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J Craniofac Surg 2022 Jun 28. Epub 2022 Jun 28.

Oxford Craniofacial Unit, Oxford University Hospitals NHS Foundation Trust, John Radcliffe Hospital.

Abstract: Pathogenic variants of the ERF gene were previously associated with craniosynostosis, craniofacial dysmorphism and Chiari malformation. This study investigates cognitive, behavioural, speech, language, and developmental outcomes in the first 5 children identified at the Oxford Craniofacial Unit as having ERF-related craniosynostosis, together with three of their carrier parents.There were no consistent findings related to overall intelligence. Read More

Neurol Res 2022 Jun 25:1-14. Epub 2022 Jun 25.

Kirikkale University Faculty of Medicine, Department of Neurosurgery, Kirikkale, Turkey.

Background: The first aim of this study was to compare the clinical data and posterior fossa morphometry obtained during the admission to the hospital between control group individuals (who had not Chiari Malformation (CM) type 1) and CM type 1 patients treated surgically or not. The second aim was to create a valid and reliable scale that can predict the decision-making for surgical intervention simply and easily in these patients.

Materials: Medical data and radiological images of 70 CM type 1 patients during their admission to the hospital were compared with the data of 69 control group individuals. Read More

Zh Vopr Neirokhir Im N N Burdenko 2022 ;86(3):92-98

Kazan State Medical University, Kazan, Russia.

Cerebellar tonsil dislocation into foramen magnum exceeding 5 mm is traditionally considered as Chiari malformation type 1. This disease requires surgical treatment in case of severe clinical manifestations. In patients with hypoplasia of posterior cranial fossa (PCF), subthreshold tonsil dislocations or tonsil position inside the foramen magnum can result clinical signs identical to Chiari malformation type 1. Read More

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 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

Orphanet J Rare Dis 2022 06 18;17(1):235. Epub 2022 Jun 18.

Operative Unit of Medical Genetics, Bianchi-Melacrino-Morelli Hospital, V. Melacrino, 89100, Reggio Calabria, Italy.

Background: Malan syndrome (MALNS) is a recently described ultrarare syndrome lacking guidelines for diagnosis, management and monitoring of evolutive complications. Less than 90 patients are reported in the literature and limited clinical information are available to assure a proper health surveillance.

Results: A multidisciplinary team with high expertise in MALNS has been launched at the "Ospedale Pediatrico Bambino Gesù", Rome, Italy. 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

J Neurol Surg A Cent Eur Neurosurg 2022 Jun 15. Epub 2022 Jun 15.

Department of Neurosurgery, Koc University Hospital, Istanbul, Turkey.

Background And Study Aims: The most used surgical procedure in the treatment of patients with Chiari type I malformation (CIM) is posterior fossa decompression. However, no consensus has been reached among surgeons regarding the superior surgical decompression approach between dural splitting and duraplasty. Thus, the aim of this study was to compare clinical and radiological outcomes between the two techniques used in consecutive patients. Read More

Neuroradiology 2022 Jun 14. Epub 2022 Jun 14.

Department of Radiology, Beth Israel Deaconess Medical Center, Harvard Medical School, WCB90, 330 Brookline Ave, Boston, MB, 02215, USA.

Purpose: Some patients with idiopathic intracranial hypertension (IIH) have cerebellar tonsillar herniation ≥ 5 mm mimicking Chiari malformation I (CMI), which can result in misdiagnosis and unjustified treatment. Our purpose was to identify IIH patients with tonsillar herniation ≥ 5 mm (IIH) and compare with CMI patients to assess imaging findings that could distinguish the two conditions.

Methods: Ninety-eight patients with IIH, 81 patients with CMI, and 99 controls were retrospectively assessed. Read More

Optom Vis Sci 2022 Jun 9. Epub 2022 Jun 9.

Illinois College of Optometry, Chicago, Illinois.

Significance: Epidural anesthesia is a safe procedure used in pregnant patients during labor. However, caution should be exercised in those patients that have concurrent Arnold Chiari malformation.

Purpose: To report a rare and atypical presentation of downbeat nystagmus, in a pregnant patient with Arnold Chiari malformation type 1 (ACM1), secondary to accidental dural puncture. 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

Surg Neurol Int 2022 20;13:205. Epub 2022 May 20.

Department of Neurosurgery, NHS, Liverpool, Merseyside, United Kingdom.

Background: We describe a case of long-standing intracranial hypotension caused by an iatrogenic arachnoid diverticulum. This case illustrates two learning points. First, excessive CSF absorption may occur through an acquired arachnoid-epidural venous plexus at a dural defect. Read More

Neurologia (Engl Ed) 2022 Jun 26;37(5):325-333. Epub 2021 May 26.

Departamento de Pediatría, Radiología y Medicina Física, Facultad de Medicina de Zaragoza, Universidad de Zaragoza, Zaragoza, Spain.

Introduction: Neurofibromatosis type 1 (NF1) is a progressive multisystem disorder following an autosomal dominant inheritance pattern that presents with multiple neurological manifestations.

Methods: We reviewed medical histories of patients with NF1 followed up at our hospital's paediatric neurology department from May 1990 to 31 December 2018. We collected data on neurological symptoms. Read More

Plast Reconstr Surg 2022 Jun 8. Epub 2022 Jun 8.

Department of Clinical Research, Medical City Dallas Hospital, Dallas, TX.

Background: Numerous children born with syndromic craniosynostosis will develop visual impairments. Based on the hypothesis that elevations in intracranial pressure might have greater impacts on vision than development, this review sought to ascertain the prevalence of optic nerve atrophy in syndromic craniosynostosis and to look for potential predictive factors.

Methods: A retrospective chart review of all children with syndromic craniosynostosis treated at a single center. 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

J Clin Sleep Med 2022 May 31. Epub 2022 May 31.

Institute of Neurology, Fondazione Policlinico Universitario "Agostino Gemelli", IRCCS, Università Cattolica del Sacro Cuore, Rome, Italy.

Study Objectives: The main aim was to evaluate the prevalence of sleep-disordered breathing (SDB) in patients with Chiari II malformation (CM-II). The secondary objectives were to evaluate the association between SDB, morphological abnormalities and neurological symptoms and to review the literature of patients with SDB and CM-II.

Methods: The study has a cross-sectional, case-control design. 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

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

J Neurosurg 2022 Apr 29:1-6. Epub 2022 Apr 29.

Objective: There is currently a lack of consensus on the utility of intraoperative neuromonitoring (IONM) for decompression of Chiari type I malformation (CM-I). Commonly used monitoring modalities include somatosensory evoked potentials (SSEPs), motor evoked potentials (MEPs), and brainstem auditory evoked potentials (BAEPs). The purpose of this study was to evaluate the utility of IONM in preventing neurological injury for CM-I decompression. 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

Ann Plast Surg 2022 05;88(3 Suppl 3):S201-S204

From the New York Presbyterian Hospital-Weill Cornell Medical Center, New York, NY.

Introduction: Patients with Ehlers-Danlos syndrome (EDS) are at elevated risk for soft tissue complications when undergoing decompression with or without fusion of the craniocervical junction. We have previously shown that muscle flap closure can decrease reoperative rates. This study investigated whether myofascial flap closure improved clinical outcomes after simple or complex surgery of the craniocervical junction in EDS patients specifically. Read More

J Clin Neurosci 2022 Jul 2;101:21-25. Epub 2022 May 2.

Department of Neurosurgery, Clinical Neuroscience Center, University of Utah, 175 N. Medical Drive East, Salt Lake City, UT 84132, USA; Department of Biomedical Engineering, University of Utah, 36 S. Wasatch Drive, Salt Lake City, UT 84112, USA. Electronic address:

Hemifacial spasm (HFS) can be associated with Chiari malformation type I (CM1), but the treatment paradigm for these concurrent conditions has not been well-defined. We sought demographical differences between patients with HFS with and without CM1 and explored optimal surgical treatments for these patients. A systematic review of peer-reviewed literature identified 8 studies with 51 patients with CM1 and HFS. Read More

Br J Neurosurg 2022 May 5:1-2. Epub 2022 May 5.

Department of Neurosurgery, General Hospital of Western Theater Command, Chengdu, China.

We report a fairly rare case of postoperative acute subdural hygromas without concurrent hydrocephalus complicating foramen magnum decompression (FMD) for Chiari malformation type I (CM-I). Full resolution of subdural hygromas was achieved through therapeutic lumbar puncture releasing bloody cerebrospinal fluid which has been scarcely advocated in managing post-FMD subdural hygromas. This outcome suggests that bloody cerebrospinal fluid may be related to the formation of subdural hygromas after FMD for CM-I and therapeutic lumbar puncture may provide an effective treatment modality in such conditions. Read More

Int J Pediatr Otorhinolaryngol 2022 Jul 26;158:111159. Epub 2022 Apr 26.

Otolaryngology, CHI Crumlin Hospital Dublin, Ireland.

Introduction: Bilateral vocal cord immobility (BVCI) is a leading cause of stridor and airway obstruction in neonates which may arise idiopathically, as a result of birth trauma, or in the setting of Central nervous system lesions such as Arnold-Chiari malformation. Although many children with BVCI may be managed conservatively, surgical intervention may be necessary in those patients with ongoing upper airway obstruction. Many interventions have been described including, tracheostomy, cordotomy, cricoid splitting procedures, and arytenoidectomy. Read More