2026 ICD-10-CM Diagnosis Code E71.541Zellweger-like syndrome

ICD-10-CM Codes›E00–E89›E70-E88›E71

ICD-10-CM E71.541
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

E71.541 is a billable ICD-10-CM diagnosis code for zellweger-like syndrome. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026). Coders also document this condition as contiguous ABCD1 DXS1357E deletion syndrome. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Other specified and unspecified nutritional and metabolic disorders.

Code Identity

ICD-10-CM Code
E71.541
Billable Status
Yes — Valid for Submission
Code Describes
Zellweger-like syndrome
Short Description
Zellweger-like syndrome
Same as the full description in the CMS dataset.
Parent Code
E71.54
Other peroxisomal disorders

Code Classification

ChapterE00–E89Endocrine, nutritional and metabolic diseases
SectionE70-E88Metabolic disorders
CategoryE71Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism
This CodeE71.541Zellweger-like syndrome

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Contiguous ABCD1 DXS1357E deletion syndrome
  • Loss of multiple peroxisomal functions
  • Zellweger-like syndrome without peroxisomal anomaly
  • Zellweger's-like syndrome

Index to Diseases and InjuriesGuidance

Alphabetical index entries that point to this code.

External Cause of Injuries IndexGuidance

References for this code in the External Cause of Injuries Index.

    • Disorder(of)
      • peroxisomal
        • Zellweger-like syndrome
    • Syndrome
      • Zellweger-like syndrome

Clinical ClassificationClinical

AHRQ’s CCSR groups this code into broader clinical categories.

CCSR END016
Other specified and unspecified nutritional and metabolic disorders
Default principal diagnosis: inpatient Yes · outpatient Yes

Clinical InformationClinical

  • Peroxisomal Disorders

    a heterogeneous group of inherited metabolic disorders marked by absent or dysfunctional peroxisomes. peroxisomal enzymatic abnormalities may be single or multiple. biosynthetic peroxisomal pathways are compromised, including the ability to synthesize ether lipids and to oxidize long-chain fatty acid precursors. diseases in this category include zellweger syndrome; infantile refsum disease; rhizomelic chondrodysplasia (chondrodysplasia punctata, rhizomelic); hyperpipecolic acidemia; neonatal adrenoleukodystrophy; and adrenoleukodystrophy (x-linked). neurologic dysfunction is a prominent feature of most peroxisomal disorders.

Patient EducationClinical

Metabolic Disorders

Metabolism is the process your body uses to get or make energy from the food you eat. Food is made up of proteins, carbohydrates, and fats. Chemicals in your digestive system break the food parts down into sugars and acids, your body's fuel.

Read the full article at MedlinePlus

Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.

Convert E71.541 to ICD-9-CMHistory

The closest ICD-9-CM equivalents under the General Equivalence Mappings.

ICD-9-CM
277.86 Peroxisomal disorders
Approximate The match is approximate rather than exact.

Code HistoryHistory

FY 2016AddedAdded to the ICD-10-CM code setEffective October 1, 2015, the first year of ICD-10-CM.
FY 2017–2025No changes
FY 2026CurrentCurrent code set, no changesEffective October 1, 2025 through September 30, 2026.

Questions About E71.541Overview

Is E71.541 (Other peroxisomal disorders) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report zellweger-like syndrome on HIPAA-covered claims from October 1, 2025 through September 30, 2026.

What is the ICD-9 equivalent of E71.541?

Under the General Equivalence Mappings, zellweger-like syndrome converts to ICD-9-CM 277.86 (peroxisomal disorders). The mapping is approximate, so confirm the match fits the documentation.

Footnotes

[1] Chronic - a chronic condition code indicates a condition lasting 12 months or longer and its effect on the patient based on one or both of the following criteria:

  • The condition results in the need for ongoing intervention with medical products,treatment, services, and special equipment
  • The condition places limitations on self-care, independent living, and social interactions.