Literature DB >> 31032440

The prevalence of Klippel-Feil syndrome in pediatric patients: analysis of 831 CT scans.

Jalea T Moses1, Devin M Williams2, Paul T Rubery2, Addisu Mesfin2.   

Abstract

BACKGROUND: To evaluate the prevalence of Klippel-Feil syndrome (KFS) in pediatric patients obtaining cervical CT imaging in the emergency room (ER).
METHODS: We evaluated CT scans of the cervical spine of pediatric patients treated in the ER of a Level I Trauma Center from January 2013 to December 2015. Along with analysis of the CT scans for KFS, the following demographics were collected: age, sex, race and ethnicity. Mechanism of injury was also established for all patients. If KFS was present, it was classified using Samartzis classification as type I (single level fusion), type II (multiple, noncontiguous fused segments) or type III (multiple, contiguous fused segments).
RESULTS: Of the 848 cervical CTs taken for pediatric ER patients during the study period, 831 were included. Of these patients, 10 had KFS, a prevalence of 1.2%. According to Samartzis classification, 9 were type I and 1 type III. The average age of patients with KFS was 16.02 years (10-18 years), with 8 males (80%) and 2 females (20%). Three had congenital fusions at vertebral levels C2-C3, two at C3-C4, three at C5-C6, one at C6-C7, and one with multiple levels of cervical fusion.
CONCLUSIONS: The prevalence of KFS amongst 831 pediatric patients, who underwent cervical CT imaging over a 3-year period, was 1.2% (approximately 1 in 83). The most commonly fused spinal levels were C2-C3 and C5-C6. The prevalence of KFS in our study was higher than previously described, and thus warrants monitoring.

Entities:  

Keywords:  Klippel-Feil syndrome (KFS); Samartzis classification; cervical CT; cervical spine imaging; cervical vertebrae; congenital cervical fusion; pediatric patients

Year:  2019        PMID: 31032440      PMCID: PMC6465471          DOI: 10.21037/jss.2019.01.02

Source DB:  PubMed          Journal:  J Spine Surg        ISSN: 2414-4630


  19 in total

1.  Klippel-Feil syndrome in the prehispanic population of El Hierro (Canary Islands).

Authors:  E González-Reimers; A Mas-Pascual; M Arnay-De-La-Rosa; J Velasco-Vásquez; M C Jiménez-Gómez
Journal:  Ann Rheum Dis       Date:  2001-02       Impact factor: 19.103

2.  THE INCIDENCE OF ACQUIRED AND CONGENITAL FUSIONS IN THE CERVICAL SPINE.

Authors:  M W BROWN; A W TEMPLETON; F J HODGES
Journal:  Am J Roentgenol Radium Ther Nucl Med       Date:  1964-12

Review 3.  CONGENITAL FUSION OF THE CERVICAL VERTEBRAE.

Authors:  S W GRAY; C B ROMAINE; J E SKANDALAKIS
Journal:  Surg Gynecol Obstet       Date:  1964-02

4.  Mutations in GDF6 are associated with vertebral segmentation defects in Klippel-Feil syndrome.

Authors:  May Tassabehji; Zhi Ming Fang; Emma N Hilton; Julie McGaughran; Zhongming Zhao; Charles E de Bock; Emma Howard; Michael Malass; Dian Donnai; Ashish Diwan; Forbes D C Manson; Dédée Murrell; Raymond A Clarke
Journal:  Hum Mutat       Date:  2008-08       Impact factor: 4.878

5.  Symptomatic cervical disc herniation in a pediatric Klippel-Feil patient: the risk of neural injury associated with extensive congenitally fused vertebrae and a hypermobile segment.

Authors:  Dino Samartzis; John P Lubicky; Jean Herman; Prakasam Kalluri; Francis H Shen
Journal:  Spine (Phila Pa 1976)       Date:  2006-05-15       Impact factor: 3.468

6.  Klippel-Feil anomaly with Sprengel anomaly, omovertebral bone, thumb abnormalities, and flexion-crease changes: novel association or syndrome?

Authors:  A R Larson; K D Josephson; R M Pauli; J M Opitz; M S Williams
Journal:  Am J Med Genet       Date:  2001-06-15

7.  Classification of congenitally fused cervical patterns in Klippel-Feil patients: epidemiology and role in the development of cervical spine-related symptoms.

Authors:  Dino Dip Samartzis; Jean Herman; John P Lubicky; Francis H Shen
Journal:  Spine (Phila Pa 1976)       Date:  2006-10-01       Impact factor: 3.468

8.  Klippel-Feil syndrome with other associated anomalies in a medieval Portuguese skeleton (13th-15th century).

Authors:  Teresa Fernandes; Catarina Costa
Journal:  J Anat       Date:  2007-09-07       Impact factor: 2.610

9.  2008 Young Investigator Award: The role of congenitally fused cervical segments upon the space available for the cord and associated symptoms in Klippel-Feil patients.

Authors:  Dino Samartzis; Prakasam Kalluri; Jean Herman; John P Lubicky; Francis H Shen
Journal:  Spine (Phila Pa 1976)       Date:  2008-06-01       Impact factor: 3.468

10.  Mutation in MEOX1 gene causes a recessive Klippel-Feil syndrome subtype.

Authors:  Fatih Bayrakli; Bulent Guclu; Cengiz Yakicier; Hatice Balaban; Ugur Kartal; Bekir Erguner; Mahmut Samil Sagiroglu; Sirin Yuksel; Ahmet Rasit Ozturk; Burak Kazanci; Unal Ozum; Hamit Zafer Kars
Journal:  BMC Genet       Date:  2013-09-28       Impact factor: 2.797

View more
  2 in total

1.  Prevalence and Risk Factors of Surgical Treatment for Klippel-Feil Syndrome.

Authors:  Linyao Ding; Xin Wang; Yu Sun; Fengshan Zhang; Shengfa Pan; Xin Chen; Yinze Diao; Yanbin Zhao; Tian Xia; Weishi Li; Feifei Zhou
Journal:  Front Surg       Date:  2022-06-07

2.  A rare case of difficult airway management in a Klippel-Feil syndrome pediatric patient with osseous torticollis undergone orthopedic surgery : Difficult airway in pediatric patient with torticollis.

Authors:  Xiaoqing Zhang; Jun Wang; Yajie Liu; Zhengqian Li; Bin Han
Journal:  BMC Anesthesiol       Date:  2021-04-19       Impact factor: 2.217

  2 in total

北京卡尤迪生物科技股份有限公司 © 2022-2023.