2027 ICD-10-CM Diagnosis Code H35.50Unspecified hereditary retinal dystrophy
ICD-10-CM Codes›H00–H59›H30-H36›H35
- Billable — Valid for Submission
- Chronic Condition
H35.50 is a billable ICD-10-CM diagnosis code for unspecified hereditary retinal dystrophy. It is valid on HIPAA claims for fiscal year 2027 (October 1, 2026 through September 30, 2027) and groups to MS-DRG 124 through 125. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Retinal and vitreous conditions.
Code Identity
Code Classification
Approximate SynonymsGuidance
Alternate terms and clinical phrases that map to this code.
- Aland Islands eye disease
- Aphonia, deafness, retinal dystrophy, bifid halluces, intellectual disability syndrome
- Ataxia with tapetoretinal degeneration syndrome
- Autosomal dominant late-onset retinal degeneration
- Autosomal recessive bestrophinopathy
- Bothnia retinal dystrophy
- Central obesity
- Choreoathetosis
- Cone dystrophy
- Cone dystrophy with supernormal rod response
- Congenital anomaly of macula
- Congenital cerebellar hypoplasia co-occurrent with tapetoretinal degeneration
- Congenital coloboma of bilateral irides
- Congenital coloboma of iris
- Congenital coloboma of iris of left eye
- Congenital coloboma of iris of right eye
- Congenital hypoplasia of external genitalia
- Congenital hypoplasia of male external genitalia
- Congenital hypoplasia of penis
- Congenital hypotrichia
- Diplegia
- Ectodermal dysplasia with ectrodactyly and macular dystrophy syndrome
- Encephalopathy, intracerebral calcification, retinal degeneration syndrome
- Familial benign flecked retina
- Familial progressive retinal dystrophy, iris coloboma, congenital cataract syndrome
- Fundus albipunctatus
- Hereditary retinal dystrophies in lipidoses
- Hereditary retinal dystrophy
- Hereditary vasopressin-related polyuria
- Hypotrichosis with juvenile macular degeneration syndrome
- Immuno-osseous dysplasia
- Infantile cerebellar and retinal degeneration
- Kandori fleck retina syndrome
- Leber's amaurosis
- Microcornea, rod-cone dystrophy, cataract, posterior staphyloma syndrome
- MORM syndrome
- Nanophthalmia
- North Carolina macular dystrophy
- Occult macular dystrophy
- Oligocone trichromacy
- Optic atrophy due to retinal dystrophy
- Paresis of left lower limb
- Paresis of right lower limb
- Progressive chorea
- Progressive retinal dystrophy due to retinol transport defect
- Retinal degeneration, nanophthalmos, glaucoma syndrome
- Retinal dystrophy
- Retinal dystrophy in cerebroretinal lipidosis
- Retinal dystrophy with inner retinal dysfunction and ganglion cell anomalies
- Retinal flecking
- Retinitis punctata albescens
- Retinohepatoendocrinologic syndrome
- Rod dystrophy
- Roifman syndrome
- Severe early childhood onset retinal dystrophy
- Severe oculo-renal-cerebellar syndrome
- Short stature, skeletal dysplasia, retinal degeneration, intellectual disability, sensorineural hearing loss syndrome
- Spastic diplegia
- Spastic paralysis
- Spastic tetraparesis
- Spondyloepiphyseal dysplasia, sensorineural hearing loss, intellectual disability, Leber congenital amaurosis syndrome
- Stargardt's disease
- Tetraparesis
- Vasopressin deficiency
- Weakness of bilateral lower limb
- Weakness of left lower limb
- Weakness of left upper limb
- Weakness of right lower limb
- Weakness of right upper limb
- X-linked intellectual disability, limb spasticity, retinal dystrophy, arginine vasopressin deficiency
Instructional NotesGuidance
Instructions from the official ICD-10-CM Tabular List that apply to H35.50: its own notes plus those printed at H35.5, H35, and Chapter 7. A note printed at a category, block or chapter applies to every code under it.
Excludes1
Not coded here: the excluded code is never reported together with this one, unless the two conditions are unrelated.
Excludes2
Not included here: the excluded condition is not part of this code, but a patient may have both, so both codes may be reported.
- certain conditions originating in the perinatal period (P04-P96)
- certain infectious and parasitic diseases (A00-B99)
- complications of pregnancy, childbirth and the puerperium (O00-O9A)
- congenital malformations, deformations, and chromosomal abnormalities (Q00-Q99)
- diabetes mellitus related eye conditions (E09.3-, E10.3-, E11.3-, E13.3-)
- endocrine, nutritional and metabolic diseases (E00-E88)
- injury (trauma) of eye and orbit (S05.-)
- injury, poisoning and certain other consequences of external causes (S00-T88)
- neoplasms (C00-D49)
- symptoms, signs and abnormal clinical and laboratory findings, not elsewhere classified (R00-R94)
- syphilis related eye disorders (A50.01, A50.3-, A51.43, A52.71)
Notes
General instructions on how these codes are used.
- Use an external cause code following the code for the eye condition, if applicable, to identify the cause of the eye condition
Source: CMS ICD-10-CM Tabular List. How to read instructional notes.
Referenced in Other NotesGuidance
Instructional notes printed at other codes that name H35.50, its category, or a range that includes it.
Index to Diseases and InjuriesGuidance
Alphabetical index entries that point to this code.
Amaurosis (acquired) (congenital) See Also: Blindness;
Leber's congenital H35.50
Best's disease H35.50
congenital amaurosis H35.50
Clinical ClassificationClinical
AHRQ’s CCSR groups this code into broader clinical categories.
Clinical InformationClinical
Vitelliform Macular Dystrophy
autosomal dominant hereditary maculopathy with childhood-onset accumulation of lipofusion in retinal pigment epithelium. affected individuals develop progressive central acuity loss, and distorted vision (metamorphopsia). it is associated with mutations in bestrophin, a chloride channel.
Patient EducationClinical
Retinal Disorders
Retinal disorders are conditions that affect the retina, the thin light-sensitive tissue at the back of your eye. The retina senses light and sends signals to your brain so you can see.
The full article covers:
- What are retinal disorders?
- What causes retinal disorders?
- What are common retinal disorders?
- What are the symptoms of retinal disorders?
- How are retinal disorders diagnosed?
- How are retinal disorders treated?
- Can retinal disorders be prevented?
Read the full article at MedlinePlus
Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.
Convert H35.50 to ICD-9-CMHistory
The closest ICD-9-CM equivalents under the General Equivalence Mappings.
Code HistoryHistory
Questions About H35.50Overview
What is the ICD-10 code for unspecified hereditary retinal dystrophy?
The ICD-10-CM code for unspecified hereditary retinal dystrophy is H35.50 (sometimes written as H3550). It is billable on HIPAA-covered claims from October 1, 2026 through September 30, 2027.
Is H35.50 (Hereditary retinal dystrophy) a billable code?
Yes. This is a billable ICD-10-CM code, specific enough to report unspecified hereditary retinal dystrophy on HIPAA-covered claims from October 1, 2026 through September 30, 2027.
What MS-DRG does H35.50 group to?
When unspecified hereditary retinal dystrophy is the principal diagnosis on an inpatient stay, it groups to MS-DRG 125, 124, with relative weights from 0.7699 to 1.2470 depending on complications. Higher weights mean higher Medicare reimbursement.
Can H35.50 be reported with a code from H31.1?
Not as a rule. The Excludes1 note at H35.5, which applies to H35.50, lists "dystrophies primarily involving Bruch's membrane (H31.1-)". An Excludes1 note means the excluded code is never reported together with this one; the only exception is when the two conditions are unrelated to each other (Official Guidelines, Section I.A.12.a).
What is the ICD-9 equivalent of H35.50?
Under the General Equivalence Mappings, unspecified hereditary retinal dystrophy converts to ICD-9-CM 362.70 (hered retin dystrphy NOS). The mapping is a direct match.
