2026 ICD-10-CM Diagnosis Code Q14.1Congenital malformation of retina

ICD-10-CM Codes›Q00-Q99›Q10-Q18›Q14

ICD-10-CM Q14.1
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

Q14.1 is a billable ICD-10-CM diagnosis code for congenital malformation of retina. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026) and groups to MS-DRG 124 through 125. The code is exempt from POA reporting. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Congenital malformations of eye, ear, face, neck.

Code Identity

ICD-10-CM Code
Q14.1
Billable Status
Yes — Valid for Submission
Code Describes
Congenital malformation of retina
Short Description
Congenital malformation of retina
Same as the full description in the CMS dataset.
Parent Code
Q14
Congenital malformations of posterior segment of eye

Code Classification

ChapterQ00-Q99Congenital malformations, deformations and chromosomal abnormalities
SectionQ10-Q18Congenital malformations of eye, ear, face and neck
CategoryQ14Congenital malformations of posterior segment of eye
This CodeQ14.1Congenital malformation of retina

Present on Admission (POA)Billing

Q14.1 is exempt from POA reporting on inpatient claims to general acute care hospitals. Review other POA exempt codes.

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Albinotic fundus
  • Angiomatosis of retina
  • Arthrogryposis with oculomotor limitation and electroretinal anomaly
  • Bilateral choroid degeneration
  • Bilateral coloboma of macula
  • Bilateral congenital anomaly of retinas
  • Bilateral congenital hypertrophy of retinal pigment epithelium
  • Brachydactyly syndrome type B
  • Chorioretinal atrophy
  • Coloboma of choroid
  • Coloboma of choroid and retina
  • Coloboma of macula with brachydactyly type B syndrome
  • Coloboma of retina
  • Congenital anomaly of left retina
  • Congenital anomaly of macula
  • Congenital anomaly of retina
  • Congenital anomaly of right retina
  • Congenital chorioretinal degeneration
  • Congenital chorioretinal degeneration of bilateral eyes
  • Congenital chorioretinal degeneration of left eye
  • Congenital chorioretinal degeneration of right eye
  • Congenital coloboma of macula lutea
  • Congenital hallux valgus
  • Congenital hypertrophy of retinal pigment epithelium
  • Congenital hypertrophy of retinal pigment epithelium of left eye
  • Congenital hypertrophy of retinal pigment epithelium of right eye
  • Congenital hypoplasia of fovea centralis
  • Congenital hypoplasia of macula lutea
  • Congenital hypoplasia of retina
  • Congenital nystagmus
  • Congenital peripapillary staphyloma
  • Congenital retinal aneurysm
  • Congenital retinal dysplasia caused by teratogenic substance
  • Congenital retinal fold
  • Congenital retinoschisis
  • Congenital stenosis of pulmonary artery
  • Congenital supravalvular pulmonary stenosis
  • Congenital vascular anomaly of eye
  • Diffuse retinal dysplasia
  • Extensive peripapillary myelinated nerve fibers of retina
  • Foveal hypoplasia with presenile cataract syndrome
  • Foveal hypoplasia, optic nerve decussation defect, anterior segment dysgenesis syndrome
  • Geographic retinal dysplasia
  • Isolated foveal hypoplasia
  • Juvenile retinoschisis
  • Macular and peripheral retinoschisis
  • Macular coloboma, cleft palate, hallux valgus syndrome
  • Macular retinoschisis
  • Microcornea
  • Microcornea with corectopia and macular hypoplasia syndrome
  • Multifocal retinal dysplasia
  • Myelinated nerve fiber layer of retina
  • Nephronophthisis
  • Oliver McFarlane syndrome
  • Peripheral retinoschisis
  • Presenile cataract
  • Pulmonary trunk stenosis
  • Renal dysplasia and retinal aplasia
  • Retinal arterial macroaneurysm with supravalvular pulmonic stenosis
  • Retinal arteriovenous malformation
  • Retinal arteriovenous shunt
  • Retinal dysplasia
  • Retinal hemangioblastomatosis
  • Retinal macroaneurysm
  • Retinal pigment deposits
  • Retinal pigment epithelial hypertrophy
  • Retinal pigmentation grouped
  • Solitary congenital hypertrophy of retinal pigment epithelium
  • Supravalvar pulmonary trunk stenosis
  • Vitreoretinal dysplasia
  • X-linked retinal dysplasia

Tabular List NotesGuidance

Coding notes and annotation back-references applicable to this code.

Inclusion Terms

  • Congenital retinal aneurysm

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.

    • Aberrant(congenital)
      • artery (peripheral)
        • retina
    • Aneurysm(anastomotic) (artery) (cirsoid) (diffuse) (false) (fusiform) (multiple) (saccular)
      • congenital (peripheral)
        • retina
    • Aneurysm(anastomotic) (artery) (cirsoid) (diffuse) (false) (fusiform) (multiple) (saccular)
      • retina
        • congenital
    • Anomaly, anomalous(congenital) (unspecified type)
      • artery (peripheral)
        • retina
    • Anomaly, anomalous(congenital) (unspecified type)
      • fovea centralis
    • Anomaly, anomalous(congenital) (unspecified type)
      • retina
    • Aplasia
      • fovea centralis (congenital)
    • Displacement, displaced
      • macula (congenital)
    • Dysplasia
      • retinal, congenital
    • Hypoplasia, hypoplastic
      • artery (peripheral)
        • retinal (congenital)
    • Imperfect
      • closure (congenital)
        • retina
    • Malformation(congenital)
      • retina
    • Medullated fibers
      • retina
    • Pigmentation(abnormal) (anomaly)
      • retina, congenital (grouped) (nevoid)
    • Retinoschisis
      • congenital
    • Stricture
      • artery
        • congenital (peripheral)
          • retinal
    • Tortuous
      • retinal vessel, congenital

Clinical ClassificationClinical

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

CCSR MAL005
Congenital malformations of eye, ear, face, neck
Default principal diagnosis: inpatient Yes · outpatient Yes

Clinical InformationClinical

  • Retinal Dysplasia

    congenital, often bilateral, retinal abnormality characterized by the arrangement of outer nuclear retinal cells in a palisading or radiating pattern surrounding a central ocular space. this disorder is sometimes hereditary.

  • Retinal Hemangioblastomatosis

    the presence of multiple hemangioblastomas in the retina. it is associated with von hippel lindau syndrome.

  • Congenital Hallux Valgus

    hallux valgus that is present at birth.

  • Nephrocystin-1|Juvenile Nephronophthisis 1 Protein|NPHP1

    nephrocystin-1 (732 aa, ~83 kda) is encoded by the human nphp1 gene. this protein is involved in the modulation of signaling.

  • Nephronophthisis

    progressive tubulointerstitial injury, inherited in an autosomal recessive pattern, caused by mutations in genes involved in ciliary function, which may result in an end stage renal failure.

  • Nephronophthisis 1|Familial Juvenile Nephronophthisis|Juvenile Nephronophthisis|NPH1

    progressive tubulointerstitial nephritis inherited in an autosomal recessive manner. it is caused by mutations in the nphp1 gene. patients present with anemia, polyuria, and polydipsia during childhood. the progressive bilateral kidney damage results in renal failure.

  • NPHP1 Gene|NPHP1|NPHP1|Nephronophthisis 1 (Juvenile) Gene

    this gene is involved in the mediation of signal transduction.

  • NPHP1 wt Allele|FLJ97602|JBTS4|NPH1|Nephronophthisis 1 (Juvenile) wt Allele|SLSN1

    human nphp1 wild-type allele is located in the vicinity of 2q13 and is approximately 83 kb in length. this allele, which encodes nephrocystin-1 protein, plays a role in the progression of adhesion-dependent signaling pathways. mutations in the gene are associated with familial juvenile nephronophthisis type 1, senior-loken syndrome type 1, and joubert syndrome type 4.

  • Microcornea

    a congenital abnormality characterized by an abnormally small cornea. the horizontal corneal diameter is less than 10mm or less than 9mm in newborns. it is associated with an increased risk of glaucoma.

Patient EducationClinical

Birth Defects

A birth defect is a problem that happens while a baby is developing in the mother's body. Most birth defects happen during the first 3 months of pregnancy. One out of every 33 babies in the United States is born with a birth defect.

The full article covers:

  • What are birth defects?
  • What causes birth defects?
  • Who is at risk of having a baby with birth defects?
  • How are birth defects diagnosed?
  • What are the treatments for birth defects?
  • Can birth defects be prevented?

Read the full article at MedlinePlus

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

Convert Q14.1 to ICD-9-CMHistory

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

ICD-9-CM
743.56 Cong retinal changes NEC
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 Q14.1Overview

Is Q14.1 (Congenital malformations of posterior segment of eye) a billable code?

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

What MS-DRG does Q14.1 group to?

When congenital malformation of retina is the principal diagnosis on an inpatient stay, it groups to MS-DRG 124, 125, with relative weights from 0.7678 to 1.3231 depending on complications. Higher weights mean higher Medicare reimbursement.

Is Q14.1 exempt from POA reporting?

Yes. CMS lists this code among those exempt from present on admission reporting, so hospitals do not assign a POA indicator for congenital malformation of retina on inpatient claims.

What is the ICD-9 equivalent of Q14.1?

Under the General Equivalence Mappings, congenital malformation of retina converts to ICD-9-CM 743.56 (cong retinal changes NEC). 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.