ID Design 2012/DOOEL Skopje
Open Access Macedonian Journal of Medical Sciences.
Klippel – Feil Syndrome
Associated with Congential Heart Disease Presentaion of Cases and a Review
of the Curent Literature
Ramush Bejiqi1*, Ragip Retkoceri1,
Hana Bejiqi2, Naim Zeka1
1Division of Cardiology, Pediatric Clinic, University Clinical
Center of Kosovo, Prishtina, Republic of Kosovo; 2Main Center of
Family Medicine, Prishtina, Republic of Kosovo
First time described in 1912, from Maurice Klippel and Andre Feil
independently, Klippel-Feil syndrome (synonyms: cervical vertebra fusion
syndrome, Klippel-Feil deformity, Klippel-Feil sequence disorder) is a bone
disorder characterized by the abnormal joining (fusion) of two or more
spinal bones in the neck (cervical vertebrae), which is present from birth.
Three major features result from this abnormality: a short neck, a limited
range of motion in the neck, and a low hairline at the back of the head.
Most affected people have one or two of these characteristic features. Less
than half of all individuals with Klippel-Feil syndrome have all three
classic features of this condition.
Since first classification from Feil in three categories (I – III) other
classification systems have been advocated to describe the anomalies,
predict the potential problems, and guide treatment decisions. Patients with
Klippel-Feil syndrome usually present with the disease during childhood, but
may present later in life. The challenge to the clinician is to recognize
the associated anomalies that can occur with Klippel-Feil syndrome and to
perform the appropriate workup for diagnosis.
Citation: Bejiqi R, Retkoceri R, Bejiqi H, Zeka N.
Klippel – Feil Syndrome Associated with Congential Heart Disease Presentaion
of Cases and a Review of the Curent Literature. OA Maced J Med Sci. http://dx.doi.org/10.3889/oamjms.2015.022
Key words: Klippel-Feil syndrome; short neck; low hairline;
congenital heart disease.
*Correspondence: Ramush Bejiqi. Division of Cardiology, Pediatric
Clinic, University Clinical Center of Kosovo, Prishtina, Republic of Kosovo.
Tel: +37744120129. E-mail: firstname.lastname@example.org
Received: 14-Jan-2015; Revised: 03-Feb-2015; Accepted: 04-Feb-2015; Online
Copyright: © 2015 Ramush Bejiqi, Ragip Retkoceri, Hana Bejiqi, Naim
Zeka. This is an open access article distributed under the terms of the
Creative Commons Attribution License, which permits unrestricted use,
distribution, and reproduction in any medium, provided the original author
and source are credited.
Competing Interests: The authors have declared that no competing
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- Bejiqi R
- Retkoceri R
- Bejiqi H
- Zeka N