AHA Coding Clinic® for ICD-10-CM and ICD-10-PCS - 2025 Issue 4; New/Revised ICD-10-CM Codes

Hao-Fountain Syndrome

Code Q87.87, Hao-Fountain Syndrome, has been created at subcategory Q87.8, Other specified congenital malformations syndromes, not elsewhere classified, to specifically identify Hao-Fountain syndrome.Hao-Fountain syndrome (HAFOUS) is a rare neurodevelopmental disorder caused by a mutation of the USP7 (ubiquitin-specific protease 7) gene on chromosome 16p13.2. Its prevalence is estimated to be 1 in 40,000. Most people with this syndrome, after onset in infancy or childhood, usually have dysmorphic facial features, global developmental delay, intellectual disability, impaired motor skills, speech delay, and behavioral abnormalities, such as autism spectrum disorder. Some patients with this disorder develop seizures...

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