ICD-10-CM 2027
ICD-10 code for first arch syndrome
Congenital malformation syndromes predominantly affecting facial appearance
From the official ICD-10-CM alphabetic index entry “Syndrome › first arch”. Page updated September 29, 2026.
About coding first arch syndrome
The ICD-10-CM code for first arch syndrome is Q87.0 (Congenital malformation syndromes predominantly affecting facial appearance).
Within Q87 (other specified congenital malformation syndromes affecting multiple systems), Q87.0 is specifically for congenital malformation syndromes predominantly affecting facial appearance. Related codes cover congenital malformation syndromes predominantly associated with short stature (Q87.1), congenital malformation syndromes predominantly involving limbs (Q87.2), congenital malformation syndromes involving early overgrowth (Q87.3), Marfan syndrome (Q87.4), other congenital malformation syndromes with other skeletal changes (Q87.5), other specified congenital malformation syndromes, not elsewhere classified (Q87.8) and Loeys-Dietz syndrome (Q87.A).
Use an additional code(s) to identify all associated manifestations.
It is not a CC or MCC, so as a secondary diagnosis it doesn't change the MS-DRG severity level.
Common questions about first arch syndrome ICD-10 codes
What is the ICD-10 code for first arch syndrome?
- Q87.0 — Congenital malformation syndromes predominantly affecting facial appearance.
Is Q87.0 billable?
- Yes. Q87.0 is a billable/specific ICD-10-CM code valid for FY2027 (October 1, 2026 – September 30, 2027).
Can Q87.0 be used as a principal diagnosis?
- Yes. The Medicare Code Editor doesn't restrict Q87.0 as a principal diagnosis.
Is Q87.0 a CC or MCC?
- No. Q87.0 is neither a CC nor an MCC under MS-DRG v44.0.