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E71.440 ICD-10-CM Code: Ruvalcaba-Myhre-Smith syndrome

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FY 2026 Apr update / Endocrine, nutritional and metabolic diseases (E00-E89) / Metabolic disorders (E70-E88)

E71.440

Billable / SpecificICD-10-CMOfficial ICD-10-CMCodebook guidance

Ruvalcaba-Myhre-Smith syndrome

Ruvalcaba-Myhre-Smith syndrome is a rare genetic disorder characterized by intellectual disability, distinctive facial features, and skeletal abnormalities. It is a type of metabolic or developmental disorder that affects multiple body systems.

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Buddy Insight

Ruvalcaba-Myhre-Smith syndrome (now recognized as part of the PTEN hamartoma tumor syndrome spectrum including Bannayan-Riley-Ruvalcaba syndrome) is characterized by macrocephaly, intestinal hamartomatous polyps, lipomas, and pigmented macules of the glans penis.

CMS-HCC V28

0

0

RAF 0

CMS-HCC V24

HCC 23

RAF 0.230

ACA/HHS

0

0

RAF 0

ESRD/PACE

HCC 23

RAF 0.0

RXHCC

HCC 43

RAF 0.0

Code Trumping

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Code Book Path

Official
E71.4Disorders of carnitine metabolism
E71.44Other secondary carnitine deficiency
E71.440Ruvalcaba-Myhre-Smith syndrome

Inclusion Terms

Official

ICD-10-CM does not list inclusion terms for E71.440 in this effective period.

Excludes 2

Official

ICD-10-CM does not list Excludes 2 notes for E71.440 in this effective period.

Related Child Codes

Official
E71.448Other secondary carnitine deficiency

Includes

Official

ICD-10-CM does not list Includes notes for E71.440 in this effective period.

Excludes 1

Official

ICD-10-CM does not list Excludes 1 notes for E71.440 in this effective period.

Code First

Official

ICD-10-CM does not list Code First sequencing instructions for E71.440 in this effective period.

Use Additional

Official

ICD-10-CM does not list Use Additional Code instructions for E71.440 in this effective period.

Code Also

Official

ICD-10-CM does not list Code Also instructions for E71.440 in this effective period.

Buddy Documentation Tip

HCC Buddy guidance
Document clinical features (macrocephaly, hamartomatous polyps, lipomas, mucocutaneous findings), PTEN gene mutation testing results, family history of PTEN-related conditions, and cancer surveillance protocol.
Record any malignancies detected during surveillance.

MEAT Support

HCC Buddy guidance
Document clinical features (macrocephaly, hamartomatous polyps, lipomas, mucocutaneous findings), PTEN gene mutation testing results, family history of PTEN-related conditions, and cancer surveillance protocol.
Record any malignancies detected during surveillance.

Audit Caution

HCC Buddy guidance
This syndrome has been reclassified in current medical literature as part of the PTEN hamartoma tumor syndrome spectrum.
Ensure documentation uses current terminology and the correct ICD-10 code for this condition.
Do not code individual manifestations without the underlying syndrome code.

Common Mistakes

HCC Buddy guidance
E71.448 (Other secondary carnitine deficiency) which is unrelated despite proximity in the code range
Q85.8 (Other phakomatoses) for related hamartoma syndromes
D12.6 (Benign neoplasm of colon, unspecified) for the polyp manifestation rather than the underlying syndrome.

Last updated: FY2026 ICD-10-CM Apr update, Apr 1, 2026 through Sep 30, 2026. CMS-HCC V28 is 100% phased in for payment year 2026.

Is E71.440 an HCC code?

Yes. E71.440 maps to Other Significant Endocrine and Metabolic Disorders under the V24 model but is not retained in V28.

HCC Category Mapping

V24HCC 23, Other Significant Endocrine and Metabolic Disorders
0.230
ESRDHCC 23, Other Significant Endocrine and Metabolic Disorders
0.000
RxHCCHCC 43, Other Significant Endocrine and Metabolic Disorders
0.000

RAF weights shown are the community, non-dual, aged base weights from the CMS risk adjustment model file. Actual per-patient RAF contribution depends on member segment, interactions, and the model year used by the payer. V28 is the CMS-HCC model phased in over payment years 2024–2026; V24 remains in use during the transition and for historical data.

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MEAT Criteria for E71.440

For E71.440to count as a valid HCC diagnosis in a given encounter, the provider's documentation must show MEAT: Monitor, Evaluate, Assess, or Treat. A diagnosis from a prior year does not carry forward automatically, it has to be re-documented and supported each calendar year.

  • MMonitor: signs, symptoms, disease progression, or lab trending documented in the note
  • EEvaluate: test results, medication response, or physical findings reviewed by the provider
  • AAssess: explicit mention in the assessment or plan with acknowledgment of status
  • TTreat: medication, referral, procedure, therapy, or counseling tied to the diagnosis

Only one of M/E/A/T is required to support the code, but the documentation must be specific enough to show that the provider actually addressed E71.440 during that encounter, not just copy-forwarded from a problem list.

Coder workflow notes

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What This Code Means

E71.440 is the ICD-10-CM diagnosis code for ruvalcaba-myhre-smith syndrome. Ruvalcaba-Myhre-Smith syndrome is a rare genetic disorder characterized by intellectual disability, distinctive facial features, and skeletal abnormalities. It is a type of metabolic or developmental disorder that affects multiple body systems. E71.440 sits in the ICD-10-CM chapter for endocrine, nutritional and metabolic diseases (e00-e89), within the section covering metabolic disorders (e70-e88).

Under the older CMS-HCC V24 model, E71.440 maps to Other Significant Endocrine and Metabolic Disorders (HCC 23) with a community, non-dual, aged base RAF weight of 0.230. V28 is the CMS-HCC risk adjustment model that reached 100% phase-in for payment year 2026, replacing V24 which was used during the PY2024–PY2025 transition.

This is a rare genetic syndrome; ensure documentation clearly identifies the diagnosis before coding to avoid confusion with other similar genetic conditions. Because E71.440 maps to a payment HCC, the provider's documentation must satisfy MEAT criteria (Monitor, Evaluate, Assess, or Treat) for the encounter to count toward the patient's Medicare Advantage risk adjustment score. When documentation is ambiguous, coders should issue a provider query rather than assume the highest-specificity variant.

HCC Buddy maintains structured V28 and V24 mapping, RAF weights, and MEAT documentation criteria for E71.440 sourced directly from the CMS-HCC risk adjustment model files and the CMS ICD-10-CM code set.

Coding Tips

  • •This is a rare genetic syndrome; ensure documentation clearly identifies the diagnosis before coding to avoid confusion with other similar genetic conditions
  • •Code E71.440 is specific to this syndrome; do not use more general metabolic disorder codes if this specific diagnosis is documented

Clinical Significance

Ruvalcaba-Myhre-Smith syndrome (now recognized as part of the PTEN hamartoma tumor syndrome spectrum including Bannayan-Riley-Ruvalcaba syndrome) is characterized by macrocephaly, intestinal hamartomatous polyps, lipomas, and pigmented macules of the glans penis. Patients carry increased cancer risk due to PTEN tumor suppressor gene mutations. Lifelong cancer surveillance is essential.

Documentation Requirements

  • ✓Document clinical features (macrocephaly, hamartomatous polyps, lipomas, mucocutaneous findings), PTEN gene mutation testing results, family history of PTEN-related conditions, and cancer surveillance protocol.
  • ✓Record any malignancies detected during surveillance.

Commonly Confused Codes

  • •E71.448 (Other secondary carnitine deficiency) which is unrelated despite proximity in the code range
  • •Q85.8 (Other phakomatoses) for related hamartoma syndromes
  • •D12.6 (Benign neoplasm of colon, unspecified) for the polyp manifestation rather than the underlying syndrome.

Child Codes

E71.440Ruvalcaba-Myhre-Smith syndrome
E71.448Other secondary carnitine deficiency

Code Hierarchy

└E71Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism└E71.4Disorders of carnitine metabolism└E71.44Other secondary carnitine deficiency└E71.440Ruvalcaba-Myhre-Smith syndrome
└E71.440Ruvalcaba-Myhre-Smith syndrome

Because E71.440 maps to a payment HCC, the documentation must also satisfy MEAT criteria (Monitor, Evaluate, Assess, or Treat) for the encounter to count toward the patient's risk adjustment score.

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