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Neurol Sci 2021 Jun 15. Epub 2021 Jun 15.

Department of Neurosurgery, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy.

Background: Syringomyelia and Chiari malformation are classified as rare diseases on Orphanet, but international guidelines on diagnostic criteria and case definition are missing.

Aim Of The Study: to reach a consensus among international experts on controversial issues in diagnosis and treatment of Chiari 1 malformation and syringomyelia in adults.

Methods: A multidisciplinary panel of the Chiari and Syringomyelia Consortium (4 neurosurgeons, 2 neurologists, 1 neuroradiologist, 1 pediatric neurologist) appointed an international Jury of experts to elaborate a consensus document. Read More

Prenat Diagn 2021 Jun 14. Epub 2021 Jun 14.

Department of Radiology, Division of Neuro- and Musculoskeletal Radiology, Medical University of Vienna, Vienna, Austria.

Objective: This systematic comparison between pre- and postnatal imaging findings and postnatal motor outcome assesses the reliability of MRI accuracy in the prognostication of the future long-term (mean, 11.4 years) ambulatory status in a historic group of postnatally repaired myelomeningocele (MMC) cases.

Methods: A retrospective, single-center study of 34 postnatally repaired MMC patients was performed. Read More

J Matern Fetal Neonatal Med 2021 Jun 13:1-6. Epub 2021 Jun 13.

Department of Neonatology, Dr Ram Manohar Lohia Hospital and Post Graduate Institute of Medical Education and Research, New Delhi, India, Atal Bihari Vajpayi Institute of Medical Sciences (ABVIMS) and Dr Ram Manohar Lohia Hospital, New Delhi, India.

Introduction: Neural tube defects constitute a major source of disability among children. Proper management requires accurate diagnosis, an assessment of the severity of the lesion, a decision whether intervention is warranted, the nature of the intervention, and educating the family of the need for lifelong medical care. But to do so, reliable data regarding presentation and outcome is very crucial. Read More

JBJS Case Connect 2021 06 11;11(2). Epub 2021 Jun 11.

Department of Orthopedic Surgery, Keio University School of Medicine, Tokyo, Japan.

Case: A 22-year-old skeletally mature man with scoliosis accompanied by syringomyelia associated with Chiari malformation type 1 was presented to our hospital. Because the maximal syrinx/cord ratio was small, and he had no neurological deficit, he underwent corrective surgery without the treatment of the syringomyelia.

Conclusion: The scoliosis was corrected without any neurological complications. Read More

JAMA Otolaryngol Head Neck Surg 2021 Jun 10. Epub 2021 Jun 10.

School of Communication Sciences and Disorders, McGill University, Montreal, Canada.

Importance: Vocal fold paralysis (VFP) results from the disruption of neural motor outputs to laryngeal muscles. Children with VFP manifest various degrees of difficulties in phonation, breathing, and swallowing. Although the etiologic characteristics and symptoms of VFP are well established in adults, corresponding clinical profiles are notably different in children. Read More

Cerebellum 2021 Jun 9. Epub 2021 Jun 9.

Department of Psychology, The University of Akron, Akron, OH, 44325-4301, USA.

Chiari malformation type I (CMI) is a neural disorder with sensory, cognitive, and motor defects, as well as headaches. Radiologically, the cerebellar tonsils extend below the foramen magnum. To date, the relationships among adult age, brain morphometry, surgical status, and symptom severity in CMI are unknown. Read More

J Neurol Surg A Cent Eur Neurosurg 2021 Jun 7. Epub 2021 Jun 7.

Department of Neurosurgery, AZ Delta, Roeselare, Belgium.

Introduction:  Chiari type I malformations can present in different ways, but the most frequent symptom is an occipitocervical headache. Hearing loss as the main presenting symptom is rare.

Case:  A young woman with progressive left-sided unilateral hearing loss was diagnosed with a Chiari type I malformation. Read More

World Neurosurg 2021 Jun 4. Epub 2021 Jun 4.

Department of Neurosurgery, Wroclaw Medical University, Borowska 213, 50-556 Wroclaw, Poland.

Background: This study aimed to compare the effectiveness of posterior fossa decompression (PFD), posterior fossa decompression with duraplasty (PFDD), and posterior fossa decompression with resection of tonsils (PFDRT) in Chiari malformation type I (CMI). Furthermore, we aimed to evaluate the evidence supporting the efficacy of filum terminale sectioning (FTS), syringo-subarachnoid shunting (SSS), and syringo-peritoneal shunting (SPS) in CMI.

Methods: PubMed, Cochrane, and Embase databases were screened for English-language studies published from inception until 11 of August 2020. Read More

Neurol Sci 2021 Jun 7. Epub 2021 Jun 7.

Neurosurgery and Pediatric Neurosurgery, Vall d'Hebron Hospital Universitari, Neurotrauma and Neurosurgery Research Unit, and Universitat Autònoma de Barcelona, Barcelona, Spain.

Background: Chiari malformation type 1 (CM1) is a rare condition where agreed classification and treatment are still missing. The goal of this study is to achieve a consensus on the diagnosis and treatment of CM1 in children.

Methods: A multidisciplinary panel formulated 57 provisional statements based on a review of the literature. Read More

Neurosurgery 2021 Jun 5. Epub 2021 Jun 5.

Department of Neurosurgery, Stanford University, Stanford, California, USA.

Background: There is a growing body of evidence suggesting not all craniotomy patients require postoperative intensive care.

Objective: To devise and implement a standardized protocol for craniotomy patients eligible to transition directly from the operating room to the ward-the Non-Intensive CarE (NICE) protocol.

Methods: We preoperatively identified patients undergoing elective craniotomy for simple neurosurgical procedures with age <65 yr and American Society of Anesthesiologists (ASA) class of 1, 2 or 3. Read More

J Neurosurg Pediatr 2021 Jun 4:1-7. Epub 2021 Jun 4.

1Division of Pediatric Neurosurgery, Primary Children's Hospital, University of Utah, Salt Lake City, Utah.

Objective: The condylar-C2 sagittal vertical alignment (C-C2SVA) describes the relationship between the occipitoatlantal joint and C2 in patients with Chiari malformation type I (CM-I). It has been suggested that a C-C2SVA ≥ 5 mm is predictive of the need for occipitocervical fusion (OCF) or ventral brainstem decompression (VBD). The authors' objective was to validate the predictive utility of the C-C2SVA by using a large, multicenter cohort of patients. Read More

J Curr Ophthalmol 2021 Jan-Mar;33(1):88-90. Epub 2021 Mar 26.

Ophthalmic Epidemiology Research Center, Research Institute for Ophthalmology and Vision Science, Shahid Beheshti University of Medical Sciences, Tehran, Iran.

Purpose: To report a case of progressive Chiari malformation type I (CIM) in a patient with unilateral sporadic retinoblastoma (RB) treated with intra-arterial chemotherapy (IAC) and enucleation.

Methods: A 5-year-old male patient with a history of RB in his left eye treated with IAC and enucleation presented to our clinic for routine RB surveillance. Radiotherapy had not been used for the treatment of his RB. Read More

J Sleep Res 2021 Jun 2:e13388. Epub 2021 Jun 2.

Assistance Publique-Hôpitaux de Paris (AP-HP), Pediatric Noninvasive Ventilation and Sleep Unit, Hôpital Necker-Enfants Malades, Paris, France.

Central sleep apneas and periodic breathing are poorly described in childhood. The aim of the study was to describe the prevalence and characteristics of central sleep apnea and periodic breathing in children with associated medical conditions, and the therapeutic management. We retrospectively reviewed all poly(somno)graphies with a central apnea index ≥ 5 events per hr in children aged > 1 month performed in a paediatric sleep laboratory over a 6-year period. Read More

Neurol Res 2021 May 30:1-7. Epub 2021 May 30.

Medeniyet University Goztepe Education and Research Hospital, Department of Neurosurgery, Istanbul, Turkey.

: Chiari malformations (CMs) are a heterogeneous group of disorders defined by anatomic anomalies of the cerebellum, brainstem, and craniovertebral junction (CVJ). The aims of this study are to establish the demographic and clinical features, incidence, surgical procedures, and outcomes in large series of old and new subtypes of CMs.: All patients were evaluated and operated on for CM-0, 1, and 1. Read More

Clin Neurol Neurosurg 2021 Jul 15;206:106686. Epub 2021 May 15.

Department of Neurosurgery, Cerrahpasa Medical Faculty, Istanbul University - Cerrahpasa, Istanbul, Turkey.

Object: Optimal surgical technique to restore the cerebrospinal fluid flow through the foramen magnum remains to be debated in Chiari malformation type 1 (CM-1) patients.

Method: This study included 46 patients with CM-1 who underwent surgical treatment by one of two methods: posterior fossa bone decompression (BD) with arachnoid preserving duraplasty (Group 1) and BD with duraplasty and arachnoid dissection (Group 2). Complaints of the patient population and neurological findings were assessed with Neck Disability Index (NDI) and Europe Quality of Life 5 Dimensions (EQ-5D) in pre- and postoperative periods. Read More

Acta Neurol Belg 2021 May 28. Epub 2021 May 28.

Centro Regionale Esperto Siringomielia e Sindrome di Chiari (CRESSC), Department of Neuroscience, AOU Città della Salute e della Scienza di Torino, Presidio CTO, Via Zuretti, 29, 10126, Turin, Italy.

In Chiari 1 Malformation (CM1) the most frequent symptom is exertional headache, but other headache types have been reported, such as migraine. This cross-sectional study is aimed to examine the prevalence of migraine in a group of CM1 headache patients and to compare clinical-demographic characteristics between migraine and non-migraine patients. 427 adults were enrolled at the multidisciplinary Chiari Center in Torino. Read More

BMJ Case Rep 2021 May 25;14(5). Epub 2021 May 25.

Paediatrics and Child Health, Stellenbosch University Faculty of Medicine and Health Sciences, Cape Town, Western Cape, South Africa.

Syringomyelia associated with tuberculous meningitis (TBM) is an extremely rare condition. Only a few adult cases have been reported. A 12-year-old woman, who previously suffered TBM at the age of 6 months, presented with a long-standing history of right upper limb panhypertrophy, dissociate anaesthesia, frequent headaches, scoliosis and acquired macrocephaly. Read More

J Vet Intern Med 2021 May 24. Epub 2021 May 24.

Department of Equine and Small Animal Medicine, Faculty of Veterinary Medicine, University of Helsinki, Helsinki, Finland.

Background: The Chihuahua dog breed is known for frequent occurrence of a bregmatic fontanelle on the dorsal skull. A common conception is that this skull defect is a clinically irrelevant finding. No studies, however, describe its prevalence or whether it is accompanied by other persistent fontanelles (PFs). Read More

J Neurol Surg B Skull Base 2021 Jun 30;82(3):277-284. Epub 2019 Sep 30.

Osaka City University Graduate School of Medicine, Neurosurgery, Osaka, Japan.

 We investigated the mechanism of ptosis of the brain stem and cerebellum (hindbrain) in Chiari malformation type I (CM-I) and classified CM-I according to pathogenesis, based on a morphometric study of the posterior cranial fossa (PCF) and craniovertebral junction (CVJ). We discuss the appropriate surgical treatment for hindbrain ptosis.  We examined 500 patients with CM-I and 100 healthy control individuals. Read More

World Neurosurg 2021 May 20;151:201-208. Epub 2021 May 20.

Division of Neurosurgery, Faculty of Medicine of the Holy House of Mercy of São Paulo, São Paulo, Brazil.

Atlas assimilation (AA) may be associated with atlantoaxial dislocation, Chiari malformation (CM), and basilar invagination. The importance of AA in the context of craniocervical junction (CVJ) anomalies is unclear. Considering this context, this study's objective is to discuss the role of AA in the management of CVJ anomalies, especially in CM. Read More

J Neurosurg Spine 2021 May 21:1-7. Epub 2021 May 21.

4Radiology, Rhode Island Hospital, The Warren Alpert Medical School of Brown University, Providence, Rhode Island; and.

Objective: The craniocervical junction (CCJ) is anatomically complex and comprises multiple joints that allow for wide head and neck movements. The thecal sac must adjust to such movements. Accordingly, the thecal sac is not rigidly attached to the bony spinal canal but instead tethered by fibrous suspension ligaments, including myodural bridges (MDBs). Read More

J Neurosurg Pediatr 2021 May 21:1-6. Epub 2021 May 21.

2Division of Neurosurgery, Children's National Health System, Washington, DC.

Objective: The authors aimed to describe the natural history and optimal management of persistent syringomyelia after suboccipital craniectomy for Chiari malformation type I (CM-I).

Methods: A cohort of all patients who presented to a tertiary pediatric hospital with newly diagnosed CM-I between 2009 and 2017 was identified. Patients with persistent or worsened syringomyelia were identified on the basis of a retrospective review of medical records and imaging studies. Read More

Pediatr Neurosurg 2021 May 17:1-6. Epub 2021 May 17.

Carolina Neurosurgery & Spine Associates, Charlotte, North Carolina, USA.

Purpose: In patients who present with headaches and Chiari 1 malformation without cranial nerve or brainstem dysfunction or syrinx formation, the decision to decompress can be difficult to make. We present a case series examining the use of acetazolamide as a diagnostic aid to determine candidacy for decompression.

Methods: A single pediatric neurosurgeon's (M. Read More

Heliyon 2021 Apr 19;7(4):e06795. Epub 2021 Apr 19.

Department of Anesthesia, Stanford University, Palo Alto, California, USA.

Cerebellar tonsillar descent can be seen on head magnetic resonance imaging in both Chiari malformation type 1 and spinal cerebrospinal fluid leak creating the potential for misdiagnosis. We report five cases of spinal cerebrospinal fluid leak at Stanford University initially misdiagnosed and treated as Chiari malformation type 1 based on cerebellar tonsillar descent demonstrated on imaging. All five cases had sustained relief at the 6-month follow up visit from epidural blood patches for the treatment of spinal cerebrospinal leak after unsuccessful suboccipital decompression surgeries. Read More

Turk Neurosurg 2020 Dec 15. Epub 2020 Dec 15.

Afyonkarahisar Health Sciences University, Faculty of Medicine, Department of Anatomy, Afyonkarahisar, Turkey.

Aim: Chiari malformations are defined as a spectrum of abnormalities of the posterior brain, including the cerebellum, brainstem, skull base, and cervical cord. Although many theories have tried to explain the etiology of Chiari malformation, its cause is still unknown. This study aimed to examine foramen magnum morphometry and shape of Chiari malformation in comparison with a control group and to contribute to the literature on whether Chiari malformation affects foramen magnum morphometry. Read More

Turk Neurosurg 2020 Dec 14. Epub 2020 Dec 14.

Uludag University Faculty of Medicine, Department of Biostatistics, Bursa, Turkey.

Aim: When cerebellar tonsils are placed ≥ 5 mm below the foramen magnum, the situation is referred to as Chiari malformation Type I (CM-I). The prevalence of CM-I has been reported as 0.6%-1%. Read More

Acta Neurochir (Wien) 2021 Jul 11;163(7):2005-2014. Epub 2021 May 11.

Department of Diagnostic Radiology, Oulu University Hospital, Oulu and Research Unit of Medical Imaging, Physics, and Technology, Oulu University Hospital and University of Oulu, Kajaanintie 50, OYS, P.O. Box 50, 90029, Oulu, Finland.

Background: The purpose of our study was to research the parameters of magnetic resonance imaging (MRI) that would predict the outcome of surgery in patients with Chiari 1 malformation (CM1) and to evaluate changes in MRI parameters after surgery.

Methods: Fifty-one patients (19 children, 13 adolescents, and 19 adults) operated on due to CM1 in Oulu University Hospital between 2004 and 2018 were evaluated. Seventeen parameters were measured from the preoperative MRI and 11 from the postoperative MRI. Read More

J Gen Fam Med 2021 May 25;22(3):158-159. Epub 2021 Jan 25.

Department of Community Medicine Management Faculty of Medicine Shimane University Izumo Japan.

Chiari type 1 deformation can show progressive degenerative neurological presentation in the elderly. Read More

Pediatr Neurosurg 2021 May 11:1-6. Epub 2021 May 11.

Division of Neurosurgery, Department of Neurosurgery, Children's Hospital of Philadelphia, University of Pennsylvania, Perelman School of Medicine, Philadelphia, Pennsylvania, USA.

Background: Myoclonus is an involuntary movement disorder characterized by semirhythmic jerking movements of muscle groups but is rarely seen in association with Chiari malformation type I (CM-1). CM-1 is a frequently encountered clinical entity in pediatric neurosurgery characterized by caudal displacement of the cerebellar tonsils with or without syringomyelia. We report a pediatric patient who presented with upper extremity myoclonus and was found to have CM-1 and a complex septated cervicothoracic syrinx eccentric to the left. Read More

PLoS One 2021 11;16(5):e0251289. Epub 2021 May 11.

Duke Molecular Physiology Institute, Duke University Medical Center, Durham, NC, United States of America.

Chiari Malformation Type 1 (CM-1) is characterized by herniation of the cerebellar tonsils below the foramen magnum and the presence of headaches and other neurologic symptoms. Cranial bone constriction is suspected to be the most common biologic mechanism leading to CM-1. However, other mechanisms may also contribute, particularly in the presence of connective tissue disorders (CTDs), such as Ehlers Danlos Syndrome (EDS). Read More