Other specified congenital malformation syndromes affecting multiple systems
In Other congenital malformations (Q80-Q89), chapter 17: Congenital Malformations, Deformations and Chromosomal Abnormalities
21 billable codes under Q87: 20 are a CC; none map to an HCC.A CC or MCC (major CC) is a secondary diagnosis that raises the MS-DRG payment.
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Instructional notes
Use additional code: add a second code for this, listed after this one
- code(s) to identify all associated manifestations
Official coding guidelines
From the ICD-10-CM Official Guidelines for Coding and Reporting, FY 2027 (CMS and NCHS). Links open the PDF at the right page.
Assign Q00-QA1 codes for documented congenital anomalies or genetic disorders at any age, coding non-inherent manifestations separately and sequencing Z38 first on the birth admission.
All codes under Q87
Q87 can't be used on a claim. Pick a billable code below; grey headings group them.
- Q87.0Congenital malformation syndromes predominantly affecting facial appearance
- Q87.1Congenital malformation syndromes predominantly associated with short stature
- Q87.11Prader-Willi syndrome
- Q87.19Other congenital malformation syndromes predominantly associated with short stature
- Q87.2Congenital malformation syndromes predominantly involving limbs
- Q87.3Congenital malformation syndromes involving early overgrowth
- Q87.4Marfan syndrome
- Q87.40Marfan syndrome, unspecified
- Q87.41Marfan syndrome with cardiovascular manifestations
- Q87.410Marfan syndrome with aortic dilation
- Q87.418Marfan syndrome with other cardiovascular manifestations
- Q87.42Marfan syndrome with ocular manifestations
- Q87.43Marfan syndrome with skeletal manifestation
- Q87.5Other congenital malformation syndromes with other skeletal changes
- Q87.8Other specified congenital malformation syndromes, not elsewhere classified
- Q87.81Alport syndrome
- Q87.82Arterial tortuosity syndrome
- Q87.83Bardet-Biedl syndrome
- Q87.84Laurence-Moon syndrome
- Q87.85MED13L syndrome
- Q87.86Kleefstra syndrome
- Q87.87Hao-Fountain Syndrome
- Q87.88CTNNB1 syndrome
- Q87.89Other specified congenital malformation syndromes, not elsewhere classified
- Q87.ALoeys-Dietz syndrome
Q87 MS-DRGs
As the principal diagnosis, the billable codes under Q87 group to these 7 MS-DRGs (v44.0). Which one applies depends on procedures performed, secondary diagnoses (CC/MCC), and the patient's age and discharge status.
- DRG 564 Other Musculoskeletal System and Connective Tissue Diagnoses with MCC (15 of 21 codes)
- DRG 565 Other Musculoskeletal System and Connective Tissue Diagnoses with CC (15 of 21 codes)
- DRG 566 Other Musculoskeletal System and Connective Tissue Diagnoses without CC/MCC (15 of 21 codes)
- DRG 306 Cardiac Congenital and Valvular Disorders with MCC (5 of 21 codes)
- DRG 307 Cardiac Congenital and Valvular Disorders without MCC (5 of 21 codes)
- DRG 052 Spinal Disorders and Injuries with CC/MCC (1 of 21 codes)
- DRG 053 Spinal Disorders and Injuries without CC/MCC (1 of 21 codes)
Other codes that refer to Q87
Notes on these codes point to Q87.
About the condition
Plain-language background from MedlinePlus (National Library of Medicine), not coding guidance.
Birth Defects
A birth defect is a problem that happens while a baby is developing in the mother's body. Most birth defects happen during the first 3 months of pregnancy. One out of every 33 babies in the United States is born with a birth defect.
Read more about birth defects on MedlinePlusCode history
Valid since FY2016, when ICD-10-CM took effect on October 1, 2015. No changes since.