This is last article in discussion of Klippel Feil Syndrome. We have discusesed cause and associated anomalies. We would be discussing Diagnosis and treatment today.
Radiological examination is important in establishing a diagnosis and in determining the extent of the deformity.
The cervical vertebrae are often obscured by the overlapping occiput and mandible; not infrequently, one may have to resort to laminography for proper visualization.
Radiographic Features
The vertebral bodies are often flattened and widened. The intervertebral discs are narrowed or obliterated. Cervical spina bifida is very common.
Hemivertabrae, cervical ribs, and platybasia have been observed in association with the Klippel-Feil syndrome.
In the differential diagnosis, one should consider
- Bilateral Sprengel deformity
- Acquired fusion of the vertebrae following fracture healing
- Inflammatory conditions such as rheumatoid arthritis or discitis.
Lateral flexion extension radiograms demonstrate vertebral instability and the level of involvement.
Treatment
Treatment consists of passive stretching exercises to obtain the maximum range of motion.
These should be started immediately after birth and continued throughout the growth period.
If there is associated kyphoscoliosis, a Milwaukee brace may be somewhat beneficial.
Instability of the cervical spine is treated by fusion. Surgical efforts are directed toward the improvement of both appearance and function.
Webbing of the skin that exaggerates the deformity can often be alleviated by plastic procedures.
Contracture of the sternocleidomastoid muscle can be corrected by its division or partial excision.
If the Klippel-Feil syndrome occurs in association with Sprengel’s deformity, the high scapula may be surgically restored to its normal position, thus increasing the apparent length of the neck.
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