icd-10-cm code for klippel-feil syndrome

What is Klippel-Feil syndrome?

Definition. Klippel-Feil Syndrome is a rare disorder characterized by the congenital fusion of two or more cervical (neck) vertebrae. It is caused by a failure in the normal segmentation or division of the cervical vertebrae during the early weeks of fetal development.

What is Klippel-Feil sequence?

Klippel-Feil sequence: Short neck, low hairline at the nape of the neck and limited movement of the head. Klippel-Feil sequence is due to a defect in the early development of the spinal column in the neck, resulting in fusion of the cervical vertebrae.

Is Klippel-Feil syndrome a neural tube defect?

Rarely, structural brain abnormalities or a type of birth defect that occurs during the development of the brain and spinal cord (neural tube defect) can occur in people with Klippel-Feil syndrome.

What is the classic triad of Klippel-Feil?

It has been propagated that KFS patients exhibit the “clinical triad” findings, known as a short neck, low posterior hairline, and a limited cervical range of motion (ROM), which aids in the identification of this syndrome [2, 3].

How is Klippel-Feil syndrome diagnosed?

Genetic testing. If at least 2 cervical vertebrae are fused and other aspects of the exam signal that the condition is congenital (from birth), Klippel-Feil syndrome is diagnosed.

What causes KFS syndrome?

In 2008, researchers determined that some cases of autosomal dominant and sporadic KFS are caused by mutations of the GDF6 gene located on chromosome 8. The GDF6 gene produces a growth factor that is involved in the production and function of cartilage in the developing embryo.

Is Klippel Feil syndrome life threatening?

Some cases of KFS can become serious and potentially life-threatening in a few different ways: Cervical myelopathy.

Is Klippel-Feil a disability?

Klippel-Feil Syndrome, a rare birth disability.

Is Klippel Feil syndrome life expectancy?

In less than 30% of cases, individuals with KFS will present with heart defects. If these heart defects are present, they often lead to a shortened life expectancy, the average being 35–45 years of age among males and 40–50 among females. This condition is similar to the heart failure seen in gigantism.

When is Klippel-Feil syndrome diagnosed?

It was first identified in 1912 by Frenchmen Maurice Klippel and André Feil. Klippel-Feil syndrome forms in utero when genes GDF6 or GDF3 mutate. The condition is present at birth, but it may go undetected for years if the symptoms are slight.

How is Klippel-Feil syndrome treated?

Physical therapy. A physical therapist or other medical professional can design a stretching and strengthening program to fit the patient's unique needs. A physical therapy program may help improve strength and mobility while reducing some types of pain and stiffness. Prescription pain medications.

What does Hydrosyringomyelia mean?

(si-ring'gō-mī-ē'lē-ă) The presence in the spinal cord of longitudinal cavities lined by dense, gliogenous tissue, which are not caused by vascular insufficiency.

What is the ICd code for Klippel-Feil syndrome?

The ICD code Q761 is used to code Klippel-Feil syndrome. Klippel–Feil syndrome is a rare disease, initially reported in 1912 by Maurice Klippel and André Feil from France, characterized by the congenital fusion of any two of the seven cervical vertebrae.:578 . The syndrome occurs in a heterogeneous group of patients unified only by the presence ...

What is billable code?

Billable codes are sufficient justification for admission to an acute care hospital when used a principal diagnosis. The Center for Medicare & Medicaid Services (CMS) requires medical coders to indicate whether or not a condition was present at the time of admission, in order to properly assign MS-DRG codes.