Klippel-Feil Syndrome

Incidence estimated at 1:40,000 live births (but milder cases go unrecognized).

Slight female predilection (63%).

Cervical spine instability and cardiopulmonary complications.

Often occurs in association with other clinical syndromes (e.g., fetal alcohol, Goldenhar).

Exacerbation of cervical spine instability during airway maneuvers, endotracheal intubation, and subsequent positioning.

Congenital abnormality consisting of the following triad of findings: Fusion of two or more cervical vertebrae, low posterior hairline, cervical immobility.

Type 1: Extensive fusion of many cervical vertebrae; type 2: Fusion at only one or two cervical interspaces; type 3: Fusion in the cervical spine and in the lower lumbar spine.

Severity ranges from mild (often not recognized until late in life) to severe (recognized at birth because of obvious deformity).

Careful preop assessment of cervical spine anatomy and degree of instability.

Review of systems for other congenital abnormalities: Renal dysfunction (64%), scoliosis (60%), deafness (30%), Sprengel scapular deformity (25–35%), congenital heart disease (4.2–14%), mental deficiency, pulmonary disability, and cleft lip and palate.

Unknown