Orthopedics - Oleksa A.P. 2006

Chest Deformities
Klippel-Feil Syndrome

Klippel-Feil syndrome was described by these authors in 1912 and is characterized by a classic triad of Clinical Features: a short neck, a low posterior hairline, and restricted HEAD mobility. This developmental malformation is caused by the synostosis of the cervical vertebrae, which are fused into a solid bone mass (Fig. 247).

According to Feil, the syndrome is classified into two types.

In Type I, the atlas and axis form a single unit that fuses with no more than four remaining cervical vertebrae. This type may also be accompanied by other congenital anomalies, such as Spina bifida, abnormally high attachment of the upper Ribs to the vertebrae, and others.

Type II involves the fusion of the atlas with the Occipital bone (occipitalization of the atlas) and the synostosis of several lower cervical vertebrae, often accompanied by additional Cervical ribs.

This syndrome may also be associated with other developmental defects, such as basilar impression (platybasia) and hemivertebrae.

Vertebral synostosis occurs during early embryonic development, preventing normal vertebral growth. Consequently, the affected vertebrae are reduced in height with absent intervertebral discs, resulting in a shortened neck.

In all patients with Klippel-Feil syndrome without exception, the neck is clinically shortened (dystrophia brevicollis) to such an extent that affected individuals are often referred to as "homme sans cou" (neckless man) or described as having a "frog neck."

In severe cases, the chin may rest against the Sternum, and the ears may Touch the shoulders. This anatomical configuration simultaneously leads to a low-set hairline. The underdevelopment and synostosis of the cervical vertebrae severely restrict head movement in all planes.

Partsch suggested that this syndrome stems from an arrested Development of the entire cervical segment. Consequently, In addition to skeletal abnormalities, patients may experience Nervous system disorders manifested by paresis, sensory disturbances, and decreased electrical excitability of Muscles. Patients typically experience no pain.

The Diagnosis of Klippel-Feil syndrome is straightforward and relies on clinical manifestations and radiographic (tomographic) findings, taking into account the congenital Nature of the defect in the patient's history.

Class="center">

Fig. 247. A 12-year-old girl with Klippel-Feil syndrome (Biezins A.P., 1968).

The syndrome rarely causes significant Complaints other than cosmetic disfigurement and restricted head rotation. However, it should be noted that secondary musculoskeletal changes develop with age, often accompanied by compensatory Scoliosis, as well as impaired pulmonary ventilation and swallowing difficulties.

Treatment involves skeletal traction during the growth period, The Use of corrective cervical collars, Therapeutic Exercises, and Muscle massage.