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Neurochirurgie 2022 Jun 22. Epub 2022 Jun 22.

Departments of Radiology, Faculty of Medicine, Bezmialem Vakif University, Istanbul, Turkey.

Background: Chiari type 1 malformation (CMI) is a disorder in which cerebellar tonsils descend below the foramen magnum. Although syringomyelia associated with CMI thought to be caused by hypoplastic posterior fossa and stenosis at the craniocervical junction; it has characteristic neurological and radiological features and the exact mechanism of syringomyelia remains unknown.

Purpose: The purposes of this study were to gain insight into morphological changes in posterior fossa and to find whether there is a difference in aqueductal stroke volume (ASV) between CMI with syrinx and without syrinx which may be an underlying mechanism of syrinx development. Read More

J Pers Med 2022 Jun 17;12(6). Epub 2022 Jun 17.

Department of Physical Medicine and Rehabilitation, Miller School of Medicine, University of Miami, Miami, FL 33136, USA.

Pediatric spina bifida (SB) and spinal cord injury (SCI) are unfortunately common in our society, and their unique findings and comorbidities warrant special consideration. This manuscript will discuss the epidemiology, pathophysiology, prevention, and management strategies for children growing and developing with these unique neuromuscular disorders. Growth and development of the maturing child places them at high risk of spinal cord tethering, syringomyelia, ascending paralysis, pressure injuries, and orthopedic abnormalities that must be addressed frequently and judiciously. Read More

J Clin Med 2022 Jun 10;11(12). Epub 2022 Jun 10.

Department of Neurosurgery, Bicêtre Hospital, AP-HP, University Paris-Saclay, 94270 Le Kremlin-Bicêtre, France.

Revision surgery after posterior fossa decompression for Chiari malformation is not uncommon and poses both strategic and technical challenges. We conducted a single-center retrospective cohort study including all adult patients who underwent revision surgery after posterior fossa decompression for Chiari type I malformation between 2010 and 2019. Among 311 consecutive patients operated on for Chiari malformation at our institution, 35 patients had a least one revision surgery with a mean follow-up of 70. Read More

Front Surg 2022 30;9:771896. Epub 2022 May 30.

Department of Hand Surgery, China-Japan Union Hospital of Jilin University, Changchun, China.

Background: Previous reports on the treatment of neuropathic arthropathy of the wrist were generally conservative, with few case reports of treatment with osteoarticular surgery.

Case Presentation: A 25-year-old right-handed male complained of unpainful swelling of the dorsal aspect of his right wrist for 3 years. He was at that time diagnosed with synovitis and radiocarpal arthritis. Read More

Orthop Surg 2022 Jun 16. Epub 2022 Jun 16.

Division of Spine Surgery, Department of Orthopaedic Surgery, Nanjing Drum Tower Hospital, The Affiliated Hospital of Nanjing University Medical School, Nanjing, China.

Objective: To analyze the intraoperative neurophysiological monitoring (IONM) data of patients with intraspinal abnormalities undergoing posterior spinal fusion and to determine how intraspinal abnormalities influence IONM results.

Methods: Patients with severe kyphoscoliosis and intraspinal abnormalities who underwent posterior spinal correction and fusion between September 2015 and January 2019 were retrospectively reviewed. Candidate intraspinal abnormalities included Chiari malformation, syringomyelia, split cord malformation, and tethered cord syndrome. Read More

Neurochirurgie 2022 Jun 10. Epub 2022 Jun 10.

Department of neurosurgery, Lariboisière hospital, 2, rue Ambroise Paré, 75010 Paris Cedex 10, France.

A 36 year old woman was referred to our department for symptomatic lumbar spinal arachnoiditis following an epidural anaesthesia for childbirth. She did not had other known causative factor and she was free of any neurological symptoms before. She rapidly developed lower limbs impairment by compressing intradural lumbar collections and arachnoiditis requiring surgical decompression and subsequently internal cerebrospinal fluid shunting for acute hydrocephalus. Read More

Surg Neurol Int 2022 20;13:217. Epub 2022 May 20.

Department of Radiology, Bumrungrad International Hospital, Bangkok, Thailand.

Background: Chiari malformation Type I (CMI) is generally considered a congenital lesion and typically associated with syringomyelia. Acquired CMI or adult Chiari malformation caused by intracranial mass is extremely rare. Brain arteriovenous malformations (AVMs) are characteristically symptomatic due to seizure, intracranial hemorrhage, or neurological deficit. Read More

Surg Neurol Int 2022 20;13:211. Epub 2022 May 20.

Department of Neurosurgery, University Hospitals Leuven, Leuven, Belgium.

Background: Megalencephaly-capillary malformation (M-CM) syndrome is a rare overgrowth syndrome characterized by macrocephaly, port-wine stains, asymmetric brain growth, hydrocephalus, and developmental delay. Cerebellar tonsil herniation is often seen, but rarely with syringomyelia.

Case Description: A newborn with M-CM syndrome developed a progressive Chiari malformation type I (CM-I) with syringomyelia. Read More

World J Stem Cells 2022 Apr;14(4):303-309

Stem Cell Treatment and Research Institute (STRI), bio Beauty and Health Company (bBHC), Seoul 04420, South Korea.

Background: Syringomyelia is a disease caused by the formation of a cavity inside the spinal cord and is accompanied by such symptoms as pain, paresthesia, and urination and defecation disorders, and in severe cases causes various paralyses. Currently, there are only surgical methods for the treatment of syringomyelia, but these methods carry the possibility of failure, recurrence, and side effects.

Case Summary: The patient was a 59-year-old woman who suffered from pain due to syringomyelia. Read More

Turk Neurosurg 2022 Jan 10. Epub 2022 Jan 10.

xuanwu hospital.

Aim: Some meta-analyses have focused on foramen magnum decompression with duraplasty (PFDD) and without duraplasty (PFD) in paediatric or mixed populations. Nevertheless, no meta-analysis has evaluated adults only. This study aimed to include new relevant findings in a systematic review to provide the first comparison of PFDD and PFD in adult CM-I. Read More

Turk J Pediatr 2022 ;64(2):385-388

Departments of Pathology, İstanbul University Cerrahpaşa Faculty of Medicine, İstanbul, Turkey.

Background: Curarino syndrome is a rare and complex anomaly with the triad of anorectal malformation, presacral mass and sacral bone deformation. The most common cause of the presacral mass is meningioma, but teratoma is the diagnosis in about one-third of the cases. Malignant transformation of teratoma in the form of carcinoma, rhabdomyosarcoma and leukemia have previously been reported on rare occasions. Read More

Paediatr Anaesth 2022 May 23. Epub 2022 May 23.

Department of Pediatric Anesthesiology, Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA.

Introduction: Posterior fossa decompression for Chiari I Malformation is a common pediatric neurosurgical procedure. We sought to identify the impact of anesthesia-related intraoperative complications on unanticipated admission to the intensive care unit and outcomes following posterior fossa decompression.

Methods: Medical records of all patients <18 years who underwent surgery for Chiari I malformation between 1/1/09 and 1/31/21 at the Ann & Robert H. Read More

World Neurosurg 2022 May 17. Epub 2022 May 17.

Department of Neurosurgery, Nanfang Hospital, Southern Medical University, Guangzhou, China; Nanfang Neurology Research Institution, Nanfang Hospital, Southern Medical University, Guangzhou, China; Nanfang Glioma Center, Guangzhou, China. Electronic address:

Background: Treatment of Chiari malformation (CM) is controversial, especially when it coexists with "stable" or Type II basilar invagination (CM + II-BI). Precise evaluation of craniovertebral junction (CVJ) stability is crucial in such patients; however, this has never been validated. This study aimed to dynamically evaluate atlanto-condyle and atlantoaxial stability by kinematic computed tomography (CT) and report its surgical treatment. Read More

Childs Nerv Syst 2022 May 19. Epub 2022 May 19.

Department of Neurologic Surgery, Mayo Clinic, Rochester, MN, USA.

Objective: Chiari I malformation is treated with suboccipital craniectomy with cervical laminectomy, a procedure which has been associated with the possibility of pre-existing or iatrogenic occipitocervical instability. The long-term risk of subsequent spinal deformity and need for occipito cervical fusion after standard Chiari decompression in pediatric patients has not yet been characterized.

Methods: We queried our institutional electronic database for patients aged 18 and under, with at least 5 years of follow-up, that underwent surgical decompression for Chiari I malformation. Read More

Childs Nerv Syst 2022 May 16. Epub 2022 May 16.

Department of Pediatric Neurosurgery, Great Ormond Street Hospital, Great Ormond Street, London, WC1N 3JH, UK.

Introduction: Atlantoaxial rotatory fixation (AARF) is a rare condition that occurs most commonly in children. The torticollis caused by AARF usually presents as abnormal posturing of the head and neck, with rotation of the chin to the opposite side. AARF in children could occur due to congenital bony malformation, minor trauma, upper respiratory tract infections (Grisel's syndrome), postoperatively after head and neck (ENT) surgery, and unknown reasons. Read More

Front Surg 2022 28;9:850879. Epub 2022 Mar 28.

Henan International Joint Laboratory of Nervous System Malformation, Zhengzhou, China.

Diagnosis of Chirai malformation type I (CM-I) is based on magnetic resonance imaging of the brain or cervical spinal cord. The main goal of surgery is to relieve the blockage to the free pulsatile flow of cerebrospinal fluid beyond the foramen magnum and to stop the progression of a syringomyelia. Despite recent advances in imaging and surgery, even today, there is no consensus on optimal management of CM-I. Read More

BMJ Open 2022 05 12;12(5):e056647. Epub 2022 May 12.

EA 3450 DevAH - Development, Adaptation and Handicap, Faculty of Medicine, University of Lorraine, Nancy, France.

Introduction: Chiari I malformation (CM1) is an anatomical abnormality characterised by the cerebellar tonsils descending at least 5 mm below the foramen magnum. CM1 causes obstruction of cerebrospinal fluid (CSF) circulation as well as direct compression on the brainstem, thus causing typical consequences (syringomyelia), and typical clinical features (characteristic headaches and neurological impairment). Surgery is the only available treatment, indicated when symptomatology is present. Read More

World Neurosurg 2022 May 5. Epub 2022 May 5.

Department of Pathology, The First Affiliated Hospital of Nanchang University, Nanchang, China; Institute of Molecular Pathology, Nanchang University, Nanchang, China.

Objective: Suboccipital decompression with duraplasty is being increasingly accepted for treating patients with Chiari malformation type I (CM-1). To date, the optimal duraplasty for CM-I has not been delineated. This study aims to compare the clinical and radiologic effects of duraplasty performed using 2 types of grafts and 2 graft fixation methods in 3 combinations. Read More

Neurochirurgie 2022 May 5. Epub 2022 May 5.

Department of Neurosurgery, BHT Clinic Istanbul TEMA Hospital, İstanbul, Turkey.

Objective: The current study aimed to assess the effects of tonsillar herniation on cervical alignment in Chiari I patients without syringomyelia using new cervical sagittal alignment parameters, such as C0-2 Cobb angle, C2-7 cobb angle, T1 slope, and C2-7 sagittal vertical axis (SVA).

Methods: Two spinal surgeons independently evaluated midline T2-weighted sagittal magnetic resonance imaging findings of 28 Chiari I patients without syringomyelia and 40 patients without tonsillar herniation but with similar complaints. Thereafter, the measured C0-2 Cobb angle, C2-7 Cobb angle T1 slope, and C2-7 SVA were compared using the t-test. Read More

Pan Afr Med J 2022 17;41:143. Epub 2022 Feb 17.

Sree Balaji Dental College and Hospital, Chennai, Tamil Nadu, India.

Neurol Clin 2022 05 31;40(2):297-307. Epub 2022 Mar 31.

National Institutes of Health, National Institute of Neurological Disorders and Stroke, Surgical Neurology Branch, 10 Center Drive, Room 3D20, Bethesda, MD, 20892 USA. Electronic address:

Chiari Malformation Type I (CMI) is a congenital malformation diagnosed by MRI findings of at least 5 mm of cerebellar ectopy below the foramen magnum. CM1 is frequently associated with syringomyelia. Herein, we discuss the history of CMI and syringomyelia, including early pathological and surgical studies. Read More

J Korean Neurosurg Soc 2022 May 25;65(3):430-438. Epub 2022 Apr 25.

Department of Neurosurgery, The First Medical Centre, Chinese PLA General Hospital, Beijing, China.

Objective: Dermoid cysts are uncommon in spinal cord tumors, and the phenomenon of their spontaneous rupture into the syrinx cavity is quite rare. We aimed to analyze the imaging characteristics and etiologies, and propose some surgical strategies, for this uncommon phenomenon.

Methods: We retrospectively reviewed 14 cases with spinal dermoid cysts that ruptured into the cervical and thoracic syrinx cavity. Read More

J Biomech 2022 May 12;137:111093. Epub 2022 Apr 12.

Neuroscience Research Australia and Prince of Wales Clinical School, University of New South Wales, Australia. Electronic address:

Chiari malformation is characterised by the herniation of the cerebellar tonsils through the foramen magnum. However, tonsillar herniation and other 2D morphometric measurements of the posterior cranial fossa (PCF) have a weak association with patients' symptoms and clinical outcomes. This study aimed to contrast current 2D metrics with a novel 3D shape analysis of the PCF and the hindbrain, to determine if 3D measurements provides further insight into the pathophysiology of Chiari. Read More

J Neurosurg Pediatr 2022 Apr 15:1-13. Epub 2022 Apr 15.

1Division of Pediatric Neurosurgery, Penn State Health Children's Hospital, Hershey, PA.

Objective: The aim of this study was to determine differences in complications and outcomes between posterior fossa decompression with duraplasty (PFDD) and without duraplasty (PFD) for the treatment of pediatric Chiari malformation type I (CM1) and syringomyelia (SM).

Methods: The authors used retrospective and prospective components of the Park-Reeves Syringomyelia Research Consortium database to identify pediatric patients with CM1-SM who received PFD or PFDD and had at least 1 year of follow-up data. Preoperative, treatment, and postoperative characteristics were recorded and compared between groups. Read More

World Neurosurg 2022 Jul 6;163:24. Epub 2022 Apr 6.

Department of Neurosurgery, Brigham and Women's Hospital, Harvard Medical School, Boston. Electronic address:

Syringomyelia is often resistant to various treatment modalities. Chiari I malformations are associated with syringomyelia in approximately 69% of operative cases. Failure to resolve syringomyelia after Chiari decompression is common. Read More

Neurospine 2022 Mar 31;19(1):108-117. Epub 2022 Mar 31.

Department of Neurosurgery, University Hospital of Bordeaux, Bordeaux, France.

Objective: Spinal cord tumors constitute a small part of spinal surgery owing to their rarity. This retrospective study describes their current management.

Methods: Forty-eight patients were treated for an intramedullary tumor between 2014 and 2020 at a single institution. Read More

Pain 2022 Mar 29. Epub 2022 Mar 29.

Core Center for Molecular Morphology, Section for Stereology and Microscopy, Aarhus University, Aarhus, Denmark.

Abstract: Central neuropathic pain is a core clinical sign of syringomyelia in humans and Cavalier King Charles Spaniel (CKCS) dogs. This histopathological study used spinal cords from CKCS dogs with syringomyelia to investigate the following conditions: (1) whether specific structural cervical spinal cord entities involved in nociception were affected by loss of neuroparenchyma or other pathological changes in CKCS dogs with pain-related behaviour and phantom scratching, (2) whether pain-related behaviour or phantom scratching correlated with loss of a specific anatomical entity or upregulation of glia cells, and (3) whether syringomyelia-related lesions affected specific functional spinal cord units of nociception. Spinal cord segments C1-C8 from CKCS dogs with magnetic resonance imaging-confirmed syringomyelia and clinical signs of pain and phantom scratching (n = 8) were compared with those from CKCS dogs without syringomyelia (n = 4). Read More

Ann Neurosci 2021 Jul 8;28(3-4):170-178. Epub 2021 Oct 8.

Department of Homeopathy, University of the Aegean, Mytilene, Greece.

Syringomyelia (SM) with Chiari malformation is a rare disease with an unpredictable course. Surgery and other interventions help reduce the severity of symptoms, but over 50% patients require re-operation. Auto-resolution is rare in this type of SM, and most cases progress to complications, which may amount to a great burden. Read More

Clin Neurol Neurosurg 2022 Mar 19;216:107214. Epub 2022 Mar 19.

Clinical Surgical Research Center, Faculty of Medicine, Chiang Mai University, Chiang Mai 50200, Thailand; Neurosurgery Unit, Department of Surgery, Faculty of Medicine, Chiang Mai University, Chiang Mai 50200, Thailand. Electronic address:

Objective: A significant number of patients with Chiari type 1 malformation (CM1) have abnormal clivo-axial angle (CXA) without other radiographic indicators of basilar invagination or craniovertebral junction (CVJ) instability. This study aimed to investigate whether abnormal CXA alone influences postoperative outcomes among patients who underwent foramen magnum decompression (FMD).

Methods: A total of 44 adult patients with symptomatic CM1 undergoing FMD without CVJ fixation were enrolled. Read More