Q87.2 Congenital malformation syndromes predominantly involving limbs

The ICD-10-CM code for Congenital malformation syndromes predominantly involving limbs is Q87.2 (FY2026). It is a billable, claim-ready diagnosis code.

Classification

Chapter

17: Congenital malformations, deformations and chromosomal abnormalities (Q00-QA0)

Section

Q80-Q89: Other congenital malformations (Q80-Q89)

Category Q87

25 codes (20 billable)

FY2026 Status

Stable since FY2024

Also Known As

ICD-10-CM Alphabetic Index entries that lead to Q87.2:

• Fong's syndrome (hereditary osteo-onychodysplasia)
• Osteo-onycho-arthro-dysplasia
• Mietens' syndrome
• Klippel-Trenaunay (-Weber) syndrome
• Osteo-onychodysplasia, hereditary
• Holt-Oram syndrome
• Rubinstein-Taybi syndrome
• Onycho-osteodysplasia

Inclusion Terms

• Holt-Oram syndrome
• Klippel-Trenaunay-Weber syndrome
• Nail patella syndrome
• Rubinstein-Taybi syndrome
• Sirenomelia syndrome
• Thrombocytopenia with absent radius [TAR] syndrome
• VATER syndrome

U.S. Hospital Utilization

• An estimated 6,070 U.S. inpatient stays in 2023 included Q87.2 among the documented diagnoses.
• 120 stays listed it as the principal diagnosis.

Source: National Inpatient Sample (NIS), Healthcare Cost and Utilization Project (HCUP), Agency for Healthcare Research and Quality, 2016–2023. National survey-weighted estimates.

Official Coding Guidelines

POA indicator for congenital conditions/anomalies and Q00-Q99 exception

Congenital conditions and anomalies Assign “Y” for congenital conditions and anomalies except for categories Q00- Q99, Congenital anomalies, which are on the exempt list. Congenital conditions are always considered present on admission.

Source: CMS — ICD-10-CM Official Guidelines for Coding and Reporting, FY2026

References

• CMS — ICD-10-CM Official Code Set and Guidelines, FY2026
• CDC/NCHS — ICD-10-CM Files and Browser Tool
• AHRQ — HCUP National Inpatient Sample, Frequencies by Diagnosis Code (2016–2023)