Research & Literature

Spinal cord detethering in children with tethered cord syndrome and Chiari type 1 malformations.

Glenn C, Cheema AA, Safavi-Abbasi S, et al.

Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia 2015; (22(11)):1749-52.

Postural Tachycardia Syndrome: Beyond Orthostatic Intolerance.

Garland EM, Celedonio JE, Raj SR

Current neurology and neuroscience reports 2015; (15(9)):60 doi:10.1007/s11910-015-0583-8.

Delayed resolution of syrinx after posterior fossa decompression without dural opening in children with Chiari malformation Type I.

Kennedy BC, Nelp TB, Kelly KM, et al.

Journal of neurosurgery. Pediatrics 2015; (16(5)):599-606 doi:10.3171/2015.4.PEDS1572.

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

Miranda SP, Kimmell KT, Silberstein HJ

World neurosurgery 2016; (85()):366.e1-4.

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

Tubbs RS

Neurosurgery clinics of North America 2015; (26(4)):487-93.

Ventral Decompression in Chiari Malformation, Basilar Invagination, and Related Disorders.

Ridder T, Anderson RC, Hankinson TC

Neurosurgery clinics of North America 2015; (26(4)):571-8.

Surgical management of syringomyelia unrelated to Chiari malformation or spinal cord injury.

Talacchi A, Meneghelli P, Borghesi I, Locatelli F

European spine journal : official publication of the European Spine Society, the European Spinal Deformity Society, and the European Section of the Cervical Spine Research Society 2016; (25(6)):1836-46 doi:10.1007/s00586-015-4262-x.

The international POTS registry: Evaluating the efficacy of an exercise training intervention in a community setting.

George SA, Bivens TB, Howden EJ, et al.

Heart rhythm 2016; (13(4)):943-50.

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

Sayal PP, Zafar A, Carroll TA

Journal of craniovertebral junction & spine 2016; (7(2)):101-4 doi:10.4103/0974-8237.181862.

Craniocervical spinal instability after type 1 Arnold Chiari decompression: a case report.

Camino Willhuber GO, Bosio ST, Puigdevall MH, et al.

Journal of pediatric orthopedics. Part B 2017; (26(1)):80-85 doi:10.1097/BPB.0000000000000346.

Severe Headache or Migraine History Is Inversely Correlated With Dietary Sodium Intake: NHANES 1999-2004: A Response.

Pogoda JM, Gross NB, Arakaki X, et al.

Headache 2016; (56(7)):1216-8 doi:10.1111/head.12868.

Are Herniated Cerebellar Tonsils the Main Culprit of Chiari Malformation Type I Symptoms? The Brainstem Compression Hypothesis seems to be Re-Elucidated and Revised.

Selcuki M, Mete M, Selcuki D

Turkish neurosurgery 2018; (28(1)):67-71 doi:10.5137/1019-5149.JTN.18349-16.1.

Ehlers-Danlos Syndrome-Hypermobility Type: A Much Neglected Multisystemic Disorder.

Gazit Y, Jacob G, Grahame R

Rambam Maimonides medical journal 2016; (7(4)) doi:10.5041/RMMJ.10261.

Hypermobile Ehlers-Danlos syndrome (a.k.a. Ehlers-Danlos syndrome Type III and Ehlers-Danlos syndrome hypermobility type): Clinical description and natural history.

Tinkle B, Castori M, Berglund B, et al.

American journal of medical genetics. Part C, Seminars in medical genetics 2017; (175(1)):48-69 doi:10.1002/ajmg.c.31538.

Orthopaedic management of the Ehlers-Danlos syndromes.

Ericson WB, Wolman R

American journal of medical genetics. Part C, Seminars in medical genetics 2017; (175(1)):188-194 doi:10.1002/ajmg.c.31551.

Treatment of Syringomyelia in Patients with Arachnoiditis at the Craniocervical Junction.

Davidoff CL, Liu S, Wong JHY, et al.

World neurosurgery 2017; (107()):565-573 doi:10.1016/j.wneu.2017.08.064.

Cyclical vomiting syndrome secondary to a Chiari I malformation-a case report.

White WL, Bagga V, Campbell DI, et al.

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery 2017; (33(12)):2083-2085 doi:10.1007/s00381-017-3589-5.

How Should Syringomyelia be Defined and Diagnosed?

Klekamp J

World neurosurgery 2018; (111()):e729-e745 doi:10.1016/j.wneu.2017.12.156.

Origin of Syrinx Fluid in Syringomyelia: A Physiological Study.

Heiss JD, Jarvis K, Smith RK, et al.

Neurosurgery 2019; (84(2)):457-468 doi:10.1093/neuros/nyy072.

The Role of Arachnoid Veils in Chiari Malformation Associated with Syringomyelia.

Ciappetta P, Signorelli F, Visocchi M

Acta neurochirurgica. Supplement 2019; (125()):97-99 doi:10.1007/978-3-319-62515-7_14.

The effect of combined exercise therapy on knee proprioception, pain intensity and quality of life in patients with hypermobility syndrome: A randomized clinical trial.

Daman M, Shiravani F, Hemmati L, Taghizadeh S

Journal of bodywork and movement therapies 2019; (23(1)):202-205 doi:10.1016/j.jbmt.2017.12.012.

[Up-to-date classification and multidisciplinary symptoms of Ehlers-Danlos syndromes].

Ralovich FV, Kiss N, Horváth K, et al.

Orvosi hetilap 2019; (160(16)):603-612 doi:10.1556/650.2019.31351.

Clinical diagnosis-part I: what is really caused by Chiari I.

Ciaramitaro P, Ferraris M, Massaro F, Garbossa D

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery 2019; (35(10)):1673-1679 doi:10.1007/s00381-019-04206-z.

Is Cervical Stabilization for All Cases of Chiari-I Malformation an Overkill? Evidence Speaks Louder Than Words!

Deora H, Behari S, Sardhara J, et al.

Neurospine 2019; (16(2)):195-206 doi:10.14245/ns.1938192.096.

Chiari I malformation in children-the natural history.

Chatrath A, Marino A, Taylor D, et al.

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery 2019; (35(10)):1793-1799 doi:10.1007/s00381-019-04310-0.

Chiari Type I Malformation with Syringomyelia Presenting with Isolated Hemi-anhidrosis: Report of a Rare Case.

Dutta G, Singh D, Jagetia A, et al.

Neurology India 2019; (67(4)):1140-1141 doi:10.4103/0028-3886.266256.

Multidimensional, patient-reported outcome after posterior fossa decompression in 79 patients with Chiari malformation type I.

De Vlieger J, Dejaegher J, Van Calenbergh F

Surgical neurology international 2019; (10()):242 doi:10.25259/SNI_377_2019.

Arnold-Chiari malformation type I and the posterior dislocation of the odontoid process aggravate prolonged weaning in a patient with severe viral pneumonia: a case report.

Ding R, Meng Y, Jia X, Ma X

BMC pulmonary medicine 2020; (20(1)):37 doi:10.1186/s12890-020-1078-1.

The Retroverted Dens: A Review of its Anatomy, Terminology, and Clinical Significance.

Ishak B, Dhaliwal G, Rengifo R, et al.

World neurosurgery 2020; (137()):304-309 doi:10.1016/j.wneu.2020.01.231.

Prevalence and Impact of Underlying Diagnosis and Comorbidities on Chiari 1 Malformation.

Sadler B, Kuensting T, Strahle J, et al.

Pediatric neurology 2020; (106()):32-37 doi:10.1016/j.pediatrneurol.2019.12.005.

Optimizing Alignment Parameters During Craniocervical Stabilization and Fusion: A Technical Note.

Henderson F, Rosenbaum R, Narayanan M, et al.

Cureus 2020; (12(3)):e7160 doi:10.7759/cureus.7160.

Timing of syrinx reduction and stabilization after posterior fossa decompression for pediatric Chiari malformation type I.

Chotai S, Chan EW, Ladner TR, et al.

Journal of neurosurgery. Pediatrics 2020; (26(2)):193-199.

Ivabradine in children with postural orthostatic tachycardia syndrome: a retrospective study.

Towheed A, Nesheiwat Z, Mangi MA, et al.

Cardiology in the young 2020; (30(7)):975-979 doi:10.1017/S1047951120001341.

Chiari 1 Formation Redefined-Clinical and Radiographic Observations in 388 Surgically Treated Patients.

Goel A, Jadhav D, Shah A, et al.

World neurosurgery 2020; (141()):e921-e934 doi:10.1016/j.wneu.2020.06.076.

Occult tethered cord syndrome: a reversible cause of paraparesis not to be missed.

Tsiptsios D, Sysoev K, Ouranidis A, et al.

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery 2020; (36(9)):1833-1834 doi:10.1007/s00381-020-04768-3.

Treatment of Postural Orthostatic Tachycardia Syndrome With Medication: A Systematic Review.

Hasan B, Almasri J, Marwa B, et al.

Journal of child neurology 2020; (35(14)):1004-1016 doi:10.1177/0883073820948679.

The battle against an invisible enemy.

Tripathi M, Mohindra S

Acta neurochirurgica 2020; (162(11)):2923-2924 doi:10.1007/s00701-020-04555-8.

Arnold-Chiari malformation type 1 as an unusual cause of acute respiratory failure: A case report.

Khatib MY, Elshafei MS, Shabana AM, et al.

Clinical case reports 2020; (8(10)):1943-1946 doi:10.1002/ccr3.3043.

Development of pre-syrinx state and syringomyelia following a minor injury: a case report.

Kleindienst A, Engelhorn T, Roeckelein V, Buchfelder M

Journal of medical case reports 2020; (14(1)):223 doi:10.1186/s13256-020-02568-6.

Prevalence of joint hypermobility syndromes in pediatric postural orthostatic tachycardia syndrome.

Boris JR, Bernadzikowski T

Autonomic neuroscience : basic & clinical 2021; (231()):102770 doi:10.1016/j.autneu.2020.102770.

Report of two cases with omovertebral bone and Klippel-Feil syndrome with craniovertebral junction instability.

Shah A, Hawaldar A, Lunawat A, et al.

Journal of craniovertebral junction & spine 2021; (12(1)):95-98 doi:10.4103/jcvjs.JCVJS_7_21.

Hypermobile Ehlers-Danlos Syndrome and Hypermobility Spectrum Disorders.

Yew KS, Kamps-Schmitt KA, Borge R

American family physician 2021; (103(8)):481-492.

Nonoperative management of enlarging syringomyelia in clinically stable patients after decompression of Chiari malformation type I.

Szuflita NS, Phan TN, Boulter JH, et al.

Journal of neurosurgery. Pediatrics 2021; (28(1)):28-33 doi:10.3171/2020.12.PEDS20621.

Role of preoperative cervical alignment on postoperative dysphagia after occipitocervical fusion.

Miyagi M, Takahashi H, Sekiya H, Ebihara S

Surgical neurology international 2021; (12()):350 doi:10.25259/SNI_547_2021.

Risk of occult spinal dysraphism based on lumbosacral cutaneous manifestations.

Shields LB, Mutchnick IS, Daniels MW, et al.

SAGE open medicine 2021; (9()):20503121211037172 doi:10.1177/20503121211037172.

Arnold-Chiari Malformation: Core Concepts.

Fons K, Jnah AJ

Neonatal network : NN 2021; (40(5)):313-320 doi:10.1891/11-T-704.

Surgical Outcomes of Adult Chiari Malformation Type 1: Experience at a Tertiary Institute.

Gündağ Papaker M, Abdallah A, Çınar İ

Cureus 2021; (13(9)):e17876 doi:10.7759/cureus.17876.

Dysautonomia in the Ehlers-Danlos syndromes and hypermobility spectrum disorders-With a focus on the postural tachycardia syndrome.

Mathias CJ, Owens A, Iodice V, Hakim A

American journal of medical genetics. Part C, Seminars in medical genetics 2021; (187(4)):510-519 doi:10.1002/ajmg.c.31951.

Occipital condyle screw fixation after posterior decompression for Chiari malformation: Technical report and application.

Chakraborty AR, Pelargos PE, Milton CK, et al.

Surgical neurology international 2021; (12()):543 doi:10.25259/SNI_26_2021.

Headache characteristics and postoperative course in Chiari I malformation.

Thunstedt DC, Schmutzer M, Fabritius MP, et al.

Cephalalgia : an international journal of headache 2022; (42(9)):879-887 doi:10.1177/03331024221079296.

Idiopathic mast cell activation syndrome is more often suspected than diagnosed-A prospective real-life study.

Buttgereit T, Gu S, Carneiro-Leão L, et al.

Allergy 2022; (77(9)):2794-2802 doi:10.1111/all.15304.

Complications and outcomes of posterior fossa decompression with duraplasty versus without duraplasty for pediatric patients with Chiari malformation type I and syringomyelia: a study from the Park-Reeves Syringomyelia Research Consortium.

Akbari SHA, Yahanda AT, Ackerman LL, et al.

Journal of neurosurgery. Pediatrics 2022; (30(1)):39-51 doi:10.3171/2022.2.PEDS21446.

Increase in clivo-axial angle is associated with clinical improvement in children undergoing occipitocervical fusion for complex Chiari malformation: patient series.

Marianayagam NJ, Chae JK, Hussain I, et al.

Journal of neurosurgery. Case lessons 2021; (2(23)):CASE21433 doi:10.3171/CASE21433.

Spinal cord stimulator for the treatment of central neuropathic pain secondary to cervical syringomyelia: illustrative case.

Schatmeyer BA, Dodin R, Kinsman M, Garcia D

Journal of neurosurgery. Case lessons 2022; (4(6)).

Management of failed Chiari decompression and intrasyringeal hemorrhage in Noonan syndrome: illustrative cases.

Falls CJ, Page PS, Greeneway GP, et al.

Journal of neurosurgery. Case lessons 2022; (3(4)).

Postural orthostatic tachycardia syndrome and post-acute COVID-19.

Amekran Y, Damoun N, El Hangouche AJ

Global cardiology science & practice 2022; (2022(1-2)):e202213 doi:10.21542/gcsp.2022.13.

Compromised Cranio-Spinal Suspension in Chiari Malformation Type 1: A Potential Role as Secondary Pathophysiology.

Shao B, Poggi JA, Amaral-Nieves N, et al.

Journal of clinical medicine 2022; (11(24)) doi:10.3390/jcm11247437.

Reference values of four measures of craniocervical stability using upright dynamic magnetic resonance imaging.

Nicholson LL, Rao PJ, Lee M, et al.

La Radiologia medica 2023; (128(3)):330-339 doi:10.1007/s11547-023-01588-8.

Fourth ventricular subarachnoid stent for Chiari malformation type I-associated persistent syringomyelia.

Han RK, Medina MP, Giantini-Larsen AM, et al.

Neurosurgical focus 2023; (54(3)):E10 doi:10.3171/2022.12.FOCUS22633.

COVID-19 Induced Postural Orthostatic Tachycardia Syndrome (POTS): A Review.

Mallick D, Goyal L, Chourasia P, et al.

Cureus 2023; (15(3)):e36955 doi:10.7759/cureus.36955.

Surgical outcomes of basilar invagination type B without atlantoaxial dislocation through simple posterior fossa decompression: a retrospective study of 18 cases.

Chen XY, Chen W, Zhao JL, et al.

Acta neurochirurgica 2023; (165(10)):3051-3063 doi:10.1007/s00701-023-05625-3.

Spontaneous improvement in syringomyelia in a patient with Chiari 1 malformation: illustrative case.

Ozturk O, Cındıl E, Emmez H, et al.

Journal of neurosurgery. Case lessons 2023; (6(3)).

Hypothesis on the pathophysiology of syringomyelia based on analysis of phase-contrast magnetic resonance imaging of Chiari-I malformation patients.

Chang HS

F1000Research 2021; (10()):996 doi:10.12688/f1000research.72823.2.

Fear avoidance, fear of falling, and pain disability in hypermobile Ehlers-Danlos syndrome and hypermobility spectrum disorders.

Chuchin JD, Ornstein TJ

Disability and rehabilitation 2024; (46(18)):4234-4245 doi:10.1080/09638288.2023.2268520.

Neuropsychiatric Manifestations of Mast Cell Activation Syndrome and Response to Mast-Cell-Directed Treatment: A Case Series.

Weinstock LB, Nelson RM, Blitshteyn S

Journal of personalized medicine 2023; (13(11)) doi:10.3390/jpm13111562.

Coronavirus disease 2019-associated persistent cough and Chiari malformation type I resulting in acute respiratory failure: illustrative case.

Piscopo AJ, Teferi N, Marincovich A, et al.

Journal of neurosurgery. Case lessons 2023; (6(25)).

Chiari Malformation Type 1 and Syringomyelia: Why Do Patients Claim for International Guidelines? Commentary on the 2021 Chiari and Syringomyelia Consensus Document.

Massimi L, Vetrano IG, Peretta P, et al.

Acta neurochirurgica. Supplement 2023; (135()):139-145 doi:10.1007/978-3-031-36084-8_23.

Craniocervical instability in patients with Ehlers-Danlos syndromes: outcomes analysis following occipito-cervical fusion.

Henderson FC, Schubart JR, Narayanan MV, et al.

Neurosurgical review 2024; (47(1)):27 doi:10.1007/s10143-023-02249-0.

Evaluation and Treatment of Patients with Small Posterior Cranial Fossa and Chiari Malformation, Types 0 and 1.

Bogdanov EI, Heiss JD

Advances and technical standards in neurosurgery 2024; (50()):307-334 doi:10.1007/978-3-031-53578-9_11.

Management of childbearing with hypermobile Ehlers-Danlos syndrome and hypermobility spectrum disorders: A scoping review and expert co-creation of evidence-based clinical guidelines.

Pezaro S, Brock I, Buckley M, et al.

PloS one 2024; (19(5)):e0302401 doi:10.1371/journal.pone.0302401.

A Cross-Sectional Study of Cardiovascular Autonomic Reactivity in Ehlers-Danlos Syndrome.

Alauddin W, Alam S, Mishra M, et al.

Cureus 2024; (16(7)):e64542 doi:10.7759/cureus.64542.

Cardiovascular, autonomic symptoms and quality of life in children with hypermobile Ehlers-Danlos syndrome.

Hertel AK, Black WR, Lytch A, et al.

SAGE open medicine 2024; (12()):20503121241287073 doi:10.1177/20503121241287073.

The Genetics of Chiari 1 Malformation.

Yan RE, Chae JK, Dahmane N, et al.

Journal of clinical medicine 2024; (13(20)) doi:10.3390/jcm13206157.

Indications for Surgery and Surgical Options in Chiari Malformation: WFNS Spine Committee Recommendations.

Visocchi M, Signorelli F, Alves ÓL, et al.

Spine 2025; (50(11)):760-766 doi:10.1097/BRS.0000000000005288.

Incidence and Management of Basilar Invagination With Associated Chiari I Malformation: WFNS Spine Committee Recommendations.

Klekamp J, Alves OL, Zileli M, et al.

Spine 2025; (50(11)):786-791 doi:10.1097/BRS.0000000000005293.

Predecompression and postdecompression cognitive and affective changes in Chiari malformation type I.

Henry LC, McDowell MM, Stephenson TL, et al.

Journal of neurosurgery 2025; (143(1)):4-12 doi:10.3171/2024.8.JNS241363.

Dysautonomia: a common comorbidity of systemic disease.

Blitshteyn S

Immunologic research 2025; (73(1)):105 doi:10.1007/s12026-025-09661-2.

The Efficacy of Atlantoaxial Facet Joint Distraction and Fixation and Foramen Magnum Decompression for Chiari Type 1 Malformation with Basilar Invagination: A Case Report.

Kohara K, Maegawa T, Okumura E, et al.

NMC case report journal 2025; (12()):283-288 doi:10.2176/jns-nmc.2025-0052.

Central arterial stiffness, flow-mediated dilation, and venous function in postural orthostatic tachycardia syndrome.

Pugh GE, Thomas KN, Fan JL, Fisher JP

American journal of physiology. Heart and circulatory physiology 2026; (330(1)):H89-H99 doi:10.1152/ajpheart.00590.2025.

Chiari Malformation Type I: A Review of Pathophysiology, Cerebrospinal Fluid Flow Dynamics, Diagnosis, Surgical Management, and Its Relationship to Syringomyelia.

Alhosani MS, Gachechiladze S

Cureus 2026; (18(1)):e101226 doi:10.7759/cureus.101226.