Klippel-Feil Syndrome (Syndrome, Klippel-Feil)

A condition characterized by shortness of the neck resulting from reduction in the number of vertebrae or the fusion of multiple hemivertebrae into one osseous mass. The hairline is low and the motion of the neck is limited. (Dorland, 27th ed)

Also Known As:

Networked: 67 relevant articles (0 outcomes, 1 trials/studies)

Disease Context: Research Results

Related Diseases

1.	Spinal Cord Diseases
2.	Hearing Loss (Hearing Impairment)
3.	Turner Syndrome (Turner's Syndrome)
4.	Platybasia (Basilar Impression)
5.	Marfan Syndrome (Marfan's Syndrome)

Experts

1.	Kim, Young Baeg: 1 article (09/2015)
2.	Park, Seung Won: 1 article (09/2015)
3.	Lee, Young Seok: 1 article (09/2015)
4.	Park, Yong Sook: 1 article (09/2015)
5.	Nam, Taek Kyun: 1 article (09/2015)
6.	Kim, Jin Bum: 1 article (09/2015)
7.	Lanna, M: 1 article (08/2015)
8.	Martinelli, P: 1 article (08/2015)
9.	Anfora, R: 1 article (08/2015)
10.	Sala, C: 1 article (08/2015)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Klippel-Feil Syndrome:

1.	AnotiaIBA 11/01/1985 - "Histologic studies of the temporal bones of an infant with Klippel-Feil syndrome whom we studied showed severe anomalies in one ear consisting of microtia, aural atresia, maldeveloped ossicles, fixed stapes, hypoplastic facial nerve, short cochlea, and large diameter of the lateral semicircular canal."
2.	Sprengel deformityIBA 11/01/2014 - "Severe Sprengel deformity associated with Klippel-Feil syndrome and a complex vascular abnormality that determined the corrective surgery technique." 12/01/2010 - "CSD combined with musculoskeletal anomalies, including clubfeet, Sprengel deformity, Klippel-Feil syndrome, and developmental dysplasia of the hip, has been described in the literature. " 08/01/2010 - "The unusual association of an omovertebral bone with Sprengel deformity and Klippel-Feil syndrome is a complex bone anomaly of unknown incidence and etiology. " 02/01/2008 - "We present a case of Klippel-Feil syndrome and Sprengel deformity with a bovine aortic arch and an aberrant course of the left subclavian artery in a 14-year-old boy. " 02/01/2008 - "Klippel-Feil syndrome and Sprengel deformity combined with an intraspinal course of the left subclavian artery and a bovine aortic arch variant."
3.	Wildervanck syndromeIBA 01/01/2015 - "Wildervanck syndrome (WS) combines features of Klippel-Feil syndrome (KFS), sixth nerve palsy, and deafness. " 09/01/2006 - "The association of hearing loss (due to agenesis of the external ear) with Klippel Feil syndrome and Stilling Duane syndrome confirmed the diagnosis of cervico-oculo-acoustic syndrome. " 09/01/2006 - "Cervico-oculo-acoustic syndrome is an uncommon syndrome consisting of Klippel Feil syndrome, deafness, and Duane syndrome. " 09/01/2007 - "In addition to these cardiovascular abnormalities, she had Wildervanck syndrome (Klippel-Feil syndrome, Duane syndrome and sensorineural hearing disturbance), blepharoptosis and short stature. " 03/01/1986 - "A review of the literature showed this lesion to be very rare, but it is more frequent in combination with other malformations such as the Klippel-Feil-syndrome, Wildervanck-syndrome and Thalidomide embryopathy. "
4.	Hereditary renal agenesisIBA 01/01/1985 - "[Unilateral renal agenesis and renal ectopia in Klippel-Feil syndrome]." 09/30/1977 - "[The Klippel-Feil syndrome and renal agenesis]." 11/01/1971 - "Klippel-Feil syndrome with renal agenesis and other anomalies." 11/01/2012 - "Klippel-Feil syndrome with unilateral renal agenesis and renal failure."
5.	Rokitansky Kuster Hauser syndromeIBA 05/01/1978 - "Full description of the case is given first: an atypical Rokitansky-Kuster-Hauser syndrome (with absence of both fallopian tubes) is associated with Turner-like dysmorphia (including a Klippel-Feil syndrome), karyotype being normal. " 05/01/1973 - "[Conjunction of Turner's syndrome, atypical Rokitansky-Kuster-Hauser syndrome and Klippel-Feil syndrome]."
6.	Growth Differentiation FactorsIBA 01/01/2013 - "Of particular interest were two regions (Chr8, Max LOD = 3.04; Chr12, Max LOD = 2.09) identified within the subset of "CTD-negative" families, both of which harbor growth differentiation factors (GDF6, GDF3) implicated in the development of Klippel-Feil syndrome (KFS). "
7.	DexmedetomidineFDA Link 07/01/2011 - "We describe a unique case in which a dexmedetomidine infusion was used as sedation for an awake fiber-optic intubation in a parturient with Klippel-Feil Syndrome, who presented for elective cesarean delivery. " 07/01/2011 - "Dexmedetomidine for an awake fiber-optic intubation of a parturient with Klippel-Feil syndrome, Type I Arnold Chiari malformation and status post released tethered spinal cord presenting for repeat cesarean section."
8.	Thalidomide (Thalomid)FDA Link 03/01/1986 - "A review of the literature showed this lesion to be very rare, but it is more frequent in combination with other malformations such as the Klippel-Feil-syndrome, Wildervanck-syndrome and Thalidomide embryopathy. "
9.	Ribosomal Proteins (Ribosomal Protein)IBA 04/15/2011 - "Can mutations in the ribosomal protein S26 (RPS26) gene lead to Klippel-Feil syndrome in Diamond-Blackfan anemia patients? "
10.	Rh-Hr Blood-Group System (Rhesus Blood-Group System)IBA 11/01/1950 - "[Klippel-Feil syndrome due to Rh factor incompatibility]."

Therapies and Procedures

1.	Airway Management 07/01/2014 - "The ideal airway management modality in pediatric patients with syndromes like Klippel-Feil syndrome is a great challenge and is technically difficult for an anesthesiologist. " 06/01/2014 - "Patients with Klippel-Feil syndrome (KFS) frequently encounter difficult airway management due to skeletal abnormalities, including fusion of two or more vertebrae and short neck. " 06/01/2014 - "[Difficult airway management using the air-Q in a patient with Klippel-Feil syndrome]." 09/01/2008 - "Congenital cervical spine fusion and airway management: a case series of Klippel-Feil syndrome." 09/01/2008 - "The electronic charts of 10 pediatric patients with Klippel-Feil syndrome (KFS) who received treatment at our institution from 2005 to 2007 were reviewed with a focus on diagnosis, level of lesion, comorbid diseases, age, procedure, history of perioperative difficulties, and airway management during general anesthesia. "
2.	Decompression 01/01/1978 - "Anterior decompression for progressive brain stem compression in the Klippel-Feil syndrome." 09/01/2015 - "Two Cases of Klippel-Feil Syndrome with Cervical Myelopathy Successfully Treated by Simple Decompression without Fixation." 09/01/2011 - "The sublabial route is an alternative approach for anterior decompression in patients with symptomatic basilar impression and altered anatomic circumstances such as that caused by Klippel-Feil syndrome." 07/01/1998 - "Thirteen cases were diagnosed with failed back surgery syndrome (FBSS), one older patient with lumbosacral radiculopathy who refused decompression, one cervical radiculopathy and Klippel-Feil syndrome, six with reflex sympathetic dystrophy (RSD), two with peripheral vascular ischemic disease, one with post-thoracotomy pain syndrome, one with leg pain following resection of angiolipoma, one with traumatic superficial peroneal neuropathy, and one with Pancoast's tumor. "
3.	Cesarean Section (Caesarean Section) 09/01/1995 - "A 34-year-old woman with severe kyphoscoliosis, an immobile cervical spine, and short stature due to the Klippel-Feil syndrome presented for elective Caesarean section. " 09/01/1995 - "Anaesthesia for caesarean section in a patient with Klippel-Feil syndrome. " 08/01/2015 - "Airway management with McGrath Series 5 video laryngoscope in a woman with Klippel-Feil syndrome requiring urgent caesarean section." 09/01/1988 - "A mother with the Klippel-Feil syndrome, congenital hydrocephalus and increased intracranial pressure presented for delivery by Caesarean section at 33 weeks because of pre-eclampsia. " 09/01/1988 - "Anaesthetic management of caesarean section for a mother with pre-eclampsia, the Klippel-Feil syndrome and congenital hydrocephalus."
4.	Arthroplasty 03/01/2011 - "Cervical arthroplasty may be justified in patients with Klippel-Feil syndrome (KFS) in order to preserve cervical motion. " 03/01/2011 - "Failure of cervical arthroplasty in a patient with adjacent segment disease associated with Klippel-Feil syndrome." 10/01/2007 - "Bryan artificial cervical disc arthroplasty in a patient with Klippel-Feil syndrome." 08/01/2007 - "Arthroplasty may be performed in selected patients with Klippel-Feil syndrome in order to restore motion and to prevent degeneration of the adjacent segment by reducing hypermobility." 08/01/2007 - "This is the first published report of a patient with Klippel-Feil syndrome treated with cervical arthroplasty. "
5.	Laryngoscopes 12/01/2013 - "Pentax-AWS video laryngoscope for tracheal intubation in a patient with Klippel-Feil syndrome." 08/01/2015 - "Airway management with McGrath Series 5 video laryngoscope in a woman with Klippel-Feil syndrome requiring urgent caesarean section."