AHA Coding Clinic® for ICD-10-CM and ICD-10-PCS - 2026 Quarter 4; New/Revised ICD-10-CM Codes

Loeys-Dietz Syndrome

Code Q87.A , Loeys-Dietz syndrome, was created to capture Loeys-Dietz syndrome (LDS), a rare, genetic autosomal dominant disorder that affects the body’s connective tissue. LDS is caused by random gene mutations occurring in the TGFBR1, TGFBR2, SMAD2, SMAD3, TGFB2, or TGFB3 genes. While most people inherit LDS, it can also occur spontaneously with no previous family history.  LDS can affect multiple body systems including craniofacial features (ocular hypertelorism, split uvula or cleft palate, craniosynostosis), musculoskeletal conditions (scoliosis, spine instabilities, chest wall deformities, joint contractures or hypermobility), gastrointestinal manifestations (eosinophilic esophagitis, inflammatory bowel disease), food or environmental allergies...

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