clinical symptoms that may be a clue to cerebral malformation include congenital hemiparesis, epilepsy and mental or psychomotor retardation. MRI is the imaging method of choice to assess cerebral malformations. (orig.)

  2. Nuclear magnetic resonance imaging of the spine

    Energy Technology Data Exchange (ETDEWEB)

    Modic, M.T.; Weinstein, M.A.; Pavlicek, W.; Starnes, D.L.; Duchesneau, P.M.; Boumphrey, F.; Hardy, R.J. Jr.

    1984-01-01

    Forty subjects were examined to determine the accuracy and clinical usefulness of nuclear magnetic resonance (NMR) examination of the spine. The NMR images were compared with plain radiographs, high-resolution computed tomograms, and myelograms. The study included 15 patients with normal spinal cord anatomy and 25 patients whose pathological conditions included canal stenosis, herniated discs, metastatic tumors, primary cord tumor, trauma, Chiari malformations, syringomyelia, and developmental disorders. Saturation recovery images were best in differentiating between soft tissue and cerebrospinal fluid. NMR was excellent for the evaluation of the foramen magnum region and is presently the modality of choice for the diagnosis of syringomyelia and Chiari malformation. NMR was accurate in diagnosing spinal cord trauma and spinal canal block.

  3. Prevalence of Congenital Malformations

    Directory of Open Access Journals (Sweden)

    Akhavan Karbasi Sedighah

    2009-05-01

    Full Text Available Congenital malformation (CM will begin to emerge as one of the major childhood health problems .Treatment and rehabilitation of children with congenital malformations are costly and complete recovery is usually impossible. The aim of this study was to determine frequency of CM in Yazd central city of the Islamic Republic of Iran to find out if there has been any difference in the rate and types of CM in this area. This descriptive-observational study carried on 4800 births delivered at all maternity hospitals in Yazd from October 2003 to June 2004. Prevalence of CM was 2.83% (2.86 % in male and 2.68 % in female out of the 136 cases 69(51.88% were males and 64 (48.12% were females and 3 with ambiguous genitalia. Positive family history of CM in sibling was in only 6 cases (4.41%.Overall, musculoskeletal (0.83%, central nerv-ous system (0.47% and genital system (0.37% were accounted as the most common. Frequency of CM was more seen in still birth (12.5% as in comparison to live birth (2.71%. There was not statistical difference be-tween prevalence of CM and neonatal's gender, gestational age, birth order and mother's age, drug ingestion, illness and parental consanguinity. In this study the overall prevalence of congenital malformation among the newborn was higher than those previous reported in Iran and determining the causes of this difference needs more extensive studies.

  4. The utility of superficial abdominal reflex in the initial diagnosis of scoliosis: a retrospective review of clinical characteristics of scoliosis with syringomyelia

    Science.gov (United States)

    2010-01-01

    Background With increasing use of magnetic resonance imaging (MRI), underlying syringomyelia is increasingly found in patients with presumed idiopathic scoliosis. To determine the indications for MRI in the differential diagnosis of scoliosis, several clinical characteristics of syringomyelia have been reported. Neurological signs, particularly abnormal superficial abdominal reflex (SAR), are important in establishing the initial diagnosis of scoliosis. However, the prevalence of abnormal SAR in patients with scoliosis and the sensitivity of this sign in predicting syringomyelia are not well known. We aimed to determine the diagnostic utility of SAR and other characteristics of syringomyelia in patients with scoliosis. Methods We reviewed the medical records of 93 patients with scoliosis, 90 of whom underwent corrective surgery. All patients underwent MRI to determine the presence of syringomyelia. Mean age at surgery was 12.5 years. Abnormal SAR was defined as unilateral or bilateral absence or hyporeflexia of SAR. We calculated indices of diagnostic utility of abnormal SAR for non-idiopathic scoliosis and for syringomyelia. Abnormal SAR, left thoracic curve pattern, gender, and curve flexibility were compared between scoliosis with syringomyelia and idiopathic scoliosis. Logistic regression analysis was performed with the existence of syringomyelia as the dependent variable and curve flexibility as the independent variable. Results Abnormal SAR was observed in 20 patients (prevalence 22%). All 6 patients with myopathic scoliosis displayed bilateral absence of SAR. The sensitivity of abnormal SAR for non-idiopathic scoliosis was 38%, with 96% specificity, 90% PPV (positive predictive value), and 60% NPV (negative predictive value). Syringomyelia was identified in 9 of the 93 patients (9.7%); 8 of these had abnormal SAR. The sensitivity of abnormal SAR for syringomyelia in presumed idiopathic scoliosis was 89%, with 95% specificity, 80% PPV, and 98% NPV. Gender

  5. Classification and diagnosis of ear malformations

    OpenAIRE

    Bartel-Friedrich, Sylva; Wulke, Cornelia

    2008-01-01

    In the ENT region 50% of the malformations affect the ear. Malformations of the outer and middle ear are predominantly unilateral (ca. 70-90%) and mostly involve the right ear. Inner ear malformations can be unilateral or bilateral. The incidence of ear malformations is approximately 1 in 3800 newborns. Ear malformations may be genetic (associated with syndromes or not, with family history, spontaneous mutations) or acquired in nature. Malformations can affect the outer ear (pinna and externa...

  6. NFIA haploinsufficiency is associated with a CNS malformation syndrome and urinary tract defects.

    Directory of Open Access Journals (Sweden)

    Weining Lu

    2007-05-01

    Full Text Available Complex central nervous system (CNS malformations frequently coexist with other developmental abnormalities, but whether the associated defects share a common genetic basis is often unclear. We describe five individuals who share phenotypically related CNS malformations and in some cases urinary tract defects, and also haploinsufficiency for the NFIA transcription factor gene due to chromosomal translocation or deletion. Two individuals have balanced translocations that disrupt NFIA. A third individual and two half-siblings in an unrelated family have interstitial microdeletions that include NFIA. All five individuals exhibit similar CNS malformations consisting of a thin, hypoplastic, or absent corpus callosum, and hydrocephalus or ventriculomegaly. The majority of these individuals also exhibit Chiari type I malformation, tethered spinal cord, and urinary tract defects that include vesicoureteral reflux. Other genes are also broken or deleted in all five individuals, and may contribute to the phenotype. However, the only common genetic defect is NFIA haploinsufficiency. In addition, previous analyses of Nfia(-/- knockout mice indicate that Nfia deficiency also results in hydrocephalus and agenesis of the corpus callosum. Further investigation of the mouse Nfia(+/- and Nfia(-/- phenotypes now reveals that, at reduced penetrance, Nfia is also required in a dosage-sensitive manner for ureteral and renal development. Nfia is expressed in the developing ureter and metanephric mesenchyme, and Nfia(+/- and Nfia(-/- mice exhibit abnormalities of the ureteropelvic and ureterovesical junctions, as well as bifid and megaureter. Collectively, the mouse Nfia mutant phenotype and the common features among these five human cases indicate that NFIA haploinsufficiency contributes to a novel human CNS malformation syndrome that can also include ureteral and renal defects.

  7. Syringomyelia secondary to cervical spondylosis: Case report and review of literature

    Directory of Open Access Journals (Sweden)

    Savitr Sastri Bhagavathula Venkata

    2014-01-01

    Full Text Available Syringomyelia secondary to cervical spondylosis is a rare entity to encounter in clinical practice. We discuss the case of a 53-year-old lady who presented with a syringomyelic syndrome and was found to have cervical spondylosis on imaging. Cine-MRI revealed an obstruction of cerebrospinal fluid (CSF flow in the cervical spinal subarachnoid space. Decompression of the same led to clinical and radiological improvement. There is a potential causal association between cervical spondylosis and syringomyelia. MRI CSF flow studies may help in deciding the course of treatment in such cases. A subset of patients with cervical spondylosis and concurrent spinal cord signal intensity changes may show reversal of the same following intervention.

  8. Anorectal malformations : A multidisciplinary approach

    NARCIS (Netherlands)

    D. van den Hondel (Desiree)

    2015-01-01

    markdownabstractAbstract The research described in this thesis was performed with the aim to evaluate and improve multidisciplinary treatment of anorectal malformation patients. An overview of current literature on treatment of anorectal malformations is given in the Preface section, which also inc

  9. Percutaneous puncture of spinal cysts in the diagnosis and therapy of syringomyelia and cystic tumors

    Energy Technology Data Exchange (ETDEWEB)

    Dietemann, J.L.; Babin, E.; Wackenheim, A.; Maitrot, D.; Bonneville, J.F.

    1982-10-01

    The experience with percutaneous puncture of intraspinal cystic lesions is reported in cases: an extradural cyst demonstrated with air, a spinal cord tumor with lipiodol and two cases of syringomyelia with metrizamide. Percutaneous puncture of cystic lesions makes possible an accurate delineation of the lesion and a histological approach through biochemical and cytological analysis of the aspirated cystic fluid, while aspiration of the fluid of cystic tumors decompresses the spinal cord and thus plays a therapeutic role.

  10. Bilateral Charcot arthropathy of shoulder secondary to syringomyelia: An unusual case report

    Directory of Open Access Journals (Sweden)

    Ashok Panagariya

    2012-01-01

    Full Text Available Neuropathic arthropathy of the shoulder is a relatively rare disorder characterized by destruction of joint secondary to loss of sensory innervation. Bilateral Charcot arthropathy is an even rarer disorder, with very few cases reported in the English literature. We herein present a case of bilateral shoulder arthropathy secondary to syringomyelia with classical clinical and radiological findings. Radiological finding on one side was of resorptive type and resorptive mixed with productive on the other side.

  11. Syringomyelia in mucopolysaccharidosis type VI (Maroteaux-Lamy syndrome): imaging findings following bone marrow transplantation

    Energy Technology Data Exchange (ETDEWEB)

    Hite, S.H. [Department of Radiology, Box 292, University of Minnesota Hospital and Clinic, 420 Delaware Street SE, Minneapolis, MN 55455 (United States); Krivit, W. [Department of Pediatrics and Institute for Human Genetics, University of Minnesota Hospital and Clinic, Minneapolis, MN (United States); Haines, S.J. [Department of Neurological Surgery, University of Minnesota Hospital and Clinic, Minneapolis, MN (United States); Whitley, C.B. [Department of Pediatrics and Institute for Human Genetics, University of Minnesota Hospital and Clinic, Minneapolis, MN (United States)

    1997-09-01

    We present the imaging findings in a patient with mucopolysaccharidosis (MPS) type VI (Maroteaux-Lamy syndrome) who developed holocord syringomyelia. This represents the only reported case of syrinx formation in a child with MPS VI. Clinical, neurologic and spinal magnetic resonance imaging findings are presented. The patient has maintained a stable clinical and neurologic course over the period following allogeneic bone marrow transplant. (orig.). With 3 figs.

  12. Direct-trauma model of posttraumatic syringomyelia with a computer-controlled motorized spinal cord impactor.

    Science.gov (United States)

    Wong, Johnny H Y; Song, Xin; Hemley, Sarah J; Bilston, Lynne E; Cheng, Shaokoon; Stoodley, Marcus A

    2016-05-01

    OBJECTIVE The pathogenesis of posttraumatic syringomyelia remains enigmatic and is not adequately explained by current theories. Experimental investigations require a reproducible animal model that replicates the human condition. Current animal models are imperfect because of their low reliability, severe neurological deficits, or dissimilar mechanism of injury. The objective of this study was to develop a reproducible rodent model of posttraumatic syringomyelia using a spinal cord impactor that produces an injury that more closely mimics the human condition and does not produce severe neurological deficits. METHODS The study consisted of 2 parts. Seventy animals were studied overall: 20 in Experiment 1 and 48 in Experiment 2 after two rats with severe deficits were killed early. Experiment 1 aimed to determine the optimal force setting for inducing a cystic cavity without neurological deficits using a computer-controlled motorized spinal cord impactor. Twenty animals received an impact that ranged from 50 to 150 kDyn. Using the optimal force for producing an initial cyst determined from Experiment 1, Experiment 2 aimed to compare the progression of cavities in animals with and those without arachnoiditis induced by kaolin. Forty-eight animals were killed at 1, 3, 6, or 12 weeks after syrinx induction. Measurements of cavity size and maximum anteroposterior and lateral diameters were evaluated using light microscopy. RESULTS In Experiment 1, cavities were present in 95% of the animals. The duration of limb weakness and spinal cord cavity size correlated with the delivered force. The optimal force chosen for Experiment 2 was 75 kDyn. In Experiment 2, cavities occurred in 92% of the animals. Animals in the kaolin groups developed larger cavities and more vacuolations and enlarged perivascular spaces than those in the nonkaolin groups. CONCLUSIONS This impact model reliably produces cavities that resemble human posttraumatic syringomyelia and is suitable for further

  13. Diagnosis and Management of Budd Chiari Syndrome: An Update

    Energy Technology Data Exchange (ETDEWEB)

    Copelan, Alexander, E-mail: alexander.copelan@beaumont.edu [William Beaumont Hospital, Diagnostic Radiology Department (United States); Remer, Erick M., E-mail: remere1@ccf.org; Sands, Mark, E-mail: sandsm@ccf.org [Cleveland Clinic, Imaging Institute (United States); Nghiem, Hanh, E-mail: HNghiem@beaumont.edu [William Beaumont Hospital, Diagnostic Radiology Department (United States); Kapoor, Baljendra, E-mail: kapoorb@ccf.org [Cleveland Clinic, Imaging Institute (United States)

    2015-02-15

    Imaging plays a crucial role in the early detection and assessment of the extent of disease in Budd Chiari syndrome (BCS). Early diagnosis and intervention to mitigate hepatic congestion is vital to restoring hepatic function and alleviating portal hypertension. Interventional radiology serves a key role in the management of these patients. The interventionist should be knowledgeable of the clinical presentation as well as key imaging findings, which often dictate the approach to treatment. This article concisely reviews the etiology, pathophysiology, and clinical presentation of BCS and provides a detailed description of imaging and treatment options, particularly interventional management.

  14. The Roads to Mitochondrial Dysfunction in a Rat Model of Posttraumatic Syringomyelia

    Directory of Open Access Journals (Sweden)

    Zhiqiang Hu

    2015-01-01

    Full Text Available The pathophysiology of posttraumatic syringomyelia is incompletely understood. We examined whether local ischemia occurs after spinal cord injury. If so, whether it causes neuronal mitochondrial dysfunction and depletion, and subsequent energy metabolism impairment results in cell starvation of energy and even cell death, contributing to the enlargement of the cavity. Local blood flow was measured in a rat model of posttraumatic syringomyelia that had received injections of quisqualic acid and kaolin. We found an 86±11% reduction of local blood flow at C8 where a cyst formed at 6 weeks after syrinx induction procedure (P<0.05, and no difference in blood flow rate between the laminectomy and intact controls. Electron microscopy confirmed irreversible neuronal mitochondrion depletion surrounding the cyst, but recoverable mitochondrial loses in laminectomy rats. Profound energy loss quantified in the spinal cord of syrinx animals, and less ATP and ADP decline observed in laminectomy rats. Our findings demonstrate that an excitotoxic injury induces local ischemia in the spinal cord and results in neuronal mitochondrial depletion, and profound ATP loss, contributing to syrinx enlargement. Ischemia did not occur following laminectomy induced trauma in which mitochondrial loss and decline in ATP were reversible. This confirms excitotoxic injury contributing to the pathology of posttraumatic syringomyelia.

  15. Management of venous malformations.

    Science.gov (United States)

    Richter, Gresham T; Braswell, Leah

    2012-12-01

    Venous malformations (VMs) frequently occur in the head and neck with a predilection for the parotid gland, submandibular triangle, buccal space, muscles of mastication, lips, and upper aerodigestive tract. They are composed of congenitally disrupted ectatic veins with inappropriate connections and tubular channels. Because VMs have poorly defined boundaries and a tendency to infiltrate normal tissue, they require calculated treatment decisions in the effort to preserve surrounding architecture. Sclerotherapy, surgical excision, neodymium:yttrium aluminum garnet laser therapy, or a combination of these modalities is employed in the management of VMs. Although many small VMs can be cured, the objective is often to control the disease with periodic therapy. Location, size, and proximity to vital structures dictate the type of therapy chosen. Vigilance with long-term follow up is important. This review outlines current diagnostic and therapeutic approaches to simple and extensive cervicofacial VMs. Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

  16. Acute Liver Failure Due to Budd-Chiari Syndrome in the Setting of Cardiac Synovial Sarcoma

    OpenAIRE

    Stine, Jonathan G.; Newton, Kelly; Vinayak, Ajeet G

    2015-01-01

    Primary malignant tumors of the heart, specifically cardiac sarcomas, are rare and mainly diagnosed at autopsy. Acute Budd-Chiari syndrome is a recognized cause of acute liver failure and has been associated with several rare cardiac tumors: atrial myxoma, caval rhabdomyosarcoma, and primary cardiac adenocarcinoma. We present the first case of a fatal, highly differentiated cardiac synovial sarcoma that presented as acute liver failure from Budd-Chiari syndrome.

  17. IMAGING EXAMINATION OF CHIARI-Ⅰ DEFORMITY%Chiari-Ⅰ型畸型的影像学检查

    Institute of Scientific and Technical Information of China (English)

    王涛; 巩若箴

    2001-01-01

    Objective:To discuss the relationship between the Chiari- Ⅰ deformity and the perioccipital foramen magnum region defor mity in imaging, clinical and pathological manifestation. Methods: Forty one cases of Chiair- Ⅰ deformity in which 28 cases had undergone MRI, 29 cases for CT, and 9 cases for both these two examinations were studied. Results: There was the tonsilla cerebelli herniation in all CT cases which had extent downward from foramen magnum, 25 to C1 bottom level, 4 down to C2 level and below. The tonsilla cerebelli as a soft mass is posterior to cervical spinal cord in magnum foramen level in all axial imaging. In 8 of 9 cases of CTM show the cervical spinal cord deformation due to tonsilla cerebelli compression. Sagittal reconstruction images show that the tonsilla cerebelli extended downward to the spinal canal from the posterior fossa. All 28 cases of MRIgroup showed wedge-shaped tonsilla herniation extending 5 ~ 8 mm down ward magnum foremen; non indicate pathologic characteristics of Chian- Ⅰ deformity. Conclusion: The cervical and medullary curvature was due to the posterior fossa invagination but not to the Chiari- Ⅰ deformity itself.

  18. Magnetic resonance imaging of spinal dysraphism

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    Akino, Minoru; Isu, Toyohiko; Iwasaki, Yoshinobu; Abe, Hiroshi; Abe, Satoru; Miyasaka, Kazuo; Nomura, Mikio; Saito, Hisatoshi.

    1988-04-01

    Nineteen patients with lumbosacral spina bifida were studied by magnetic resonance imaging (MRI) and were divided into two groups: those with lumbosacral lipoma and those with meningomyelocele. All of the patients with meningomyelocele underwent surgery soon after birth for closure of the skin defect. Whenever possible, examination was not confined to the lumbosacral area but also included the brain and other portions of the spinal cord. Certain similarities and differences in pathology were ascertained in the two groups. The tethered cords were the same in both groups. However, Chiari malformations were observed only in patients with meningomyelocele, and hydrocephalus occurred only in patients with Chiari malformations. Syringomyelia and scoliosis were detected in both groups, but scoliosis was more prevalent in the meningomyelocele group. There appeared to be a correlation between scoliosis and syringomyelia; in five of the seven cases of syringomyelia, the locations of the scoliosis and syringomyelia were the same. With MRI, these complex pathologies, including tethered cord, syringomyelia, scoliosis, Chiari malformations, and hydrocephalus, were easily visualized. The superiority of MRI over conventional X-ray technology has been well established. First, a direct image of the spinal cord is obtained. Second, there is no necessity for injection of contrast material into the intrathecal space. Third, any scanning field is possible. There are also some disadvantages with MRI. First, the spatial resolution is inferior to that of high-resolution computed tomography. Second, MRI cannot provide information concerning bone cortex. Therefore, bone involvement cannot be accurately diagnosed. However, in the assessment of spinal dysraphism, MRI is an excellent diagnostic tool and should be the preferred method of diagnosing spinal dysraphism.

  19. Cerebellar Malformations and Cognitive Disdorders

    Directory of Open Access Journals (Sweden)

    J Gordon Millichap

    2007-10-01

    Full Text Available The behavioral developmental profile of 27 children and adults (17 males and 10 females with congenital cerebellar malformations was determined in a clinical, neuroradiological and neuropsychological study at the Scientific Institute 'E Medea', University of Milano, Italy.

  20. Are Established Methods of Physiotherapeutic Management for Long-term Neurological Conditions Applicable to 'Orphan' Conditions such as Syringomyelia?

    Science.gov (United States)

    Smith, Rebecca; Jones, Gareth; Curtis, Alexandra; Murphy, Hollie; Flint, Graham

    2016-03-01

    Syringomyelia is a rare or 'orphan' condition with the potential to cause significant disability and detrimental effects to quality of life. Syringomyelia shares similar symptoms to those common in other long-term neurological conditions, including spinal cord injury and multiple sclerosis. In these more prevalent conditions, physiotherapy is utilized widely and is effective in optimizing physical, psychological and social parameters. Therefore, we theorized that physiotherapy might be transferable to, and beneficial to syringomyelia patients. As a paucity of literature exists in this area, we aimed to evaluate the existing uptake and perceived efficacy of physiotherapy. An exploratory, mixed methodology was selected to derive sufficient qualitative data for analysis. Specifically designed questionnaires and semi-structured interviews yielded data on uptake and perceived physiotherapy efficacy. One hundred patients from a National Health Service tertiary syringomyelia service were invited to participate. The questionnaire and interviews were completed by 49 and 20 patients, respectively. Of the small number of patients receiving physiotherapy, the majority reported beneficial effects on pain modulation and quality of life. Stretching and hydrotherapy were deemed effective for relief of pain and stiffness. Additionally, physiotherapy was reported to provide similar benefits to surgical intervention. Syringomyelia patients report physiotherapy to provide benefits for symptom management and quality of life. Such findings suggest that established rehabilitation techniques in more common conditions may be transferable to those less prevalent. Uptake of physiotherapy was limited, seemingly because of inadequate information, knowledge and resources. To address these deficiencies, further studies should be planned investigating the effectiveness of physiotherapy modalities, such as hydrotherapy, in parallel or in conjunction with surgery and/or pharmacology. Additionally

  1. Anorectal malformations in neonates

    Directory of Open Access Journals (Sweden)

    Bilal Mirza

    2011-01-01

    Full Text Available Background : Anorectal malformations (ARM are associated with congenital anomalies and other risk factors, yielding a poor prognosis, especially in neonatal life. Objectives: This study was performed to identify the congenital anomalies as a factor of poor prognosis (mortality in such patients. Settings: Department of Pediatric surgery, The Children′s Hospital and The Institute of Child Health, Lahore. Design: Prospective observational study, with statistical support. Materials and Methods: The information on the demography, clinical features, investigations, management performed, and outcome was entered in the designed proforma and analysed with the help of statistical software EpiInfo version 3.5.1. Statistical test: Chi-square test was used to determine statistical significance of the results. Results : Of 100 neonates with ARM, 77 were male and 23, female (3.4:1. The mean age at presentation was 3.4 days (range, 12 hrs to 28 days. In 60 patients (60%, the presentation was imperforate anus without a clinically identified fistula. In 28 patients (28%, associated anomalies were present. The common associated anomalies were urogenital (10%, cardiovascular (8%, and gastrointestinal (6%. Down′s syndrome was present in 8 (8% patients. A total of 15 (15% deaths occurred in this study. In patients having associated congenital anomalies, 11 deaths occurred, whereas, 4 deaths were in patients without associated anomalies (P < 0.5. Conclusion : The mortality is higher in neonates with ARM having associated congenital anomalies.

  2. A feline case of isolated fourth ventricle with syringomyelia suspected to be related with feline infectious peritonitis.

    Science.gov (United States)

    Kitagawa, Masato; Okada, Midori; Sato, Tsuneo; Kanayama, Kiichi; Sakai, Takeo

    2007-07-01

    A one-year-old female cat was unable to stand. Magnetic resonance imaging was performed, and an enlargement of the lateral, third, and fourth ventricles and syringomyelia were detected. The cat was diagnosed with an isolated fourth ventricle (IFV) with syringomyelia. The serum isoantibody test for the feline infectious peritonitis (FIP) virus was 1:3,200. After the cat died, a pathological examination revealed nonsuppurative encephalomyelitis. We suspected that the IFV, detected in the cat, was associated with FIP encephalomyelitis. To our knowledge, there has been no report on IFV in veterinary medicine.

  3. A Case of Syringomyelia%脊髓空洞症1例

    Institute of Scientific and Technical Information of China (English)

    李灵匀; 胡雪艳

    2009-01-01

    患者女,50岁.左侧背部反复出现水疱,破溃、结痂8年.左侧C7-T6触觉迟钝,痛、温觉减退.MRI示:小脑扁桃体下端变尖,疝入椎管内,颈髓中央管扩张.诊断:Chiari I畸形,脊髓空洞症.

  4. Diagnosis and surgical treatment of terminal syringomyelia within spinal cord combined with tethered cord syndrome

    Directory of Open Access Journals (Sweden)

    Jing-cheng XIE

    2016-04-01

    Full Text Available Objective To summarize the clinical manifestations, imaging characteristics and experience of surgical treatment of spinal cord terminal syringomyelia with tethered cord syndrome (TCS.  Methods and Results Clinical data of 10 patients with spinal cord syringomyelia combined with TCS surgically treated under microscope from January 1999 to March 2014 in our hospital were retrospectively analyzed. There were 3 males and 7 females with average age of 15.06 years old (ranged from 2 to 35 years old. The course of disease ranged from 3 months to 20 years (average 42.17 months. Among those patients, one patient presented hydromyelia, 8 patients suffered from meningeal cyst within the sacral canal, and one patient were concurrent with sacral dermal sinus. The weakness of lower extremities, especially distal limbs, was the main clinical manifestation. Five patients were accompanied with bowel and bladder dysfunction and 5 patients with sensory disturbance below the level of syringomyelia, especially hypesthesia. Preoperative MRI showed conus medullaris disappeared at the end of spinal cord, and there was fluid signal in the lower spinal cord with hypo-intensity signal in T1WI and hyper-intensity signal in T2WI without enhancement. All patients underwent surgical procedures. Under microscope, filum terminale was cut off, drainage was performed, meningeal cyst within the sacral canal was removed, and tethered cord was released. The success rate of operations was 100%. The duration of surgery ranged from 1.52 to 3.07 h (average 2.15 h, with average intraoperative blood loss 220 ml (ranged from 100 to 410 ml. The tethering filum had been totally resected and histological examination showed typical filum tissue in all cases. No operative complication was found. Visual Analogue Scale (VAS score was decreased, and the lower limbs weakness as well as bowel and bladder dysfunction was gradually relieved after operation. The period of follow-up was ranged from 6

  5. Budd-Chiari syndrome: Etiology, pathogenesis and diagnosis

    Science.gov (United States)

    Aydinli, Musa; Bayraktar, Yusuf

    2007-01-01

    Budd-Chiari syndrome is a congestive hepatopathy caused by blockage of hepatic veins. This syndrome occurs in 1/100 000 in the general population. Hypercoagulable state could be identified in 75% of the patients; more than one etiologic factor may play a role in 25% of the patients. Primary myeloproliferative diseases are the leading cause of the disease. Two of the hepatic veins must be blocked for clinically evident disease. Liver congestion and hypoxic damage of hepatocytes eventually result in predominantly centrilobular fibrosis. Doppler ultrasonography of the liver should be the initial diagnostic procedure. Hepatic venography is the reference procedure if required. Additionally liver biopsy may be helpful for differential diagnosis. The prognosis of the chronic form is acceptable compared to other chronic liver diseases. PMID:17569137

  6. Budd-Chiari syndrome: Etiology, pathogenesis and diagnosis

    Institute of Scientific and Technical Information of China (English)

    Musa Aydinli; Yusuf Bayraktar

    2007-01-01

    Budd-Chiari syndrome is a congestive hepatopathy caused by blockage of hepatic veins. This syndrome occurs in 1/100000 in the general population. Hypercoagulable state could be identified in 75% of the patients; more than one etiologic factor may play a role in 25% of the patients. Primary myeloproliferative diseases are the leading cause of the disease. Two of the hepatic veins must be blocked for clinically evident disease. Liver congestion and hypoxic damage of hepatocytes eventually result in predominantly centrilobular fibrosis. Doppler ultrasonography of the liver should be the initial diagnostic procedure. Hepatic venography is the reference procedure if required. Additionally liver biopsy may be helpful for differential diagnosis. The prognosis of the chronic form is acceptable compared to other chronic liver diseases.

  7. Budd-Chiari Syndrome: Two Cases with Different Courses

    Directory of Open Access Journals (Sweden)

    Shinjiro Inomata

    2008-08-01

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