2025 ICD-10-CM code Q13.81
(Active) Effective Date: N/A Revision Date: N/A Congenital malformations - Congenital malformations of eye, ear, face and neck Congenital malformations, deformations and chromosomal abnormalities (Q00-Q99) Feed
Rieger's anomaly, a congenital ocular defect characterized by severe anterior chamber deformity with iris stromal atrophy, hole or pseudo-hole formation, and corectopia.
Modifiers may be applicable depending on the specific circumstances of the encounter and services provided. Refer to the current CPT and ICD-10-CM coding guidelines.
Medical necessity for coding Q13.81 is established by the presence of the characteristic clinical findings of Rieger's anomaly, documented through a comprehensive ophthalmologic examination.Further investigations like genetic testing might be medically necessary to determine if systemic involvement is present and to differentiate it from Axenfeld-Rieger syndrome.
Ophthalmologist, geneticist (if systemic involvement suspected)
- Congenital malformations, deformations and chromosomal abnormalities (Q00-Q99)
- Congenital malformations of eye, ear, face and neck (Q10-Q18)
In simple words: Rieger's anomaly is a birth defect affecting the front part of the eye.It causes changes in the iris (the colored part of the eye), making it thin and possibly causing holes or an off-center pupil. This can lead to glaucoma, a condition that increases pressure inside the eye.
Rieger's anomaly is a congenital ocular defect resulting from anterior segment dysgenesis.It's characterized by a severely deformed anterior chamber of the eye, prominent iris strands, and significant atrophy of the iris stroma.This atrophy leads to the formation of holes or pseudo-holes in the iris and corectopia (off-center pupil).It often co-occurs with Axenfeld's anomaly. Glaucoma is a frequent complication.
Example 1: A newborn infant is diagnosed with Rieger's anomaly during a routine eye examination. The anomaly is unilateral, and the child exhibits mild corectopia in the affected eye. No associated systemic findings are noted., A 5-year-old child presents with Rieger's anomaly, characterized by significant iris atrophy and corectopia in both eyes. The child is diagnosed with glaucoma and undergoes angle bypass surgery., A teenager is diagnosed with Rieger's anomaly, showing marked iris stromal atrophy, multiple pseudo-holes in the iris, and glaucoma in the right eye.Genetic testing reveals a mutation consistent with Axenfeld-Rieger syndrome. Further evaluation reveals associated dental anomalies and redundant periumbilical skin.
Detailed ophthalmologic examination findings including slit-lamp biomicroscopy for assessment of the anterior chamber, iris, pupil, and cornea.Documentation of associated glaucoma and its severity.Genetic testing results and systemic examination findings (if applicable).
** Rieger's anomaly is often part of a broader spectrum of disorders known as Axenfeld-Rieger syndrome.Careful clinical evaluation is essential to determine the complete diagnosis and code appropriately.Consider genetic counseling and testing for family members.
- Payment Status: Active
- Specialties:Ophthalmology, Genetics
- Place of Service:Office, Hospital (Inpatient or Outpatient), Ambulatory Surgical Center