4,904 results match your criteria Syringomyelia


A retrospective study on outcomes following posterior fossa decompression with dural splitting surgery in patients with Chiari type I malformation.

Clin Neurol Neurosurg 2020 Jun 26;196:106035. Epub 2020 Jun 26.

Department of Neurosurgery, Shanghai Tenth People's Hospital, Tongji University School of Medicine, Shanghai 200072, People's Republic of China. Electronic address:

Background: Chiari type I malformation (CM I) is a congenital defect of the skull base and brain. Posterior fossa decompression alone or with duraplasty and partial resection of cerebellar tonsils is a standard approach. The effectiveness of other approaches such as the dural splitting technique is still under the study. Read More

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http://dx.doi.org/10.1016/j.clineuro.2020.106035DOI Listing

Late-Onset Tethered Cord Syndrome in a Patient with Spina Bifida: A Case Report.

Sisli Etfal Hastan Tip Bul 2018 29;52(2):138-141. Epub 2018 May 29.

Department of Physical Medicine and Rehabilitation, Istanbul Medipol University, Faculty of Medicine, Istanbul, Turkey.

Tethered cord is a clinical syndrome associated with short and thick filum terminale. It can occur because of congenital or acquired reasons and can lead to progressive neurological deficits. A thorough medical history, physcial examination, imaging, and electrophysiological tests are heplful in its diagnosis. Read More

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http://dx.doi.org/10.14744/SEMB.2017.64936DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7315062PMC

Autonomic dysreflexia triggered by breastfeeding in a non-plegic patient with syringomyelia: A case report.

Int J Gynaecol Obstet 2020 Jun 29. Epub 2020 Jun 29.

First Department of Obstetrics and Gynecology, Papageorgiou General Hospital, Aristotle University of Thessaloniki, Thessaloniki, Greece.

Postnatally developed autonomic dysreflexia (AD) can be a rare manifestation of breastfeeding.[1] Although antepartum AD and postpartum exacerbations have been previously reported in the literature, postnatally developed AD triggered by breastfeeding is an extremely rare occurrence. To the best of our knowledge, the present study reports the first non-plegic post-traumatic syringomyelia patient with postnatally developed AD triggered by breastfeeding. Read More

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http://dx.doi.org/10.1002/ijgo.13284DOI Listing

The SHORE score: a novel predictive tool for improvement after decompression surgery in adult Chiari malformation type I.

World Neurosurg 2020 Jun 26. Epub 2020 Jun 26.

Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, MD. Electronic address:

Background: A practical scoring system predicting significant improvement after surgical decompression in adult Chiari malformation type I (CM-1) based on validated outcome measures is lacking. We aimed to develop a simple score and improvement calculator to facilitate the decision-making process in symptomatic CM-1 patients.

Methods: We evaluated adult CM-1 patients who presented to our institution between September 2006 and September 2018 and underwent surgical decompression. Read More

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http://dx.doi.org/10.1016/j.wneu.2020.06.175DOI Listing

Prospective Follow-up of Intramedullary Slitlike Cavities: A Consecutive Series of 48 Patients.

Front Neurol 2020 12;11:495. Epub 2020 Jun 12.

Neurosurgery Department, Bicêtre University Hospital, Assistance Publique Hôpitaux de Paris (APHP), Université Paris-Sud, Le Kremlin-Bicêtre, France.

Predicting whether intramedullary slitlike cavity (SC) will worsen over time or remain stable is an outstanding clinical challenge. The aim of this study was to identify early features of SC (clinical and magnetic resonance imaging [MRI] findings). We prospectively included all patients referred to our institution following the discovery of a SC and divided them in two groups: typical SC (defined as a cavity spanning fewer than three vertebrae, not enlarging the spinal cord, and located at the midline between the anterior third and posterior two-thirds of the spinal cord) or atypical SC (all others). Read More

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http://dx.doi.org/10.3389/fneur.2020.00495DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7304370PMC

Risk factors of unplanned neurosurgery for scoliotic patients with Chiari malformation type I and syringomyelia after spinal deformity correction.

Clin Neurol Neurosurg 2020 Jun 20;196:106014. Epub 2020 Jun 20.

Department of Orthopedic Surgery and Orthopedic Research Institute, West China Hospital and West China School of Medicine, Sichuan University, Chengdu, China. Electronic address:

Objectives: It remains unclear which subgroups of scoliotic patients with CMI and syringomyelia are more likely to undergo unplanned neurosurgery after spinal deformity correction. The purpose of this study is to explore risk factors of unplanned neurosurgery for scoliotic patients with CMI and syringomyelia after spinal deformity correction.

Patients And Methods: This cohort consisted of 62 scoliotic patients with CMI and syringomyelia who underwent spinal deformity surgery with a mean follow-up of 4. Read More

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http://dx.doi.org/10.1016/j.clineuro.2020.106014DOI Listing

Secondary tethered cord syndrome in adult patients: retethering rates, long-term clinical outcome, and the effect of intraoperative neuromonitoring.

Acta Neurochir (Wien) 2020 Jun 25. Epub 2020 Jun 25.

Department of Pediatric Neurosurgery, Charité - Universitätsmedizin Berlin, Augustenburger Platz 1, 13353, Berlin, Germany.

Background: The strategy for surgical treatment of tethered cord syndrome in pediatric patients is well established but still bares challenges for adult patients. This retrospective study was performed to assess the surgical outcome of adult patients with a secondary tethered cord syndrome and to evaluate the benefit of intraoperative neuromonitoring.

Methods: Clinical charts of 32 consecutive adult patients who underwent in total 38 surgical untethering procedures at our facility between 2008 and 2018 were retrospectively analyzed. Read More

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http://dx.doi.org/10.1007/s00701-020-04464-wDOI Listing

Cerebrospinal fluid levels of GFAP and pNF-H are elevated in patients with chronic spinal cord injury and neurological deterioration.

Acta Neurochir (Wien) 2020 Jun 25. Epub 2020 Jun 25.

Department of Neuroscience, Neurosurgery, Uppsala University, Uppsala, Sweden.

Background: Years after a traumatic spinal cord injury (SCI), a subset of patients may develop progressive clinical deterioration due to intradural scar formation and spinal cord tethering, with or without an associated syringomyelia. Meningitis, intradural hemorrhages, or intradural tumor surgery may also trigger glial scar formation and spinal cord tethering, leading to neurological worsening. Surgery is the treatment of choice in these chronic SCI patients. Read More

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http://dx.doi.org/10.1007/s00701-020-04422-6DOI Listing

Posterior fossa decompression with duraplasty in Chiari malformation type 1: a systematic review and meta-analysis.

Acta Neurochir (Wien) 2020 Jun 23. Epub 2020 Jun 23.

Royal Sussex County Hospital, Brighton and Sussex University Hospitals NHS Trust, Brighton, UK.

Background: Surgery for symptomatic Chiari type I malformation (CM-I) patients include posterior fossa decompression (PFD) involving craniectomy with or without dural opening, and posterior fossa decompression with duraplasty (PFDD). This review aims to examine the evidence to aid surgical decision-making.

Methods: A medical database search was expanded to include article references to identify all relevant published case series. Read More

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http://dx.doi.org/10.1007/s00701-020-04403-9DOI Listing

Chiari 1 formation- redefined- Clinical and radiographic observations in 388 surgically treated cases.

World Neurosurg 2020 Jun 17. Epub 2020 Jun 17.

Department of Neurosurgery, Lilavati Hospital and Research Centre, Bandra (E), Mumbai.

Objective: The subject of Chiari formation is revisited and redefined. Results of surgical treatment of patients with Chiari formation by atlantoaxial fixation are presented.

Material And Methods: Three hundred and eighty eight patients with Chiari formation were surgically treated during the period 2010 to June 2019 and have been analyzed. Read More

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http://dx.doi.org/10.1016/j.wneu.2020.06.076DOI Listing

Surgical Scoliosis Correction in Chiari-I Malformation with Syringomyelia Versus Idiopathic Syringomyelia.

J Bone Joint Surg Am 2020 Jun 10. Epub 2020 Jun 10.

Department of Orthopedics, Peking Union Medical College Hospital and Graduate School of Peking Union Medical College, Peking Union Medical College, Chinese Academy of Medical Science, Beijing, People's Republic of China.

Background: Both patients with Chiari-I malformation (CIM) with syringomyelia and those with idiopathic syringomyelia (ISm) have a syrinx and can have scoliosis as well. However, there is no literature regarding differences between CIM and ISm in terms of radiographic outcomes and surgical complications after posterior fusion, to our knowledge. The aim of the present study was to compare radiographic features, clinical outcomes, and surgical complications after posterior spinal fusion between patients with CIM-associated scoliosis and those with ISm-associated scoliosis. Read More

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http://dx.doi.org/10.2106/JBJS.20.00058DOI Listing

Clinicoradiographic data and management of children with Chiari malformation type 1 and 1.5: an Italian case series.

Acta Neurol Belg 2020 Jun 10. Epub 2020 Jun 10.

Department of General Surgery and Medical Surgical Specialties, Section of Orthopaedics and Traumatologic Surgery, University of Catania, 95123, Catania, Italy.

Introduction: The widespread use of imaging has increased Chiari malformation (CM) diagnosis. CM shows clinical heterogeneity that makes management controversial. We aimed to evaluate the occurrence and clinical and radiographic presentation of children with CM-1 and CM-1. Read More

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http://dx.doi.org/10.1007/s13760-020-01398-zDOI Listing

[Cerebrospinal fluid flow dynamics and the potential of phase-contrast MRI in syringomyelia].

Ugeskr Laeger 2020 May;182(22)

Cerebrospinal fluid flow dynamics are not fully understood. However, it is well known, that flow dynamics play a crucial role in development and treatment of a wide variety of diseases in the central nervous system, among them, syringomyelia. Hence, in-depth knowledge of the mechanisms underlying flow dynamics is of great interest to improve diagnostics, prognostics, and to assess treatment efficacy. Read More

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Chiari malformation type I and basilar invagination originating from atlantoaxial instability: a literature review and critical analysis.

Acta Neurochir (Wien) 2020 Jul 6;162(7):1553-1563. Epub 2020 Jun 6.

Department of Neurosurgery, Klinikum rechts der Isar, Technical University Munich School of Medicine, Ismaninger Str. 22, 81675, Munich, Germany.

Introduction: Recently, a novel hypothesis has been proposed concerning the origin of craniovertebral junction (CVJ) abnormalities. Commonly found in patients with these entities, atlantoaxial instability has been suspected to cause both Chiari malformation type I and basilar invagination, which renders the tried and tested surgical decompression strategy ineffective. In turn, C1-2 fusion is proposed as a single solution for all CVJ abnormalities, and a revised definition of atlantoaxial instability sees patients both with and without radiographic evidence of instability undergo fusion, instead relying on the intraoperative assessment of the atlantoaxial joints to confirm instability. Read More

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http://dx.doi.org/10.1007/s00701-020-04429-zDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7295832PMC

External syringomyelia in longstanding benign foramen magnum tumors.

Surg Neurol Int 2020 2;11:92. Epub 2020 May 2.

Department of Neurosurgery, King Edward VII Memorial Hospital, Mumbai, Maharashtra, India.

Background: The effect of benign foramen magnum tumours on cranial and spinal dimensions and cerebrospinal fluid (CSF) spaces is unclear. In this study, we measured alterations in cerebrospinal fluid (CSF) spaces in the spinal canal and in the posterior cranial fossa distant from the site of benign foramen magnum tumors.

Methods: Twenty-nine magnetic resonance imaging scans of patients with foramen magnum tumors (8 meningiomas and 21 C2 neurinomas) were identified for radiological morphometric analysis and compared with normal control scans. Read More

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http://dx.doi.org/10.25259/SNI_106_2020DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7265472PMC

Hindbrain-related syringomyelia and raised intra-abdominal pressure: implications for safety of laparoscopic and robotic surgery.

Ann Ital Chir 2020 May 25;9. Epub 2020 May 25.

Background: The causative role played by intra-abdominal pressure (IAP) in the syringogenesis of the Chiari 1 malformation syringomyelia has been still not adequately studied. The aim of this study is to validate the transmedullary theory about the hindbrain-related syrinx, also discussing the implications for safety of these patients related to the use of high-pressure CO2 pneumoperitoneum during laparoscopic and robotic surgery.

Methods: Fourteen patients with a hindbrain-related syrinx were candidate for a posterior fossa decompression. Read More

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Syringomyelia as a delayed complication of lumbar-sacral adhesive arachnoiditis in Pott's disease.

BMJ Case Rep 2020 May 27;13(5). Epub 2020 May 27.

Department of General Medicine, Government Medical College and Hospital, Chandigarh, India.

Syringomyelia is the development of a fluid-filled cyst (syrinx) within the spinal cord and is an extremely rare chronic manifestation of tuberculosis. The syrinx so formed may expand over time, causing compression or destruction of spinal tracts and surrounding nerve roots. Development of syringomyelia in a patient of Pott's disease is particularly infrequent. Read More

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http://dx.doi.org/10.1136/bcr-2019-234032DOI Listing

Comparison of surgical outcomes in patients with Chiari Type I malformation receiving posterior fossa decompression with and without duraplasty.

Childs Nerv Syst 2020 Jul 25;36(7):1399-1405. Epub 2020 May 25.

Donald & Barbara Zucker School of Medicine at Hofstra/Northwell, 500 Hofstra Boulevard, Hempstead, NY, 11549, USA.

Purpose: The goals of this study were to compare clinical outcomes in patients with Chiari Malformation Type I (CMI) receiving posterior fossa decompression with (PFDD) or without duraplasty (PFD).

Methods: We conducted a retrospective analysis of 178 consecutive cases of 157 patients undergoing PFDD or PFD for CMI at Cohen Children's Medical Center between 2007 and 2017. Clinical improvement was defined as a clear reduction of preoperative symptoms after surgery as reported by patients and parents. Read More

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http://dx.doi.org/10.1007/s00381-020-04667-7DOI Listing

Reports of a Conus Cauda Tumor with Holocord Syrinx in an Adolescent Girl.

J Pediatr Neurosci 2020 Jan-Mar;15(1):51-53. Epub 2020 Mar 18.

Department of Pediatrics, K. S. Hegde Medical Academy (KSHEMA), Mangaluru, Karnataka, India.

Introduction: Spinal cord ependymoma seldom presents with holocord syringomyelia in pediatric age-group. Association of ependymoma with a lipoma is also rare. The child presented critically ill with polymicrobial pneumonia, and the neurologic findings were missed until recovery. Read More

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http://dx.doi.org/10.4103/JPN.JPN_96_19DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7227757PMC

Communicating Syringomyelia.

World Neurosurg 2020 May 17;140:96-100. Epub 2020 May 17.

Department of Neurosurgery, Osaka General Medical Center, Osaka, Japan.

Background: Communicating syringomyelia can develop in association with hydrocephalus, with communication between syringomyelia and the fourth ventricle a representative neuroimaging finding.

Case Description: A 51-year-old woman presented with slowly progressive bladder dysfunction and scoliosis. She had a nonfunctioning cerebrospinal fluid shunt that had been placed after birth for neonatal hydrocephalus. Read More

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http://dx.doi.org/10.1016/j.wneu.2020.05.073DOI Listing

Additional Assessment of Developed Occipital Sinus Using Intraoperative Indocyanine Green Videoangiography for a Safe Foramen Magnum Decompression-Technical Case Report.

Oper Neurosurg (Hagerstown) 2020 May 18. Epub 2020 May 18.

Department of Neurosurgery, Nara Medical University, Kashihara, Japan.

Background And Importance: Although foramen magnum decompression (FMD) with expansive duraplasty is a popular procedure for treating chiari malformation (CM), the common Y-shaped dural incision can lead to a life-threatening cerebral venous circulation disturbance in patients with a developed occipital sinus. Here, we describe the effectiveness of intraoperative indocyanine green video angiography (ICG-VA) for a CM type 1 (CM1) patient with a highly developed unilateral occipital sinus.

Clinical Presentation: A 40-yr-old woman presented with sensory disturbance on the left side of the body. Read More

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http://dx.doi.org/10.1093/ons/opaa125DOI Listing

Commentary on surgical treatment of scoliosis-associated with syringomyelia: The role of syrinx size.

Neurol India 2020 Mar-Apr;68(2):305-306

Vice-Chair and Director of Complex Spinal Disorders, Professor Department of Neurosurgery, Louisiana State University Health Sciences Center-Shreveport, LA 71101, United States.

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http://dx.doi.org/10.4103/0028-3886.284370DOI Listing

Medical infrared thermal imaging of syringomyelia in the Cavalier King Charles Spaniel.

BMC Vet Res 2020 May 14;16(1):137. Epub 2020 May 14.

The Canine Chiari Institute at Long Island Veterinary Specialists, 163 South Service Road, Plainview, NY, 11803, USA.

Background: Medical infrared thermal imaging (MITI) is a non-invasive imaging modality gaining popularity in the veterinary field. An infrared camera captures emission of heat and creates a color map in the form of a thermogram. Topical heat emission is influenced by localized disease processes as a result of autonomic nervous system imbalance. Read More

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http://dx.doi.org/10.1186/s12917-020-02354-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7227106PMC

Perioperative Halo-Gravity Traction in the Treatment of Scoliosis with Intraspinal Anomalies.

World Neurosurg 2020 May 11. Epub 2020 May 11.

Department of Spinal Surgery, HongHui Hospital, Xi'an Jiaotong University, Xi'an, Shaanxi, China. Electronic address:

Objective: To investigate the efficacy and safety of preoperative halo-gravity traction and 1-stage posterior surgery for the treatment of scoliosis with intramedullary anomalies.

Methods: A total of 11 patients with scoliosis with intramedullary anomalies were evaluated. All patients were treated with preoperative halo-gravity traction and 1-stage posterior surgery. Read More

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http://dx.doi.org/10.1016/j.wneu.2020.04.242DOI Listing

Resection of hip heterotrophic ossification leads to resolution of autonomic nervous system dysfunction in a patient with spinal Charcot arthropathy: a case report.

Spinal Cord Ser Cases 2020 May 13;6(1):41. Epub 2020 May 13.

Surgical Services, VA Palo Alto Health Care System, Palo Alto, CA, USA.

Introduction: Patients with complete spinal cord injury (SCI) may develop concurrent sequalae that interact and share symptoms; thus, a careful approach to diagnosis and management of new symptoms is crucial.

Case Presentation: A patient with prior T4 complete SCI presented with progressive autonomic nervous system (ANS) dysfunction. The initial differential diagnosis included syringomyelia and lumbar Charcot arthropathy. Read More

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http://dx.doi.org/10.1038/s41394-020-0286-5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7220901PMC

Resolution of Tonsillar Herniation and Syringomyelia Following Resection of a Large Anterior Frontal Parasagittal Meningioma.

Cureus 2020 Apr 11;12(4):e7636. Epub 2020 Apr 11.

Neurosurgery, New York University (NYU) School of Medicine, New York, USA.

Chiari I malformation is the herniation of cerebellar tonsils below the level of the foramen magnum due to congenital or acquired pathologies. Acquired Chiari I malformation (ACM) may occur secondary to space-occupying lesions (SOLs), such as intracranial tumors due to elevated intracranial pressure (ICP), and can be accompanied by syringomyelia. ACM and syringomyelia have been shown to resolve after resection of the SOL, without the need for adjuvant posterior fossa decompression. Read More

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http://dx.doi.org/10.7759/cureus.7636DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7213766PMC

The Filum disease and the Neuro-Cranio-vertebral syndrome: definition, clinical picture and imaging features.

BMC Neurol 2020 May 11;20(1):175. Epub 2020 May 11.

Anesthesia Department, CIMA Hospital, Barcelona, Spain.

Background: We propose two new concepts, the Filum Disease (FD) and the Neuro-cranio-vertebral syndrome (NCVS), that group together conditions thus far considered idiopathic, such as Arnold-Chiari Syndrome Type I (ACSI), Idiopathic Syringomyelia (ISM), Idiopathic Scoliosis (IS), Basilar Impression (BI), Platybasia (PTB) Retroflexed Odontoid (RO) and Brainstem Kinking (BSK).

Method: We describe the symptomatology, the clinical course and the neurological signs of the new nosological entities as well as the changes visible on imaging studies in a series of 373 patients.

Results: Our series included 72% women with a mean age of 33. Read More

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http://dx.doi.org/10.1186/s12883-020-01743-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7212596PMC

Rare dorsal thoracic arachnoid web mimics spinal cord herniation on imaging.

Surg Neurol Int 2020 11;11:66. Epub 2020 Apr 11.

Department of Neurosurgery, University of Louisville, Louisville, Kentucky, United States.

Background: Dorsal arachnoid webs (DAWs) are rare clinical entities that can mimic other conditions on magnetic resonance imaging (MRI). Here, we present a case of DAW that was misdiagnosed on MR as a ventral cord herniation.

Case Description: A 35-year-old female presented with a 1-year history of lower extremity weakness and numbness. Read More

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http://dx.doi.org/10.25259/SNI_98_2020DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7193193PMC

A Novel Risk Calculator for Suboccipital Decompression for Adult Chiari Malformation.

World Neurosurg 2020 Apr 29. Epub 2020 Apr 29.

Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, MD. Electronic address:

Background: Patient counselling and selection for surgical therapy in adult Chiari malformation type I (CM-1) remains debatable. We aimed to develop a clinical calculator predicting the risk of non-home discharge and re-operation using the American College of Surgeons - National Surgical Quality Improvement Program (ACS-NSQIP) database.

Methods: The database from years 2011 through 2017 was queried to identify the subset of CM-1 patients undergoing suboccipital decompression. Read More

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http://dx.doi.org/10.1016/j.wneu.2020.04.169DOI Listing

External validation of current prediction systems of improvement after decompression surgery in Chiari malformation type I patients: can we do better?

J Neurosurg 2020 May 1:1-6. Epub 2020 May 1.

1Department of Neurosurgery, Johns Hopkins University School of Medicine.

Objective: The Chiari Severity Index (CSI) and points-based algorithm of Thakar et al. are two prognostic tools that have been developed to predict the likelihood of improvement after suboccipital decompression in adult patients with Chiari malformation type I (CM-I). This study aimed to externally validate and critically evaluate these algorithms in the interest of guiding the development of improved prediction systems. Read More

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http://dx.doi.org/10.3171/2020.2.JNS20181DOI Listing

Syringo-Subarachnoid Shunt Placement: A Minimally Invasive Technique Using Fixed Tubular Retractors-Three Case Reports and Literature Review.

Int J Spine Surg 2020 Apr 30;14(2):133-139. Epub 2020 Apr 30.

Department of Neurosurgery, Aster CMI Hospital, Karnataka, India.

Background: Placement of a syringo-subarachnoid shunt as a surgical management for syringomyelia has been well described in the literature. Good results in terms of clinical and radiological improvement have been documented especially for posttraumatic syringomyelia. Traditionally, this has been performed using open approaches which are fraught with risks of cerebrospinal fluid leak, delayed wound healing, and increased postoperative pain. Read More

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http://dx.doi.org/10.14444/7020DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7188106PMC

Cervical Abnormalities in Severe Spinal Deformity: A 10-year MRI Review.

Orthop Surg 2020 Jun 29;12(3):761-769. Epub 2020 Apr 29.

Department of Orthopaedics, The 2nd Affiliated Hospital of Kunming Medical University, Kunming, China.

Objective: To investigate the incidence of cervical anomalies (CA), including cervical intraspinal neural axis abnormalities (CIINAA) and/or cervical osseous abnormalities (COA), and the clinical relevance in severe spinal deformities (SSD) at a single center.

Methods: A retrospective study of SSD admitted for spinal surgery from January 2003 to January 2015 was conducted at a single center.

Inclusion Criteria: patients who present with coronal Cobb over 90° (and/or sagittal cobb ≥90°); and patients with complete imaging and clinical data preoperatively. Read More

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http://dx.doi.org/10.1111/os.12673DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7307244PMC

Holocord Syrinx Associated with Shunt Malfunction in Tetraventricular Hydrocephalus.

World Neurosurg 2020 Apr 27;139:382-386. Epub 2020 Apr 27.

Department of Neurosciences, Department of Neurosurgery, Santobono-Pausilipon Children's Hospital, Naples, Italy.

We present an uncommon case of holocord, "rosary bead-like," multiloculated syringomyelia consistent with a fourth ventricle outlet obstruction in a 2-month-old infant who was previously shunted for posthemorrhagic hydrocephalus of prematurity. The shunt malfunction resulted in a syringomyelia because of the simultaneous obstruction of the 3 outlets of the fourth ventricle, resulting in an enlargement of the central canal of the spinal cord. The syringomyelia resolved with shunt revision, avoiding further complex surgical procedures. Read More

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http://dx.doi.org/10.1016/j.wneu.2020.04.158DOI Listing

Neuraxial dysraphism in associated syndrome due to improper mesenchymal transition.

Neurol Genet 2020 Jun 1;6(3):e414. Epub 2020 Apr 1.

National Institutes of Health (J.S.R., A.J.C., H.W., Z.Z.), National Cancer Institute Neuro-Oncology Branch; National Institutes of Health (D.P.A., J.D.H.), National Institute of Neurological Disorders and Stroke, Surgical Neurology Branch; National Institutes of Health (Y.P., A.J., K.P.), Eunice Kennedy Shriver National Institute of Child Health and Human Development, Section on Medical Neuroendocrinology; Georgetown Hospital (M.A.N.), Internal Medicine and Pediatrics, Washington DC; National Institutes of Health (J.P.M., D.R.D.), National Institute of Neurological Disorders and Stroke, Mouse Imaging Facility, Bethesda, MD; George Washington University (J.G.S.), Radiology, Washington DC; National Library of Medicine (J.G.S.), MedPix®; National Institutes of Health (M.M.M.), Center for Cancer Research, National Cancer Institute, Laboratory of Pathology; and National Institutes of Health (R.H.K., B.A.K.), National Heart Lung and Blood Institute, Translational Vascular Medicine Branch, Bethesda, MD.

Objective: To investigate the effect of somatic, postzygotic, gain-of-function mutation of Endothelial Per-Arnt-Sim (PAS) domain protein 1 () encoding hypoxia-inducible factor-2α (HIF-2α) on posterior fossa development and spinal dysraphism in gain-of-function syndrome, which consists of multiple paragangliomas, somatostatinoma, and polycythemia.

Methods: Patients referred to our institution for evaluation of new, recurrent, and/or metastatic paragangliomas/pheochromocytoma were confirmed for gain-of-function syndrome by identification of the gain-of-function mutation in resected tumors and/or circulating leukocytes. The posterior fossa, its contents, and the spine were evaluated retrospectively on available MRI and CT images of the head and neck performed for tumor staging and restaging. Read More

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http://dx.doi.org/10.1212/NXG.0000000000000414DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7164966PMC

Syringomyelia in the Tethered Spinal Cords.

J Korean Neurosurg Soc 2020 May 27;63(3):338-341. Epub 2020 Apr 27.

Division of Pediatric Neurosurgery, Seoul National University Children's Hospital, Seoul, Korea.

Cases of syringomyelia associated with spinal dysraphism are distinct from those associated with hindbrain herniation or arachnoiditis in terms of the suspected pathogenetic mechanism. The symptoms of terminal syringomyelia are difficult to differentiate from the symptoms caused by spinal dysraphism. Nonetheless, syringomyelia has important clinical implications, as it is an important sign of cord tethering. Read More

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http://dx.doi.org/10.3340/jkns.2020.0097DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7218206PMC

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

J Neurosurg Pediatr 2020 Apr 24:1-7. Epub 2020 Apr 24.

1Department of Neurosurgery, Vanderbilt University Medical Center.

Objective: The aim of this study was to determine the timeline of syrinx regression and to identify factors mitigating syrinx resolution in pediatric patients with Chiari malformation type I (CM-I) undergoing posterior fossa decompression (PFD).

Methods: The authors conducted a retrospective review of records from pediatric patients (< 18 years old) undergoing PFD for the treatment of CM-I/syringomyelia (SM) between 1998 and 2015. Patient demographic, clinical, radiological, and surgical variables were collected and analyzed. Read More

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http://dx.doi.org/10.3171/2020.2.PEDS19366DOI Listing

Comparison of high-field MRI and multidetector CT for grading Chiari-like malformation and syringomyelia in Cavalier King Charles Spaniels.

Vet Radiol Ultrasound 2020 Apr 24. Epub 2020 Apr 24.

Department of Veterinary Clinical Sciences, College of Veterinary Medicine, The Ohio State University, Columbus, Ohio.

Chiari-like malformation (CM) and syringomyelia (SM) are common illnesses that can cause debilitating neuropathic pain in Cavalier King Charles spaniels (CKCS). The current imaging modality to screen CKCS for CM/SM is MRI of the brain and cervical spine. Magnetic resonance imaging provides good soft tissue detail and contrast of the cerebellum and cervical spinal cord. Read More

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http://dx.doi.org/10.1111/vru.12862DOI Listing

Posterior spinal fusion for severe kyphoscoliosis in a Loeys-Dietz syndrome patient with a large syringomyelia.

J Clin Neurosci 2020 Jun 21;76:211-213. Epub 2020 Apr 21.

Department of Orthopaedic Surgery, Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto, Nagano 390-8621, Japan.

Spinal deformity is a characteristic feature of Loeys-Dietz syndrome (LDS). Surgical correction in LDS is indicated when the deformity is progressive to avoid neurological deficits, respiratory impairment, and back pain. However, few reports exist on the surgical treatment of spinal deformity in LDS, and no therapeutic standards have been established. Read More

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http://dx.doi.org/10.1016/j.jocn.2020.04.017DOI Listing

Continuous Hypodynamic Change of Cerebrospinal Fluid Flow as A Potential Factor Working for Experimental Scoliotic Formation.

Sci Rep 2020 Apr 22;10(1):6821. Epub 2020 Apr 22.

Department of Orthopaedics, The 2nd Affiliated Hospital of Kunming Medical University, Kunming, 6500101, Yunnan, People's Republic of China.

Scoliosis is often associated with syringomyelia (SM). As an important role in SM formation, the influence from abnormal cerebrospinal fluid (CSF) flow is still unclear to scoliosis. The aim of this experimental work is to explore the connection between CSF flow and scoliosis through imaging and histological analysis on the basis of a kaolin-induced scoliotic rabbit model. Read More

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http://dx.doi.org/10.1038/s41598-020-63822-xDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7176657PMC

Surgical outcomes by sectioning a filum terminale in patients with terminal syringomyelia.

Childs Nerv Syst 2020 Apr 21. Epub 2020 Apr 21.

Division of Neurosurgery, National Center for Child Health and Development, Okura 2-10-1, Setagaya-ku, Tokyo, 157-8535, Japan.

Background: Although terminal syringomyelia suggests a tethered spinal cord, a consensus has still not been established for surgical indications, when there are no associated abnormalities or only minor associated abnormalities. We analyzed surgical outcomes of sectioning a filum terminale in patients with terminal syringomyelia to elucidate the significance of untethering surgery.

Methods: Fifty-seven pediatric patients with terminal syringomyelia who underwent untethering via sectioning the filum terminale between 2007 and 2018 were retrospectively analyzed. Read More

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http://dx.doi.org/10.1007/s00381-020-04615-5DOI Listing

Chiari I malformation and pregnancy: a comprehensive review of the literature to address common questions and to guide management.

Acta Neurochir (Wien) 2020 Jul 18;162(7):1565-1573. Epub 2020 Apr 18.

Department of Surgery, Division of Neurosurgery, DeGroote Medical School, McMaster University, Hamilton, ON, Canada.

Background: The optimal management of Chiari I malformation during pregnancy remains uncertain. Labor contractions, which increase intracranial pressure, and neuraxial anesthesia both carry the theoretical risk of brainstem herniation given the altered CSF dynamics inherent to the condition. Mode of delivery and planned anesthesia, therefore, require forethought to avoid potentially life-threatening complications. Read More

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http://dx.doi.org/10.1007/s00701-020-04308-7DOI Listing

Minimally invasive foramen magnum durectomy and obexostomy for treatment of craniocervical junction-related syringomyelia in adults: case series and midterm follow-up.

J Neurosurg Spine 2020 Apr 17:1-10. Epub 2020 Apr 17.

1Department of Neurosurgery, Hospital das Clínicas of University of São Paulo Medical School, São Paulo.

Objective: Craniocervical junction-related syringomyelia (CCJS) is the most common form of syringomyelia. Approximately 30% of patients treated with foramen magnum decompression (FMD) will show persistence, recurrence, or progression of the syrinx. The authors present a pilot study with a new minimally invasive surgery technique targeting the pathophysiology of CCJS in adult patients. Read More

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http://dx.doi.org/10.3171/2020.2.SPINE2032DOI Listing

Posterior Calvarial Augmentation for Chiari Malformation Type 1 Refractory to Foramen Magnum Decompression.

World Neurosurg 2020 Apr 13;139:70-74. Epub 2020 Apr 13.

Department of Neurosurgery, Birmingham Children's Hospital, Birmingham, United Kingdom.

Background: Chiari 1 malformation is a structural abnormality of the hindbrain and posterior fossa characterized by herniation of the cerebellar tonsils through the foramen magnum. Although asymptomatic in some cases, hindbrain herniation can be associated with disruption of cerebrospinal fluid flow dynamics at the craniovertebral junction and syrinx formation, leading to symptoms. Foramen magnum decompression with or without duraplasty has been the most commonly performed surgical procedure in the management of this condition. Read More

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http://dx.doi.org/10.1016/j.wneu.2020.03.218DOI Listing
April 2020
2.417 Impact Factor

Obex position is associated with syringomyelia and use of posterior fossa decompression among patients with Chiari I malformation.

J Neurosurg Pediatr 2020 Apr 10:1-8. Epub 2020 Apr 10.

Departments of1Neurological Surgery.

Objective: Chiari I malformation (CM-I) has traditionally been defined by measuring the position of the cerebellar tonsils relative to the foramen magnum. The relationships of tonsillar position to clinical presentation, syringomyelia, scoliosis, and the use of posterior fossa decompression (PFD) surgery have been studied extensively and yielded inconsistent results. Obex position has been proposed as a useful adjunctive descriptor for CM-I and may be associated with clinical disease severity. Read More

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http://dx.doi.org/10.3171/2020.2.PEDS19486DOI Listing

Radiological and clinical associations with scoliosis outcomes after posterior fossa decompression in patients with Chiari malformation and syrinx from the Park-Reeves Syringomyelia Research Consortium.

J Neurosurg Pediatr 2020 Apr 10:1-7. Epub 2020 Apr 10.

1Department of Neurological Surgery, Washington University School of Medicine, St. Louis, Missouri.

Objective: In patients with Chiari malformation type I (CM-I) and a syrinx who also have scoliosis, clinical and radiological predictors of curve regression after posterior fossa decompression are not well known. Prior reports indicate that age younger than 10 years and a curve magnitude < 35° are favorable predictors of curve regression following surgery. The aim of this study was to determine baseline radiological factors, including craniocervical junction alignment, that might predict curve stability or improvement after posterior fossa decompression. Read More

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http://dx.doi.org/10.3171/2020.1.PEDS18755DOI Listing

Ten-Segment Intramedullary Ependymoma and Whole Spinal Syringomyelia.

World Neurosurg 2020 Apr 3;139:20-22. Epub 2020 Apr 3.

Department of Neurosurgery, Fudan University Huashan Hospital, Shanghai, China. Electronic address:

Background: Ten-segment intramedullary tumors are rare lesions in adults.

Case Description: In this report, we describe the case of a 30-year-old woman who presented with a 2-year history of right lower limb numbness. Spinal magnetic resonance imaging showed an expansive 10-segment intramedullary lesion and syringomyelia. Read More

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http://dx.doi.org/10.1016/j.wneu.2020.03.149DOI Listing