Health Information Management

Kabuki syndrome

JustCoding News: Outpatient, June 30, 2010

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Kabuki syndrome, previously known as Kabuki makeup syndrome or Niikawa-Kurok syndrome, is a rare pediatric congenital disorder that typically includes mental retardation, unusual facial features (e.g., arched eyebrows, thick eyelashes, etc.), skeletal abnormalities, and ridges on the skin. Its causes are unknown, however, it is believed to be an inherited disorder.

There are no additional types/stages of Kabuki syndrome.

Signs and Symptoms
The syndrome affects each individual differently. However, following are some of the more common symptoms:

  • Heart defects
  • Urinary tract anomalies
  • Hearing loss
  • Hypotonia
  • Postnatal growth deficiency

Additional characteristics may include:

  • Skeletal abnormality
  • Cleft lip and palate
  • Joint laxity
  • Short stature
  • Recurring ear infections in infancy
  • Mild to moderate intellectual disability


Treatment for Kabuki syndrome is based on the individual problems the child experiences, and most likely involves a multidisciplinary team of specialists.

In addition to surgery or medication, treatment for Kabuki syndrome may also include:

  • Early intervention. Specialists provide therapy for cognitive and physical developmental problems, and provide education and support to parents.
  • Physical and occupational therapy. This strengthens muscle tone and lessens the chance of joint dislocations. Occupational therapy helps develop coordination and fine-motor skills.
  • Speech therapy. Problems with the palate, hearing loss, and hypotonia can all contribute to problems with speech.
  • Sensory integration therapy. This treatment helps children who experience sensory problems such as an aversion to the feeling of certain textures.

Documentation and coding

ICD-9-CM coding

  • Kabuki syndrome: Report code 759.89 Other specified anomalies
  • The Tabular list identifies “Congenital malformation syndromes affecting multiple systems, not elsewhere classified” as an example condition for the code.
  • For inpatient coding, Kabuki syndrome may be a CC and may affect DRG assignment.

ICD-10-CM coding

  • Kabuki syndrome: Report code Q89.8 Other specified anomalies. Use additional code(s) to identify all associated manifestations

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