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| arnold chiari malformationSummarySummary: A group of congenital malformations involving the brainstem, cerebellum, upper spinal cord, and surrounding bony structures. Type II is the most common, and features compression of the medulla and cerebellar tonsils into the upper cervical spinal canal and an associated MENINGOMYELOCELE. Type I features similar, but less severe malformations and is without an associated meningomyelocele. Type III has the features of type II with an additional herniation of the entire cerebellum through the bony defect involving the foramen magnum, forming an ENCEPHALOCELE. Type IV is a form a cerebellar hypoplasia. Clinical manifestations of types I-III include TORTICOLLIS; opisthotonus; HEADACHE; VERTIGO; VOCAL CORD PARALYSIS; APNEA; NYSTAGMUS, CONGENITAL; swallowing difficulties; and ATAXIA. (From Menkes, Textbook of Child Neurology, 5th ed, p261; Davis, Textbook of Neuropathology, 2nd ed, pp236-46) Top Publications
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Publications
Chiari I malformation associated with syringomyelia: a retrospective study of 316 surgically treated patientsZ Q Zhang
Department of Neurosurgery, Qilu Hospital of Shandong University, Jinan, PR China
Spinal Cord 46:358-63. 2008..The objective of this article is to probe the surgical procedures of treatment for Chiari I malformation (CMI) associated with syringomyelia and evaluate their efficacy based on clinical and neuroradiological results...
Treatment of the Chiari malformation with bone decompression without durotomy in children and young adultsHector E James
Department of Pediatrics, Children s Hospital and University of California, San Diego, USA
Childs Nerv Syst 18:202-6. 2002..The purpose of this study was to provide more information on alternative operative approaches to the treatment of symptomatic Chiari type I and/or type II malformations in children and young adults...
Scoliosis associated with Chiari 1 malformations: the effect of suboccipital decompression on scoliosis curve progression: a preliminary studyDouglas Brockmeyer
Department of Neurosurgery, University of Utah and Primary Children s Medical Center, Salt Lake City, Utah 84113 1103, USA
Spine (Phila Pa 1976) 28:2505-9. 2003..A retrospective review of the effect of suboccipital decompression and duraplasty on curve progression in 22 patients who presented with scoliosis, syringomyelia, and a Chiari 1 malformation...
Chiari malformation: CSF flow dynamics in the craniocervical junction and syrinxD E Sakas
Department of Neurosurgery, University of Athens, Evangelismos General Hospital, Athens, Greece
Acta Neurochir (Wien) 147:1223-33. 2005..SPAMM can be used to assess whether CCJD has restored CSF flow, predict outcome and provide pathophysiological insights in ChM and syringomyelia...
Hindbrain decompression for Chiari-syringomyelia complex: an outcome analysis comparing surgical techniquesC Hayhurst
Department of Neurosurgery, Walton Centre for Neurology and Neurosurgery, Liverpool, UK
Br J Neurosurg 22:86-91. 2008..Although this is a retrospective study it suggests that bone only decompression should be reserved for patients with isolated headache...
Sleep disorder: a possible cause of attention deficit in children and adolescents with Chiari malformation type IIPaulo Sérgio Azeredo Henriques Filho
School of Medicine, Brasilia University, Brasilia, DF, Brazil
Arq Neuropsiquiatr 67:29-34. 2009..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...
The natural history of the Chiari Type I anomalyFederica Novegno
Department of Pediatric Neurosurgery, Catholic University Medical School, Largo A Gemelli 1, Rome, Italy
J Neurosurg Pediatr 2:179-87. 2008..The decision of whether or not to operate is more difficult in these patients than in those with clear symptoms because of the lack of information about the natural course of this disease...
Chiari I anatomy after ventriculoperitoneal shunting: posterior fossa volumetric evaluation with MRIFerdnand C Osuagwu
Department of Anatomy, University of Ibadan, Ibadan, Nigeria
Childs Nerv Syst 22:1451-6. 2006..DISCUSSION: Chiari I anatomy after a ventriculoperitoneal shunt could develop in children and we propose a "posterior cranial fossa disproportion" rather than a "cephalocranial disproportion."..
Chiari type I malformation in a pediatric populationLeslie A Aitken
Department of Pediatrics, University of California San Francisco, San Francisco, CA 94117 0137, USA
Pediatr Neurol 40:449-54. 2009..Larger and longer-term studies are needed to determine the prognosis and optimal treatment of pediatric Chiari I...
Chiari I malformation in the very young child: the spectrum of presentations and experience in 31 children under age 6 yearsJeremy D W Greenlee
Department of Neurosurgery, University of Iowa Hospitals and Clinics, Iowa City, Iowa 52242, USA
Pediatrics 110:1212-9. 2002..CONCLUSIONS: Very young children presenting with oropharyngeal dysfunction, pain worsened by valsalva, or scoliosis should prompt the clinician to consider CM I as a possible cause...
Association of Chiari malformation type I and tethered cord syndrome: preliminary results of sectioning filum terminaleThomas H Milhorat
Department of Neurosurgery, The Chiari Institute, Harvey Cushing Institutes of Neuroscience, North Shore Long Island Jewish Health System, Manhasset, NY 11030, USA
Surg Neurol 72:20-35. 2009..The pathogenesis of CM-I is incompletely understood. We describe an association of CM-I and TCS that occurs in a subset of patients with normal size of the PCF...
The effects of posterior fossa decompressive surgery in adult patients with Chiari malformation and sleep apneaRicardo Vieira Botelho
Neurosurgical Service, Hospital do Servidor Público do Estado de São Paulo, Sao Paulo, Brazil
J Neurosurg 112:800-7. 2010..The objective of this work was to study the effect of posterior fossa decompressive surgery on respiratory parameters during sleep in patients with craniovertebral junction malformations and breathing-related sleep disorders...
The role of cine flow MRI in children with Chiari I malformationEnrique C G Ventureyra
Division of Neurosurgery, Children s Hospital of Eastern Ontario, University of Ottawa, 401 Smyth Road, Ottawa, Ontario, KIH 8LI Canada
Childs Nerv Syst 19:109-13. 2003..The present study attempts to analyze the role of cine flow MRI in Chiari I malformations...
Posterior fossa volume in children with Chiari malformation Type ISpyros Sgouros
Department of Neurosurgery and Neuroscience Informatics Laboratory of the Institute of Child Health, Birmingham Children s Hospital, Birmingham, England
J Neurosurg 105:101-6. 2006..They also investigated the role of syringomyelia in posterior fossa development...
Posterior fossa dimension and volume estimates in pediatric patients with Chiari I malformationsT Trigylidas
Division of Neurosurgery, Children s Hospital of Eastern Ontario, University of Ottawa, 401 Smyth Road, Ottawa, ON K1H 8L1, Canada
Childs Nerv Syst 24:329-36. 2008..The combination of neuroradiological and clinical findings is used to re-examine the criteria used for diagnosis and treatment of pediatric CMI patients...
Arnold Chiari type 1 malformation presenting with sleep disordered breathing in well childrenC Murray
Children's Hospital at Westmead, Sydney, Australia
Arch Dis Child 91:342-3. 2006..Each had normal neurological examinations and an Arnold Chiari type 1 malformation was confirmed on magnetic resonance imaging. All underwent urgent posterior fossa decompression which normalised their sleep breathing...
Surgical experience in 130 pediatric patients with Chiari I malformationsR Shane Tubbs
Department of Cell Biology, University of Alabama at Birmingham, Birmingham, Alabama, USA
J Neurosurg 99:291-6. 2003..The aim of this study was to present the long-term findings of a surgical series of pediatric patients with Chiari I malformations...
Osteopetrosis with Arnold Chiari malformation type I and brain stem compressionM L Kulkarni
Department of Pediatrics J J M Medical College, Davangere, Karnataka, India
Indian J Pediatr 74:412-5. 2007..The association of Type 1 Arnold Chiari malformation with osteopetrosis resulting in brain stem compression syndrome is an extremely rare entity.
Thoracic meningocele, meningomyelocele or myelocystocele? Diagnostic difficulties, consequent implications and treatmentM P Arts
Department of Pediatric Neurosurgery, Erasmus Medical Center, Sophia Children Hospital, Rotterdam, The Netherlands
Pediatr Neurosurg 40:75-9. 2004..In this case report, we present a child born with a thoracic myelocystocele, the diagnostic difficulties, consequent implications and surgical treatment...
Syringomyelia in twin brothers discordant for Chiari I malformation: case reportR Shane Tubbs
Department of Cell Biology, University of Alabama at Birmingham, USA
J Child Neurol 19:459-62. 2004....
Urgent surgical intervention in pediatric patients with Chiari malformation type I. Report of two casesJohn C Wellons
Division of Neurosurgery, Section of Pediatric Neurosurgery, Children s Hospital, Birmingham, Alabama 35233, USA
J Neurosurg 107:49-52. 2007..Although seemingly rare, the clinician should consider CM-I in the differential diagnosis in pediatric patients presenting with acute brainstem or long tract signs...
Foramen magnum decompression for the treatment of Arnold Chiari malformation type I with associated syringomyelia in an elderly patientI Takigami
Department of Orthopaedic Surgery, Gifu University School of Medicine, Gifu City, 501-1194, Gifu, Japan
Spinal Cord 43:249-51. 2005STUDY DESIGN: Report of a rare case of an elderly patient with late onset of Arnold Chiari malformation type I with associated syringomyelia that was successfully treated with foramen magnum decompression...
Chiari Type II malformation: past, present, and futureKevin L Stevenson
Children s Healthcare of Atlanta, Atlanta, Georgia, USA
Neurosurg Focus 16:E5. 2004..A thorough understanding of the embryology, anatomy, symptomatology, and surgical treatment is necessary to care optimally for children with myelomeningocele and prevent significant morbidity and mortality...
Factors influencing the outcome in symptomatic Chiari I malformationPankaj Arora
Department of Neurosurgery, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Rae Bareli Road, Lucknow - 226 014, India
Neurol India 52:470-4. 2004..001), and basilar invagination (P value=0.048). The effect of syringomyelia in predicting the clinical outcome could not be determined due to the differences in the number of patients in the groups with or without syringomyelia...
Arachnoid veils and the Chiari I malformationR Shane Tubbs
Department of Cell Biology, University of Alabama at Birmingham, USA
J Neurosurg 100:465-7. 2004..The literature contains scant data regarding variations in anatomy at the level of the foramen of Magendie in patients with Chiari I malformation and syringomyelia...
Cluster-like headache: association with cervical syringomyelia and Arnold-Chiari malformationM Seijo-Martinez
Neurology Service, Complexo Hospitalario de Pontevedra, Pontevedra, Spain
Cephalalgia 24:140-2. 2004
Persistent syringomyelia following pediatric Chiari I decompression: radiological and surgical findingsR Shane Tubbs
Department of Cell Biology, University of Alabama at Birmingham, USA
J Neurosurg 100:460-4. 2004..The authors have analyzed this smaller group of patients to look for possible radiological or surgical findings that may aid in predicting which patients are less likely to respond to the initial first decompressive procedure...
Noncommunicating cysts and cerebrospinal fluid flow dynamics in a patient with a Chiari I malformation and syringomyelia--part IITerry Lichtor
Department of Neurological Surgery, Rush University Medical Center, Chicago, IL 60612, USA
Spine 30:1466-72. 2005
Surgical results of posterior fossa decompression for patients with Chiari I malformationRamon Navarro
Division of Pediatric Neurosurgery, Children's Memorial Hospital, Northwestern University Medical School, 2300 Children's Plaza, Box #28, Chicago, IL 60614, USA
Childs Nerv Syst 20:349-56. 2004..This less invasive approach minimizes complications associated with dural opening and offers comparable success rates...
Arnold-Chiari malformation with syrinx presenting as carpal tunnel syndrome: a case reportMark J Ziadeh
Department of Physical Medicine and Rehabilitation, University of Michigan, Ann Arbor 48109-0718, USA
Arch Phys Med Rehabil 85:158-61. 2004..Neurosurgical decompression led to a decrease in symptoms. A meticulous history and physical examination should be performed on patients with presumed carpal tunnel syndrome with an atypical onset of symptoms or response to therapy...
Chiari I malformation related syringomyelia: radionuclide cisternography as a predictor of outcomeP Arora
Department of Neurosurgery, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India
Acta Neurochir (Wien) 146:119-30. 2004..The symptomatology related to brain-stem compression immediately responds to the surgical procedure but the syrinx-induced signs and symptoms of spinal cord dysfunction persist...
Effect of craniocervical decompression on peak CSF velocities in symptomatic patients with Chiari I malformationMaria T Dolar
Department of Radiology, University of Wisconsin, Madison, WI, USA
AJNR Am J Neuroradiol 25:142-5. 2004..The study supports the hypothesis that successful treatment of the Chiari I malformation is associated with improvement in CSF flow patterns...
Preoperative MRI analysis of patients with idiopathic scoliosis: a prospective studyMasatoshi Inoue
Department of Orthopedic Surgery, Graduate School of Medicine, Chiba University, Chiba, Japan
Spine (Phila Pa 1976) 30:108-14. 2005..A prospective trial of preoperative MRI study in patients with "idiopathic" scoliosis...
"Syringomyelia with Chiari malformation; 3 unusual cases with implication of pathogenesis"C Di Rocco
Acta Neurochir (Wien) 147:221. 2005
Relevance of surgical strategies for the management of pediatric Chiari type I malformationMarcelo Galarza
Department of Neurosurgery, Children s Hospital of Turin, Turin, Italy
Childs Nerv Syst 23:691-6. 2007..In face of continuing controversy to the optimal treatment of Chiari type I malformation, the authors analyzed three different surgical strategies...
Sleep-disordered breathing in patients with Chiari malformation: improvement after surgeryF Gagnadoux
Department de Pneumology, Centre Hospitalier Universitaire, Angers, France
Neurology 66:136-8. 2006..In six patients with sleep apnea syndrome, PSG was repeated an average of 203 days after decompression surgery. Postoperative PSG showed a decrease in the central apnea index from 14.9 +/- 5.5 to 1.3 +/- 0.6 (p = 0.03)...
Relationship of cine phase-contrast magnetic resonance imaging with outcome after decompression for Chiari I malformationsMatthew J McGirt
Department of Neurosurgery, The Johns Hopkins Hospital, Baltimore, Maryland, USA
Neurosurgery 59:140-6; discussion 140-6. 2006..Cine phase-contrast magnetic resonance imaging may be a valuable tool in identifying patients who are less likely to respond to surgical decompression for Chiari I malformation...
Scoliosis in children with Chiari I-related syringomyeliaRanjeev Bhangoo
Department of Paediatric Neurosurgery, Birmingham Children's Hospital, Steelhouse Lane, Birmingham, B4 6NH, UK
Childs Nerv Syst 22:1154-7. 2006..CONCLUSIONS: Cranio-vertebral decompression for Chiari I may prevent the need for corrective scoliosis surgery when performed before the age of ten and below a Cobb angle of 30 degrees...
Honored guest presentation: lifetime experiences and where we are going: Chiari I with syringohydromyelia--controversies and development of decision treesArnold H Menezes
Department of Neurosurgery, University of Iowa Hospitals and Clinics, University of Iowa Carver College of Medicine, Iowa City, USA
Clin Neurosurg 52:297-305. 2005
Chiari I malformation accompanied by assimilation of the atlas, Klippel-Feil syndrome, and syringomyelia: case reportMasahiro Kagawa
Department of Neurological Surgery, Kagawa University, Miki cho, Kagawa, 761 0793, Japan
Surg Neurol 65:497-502; discussion 502. 2006..We report a case of Chiari I malformation accompanied by assimilation of the atlas, Klippel-Feil syndrome, and syringomyelia...
Infratentorial hygromaIgnacio Arrese
J Neurosurg 104:856-7; author reply 857. 2006
Cervical myelomeningoceleZohreh Habibi
Department of Neurosurgery, Children's Hospital Medical Center, Tehran University of Medical Science, Tehran, Iran
Neurosurgery 58:1168-75; discussion 1168-75. 2006..We advise thorough preoperative evaluation of the brain, spinal column, and urinary system. Intradural exploration to release any potential adhesion bands as well as correcting associated anomalies is recommended in all cMMC operations...
Surgical outcomes in adult patients with syringomyelia associated with Chiari malformation type I: the relationship between scoliosis and neurological findingsAtsushi Ono
Department of Orthopaedic Surgery, Hirosaki University School of Medicine, Japan
J Neurosurg Spine 6:216-21. 2007..In the present study of adult syringomyelia associated with Chiari malformation Type I, the authors investigated the relationships among the syrinx, scoliosis, and neurological data...
Posterior cranial fossa box expansion leads to resolution of symptomatic cerebellar ptosis following Chiari I malformation repairJustin B Heller
Division of Plastic and Reconstructive Surgery, Department of Neurosurgery, University of California, Los Angeles, California 90095, USA
J Craniofac Surg 18:274-80. 2007..One out of seven patients had return of headache in a more mild form. Thus, in our patient group the posterior fossa box reconstruction led to resolution of symptomatic cerebellar ptosis following Chiari I malformation repair...
Atypical syringomyelia without cavity in a patient with Chiari malformation and hydrocephalusRong Wang
Department of Spine Surgery, Drum Tower Hospital, College of Medicine, Nanjing University, Nanjing, China
Spine (Phila Pa 1976) 32:E467-70. 2007..A case report is presented...
A detailed morphologic and functional magnetic resonance imaging study of the craniocervical junction in adolescent idiopathic scoliosisWinnie C W Chu
Department of Diagnostic Radiology and Organ Imaging, Chinese University of Hong Kong, Prince of Wales Hospital, Shatin, Hong Kong
Spine (Phila Pa 1976) 32:1667-74. 2007..Conventional and phase-contrast magnetic resonance (MR) imaging were used to evaluate the morphology and cerebrospinal fluid (CSF) flow dynamics at craniocervical junction in adolescent idiopathic scoliosis (AIS)...
Syringomyelia with Chiari malformation; 3 unusual cases with implications for pathogenesisB K Owler
Departments of Neurosurgery and Neurology, Institute of Clinical Neurosciences, Royal Prince Alfred Hospital, Sydney, Australia
Acta Neurochir (Wien) 146:1137-43; discussion 1143. 2004..These cases provide a basis for discussion of the pathogenesis and management of syringomyelia and the Chiari malformation in such cases...
Foramen magnum cerebrospinal fluid flow characteristics in children with Chiari I malformation before and after craniocervical decompressionBermans J Iskandar
Department of Neurosurgery, University of Wisconsin, Madison, Wisconsin 53792, USA
J Neurosurg 101:169-78. 2004..The authors used temporally and spatially resolved flow analyses to assess the characteristics of CSF flow in children with Chiari I malformation and the changes in these flow characteristics that occur after suboccipital decompression...
A critical analysis of the Chiari 1.5 malformationR Shane Tubbs
Department of Cell Biology, University of Alabama at Birmingham, Alabama, USA
J Neurosurg 101:179-83. 2004..In an attempt to elucidate further the best surgical strategy and to refine the descriptive terminology, the authors retrospectively analyzed all cases at their institutions in which this form of hindbrain herniation was diagnosed...
Dura-splitting decompression of the craniocervical junction: reduced operative time, hospital stay, and cost with equivalent early outcomeFarhad M Limonadi
Division of Pediatric Neurosurgery, Doernbecher Children's Hospital and Oregon Health and Science University, Portland, Oregon 97239, USA
J Neurosurg 101:184-8. 2004..A randomized controlled trial of dura-splitting decompression in a uniform population of patients with Chiari I malformation is indicated...
Noncommunicating cysts and cerebrospinal fluid flow dynamics in am patient with a Chiari I malformation and syringomyelia--part ITerry Lichtor
Department of Neurological Surgery, Rush University Medical Center, John H. Stroger Hospital of Cook County and Hektoen Institute for Medical Research, Chicago, IL 60612, USA
Spine 30:1335-40. 2005
Relevance of surgical strategies for the management of paediatric Chiari type I malformationRamon Navarro
Childs Nerv Syst 23:725-6; author reply 727. 2007
Trigeminal neurinoma associated with Chiari malformation and syringomyeliaA Goel
Department of Neurosurgery, Seth G S Medical College and King Edward VII Memorial Hospital, Parel, Mumbai, India
J Clin Neurosci 12:608-10. 2005..Within two months of tumor resection, both the Chiari malformation and the syringomyelia resolved. The association of an intracranial space-occupying lesion with Chiari malformation and syringomyelia is reviewed...
Treatment of Chiari I malformation in patients with and without syringomyelia: a consecutive series of 66 casesJ C Alzate
Institute for Neurology and Neurosurgery, Singer Division, Department of Pediatric Neurosurgery, Beth Israel Medical Center, New York, New York 10128, USA
Neurosurg Focus 11:E3. 2001..Primary placement of a shunt in the presence of a sufficiently large syrinx appears to be beneficial. The question of if and when to place a shunt, however, requires further, preferably prospective, investigation...
Chiari malformation, cervical disc prolapse and syringomyelia--always think twiceJonathon R Ball
Department of Neurosurgery, Royal North Shore Hospital, Pacific Highway, St Leonards, 2065 New South Wales, Australia
J Clin Neurosci 15:474-6. 2008..Chiari I malformations are increasingly incidentally detected and asymptomatic. This first report of arthroplasty for cervical spondylosis associated with syringomyelia adds to the growing body of experience with this new technology...
Initial management of hydrocephalus associated with Chiari malformation Type I-syringomyelia complex via endoscopic third ventriculostomy: an outcome analysisCaroline Hayhurst
Department of Neurosurgery, The Walton Centre for Neurology and Neurosurgery, Liverpool, UK
J Neurosurg 108:1211-4. 2008..The aim of this study was to evaluate the efficacy of endoscopic third ventriculostomy (ETV) in patients with Chiari malformation Type I (CM-I) and hydrocephalus with or without syringomyelia...
Syringobulbia in a pediatric populationJeremy D W Greenlee
Department of Neurosurgery, University of Iowa Hospitals and Clinics, Iowa City, Iowa 52242, USA
Neurosurgery 57:1147-53; discussion 1147-53. 2005..CONCLUSION: Syringobulbia is strongly associated with Chiari malformation and syringomyelia, and patients often present because of cranial nerve palsies. Posterior fossa decompression is a safe and effective treatment...
Curve progression in scoliosis associated with Chiari I malformation following suboccipital decompressionFrances A Farley
Department of Orthopaedic Surgery, University of Michigan, Ann Arbor, USA
J Spinal Disord Tech 15:410-4. 2002..Eight also had syringomyelia. Despite initial curve stabilization, at final follow-up eight curves were of the magnitude to require spinal fusion. Neither bracing nor secondary neurosurgical procedures arrested progressive curves...
Cranio-cervical decompression for Chiari type I-malformation, adding extreme lateral foramen magnum opening and expansile duroplasty with arachnoid preservation. Technique and long-term functional results in 44 consecutive adult cases -- comparison with lM Sindou
Department of Neurosurgery, , University of Lyon, Lyon, France
Acta Neurochir (Wien) 144:1005-19. 2002....
The Chiari I malformation and the neurotologistArvind Kumar
Department of Otolaryngology, Head and Neck Surgery, University of Illinois at Chicago, 60612, USA
Otol Neurotol 23:727-35. 2002..The second objective is to report how the results of a vestibular evaluation can help neurosurgeons decide on the need for surgical treatment...
Endoscopic third ventriculostomy for treatment of noncommunicating syringomyelia associated with a Chiari I malformation and hydrocephalus: case report and pathophysiological considerationsPhilippe Metellus
Department of Neurosurgery, Timone Hospital, Marseille, France
Neurosurgery 51:500-3; discussion 503-4. 2002..The successful use of this technique in such a case has not been previously described, and the results allow us to speculate on the pathophysiological mechanism involved...
Craniocervical junction fusions in patients with hindbrain herniation and syringohydromyeliaAlbert J Fenoy
Department of Neurosurgery, University of Iowa Hospitals and Clinics, Iowa City, Iowa, USA
J Neurosurg Spine 9:1-9. 2008..The authors surveyed the indications for CCJ fusions in this population...
Chiari I malformation associated with syringomyelia and scoliosis: a twenty-year review of surgical and nonsurgical treatment in a pediatric populationJames M Eule
Department of Orthopedic Surgery, University of Colorado, Denver, Colorado, USA
Spine 27:1451-5. 2002..Each of these patients underwent decompression before 8 years of age and before the curve was severe. However, this series represents a few patients demonstrating this trend, and further follow-up and investigation are warranted...
[Syringomyelia and Chiari's malformation in a child with scoliosis]A Weijenberg
Universitair Medisch Centrum Groningen, Groningen
Ned Tijdschr Geneeskd 152:1339-43. 2008..In children with structural scoliosis, ancillary investigation in the way of neuroimaging of the spinal cord should be considered to exclude underlying spinal cord pathology, even in the absence of neurological abnormalities...
Treatment of Chiari malformation, syringomyelia and hydrocephalus by neuroendoscopic third ventriculostomyN Buxton
Department of Neurosurgery, University Hospital Nottingham, UK
Minim Invasive Neurosurg 45:231-4. 2002..Two similar cases are discussed. The technique is advocated for the treatment of such complex dysraphic conditions...
Surgical prognosis in hindbrain related syringomyeliaS Asgari
Department of Neurosurgery, University Hospital Essen, Hufelandstr 55, Essen, Germany
Acta Neurol Scand 107:12-21. 2003..The recommended operative treatment against hindbrain related syringomyelia is suboccipital decompression. The aim of the study was to define prognostic factors for surgical outcome in this disease...
Acute external hydrocephalus complicating craniocervical decompression for syringomyelia-Chiari I complex: case report and review of the literaturePaolo Perrini
Department of Neurosurgery, Hope Hospital, Salford, Manchester, UK
Neurosurg Rev 31:331-5. 2008....
Time course of syringomyelia resolution following decompression of Chiari malformation Type INicholas M Wetjen
Department of Neurologic Surgery, Mayo Clinic, Rochester, Minnesota, USA
J Neurosurg Pediatr 1:118-23. 2008....
Presyrinx in children with Chiari malformationsS Goh
School of Medicine, University of California San Francisco, San Francisco, CA 94143, USA
Neurology 71:351-6. 2008..Only three pediatric cases have been reported previously. We describe the clinical and radiologic features of presyrinx in six pediatric patients...
Comparison of posterior fossa decompression with and without duraplasty for the surgical treatment of Chiari malformation Type I in pediatric patients: a meta-analysisSusan R Durham
Department of Surgery Pediatric Neurosurgery, Dartmouth Medical School, Hanover, NH, USA
J Neurosurg Pediatr 2:42-9. 2008..The objective of this meta-analysis was to compare the outcome of posterior fossa decompression with duraplasty (PFDD) and posterior fossa decompression without duraplasty (PFD) for the treatment of CM-I in children...
Ultrastructural pathology of Golgi apparatus of nerve cells in human brain edema associated to brain congenital malformations, tumours and traumaO J Castejon
Institute of Biological Investigations, Faculty of Medicine, University of Zulia, Maracaibo, Venezuela
J Submicrosc Cytol Pathol 31:203-13. 1999..The ischemia and anoxia associated to the vasogenic and cytotoxic brain edema induced enlargement, fragmentation and disappearance of stacked Golgi cisternae...
Prevalence of neural axis abnormalities in patients with infantile idiopathic scoliosisMatthew B Dobbs
Department of Orthopaedic Surgery, Washington University School of Medicine, Saint Luis, Missouri 63110, USA
J Bone Joint Surg Am 84:2230-4. 2002..In two previous studies involving a total of only ten patients with infantile idiopathic scoliosis, five patients were noted to have a neural axis abnormality on magnetic resonance images...
[Surgical management of syringomyelia associated with Arnold-Chiari malformation, primary IgA deficiency and chromosomal abnormality--a case report]A Keyaki
Dpartment of Neurosurgery and Neurology, Tenri Hospital, Nara, Japan
Nihon Geka Hokan 59:161-7. 1990..The association of these three anomalies is very rare and genetical relationship is not known. From surgical point of view, it is conceivable that preoperative management in a case of asymptomatic IgA deficiency is uneventful...
Spondylolysis associated with Arnold-Chiari malformation and syringomyelia. A report of two casesJ D Bowen
Department of Physical Medicine and Rehabilitation, Mayo Clinic, Rochester, Minnesota, USA
Spine (Phila Pa 1976) 22:2458-63. 1997..e., Arnold-Chiari malformation Type I)...
Auditory brainstem response abnormalities and hearing loss in children with craniosynostosisMichael W Church
Department of Obstetrics and Gynecology, Wayne State University School of Medicine, Detroit, Michigan, USA
Pediatrics 119:e1351-60. 2007..Our objective was to evaluate such patients for auditory brainstem response and hearing disorders with the long-term goal of improving patient evaluation and management...
[Platybasia, basilar groove, odontoid process and kinking of the brainstem: a common etiology with idiopathic syringomyelia, scoliosis and Chiari malformations]M B Royo-Salvador
Servicio de Neurocirugia, Clinica Tres Torres, Barcelona, Espanña
Rev Neurol 24:1241-50. 1996..Confirmation is based on an abnormally low position of the conus medullaris (CMB) in the patients with SMI...
Bilateral congenital vocal cord paralysis: a 16-year institutional reviewR Christopher Miyamoto
Department of Otolaryngology and Communication Disorders, Children s Hospital Boston, Harvard Medical School, Boston, Massachusetts, USA
Otolaryngol Head Neck Surg 133:241-5. 2005..To review the management and outcome of bilateral congenital true vocal cord paralysis in 22 patients treated over a 16-year period and to review the role of tracheostomy in these patients...
In utero repair of myelomeningocele: experimental pathophysiology, initial clinical experience, and outcomesDiana L Farmer
Departments of Surgery and Neurosurgery and the Fetal Treatment Center, University of California, San Francisco, CA 94143 0570, USA
Arch Surg 138:872-8. 2003..Experimental work raises the possibility that in utero repair of myelomeningocele (MMC) may improve lower extremity, bladder, and bowel function, ameliorate the Arnold-Chiari malformation, and decrease the need for postnatal shunting...
Axillary vein thrombosis as a manifestation of rapidly progressive neuropathic arthropathy of the shoulder associated with syringomyeliaAntonio G Tristano
Department of Internal Medicine, Hospital Dr Domingo Luciani, Caracas, Venezuela
Mayo Clin Proc 80:416-8. 2005..To our knowledge, this is the first report of a patient with this constellation of findings...
[A new surgical treatment for syringomyelia, scoliosis, Arnold-Chiari malformation, kinking of the brainstem, odontoid recess, idiopathic basilar impression and platybasia]M B Royo Salvador
, , Barcelona, Jefe del,
Rev Neurol 25:523-30. 1997..on medullary traction as responsible for idiopathic syringomyelia (SMI), idiopathic scoliosis (ESCID), Arnold Chiari malformation (ARCH), platybasia (PTB), basilar impression (IMB), odontoid recess (RTO) kinking of the brain stem (KTC)..
Blepharoclonus and Arnold-Chiari malformationD E Jacome
Department of Medicine, Franklin Medical Center, 164 High Street, Greenfield, Massachusetts 01301, USA
Acta Neurol Scand 104:113-7. 2001..BLC may follow major head trauma. Four patients with Arnold-Chiari malformation (ACM) and BLC are described...
[Tinnitus treatment: neurosurgical management]A Londero
Service d ORL et de chirurgie cervicofaciale, Hopital Europeen Georges Pompidou, 75908 Paris Cedex 15, France
Neurochirurgie 55:248-58. 2009..However, in the near future better knowledge of tinnitus pathophysiology and innovative therapeutic tools could emerge from neuromodulation techniques such as repeated transcranial magnetic or epidural electric stimulation...
Emergence of oropharyngeal, laryngeal and swallowing activity in the developing fetal upper aerodigestive tract: an ultrasound evaluationJeri L Miller
Department of Rehabilitation Medicine, Physical Disabilities Branch, Room S6235, Building 10, Warren G Magnuson Clinical Center, National Institutes of Health, 9000 Rockville Pike, Bethesda, MD 20892, USA
Early Hum Dev 71:61-87. 2003..controls were compared to seven cases at risk for postnatal feeding and swallowing dysfunction (Type II Arnold Chiari Malformation, trisomy 18, polyhydramnios, intrauterine growth restriction, Brachmann-de Lange Syndrome)...
Neuropathic arthropathy progressing with multiple joint involvement in the upper extremity due to syringomyelia and type I Arnold-Chiari malformationBaris Nacir
2nd Department of Physical Medicine and Rehabilitation, Ankara Training and Research Hospital, Ministry of Health, 06340, Cebeci, Ankara, Turkey
Rheumatol Int 30:979-83. 2010..The characteristics of this presented case is the presence of Arnold-Chiari malformation accompanying with syringomyelia and involvement of the shoulder, elbow and hand (multiple joint involvement)...
Headache in Ehlers-Danlos syndromeD E Jacome
Franklin Medical Center, Department of Medicine, Greenfield, MA, USA
Cephalalgia 19:791-6. 1999..The clinical data collected on 18 patients with EDS and chronic headaches is reported...
[Clinico-epidemiologic study of infantile hydrocephalus in Japan]T Negoro
Department of Pediatrics, Nagoya
No To Hattatsu 26:211-5. 1994..3) Clinico-epidemiologic features of intractable hydrocephalus based on the diagnostic criteria of the research committee was investigated comparing intractable with non-intractable groups.(ABSTRACT TRUNCATED AT 250 WORDS)..
Relation between intracranial volume and the surface area of the foramen magnumNiyazi Acer
School of Health Sciences, Mugla University, Mugla, Turkey
J Craniofac Surg 17:326-30. 2006..The cephalometry could apply on living subjects and, thereby, our findings could provide some data to evaluate the etiology of Arnold Chiari malformation and achondroplasia for living subjects.
Congenital vocal cord paralysisMehmet Ada
Otolaryngology Head and Neck Surgery Department, Cerrahpasa Medical School, Istanbul University, Istanbul, Turkey
J Craniofac Surg 21:273-4. 2010..In this article, we report 2 patients with idiopathic congenital vocal cord paralysis. One of them has the unilateral vocal cord paralysis, and the other, bilateral...
The value of transcranial cerebral sonography in diagnosing neurootological disordersJoel F Lehrer
Holy Name Hospital, Teaneck, New Jersey, USA
Int Tinnitus J 15:164-7. 2009..Furthermore, TCCS is a valuable addition to neurootologists' test batteries...
Cranial malformations in related white lions (Panthera leo krugeri)F E Scaglione
Dipartimento di Patologia Animale, Universita degli Studi di Torino, Via L da Vinci 44, 10095 Grugliasco, Italy
Vet Pathol 47:1095-9. 2010..Paternal consanguinity of the parents, along with inbreeding among white lions in general, could account for the high incidence of congenital malformations of the head in this pride of white lions...
[Our experience with the programmable Codman-Medos valve: review of 125 shunts]G Catalan
Servicio de Neurocirugia, Hospital de Cruces, Plaza de Cruces, s n E 48903 Barakaldo, Vizcaya
Rev Neurol 31:1136-42. 2000..Programmable valves are a possible solution in cases of excessive or insufficient draining...
Pulsatile cerebrospinal fluid flow measurement using phase-contrast magnetic resonance imaging in patients with cervical myelopathyRyoichi Shibuya
Department of Orthopaedic Surgery, Toyonaka Municipal Hospital, Toyonaka, Japan
Spine (Phila Pa 1976) 27:1087-93. 2002..A technical report is presented...
Cystoperitoneal shunt as alternative treatment for a giant thoracic meningocele in a patient with neurofibromatosisDimitri J Vanhauwaert
Department of Neurosurgery, Heilig Hart Ziekenhuis, Roeselare, Belgium, 8800
Surg Neurol 69:535-7. 2008..Usually, these meningoceles remain asymptomatic, but back pain, headache, cough, and dyspnea are possible manifestations. Often, there is an associated kyphoscoliotic deformity of the thoracic spine...
[Familial spastic paraplegia with severe amyotrophy of the hands. (Silver syndrome?)]I Feki
Service de Neurologie, CHU Habib Bourguiba, Sfax, Tunisie
Rev Neurol (Paris) 163:476-9. 2007..Abnormalities of the cranio-vertebral junction, Arnold-Chiari malformation, syringomyelia and familial juvenile amyotrophic lateral sclerosis (ALS) were excluded and the diagnosis of Silver's syndrome was evoked...
Sagittal profiles of the spine in scoliosis associated with an Arnold-Chiari malformation with or without syringomyeliaRandall T Loder
Shriners Hospital for Children Twin Cities, Minneapolis, Minnesota 55414, USA
J Pediatr Orthop 22:483-91. 2002..0001). When cervical lordosis is >0 degrees or thoracic kyphosis is >40 degrees (Cobb method), the clinician should strongly suspect the presence of an Arnold-Chiari I malformation with or without syringomyelia...
Somatosensory evoked potentials in Arnold-Chiari malformationRainer Boor
Department of Pediatric Neurology, University Children s Hospital, Johannes Gutenberg University, Langenbeckstrasse 1, D 55101 Mainz, Germany
Brain Dev 26:99-104. 2004..9), but not in the younger age group. Abnormal somatosensory conduction reflects dysfunction of the brainstem or the upper cervical cord and may be clinically useful to assess patients with late onset symptomatic AC malformation...
Spontaneous intracranial hypotension: headache with a reversible Arnold-Chiari malformationS E Kasner
Department of Neurology, Hospital of the University of Pennsylvania, Philadelphia 19104, USA
Headache 35:557-9. 1995..Symptomatic relief was sustained only with long-term theophylline administration. The apparent Arnold-Chiari malformation resolved with treatment of the low CSF pressure...
Treatment of downbeat nystagmus with 3,4-diaminopyridine: a placebo-controlled studyM Strupp
Department of Neurology, University of Munich, Germany
Neurology 61:165-70. 2003..These drugs were tested under the assumption that DBN was caused by a disinhibition of a physiologic inhibitory cerebellar input on vestibular nuclei...
Orofacial pain as the sole manifestation of syringobulbia-syringomyelia associated with Arnold-Chiari malformationM Penarrocha
Oral Medicine Valencia University Medical and Dental School, Valencia, Spain
J Orofac Pain 15:170-3. 2001..The symptoms were resolved following neurosurgical management with amplification of the foramen magnum...
Evaluation of intrasyrinx fluid motion by spatial modulation of magnetization-magnetic resonance imaging in syringomyelia with long-term follow-up: a predictor of postoperative prognosis?Chul Hwan Park
Department of Diagnostic Radiology and Research Institute of Radiological Science, Brain Korea 21 Project for Medical Science, Yonsei University College of Medicine, Yongdong Severance Hospital, Kangnam Gu, Seoul, South Korea
J Comput Assist Tomogr 32:135-40. 2008..To evaluate the use of measuring intrasyrinx fluid motion in syringomyelia by spatial modulation of magnetization (SPAMM)-magnetic resonance imaging (MRI) for predicting postoperative results...
Adolescent idiopathic scoliosis and the presence of spinal cord abnormalities. Preoperative magnetic resonance imaging analysisB Maiocco
Department of Orthopedics, Ochsner Clinic, New Orleans, Louisiana, USA
Spine (Phila Pa 1976) 22:2537-41. 1997..It appears from the results of this study that by following these guidelines, small syrinxes may remain undetected, but the clinical significance of this is unknown...
Research Grants
- Diffusion Tensor Imaging of Wallerian Degeneration in MSKhader Hasan; Fiscal Year: 2009..The DTI measures, after accounting for the age and gender dependence will be correlated with the clinical measures. ..
- Noninvasive Measurements of Intracranial PressureNoam Alperin; Fiscal Year: 2004..3. Quantify and demonstrate improved reproducibility of the volumetric flow rates and the resulting ICP values. ..
- Development and Early Clinical Evaluation of Noninvasive MRI Measurement of ICPNoam Alperin; Fiscal Year: 2006..Preliminary results demonstrated lesser inter-individual variability of the normalized tCBF measurement. ..
- Multicenter Trial of Fetal Myelomeningocele RepairDiana Farmer; Fiscal Year: 2007..In summary, the proposed trial will be able to demonstrate whether fetal intervention offers improved outcome with a reasonable quality of life for spina bifida children. ..
- PENNSYLVANIA ABUSIVE HEAD TRAUMA PREVENTION PROGRAMMark Dias; Fiscal Year: 2007....
- In Vivo Imaging of Post-Traumatic Cerebral Amyloid DepositionMarvin Bergsneider; Fiscal Year: 2007..The results may have important public health implications in that improved understanding of the pathological consequences of cerebral amyloid deposition may lead to medical treatments that will improve outcome following TBI. ..
- Multicenter Trial of Fetal Myelomeningocele RepairN Adzick; Fiscal Year: 2007..In summary, the proposed trial will be able to demonstrate whether fetal intervention offers improved outcome with a reasonable quality of life for spina bifida children. ..
- Folic Acid Enhances Repair Mechanism in the Adult CNSBermans J Iskandar; Fiscal Year: 2010....
- In Vivo CSF Shunt Hydrodynamics in HydrocephalusMarvin Bergsneider; Fiscal Year: 2010..Documenting definitive evidence of the tuned-dynamic absorber model could have fundamental implications to all ICP disorders. ..
- Diffusion Tensor Imaging of Wallerian Degeneration in MSKhader Hasan; Fiscal Year: 2007..This tool will also help in the fusion of multi-modal MRI data for a robust segmentation of the subregions of CC and 1C. The DTI measures, after accounting for the age and gender dependence will be correlated with the clinical measures. ..
- BAEP SCREENING OF NEONATESMICHAEL CHURCH; Fiscal Year: 2001..This proposal will address these issues as well. Potentially confounding variables will be gathered and their influence assessed and statistically controlled. Appropriate clinical interventions for mother and infant will be made. ..
- A HEMODYNAMIC MODEL OF INTRACRANIAL PRESSURE DYNAMICSMarvin Bergsneider; Fiscal Year: 2002..An improved understanding of these disorders will offer new and better treatment modalities for millions of affected patients. ..
- Multicenter Trial of Fetal Myelomeningocele RepairN Adzick; Fiscal Year: 2006..In summary, the proposed trial is expected to demonstrate whether fetal intervention offers improved outcome with a reasonable quality of life for spina bifida children. ..
- Multicenter Trial of Fetal Myelomeningocele RepairDiana Farmer; Fiscal Year: 2006..In summary, the proposed trial is expected to demonstrate whether fetal intervention offers improved outcome with a reasonable quality of life for spina bifida children. ..
- In Vivo CSF Shunt Hydrodynamics in HydrocephalusMarvin Bergsneider; Fiscal Year: 2007..Documenting definitive evidence of the tuned-dynamic absorber model could have fundamental implications to all ICP disorders. ..
