E71.510 Zellweger syndrome

Billable ICD-10-CM code, FY2026. Acts as a Complication or Comorbidity (CC) as a secondary diagnosis.

Applies from E71.5 Peroxisomal disorders

Excludes1 (never code together)

• Schilder's disease (G37.0)

Applies from E71.51 Disorders of peroxisome biogenesis

Applicable to

• Group 1 peroxisomal disorders

Excludes1 (never code together)

• Refsum's disease (G60.1)

Risk adjustment (HCC)

• CMS-HCC V22: HCC 23 — Other Significant Endocrine and Metabolic Disorders
• RxHCC V08: HCC 43 — Pituitary, Adrenal Gland, and Other Endocrine and Metabolic Disorders
• ESRD V24: HCC 23

MS-DRG index

Defines the logic of DRG(s) 642 in MDC 10.

Approximate ICD-9-CM (GEMs)

• 277.86 Peroxisomal disorders (approximate)

Code annotations containing back-references to E71.510

Back-references to E71.510

• Type 1 Excludes: E72.3 Disorders of lysine and hydroxylysine metabolism ("Zellweger syndrome (E71.510)")
• Type 1 Excludes: Q87.8 Other specified congenital malformation syndromes, not elsewhere classified ("Zellweger syndrome (E71.510)")

Back-references to E71.5

• Type 1 Excludes: E71.3 Disorders of fatty-acid metabolism ("peroxisomal disorders (E71.5)")

Back-references to E71

• Type 2 Excludes: G11 Hereditary ataxia ("metabolic disorders (E70-E88)")
• Code First: G63 Polyneuropathy in diseases classified elsewhere ("metabolic diseases (E70-E88)")
• Type 2 Excludes: G71 Primary disorders of muscles ("metabolic disorders (E70-E88)")
• Code First: H42 Glaucoma in diseases classified elsewhere ("specified metabolic disorder (E70-E88)")
• Type 2 Excludes: N25.0 Renal osteodystrophy ("metabolic disorders classifiable to E70-E88")
• Type 2 Excludes: N25.81 Secondary hyperparathyroidism of renal origin ("metabolic disorders classifiable to E70-E88")
• Type 1 Excludes: P59 Neonatal jaundice from other and unspecified causes ("jaundice due to inborn errors of metabolism (E70-E88)")
• Type 1 Excludes: R79 Other abnormal findings of blood chemistry ("specific findings indicating disorder of amino-acid metabolism (E70-E72)")
• Type 1 Excludes: R79.83 Abnormal findings of blood amino-acid level ("disorders of amino-acid metabolism (E70-E72)")

Diagnosis Index entries for E71.510

• Disorder (of) › peroxisomal › biogenesis › Zellweger syndrome
• Syndrome › Zellweger syndrome
• Zellweger's syndrome

Nearby codes

• 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 (this code)
• 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