1. ICD-10-CM Codes
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2. E00-E89 Endocrine, nutritional and metabolic diseases
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3. E70-E88 Metabolic disorders
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4. E76- Disorders of glycosaminoglycan metabolism
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2018 ICD-10-CM Diagnosis Code E76.219

Morquio mucopolysaccharidoses, unspecified

2016 2017 2018 Billable/Specific Code

• E76.219 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.
• The 2018 edition of ICD-10-CM E76.219 became effective on October 1, 2017.
• This is the American ICD-10-CM version of E76.219 - other international versions of ICD-10 E76.219 may differ.

• Morquio syndrome
• Mucopolysaccharidosis mps-iv-a
• Mucopolysaccharidosis mps-iv-b

• A rare autosomal recessive lysosomal storage disease characterized by abnormal skeletal developments, dwarfism, heart disorders, and central nervous system deficits.
• An inborn error of metabolism characterized by faulty degradation of keratan sulfate with lysosomal accumulation and mucopolysaccharidosis, keratansulfaturia. Clinical features include short trunk dwarfism, dysostosis multiplex, progressive spinal deformity, short neck, pectus carinatum, genu valgum, pes planus, and odontoid hypoplasia with varying degrees of severity. Mental development is usually normal but progressive intellectual deterioration was reported in type b. Two types are recognized according to the enzymes involved: type a: synonyms: morquio syndrome a galactosamine-4-sulfatase (galns) deficiency mucopolysaccharidosis (mps) iv a this type is caused by galactosamine-6-sulfate sulfatase (ec 3.16.4) and is more severe than type b. It is marked by shortness and hyperextension of the neck causing the head to appear as if it were resting directly on the shoulders, short trunk, long extremities with excessive joint mobility, kyphosis or kyphoscoliosis, pectus carinatum, the sternum extending from clavicular junction and angling downward in midsection, spinal cord compression associated with atlantoaxial dislocation and thoracolumbar gibbus, protruding abdomen, and clouding of the cornea type b: synonyms: morquio syndrome b morquio-like syndrome beta-galactosidase deficiency mucopolysaccharidosis (mps) iv b this type is caused by beta-galactosidase (ec 3.2.1.23) deficiency and is marked a milder phenotype consisting of dysostosis multiplex, pectus carinatum, odontoid hypoplasia, kyphosis, genua valga, platyspondyly, and corneal clouding.
• Genetic disorder of mucopolysaccharide metabolism characterized by skeletal abnormalities, joint instability, development of cervical myelopathy, and excessive urinary keratan sulfate.
• Genetic disorder of mucopolysaccharide metabolism characterized by skeletal abnormalities, joint instability, development of cervical myelopathy, and excessive urinary keratan sulfate. There are two biochemically distinct forms, each due to a deficiency of a different enzyme.

• 642 Inborn and other disorders of metabolism

Convert E76.219 to ICD-9-CM

• 2016 (effective 10/1/2015): New code (first year of non-draft ICD-10-CM)
• 2017 (effective 10/1/2016): No change
• 2018 (effective 10/1/2017): No change

• Chondro-osteodysplasia E76.219 (Morquio-Brailsford type)
• Kyphosis, kyphotic (acquired) M40.209
  ICD-10-CM Diagnosis Code M40.209

  Unspecified kyphosis, site unspecified

  2016 2017 2018 Billable/Specific Code
  • Morquio-Brailsford type E76.219 (spinal) - see also subcategory M49.8
• Mucopolysaccharidosis E76.3
  ICD-10-CM Diagnosis Code E76.3

  Mucopolysaccharidosis, unspecified

  2016 2017 2018 Billable/Specific Code
  • Morquio syndrome E76.219
  • type
    • IV E76.219

Reimbursement claims with a date of service on or after October 1, 2015 require the use of ICD-10-CM codes.