Q87.86 Kleefstra syndrome

Billable ICD-10-CM code, FY2026. Acts as a Complication or Comorbidity (CC) as a secondary diagnosis. Exempt from present-on-admission (POA) reporting.

Applies from Q87 Other specified congenital malformation syndromes affecting multiple systems

Use additional code

• code(s) to identify all associated manifestations

Applies from Q87.8 Other specified congenital malformation syndromes, not elsewhere classified

Excludes1 (never code together)

• Zellweger syndrome (E71.510)

Risk adjustment (HCC)

• RxHCC V08: HCC 148 — Mild or Unspecified Intellectual Disability/Developmental Disorder

MS-DRG index

Defines the logic of DRG(s) 564-566 in MDC 08.

Code annotations containing back-references to Q87.86

Back-references to Q87

• Type 1 Excludes: Q67 Congenital musculoskeletal deformities of head, face, spine and chest ("congenital malformation syndromes classified to Q87.-")
• Type 1 Excludes: Q75 Other congenital malformations of skull and face bones ("congenital malformation syndromes classified to Q87.-")
• Type 1 Excludes: Q89.7 Multiple congenital malformations, not elsewhere classified ("congenital malformation syndromes affecting multiple systems (Q87.-)")

Diagnosis Index entries for Q87.86

• Syndrome › Kleefstra

Nearby codes

• Q87.81 Alport syndrome
• Q87.82 Arterial tortuosity syndrome
• Q87.83 Bardet-Biedl syndrome
• Q87.84 Laurence-Moon syndrome
• Q87.85 MED13L syndrome
• Q87.86 Kleefstra syndrome (this code)
• Q87.87 Hao-Fountain Syndrome
• Q87.88 CTNNB1 syndrome
• Q87.89 Other specified congenital malformation syndromes, not elsewhere classified