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Klippel-feil syndrome

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https://www.readbyqxmd.com/read/28410903/l-ut%C3%A3-rus-unicorne-et-le-syndrome-de-klippel-feil
#1
Areiyu Zhang
No abstract text is available yet for this article.
April 11, 2017: Journal of Obstetrics and Gynaecology Canada: JOGC, Journal D'obstétrique et Gynécologie du Canada: JOGC
https://www.readbyqxmd.com/read/28410901/unicornuate-uterus-in-klippel-feil-syndrome
#2
Areiyu Zhang, Luz Arbelaez
No abstract text is available yet for this article.
April 11, 2017: Journal of Obstetrics and Gynaecology Canada: JOGC, Journal D'obstétrique et Gynécologie du Canada: JOGC
https://www.readbyqxmd.com/read/28387188/deep-sedation-technique-for-dental-rehabilitation-of-a-patient-with-klippel-feil-syndrome
#3
Halima Abukabbos, Michael Mahla, Abi O Adewumi
Klippel-Feil syndrome (KFS) is a congenital anomaly characterized by a defect in the formation or segmentation of the cervical vertebrae, resulting in their fusion. The clinical triad of the syndrome consists of short neck, low posterior hairline, and limited neck movement, although fewer than 50 percent of patients demonstrate all three clinical features. The short neck and its immobility and instability present a significant challenge for endotracheal intubation. The purpose of this paper is to describe the management of a 13-year-old patient with KFS, extensive dental caries, and restricted mouth opening using a deep sedation technique in the operating room, which allowed successful completion of dental treatment...
January 15, 2017: Journal of Dentistry for Children
https://www.readbyqxmd.com/read/28357553/misdiagnosed-syrinx-in-a-patient-with-neuroschisis-and-klippel-feil-syndrome-case-report
#4
Vlad Voin, Nitsa Topale, Joe Iwanaga, Rod J Oskouian, R Shane Tubbs
INTRODUCTION: Patients with Klippel-Feil syndrome can present with a myriad of symptoms and imaging findings. Herein, we present a case of Klippel-Feil syndrome that was initially misdiagnosed with syringomyelia. We review this case and discuss the imaging findings. CONCLUSIONS: Neuoroschisis should be suspected in patients with Klippel-Feil syndrome and differentiated from syringomyelia.
March 29, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28250492/klippel-feil-syndrome-interchange-of-plan-a-and-b-for-airway-management-in-the-same-patient-under-different-circumstances
#5
Karri Pavani, Handattu Mahabaleswara Krishna
No abstract text is available yet for this article.
February 2017: Indian Journal of Anaesthesia
https://www.readbyqxmd.com/read/28217386/true-oblique-axis-fracture-associated-with-congenital-anomalies-of-the-upper-cervical-spine-case-report-of-an-unusual-fracture-pattern
#6
Luis A Robles
BACKGROUND: Acute traumatic axis fractures are common cervical spine injuries often caused by road accidents or falls. They are usually classified into three different types, namely, odontoid fractures, Hangman's fractures, and miscellaneous fractures. Congenital malformations of the craniovertebral junction (CVJ), although typically asymptomatic, may result in neural compression or instability, especially following trauma. Here, the authors present an unusual oblique axis fracture occurring in conjunction with several malformations of the upper cervical spine...
2017: Surgical Neurology International
https://www.readbyqxmd.com/read/28199244/crisis-management-of-accidental-extubation-in-a-prone-positioned-patient-with-klippel-feil-syndrome-erratum
#7
(no author information available yet)
No abstract text is available yet for this article.
February 15, 2017: A & A Case Reports
https://www.readbyqxmd.com/read/28133560/endoscopic-resection-of-skull-base-teratoma-in-klippel-feil-syndrome-through-use-of-combined-ultrasonic-and-bipolar-diathermy-platforms
#8
Justin A Edward, Alkis J Psaltis, Ryan A Williams, Gregory W Charville, Robert L Dodd, Jayakar V Nayak
Klippel-Feil syndrome (KFS) is associated with numerous craniofacial abnormalities but rarely with skull base tumor formation. We report an unusual and dramatic case of a symptomatic, mature skull base teratoma in an adult patient with KFS, with extension through the basisphenoid to obstruct the nasopharynx. This benign lesion was associated with midline palatal and cerebral defects, most notably pituitary and vertebrobasilar arteriolar duplications. A multidisciplinary workup and a complete endoscopic, transnasal surgical approach between otolaryngology and neurosurgery were undertaken...
2017: Case Reports in Otolaryngology
https://www.readbyqxmd.com/read/28130167/congenital-cervical-fusion-as-a-risk-factor-for-development-of-degenerative-cervical-myelopathy
#9
Aria Nouri, Allan R Martin, Stefan F Lange, Mark R N Kotter, David J Mikulis, Michael G Fehlings
BACKGROUND: Congenital fusion of cervical vertebrae, including Klippel-Feil syndrome (KFS), is a suspected risk factor for development of degenerative cervical myelopathy (DCM). We aimed to establish prevalence and degenerative patterns of congenital cervical fusion (CCF) among a global cohort of patients with DCM. METHODS: Data from 3 prospective DCM studies were merged, including clinical data for 813 patients and imaging for 592 patients. CCF was diagnosed by presence of fused cervical vertebrae without signs of degenerative fusion...
April 2017: World Neurosurgery
https://www.readbyqxmd.com/read/27869419/-klippel-feil-syndrome-with-tracheoesophageal-fistula-bifid-thumb-and-cerebral-angiolipoma
#10
Eliéxer Urdaneta Carruyo, Gustavo Rojas Zerpa, Adriana Urdaneta Contreras, Malvy Maldonado Alviarez, Miguel Brito Rodríguez
The Klippel-Feil syndrome is a congenital malformation of the skull flap involving complex cervical vertebrae and organs, characterized by a classic triad: short neck, limitation of movement of the head due to cervical spine fusion and low hairline in occipital region. It results from an error in the axial skeleton segmentation of the embryo; its incidence is estimated at 1/40,000-42,000 births and predominates in females. The aim of this paper is to describe the clinical picture of a patient with Klippel-Feil syndrome and multiple malformations, including tracheoesophageal fistula, bifid thumb and intracranial lipomas/angiolipomas,that have not been previously described in the syndrome, so it is considered an exceptional finding...
December 1, 2016: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/27861250/mri-analysis-of-the-combined-prospectively-collected-aospine-north-america-and-international-data-the-prevalence-and-spectrum-of-pathologies-in-a-global-cohort-of-patients-with-degenerative-cervical-myelopathy
#11
Aria Nouri, Allan Martin, Lindsay Tetreault, Anick Nater, So Kato, Hiroaki Nakashima, Narihito Nagoshi, Hamed Reihani-Kermani, Michael G Fehlings
STUDY DESIGN: Ambispective Analysis OBJECTIVE.: To report the global prevalence of specific degenerative cervical pathologies in patients with DCM through detailed review of MRIs SUMMARY OF BACKGROUND DATA.: Degenerative Cervical Myelopathy (DCM) encompasses a spectrum of age-related conditions that result in progressive spinal cord injury. METHODS: MRIs of 458 patients (age 56.4 +/-11.8, 285 Male, 173 Female) patients were reviewed for specific degenerative features, directionality of cord compression, levels of spinal cord compression, and signal changes on T2WI and T1WI...
November 16, 2016: Spine
https://www.readbyqxmd.com/read/27847299/an-mrspec-database-query-and-visualization-engine-with-applications-as-a-clinical-diagnostic-and-research-tool
#12
Filip Miscevic, Justin Foong, Benjamin Schmitt, Susan Blaser, Michael Brudno, Andreas Schulze
PURPOSE: Proton magnetic resonance spectroscopy (MRspec), one of the very few techniques for in vivo assessment of neuro-metabolic profiles, is often complicated by lack of standard population norms and paucity of computational tools. METHODS: 7035 scans and clinical information from 4430 pediatric patients were collected from 2008 to 2014. Scans were conducted using a 1.5T (n=3664) or 3T scanner (n=3371), and with either a long (144ms, n=5559) or short echo time (35ms, n=1476)...
December 2016: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/27673225/poster-484-3d-printed-orthotic-designed-for-klippel-feil-syndrome-affected-hypoplastic-thumb-a-case-report
#13
Brian W Lee, Dona Rani C Kathirithamby, Cesar Colasante, Kyle Menze, Kyle Silva, Louis Dizon, Jared R Levin, Edward Alexeev
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/27591474/airway-management-in-newborn-with-klippel-feil-syndrome
#14
Nuray Altay, Hasan H Yüce, Harun Aydoğan, Mustafa E Dörterler
Klippel-Feil syndrome (KFS) has a classical triad that includes short neck, low hair line and restriction in neck motion and is among one of the congenital causes of difficult airway. Herein, we present a 26-day, 3300g newborn with KFS who was planned to be operated for correction of an intestinal obstruction. She had features of severe KFS. Anesthesia was induced by inhalation of sevoflurane 2-3% in percentage 100 oxygen. Sevoflurane inhalation was stopped after 2min. Her Cornmack Lehane score was 2 and oral intubation was performed with 3...
September 2016: Brazilian Journal of Anesthesiology
https://www.readbyqxmd.com/read/27539778/extremely-large-amount-of-fused-segments-in-a-rare-case-of-klippel-feil-syndrome
#15
Rong Xing, Qingquan Kong
No abstract text is available yet for this article.
August 2016: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
https://www.readbyqxmd.com/read/27465418/surgical-treatment-of-occipitocervical-dislocation-with-atlas-assimilation-and-klippel-feil-syndrome-using-occipitalized-c1-lateral-mass-and-c2-fixation-and-reduction-technique
#16
Yi-Heng Yin, Guang-Yu Qiao, Xin-Guang Yu
OBJECTIVE: To introduce and assess a surgical treatment of occipitocervical (OC) dislocation with atlas assimilation and Klippel-Feil syndrome (KFS) using occipitalized C1 lateral mass and C2 fixation and reduction technique. METHODS: From January 2007 to August 2013, 58 symptomatic patients with OC dislocation and KFS of C2-3 congenital fusion and atlas assimilation were surgically treated in our institution via this technique. After opening the C1-2 facet joints via a posterior approach, OC reduction was conducted by intraoperative manipulation and C1 lateral mass and C2 pedicle screw and rod fixation...
November 2016: World Neurosurgery
https://www.readbyqxmd.com/read/27444288/the-intriguing-history-of-vertebral-fusion-anomalies-the-klippel-feil-syndrome
#17
REVIEW
Erfanul Saker, Marios Loukas, Rod J Oskouian, R Shane Tubbs
INTRODUCTION: Our knowledge and understanding of vertebral fusion, defined and eponymously known as Klippel-Feil syndrome in the early 1900s, have a long history. This uncommon finding has been identified as early as 500 B.C. in an Egyptian mummy. Many more examples of spinal vertebra fusion have been described by Greek historians and recovered by archeologists demonstrating this entity's rich history. CONCLUSIONS: Klippel-Feil syndrome is a rare skeletal anomaly characterized by abnormal fusion of two or more vertebrae...
September 2016: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/27399514/316%C3%A2-magnetic-resonance-imaging-analysis-of-the-combined-aospine-north-america-and-international-studies-part-i-the-prevalence-and-spectrum-of-pathologies-in-a-global-cohort-of-patients-with-degenerative-cervical-myelopathy
#18
Aria Nouri, Allan R Martin, Lindsay Tetreault, Anick Nater, So Kato, Hiroaki Nakashima, Narihito Nagoshi, Hamed Reihani-Kermani, Michael G Fehlings
INTRODUCTION: Degenerative cervical myelopathy (DCM) encompasses a spectrum of age-related conditions that result in progressive spinal cord injury through static and dynamic injury mechanisms. Through detailed review of MRIs from prospective AOSpine multicenter studies, the global prevalence of degenerative cervical pathologies of surgically treated DCM patients is reported. METHODS: MRIs of 458 patients were obtained from North America (n = 197), Europe (n = 92), Latin America (n = 57), and Asia-Pacific (n = 112) and assessed for the type of pathology, source of stenosis, level of maximum cord compression, levels of spinal cord compression (SCC), presence of signal changes on T2-weighted images (T2WI) and T1-weighted images, and the levels of T2WI signal change...
August 2016: Neurosurgery
https://www.readbyqxmd.com/read/27390547/a-case-of-anomalous-origin-and-course-of-vertebral-artery-in-a-patient-with-klippel-feil-syndrome
#19
Onur Levent Ulusoy, Hadi Sasani, Sezgi Burçin Barlas, Ayhan Mutlu, Mehdi Sasani
Patients with Klippel-Feil syndrome (KFS) have an increased incidence of vascular anomalies as well as vertebral artery (VA) anomalies. In this article, we presented imaging findings of a 15-year-old female patient with KFS with a rare association of extraforaminal cranially ascending right VA that originated from the ipsilateral carotid bulb. Trifurcation of the carotid bulb with VA is a very unusual variation and to the best of our knowledge, right-sided one has not been reported in the literature.
July 2016: Korean Journal of Radiology: Official Journal of the Korean Radiological Society
https://www.readbyqxmd.com/read/27364256/monsters-and-the-case-of-l-joseph-andr%C3%A3-feil-s-thesis-on-the-origin-of-the-klippel-feil-syndrome-and-a-social-transformation-of-medicine
#20
Evgenii Belykh, Kashif Malik, Isabelle Simoneau, Kaan Yagmurlu, Ting Lei, Daniel D Cavalcanti, Vadim A Byvaltsev, Nicholas Theodore, Mark C Preul
André Feil (1884-1955) was a French physician best recognized for his description, coauthored with Maurice Klippel, of patients with congenital fusion of cervical vertebrae, a condition currently known as Klippel-Feil syndrome. However, little is known about his background aside from the fact that he was a student of Klippel and a physician who took a keen interest in describing congenital anomalies. Despite the relative lack of information on Feil, his contributions to the fields of spinal disease and teratology extended far beyond science to play an integral role in changing the misguided perception shrouding patients with disfigurements, defects, deformities, and so-called monstrous births...
July 2016: Neurosurgical Focus
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