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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Article Overview
The article introduces Loeys-Dietz syndrome as a rare connective tissue condition and surveys the range of body systems it may affect. It also touches on the condition’s genetic background and the broad focus of ongoing care.
Why This Topic Matters
The article offers a concise orientation to a rare condition with varied manifestations, including cardiovascular concerns, for readers seeking general clinical and coding context.
What You Will Learn
- The genetic background discussed for Loeys-Dietz syndrome.
- The broad categories of manifestations described across body systems.
- The general care context addressed in the article.
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Who Should Read This
- Medical coders
- Healthcare professionals
- Readers seeking general information about rare genetic conditions
Codes Discussed
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