The Long-Term Follow-Up of Patients With Klippel-Feil Syndrome and Congenital Scoliosis

Theiss, Steven M.; Smith, Michael D.; Winter, Robert B.

doi:10.1097/00007632-199706010-00009

Cited by 69 publications

(42 citation statements)

References 18 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…5 Neurological sequelae can arise later in life, with an estimated incidence of between 22% and 43%. 7,18,19 Pizzutillo et al 20 reported that basilar impression and hypermobility at the upper motion segment were often responsible for myelopathy, but other possible causes of neurological deficits include craniovertebral abnormalities, spinal stenosis and scoliosis. 7,9 Spontaneous fusion of C2 and C3 with occipitalisation of C1 is strongly associated with instability and myelopathy.…”

Section: Discussionmentioning

confidence: 99%

Posterior occipitocervical fixation under skull-femoral traction for the treatment of basilar impression in a child with Klippel–Feil syndrome

Dokai

Nagashima

Nanjo

et al. 2011

The Journal of Bone and Joint Surgery. British volume

View full text Add to dashboard Cite

We present the case of a 15-year-old boy with symptoms due to Klippel-Feil syndrome. Radiographs and CT scans demonstrated basilar impression, occipitalisation of C1 and fusion of C2/C3. MRI showed ventral compression of the medullocervical junction. Skull traction was undertaken pre-operatively to determine whether the basilar impression could be safely reduced. During traction, the C3/C4 junction migrated 12 mm caudally and spasticity resolved. Peri-operative skull-femoral traction enabled posterior occipitocervical fixation without decompression. Following surgery, cervical alignment was restored and spasticity remained absent. One year after surgery he was not limited in his activities. The surgical strategy for patients with basilar impression and congenital anomalies remains controversial. The anterior approach with decompression is often recommended for patients with ventral compression of the medullocervical region, but such procedures are technically demanding and carry a significant risk of complications. Our surgical strategy was an alternative solution. Prior to a posterior cervical fixation, without decompression, skull traction was used to confirm that the deformity was reducible and effective in resolving associated myelopathy.

show abstract

Section: Discussionmentioning

confidence: 99%

Posterior occipitocervical fixation under skull-femoral traction for the treatment of basilar impression in a child with Klippel–Feil syndrome

Dokai

Nagashima

Nanjo

et al. 2011

The Journal of Bone and Joint Surgery. British volume

View full text Add to dashboard Cite

show abstract

“…Children with Type I or Type III KlippelFeil syndrome usually suffer from severe accessory defects (Barnes, 1994); not all of them become manifest in bones. In the literature, abnormalities such as neck pain, hypomobility of the neck, fusion of the occipito-atlantal joint, basilar impression, eight cervical vertebrae (Gunderson et al, 1967), skoliosis (often caused by hemivertebrae; Resnick, 2002), renal anomalies, Sprengel's deformity, hearing impairment, synkinesis or heart defects (Hensinger et al, 1974), vasculopathies, diseases of the central nervous system and the skeletal system (Theiss et al, 1997) have all been reported. Furthermore, spina bifida, menigocele, deafness or deafmuteness is frequently mentioned (Everberg et al, 1962), as well as os odontoideum (this is a separation of the odontoid process from the body of the axis; Sherk & Dawoud, 1981).…”

Section: Discussionmentioning

confidence: 99%

Klippel‐Feil syndrome in an Early Hungarian Period juvenile skeleton from Austria

Pany

Teschler-Nicola

2006

Intl J of Osteoarchaeology

View full text Add to dashboard Cite

The excellently preserved skeleton of a juvenile from the Early Hungarian Period (10 th century AD), recently excavated in Gnadendorf (Lower Austria), displays typical symptoms of Type II congenital 'Klippel-Feil syndrome' (KFS): fused cervical vertebrae 2 and 3, and fused thoracic vertebrae 2 and 3. Other features observed in this skeleton clinically reported for KFS include a basilar impression and a spina bifida occulta. The bilateral symmetrical hypoplasia of the basilar part of the occipital bone is probably also linked to this syndrome, as well as the constricted external acoustic meati, which almost certainly led to hearing impairment. A cranial lesion and its possible consequences are discussed in the context of the specific KFS alterations.

show abstract

“…68 Currently, Klippel-Feil syndrome is observed as a syndrome that unites a heterogeneous group of patients only by the presence of a congenital synostosis of two or more cervical vertebrae. 62,67,69 Attempts have been made to further subdivide this heterogeneous group of patients. For example, Samartzis and colleagues proposed a classification method based on radiological findings found in patients with Klippel-Feil syndrome.…”

Section: Transition To a Modern View Of Klippel-feil Syndromementioning

confidence: 99%

Monsters and the case of L. Joseph: André Feil's thesis on the origin of the Klippel-Feil syndrome and a social transformation of medicine

Belykh

Malik

Simoneau

et al. 2016

FOC

View full text Add to dashboard Cite

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. In particular, Feil's 1919 medical school thesis on cervical abnormalities was a critical publication in defying long-held theory and opinion that human “monstrosities,” anomalies, developmental abnormalities, and altered congenital physicality were a consequence of sinful behavior or a reversion to a primitive state. Indeed, his thesis on a spinal deformity centering on his patient, L. Joseph, was at the vanguard for a new view of a patient as nothing less than fully human, no matter his or her physicality or appearance.

show abstract

The Long-Term Follow-Up of Patients With Klippel-Feil Syndrome and Congenital Scoliosis

Cited by 69 publications

References 18 publications

Posterior occipitocervical fixation under skull-femoral traction for the treatment of basilar impression in a child with Klippel–Feil syndrome

Posterior occipitocervical fixation under skull-femoral traction for the treatment of basilar impression in a child with Klippel–Feil syndrome

Klippel‐Feil syndrome in an Early Hungarian Period juvenile skeleton from Austria

Monsters and the case of L. Joseph: André Feil's thesis on the origin of the Klippel-Feil syndrome and a social transformation of medicine

Contact Info

Product

Resources

About