2019 ICD-10-CM Diagnosis Code E71.440

Ruvalcaba-Myhre-Smith syndrome

    2016 2017 2018 2019 Billable/Specific Code
  • E71.440 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.
  • The 2019 edition of ICD-10-CM E71.440 became effective on October 1, 2018.
  • This is the American ICD-10-CM version of E71.440 - other international versions of ICD-10 E71.440 may differ.
The following code(s) above E71.440 contain annotation back-references
Annotation Back-References
In this context, annotation back-references refer to codes that contain:
  • Applicable To annotations, or
  • Code Also annotations, or
  • Code First annotations, or
  • Excludes1 annotations, or
  • Excludes2 annotations, or
  • Includes annotations, or
  • Note annotations, or
  • Use Additional annotations
that may be applicable to E71.440:
  • E00-E89
    2019 ICD-10-CM Range E00-E89

    Endocrine, nutritional and metabolic diseases

    Note
    • All neoplasms, whether functionally active or not, are classified in Chapter 2. Appropriate codes in this chapter (i.e. E05.8, E07.0, E16-E31, E34.-) may be used as additional codes to indicate either functional activity by neoplasms and ectopic endocrine tissue or hyperfunction and hypofunction of endocrine glands associated with neoplasms and other conditions classified elsewhere.
    Type 1 Excludes
    • transitory endocrine and metabolic disorders specific to newborn (P70-P74)
    Endocrine, nutritional and metabolic diseases
  • E70-E88
    2019 ICD-10-CM Range E70-E88

    Metabolic disorders

    Type 1 Excludes
    • androgen insensitivity syndrome (E34.5-)
    • congenital adrenal hyperplasia (E25.0)
    • Ehlers-Danlos syndrome (Q79.6)
    • hemolytic anemias attributable to enzyme disorders (D55.-)
    • Marfan's syndrome (Q87.4)
    • 5-alpha-reductase deficiency (E29.1)
    Metabolic disorders
  • E71.4
    ICD-10-CM Diagnosis Code E71.4

    Disorders of carnitine metabolism

      2016 2017 2018 2019 Non-Billable/Non-Specific Code
    Type 1 Excludes
    • Muscle carnitine palmitoyltransferase deficiency (E71.314)
    Disorders of carnitine metabolism
Approximate Synonyms
  • Ruvalcaba myhre smith syndrome
  • Ruvalcaba-myhre syndrome
Clinical Information
  • A genetic syndrome caused by mutations in the pten gene. It is characterized by macrocephaly and the presence of hamartomas.
  • A syndrome originally reported (ruvalcaba) as an association of macrocephaly, intestinal polyposis, and pigmentation of the penis, bannayan later described it as a separate entity of macrocephaly, multiple lipomas, and hemangiomas, and riley and smith observed a concurrence of macrocephaly, pseudoepithelioma, and multiple hemangioma. The three conditions were recognized by dvir as different expressions of the same syndrome. The symptoms usually first become apparent in infancy but they may also appear later in life.
ICD-10-CM E71.440 is grouped within Diagnostic Related Group(s) (MS-DRG v36.0):
  • 642 Inborn and other disorders of metabolism

Convert E71.440 to ICD-9-CM

Code History
  • 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
  • 2019 (effective 10/1/2018): No change

Diagnosis Index entries containing back-references to E71.440:
  • Ruvalcaba-Myhre-Smith syndrome E71.440
  • Syndrome - see also Disease
    • Ruvalcaba-Myhre-Smith E71.440

ICD-10-CM Codes Adjacent To E71.440
E71.314 Muscle carnitine palmitoyltransferase deficiency
E71.318 Other disorders of fatty-acid oxidation
E71.32 Disorders of ketone metabolism
E71.39 Other disorders of fatty-acid metabolism
E71.4 Disorders of carnitine metabolism
E71.40 Disorder of carnitine metabolism, unspecified
E71.41 Primary carnitine deficiency
E71.42 Carnitine deficiency due to inborn errors of metabolism
E71.43 Iatrogenic carnitine deficiency
E71.44 Other secondary carnitine deficiency
E71.440 Ruvalcaba-Myhre-Smith syndrome
E71.448 Other secondary carnitine deficiency
E71.5 Peroxisomal disorders
E71.50 Peroxisomal disorder, unspecified
E71.51 Disorders of peroxisome biogenesis
E71.510 Zellweger syndrome
E71.511 Neonatal adrenoleukodystrophy
E71.518 Other disorders of peroxisome biogenesis
E71.52 X-linked adrenoleukodystrophy
E71.520 Childhood cerebral X-linked adrenoleukodystrophy
E71.521 Adolescent X-linked adrenoleukodystrophy

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