4,817 results match your criteria Chiari Malformation


Posterior calvarial distraction for complex craniosynostosis and cerebellar tonsillar herniation.

J Neurosurg Pediatr 2020 Jul 10:1-10. Epub 2020 Jul 10.

Departments of1Neurosurgery and.

Objective: Children with syndromic, multisuture, and lambdoid craniosynostosis undergoing calvarial surgery often have Chiari malformation type I (CM-I) (or cerebellar tonsillar herniation). The optimal management of this patient group, including the surgical techniques and timing of surgery, remains uncertain. Posterior calvarial distraction (PCD) is an effective method to increase the supratentorial cranial volume and improve raised intracranial pressure in children with complex craniosynostosis. Read More

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http://dx.doi.org/10.3171/2020.4.PEDS19742DOI Listing

Acquired Chiari malformation following spinal cord injury-a case series.

Spinal Cord Ser Cases 2019 Jul 9;5(1):65. Epub 2019 Jul 9.

Stoke Mandeville Hospital, Buckinghamshire Healthcare NHS Trust, Aylesbury, UK.

Introduction: Chiari malformation is characterized by caudal descent of the cerebellar tonsils through the foramen magnum. Acquired Chiari malformations (ACM) have previously been described after a variety of pathologies including lumbar puncture, cerebrospinal fluid (CSF) drainage, lumboperitoneal shunts, and conditions causing craniocephalic disproportion.

Case Presentation: We present four cases of ACM following spinal cord injury (SCI), which has not previously been described in the literature. Read More

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http://dx.doi.org/10.1038/s41394-019-0211-yDOI Listing

Adults with spina bifida: A cross-sectional study of health issues and living conditions.

Brain Behav 2020 Jul 7:e01736. Epub 2020 Jul 7.

Rehab Station Stockholm, Spinalis Outpatient Clinic, Solna, Sweden.

Objective: To describe health issues and living conditions in a cohort of adults living with Spina bifida.

Material And Methods: A cross-sectional study was conducted by a multidisciplinary team. Adults with spina bifida (n = 219) were invited to participate. Read More

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http://dx.doi.org/10.1002/brb3.1736DOI Listing

Cervical myelomeningocele with CSF leakage: a case-based review.

Childs Nerv Syst 2020 Jul 4. Epub 2020 Jul 4.

Neurosurgery Department, Hospital Regional Universitario de Málaga, Málaga, Spain.

Background: The cystic spinal dysraphism of the cervical and upper thoracic region (CDCT) accounts for only 3.9 to 8% of spina bifida cystica lesions. The presence of external cerebrospinal fluid (CSF) leakage is infrequent and very few authors have reported about surgical complications. Read More

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http://dx.doi.org/10.1007/s00381-020-04743-yDOI Listing

Central sleep apnea and Chiari 1 malformation in a pediatric patient with Klippel-Feil syndrome.

J Clin Sleep Med 2020 Jul 6. Epub 2020 Jul 6.

Department of Sleep Medicine, Baylor College of Medicine, Houston, Texas.

None: Klippel-Feil sequence (KFS) is a rare congenital condition that presents with congenital cervical spine fusion, reduced cervical spine flexion, and low posterior hairline. Chiari malformation type 1 (CM1) and sleep-disordered breathing (SDB) are frequent co-morbidities of KFS. The pathological basis of the connection between CM1 and SDB in the setting of KFS is not clearly understood. Read More

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http://dx.doi.org/10.5664/jcsm.8650DOI Listing

A retrospective study on outcomes following posterior fossa decompression with dural splitting surgery in patients with Chiari type I malformation.

Clin Neurol Neurosurg 2020 Jun 26;196:106035. Epub 2020 Jun 26.

Department of Neurosurgery, Shanghai Tenth People's Hospital, Tongji University School of Medicine, Shanghai 200072, People's Republic of China. Electronic address:

Background: Chiari type I malformation (CM I) is a congenital defect of the skull base and brain. Posterior fossa decompression alone or with duraplasty and partial resection of cerebellar tonsils is a standard approach. The effectiveness of other approaches such as the dural splitting technique is still under the study. Read More

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http://dx.doi.org/10.1016/j.clineuro.2020.106035DOI Listing
June 2020
1.248 Impact Factor

Prenatal visualization of the torcular herophili by means of a Doppler technology highly sensitive for low velocity flow in the expert assessment of the posterior fossa: a prospective study.

BJOG 2020 Jul 3. Epub 2020 Jul 3.

Department of Medicine and Surgery, Unit of Surgical Sciences, Obstetrics and Gynecology, University of Parma, Parma, Italy.

Objective: To evaluate the usefulness of a Doppler technology highly sensitive for low velocity flow in the antenatal imaging of the torcular herophili (TH) in the second trimester of pregnancy.

Design: Prospective study.

Setting: Referral Fetal Medicine Unit. Read More

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http://dx.doi.org/10.1111/1471-0528.16392DOI Listing

The SHORE score: a novel predictive tool for improvement after decompression surgery in adult Chiari malformation type I.

World Neurosurg 2020 Jun 26. Epub 2020 Jun 26.

Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, MD. Electronic address:

Background: A practical scoring system predicting significant improvement after surgical decompression in adult Chiari malformation type I (CM-1) based on validated outcome measures is lacking. We aimed to develop a simple score and improvement calculator to facilitate the decision-making process in symptomatic CM-1 patients.

Methods: We evaluated adult CM-1 patients who presented to our institution between September 2006 and September 2018 and underwent surgical decompression. Read More

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http://dx.doi.org/10.1016/j.wneu.2020.06.175DOI Listing

Endoscopic endonasal approaches to the craniovertebral junction: The Otolaryngologist's perspective.

World J Otorhinolaryngol Head Neck Surg 2020 Jun 16;6(2):94-99. Epub 2020 Mar 16.

Department of Otolaryngology - Head & Neck Surgery, Weill Cornell Medical College - New York, NY, USA.

Objective: To review indications and techniques for the endoscopic endonasal approach to the craniovertebral junction (CVJ), analyze postoperative outcomes, and discuss important technical considerations.

Methods: A retrospective analysis was performed on all patients undergoing endonasal endoscopic approaches to the CVJ from May 2007 to June 2017. Demographic information, presenting symptoms, imaging results, treatment course, postoperative functional status, and follow-up were recorded. Read More

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http://dx.doi.org/10.1016/j.wjorl.2020.01.001DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7296474PMC

Risk factors of unplanned neurosurgery for scoliotic patients with Chiari malformation type I and syringomyelia after spinal deformity correction.

Clin Neurol Neurosurg 2020 Jun 20;196:106014. Epub 2020 Jun 20.

Department of Orthopedic Surgery and Orthopedic Research Institute, West China Hospital and West China School of Medicine, Sichuan University, Chengdu, China. Electronic address:

Objectives: It remains unclear which subgroups of scoliotic patients with CMI and syringomyelia are more likely to undergo unplanned neurosurgery after spinal deformity correction. The purpose of this study is to explore risk factors of unplanned neurosurgery for scoliotic patients with CMI and syringomyelia after spinal deformity correction.

Patients And Methods: This cohort consisted of 62 scoliotic patients with CMI and syringomyelia who underwent spinal deformity surgery with a mean follow-up of 4. Read More

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http://dx.doi.org/10.1016/j.clineuro.2020.106014DOI Listing

Confounding clinical presentation and different disease progression in CMT4B1.

Neuromuscul Disord 2020 May 16. Epub 2020 May 16.

APHP, Centre de référence des maladies neuromusculaires, Institut de Myologie, Sorbonne Université, APHP, Hôpital Pitié-Salpêtrière; Paris, France. Electronic address:

We report seven Charcot-Marie-Tooth 4B1 (CMT4B1) patients from four families with distinctive features, presenting with severe distal weakness and cranial nerve involvement. Patient from family 1 presented with congenital varus foot deformity, progressive distal and proximal weakness leading to loss of ambulation at 14 years, bilateral facial palsy and prominent bulbar involvement. In three siblings from family 2, still ambulant in the second decade, neuropathy was associated with marked sweating and Arnold-Chiari syndrome. Read More

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http://dx.doi.org/10.1016/j.nmd.2020.05.003DOI Listing

Posterior cranial fossa maldevelopment in infants with repaired open myelomeningoceles. Double trouble or a dynamic process of posterior cranial fossa abnormalities?

World Neurosurg 2020 Jun 22. Epub 2020 Jun 22.

Fondazione Policlinico Universitario Agostino Gemelli IRCC, Roma-UOC Radiologia e Neuroradiologia, Polo Diagnostica per immagini, radioterapia, oncologia ed ematologia, Area diagnostica per immagini, Rome, Italy; Istituto di Radiologia, Università Cattolica del Sacro Cuore, Rome, Italy.

Objective: Two degrees of posterior cranial fossa (PCF) maldevelopment can be hypothesized in children with myelomeningocele (MMC). This paper investigate the PCF deformation by quantitative MRI analysis in MMC subjects with and without Chiari 2 malformation (CM2).

Methods: Posterior cranial fossa volume (PCFV), posterior cranial fossa brain volume (PCFBV), lengths of PCF, ventriculomegaly, level and extension of the dysraphism were analyzed in magnetic resonance imaging (MRI) of 51 newborns with MMC surgically repaired at birth (and 41 controls). Read More

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http://dx.doi.org/10.1016/j.wneu.2020.06.106DOI Listing

Posterior fossa decompression with duraplasty in Chiari malformation type 1: a systematic review and meta-analysis.

Acta Neurochir (Wien) 2020 Jun 23. Epub 2020 Jun 23.

Royal Sussex County Hospital, Brighton and Sussex University Hospitals NHS Trust, Brighton, UK.

Background: Surgery for symptomatic Chiari type I malformation (CM-I) patients include posterior fossa decompression (PFD) involving craniectomy with or without dural opening, and posterior fossa decompression with duraplasty (PFDD). This review aims to examine the evidence to aid surgical decision-making.

Methods: A medical database search was expanded to include article references to identify all relevant published case series. Read More

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http://dx.doi.org/10.1007/s00701-020-04403-9DOI Listing

INCIDENTAL FINDINGS ON BRAIN MAGNETIC RESONANCE IMAGING (MRI) IN PEDIATRIC ENDOCRINE PATIENTS.

Endocr Pract 2020 Jun 23. Epub 2020 Jun 23.

From: Pediatric Endocrinology and Diabetes Unit.

To explore incidental findings on brain magnetic resonance imaging (MRI) studies of pediatric patients referred due to endocrine disorders. A retrospective, observational study conducted in a tertiary referral center. The neuroimaging database of 17,445 brain MRI studies of 11,011 pediatric patients were searched for cases with endocrine referrals and without medical history of malignancy, genetic syndromes and/or neurological comorbidities. Read More

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http://dx.doi.org/10.4158/EP-2020-0208DOI Listing

Spontaneous cerebrospinal fluid leak via foramen rotundum in a non-obese male presented as pseudo-Chiari malformation type I: a case report and literature review.

J Int Med Res 2020 Jun;48(6):300060520924212

Department of Otolaryngology, University of Medicine and Pharmacy at Ho Chi Minh City, Ho Chi Minh City, Vietnam.

Spontaneous cerebrospinal fluid (sCSF) leak from the skull base has been previously reported, but there are few reports of sCSF leak from the foramen rotundum due to its rare occurrence. This case report describes a 15-year-old male patient that presented with left side watery rhinorrhoea that had been present since he was 4 years of age and a history of repeated bouts of meningitis of unknown cause. A discharge sample from the nose tested positive for beta-2 transferrin. Read More

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http://dx.doi.org/10.1177/0300060520924212DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7313344PMC

Outcomes of dura-splitting technique compared to conventional duraplasty technique in the treatment of adult Chiari I malformation: a systematic review and meta-analysis.

Neurosurg Rev 2020 Jun 22. Epub 2020 Jun 22.

Faculty of Medicine, Islamic Azad University of Mashhad, Mashhad, Iran.

Chiari malformation type I is a developmental abnormality with an array of surgical techniques introduced for the management of it. The most common technique is foramen magnum decompression with duraplasty. Dura-splitting technique as one of the non-dura-opening techniques is a less known procedure that spares the internal layer of the dura and can theoretically result in fewer complications compared to duraplasty. Read More

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http://dx.doi.org/10.1007/s10143-020-01334-yDOI Listing

Surgical Scoliosis Correction in Chiari-I Malformation with Syringomyelia Versus Idiopathic Syringomyelia.

J Bone Joint Surg Am 2020 Jun 10. Epub 2020 Jun 10.

Department of Orthopedics, Peking Union Medical College Hospital and Graduate School of Peking Union Medical College, Peking Union Medical College, Chinese Academy of Medical Science, Beijing, People's Republic of China.

Background: Both patients with Chiari-I malformation (CIM) with syringomyelia and those with idiopathic syringomyelia (ISm) have a syrinx and can have scoliosis as well. However, there is no literature regarding differences between CIM and ISm in terms of radiographic outcomes and surgical complications after posterior fusion, to our knowledge. The aim of the present study was to compare radiographic features, clinical outcomes, and surgical complications after posterior spinal fusion between patients with CIM-associated scoliosis and those with ISm-associated scoliosis. Read More

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http://dx.doi.org/10.2106/JBJS.20.00058DOI Listing

Clinicoradiographic data and management of children with Chiari malformation type 1 and 1.5: an Italian case series.

Acta Neurol Belg 2020 Jun 10. Epub 2020 Jun 10.

Department of General Surgery and Medical Surgical Specialties, Section of Orthopaedics and Traumatologic Surgery, University of Catania, 95123, Catania, Italy.

Introduction: The widespread use of imaging has increased Chiari malformation (CM) diagnosis. CM shows clinical heterogeneity that makes management controversial. We aimed to evaluate the occurrence and clinical and radiographic presentation of children with CM-1 and CM-1. Read More

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http://dx.doi.org/10.1007/s13760-020-01398-zDOI Listing

Severe Cerebellar Degeneration and Chiari I Malformation - Speculative pathophysiology based on a systematic review.

Rev Assoc Med Bras (1992) 2020 Mar;66(3):375-379

. Professor de Neurocirurgia - Universidade de Campinas (UNICAMP), Campinas-SP Brasil.

Background: Symptomatic Chiari Type I Malformation (CM) is treated with posterior fossa decompression with or without duroplasty. We have noticed some cases with concomitant severe cerebellar ataxia due to cerebellar atrophy. The aim of this study is to review the literature of CM associated with severe cerebellar atrophy and discuss its potential physiopathology. Read More

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http://dx.doi.org/10.1590/1806-9282.66.3.375DOI Listing

Pre-operative headaches and obstructive hydrocephalus predict an extended length of stay following suboccipital decompression for pediatric Chiari I malformation.

Childs Nerv Syst 2020 Jun 9. Epub 2020 Jun 9.

Department of Neurosurgery, Yale University School of Medicine, 333 Cedar Street, New Haven, CT, 06520, USA.

Purpose: For young children and adolescents with Chiari malformation type I (CM-I), the determinants of extended length of hospital stay (LOS) after neurosurgical suboccipital decompression are obscure. Here, we investigate the impact of patient- and hospital-level risk factors on extended LOS following surgical decompression for CM-I in young children to adolescents.

Methods: The Kids' Inpatient Database year 2012 was queried. Read More

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http://dx.doi.org/10.1007/s00381-020-04688-2DOI Listing

Chiari malformation type I and basilar invagination originating from atlantoaxial instability: a literature review and critical analysis.

Acta Neurochir (Wien) 2020 Jul 6;162(7):1553-1563. Epub 2020 Jun 6.

Department of Neurosurgery, Klinikum rechts der Isar, Technical University Munich School of Medicine, Ismaninger Str. 22, 81675, Munich, Germany.

Introduction: Recently, a novel hypothesis has been proposed concerning the origin of craniovertebral junction (CVJ) abnormalities. Commonly found in patients with these entities, atlantoaxial instability has been suspected to cause both Chiari malformation type I and basilar invagination, which renders the tried and tested surgical decompression strategy ineffective. In turn, C1-2 fusion is proposed as a single solution for all CVJ abnormalities, and a revised definition of atlantoaxial instability sees patients both with and without radiographic evidence of instability undergo fusion, instead relying on the intraoperative assessment of the atlantoaxial joints to confirm instability. Read More

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http://dx.doi.org/10.1007/s00701-020-04429-zDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7295832PMC

The novel duplication HRAS c.186_206dup p.(Glu62_Arg68dup): clinical and functional aspects.

Eur J Hum Genet 2020 Jun 4. Epub 2020 Jun 4.

Institute of Human Genetics, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.

Specific activating missense HRAS variants cause Costello syndrome (CS), a RASopathy with recognizable facial features. The majority of these dominant disease causing variants affect the glycine residues in position 12 or 13. A clinically suspected CS diagnosis can be confirmed through identification of a dominant pathogenic HRAS variant. Read More

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http://dx.doi.org/10.1038/s41431-020-0662-4DOI Listing

Paradoxical Vocal Cord Motion Presaging Bilateral Vocal Cord Paresis in an Infant.

Cureus 2020 Apr 27;12(4):e7853. Epub 2020 Apr 27.

Otolaryngology, Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, Buffalo, USA.

Paradoxical vocal cord motion (PVCM) is a condition characterized by inappropriate adduction of the vocal cords during respiration. Usually seen in children and adolescents, PVCM presentation in infants is uncommon. Once thought to be a product of psychiatric disease, there are now several other proposed etiologies including irritant-induced and secondary to neurologic disease. Read More

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http://dx.doi.org/10.7759/cureus.7853DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7255064PMC

Surgical management of Chiari malformation type II.

Childs Nerv Syst 2020 May 30. Epub 2020 May 30.

Department of Neurosurgery, ASST Niguarda, P.za Ospedale Maggiore 3, 20162, Milan, Italy.

Background: Chiari malformation type II is present in almost all patients with myelomeningocele but usually remains asymptomatic. Symptoms are generally more severe in neonates, who have the worst prognosis. The association symptoms/hydrocephalus is well known, and first treatment usually consists of ensuring adequate ventricular drainage. Read More

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http://dx.doi.org/10.1007/s00381-020-04675-7DOI Listing
May 2020
1.163 Impact Factor

Hindbrain-related syringomyelia and raised intra-abdominal pressure: implications for safety of laparoscopic and robotic surgery.

Ann Ital Chir 2020 May 25;9. Epub 2020 May 25.

Background: The causative role played by intra-abdominal pressure (IAP) in the syringogenesis of the Chiari 1 malformation syringomyelia has been still not adequately studied. The aim of this study is to validate the transmedullary theory about the hindbrain-related syrinx, also discussing the implications for safety of these patients related to the use of high-pressure CO2 pneumoperitoneum during laparoscopic and robotic surgery.

Methods: Fourteen patients with a hindbrain-related syrinx were candidate for a posterior fossa decompression. Read More

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Comparison of surgical outcomes in patients with Chiari Type I malformation receiving posterior fossa decompression with and without duraplasty.

Childs Nerv Syst 2020 Jul 25;36(7):1399-1405. Epub 2020 May 25.

Donald & Barbara Zucker School of Medicine at Hofstra/Northwell, 500 Hofstra Boulevard, Hempstead, NY, 11549, USA.

Purpose: The goals of this study were to compare clinical outcomes in patients with Chiari Malformation Type I (CMI) receiving posterior fossa decompression with (PFDD) or without duraplasty (PFD).

Methods: We conducted a retrospective analysis of 178 consecutive cases of 157 patients undergoing PFDD or PFD for CMI at Cohen Children's Medical Center between 2007 and 2017. Clinical improvement was defined as a clear reduction of preoperative symptoms after surgery as reported by patients and parents. Read More

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http://dx.doi.org/10.1007/s00381-020-04667-7DOI Listing

Cerebellar syndrome after occipital nerve block. A case report.

Cephalalgia 2020 May 25:333102420929015. Epub 2020 May 25.

Neurology Unit, Department of Medicine, Hopitaux Robert Schuman, Luxembourg, Luxembourg.

Background: Occipital nerve blocks are commonly used in the treatment of different types of refractory headaches. The procedure is considered safe, and serious complications have rarely been described.

Case Presentation: We report a serious complication of occipital nerve blockade secondary to the penetration of local anesthetic and non-steroidal anti-inflammatory drugs into the posterior fossa in a patient affected by type I Arnold Chiari malformation. Read More

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http://dx.doi.org/10.1177/0333102420929015DOI Listing

Chiari type I malformation: role of the Chiari Severity Index and Chicago Chiari Outcome Scale.

J Neurosurg Pediatr 2020 May 22:1-7. Epub 2020 May 22.

1Surgical Outcome Center for Kids, Monroe Carell Jr. Children's Hospital at Vanderbilt, Nashville, Tennessee.

Objective: The authors aimed to determine whether the Chiari Severity Index (CSI), and other clinical variables, can be used as a predictor of postoperative outcomes for Chiari type I malformation (CM-I) using the modified Chicago Chiari Outcome Scale (mCCOS) as the postoperative measure.

Methods: The cohort included patients 18 years of age and younger who were treated for CM-I between 2010 and 2015 who had at least 12 months of clinical and radiographic follow-up. CSI grades were assigned using preoperative clinical and neuroimaging data. Read More

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http://dx.doi.org/10.3171/2020.2.PEDS19770DOI Listing

Chiari I malformation with neurogenic hypertension after suboccipital decompression.

Childs Nerv Syst 2020 May 20. Epub 2020 May 20.

Pediatric Neurosurgery Department, National Neuroscience Institute, King Fahad Medical City, Riyadh, Saudi Arabia.

Patients with Chiari I malformations have a spectrum of symptoms related to craniocervical structural compression. Neurogenic hypertension has been reported in some patients with this disorder, with resolution of hypertension after suboccipital decompressions in some cases. The pathophysiology of neurogenic hypertension in these patients is thought to be related to ventrolateral medullary compression. Read More

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http://dx.doi.org/10.1007/s00381-020-04670-yDOI Listing

Pathological and Incidental Findings in 403 Taiwanese Girls With Central Precocious Puberty at Initial Diagnosis.

Front Endocrinol (Lausanne) 2020 5;11:256. Epub 2020 May 5.

Department of Pediatrics, Chang Gung Memorial Hospital, Taoyuan, Taiwan.

Whether girls with central precocious puberty (CPP) should undergo routine brain magnetic resonance imaging (MRI) to identify potential intracranial pathologies is controversial. To evaluate the brain MRI results of girls with CPP and identify the clinical and endocrine predictors of brain abnormalities. This retrospective study obtained data from pediatric endocrine clinics at Chang Gung Children's Hospital. Read More

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http://dx.doi.org/10.3389/fendo.2020.00256DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7214687PMC

Correction to: Clinical Chiari syndrome or anatomical Chiari malformation? A conundrum revisited.

Acta Neurochir (Wien) 2020 Jul;162(7):1529

Department of Neurosurgery Western General Hospital Edinburgh, Edinburgh, UK.

An incorrect set of affiliations for two authors (Tiit Illimar Mathiesen and Andreas K. Demetriades). Read More

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http://dx.doi.org/10.1007/s00701-020-04389-4DOI Listing

Additional Assessment of Developed Occipital Sinus Using Intraoperative Indocyanine Green Videoangiography for a Safe Foramen Magnum Decompression-Technical Case Report.

Oper Neurosurg (Hagerstown) 2020 May 18. Epub 2020 May 18.

Department of Neurosurgery, Nara Medical University, Kashihara, Japan.

Background And Importance: Although foramen magnum decompression (FMD) with expansive duraplasty is a popular procedure for treating chiari malformation (CM), the common Y-shaped dural incision can lead to a life-threatening cerebral venous circulation disturbance in patients with a developed occipital sinus. Here, we describe the effectiveness of intraoperative indocyanine green video angiography (ICG-VA) for a CM type 1 (CM1) patient with a highly developed unilateral occipital sinus.

Clinical Presentation: A 40-yr-old woman presented with sensory disturbance on the left side of the body. Read More

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http://dx.doi.org/10.1093/ons/opaa125DOI Listing

Clivus length distinguishes between asymptomatic healthy controls and symptomatic adult women with Chiari malformation type I.

Neuroradiology 2020 May 16. Epub 2020 May 16.

Conquer Chiari Research Center, Department of Biomedical Engineering, The University of Akron, Akron, OH, USA.

Purpose: While the presence of cerebellar tonsillar descent in radiological images has been used as evidence of Chiari malformation type I (CMI), tonsillar ectopia alone is insufficient to identify individuals with symptomatic CMI. This study sought to identify differences in brain morphology between symptomatic CMI and healthy controls in adult females.

Methods: Two hundred and ten adult females with symptomatic CMI and 90 age- and body mass index-matched asymptomatic female controls were compared using seven brain morphometric measures visible on magnetic resonance images. Read More

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http://dx.doi.org/10.1007/s00234-020-02453-5DOI Listing

Determinants of Chiari I progression in pregnancy.

J Clin Neurosci 2020 Jul 7;77:1-7. Epub 2020 May 7.

Department of Pediatric Neurosurgery, Shriners Hospital for Children Philadelphia, PA, USA.

Chiari Malformation type 1 (CM-I) is congenital or an acquired anomaly of the hind brain; develops when the cerebellar tonsils recede downwards below the foramen magnum. Recurrent post tussive suboccipital headache is the common presentation in a pregnant woman and the diagnosis is usually missed or delayed due to lack of formal understanding of this neurological pathology. Much has been written regarding presentation, morphology and the treatment of CM-I; however, little is known when the etiology is acquired or an iatrogenic in its evolution. Read More

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http://dx.doi.org/10.1016/j.jocn.2020.05.026DOI Listing

Medical infrared thermal imaging of syringomyelia in the Cavalier King Charles Spaniel.

BMC Vet Res 2020 May 14;16(1):137. Epub 2020 May 14.

The Canine Chiari Institute at Long Island Veterinary Specialists, 163 South Service Road, Plainview, NY, 11803, USA.

Background: Medical infrared thermal imaging (MITI) is a non-invasive imaging modality gaining popularity in the veterinary field. An infrared camera captures emission of heat and creates a color map in the form of a thermogram. Topical heat emission is influenced by localized disease processes as a result of autonomic nervous system imbalance. Read More

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http://dx.doi.org/10.1186/s12917-020-02354-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7227106PMC

Resolution of Tonsillar Herniation and Syringomyelia Following Resection of a Large Anterior Frontal Parasagittal Meningioma.

Cureus 2020 Apr 11;12(4):e7636. Epub 2020 Apr 11.

Neurosurgery, New York University (NYU) School of Medicine, New York, USA.

Chiari I malformation is the herniation of cerebellar tonsils below the level of the foramen magnum due to congenital or acquired pathologies. Acquired Chiari I malformation (ACM) may occur secondary to space-occupying lesions (SOLs), such as intracranial tumors due to elevated intracranial pressure (ICP), and can be accompanied by syringomyelia. ACM and syringomyelia have been shown to resolve after resection of the SOL, without the need for adjuvant posterior fossa decompression. Read More

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http://dx.doi.org/10.7759/cureus.7636DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7213766PMC

Evidence of Neural Microstructure Abnormalities in Type I Chiari Malformation: Associations Among Fiber Tract Integrity, Pain, and Cognitive Dysfunction.

Pain Med 2020 May 10. Epub 2020 May 10.

Department of Neurosurgery, Johns Hopkins Medical Center, Baltimore, Maryland, USA.

Background: Previous case-control investigations of type I Chiari malformation (CMI) have reported cognitive deficits and microstructural white matter abnormalities, as measured by diffusion tensor imaging (DTI). CMI is also typically associated with pain, including occipital headache, but the relationship between pain symptoms and microstructure is not known.

Methods: Eighteen female CMI patients and 18 adult age- and education-matched control participants underwent DTI, were tested using digit symbol coding and digit span tasks, and completed a self-report measure of chronic pain. Read More

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http://dx.doi.org/10.1093/pm/pnaa094DOI Listing

Suboccipital decompressive craniectomy in a case of complex Chiari malformation complicated with influenza A (H1N1) necrotizing encephalopathy.

Childs Nerv Syst 2020 Jul 6;36(7):1335-1336. Epub 2020 May 6.

Department of Neurosurgery, Aydın Adnan Menderes University Faculty of Medicine, 09100 Efeler, Aydın, Turkey.

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http://dx.doi.org/10.1007/s00381-020-04659-7DOI Listing

[Etiological diagnosis and clinical evaluation of isolated fetal ascites].

Zhonghua Fu Chan Ke Za Zhi 2020 Apr;55(4):246-252

Department of Obstetrics and Gynecology, Provincial Hospital Affiliated to Shandong University, Jinan 250021, China.

To explore the correlation between prenatal clinical data with etiological diagnosis and neonatal outcome in isolated fetal ascites. Totally, 36 pregnancy cases diagnosed as isolated fetal ascites by ultrasound in Provincial Hospital Affiliated to Shandong University from June 22nd, 2016 to September 28th, 2018 were collected. Invasive prenatal diagnosis was performed by taking fetal cord blood, amniotic fluid, and fetal ascites respectively for cytogenetics, molecular genetics and biochemical examination and the impact of intrauterine therapeutic procedures on neonatal outcomes was evaluated as well. Read More

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http://dx.doi.org/10.3760/cma.j.cn112141-20190605-00312DOI Listing

Ocular Findings in the 16p11.2 Microdeletion Syndrome: A Case Report and Literature Review.

Case Rep Pediatr 2020 20;2020:2031701. Epub 2020 Apr 20.

Department of Human and Molecular Genetics, Virginia Commonwealth University School of Medicine, Richmond, VA, USA.

The recurrent 16p11.2 microdeletion is characterized by developmental delays and a wide spectrum of congenital anomalies. It has been well reported that individuals with this ∼593-kb interstitial deletion have an increased susceptibility toward the autism spectrum disorder (ASD). Read More

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http://dx.doi.org/10.1155/2020/2031701DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7189309PMC

A Novel Risk Calculator for Suboccipital Decompression for Adult Chiari Malformation.

World Neurosurg 2020 Apr 29. Epub 2020 Apr 29.

Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, MD. Electronic address:

Background: Patient counselling and selection for surgical therapy in adult Chiari malformation type I (CM-1) remains debatable. We aimed to develop a clinical calculator predicting the risk of non-home discharge and re-operation using the American College of Surgeons - National Surgical Quality Improvement Program (ACS-NSQIP) database.

Methods: The database from years 2011 through 2017 was queried to identify the subset of CM-1 patients undergoing suboccipital decompression. Read More

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http://dx.doi.org/10.1016/j.wneu.2020.04.169DOI Listing

External validation of current prediction systems of improvement after decompression surgery in Chiari malformation type I patients: can we do better?

J Neurosurg 2020 May 1:1-6. Epub 2020 May 1.

1Department of Neurosurgery, Johns Hopkins University School of Medicine.

Objective: The Chiari Severity Index (CSI) and points-based algorithm of Thakar et al. are two prognostic tools that have been developed to predict the likelihood of improvement after suboccipital decompression in adult patients with Chiari malformation type I (CM-I). This study aimed to externally validate and critically evaluate these algorithms in the interest of guiding the development of improved prediction systems. Read More

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http://dx.doi.org/10.3171/2020.2.JNS20181DOI Listing

A Rare Cause of Adrenal Insufficiency - Isolated ACTH Deficiency Due to TBX19 Mutation: Long-Term Follow-Up of Two Cases and Review of the Literature.

Horm Res Paediatr 2019 28;92(6):395-403. Epub 2020 Apr 28.

Department of Pediatric Endocrinology, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey.

Introduction: Isolated adrenocorticotropic hormone (ACTH) deficiency (IAD) is a rare cause of adrenal insufficiency and T-box pituitary restricted transcription factor (TBX19) mutations are responsible for two-thirds of the neonatal onset form of the disease. IAD presents with hypoglycemia and prolonged jaundice in the neonatal period. TBX19 is important for both pro-opiomelanocortin (POMC) gene transcription and differentiation of POMC-expressing cells. Read More

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http://dx.doi.org/10.1159/000506740DOI Listing

Myelomeningocele with Associated Anomalies – Case Report and Literature Review.

Folia Med (Plovdiv) 2019 09;61(3):462-466

Medical University, Plovdiv, Bulgaria.

Myelomeningocele is a common defect of the development of the neural tube. It is a complex congenital malformation of the central nervous system (CNS) that can be associated with other concurrent anomalies. We report on a case of lumbar myelomeningocele with concomitant CNS malformations we followed up over a period of 15 years. Read More

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http://dx.doi.org/10.3897/folmed.61.e39415DOI Listing
September 2019

MRI utilization and rates of abnormal pretreatment MRI findings in early-onset scoliosis: review of a global cohort.

Spine Deform 2020 Apr 24. Epub 2020 Apr 24.

Department of Orthopaedic Surgery, University of California, San Diego, San Diego, CA, USA.

Study Design: Retrospective review OBJECTIVES: To report the frequency of pretreatment magnetic resonance imaging (MRI) utilization and rates and types of intra-spinal abnormalities identified on MRI in patients with early-onset scoliosis (EOS). MRI can help identify spinal cord abnormalities in patients with EOS.

Methods: We reviewed data from patients enrolled from 1993-2018 in an international EOS registry. Read More

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http://dx.doi.org/10.1007/s43390-020-00115-3DOI Listing

The impact of depression on surgical outcome in Chiari malformation type I: an assessment based on the Chicago Chiari Outcome Scale.

J Neurosurg Spine 2020 Apr 24:1-8. Epub 2020 Apr 24.

1Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore.

Objective: The effect of depression on outcomes in Chiari malformation type I (CM-1) is unclear. The authors sought to determine whether depression affects outcome in a surgical cohort of CM-1 patients by using a validated outcome assessment tool, the Chicago Chiari Outcome Scale (CCOS).

Methods: The authors performed a retrospective analysis of a prospectively maintained database of 149 adult CM-1 patients undergoing suboccipital decompression with duraplasty and cranioplasty. Read More

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http://dx.doi.org/10.3171/2020.2.SPINE2069DOI Listing

Timing of syrinx reduction and stabilization after posterior fossa decompression for pediatric Chiari malformation type I.

J Neurosurg Pediatr 2020 Apr 24:1-7. Epub 2020 Apr 24.

1Department of Neurosurgery, Vanderbilt University Medical Center.

Objective: The aim of this study was to determine the timeline of syrinx regression and to identify factors mitigating syrinx resolution in pediatric patients with Chiari malformation type I (CM-I) undergoing posterior fossa decompression (PFD).

Methods: The authors conducted a retrospective review of records from pediatric patients (< 18 years old) undergoing PFD for the treatment of CM-I/syringomyelia (SM) between 1998 and 2015. Patient demographic, clinical, radiological, and surgical variables were collected and analyzed. Read More

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http://dx.doi.org/10.3171/2020.2.PEDS19366DOI Listing

Comparison of high-field MRI and multidetector CT for grading Chiari-like malformation and syringomyelia in Cavalier King Charles Spaniels.

Vet Radiol Ultrasound 2020 Apr 24. Epub 2020 Apr 24.

Department of Veterinary Clinical Sciences, College of Veterinary Medicine, The Ohio State University, Columbus, Ohio.

Chiari-like malformation (CM) and syringomyelia (SM) are common illnesses that can cause debilitating neuropathic pain in Cavalier King Charles spaniels (CKCS). The current imaging modality to screen CKCS for CM/SM is MRI of the brain and cervical spine. Magnetic resonance imaging provides good soft tissue detail and contrast of the cerebellum and cervical spinal cord. Read More

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http://dx.doi.org/10.1111/vru.12862DOI Listing