ICD-10-CM 2027
ICD-10 code for Tapetoretinal dystrophy
From the official ICD-10-CM alphabetic index entry “Dystrophy, dystrophia › tapetoretinal”. Page updated September 29, 2026.
About coding Tapetoretinal dystrophy
The ICD-10-CM code for Tapetoretinal dystrophy is H35.54 (Dystrophies primarily involving the retinal pigment epithelium).
Within H35.5 (hereditary retinal dystrophy), H35.54 is specifically for dystrophies primarily involving the retinal pigment epithelium. Related codes cover unspecified hereditary retinal dystrophy (H35.50), vitreoretinal dystrophy (H35.51), pigmentary retinal dystrophy (H35.52) and other dystrophies primarily involving the sensory retina (H35.53).
It is not a CC or MCC, so as a secondary diagnosis it doesn't change the MS-DRG severity level.
In MS-DRG v44.0, it is part of the grouping logic for DRG 124 (Other Disorders of the Eye with MCC or Thrombolytic Agent, relative weight 1.2471) and DRG 125 (Other Disorders of the Eye without MCC, relative weight 0.7702), in MDC 02 (Diseases and Disorders of the Eye), as a principal or secondary diagnosis. The DRG a claim lands in depends on the principal diagnosis, procedures and any CC/MCC secondary diagnoses.
Common questions about Tapetoretinal dystrophy ICD-10 codes
What is the ICD-10 code for Tapetoretinal dystrophy?
- H35.54 — Dystrophies primarily involving the retinal pigment epithelium.
Is H35.54 billable?
- Yes. H35.54 is a billable/specific ICD-10-CM code valid for FY2027 (October 1, 2026 – September 30, 2027).
Can H35.54 be used as a principal diagnosis?
- Yes. The Medicare Code Editor doesn't restrict H35.54 as a principal diagnosis.
Is H35.54 a CC or MCC?
- No. H35.54 is neither a CC nor an MCC under MS-DRG v44.0.