Klippel-Feil syndrome


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Related to Klippel-Feil syndrome: Klippel-Trenaunay Syndrome

Klippel-Feil syndrome

 [klĭ-pel´ fīl]
shortness of the neck due to reduction in the number of cervical vertebrae or the fusion of multiple hemivertebrae into one osseous mass, with limitation of neck motion and low hairline.

Klip·pel-Feil syn·drome

(klip'ĕl fīl), [MIM*148900]
a congenital defect manifested as a short neck, fusion of cervical vertebrae, and abnormalities of the brainstem and cerebellum; autosomal dominant inheritance, with most cases sporadic.

Klippel-Feil syndrome

[klipel′ fel′, klip′əl fīl′]
Etymology: Maurice Klippel, French neurologist, 1858-1942; Andre Feil, French neurologist, b. 1884
a condition of short neck and limited neck movements because of congenital fusion of the cervical vertebrae or reduction in the number of cervical vertebrae. Also called Klippel-Feil disease, Klippel-Feil malformation, Klippel's disease. See congenital short neck syndrome.

Klippel-Feil syndrome

A condition (OMIM:214300) characterised by short neck, low hairline and decreased cervical motion.
 
Associated conditions
Congenital scoliosis (60%, most need treatment); Sprengel's deformity (failure of scapula to descend, remains attached to cervical spine by omovertebral bone or fibrous band) (33%); renal disease, often with aplasia (33%); synkinesis (mirror movements); congenital heart disease; brain stem defects; congenital cervical stenosis; syndactyly and hypoplastic thumb; hearing loss (30%).

Pathogenesis
Klippel-Feil syndrome is characterised by congenital failure of normal segmentation of cervical somites at 3–8 weeks's gestation, resulting in two or more fused cervical segments.

Klippel-Feil sequence/syndrome

 The combination of short neck, low hairline at nape of the neck and limited movement of head, due to a defect in the early development of the spinal column in the neck–the cervival vertebrae

Klip·pel-Feil syn·drome

(klipĕl fīl sindrōm)
A congenital abnormality of the spine characterized by a reduction in the number of cervical vertebrae and their fusion. See this page.

Feil,

André, French physician, 1884–.
Feil-Klippel syndrome - Synonym(s): Klippel-Feil syndrome
Klippel-Feil syndrome - see under Klippel

Klippel,

Maurice, French neurologist, 1858-1942.
Feil-Klippel syndrome - Synonym(s): Klippel-Feil syndrome
Klippel-Feil syndrome - a congenital defect manifested as a short neck, extensive fusion of the cervical vertebrae, and abnormalities of the brainstem and cerebellum. Synonym(s): cervical fusion syndrome; Feil-Klippel syndrome
Klippel-Trenaunay-Weber syndrome - an anomaly of the extremity in which there is a combination of angiomatosis and anomalous development of the underlying bone and muscle, sometimes associated with localized gigantism. Synonym(s): angioosteohypertrophy syndrome; congenital dysplastic angiectasia; hemangiectatic hypertrophy

Klip·pel-Feil syn·drome

(klipĕl fīl sindrōm) [MIM*148900]
A congenital abnormality of the spine characterized by a reduction in the number of cervical vertebrae and their fusion.
References in periodicals archive ?
Cervical Klippel-Feil syndrome predisposing an elderly African man to central cord myelopathy following minor trauma.
Instability of the cervical spine and neurological involvement in Klippel-Feil syndrome.
Type 3 is where thoracic or lumbar spine abnormalities are associated with Type 1 or 2 Klippel-Feil syndrome.
Klippel-Feil syndrome is a rare condition with an estimated incidence of 1 in 40,000 births (3).
We recently had a patient with adult Klippel-Feil syndrome who developed intraoperative haemodynamic instability during an elective C1 dorsal laminectomy and occipito-cervical stabilisation in the prone position.
She had been diagnosed with Type 1 Klippel-Feil syndrome some years before.
There are other issues to consider in patients with Klippel-Feil syndrome presenting for anaesthesia.
Anaesthetic considerations in Klippel-Feil syndrome.
Anesthetic management of a child with klippel-Feil syndrome in the radiology suite.
Anesthetic management of a child with Arnold-Chiari malformation and Klippel-Feil syndrome.
Thoracic epidural anaesthesia for bilateral reduction mammoplasty in a patient with Klippel-Feil syndrome.