Klippel-Feil Syndrome

Going back to the year of 1912, two people have discovered a rare syndrome described as people with features of webbed neck accompanied with decreased range of motion in the cervical spine and a low hairline.

The proponents of this syndrome are Maurice Klippel and Andre Fiel. Fiel further classified these into 3 categories:

  1. Type I – fusion of the cervical spine
  2. Type II – one or two vertebrae
  3. Type III – anomalies o thoracic and lumbar spine combined with Type I or Type 2

Another classification by Samartzis and colleagues

  1. Type I – single-level fusion
  2. Type II – multiple, noncontiguous fused segments
  3. Type III – multiple, contiguous fused segments

This is called a syndrome since the etiology is still unkown. Some have proposed that it has something to do with genetics or with due to fetal alcohol syndrome.

Signs and Symptoms:

  1. Short neck
  2. Decreased cervical range of motion
  3. Spinal involvement
  4. Low hair line
  5. Loss of flexion and extension
  6. Torticollis or facial asymmetry
  7. Scoliosis

Diagnostic Evaluation:

A radiograph or series or x-ray imaging is used in order to know the extent of the syndrome as well as the complication in the internal organs.

An intravenous pyelogram can be used in order to check the kidney’s involvement, if the circulation to the kidney is affected, the kidney is described as “horse-shoe kidbey”.

Surgical management:

Surgical intervention may involve proper assessment of the patient since intubation may pose a risk when there is cervical or occipitocervical involvement. Deformity may progress due to the potential growth of the anomalies, surgical intervention can be done for cases with stable cervical spine only.

Nursing Management:

  1. Assist during the physical therapy examination, alley fears or anxiety that goes along with the patient as new procedures are introduced.
  2. Assist the patient with the activities of daily living as well as range of motion exercises as well as stretching.
  3. Encourage proper follow-up with specialists (opthamologist, orthopedics and neurologist

Daisy Jane Antipuesto RN MN

Currently a Nursing Local Board Examination Reviewer. Subjects handled are Pediatric, Obstetric and Psychiatric Nursing. Previous work experiences include: Clinical instructor/lecturer, clinical coordinator (Level II), caregiver instructor/lecturer, NC2 examination reviewer and staff/clinic nurse. Areas of specialization: Emergency room, Orthopedic Ward and Delivery Room. Also an IELTS passer.

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