Q87.11

Billable

Prader-Willi syndrome

HCC Category Mapping

RxHCCHCC 148 — Mild Intellectual Disabilities

0.000

What This Code Means

Prader-Willi syndrome is an inherited genetic disorder characterized by severe obesity, intellectual disability, short stature, and distinctive facial features due to loss of paternal gene expression.

Coding Tips

•Document the genetic confirmation method (deletion, uniparental disomy, or imprinting defect) when available
•Code associated complications separately (obesity, diabetes, hypogonadism) for comprehensive documentation

Clinical Significance

Prader-Willi syndrome is a complex genetic disorder with significant implications for growth, behavior, and metabolic function, requiring lifelong multidisciplinary management including endocrinology, nutrition, and behavioral support. The condition carries substantial risk for life-threatening obesity and requires specialized care approaches throughout all life stages from infancy to adulthood.

Documentation Requirements

✓Genetic confirmation of PWS (deletion, uniparental disomy, etc.)
✓Characteristic neonatal features (hypotonia, feeding difficulties)
✓Hyperphagia and obesity development
✓Short stature and growth hormone deficiency
✓Intellectual disability or developmental delays
✓Behavioral characteristics and management needs
✓Endocrine abnormalities documented
✓Multidisciplinary care team involvement

Commonly Confused Codes

E66.09 — Obesity, unspecified (symptom, not underlying syndrome)F70 — Mild intellectual disabilities (manifestation, not primary diagnosis)E23.0 — Hypopituitarism (endocrine manifestation)Q87.19 — Other congenital malformation syndromes with growth disorders

Code Hierarchy

└Q87Other specified congenital malformation syndromes affecting multiple systems └Q87.1Congenital malformation syndromes predominantly associated with short stature └Q87.11Prader-Willi syndrome

└Q87.11Prader-Willi syndrome