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JBJS Case Connect 2021 Jun 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

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

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

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

World Neurosurg 2021 May 27. Epub 2021 May 27.

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

Objective: Single-stage spinal correction without previous neurosurgical intervention has been attempted in scoliotic patients associated with syringomyelia (SM). But there is still lack of evidence to demonstrate its potential influences to associated SM from direct spinal correction. The aim of this study is to explore the role of spinal-shortening in the prognosis of SM-associated scoliosis after single-stage spinal correction without any previous neurosurgical intervention. 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

BMJ Case Rep 2021 May 24;14(5). Epub 2021 May 24.

Radiology, LTMMC & GH, Mumbai, India.

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 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

Neurosurg Rev 2021 May 14. Epub 2021 May 14.

Department of Neurosurgery, Peking University Third Hospital, Peking University, Beijing, China.

Multiple factors, such as tumor size, lateralization, tumor location, accompanying syringomyelia, and regional spinal cord atrophy, may affect the resectability and clinical prognosis of intramedullary spinal cord ependymomas. However, whether long-segmental involvement of the spinal cord may impair functional outcomes remains unclear. This study was aimed to compare perioperative neurological functions and long-term surgical outcomes between multisegmental ependymomas and their monosegmental counterparts. 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

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

JBJS Case Connect 2021 03 5;11(1). Epub 2021 Mar 5.

Department of Orthopaedics, T.N.M.C. and B.Y.L. Nair Ch. Hospital, Mumbai, Maharashtra, India.

Case: The authors present a case of syringobulbia in a setting of elbow arthropathy due to syringomyelia. The patient had painless elbow instability with subtle neurological findings such as ulnar neuropathy, palatal palsy, and dysphonia. As she denied surgery, she was managed with physiotherapy and orthosis. Read More

Proc (Bayl Univ Med Cent) 2021 Jan 22;34(3):397-398. Epub 2021 Jan 22.

Department of Internal Medicine-Pediatrics, Wayne State University/Detroit Medical Center, Detroit, Michigan.

A patient with a syringopleural shunt with dyspnea and cough was found to have a recurrent large pleural effusion. Computed tomography of the thorax revealed the syringopleural shunt catheter abutting the pleural effusion, and beta-2 transferrin was detected in the pleural fluid, suggesting that the recurrent symptomatic pleural effusion was secondary to the syringopleural shunt. Physicians should be cognizant of this potential long-term complication of syringopleural shunts. Read More

World Neurosurg 2021 Apr 30. Epub 2021 Apr 30.

Birmingham Women's and Children's Hospital NHS Foundation Trust, Birmingham, United Kingdom.

Background: The best surgical treatment for adult Chiari malformation type 1 remains widely debated.

Objective: This study aimed to assess the efficacy of posterior fossa decompression and duraplasty with arachnoid preservation compared with arachnoid dissection for the treatment of adult Chiari malformation type 1.

Methods: Two reviewers (M. Read More

J Vet Intern Med 2021 May 3. Epub 2021 May 3.

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

Background: Persistent fontanelles (PFs) are, in Chihuahuas, almost ubiquitous. Furthermore, Chihuahuas are predisposed to other craniomorphological abnormalities, including syringomyelia (SM), ventriculomegaly, and craniocervical junction (CCJ) overcrowding resulting in neural tissue deviation. It is, however, undetermined if PFs are more common in dogs with these structural abnormalities, and their etiology is unknown. Read More

Acta Neurochir (Wien) 2021 06 21;163(6):1593-1601. Epub 2021 Apr 21.

Department of Neurosurgery, The Jikei University School of Medicine, 3-25-8 Nishishinbashi, Minato-ku, Tokyo, 105-8461, Japan.

Background: It is important to distinguish foramen magnum arachnoiditis (FMA) from Chiari malformation (CM) before surgery because the operative strategies for these diseases differ. In the current study, we compared pretreatment magnetic resonance imaging (MRI) of FMA with CM and investigated the MRI findings useful to differentiate between these diseases.

Methods: We retrospectively reviewed patients with FMA or CM aged ≥ 18 years who underwent surgeries at our institution between 2007 and 2019. Read More

J Clin Neurosci 2021 May 5;87:20-25. Epub 2021 Mar 5.

Division of Neurosciences Critical Care, Department of Neurology, Neurosurgery, Anesthesiology and Critical Care Medicine and Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, MD, United States. Electronic address:

Determinants of tuberculosis (TB) syringomyelia, its management options and outcomes are still under investigation. The aim of this study is to present a case of TB syringomyelia with markedly improved symptoms status-post surgery and to understand the clinical characteristics and outcomes of 33 TB syringomyelia cases reported in the literature. Specifically, we examined the differences between patients who were managed medically and those who underwent surgical intervention. Read More

J Craniovertebr Junction Spine 2021 Jan-Mar;12(1):99-101. Epub 2021 Mar 4.

Department of Neurosurgery, K.E.M. Hospital and Seth G.S. Medical College, Mumbai, Maharashtra, India.

A 16-year-old male presented with primary complaint of worsening dorsal spinal kyphoscoliosis (SKS) for 3 years. More recently, he developed spasticity in legs, breathlessness on mild exertion, and sleep apneas. Apart from SKS, investigations revealed rotatory atlantoaxial dislocation. Read More

J Craniovertebr Junction Spine 2021 Jan-Mar;12(1):15-25. Epub 2021 Mar 4.

Department of Neurological Surgery, University of Illinois at Chicago, Chicago, IL, USA.

Background: Pathophysiological mechanisms underlying the syringomyelia associated with Chiari I malformation (CM-1) are still not completely understood, and reliable predictors of the outcome of posterior fossa decompression (PFD) are lacking accordingly. The reported prospective case-series study aimed to prove the existence of a pulsatile, biphasic systolic-diastolic cerebrospinal fluid (CSF) dynamics inside the syrinx associated with CM-1 and to assess its predictive value of patients' outcome after PFD. Insights into the syringogenesis are also reported. Read More

Global Spine J 2021 Apr 13:21925682211009175. Epub 2021 Apr 13.

Department of Neurosurgery, 104607PLA General Hospital, Haidian District, Beijing, China.

Study Design: Retrospective cohort study.

Objectives: We aimed to evaluate the effectiveness of terminal ventriculostomy in treating tethered cord syndrome (TCS) combined with terminal syringomyelia (TS) and describe "V"-type ostomy as an effective surgical method to avoid relapsing syringomyelia based on terminal ventriculostomy.

Methods: We retrospectively analyzed the clinical and radiological data of 28 patients admitted to the Department of Neurosurgery, PLA General Hospital who had been diagnosed with TCS combined with TS and underwent terminal ventriculostomy-associated "V"-type ostomy between January 2011 and January 2016. Read More

Eur Spine J 2021 Apr 10. Epub 2021 Apr 10.

Department of Neurosurgery, Division of Spine, China, International Neurological Institute, Xuanwu Hospital, Capital Medical University, 45 Changchun Street, Beijing, 100053, People's Republic of China.

J Neurosurg 2021 Apr 9:1-14. Epub 2021 Apr 9.

Departments of1Neurological Surgery and.

Objective: Posterior fossa decompression with duraplasty (PFDD) is often used for Chiari malformation type I (CM-I), but outcomes associated with different dural graft materials are not well characterized. In this meta-analysis, the authors examined complication rates and outcomes after PFDD for CM-I for autografts and four types of nonautologous grafts.

Methods: A literature search of numerous electronic databases (Ovid Medline, Embase, Scopus, Cochrane Database of Systematic Reviews, Cochrane Central Register of Controlled Trials, Database of Abstracts of Reviews of Effects, Health Technology Assessment Database, NHS Economic Evaluation Database, and ClinicalTrials. Read More

Neural Regen Res 2021 Nov;16(11):2293-2298

Department of Chemical, Biomolecular, and Corrosion Engineering, Integrated Bioscience Program, The University of Akron, Akron, OH, USA.

Precise assessment of spinal cord cystic lesions is crucial to formulate effective therapeutic strategies, yet histological assessment of the lesion remains the primary method despite numerous studies showing inconsistent results regarding estimation of lesion size via histology. On the other hand, despite numerous advances in micro-computed tomography (micro-CT) imaging and analysis that have allowed precise measurements of lesion size, there is not enough published data on its application to estimate intraspinal lesion size in laboratory animal models. This work attempts to show that micro-CT can be valuable for spinal cord injury research by demonstrating accurate estimation of syrinx size and compares between micro-CT and traditional histological analysis. Read More

BMC Pregnancy Childbirth 2021 Apr 1;21(1):272. Epub 2021 Apr 1.

Department of Anesthesiology, The Obstetrics and Gynecology Hospital of Fudan University, Shanghai, China.

Background: The preferable choice of anesthesia for the patients with congenital atlantoaxial dislocation (CAAD) and type I Arnold Chiari malformations (ACM-I) has been a very confusing issue in clinical practice. We describe the successful administration of combined spinal-epidural anesthesia for a woman with CAAD and ACM-1 accompanied by syringomyelia.

Case Presentation: Our case report presents the successful management of a challenging obstetric patient with CAAD and ACM-1 accompanied by syringomyelia. Read More

Clin Case Rep 2021 Mar 2;9(3):1571-1576. Epub 2021 Feb 2.

Normal Physiology Department Kazan State Medical University Kazan Russia.

A comprehensive work-up, clinical correlation, and differential diagnosis are needed to determine if abnormal findings such us hydromyelia in ALS patients are causative or incidental in order to rule out other, more curable conditions that resemble ALS. Read More

Medicine (Baltimore) 2021 Mar;100(12):e25202

Department of Orthopedics, The Second Affiliated Hospital of Bengbu Medical College, Bengbu.

Abstract: To introduce a novel technique of using individualized 3D printing occipitocervical fusion instrument (3D-OCF) for the treatment of upper cervical deformity with atlantoaxial joint dislocation.The surgery for deformity of the craniocervical junction area is a challenge in the field of spine. If the surgical deviation is too large to injure the spinal cord or vertebral artery, it will cause catastrophic damage to the patient. Read More