Introduction:
Klipple-Feil Syndrome is a congenital malformation.It is first described by Maurice Klippel and Andre Feil in 1912.68% found in females. It may be because of problem in the early neural tube development.
Features:
1.short webbed neck
2,fused cervical vertebrae
3. secondary torticollis
4. facial asymmetry
5.low hairline
Frequency:
1 in 42000 live births
Types:
Type I - Single-level fusion
Type II - Multiple, noncontinuous fused segments
Type III - Multiple, contiguous fused segments
Associated anomalies:
Cleft palate
deafness
ventricular septal defect
rib defects
fetal alcohol syndrome
syringomyelia
scoliosis
cervical meningomyelocele
Sprengel anomaly
Presentation:
short neck
Decreased neck movements (ROM)
Facial asymmetry
toticollis
Investigations:
Lateral flexion-extension radiographs of the cervical spine
CT scan of cervical spine/whole spine
Ultrasonography for renal anomalies
Management:
Hyper-mobility of upper cervical spine are at risk of developing neurologic development, While hyper-mobility of lower cervical spine are exposed to early degenerative disease and its consequences.
Syptomatic spinal fusion my require decompression
