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Arnold chiari malformation

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https://www.readbyqxmd.com/read/28321315/prolonged-expiratory-apnoea-with-cyanosis-in-arnold-chiari-ii-malformation
#1
Adnan Zafar, Nahin Hussain
Apnoea associated with Arnold Chiari malformation is a known entity and can be obstructive or central. Differentiating between two types is vital to deciding management pathway and prognosticating disease process.
March 2017: JRSM Open
https://www.readbyqxmd.com/read/28217389/management-of-parturients-in-active-labor-with-arnold-chiari-malformation-tonsillar-herniation-and-syringomyelia
#2
Ramsis F Ghaly, Tatiana Tverdohleb, Kenneth D Candido, Nebojsa Nick Knezevic
BACKGROUND: Arnold-Chiari malformation Type 1 (ACM-1) in parturients is a topic of ongoing discussion between obstetricians and anesthesiologists. The primary unanswered question remains; How should the anesthesia provider proceed with labor analgesia and anesthesia for cesarean section when confronted with an advanced, asymptomatic, or minimally symptomatic case of ACM-1 during labor? CASE DESCRIPTION: A 24-year-old, ASA II, G1P0 full-term parturient presented to Labor and Delivery for vaginal delivery...
2017: Surgical Neurology International
https://www.readbyqxmd.com/read/28121970/resorbable-mesh-cranioplasty-repair-of-bilateral-cerebrospinal-fluid-leaks-following-pediatric-simultaneous-bilateral-auditory-brainstem-implant-surgery
#3
Giacomo Colletti, Marco Mandalà, Vittorio Colletti, Alberto Deganello, Fabiana Allevi, Liliana Colletti
OBJECTIVE: To present a child with cochlear nerve deficiency (CND) who received simultaneous bilateral simultaneous auditory brainstem implants (BS-ABI) and subsequently presented with bilateral cerebrospinal fluid (CSF) leaks unresponsive to standard treatments. To propose a novel rigid retrosigmoid cranioplasty for treating and preventing CSF leaks in children at high risk for this complication. PATIENT: A 3.5-year-old child with CND, vertebral defects, anal atresia, cardiac defects, tracheo-esophageal fistula, renal anomalies, and limb abnormalities, coloboma, heart defect, atresia choanae, retarded growth and development, genital abnormality, and ear abnormality, Arnold Chiari malformation, previous treated tracheo-esophageal fistula underwent BS-ABI...
April 2017: Otology & Neurotology
https://www.readbyqxmd.com/read/27981932/g6pd-deficiency-with-arnold-chiari-malformation
#4
Shilpi Verma, Pradeep Kumar Bhatia, Vandana Sharma, Priyanka Sethi, Yogendra Raj Singh
A neonate with glucose-6-phosphate dehydrogenase (G6PD) deficiency and Arnold-Chiari Malformation (ACM) type 2 underwent lumbar meningomyelocele (MMC) repair. Patients with G6PD deficiency are prone to develop haemolysis following any kind of oxidative stress and in ACM, there is a disturbed cranio-spinal pressure relationship. The neonate was managed under general anaesthesia with propofol for induction as well as for maintenance along with fentanyl and oxygen-nitrous mixture.
November 2016: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
https://www.readbyqxmd.com/read/27858370/intracranial-hypertension-in-cystinosis-is-a-challenge-experience-in-a-children-s-hospital
#5
Nieves Martín-Begué, Silvia Alarcón, Charlotte Wolley-Dod, Luis Enrique Lara, Álvaro Madrid, Paola Cano, Mireia Del Toro, Gema Ariceta
BACKGROUND: Cystinosis is a rare systemic lysosomal disease affecting mainly the kidney and eye. Ocular involvement in cystinosis is universal being the presence of cystine crystals in the cornea a diagnostic criterion and one of the earliest manifestations of the disease. Neuro-ophthalmologic manifestations are considered a rare and late complication in these patients. The aim of this article is to report the unexpectedly high incidence of intracranial hypertension in children with cystinosis at our centre...
November 18, 2016: JIMD Reports
https://www.readbyqxmd.com/read/27857798/not-all-cases-of-nyctalopia-are-benign-unusual-and-serendipitous-presentation-of-arnold-chiari-type-1-malformation-at-a-pediatric-tertiary-care-center
#6
Kailash Chandra Patra, Abhijeet Prakash Kirtane
The Arnold-Chiari Type 1 malformation (CM1) is a rare congenital abnormality characterized by ectopia or caudal herniation of the cerebellar tonsils through the foramen magnum into the cervical spine, resulting in crowding at the craniocervical junction. It seldom presents in childhood with symptoms and a normal neurological examination. More typically, CM1 presents in young adult women with neurological symptoms including a headache, cervical pain, cranial nerve palsies, neurosensory deficit, and ataxia. Ocular manifestations associated with Chiari I include third and sixth cranial nerve paresis and convergence/divergence abnormalities...
July 2016: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/27823711/central-sleep-apnea-in-children-experience-at-a-single-center
#7
Orlane Felix, Alessandro Amaddeo, Jorge Olmo Arroyo, Michel Zerah, Stephanie Puget, Valerie Cormier-Daire, Genevieve Baujat, Graziella Pinto, Marta Fernandez-Bolanos, Brigitte Fauroux
OBJECTIVE: Central sleep apnea (CSA) syndromes are rare in children and data in children over one year of age are scarce. The aim of the study was to describe the sleep characteristics, underlying disorders, management, and outcome of children with CSA. PATIENTS/METHODS: A retrospective chart review of all children >1 year of age, diagnosed with CSA on a laboratory sleep study during a 20-month period, was performed. CSA was defined by a central apnea index (CAI) >5 events/h...
September 2016: Sleep Medicine
https://www.readbyqxmd.com/read/27790480/bilateral-vocal-cord-palsy-with-arnold-chiari-malformation-a-rare-case-series
#8
Nikhil Arora, Ruchika Juneja, Ravi Meher, Eishaan K Bhargava
Stridor in paediatric age group is not an uncommon presentation to the ENT emergency. The range of differential diagnosis is vast. The presentation may vary from noisy breathing to severe respiratory distress and apnea. Early and meticulous diagnosis is crucial for the management as the condition may be life threatening. We report a rare case series of 3 infants with Arnold Chiari Malformation who presented to the hospital with stridor and were diagnosed with bilateral vocal cord palsy. These 3 infants had similar underlying neurological condition with hydrocephalus and raised intracranial pressure...
September 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/27777155/shunting-of-syringomyelic-cavities-by-using-a-myringotomy-tube-technical-note-and-long-term-results
#9
Dorota Tassigny, Basel Abu-Serieh, Daniella Tsague Fofe, Jacques Born, Germain Milbouw
BACKGROUND: Syringomyelia is a progressive cerebrospinal fluid disorder that can lead to irreversible spinal cord injury. To date, the optimal management of syringomyelic cavities remains controversial. Multiple studies have emphasized the importance of the craniocervical decompression or shunting procedures; however, except for syrinx related to Chiari malformation, nearly one-half of patients need to undergo reoperation. The purpose of the present study was to describe a simple and efficient surgical technique and to report the long-term radioclinical outcomes...
February 2017: World Neurosurgery
https://www.readbyqxmd.com/read/27673019/poster-262-autonomic-dysreflexia-associated-with-an-arnold-chiari-1-malformation-a-case-report
#10
Zachary C Fausel, Dale C Strasser
No abstract text is available yet for this article.
September 2016: PM & R: the Journal of Injury, Function, and Rehabilitation
https://www.readbyqxmd.com/read/27489065/urgent-decisions-and-a-tight-spot-embolic-infarction-of-a-herniated-cerebellar-tonsil
#11
Ruth Mc Donagh, David Bradley, Joseph Augustine Harbison
A previously well 30-year-old woman presented at 17:30 with a sudden onset of dizziness, ataxia and headache. She was initially investigated with a CT scan of the brain and lumbar puncture, which yielded no diagnosis. Subsequent MR scan revealed multiple posterior circulation infarcts, along with a previously undiagnosed Arnold-Chiari 2 malformation with an associated syrinx of her cervical and thoracic spine. The infarct involved one of the herniated cerebellar tonsils. Oedema of an infarct in the herniated tonsils caused compression of the medulla at the foramen magnum, with associated neurological symptoms including Lhermitte's phenomenon and headache on valsalva manoeuvre...
August 3, 2016: BMJ Case Reports
https://www.readbyqxmd.com/read/27418995/unusual-ventilatory-response-to-exercise-in-patient-with-arnold-chiari-type-1-malformation-after-posterior-fossa-decompression
#12
Keely Smith, Ana M Gomez-Rubio, Tomika S Harris, Lauren E Brooks, Ricardo A Mosquera
We present a case of a 17-year-old Hispanic male with Arnold-Chiari Type 1 [AC-Type 1] with syringomyelia, status post decompression, who complains of exercise intolerance, headaches, and fatigue with exertion. The patient was found to have diurnal hypercapnia and nocturnal alveolar hypoventilation. Cardiopulmonary testing revealed blunting of the ventilatory response to the rise in carbon dioxide (CO2) resulting in failure of the parallel correlation between increased CO2 levels and ventilation; the expected vertical relationship between PETCO2 and minute ventilation during exercise was replaced with an almost horizontal relationship...
2016: Case Reports in Pediatrics
https://www.readbyqxmd.com/read/27399450/171%C3%A2-resurgery-in-craniovertebral-junction-abnormalities
#13
Pavaman Pandit Sindgikar, Kuntal Kanti Das, Awadesh K Jaiswal, Rabi Narayan Sahu, Arun K Srivastava, Anant Mehrotra, Jayesh Chunilal Sardhara, Kamlesh Singh Bhaisora, Sanjay Behari
INTRODUCTION: Surgery for craniovertebral junction (CVJ) abnormalities like atlantoaxial dislocation (AAD) with or without basilar invagination (BI) and/or with or without associated Arnold-Chiari malformation (ACM) cause high cervical myelopathy. Occasionally, mechanical factors such as inadequate canal decompression, torticollis, and/or scoliosis may lead to lack of improvement following the primary surgery. Also, implant-related factors requiring its revision/removal or surgical site infections may cause patient to undergo resurgery...
August 2016: Neurosurgery
https://www.readbyqxmd.com/read/27315431/bilateral-vocal-cord-palsy-causing-stridor-as-the-only-symptom-of-syringomyelia-and-chiari-i-malformation-a-case-report
#14
Saif Yousif, Mark Walsh, Hannah Burns
INTRODUCTION: Bilateral vocal cord palsy is a condition which has many causes (Gupta et al., 2012) [1]. Syringomyelia is an uncommon condition which describes the formation of fluid filled cavity, occupying the spinal cord (Chang, 2003) [2]. It rarely manifests itself as subacute onset of stridor. PRESENTATION OF CASE: We present the case of a three year old female who presented for evaluation of her speech and language delay, when incidentally it was made note of her loud breathing which had previously been managed as bronchiolitis by her general practitioner...
2016: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/27299074/bilateral-femoral-neck-fractures-in-a-young-patient-suffering-from-hypophosphatasia-due-to-a-first-time-epileptic-seizure
#15
N Sharma, E Bache, T Clare
INTRODUCTION: We report a case of an adolescent sustaining bilateral femoral neck fractures due to a first time epileptic seizure, as a result of expansion of his known syrinx. CASE REPORT: A 19-year-old patient suffering from hypophosphatasia (HPP), Arnold-Chiari malformation, and a ventriculoperitoneal shunt sustained a trivial fall with profound pain and an inability to mobilize. Radiographs demonstrated a right-sided Garden-4 femoral neck and left-sided multi-fragmentary intracapsular/extracapsular fractures...
July 2015: Journal of Orthopaedic Case Reports
https://www.readbyqxmd.com/read/27258364/craniocervical-spinal-instability-after-type-1-arnold-chiari-decompression-a-case-report
#16
Gaston O Camino Willhuber, Santiago T Bosio, Miguel H Puigdevall, Carolina Halliburton, Carlos A Sola, Ruben A Maenza
To present and describe an unusual case of spinal instability after craniocervical spinal decompression for a type-1 Chiari malformation. Type-1 Chiari malformation is a craniocervical disorder characterized by tonsillar displacement greater than 5 mm into the vertebral canal; posterior fossa decompression is the most common surgical treatment for this condition. Postoperative complications have been described: cerebrospinal fluid leak, pseudomeningocele, aseptic meningitis, wound infection, and neurological deficit...
January 2017: Journal of Pediatric Orthopedics. Part B
https://www.readbyqxmd.com/read/27149441/arnold-chiari-malformation-with-sponastrime-spondylar-and-nasal-changes-with-striations-of-the-metaphyses-dysplasia-a-case-report
#17
Je Hoon Jeong, A Leum Lee, Sung Yoon Cho, Dong Kyu Jin, Soo-Bin Im
SPOndylar and NAsal changes, with STRIations of the Metaphyses (SPONASTRIME) dysplasia (SD) is a dwarfing autosomal recessive syndrome, characterized by a variety of clinical and radiographic features, which form the basis for diagnosis. We describe the presentation of an Arnold Chiari malformation in a patient with a clinical diagnosis of SD. The malformation was successfully treated by decompression of the foramen magnum and elevation of the cerebellum, with complete resolution of pain.We report a rare case of Arnold Chiari malformation in a patient presenting with clinical and radiographic features strongly suggestive of SD and be successfully treated...
May 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27141649/-a-destructive-shoulder-arthropathy
#18
L Ouhadi, M Gaudreault, S Mottard, Ph Gillet
Charcot arthropathy is a progressive, chronic and degenerative destruction of one or several joints caused by a central or peripheral neurological disorder. Approximately 25 % of the patients with syringomyelia develop this arthropathy located in the upper limb in 80 % of the cases. An early etiological diagnosis is essential to begin the treatment of the underlying neurological disorder. Afterwards, a conservative treatment of the arthropathy is preferred. We report the story of a patient with an arthropathy of the left shoulder due to Arnold-Chiari's malformation of type I with syringomyelia...
February 2016: Revue Médicale de Liège
https://www.readbyqxmd.com/read/27121616/the-prevalence-of-intraspinal-anomalies-in-infantile-and-juvenile-patients-with-presumed-idiopathic-scoliosis-a-mri-based-analysis-of-504-patients
#19
Wen Zhang, Shifu Sha, Leilei Xu, Zhen Liu, Yong Qiu, Zezhang Zhu
BACKGROUND: Though several studies have reported the incidence of intraspinal neural axis abnormalities in infantile and juvenile "presumed idiopathic" scoliosis, there has been a varying prevalence ranging from 11.1 to 26.0% based on a limited sample size. Therefore, such inconclusive findings have resulted in some questions on the MRI-associated role in the management of these patients. We aimed to investigate the prevalence and distribution of intraspinal anomalies in the infantile and juvenile patients with "presumed idiopathic" scoliosis and to explore the radiographic and clinical indicators with large sample size...
April 27, 2016: BMC Musculoskeletal Disorders
https://www.readbyqxmd.com/read/27010296/progressive-syringohydromyelia-and-degenerative-axonopathy-in-a-bobcat-lynx-rufus-following-surgical-correction-of-a-chiari-like-malformation
#20
Ryan Sadler, Juergen Schumacher, Edward Ramsay, Brynn McCleery, Katherine Baine, William Thomas, Michelle Nobrega-Lee, George A Henry, Shelley J Newman
A 3-yr-old male captive bobcat (Lynx rufus) presented with chronic ataxia and right-sided head tilt. Magnetic resonance imaging (MRI) revealed cerebellar crowding and compression consistent with Chiari-like malformation. The clinical signs did not improve after surgical occipital craniectomy, and 2 mo postoperatively a second MRI showed hydromyelia and continued cerebellar compression. The bobcat was euthanized, and necropsy showed chronic focal cerebellar herniation and chronic multifocal atlanto-occipital joint osteophyte proliferation...
March 2016: Journal of Zoo and Wildlife Medicine: Official Publication of the American Association of Zoo Veterinarians
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