Q87.89 Other specified congenital malformation syndromes, not elsewhere classified

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)

MS-DRG index

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

Approximate ICD-9-CM (GEMs)

• 759.89 Other specified congenital anomalies (approximate)

Code annotations containing back-references to Q87.89

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 Q87.89

• Birt-Hogg-Dube syndrome
• Cantrell's syndrome
• Cerebro-hepato-renal syndrome
• Syndrome › basal cell nevus
• Syndrome › Birt-Hogg-Dube syndrome
• Syndrome › cardiofaciocutaneous
• Syndrome › congenital › affecting multiple systems NEC
• Syndrome › congenital › muscular hypertrophy-cerebral
• Syndrome › Glass
• Syndrome › Gorlin's
• Syndrome › Oliver-McFarlane
• Syndrome › popliteal › web
• Syndrome › SATB2-associated
• Syndrome › Snyder-Robinson
• Web, webbed (congenital) › popliteal syndrome

Nearby codes

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