2024 ICD-10-CM Diagnosis Code H35.52

Approximate Synonyms

The following clinical terms are approximate synonyms or lay terms that might be used to identify the correct diagnosis code:

• Autosomal dominant retinitis pigmentosa
• Autosomal dominant retinitis pigmentosa
• Autosomal recessive leukoencephalopathy, ischemic stroke, retinitis pigmentosa syndrome
• Autosomal recessive posterior column ataxia and retinitis pigmentosa
• Autosomal recessive retinitis pigmentosa
• Brachydactyly, short stature, retinitis pigmentosa syndrome
• Butterfly-shaped pigmentary macular dystrophy
• Cholestasis with pigmentary retinopathy and cleft palate syndrome
• Disorder due to abnormality of dermal elastin
• Drusen of optic disc
• Early onset cerebellar ataxia with retinitis pigmentosa and optic atrophy
• Hereditary cerebellar atrophy
• Hereditary motor and sensory neuropathy with retinitis pigmentosa
• Hypogonadotropic hypogonadism retinitis pigmentosa syndrome
• Infantile and/or juvenile cataract
• Juvenile cataract
• Macular pigment deposit
• Microphthalmia, retinitis pigmentosa, foveoschisis, optic disc drusen syndrome
• Mitochondrial myopathy, cerebellar ataxia, pigmentary retinopathy syndrome
• Muscular atrophy, ataxia, retinitis pigmentosa, and diabetes mellitus
• Neurogenic muscle weakness, ataxia and retinitis pigmentosa
• Oculotrichodysplasia
• Osteochondrodysplatic nanism, deafness, retinitis pigmentosa syndrome
• Pattern dystrophy of macula
• Polyneuropathy, hearing loss, ataxia, retinitis pigmentosa, cataract syndrome
• Primary ciliary dyskinesia and retinitis pigmentosa syndrome
• Pseudoxanthoma elasticum-like skin manifestations with retinitis pigmentosa
• Retinal dystrophy due to systemic disorder
• Retinal pigment deposits
• Retinal pigment deposits
• Retinal pigment epithelial dystrophy
• Retinal pigment epithelial dystrophy
• Retinitis pigmentosa
• Retinitis pigmentosa due to systemic disease
• Retinitis pigmentosa of bilateral eyes
• Retinitis pigmentosa of left eye
• Retinitis pigmentosa of right eye
• Retinitis pigmentosa, hearing loss, premature aging, short stature, facial dysmorphism syndrome
• Retinitis pigmentosa, intellectual disability, deafness, hypogenitalism syndrome
• Retinitis pigmentosa, juvenile cataract, short stature, intellectual disability syndrome
• Retinitis pigmentosa-deafness syndrome
• Retinitis pigmentosa-deafness syndrome type 3
• RHYNS syndrome
• Saldino-Mainzer dysplasia
• Spastic tetraplegia
• Spastic tetraplegia, retinitis pigmentosa, intellectual disability syndrome
• Tapetoretinal dystrophy
• Uncombable hair, retinal pigmentary dystrophy, dental anomaly and brachydactyly syndrome
• X-linked retinitis pigmentosa
• X-linked retinitis pigmentosa
• X-linked retinitis pigmentosa heterozygote

Clinical Information

• Retinitis Pigmentosa

  hereditary, progressive degeneration of the retina due to death of rod photoreceptors initially and subsequent death of cone photoreceptors. it is characterized by deposition of pigment in the retina.

• Usher Syndromes

  autosomal recessive hereditary disorders characterized by congenital sensorineural hearing loss and retinitis pigmentosa. genetically and symptomatically heterogeneous, clinical classes include type i, type ii, and type iii. their severity, age of onset of retinitis pigmentosa and the degree of vestibular dysfunction are variable.

• Ceramide Kinase-like Protein|Retinitis Pigmentosa 26 (Autosomal Recessive)

  ceramide kinase-like protein (558 aa, ~63 kda) is encoded by the human cerkl gene. this protein plays a role in protecting cells from apoptosis in oxidative stress conditions.

• EYS wt Allele|C6orf178|C6orf179|C6orf180|Chromosome 6 Open Reading Frame 178 Gene|Chromosome 6 Open Reading Frame 179 Gene|Chromosome 6 Open Reading Frame 180 Gene|EGF-Like-Domain, Multiple 10 Gene|EGF-Like-Domain, Multiple 11 Gene|EGFL10|EGFL11|Eyes Shut Homolog (Drosophila) Gene|Eyes Shut Homolog wt Allele|Eyes Shut, Drosophila, Homolog of Gene|RP25|Retinitis Pigmentosa 25 (Autosomal Recessive) Gene|SPAM|Spacemaker Gene|UNQ9424/PRO34591|bA166P24.2|bA307F22.3|bA74E24.1|dJ1018A4.2|dJ22I17.2|dJ303F19.1

  human eys wild-type allele is located in the vicinity of 6q12 and is approximately 1987 kb in length. this allele, which encodes protein eyes shut homolog, is involved in retinal function. mutation of the gene is associated with autosomal recessive retinitis pigmentosa 25.

• OFD1 wt Allele|71-7A|CXorf5|Chromosome X Open Reading Frame 5 Gene|JBTS10|MGC117039|MGC117040|Oral-Facial-Digital Syndrome 1 wt Allele|Retinitis Pigmentosa 23 (X-Linked Recessive) Gene|SGBS2

  human ofd1 wild-type allele is located in the vicinity of xp22 and is approximately 35 kb in length. this allele, which encodes oral-facial-digital syndrome 1, may be involved in the mediation of embryonic development. mutations in the gene are associated with both oral-facial-digital syndrome type i and simpson-golabi-behmel syndrome type 2.

• POLR1D wt Allele|AC19|MGC9850|POLR1C|Polymerase (RNA) I Polypeptide D, 16kDa Gene|Polymerase (RNA) I Subunit D Gene|Polymerase I, RNA, Subunit D Gene|RNA Polymerase A, 16-kD, Mouse, Homolog of Gene|RNA Polymerase I and III Subunit D wt Allele|RPA16|RPA9|RPAC2|RPC16|RPO1-3|Retinitis Pigmentosa 25 (Autosomal Recessive) Gene|TCS2

  human polr1d wild-type allele is located in the vicinity of 13q12.2 and is approximately 123 kb in length. this allele, which encodes dna-directed rna polymerases i and iii subunit rpac2 protein, is involved in rna polymerase i- and iii-dependent transcription of rrna and other small rnas. mutation of the gene is associated with treacher collins syndrome 2.

• Retinitis Pigmentosa

  a rare inherited retinal dystrophy disorder characterized by spots of black bone-spicule pigmentation of the retinal pigment epithelium. it is manifested with decreased vision in low light or in the night, followed by decreased peripheral vision, and, eventual decreased central vision. it may lead to blindness.

• RHO wt Allele|CSNBAD1|OPN2|Opsin 2, Rod Pigment Gene|RP4|Retinitis Pigmentosa 4, Autosomal Dominant Gene|Rhodopsin wt Allele

  human rho wild-type allele is located in the vicinity of 3q22.1 and is approximately 7 kb in length. this allele, which encodes rhodopsin protein, is involved in photoreceptor cell activity and maintenance. mutation of the gene is associated with congenital stationary night blindness, retinitis pigmentosa 4, and retinitis punctata albescens.

• RP2 Gene|RP2|RP2|Retinitis Pigmentosa 2 (X-Linked Recessive) Gene

  this gene plays a role in development.

• RP2 wt Allele|DELXp11.3|NME10|Retinitis Pigmentosa 2 (X-Linked Recessive) wt Allele|TBCCD2|XRP2

  human rp2 wild-type allele is located within xp11.4-xp11.21 and is approximately 45 kb in length. this allele, which encodes protein xrp2, plays a role in photoreceptor development. mutations in this gene are associated with x-linked mental retardation with retinitis pigmentosa.

H35.52 is grouped with Diagnostic Related Groups - MS-DRG Mapping

Diagnostic Related Groups (DRGs) are a classification system used to group together diagnosis codes for the purpose of reimbursement and healthcare management. DRGs are used to standardize the way hospitals and other providers are paid for inpatient services. In this system patients are grouped together based on their principal diagnosis in areas referred to as Major Diagnostic Categories (MDC). Once a patient is assigned a particular diagnostic category, providers receive a predetermined payment rate for the services rendered. This reimbursement rate is often fixed and is meant to cover the cost of care for that patient. Reimbursement is also affected by the relative weight of a diagnostic related group based on the severity of a patient's illness and the associated cost of care during hospitalization.

Diagnostic related groups encourage healthcare providers to focus on quality and efficiency in patient care while managing costs effectively. The diagnosis code is present in the following groups for version MS-DRG V41.0 applicable from 10/01/2023 through 09/30/2024.

MS-DRG	MS-DRG Title	MCD	Relative Weight
124	OTHER DISORDERS OF THE EYE WITH MCC OR THROMBOLYTIC AGENT	02	1.3219
125	OTHER DISORDERS OF THE EYE WITHOUT MCC	02	0.7975

The relative weight of a diagnostic related group determines the reimbursement rate based on the severity of a patient's illness and the associated cost of care during hospitalization.

Convert H35.52 to ICD-9-CM

• ICD-9-CM Code: 362.74 - Pigment retina dystrophy

Patient Education

Retinal Disorders

The retina is a layer of tissue in the back of your eye that senses light and sends images to your brain. In the center of this nerve tissue is the macula. It provides the sharp, central vision needed for reading, driving and seeing fine detail.

Retinal disorders affect this vital tissue. They can affect your vision, and some can be serious enough to cause blindness. Examples are:

• Macular degeneration - a disease that destroys your sharp, central vision
• Diabetic eye disease
• Retinal detachment - a medical emergency, when the retina is pulled away from the back of the eye
• Retinoblastoma - cancer of the retina. It is most common in young children.
• Macular pucker - scar tissue on the macula
• Macular hole - a small break in the macula that usually happens to people over 60
• Floaters - cobwebs or specks in your field of vision

NIH: National Eye Institute

[Learn More in MedlinePlus]

Cone-rod dystrophy

Cone-rod dystrophy is a group of related eye disorders that causes vision loss, which becomes more severe over time. These disorders affect the retina, which is the layer of light-sensitive tissue at the back of the eye. In people with cone-rod dystrophy, vision loss occurs as the light-sensing cells of the retina gradually deteriorate.

The first signs and symptoms of cone-rod dystrophy, which often occur in childhood, are usually decreased sharpness of vision (visual acuity) and increased sensitivity to light (photophobia). These features are typically followed by impaired color vision (dyschromatopsia), blind spots (scotomas) in the center of the visual field, and partial side (peripheral) vision loss. Over time, affected individuals develop night blindness and a worsening of their peripheral vision, which can limit independent mobility. Decreasing visual acuity makes reading increasingly difficult and most affected individuals are legally blind by mid-adulthood. As the condition progresses, individuals may develop involuntary eye movements (nystagmus).

There are more than 30 types of cone-rod dystrophy, which are distinguished by their genetic cause and their pattern of inheritance: autosomal recessive, autosomal dominant, and X-linked. Additionally, cone-rod dystrophy can occur alone without any other signs and symptoms or it can occur as part of a syndrome that affects multiple parts of the body.

[Learn More in MedlinePlus]

Fundus albipunctatus

Fundus albipunctatus is an eye disorder characterized by an impaired ability to see in low light (night blindness) and the presence of whitish-yellow flecks in the retina, which is the specialized light-sensitive tissue in the inner lining of the back of the eye (the fundus). The flecks are detected during an eye examination.

Individuals with fundus albipunctatus experience night blindness from an early age. In particular, they have delayed dark adaptation, which means they have trouble adapting from bright light to dark conditions, such as when driving into a dark tunnel on a sunny day. It often takes hours for adaptation to occur. Their vision in bright light is usually normal.

The flecks are especially abundant near the outer edge (the periphery) of the retina. Their density varies among affected individuals; some people have numerous flecks that overlap, while others have fewer. For unknown reasons, the flecks get smaller or fade with age in some affected individuals, although night vision does not improve.

While fundus albipunctatus typically does not worsen (progress) over time, some individuals with the condition develop other eye conditions, such as breakdown of the central region of the retina known as the macula (macular degeneration) with loss of specialized light receptor cells called cones, which can affect vision in bright light.

[Learn More in MedlinePlus]

Retinitis pigmentosa

Retinitis pigmentosa is a group of related eye disorders that cause progressive vision loss. These disorders affect the retina, which is the layer of light-sensitive tissue at the back of the eye. In people with retinitis pigmentosa, vision loss occurs as the light-sensing cells of the retina gradually deteriorate.

The first sign of retinitis pigmentosa is usually a loss of night vision, which becomes apparent in childhood. Problems with night vision can make it difficult to navigate in low light. Later, the disease causes blind spots to develop in the side (peripheral) vision. Over time, these blind spots merge to produce tunnel vision. The disease progresses over years or decades to affect central vision, which is needed for detailed tasks such as reading, driving, and recognizing faces. In adulthood, many people with retinitis pigmentosa become legally blind.

The signs and symptoms of retinitis pigmentosa are most often limited to vision loss. When the disorder occurs by itself, it is described as nonsyndromic. Researchers have identified several major types of nonsyndromic retinitis pigmentosa, which are usually distinguished by their pattern of inheritance: autosomal dominant, autosomal recessive, or X-linked.

Less commonly, retinitis pigmentosa occurs as part of syndromes that affect other organs and tissues in the body. These forms of the disease are described as syndromic. The most common form of syndromic retinitis pigmentosa is Usher syndrome, which is characterized by the combination of vision loss and hearing loss beginning early in life. Retinitis pigmentosa is also a feature of several other genetic syndromes, including Bardet-Biedl syndrome; Refsum disease; and neuropathy, ataxia, and retinitis pigmentosa (NARP).

[Learn More in MedlinePlus]

Code History

• FY 2024 - No Change, effective from 10/1/2023 through 9/30/2024
• FY 2023 - No Change, effective from 10/1/2022 through 9/30/2023
• FY 2022 - No Change, effective from 10/1/2021 through 9/30/2022
• FY 2021 - No Change, effective from 10/1/2020 through 9/30/2021
• FY 2020 - No Change, effective from 10/1/2019 through 9/30/2020
• FY 2019 - No Change, effective from 10/1/2018 through 9/30/2019
• FY 2018 - No Change, effective from 10/1/2017 through 9/30/2018
• FY 2017 - No Change, effective from 10/1/2016 through 9/30/2017
• FY 2016 - New Code, effective from 10/1/2015 through 9/30/2016. This was the first year ICD-10-CM was implemented into the HIPAA code set.