4,442 results match your criteria Chiari Malformation


Hyperinsulinaemic hypoglycaemia: A new presentation of 16p11.2 deletion syndrome.

Clin Endocrinol (Oxf) 2019 Feb 18. Epub 2019 Feb 18.

Endocrinology Department, Great Ormond Street Hospital for Children, NHS Foundation Trust, London, UK.

16p11.2 microdeletion syndrome is a recognisable chromosomal anomaly caused by microdeletions in the 16p11.2 locus. Read More

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http://dx.doi.org/10.1111/cen.13951DOI Listing
February 2019

Expanding the genetic and clinical spectrum of the NONO-associated X-linked intellectual disability syndrome.

Am J Med Genet A 2019 Feb 17. Epub 2019 Feb 17.

Division of Medical Genetics, University of Utah, Salt Lake City, Utah.

The NONO gene encodes a nuclear protein involved in RNA metabolism. Hemizygous loss-of-function NONO variants have been associated with syndromic intellectual disability and with left ventricular noncompaction (LVNC). A two-year-old boy presented to the University of Utah's Penelope Undiagnosed Disease Program with developmental delay, nonfamilial features, relative macrocephaly, and dilated cardiomyopathy with LVNC and Ebstein anomaly. Read More

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http://dx.doi.org/10.1002/ajmg.a.61091DOI Listing
February 2019
1 Read

Surgical Experience in Pediatric Patients with Chiari-I Malformations Aged ≤18 Years.

J Neurosci Rural Pract 2019 Jan-Mar;10(1):85-88

Department of Neurosurgery, Maharaja Agrasen Hospital, New Delhi, India.

Objective: The objective of this study was to retrospectively study Chiari I malformation patients (<18 years) treated surgically.

Materials And Methods: Chiari I malformation patients (<18 years) treated surgically at our institute were retrospectively studied.

Results: During the study period between January 1999 and June 2011, fifty patients, aged ≤18 years with Chiari malformation, were treated surgically and formed the basis for this series. Read More

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http://dx.doi.org/10.4103/jnrp.jnrp_160_18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6337998PMC
February 2019

ERF-related craniosynostosis: The phenotypic and developmental profile of a new craniosynostosis syndrome.

Am J Med Genet A 2019 Feb 13. Epub 2019 Feb 13.

Clinical Genetics Service, Great Ormond Street Hospital, London, United Kingdom.

Mutations in the ERF gene, coding for ETS2 repressor factor, a member of the ETS family of transcription factors cause a recently recognized syndromic form of craniosynostosis (CRS4) with facial dysmorphism, Chiari-1 malformation, speech and language delay, and learning difficulties and/or behavioral problems. The overall prevalence of ERF mutations in patients with syndromic craniosynostosis is around 2%, and 0.7% in clinically nonsyndromic craniosynostosis. Read More

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http://dx.doi.org/10.1002/ajmg.a.61073DOI Listing
February 2019
1 Read

Chiari malformations in adults: A single center surgical experience with special emphasis on the kinetics of clinical improvement.

Neurochirurgie 2019 Feb 8. Epub 2019 Feb 8.

Service de neurochirurgie, hôpital Nord, 13015 Marseille, France.

Background: The Chiari malformation type I (CM-I) is the most commonly found type in adults. The efforts to further improve the treatment offered for these malformations are hampered by the existence of controversial methods and the absence of a uniform scoring system to evaluate clinical outcomes.

Objectives: The goal of our study is to analyze the clinical and radiological data concerning patients operated for CM and to expose surgical techniques. Read More

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http://dx.doi.org/10.1016/j.neuchi.2018.10.010DOI Listing
February 2019
1 Read

Posterior fossa decompression for Chiari malformation type I: clinical and radiological presentation, outcome and complications in a retrospective series of 105 procedures.

Acta Neurol Belg 2019 Feb 8. Epub 2019 Feb 8.

Department of Neurosurgery, University Hospitals, Leuven, Belgium.

Objective: Determining clinical and radiological characteristics, complication rates and outcome for patients undergoing posterior fossa decompression (PFD) and duraplasty for Chiari malformation type I (CM-I).

Methods And Materials: Retrospective, single-university hospital study of all PFDs for CM-I between January 1995 and December 2016.

Results: PFD was performed in 105 patients with CM-I (n = 105), of whom 62 suffered from associated syringomyelia and 37 were pediatric cases. Read More

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http://dx.doi.org/10.1007/s13760-019-01086-7DOI Listing
February 2019
1 Read

An unusual presentation of bobble-head doll syndrome in a patient with hydranencephaly and Chiari 3 malformation.

Childs Nerv Syst 2019 Feb 6. Epub 2019 Feb 6.

Department of Surgery, Division of Neurosurgery, College of Health Sciences, University of Zimbabwe, P.O Box A178, Avondale, Harare, Zimbabwe.

Bobble-head doll syndrome is a rare movement disorder that is usually associated with lesions involving the third ventricle. It is characterised by stereotypical rhythmic up-and-down or side-to-side head movements. The pathophysiology and anatomical basis for this unusual manifestation is still a subject of intense scrutiny. Read More

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http://dx.doi.org/10.1007/s00381-019-04054-xDOI Listing
February 2019

Development of Common Data Elements for Use in Chiari Malformation Type I Clinical Research: An NIH/NINDS Project.

Neurosurgery 2019 Jan 23. Epub 2019 Jan 23.

Division of Neuroscience, National Institutes of Health/National Institute of Neurological Disorders and Stroke, Bethesda, Maryland.

The management of Chiari I malformation (CMI) is controversial because treatment methods vary and treatment decisions rest on incomplete understanding of its complex symptom patterns, etiologies, and natural history. Validity of studies that attempt to compare treatment of CMI has been limited because of variable terminology and methods used to describe study subjects. The goal of this project was to standardize terminology and methods by developing a comprehensive set of Common Data Elements (CDEs), data definitions, case report forms (CRFs), and outcome measure recommendations for use in CMI clinical research, as part of the CDE project at the National Institute of Neurological Disorders and Stroke (NINDS) of the US National Institutes of Health. Read More

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http://dx.doi.org/10.1093/neuros/nyy475DOI Listing
January 2019
2 Reads
3.620 Impact Factor

The value of CSF flow studies in the management of CSF disorders in children: a pictorial review.

Insights Imaging 2019 Jan 28;10(1). Epub 2019 Jan 28.

Department of Radiodiagnosis, Pediatric Radiology section, Faculty of Medicine, Ain-Shams University, Abbasia, Cairo, 11657, Egypt.

CSF flow disorders are frequently encountered in children. The advent of MR technology with the emergence of new pulse sequences allowed better understanding of CSF flow dynamics. In this pictorial review, we aim to conduct a comprehensive review of the MR protocol used to study CSF flow disorders and to discuss the utility of each pulse sequence in the adopted protocol. Read More

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http://dx.doi.org/10.1186/s13244-019-0686-xDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6352391PMC
January 2019
1 Read

Anterior Spinal Overgrowth of the Thoracic Spine May Not Be Involved in the Initiation of Adolescent Idiopathic Scoliosis.

World Neurosurg 2019 Jan 25. Epub 2019 Jan 25.

Spine Surgery, Drum Tower Hospital of Nanjing University Medical School, Nanjing, China. Electronic address:

Objective: To compare vertebral morphology among patients with adolescent idiopathic scoliosis (AIS), patients with Chiari I malformation (CMS)-associated scoliosis, and normal control subjects, with the goal of determining the role of anterior column overgrowth in the development of AIS.

Methods: One hundred and forty adolescent girls were enrolled (50 with AIS, 40 with CMS, and 50 control subjects). Thoracic computed tomography images were obtained for all subjects. Read More

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http://dx.doi.org/10.1016/j.wneu.2019.01.071DOI Listing
January 2019
1 Read

Appropriate surgical procedures for Chiari type 1 malformation and associated syrinx based on radiological characteristics of the craniovertebral junction.

Neurosurg Rev 2019 Jan 25. Epub 2019 Jan 25.

Department of Neurosurgery, Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto, 390-8621, Japan.

Several surgical procedures can be applied for syrinx associated with Chiari type 1 malformation; however, it remains controversial as to which approach is the most effective. Here, we evaluated the indications and limitations of foramen magnum decompression (FMD) with or without dural plasty. Forty patients with Chiari type 1 malformation were surgically treated and followed up for > 12 months. Read More

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http://dx.doi.org/10.1007/s10143-019-01079-3DOI Listing
January 2019

Clinical deterioration despite syringomyelia resolution after successful foramen magnum decompression for Chiari malformation - Case series.

Eur J Paediatr Neurol 2019 Jan 14. Epub 2019 Jan 14.

Department of Paediatric Neurosciences, Royal Hospital for Sick Children, Edinburgh, UK.

Introduction: Neurosurgical treatment is recommended for symptomatic syringomyelia and the post-operative radiological resolution of the syringomyelia is associated with an improvement or at least stability of the patient's pre-operative symptoms.

Methods: We reviewed syringomyelia treatment in our centre over the last five years for clinical outcome, surgical complications, post operative MRI and long term symptom resolution.

Results: 50 cases of symptomatic syringomyelia underwent foramen magnum decompression and expansile watertight duroplasty. Read More

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http://dx.doi.org/10.1016/j.ejpn.2019.01.003DOI Listing
January 2019

Encysted hydrocele of the canal of Nuck in an 11-month-old child with a past history of duodenal atresia and Arnold-Chiari malformation: A case report.

Medicine (Baltimore) 2019 Jan;98(4):e14232

College of Medicine, Qatar University, Doha, Qatar.

Rationale: Hydrocele of the canal of Nuck is a rare developmental disorder and represents of a homolog of hydrocele of spermatic cord in males. Hydrocele of the canal of Nuck is a very rare cause of inguinal swelling in female infants and children. It results from the failure of obliteration of the distal portion of evaginated parietal peritoneum within the inguinal canal, which forms a sac containing fluid. Read More

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http://dx.doi.org/10.1097/MD.0000000000014232DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6358343PMC
January 2019
1 Read
5.723 Impact Factor

[Correlation between syrinx resolution after posterior fossa decompression and cervical sagittal profile change in adolescents with Chiari malformation and syringomyelia].

Zhonghua Yi Xue Za Zhi 2019 Jan;99(3):183-187

Department of Spinal Surgery, Drum Tower Hospital Clinical College of Nanjing Medical University, Nanjing 210008, China.

To evaluate the long term change of the cervical sagittal profile in adolescent Chiari malformation type Ⅰ (CMI)/syringomyelia undergoing posterior fossa decompression (PFD) and to further evaluate the correlation between the syrinx resolution and cervical sagittal profile. A retrospective radiographic study was performed in 32 adolescents undergoing PFD for CMI/syringomyelia from October 2011 to August 2015 with a minimum 2-year follow-up. There were 23 males and 9 females, with a mean age of (13. Read More

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http://dx.doi.org/10.3760/cma.j.issn.0376-2491.2019.03.006DOI Listing
January 2019

Syringo-subarachnoid shunt: how I do it.

Acta Neurochir (Wien) 2019 Feb 22;161(2):367-370. Epub 2019 Jan 22.

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

Background: Syringo-subarachnoid shunt (SSS) is a valid method for the treatment of syringomyelia persisting after foramen magnum decompression (FMD) for Chiari I malformation.

Method: We give a brief overview on indication and outcome of SSS, followed by a detailed description of the surgical anatomy, and of the microsurgical technique. In particular, we highlight some key points for complication avoidance. Read More

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http://dx.doi.org/10.1007/s00701-019-03810-xDOI Listing
February 2019

Neural Axis Abnormalities in Patients With Adolescent Idiopathic Scoliosis: Is Routine Magnetic Resonance Imaging Indicated Irrespective of Curve Severity?

Neurospine 2018 Oct 15. Epub 2018 Oct 15.

Department of Orthopedic Surgery, Spine Unit, Rigshospitalet, University of Copenhagen, Copenhagen, Denmark.

Objective: MRI-verified neural axis abnormality (NAA) has been described in adolescent idiopathic scoliosis (AIS) and several risk factors have been associated with the presence of NAA in AIS-patients. However, the clinical significance of these findings is not clear. The purpose of the present study was to determine the prevalence of NAAs in a large consecutive cohort of AIS patients and evaluate the clinical significance of previously proposed risk factors. Read More

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http://e-neurospine.org/journal/view.php?doi=10.14245/ns.183
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http://dx.doi.org/10.14245/ns.1836154.077DOI Listing
October 2018
10 Reads

Comparison of Dural Splitting and Duraplasty in Patients with Chiari Type I Malformation: Relationship between Tonsillo-Dural Distance and Syrinx Cavity.

Turk Neurosurg 2018 Jun 21. Epub 2018 Jun 21.

Mustafa Kemal University Medical Faculty.

Aim: Chiari Malformation Type I is associated with anomaly of hindbrain with displacement of the cerebellar tonsils into the upper cervical canal.The dural splitting and duraplasty are common methods which were used in the surgical treatment for the patients with Chiari Malformation Type I. In this study, we aimed to comparison two surgical methods in terms of clinical and radiological results. Read More

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http://dx.doi.org/10.5137/1019-5149.JTN.23319-18.2DOI Listing

Pilot study of head conformation changes over time in the Cavalier King Charles spaniel breed.

Vet Rec 2019 Jan 11;184(4):122. Epub 2019 Jan 11.

Faculty of Health and Medical Sciences, School of Veterinary Medicine, University of Surrey, Guildford, UK.

Modern interpretation of head conformation in the Cavalier King Charles spaniel (CKCS) has favoured a smaller, more exaggerated, brachycephalic type than originally described in the 1929 breed standard. Recent research studies identified brachycephaly and reduced hind cranium as two conformational (dysmorphic) features that increase risk for symptomatic Chiari-like malformation and secondary syringomyelia (SM). A prospective pilot study investigated the hypothesis that dysmorphic head features could be assessed visually and correlated with risk of SM. Read More

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http://dx.doi.org/10.1136/vr.105135DOI Listing
January 2019

Cervical medullary syndrome secondary to craniocervical instability and ventral brainstem compression in hereditary hypermobility connective tissue disorders: 5-year follow-up after craniocervical reduction, fusion, and stabilization.

Neurosurg Rev 2019 Jan 9. Epub 2019 Jan 9.

Medical University of South Carolina, Charleston, SC, USA.

A great deal of literature has drawn attention to the "complex Chiari," wherein the presence of instability or ventral brainstem compression prompts consideration for addressing both concerns at the time of surgery. This report addresses the clinical and radiological features and surgical outcomes in a consecutive series of subjects with hereditary connective tissue disorders (HCTD) and Chiari malformation. In 2011 and 2012, 22 consecutive patients with cervical medullary syndrome and geneticist-confirmed hereditary connective tissue disorder (HCTD), with Chiari malformation (type 1 or 0) and kyphotic clivo-axial angle (CXA) enrolled in the IRB-approved study (IRB# 10-036-06: GBMC). Read More

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http://link.springer.com/10.1007/s10143-018-01070-4
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http://dx.doi.org/10.1007/s10143-018-01070-4DOI Listing
January 2019
15 Reads

A Conditional Inference Tree Model for Predicting Sleep-Related Breathing Disorders in Patients With Chiari Malformation Type 1: Description and External Validation.

J Clin Sleep Med 2019 Jan 15;15(1):89-99. Epub 2019 Jan 15.

Neurotraumatology and Neurosurgery Research Unit (UNINN), Vall d'Hebron University Hospital, Barcelona, Spain.

Study Objectives: The aim of this study is to generate and validate supervised machine learning algorithms to detect patients with Chiari malformation (CM) 1 or 1.5 at high risk of the development of sleep-related breathing disorders (SRBD) using clinical and neuroradiological parameters.

Methods: We prospectively included two independent datasets. Read More

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http://dx.doi.org/10.5664/jcsm.7578DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6329533PMC
January 2019
2 Reads

Sensory dysphagia: A case series and proposed classification of an under recognized swallowing disorder.

Head Neck 2019 Jan 8. Epub 2019 Jan 8.

Department of Neurology, University of Massachusetts Medical School, Worcester, Massachusetts.

Background: Although sensory feedback is a vital regulator of deglutition, it is not comprehensively considered in the standard dysphagia evaluation. Difficulty swallowing secondary to sensory loss may be termed "sensory dysphagia" and may account for cases receiving diagnoses of exclusion, like functional or idiopathic dysphagia.

Methods And Results: Three cases of idiopathic dysphagia were suspected to have sensory dysphagia. Read More

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http://doi.wiley.com/10.1002/hed.25588
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http://dx.doi.org/10.1002/hed.25588DOI Listing
January 2019
4 Reads

Giant Tongue in a Patient With Chiari Malformation and Neuroleptic-Induced Tardive Dyskinesia.

J Craniofac Surg 2018 Dec 29. Epub 2018 Dec 29.

Department of Plastic, Reconstructive and Maxillo-Facial Surgery, and Burn Unit, Centro Hospitalar de São João, Porto Medical School, Alameda Professor Hernâni Monteiro, Porto, Portugal.

A 68-year-old woman, presented with a squamous cell carcinoma of the malar region, and underwent wide local excision. During her clinical examination, repetitive protrusion and intrusion of the tongue as well as stereotypic, abnormal movements of the mouth and lips were observed, in a pattern that resembled chewing, sucking or lip pursing; dyskinesias ceased when she was speaking or bringing food to the mouth. She was unaware of the movements and the tongue was observed to move similar to choreiform movements, while revealing a giant "snake-like" macroglossia. Read More

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http://dx.doi.org/10.1097/SCS.0000000000005066DOI Listing
December 2018

Kyphoscoliosis with Klippel-Trenaunay syndrome: a case report and literature review.

BMC Musculoskelet Disord 2019 Jan 5;20(1):10. Epub 2019 Jan 5.

Department of Orthopaedic Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing, 100730, China.

Background: Klippel-Trenaunay syndrome (KTS) is a rare congenital syndrome characterized by the triad of venous varicosities, capillary malformations and limb hypertrophy. However, KTS may rarely occur in combination with kyphoscoliosis.

Case Presentation: We presented an 18-year-old female with KTS and kyphoscoliosis. Read More

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https://bmcmusculoskeletdisord.biomedcentral.com/articles/10
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http://dx.doi.org/10.1186/s12891-018-2393-zDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6320630PMC
January 2019
10 Reads

Patients with "benign" Chiari I malformations require surgical decompression at a low rate.

J Neurosurg Pediatr 2019 Jan 4:1-9. Epub 2019 Jan 4.

1Department of Neurosurgery and Division of Pediatric Neurosurgery, University of Alabama at Birmingham, Alabama; and.

OBJECTIVEThere are sparse published data on the natural history of "benign" Chiari I malformation (CM-I)-i.e., Chiari with minimal or no symptoms at presentation and no imaging evidence of syrinx, hydrocephalus, or spinal cord signal abnormality. Read More

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http://dx.doi.org/10.3171/2018.10.PEDS18407DOI Listing
January 2019
1 Read

Perverted Downward Corrective Saccades During Horizontal Head Impulses in Chiari Malformation.

Cerebellum 2019 Jan 4. Epub 2019 Jan 4.

Department of Neurology, Seoul National University College of Medicine, Seoul, South Korea.

The mechanism of perverted vertical responses during horizontal head impulse tests (HITs) requires further elucidation. A 47-year-old woman with a Chiari malformation showed alternating skew deviation, downbeat nystagmus with an increasing slow phase velocity, impaired smooth pursuit, and upward ocular deviation during horizontal HITs and corrective downward saccades in the presence of normal bithermal caloric tests and intact tilt suppressions of the post-rotatory nystagmus. These findings suggest dysfunction of the inferior cerebellum including the tonsil, nodulus, and uvula. Read More

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http://link.springer.com/10.1007/s12311-018-1000-z
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http://dx.doi.org/10.1007/s12311-018-1000-zDOI Listing
January 2019
8 Reads

Shunt dependency syndrome and acquired Chiari malformation secondary to cerebrospinal fluid diversion procedures: a 9-year longitudinal observation.

Childs Nerv Syst 2019 Jan 4. Epub 2019 Jan 4.

Department of Neurosurgery, China National Clinical Research Center for Neurological Diseases, Beijing Tiantan Hospital, Capital Medical University, Beijing, China.

Background: Shunt dependency syndrome is a rare long-term complication of cystoperitoneal (CP) shunting for intracranial arachnoid cysts, which is characterized by acute intracranial hypertension with normal-sized or small ventricles. Additionally, acquired Chiari type I malformations (ACIM) could be infrequently secondary to extrathecal shunt drainage of cerebrospinal fluid.

Case Report: We described a 12-year-old boy who developed shunt dependency syndrome following a CP shunting for treating a temporal arachnoid cyst. Read More

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http://dx.doi.org/10.1007/s00381-018-4014-4DOI Listing
January 2019
3 Reads

Stability-Sparing Endoscopic Endonasal Odontoidectomy in a Malformative Craniovertebral Junction: Case Report and Biomechanical Considerations.

Acta Neurochir Suppl 2019 ;125:229-233

Neurosurgical Unit, Surgical Department, Azienda Ospedaliera SS Antonio e Biagio e Cesare Arrigo, Alessandria, Italy.

Background: The craniovertebral junction (CVJ) is often involved in a wide range of congenital, developmental and acquired pathologies that can create bony and ligamentous instability or cause direct compression on the medulla and cervical spine cord, resulting in significant impairment. Atlas assimilation is the most common malformation in the CVJ and can be frequently associated with basilar invagination (BI) and Chiari malformation (CM) type I. Posterior atlas assimilation more frequently leads to BI type II with a mass effect on neural structures but usually no signs of biomechanical instability. Read More

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http://dx.doi.org/10.1007/978-3-319-62515-7_32DOI Listing
January 2019
1 Read

Surgical Treatment of Chiari Malformation in Adults: Comparison of Surgical Techniques Described in the Literature and Our Experience.

Acta Neurochir Suppl 2019 ;125:139-143

Department of Neurosurgery, Policlinico "A. Gemelli", University "Cattolica", Rome, Italy.

This paper has been edited for clarity, correctness and consistency with our house style. Please check it carefully to make sure the intended meaning has been preserved. If the intended meaning has been inadvertently altered by the editing changes, please make any corrections needed. Read More

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http://dx.doi.org/10.1007/978-3-319-62515-7_20DOI Listing
January 2019

Treatment of Holocord Syringomyelia-Chiari Complex by Posterior Fossa Decompression and a Syringosubarachnoid Shunt in a Single-Stage Single Approach.

Acta Neurochir Suppl 2019 ;125:133-138

Division of Neurosurgery, University of Messina, Messina, Italy.

Background: Posterior fossa decompression with expansive duraplasty is the first-line surgical approach for the treatment of symptomatic syringomyelia associated with Chiari malformation. Despite good decompression, the clinical failure rate is reported to be up to 26%. A syringosubarachnoid (S-S) shunt may be used as a secondary option. Read More

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http://link.springer.com/10.1007/978-3-319-62515-7_19
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http://dx.doi.org/10.1007/978-3-319-62515-7_19DOI Listing
January 2019
1 Read

Surgical Treatment in Symptomatic Chiari Malformation Type I: A Series of 25 Adult Patients Treated with Cerebellar Tonsil Shrinkage.

Acta Neurochir Suppl 2019 ;125:125-131

Division of Neurosurgery, ARNAS Civico Hospital, Palermo, Italy.

Background: The variety of symptoms and radiological findings in patients with Chiari malformation type I makes both the indication for surgery and the technical modality controversial. We report our 5-year experience, describing our technique and critically evaluating the clinical results.

Methods: Between 2012 and 2016, 25 patients (15 female and 10 male; mean age 39. Read More

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http://dx.doi.org/10.1007/978-3-319-62515-7_18DOI Listing
January 2019

Bony Decompression for Chiari Malformation Type I: Long-Term Follow-Up.

Acta Neurochir Suppl 2019 ;125:119-124

Paediatric Neurosurgery, Agostino Gemelli Hospital Foundation, Institute of Neurosurgery, Catholic University of Rome, Rome, Italy.

Background: Several surgical techniques are used for the management of Chiari malformation type I (CM-I). Bony posterior fossa decompression is considered a good option in children, though with a higher risk of requiring reoperation. However, there is not enough evidence from the series in the literature, which are often limited by inadequate follow-up. Read More

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http://dx.doi.org/10.1007/978-3-319-62515-7_17DOI Listing
January 2019

The Relationship Between Basilar Invagination and Chiari Malformation Type I: A Narrative Review.

Acta Neurochir Suppl 2019 ;125:111-118

Department of Neurosurgery, Xinhua Hospital, Affiliated to Shanghai Jiao Tong University School of Medicine, Shanghai, P. R. China.

Basilar invagination (BI) and Chiari malformation type I CM-I) are the most common adult craniovertebral junction malformations, and they are frequently associated with each other and present synchronously. The relationship between BI and CM-I has remained incompletely understood, and the choice of surgical strategy has remained controversial. This brief review focuses on the different aspects of BI and CM-I, and further discusses the relationship between these two concomitant pathologies on the basis of the concepts proposed over the last three decades. Read More

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http://link.springer.com/10.1007/978-3-319-62515-7_16
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http://dx.doi.org/10.1007/978-3-319-62515-7_16DOI Listing
January 2019
9 Reads

Atlantoaxial Fixation for Treatment of Chiari Formation and Syringomyelia with No Craniovertebral Bone Anomaly: Report of an Experience with 57 Cases.

Acta Neurochir Suppl 2019 ;125:101-110

Department of Neurosurgery, KEM Hospital and Seth GS Medical College, Mumbai, India.

Aim: In this paper we evaluate the role of atlantoaxial instability in the pathogenesis of Chiari formation type I and the role of atlantoaxial stabilization for treatment of this condition in cases with no obvious bone malformation in the region of the craniovertebral junction.

Materials, Methods And Results: During the period from January 2010 to July 2016, we identified 57 cases of Chiari formation where there was no bone malformation or evidence of craniovertebral junction instability that could be diagnosed on the basis of an abnormal increase in the atlantodental interval on dynamic imaging. Forty-eight of these patients had syringomyelia. Read More

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http://dx.doi.org/10.1007/978-3-319-62515-7_15DOI Listing
January 2019

The Role of Arachnoid Veils in Chiari Malformation Associated with Syringomyelia.

Acta Neurochir Suppl 2019 ;125:97-99

Institute of Neurosurgery, Catholic University School of Medicine, Rome, Italy.

Chiari malformation type I (CM-I), or hindbrain herniation syndrome, has traditionally been defined as a dislocation of the cerebellar tonsils 5 mm or more below the foramen magnum on sagittal magnetic resonance imaging (MRI) [1, 2]. An association of this anomaly with syringomyelia is observed in 45-68% of patients [3, 4]. Read More

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http://dx.doi.org/10.1007/978-3-319-62515-7_14DOI Listing
January 2019

Chiari Malformations.

Acta Neurochir Suppl 2019 ;125:89-95

Department of Neurology and Psychiatry, Division of Neurosurgery, "Sapienza" University of Rome, Rome, Italy.

Background: Chiari malformations (CM) represent a group of anomalies characterized by descent of the cerebellar tonsils or vermis into the cervical spinal canal. These malformations can be associated with abnormalities such as hydrocephalus, spina bifida, hydromyelia, syringomyelia, curvature of the spine (kyphosis and scoliosis) and tethered cord syndrome. Hereditary syndromes and other disorders that affect growth and bone formation-such as craniosynostosis, Ehlers-Danlos syndromes and Klippel-Feil syndrome-can also be associated with CM. Read More

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http://link.springer.com/10.1007/978-3-319-62515-7_13
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http://dx.doi.org/10.1007/978-3-319-62515-7_13DOI Listing
January 2019
9 Reads

Type II Abernethy Malformation in a Patient with Primary Budd-Chiari Syndrome.

Ann Hepatol 2018 Dec;18(1):246-249

Department of Intervention, First Affiliated Hospital of Zhengzhou University, Zhengzhou, Henan, China.

Budd-Chiari syndrome (BCS) is a heterogeneous group of disorders characterized by hepatic venous outflow obstruction. Abernethy malformation is a congenital vascular malformation defined by diversion of portal blood away from the liver. Both conditions are rare vascular diseases. Read More

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http://dx.doi.org/10.5604/01.3001.0012.7933DOI Listing
December 2018
2 Reads

Central Hypothyroidism and Novel Clinical Phenotypes in Hemizygous Truncation of .

J Endocr Soc 2019 Jan 23;3(1):119-128. Epub 2018 Nov 23.

Thyroid Molecular Laboratory, Institute for Medical and Molecular Genetics (INGEMM), La Paz University Hospital, Autonomous University of Madrid, Madrid, Spain.

Transducin -like 1 X-linked () gene encodes a subunit of the nuclear corepressor-silencing mediator for retinoid and thyroid hormone receptor complex (NCoR-SMRT) involved in repression of thyroid hormone action in the pituitary and hypothalamus. defects were recently associated with central hypothyroidism and hearing loss. The current study aims to describe the clinical and genetic characterization of a male diagnosed with central hypothyroidism through thyroid hormone profiling, TRH test, brain MRI, audiometry, and psychological evaluation. Read More

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https://academic.oup.com/jes/article/3/1/119/5198610
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http://dx.doi.org/10.1210/js.2018-00144DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6300407PMC
January 2019
5 Reads

Review of first clinical experiences with a 1.5 Tesla ceiling-mounted moveable intraoperative MRI system in Europe.

Bosn J Basic Med Sci 2018 Dec 27. Epub 2018 Dec 27.

Department of Neurosurgery, University of Tuebingen Medical Center, Germany.

High-field intraoperative MRI (iMRI) systems provide excellent imaging quality and are used for resection control and update of image guidance systems in a number of centers. A ceiling-mounted intraoperative MRI system has several advantages compared to a conventional iMRI system. In this article, we report on first clinical experience with using such a state-of-the-art, the 1. Read More

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http://dx.doi.org/10.17305/bjbms.2018.3777DOI Listing
December 2018
2 Reads

Incidence of Intraspinal and Extraspinal MRI Abnormalities in Patients With Adolescent Idiopathic Scoliosis.

Spine Deform 2019 01;7(1):47-52

Division of Pediatric Orthopaedic Surgery, Hospital for Special Surgery, 535 East 70th Street, New York, NY 10021, USA.

Study Design: Retrospective study with follow-up.

Objectives: The purpose of this study was to determine the incidence of intraspinal and extraspinal MRI abnormalities in a consecutive series of patients with adolescent idiopathic scoliosis (AIS) and to describe the evaluation and management of these abnormalities.

Summary Of Background Data: Indications for preoperative magnetic resonance imaging (MRI) in patients with AIS remain controversial. Read More

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http://dx.doi.org/10.1016/j.jspd.2018.06.006DOI Listing
January 2019
2 Reads

[The simple posterior operation for different types of malformations in the craniocervical junction with atlantoaxial dislocation].

Authors:
Y Y Li M H Li N Z Yu

Zhonghua Yi Xue Za Zhi 2018 Dec;98(47):3888-3891

Department of Neurosurgery, the First Affiliated Hospital of Nanchang University, Nanchang 330006, China.

To explore the therapeutic effect of simple posterior reduction and decompression technique for different types of malformations in the craniocervical junction with atlantoaxial dislocation. The clinical data of 34 cases of different malformations in the craniocervical junction who were treated by the simple posterior internal fixation and decompression surgery in the department of neurosurgery of the first affiliated hospital of Nanchang University from March 2014 to March 2017 were analyzed retrospectively.In 34 cases, including 26 cases of basilar invagination with atlantoaxial dislocation (19 cases with atlanto occipital fusion, 12 cases with Chiari malformation, 13 cases with syringomyelia), 8 cases of odontoid malformation with atlantoaxial dislocation (2 cases with Chiari malformation, 1 case with flat skull base, 1 case with malformation of atlas). Read More

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http://dx.doi.org/10.3760/cma.j.issn.0376-2491.2018.47.015DOI Listing
December 2018
3 Reads

Unexpected Progression of Tonsillar Herniation in Two Pediatric Cases with Chiari Malformation Type I and Review of the Literature.

Pediatr Neurosurg 2018 Dec 21:1-6. Epub 2018 Dec 21.

Background: Chiari malformation type 1 (CM-1) is a generally congenital, rarely acquired disease characterized with 5 mm or more displacement of cerebellar tonsils through foramen magnum.

Methods: Here, we report about 2 patients with CM-1 progressed in the degree of tonsillar herniation from our clinic, whereas increasing in prolapse of tonsillar herniation after diagnosis is extremely uncommon.

Results: The first patient aged 17 years was diagnosed with CM-1 in 2009 and was operated due to progression of 5 mm radiologically and worsening symptoms in 2014. Read More

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http://dx.doi.org/10.1159/000495066DOI Listing
December 2018

Headaches in Loyes-Dietz Syndrome.

Authors:
Debopam Samanta

J Child Neurol 2018 Dec 19:883073818815039. Epub 2018 Dec 19.

1 Department of Pediatrics, Neurology Section, University of Arkansas for Medical Sciences, Little Rock, AR, USA.

Loeys-Dietz syndrome is a rare connective tissue disorder characterized by cardiovascular, craniofacial, skeletal, and neurocognitive abnormalities. Recurrent headaches may constitute the neurological presentation of Loyes-Dietz syndrome in the absence of an intracranial aneurysm, subarachnoid hemorrhage, or dissection. The etiology of headaches occurring in Loeys-Dietz syndrome can be diverse but underrecognized and underreported. Read More

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http://dx.doi.org/10.1177/0883073818815039DOI Listing
December 2018
7 Reads

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
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6296028PMC
December 2018
1 Read

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
2 Reads

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 Reads
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
28 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
3 Reads

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
6 Reads

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
3 Reads