4,396 results match your criteria Chiari Malformation


Cerebellar tonsil ectopia measurement in type I Chiari malformation patients show poor inter-operator reliability.

Fluids Barriers CNS 2018 Dec 17;15(1):33. Epub 2018 Dec 17.

Department of Biological Engineering, University of Idaho, 875 Perimeter Drive MS 0904, Moscow, ID, 83844-0904, USA.

Background: Type 1 Chiari malformation (CM-I) has been historically defined by cerebellar tonsillar position (TP) greater than 3-5 mm below the foramen magnum (FM). Often, the radiographic findings are highly variable, which may influence the clinical course and patient outcome. In this study, we evaluate the inter-operator reliability (reproducibility) of MRI-based measurement of TP in CM-I patients and healthy controls. Read More

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http://dx.doi.org/10.1186/s12987-018-0118-1DOI Listing
December 2018

Treatment failure of syringomyelia associated with Chiari I malformation following foramen magnum decompression: how should we proceed?

Neurosurg Rev 2018 Dec 15. Epub 2018 Dec 15.

Departments of Neurosurgery and Pediatric Neurosurgery, Tel-Aviv Medical Center and Dana Children's Hospital Tel Aviv, Tel Aviv University, 6 Weizmann Street, Tel Aviv, Israel.

The preferred treatment of patients with persistent, recurrent, or progressive syringomyelia after foramen magnum decompression (FMD) for Chiari I (CMI)-associated syringomyelia is controversial, and may include redo FMD, stabilization, or shunting procedures (such as syringopleural or syringo-subarachnoid shunts). We describe our experience in treating these patients and discuss the treatment modalities for these patients. We retrospectively collected data of CMI patients with persistent, recurrent, or progressive syringomyelia after FMD. Read More

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http://dx.doi.org/10.1007/s10143-018-01066-0DOI Listing
December 2018

Selective thoracic fusion for adolescent thoracic scoliosis secondary to Chiari I malformation: a comparison between the left and the right curves.

Eur Spine J 2018 Dec 14. Epub 2018 Dec 14.

Department of Spine Surgery, The Affiliated Drum Tower Clinical Medical College of Nanjing Medical University, Nanjing, China.

Purpose: The aim of this study was to compare the clinical outcomes of selective thoracic fusion in the surgical treatment of Chiari malformation type I (CMI) adolescents with different curve patterns.

Methods: Sixty-three CMI patients with left thoracic curve (LTC) and 63 age- and curve-magnitude-matched CMI patients with right thoracic curve (RTC) were recruited. Selective thoracic fusion was performed for two groups. Read More

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http://dx.doi.org/10.1007/s00586-018-5855-yDOI Listing
December 2018
2.473 Impact Factor

Clinical Application of Diagnostic Imaging of Chiari-Like Malformation and Syringomyelia.

Front Vet Sci 2018 28;5:280. Epub 2018 Nov 28.

School of Veterinary Medicine, Faculty of Health & Medical Sciences, University of Surrey, Guildford, United Kingdom.

Chiari-like malformation (CM) and syringomyelia (SM) is a frequent diagnosis in predisposed brachycephalic toy breeds since increased availability of MRI. However, the relevance of that MRI diagnosis has been questioned as CM, defined as identification of a cerebellar herniation, is ubiquitous in some breeds and SM can be asymptomatic. This article reviews the current knowledge of neuroanatomical changes in symptomatic CM and SM and diagnostic imaging modalities used for the clinical diagnosis of CM-pain or myelopathy related to SM. Read More

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https://www.frontiersin.org/article/10.3389/fvets.2018.00280
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http://dx.doi.org/10.3389/fvets.2018.00280DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6279941PMC
November 2018
9 Reads

Tonsillar herniation as a complication of lumboperitoneal shunt: case report and literature review.

Br J Neurosurg 2018 Dec 6:1-4. Epub 2018 Dec 6.

a Department of Neurosurgery, An Nan Hospital , China Medical University , Tainan , Taiwan.

Tonsillar herniation is a rare and seldom reported complication after lumboperitoneal (LP) shunting. There have been only few reports that have presented possible options for treatment with varying degrees of success. In this report, we describe a rare case of tonsillar herniation after LP shunting and review related literature. Read More

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http://dx.doi.org/10.1080/02688697.2018.1538481DOI Listing
December 2018

Kyphectomy in neonates with meningomyelocele.

Childs Nerv Syst 2018 Dec 11. Epub 2018 Dec 11.

School of Medicine, Department of Neonatology, Celal Bayar University, Manisa, Turkey.

Purpose: Kyphosis is the most severe spinal deformity associated with meningomyelocele (MMC) and is seen in approximately 15% of neonates. Our purpose is to present our clinical experience, to discuss the technique and deformity correction in kyphectomy in neonates with MMC, and to assess its long-term outcomes.

Method: In this prospective study, the authors reviewed eight cases submitted to surgery between 2013 and 2015. Read More

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http://link.springer.com/10.1007/s00381-018-4006-4
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http://dx.doi.org/10.1007/s00381-018-4006-4DOI Listing
December 2018
1 Read

Outcomes and resource utilization in surgery for Chiari I malformation in a national network of children's hospitals.

Childs Nerv Syst 2018 Dec 10. Epub 2018 Dec 10.

Department of Neurosurgery, Baylor College of Medicine, Division of Pediatric Neurosurgery, Texas Children's Hospital, Houston, TX, USA.

Introduction: Chiari malformation type 1 (CM-1) is a common congenital or acquired malformation of the posterior fossa. We aimed to characterize preoperative risk factors, perioperative complications, and postoperative outcomes related to CM-1 surgery in pediatric populations across a nationwide network of pediatric hospitals in the United States (US).

Methods: The Children's Hospital Association's Pediatric Health Information System (PHIS) database was used to examine patients < 21 years old in the US-based nationwide database who underwent inpatient surgery for CM-1 from 2007 to 2015. Read More

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http://dx.doi.org/10.1007/s00381-018-4012-6DOI Listing
December 2018

Quantification of Cerebellar Crowding in Type I Chiari Malformation.

Ann Biomed Eng 2018 Dec 7. Epub 2018 Dec 7.

Department of Mechanical Engineering, The University of Akron, Akron, OH, 44325-3903, USA.

This study was focused on a semi-automated morphometric analysis of the cerebellum in the mid-sagittal plane as an alternative to tonsillar descent alone in the evaluation of Chiari malformation type 1 (CMI) patients. Morphometric analyses of posterior fossa structures were performed on mid-sagittal MRI images of 375 individuals (females, > 18 years, 235 CMI and 140 healthy controls). Twenty-six parameters including linear, angular and area measurements together with non-dimensional ratios were calculated. Read More

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http://dx.doi.org/10.1007/s10439-018-02175-zDOI Listing
December 2018

Dynamic Cerebellar Tonsils in Chiari Malformation.

J Pediatr 2018 Dec 7. Epub 2018 Dec 7.

College of Medicine Medical University of South Carolina Charleston, South Carolina.

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http://dx.doi.org/10.1016/j.jpeds.2018.11.020DOI Listing
December 2018

Clinical spectrum of PTEN mutation in pediatric patients. A bicenter experience.

Eur J Med Genet 2018 Dec 4. Epub 2018 Dec 4.

Pediatric Highly Intensive Care Unit, Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.

Objective Of The Study: To give a full overview of the clinical presentation of PTEN mutations in pediatric patients and to propose a pediatric follow-up protocol.

Methods: Recruitment of 16 PTEN mutated children (age 6 months-11 years) from two pediatric centers in Milan (Italy) between 2006 and 2017. All the patients underwent clinical and neurologic evaluations, cognitive and behavioral tests, and brain MRI; they are currently following an oncologic follow-up. Read More

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http://dx.doi.org/10.1016/j.ejmg.2018.12.001DOI Listing
December 2018
2 Reads

Intracranial hypertension after Chiari decompression resolving after removal of a levonorgestrel-releasing intrauterine device: case report.

Authors:

J Neurosurg 2018 Oct 1:1-4. Epub 2018 Oct 1.

Levonorgestrel-releasing intrauterine devices (LIUDs) are thought to release this progestin locally in the uterus to limit side effects. Authors here present a case of treatment-refractory hydrocephalus and pseudomeningocele (PMC), both of which fully resolved after LIUD removal.A 35-year-old woman with an implanted LIUD developed symptomatic PMC and hydrocephalus after suboccipital craniectomy for Chiari malformation type I. Read More

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http://dx.doi.org/10.3171/2018.5.JNS18315DOI Listing
October 2018
1 Read

A familial syndrome of hypothalamic hamartomas, polydactyly, and SMO mutations: a clinical report of 2 cases.

Authors:

J Neurosurg Pediatr 2018 Oct 1:1-6. Epub 2018 Oct 1.

Hypothalamic hamartomas are benign tumors known to cause gelastic or dacrystic seizures, precocious puberty, developmental delay, and medically refractory epilepsy. These tumors are most often sporadic but rarely can be associated with Pallister-Hall syndrome, an autosomal dominant familial syndrome caused by truncation of glioblastoma transcription factor 3, a downstream effector in the sonic hedgehog pathway. In this clinical report, the authors describe two brothers with a different familial syndrome. Read More

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http://dx.doi.org/10.3171/2018.7.PEDS18292DOI Listing
October 2018
1 Read

Fourth ventricle stent placement for treatment of recurrent syringomyelia in patients with type I Chiari malformations.

Authors:

J Neurosurg Pediatr 2018 Oct 1:1-7. Epub 2018 Oct 1.

OBJECTIVEIn patients with syringomyelia and type I Chiari malformation (CM-I) who have required reoperation because of persistent, recurrent, or expanding syrinx, the senior author placed a stent from the fourth ventricle to the cervical subarachnoid space in hopes of promoting circulation of CSF out of the ventricle and away from the central canal of the spinal cord. This study was undertaken to determine the long-term success of this operative stratagem in eliminating the syrinx, as well as to document the complications that occurred following stent placement. The technique utilized for placement of fourth ventricle stents is presented. Read More

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http://dx.doi.org/10.3171/2018.7.PEDS18312DOI Listing
October 2018
1 Read

Multisegmental Lumbar Corporectomy and Transcorporal Fixation for Correction of Extreme Thoracolumbar Kyphosis in Myelomeningocele with Chronic Decubitus.

Pediatr Neurosurg 2018 Nov 27:1-5. Epub 2018 Nov 27.

Department of Neurosurgery, Hannover Medical School, Hannover, Germany.

We introduce a novel technique for the treatment of severe kyphosis in myelomeningocele. A 5-year-old paraplegic boy with myelomeningocele presented with severe thoracolumbar kyphosis and a chronic ulcus at the site of the gibbus. The myelomeningocele had been treated during his first week of life, and an accompanying Chiari type II malformation had been treated by ventriculoperitoneal shunting. Read More

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http://dx.doi.org/10.1159/000494564DOI Listing
November 2018
5 Reads

Sequential Neuroimaging of the Fetus and Newborn With In Utero Zika Virus Exposure.

JAMA Pediatr 2018 Nov 26. Epub 2018 Nov 26.

Division of Fetal and Transitional Medicine, Children's National Health System, Washington, DC.

Importance: The evolution of fetal brain injury by Zika virus (ZIKV) infection is not well described.

Objectives: To perform longitudinal neuroimaging of fetuses and infants exposed to in utero maternal ZIKV infection using concomitant magnetic resonance imaging (MRI) and ultrasonography (US), as well as to determine the duration of viremia in pregnant women with ZIKV infection and whether the duration of viremia correlated with fetal and/or infant brain abnormalities.

Design, Setting, And Participants: A cohort of 82 pregnant women with clinical criteria for probable ZIKV infection in Barranquilla, Colombia, and Washington, DC, were enrolled from June 15, 2016, through June 27, 2017, with Colombian women identified by community recruitment and physician referral and travel-related cases of American women recruited from a Congenital Zika Program. Read More

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http://archpedi.jamanetwork.com/article.aspx?doi=10.1001/jam
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http://dx.doi.org/10.1001/jamapediatrics.2018.4138DOI Listing
November 2018
4 Reads

First-in-Man Clinical Experience Using a High-Definition 3-Dimensional Exoscope System for Microneurosurgery.

Oper Neurosurg (Hagerstown) 2018 Nov 26. Epub 2018 Nov 26.

Department of Neurosurgery, Lenox Hill Hospital, Zucker School of Medicine at Hofstra/Northwell, New York, New York.

Background: During its development and preclinical assessment, a novel, 3-dimensional (3D), high-definition (4K-HD) exoscope system was formerly shown to provide an immersive surgical experience, while maintaining a portable, low-profile design.

Objective: To assess the clinical applicability of this 3D 4K-HD exoscope via first-in-man surgical use.

Methods: The operative workflow, functionality, and visual haptics of the 3D 4K-HD exoscope were assessed in a variety of microneurosurgical cases at 2 US centers. Read More

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https://academic.oup.com/ons/advance-article/doi/10.1093/ons
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http://dx.doi.org/10.1093/ons/opy320DOI Listing
November 2018
7 Reads

Secondary sacral tethered cord syndrome after treatment of secondary cervical syringomyelia.

J Clin Neurosci 2018 Nov 20. Epub 2018 Nov 20.

Department of Neurosurgery, HELIOS Klinikum, Erfurt, Germany.

A female patient operated at the age of 11 on a pilocytic astrocytoma in the posterior fossa, was re-operated for a recurrence 9 years later. A Torkildsen drain was placed in the 4th ventricle and she remained asymptomatic for 15 years before presenting again with acute hydrocephalus, tonsillar herniation, and a massive cervicothoracic syrinx. The symptoms retreated following craniocervical untethering and decompression. Read More

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http://dx.doi.org/10.1016/j.jocn.2018.11.010DOI Listing
November 2018
1 Read

Swallowing Dysfunction in Adult Patients with Chiari I Malformation.

J Neurol Surg B Skull Base 2018 Dec 25;79(6):606-613. Epub 2018 May 25.

Department of Neurosurgery, Sahlgrenska University Hospital and Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden.

 Swallowing difficulties have been reported in patients with Chiari I malformation (CMI) with a prevalence of 4 to 47%, but existing evidence is based only on case reports. We aimed to prospectively study swallowing function in adult patients with CMI before and 3 months after surgical decompression.  We included all adult patients diagnosed with CMI from September 2015 to October 2017 who underwent a planned surgery at Sahlgrenska University Hospital, Sweden. Read More

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http://dx.doi.org/10.1055/s-0038-1655758DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6239883PMC
December 2018
5 Reads

Choosing Wisely Canada: Pediatric Neurosurgery Recommendations.

Paediatr Child Health 2018 Sep 1;23(6):383-387. Epub 2018 Mar 1.

Division of Neurosurgery, Hospital For Sick Children, Toronto, Ontario.

Objectives: Choosing Wisely Canada is an evidence-based, patient-focused, physician-led campaign to improve the delivery of medical care in Canada. The goal of this study was to produce Canadian recommendations for physicians treating patients with selected paediatric neurosurgery issues.

Methods: Paediatric neurosurgeons practicing in Canada were invited to participate. Read More

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https://academic.oup.com/pch/article/23/6/383/4916924
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http://dx.doi.org/10.1093/pch/pxy012DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6234424PMC
September 2018
5 Reads

Comparison decompression by duraplasty or cerebellar tonsillectomy for Chiari malformation-I complicated with syringomyelia.

Clin Neurol Neurosurg 2018 Nov 10;176:1-7. Epub 2018 Nov 10.

Department of Neurosurgery, The First Affiliated Hospital of Zhengzhou University, China. Electronic address:

Objective: The current study aimed to assess the two surgical procedures of posterior fossa decompression (PDF) in treating Chiari malformation type I (CM-1) complicated by syringomyelia (SM), and to evaluate the postoperative complications, surgical effects and prognosis.

Patients And Methods: A retrospective study was performed on 115 adult CM-I patients undergoing surgical treatment from November 2013 to November 2016 in a single comprehensive hospital. These patients underwent the surgical procedure of either posterior fossa decompression with duraplasty (PFDD) or posterior fossa decompression combined with the resection of tonsils (PFDRT) by five experienced neurosurgeon in a single center. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S03038467183043
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http://dx.doi.org/10.1016/j.clineuro.2018.11.008DOI Listing
November 2018
6 Reads

A fetal case of microphthalmia and limb anomalies with abnormal neuronal migration associated with SMOC1 biallelic variants.

Eur J Med Genet 2018 Nov 13. Epub 2018 Nov 13.

University of Torino, Department of Medical Sciences, 10126, Torino, Italy.

Microphthalmia with limb anomalies (MLA, OMIM, 206920) is a rare autosomal-recessive disease caused by biallelic pathogenic variants in the SMOC1 gene. It is characterized by ocular disorders (microphtalmia or anophtalmia) and limb anomalies (oligodactyly, syndactyly, and synostosis of the 4th and 5th metacarpals), variably associated with long bone hypoplasia, horseshoe kidney, venous anomalies, vertebral anomalies, developmental delay, and intellectual disability. Here, we report the case of a woman who interrupted her pregnancy after ultrasound scans revealed a depression of the frontal bone, posterior fossa anomalies, cerebral ventricular enlargement, cleft spine involving the sacral and lower-lumbar vertebrae, and bilateral microphthalmia. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S17697212183039
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http://dx.doi.org/10.1016/j.ejmg.2018.11.012DOI Listing
November 2018
6 Reads

Basilar impression in osteogenesis imperfecta treated with staged halo traction and posterior decompression with short-segment fusion.

J Craniovertebr Junction Spine 2018 Jul-Sep;9(3):212-215

Department of Orthopaedics, Nemours/Alfred I. Dupont Hospital for Children, Wilmington, Delaware, USA.

Basilar impression is a cranial base abnormality associated with osteogenesis imperfecta (OI) with serious neurologic implications but controversial treatment options. Combined anterior and posterior decompression with long-segment posterior fusion is often recommended. We report a patient with OI (Sillence type III) with basilar impression treated with halo traction followed by posterior surgery. Read More

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http://www.jcvjs.com/text.asp?2018/9/3/212/242819
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http://dx.doi.org/10.4103/jcvjs.JCVJS_63_18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6187899PMC
November 2018
2 Reads

Anesthetic management of parturients with Arnold Chiari malformation-I: a multicenter retrospective study.

Int J Obstet Anesth 2018 Oct 10. Epub 2018 Oct 10.

Department of Anesthesiology, Duke University Hospital, Durham, NC, United States.

Background: Consensus regarding the safest mode of delivery and anesthetic management for parturients with Arnold Chiari malformation-I (ACM-I) remains controversial. This study assessed their anesthetic management and reported anesthetic complications during hospitalization for delivery.

Methods: This was a multicenter, retrospective, cohort study of patients with ACM-I undergoing vaginal or cesarean delivery. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S0959289X183027
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http://dx.doi.org/10.1016/j.ijoa.2018.10.002DOI Listing
October 2018
7 Reads

Comparison between decompressed and non-decompressed Chiari Malformation type I patients: A neuropsychological study.

Neuropsychologia 2018 Dec 6;121:135-143. Epub 2018 Nov 6.

Neuro-e-Motion Research Team, Faculty of Psychology and Education, University of Deusto, Av. Universidades, 24, 48007 Bilbao, Spain. Electronic address:

Background: Previous studies have suggested an association of Chiari Malformation type I (CM-I) and cognitive deficits. CM-I is a neurological disorder characterized by a descent of cerebellar tonsils into the foramen magnum, resulting in overcrowding of the upper cervical spine region. Posterior fossa decompression (PFD) is the surgical treatment of choice, however, the literature on the consequences for patients is mainly reduced to the assessment of physical symptoms. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00283932183050
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http://dx.doi.org/10.1016/j.neuropsychologia.2018.11.002DOI Listing
December 2018
2 Reads

Headache, Chiari I malformation and foramen magnum decompression.

Curr Opin Pediatr 2018 Dec;30(6):786-790

Department of Neurosurgery, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts, USA.

Purpose Of Review: With increasing use of MRI, more patients are being diagnosed with Chiari I malformation (CM1), many of whom are asymptomatic. When symptoms are present, headache is the most frequent presenting feature, although symptoms can be variable. The purpose of this review is to help primary care clinicians better understand the relationship between an individual's presenting symptoms and the presence of radiographic CM1, and to describe the expected outcomes for patients who undergo decompression surgery. Read More

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http://dx.doi.org/10.1097/MOP.0000000000000679DOI Listing
December 2018
5 Reads
2.530 Impact Factor

Bilambdoid and sagittal synostosis: Report of 39 cases.

Surg Neurol Int 2018 11;9:206. Epub 2018 Oct 11.

Craniofacial Unit, Department of Pediatric Neurosurgery, Hôpital Necker-Enfants Malades, Paris, France.

Background: Bilambdoid and sagittal synostosis (BLSS), also called "Mercedes Benz synostosis," is a multisutural craniosynostosis that has been described as a specific entity. However, this synostotic pattern can also be found in syndromic craniostenosis. To better define this entity we reviewed our experience with bilambdoid and sagittal synostosis. Read More

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http://dx.doi.org/10.4103/sni.sni_454_17DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6194734PMC
October 2018
8 Reads

[Cervico-occipital junction malformation and vitiligo].

Pan Afr Med J 2018 20;30:146. Epub 2018 Jun 20.

Departement of Dermatology, Military Hospital My Ismail, Meknes, Morocco.

Vitiligo is a relatively common multifactorial polygenetic dermatosis (0.5%-2% of general population) characterized by segmental or nonsegmental cutaneous depigmentation. Chiari malformation is a congenital cervico-occipital junction disease characterized by a migration of a part of the cerebellum through the foramen magnum. Read More

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http://dx.doi.org/10.11604/pamj.2018.30.146.15610DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6201618PMC
November 2018
5 Reads

Management and outcomes of pediatric vocal cord paresis in Chiari malformation.

Int J Pediatr Otorhinolaryngol 2018 Dec 18;115:49-53. Epub 2018 Sep 18.

Medical College of Wisconsin, Department of Otolaryngology, Milwaukee, WI, USA. Electronic address:

Introduction: Pediatric vocal cord paresis (VCP) has a variety of etiologies, including congenital neurologic disease. Arnold-Chiari Malformation (ACM) is one such disease with known VCP association. However, the natural history, need for tracheostomy, and rate of decannulation in this patient population is not well characterized. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S01655876183046
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http://dx.doi.org/10.1016/j.ijporl.2018.09.014DOI Listing
December 2018
4 Reads

High Incidence of Cranial Synostosis and Chiari I Malformation in Children With X-Linked Hypophosphatemic Rickets (XLHR).

J Bone Miner Res 2018 Oct 23. Epub 2018 Oct 23.

Pediatric Neurosurgery, Hôpital Femme Mère Enfant, Hospices Civiles de Lyon and University Claude Bernard Lyon 1, Bron Cedex, France.

X-linked hypophosphatemic rickets (XLHR) represents the most common form of genetic hypophosphatemia and causes rickets and osteomalacia in children because of increased FGF23 secretion and renal phosphate wasting. Even though cranial vault and craniovertebral anomalies of potential neurosurgical interest, namely early closure of the cranial sutures and Chiari type I malformation, have been observed in children with XLHR, their actual incidence and characteristics are not established. The aims of this study were to analyze the incidence of cranial and cervico-occipital junction (COJ) anomalies in children with XLHR and describe its features. Read More

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http://dx.doi.org/10.1002/jbmr.3614DOI Listing
October 2018
9 Reads

Dizygotic opposite-sex twins with surgically repaired concordant myelomeningocele conceived by in vitro fertilization using intracytoplasmic sperm injection: a case report and review of the literature.

Childs Nerv Syst 2018 Oct 22. Epub 2018 Oct 22.

Department of Neurosurgery, Division of Pediatric Neurosurgery, University Hospital and Children's Hospital of Basel, Spitalstrasse 21, 4031, Basel, Switzerland.

Background: Myelomeningocele (MMC) is a common subtype of congenital neural tube defects (NTD). Although congenital malformations including NTD are more common in twins, concordance, especially in dizygotic twins, is extremely rare and is found mostly in same-sex twins. The role of genetic and environmental factors in the etiology of MMC is unclear. Read More

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http://dx.doi.org/10.1007/s00381-018-3990-8DOI Listing
October 2018
2 Reads

Clinical Significance of Variable Clivus Gradients in Patients with Chiari Malformation Type I After Surgical Decompression: A Retrospective Analysis.

World Neurosurg 2018 Oct 19. Epub 2018 Oct 19.

Department of Neurosurgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China. Electronic address:

Objective: Previous studies have shown that the clivus angle gradient is significantly decreased in patients with Chiari malformation type I (CMI) with an associated syrinx compared with patients with CMI only and a healthy population. To date, the relationship between the clivus gradient and clinical outcomes has remained unclear. The objective of the present study was to investigate whether different clivus gradients (∠α) in CMI after posterior fossa decompression will lead to different clinical outcomes. Read More

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http://dx.doi.org/10.1016/j.wneu.2018.10.068DOI Listing
October 2018
10 Reads

Myelomeningocele Versus Myelocele on Fetal MR Images: Are There Differences in Brain Findings?

AJR Am J Roentgenol 2018 Dec 17;211(6):1376-1380. Epub 2018 Oct 17.

1 Department of Radiology and Medical Imaging, Cincinnati Children's Hospital Medical Center, 3333 Burnet Ave, Cincinnati, OH 45229-3026.

Objective: The purpose of this study was to examine differences between patients with myelomeningocele and those with myelocele with respect to brain imaging findings at fetal MRI.

Materials And Methods: A single-center retrospective analysis was performed of fetal MRI examinations revealing open spinal dysraphism from 2004 through 2016 with available diagnostic postnatal spinal MR images in conjunction with neurosurgical follow-up findings. Images were reviewed by two board-certified fellowship-trained pediatric neuroradiologists. Read More

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https://www.ajronline.org/doi/10.2214/AJR.18.20088
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http://dx.doi.org/10.2214/AJR.18.20088DOI Listing
December 2018
8 Reads

Minimally-invasive approach to posterior fossa decompression: Initial experience in Adult Chiari Type 1 Malformation patients.

J Clin Neurosci 2018 Oct 4;56:90-94. Epub 2018 Jul 4.

Division of Neurosurgery, National University Health System, 1E Kent Ridge Road, 119228, Singapore; Department of Neurological Surgery, School of Medicine and Public Health, University of Wisconsin, 750 Highland Avenue, 53726 Madison, WI, USA.

We report our initial experience using Minimally-Invasive Surgery (MIS) technique for Posterior Fossa Decompression (PFD) in Adult Chiari 1 Malformation (C1M) patients. Five subjects who were treated with MIS PFD at our center and followed up over a 5-year period. Another nine subjects who were treated with Open PFD and follow up over the same period were used for comparison. Read More

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http://dx.doi.org/10.1016/j.jocn.2018.06.054DOI Listing
October 2018

Familial Chiari Type 1: A Molecular Karyotyping Study in a Turkish Family and Review of the Literature.

World Neurosurg 2019 Jan 11;121:e852-e857. Epub 2018 Oct 11.

Department of Neurological Surgery, TR University of Health Sciences, Istanbul Fatih Sultan Mehmet Education and Research Hospital, Istanbul, Turkey.

Background: The etiology of Chiari I malformation (CMI) has not been fully elucidated. Therefore, we performed a genetic study of a Turkish family in which 3 sisters had a diagnosis of CMI with or without syringomyelia.

Methods: In a family with 7 children, 4 daughters complained of occipital headaches. Read More

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http://dx.doi.org/10.1016/j.wneu.2018.09.235DOI Listing
January 2019
2 Reads

Investigation of sensory thresholds in Cavalier King Charles Spaniels with and without Chiari-like malformations and syringomyelia.

J Vet Intern Med 2018 Nov 11;32(6):2021-2028. Epub 2018 Oct 11.

Department of Clinical Sciences, College of Veterinary Medicine, North Carolina State University, Raleigh, North Carolina.

Background: Cavalier King Charles Spaniels (CKCS) suffer pain associated with Chiari-like malformation and syringomyelia (CMSM). People suffer from a similar condition and describe numerous sensory abnormalities. Sensory changes have not been quantified in affected CKCS. Read More

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http://doi.wiley.com/10.1111/jvim.15297
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http://dx.doi.org/10.1111/jvim.15297DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6272044PMC
November 2018
2 Reads

Chiari Type I Malformation Associated With Verbal Fluency Impairment.

J Speech Lang Hear Res 2018 Oct;61(10):2458-2466

Department of Personality, Evaluation and Psychological Treatments, Faculty of Psychology and Education, University of Deusto, Bilbao, Spain.

Purpose: Chiari malformation (CM) Type I is a rare disorder that implies an anomaly in the craniocervical junction, where one or both cerebellar tonsils are displaced below the foramen magnum into the cervical spinal channel. Research carried out regarding cognitive symptoms such as verbal fluency is scarce. The aim of this study was to investigate whether verbal fluency is impaired in a CM clinical group compared to a group of healthy control individuals while controlling for depression and anxiety symptomatology. Read More

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http://jslhr.pubs.asha.org/article.aspx?doi=10.1044/2018_JSL
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http://dx.doi.org/10.1044/2018_JSLHR-S-17-0465DOI Listing
October 2018
7 Reads

Management of Anesthesia and Delivery in Women With Chiari I Malformations.

Obstet Gynecol 2018 Oct 5. Epub 2018 Oct 5.

Department of Neurology, Division of Women's Neurology, the Department of Neurology, and the Departments of Anesthesiology and Bioengineering, McGowan Institute for Regenerative Medicine, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania; the Department of Neurology, Brigham & Women's Hospital, Harvard Medical School, Boston, Massachusetts; and the Department of Bioengineering, University of Pittsburgh, and the Department of Obstetrics, Gynecology and Reproductive Sciences, Division of Maternal-Fetal Medicine, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania.

Objective: To estimate whether vaginal delivery or neuraxial anesthesia poses a risk of neurologic deterioration in women with uncorrected Chiari I malformation.

Methods: To assemble this case series, electronic record databases were used to identify women with Chiari I malformation who delivered on two busy tertiary care obstetric services over a 5-year period from January 2010 through December 2015. Women who had undergone surgical decompression were not included in the study. Read More

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http://dx.doi.org/10.1097/AOG.0000000000002943DOI Listing
October 2018
3 Reads

Chiari 1 Malformation Surgery: Comparing Non-violation of the Arachnoid versus Arachnoid Opening and Thermocoagulation of the Tonsils.

World Neurosurg 2019 Jan 4;121:e605-e613. Epub 2018 Oct 4.

Department of Anatomy, Universidade Federal da Paraíba, João Pessoa, Paraíba, Brazil.

Objective: Evidence is lacking concerning the myriad surgical techniques for type 1 Chiari malformation. This study evaluated the impact of arachnoid violation with tonsil thermocoagulation during surgical craniovertebral junction decompression.

Methods: The evaluation included aspects of the neurologic examination and parameters of cerebrospinal fluid flow on magnetic resonance imaging during preoperative and postoperative periods. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S18788750183223
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http://dx.doi.org/10.1016/j.wneu.2018.09.175DOI Listing
January 2019
2 Reads

Chiari malformation clusters describe differing presence of concurrent anomalies based on Chiari type.

J Clin Neurosci 2018 Dec 29;58:165-171. Epub 2018 Sep 29.

Department of Orthopaedic and Neurologic Surgery, NYU Langone Orthopedic Hospital, NYU Langone Medical Center, New York, NY, USA. Electronic address:

Chiari malformations are structural defects in the posterior fossa where the cerebellum displaces caudally into the foramen magnum and upper spinal canal. These malformations are classified by severity as Types 1-4, each presenting with different associated and/or concurrent conditions and anomalies. The aim of this study was to utilize a nationwide database to study patients with Chiari malformations including their concurrent diagnoses and associated anomalies. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S09675868183054
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http://dx.doi.org/10.1016/j.jocn.2018.06.045DOI Listing
December 2018
3 Reads

Spontaneous Pneumocephalus Secondary to Leaking Myelomeningocele.

J Pediatr Neurosci 2018 Jul-Sep;13(3):329-330

Department of Neurosurgery, Government Medical College, Thiruvananthapuram, Kerala, India.

Myelomeningocele is one among the most common forms of spinal dysraphism. We report a newborn male child with leaking myelomeningocele who presented with apneic spells. He underwent a magnetic resonance imaging of the neuraxis, which revealed Chiari malformation and severe hydromyelia along with pneumocephalus. Read More

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http://www.pediatricneurosciences.com/text.asp?2018/13/3/329
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http://dx.doi.org/10.4103/JPN.JPN_17_18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6144603PMC
October 2018
7 Reads

Giant Occipital Encephalocele with Chiari Malformation Type 3.

J Neurosci Rural Pract 2018 Oct-Dec;9(4):619-621

Department of Neurosciences, School of Medical Sciences, Universiti Sains Malaysia, Health Campus, Kelantan, Malaysia.

Encephaloceles are rare embryological mesenchymal developmental anomalies resulting from inappropriate ossification in the skull through which herniate the intracranial contents of the sac. Occipital encephaloceles are described as giant when they are larger than the head from which they arise, and they pose a great surgical challenge. Herein, we present a case of a giant occipital encephalocele in a neonate with Chiari malformation Type 3 to highlight the problems encountered in its management and the outcome of the surgery. Read More

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http://www.ruralneuropractice.com/text.asp?2018/9/4/619/2398
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http://dx.doi.org/10.4103/jnrp.jnrp_103_18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6126286PMC
October 2018
3 Reads

Response of Syrinx Associated with Chiari I Malformation to Posterior Fossa Decompression with or without Duraplasty and Correlation with Functional Outcome: A Prospective Study of 22 Patients.

J Neurosci Rural Pract 2018 Oct-Dec;9(4):587-592

Department of Neuroimaging and Interventional Neuroradiology, National Institute of Mental Health and Neurosciences, Bengaluru, Karnataka, India.

Background: The aim of the study is to correlate the surgical outcome with radiological changes in patients with Chiari I malformation (CMI) with syrinx. We also compared long-term functional and radiographic outcome in CM1 patients treated with posterior fossa decompression (PFD) with or without duroplasty.

Patients And Methods: From December 2013 to October 2015, 22 patients who underwent surgery with the diagnosis of CMI and syrinx were included in the study. Read More

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http://www.ruralneuropractice.com/text.asp?2018/9/4/587/2398
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http://dx.doi.org/10.4103/jnrp.jnrp_10_18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6126299PMC
October 2018
1 Read

The role of lumboperitoneal shunts in managing chronic hydrocephalus with slit ventricles.

J Neurosurg Pediatr 2018 Sep 21:1-6. Epub 2018 Sep 21.

Departments of Neurosurgery and Pediatric Neurosurgery, Wayne State University School of Medicine and Children's Hospital of Michigan, Detroit; and.

Objective: Lumboperitoneal (LP) shunts have a role not only in pseudotumor cerebri, but also in patients with slit-like ventricles who are treated with CSF shunting on a chronic basis. Hesitation to utilize LP shunts is based on previous conventional beliefs including the tendency for overdrainage, difficulties accessing the shunt to tap or revise, and risk of progressive cerebellar tonsillar herniation. The authors hypothesized that the use of horizontal-vertical (HV) valves may reduce the risk of these complications, particularly overdrainage and development of Chiari malformation. Read More

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http://dx.doi.org/10.3171/2018.6.PEDS17642DOI Listing
September 2018
5 Reads
1.370 Impact Factor

Intracranial hypertension following sacrifice of occipital and marginal sinuses during posterior fossa decompression for Chiari I malformation: case report.

J Neurosurg Pediatr 2018 Sep 21:1-4. Epub 2018 Sep 21.

Departments of 1 Pediatric Neurosurgery.

The occipital and marginal sinuses, when present, must be sacrificed in order to open the dura in most posterior fossa surgeries in the pediatric population, including posterior fossa decompression for Type I Chiari malformation (CM-I) with duraplasty. Apart from the immediate risk of hemorrhage, the voluntary occlusion of this structure is almost universally well tolerated. The authors report a case of intracranial hypertension following the sacrifice of occipital and marginal sinuses following posterior fossa decompression with duraplasty for CM-I. Read More

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http://thejns.org/doi/10.3171/2018.7.PEDS18237
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http://dx.doi.org/10.3171/2018.7.PEDS18237DOI Listing
September 2018
9 Reads
1.370 Impact Factor

Imaging of Clival Hypoplasia in CHARGE Syndrome and Hypothesis for Development: A Case-Control Study.

AJNR Am J Neuroradiol 2018 Oct 20;39(10):1938-1942. Epub 2018 Sep 20.

Radiology (L.C.M.), University of Groningen, University Medical Center Groningen, Groningen, the Netherlands.

Background And Purpose: We present the largest case series to date on basiocciput abnormalities in CHARGE syndrome (Coloboma of the eye, Heart defects, Atresia of the choanae, Retardation of growth and/or development, Genital and/or urinary abnormalities, and Ear abnormalities and/or deafness). We aimed to show that basiocciput abnormalities are common and may aid in diagnosis. We furthermore explored whether clivus size correlates with the type of chromodomain-helicase-DNA binding protein 7 gene () mutation, which causes CHARGE syndrome, and with clinical criteria according to Blake et al and Verloes. Read More

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http://dx.doi.org/10.3174/ajnr.A5810DOI Listing
October 2018
7 Reads

Cervico-medullary compression ratio: A novel radiological parameter correlating with clinical severity in Chiari type 1 malformation.

Clin Neurol Neurosurg 2018 Nov 10;174:123-128. Epub 2018 Sep 10.

Ministry of Health, Health Sciences University, Diskapi Yildirim Beyazit Training and Research Hospital, Department of Neurosurgery, Ankara, Turkey.

Objectives: Chiari malformation type 1 (CM-1) is associated with cough headache, intracranial hypertension, cerebellar and spinal cord symptoms/signs. Herniated cerebellar tonsil length (HCTL) is widely used radiological parameter to determine the severity of CM-1, but with limited utility due to its weak correlation with some clinico-radiological findings. In this study, we aimed to evaluate a novel, practical parameter (cervico-medullary compression ratio; "CMCR") for its relationship with clinico-radiological findings in CM-1. Read More

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http://dx.doi.org/10.1016/j.clineuro.2018.09.016DOI Listing
November 2018
4 Reads

A rare case of Chiari malformation secondary to Paget's disease of skull.

Neurol India 2018 Sep-Oct;66(5):1510-1511

Department of Neurosurgery, Longyan First Hospital, Fujian Medical University, Longyan, Fujian, People's Republic of China.

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http://dx.doi.org/10.4103/0028-3886.241388DOI Listing
September 2018
2 Reads

Management and birth outcomes of pregnant women with Chiari malformations: A 14 years retrospective case series.

Eur J Obstet Gynecol Reprod Biol 2018 Nov 10;230:1-5. Epub 2018 Sep 10.

Department of obstetrics and gynaecology, Birmingham Womens Hospital, Birmingham, UK; Birmingham University, Birmingham, UK.

Objective: The management of Chiari malformations in pregnancy is challenging due to the perceived risk of adverse maternal neurological outcomes and raising intracranial pressure during labour. Our aim was to evaluate the management and health outcomes of pregnant women cared for at a regional referral centre and highlight elements of best practice.

Study Design: A retrospective case series of all pregnant women diagnosed with Chiari malformation over fourteen years (January 2004-June 2018) at the Birmingham Women's Hospital - UK. Read More

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http://dx.doi.org/10.1016/j.ejogrb.2018.09.006DOI Listing
November 2018
2 Reads

Use of ventilation bag for the respiratory support during magnetic resonance imaging in Arnold-Chiari ventilated patients, a case report.

J Spinal Cord Med 2018 Sep 12:1-4. Epub 2018 Sep 12.

a Neurorehabilitation Department , Fondazione Ospedale San Camillo IRCCS , Venezia , Italia.

Context: Magnetic Resonance Imaging (MRI) is an essential diagnostic tool for neuroimaging tissues such as the spinal cord. Unfortunately, the use of MRI may be limited in ventilated patients, who cannot maintain the supine position in spontaneous breathing for the whole duration of the exam (i.e. Read More

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http://dx.doi.org/10.1080/10790268.2018.1519997DOI Listing
September 2018
1 Read

Physical therapy examination and management of a 48-year-old male with vertigo, cephalalgia, and cervicalgia secondary to unilateral vestibular hypofunction.

Physiother Theory Pract 2018 Sep 10:1-12. Epub 2018 Sep 10.

c Department of Physical Therapy , Angelo State University, Texas Tech University System , San Angelo , TX , USA.

Purpose: This case report presents evidence-based physical therapy assessments and interventions for a patient with unilateral vestibular hypofunction (UVH). UVH is the result of peripheral vestibular dysfunction in the inner ear.

Case Description: The patient was a 48-year-old male with symptoms of dizziness, cephalalgia, and cervicalgia. Read More

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http://dx.doi.org/10.1080/09593985.2018.1517848DOI Listing
September 2018
2 Reads