756.16 is a legacy non-billable code used to specify a medical diagnosis of klippel-feil syndrome. This code was replaced on September 30, 2015 by its ICD-10 equivalent.
When Klippel-Feil syndrome is a feature of another disorder, such as fetal alcohol syndrome, Goldenhar syndrome, Wildervanck syndrome or hemifacial microsomia, among others, it is caused by mutations in genes involved in the other disorder.
Prognosis. The long-term outlook ( prognosis) for people with Klippel Feil syndrome varies depending on the specific features and severity in each affected person. While all affected people have fusion of at least two vertebrae of the neck, additional signs and symptoms (if present) can vary greatly.
As of October 2015, ICD-9 codes are no longer used for medical coding. Instead, use this equivalent ICD-10-CM code, which is an exact match to ICD-9 code 756.16:
Billable codes are sufficient justification for admission to an acute care hospital when used a principal diagnosis.
Listen. Klippel Feil syndrome (KFS) is a condition affecting the development of the bones in the spine. People with KFS are born with abnormal fusion of at least two spinal bones (vertebrae) in the neck. Common features may include a short neck, low hairline at the back of the head, and restricted movement of the upper spine.
Klippel Feil syndrome (KFS) is typically diagnosed based on a clinical examination, symptoms and imaging studies ( X-rays, MRI or CT scan ). Additional studies may be necessary to look at other parts of the skeleton and other body systems. Some people with KFS have few or no symptoms, and are diagnosed by chance after having imaging studies for some other reason. [1] [2]
Most estimates suggest that 1 in 40,000 to 1 in 42,000 people have Klippel Feil syndrome (KFS). [3] [5] One study suggested a higher incidence of about 1 in 21,000 people [3] based on clinical examinations of people with and without symptoms. The exact incidence of KFS is unknown.
Some people with KFS have no symptoms. Others may have frequent headaches, back and neck pain, and other nerve issues. People with KFS are at risk for severe spinal injury. KFS can occur along with other types of birth defects, and sometimes KFS occurs as a feature of another disorder or syndrome.
Listen. The exact underlying causes and mechanisms of Klippel Feil syndrome (KFS) are not well understood. Most cases of isolated KFS (meaning not associated with another syndrome) are sporadic, meaning there is no known family history or obvious genetic cause . [4] .