Q87.1Congenital malformation syndromes predominantly associated with short stature

Q87.1 is the ICD-10-CM diagnosis code for congenital malformation syndromes predominantly associated with short stature. It belongs to category Q87 (other specified congenital malformation syndromes affecting multiple systems), block Q80-Q89 (other congenital malformations) and chapter 17 (congenital malformations, deformations, chromosomal abnormalities, and genetic disorders). It is a header code, so it can't be reported by itself; one of its 2 subcodes must be used instead.

Within Q87 (other specified congenital malformation syndromes affecting multiple systems), Q87.1 is specifically for congenital malformation syndromes predominantly associated with short stature. Related codes cover congenital malformation syndromes predominantly affecting facial appearance (Q87.0), 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).

To report this condition, pick the subcode that matches the documentation: Q87.11 (syndrome) and Q87.19 (stature).

Don't report Q87.1 together with the conditions in its Excludes1 note: Ellis-van Creveld syndrome (Q77.6) and Smith-Lemli-Opitz syndrome (E78.72).

Use an additional code(s) to identify all associated manifestations.

It has been part of ICD-10-CM since the code set took effect on October 1, 2015, and changed in FY2020 (expanded into subcodes).

Before ICD-10, this condition was coded in ICD-9-CM as 759.81 (Prader-Willi syndrome).