Short answer · Medically reviewed summary · Last updated: 2026-05-08
Maroteaux-Lamy Syndrome, also known as Mucopolysaccharidosis type VI (MPS VI), is officially classified under ICD-10 code E76.210 and ICD-9 code 277.5. These codes are essential for medical billing, insurance authorization for enzyme replacement therapy, and clinical documentation for patients with this rare lysosomal storage disorder. What is the clinical significance of Maroteaux-Lamy Syndrome? Maroteaux-Lamy Syndrome is a progressive condition caused by a deficiency in the enzyme arylsulfatase B.
ICD10 code of Maroteaux-Lamy Syndrome and ICD9 code
ICD-10 and ICD-9 codes for Maroteaux-Lamy Syndrome, with classification details for clinicians, coders and patients.
Maroteaux-Lamy Syndrome, also known as Mucopolysaccharidosis type VI (MPS VI), is officially classified under ICD-10 code E76.210 and ICD-9 code 277.5. These codes are essential for medical billing, insurance authorization for enzyme replacement therapy, and clinical documentation for patients with this rare lysosomal storage disorder.
What is the clinical significance of Maroteaux-Lamy Syndrome?
Maroteaux-Lamy Syndrome is a progressive condition caused by a deficiency in the enzyme arylsulfatase B. This deficiency leads to the accumulation of glycosaminoglycans (GAGs) in various tissues, causing skeletal abnormalities, corneal clouding, and organ enlargement. Because Maroteaux-Lamy Syndrome is a multisystemic disease, accurate coding is vital to ensure patients receive multidisciplinary care, including cardiology, orthopedics, and ophthalmology services.
How does the diagnosis of Maroteaux-Lamy Syndrome impact medical coding?
When clinicians record Maroteaux-Lamy Syndrome, they must use the specific ICD-10 code E76.210 to distinguish it from other mucopolysaccharidoses. Proper coding facilitates access to specialized treatments like Naglazyme (galsulfase), which is the standard enzyme replacement therapy for Maroteaux-Lamy Syndrome. At DiseaseMaps.org, three community members currently share their experiences navigating these diagnostic and treatment paths, highlighting the importance of precise medical identification.
What are common features associated with Maroteaux-Lamy Syndrome?
While the severity of Maroteaux-Lamy Syndrome varies significantly between individuals, common clinical manifestations often documented alongside the ICD code include:
- Skeletal dysostosis: Joint stiffness and progressive bone deformities.
- Cardiac involvement: Valvular heart disease, particularly involving the mitral and aortic valves.
- Ocular issues: Progressive corneal clouding that may impair visual acuity.
- Respiratory challenges: Potential airway obstruction due to tissue thickening.
- Hepatosplenomegaly: Enlargement of the liver and spleen.
Next steps
- Consult with a metabolic specialist or geneticist to confirm the diagnosis and ensure the correct ICD-10 coding is reflected in your medical records.
- Connect with the 3 members of our DiseaseMaps community who are living with Maroteaux-Lamy Syndrome to share resources and experiences.
- Review your insurance coverage to ensure that treatments coded under E76.210 are fully supported.
- Visit the National MPS Society website for updated clinical guidelines and support resources.
Medical disclaimer: This information is for educational purposes only and does not constitute professional medical advice, diagnosis, or treatment; always seek the advice of your physician or other qualified health provider with any questions regarding a medical condition.
References
- National Organization for Rare Disorders (NORD): MPS VI (Maroteaux-Lamy Syndrome)
- NIH Genetic and Rare Diseases Information Center (GARD): Mucopolysaccharidosis type VI
- Orphanet: Mucopolysaccharidosis type VI (ORPHA:582)
- OMIM (Online Mendelian Inheritance in Man): #253200 (Mucopolysaccharidosis, Type VI)
