Acta Scientific Ophthalmology (ISSN: 2582-3191)

Review Article Volume 4 Issue 10

Axenfeld Rieger Syndrome-A Rare Syndrome Revisited

Deepti Parmar1, Vinod Sharma2 and Kalpana Sharma3*

1Senior Resident, Department of Ophthalmology, Indira Gandhi Medical College, Shimla, India
2Associate Professor, Department of Ophthalmology, Indira Gandhi Medical College, Shimla, India
3Assistant Professor, Department of Ophthalmology, Indira Gandhi Medical College, Shimla, India

*Corresponding Author: Kalpana Sharma, Assistant Professor, Department of Ophthalmology, Indira Gandhi Medical College, Shimla, India.

Received: August 24, 2021; Published: September 06, 2021

Abstract

  Axenfeld Rieger Syndrome is an anterior segment dysgenesis accompanied by systemic abnormalities. We report a case of 17 years old male with iris coloboma in right eye and anteriorly displaced Schwalbe’s line in both eyes. Intraocular pressure was raised in both eyes. Patient was diagnosed case of ventricular septal defect (VSD) with patent ductus arteriosus (PDA) for which he was operated six years back. On physical examination he had scoliosis and on Oro dental examination maxillary hypoplasia was seen. Patient was diagnosed as Axenfeld Rieger Syndrome with glaucoma and was treated with antiglaucoma drugs. This article aims to discuss the clinical features and management of this rare congenital anomaly.

Keywords: Axenfeld Rieger Syndrome; Patent Ductus Arteriosus (PDA); Ventricular Septal Defect (VSD)

References

  1. Axenfeld TH. “Embryotoxon cornea posterius”. Klinische Monatsblatter fur Augenheilkunde 65 (1920): 381-382.
  2. Waring GO., et al. “Anterior chamber cleavage syndrome”. Survey of Ophthalmology 20 (1975): 3-27.
  3. Alward LWM. “Axenfeld–Rieger syndrome in the age of molecular genetics”. American Journal of Ophthalmology 130 (2000): 107-115.
  4. Azuma N and Yamada M. “Missense mutation at the C terminus of the PAX6 gene in ocular anterior segment anomalies”. Investigative Ophthalmology and Visual Science 39 (1998): 828-830.
  5. Johnston MC., et al. “Origins of avian ocular and periocular tissue”. Experimental Eye Research 29 (1979): 27-43.
  6. Shields MB. “Axenfeld–Rieger syndrome: a theory of mechanism and distinctions from the iridocorneal endothelial syndrome”. Transactions of the American Ophthalmological Society 81 (1983): 736-784.
  7. Gould DB and John SW. “Anterior segment dysgenesis and the developmental glaucomas are complex traits”. Human Molecular Genetics 10 (2002): 1185-1193.
  8. Mandal Prasad Naduvilath. “Surgical Results and Complications of Mitomycin C-Augmented Trabeculectomy in Refractory Developmental Glaucoma”. Ophthalmic Surgery, Lasers, and Imaging 6 (1999): 473-480.

Citation

Citation: Kalpana Sharma., et al. “Axenfeld Rieger Syndrome-A Rare Syndrome Revisited”. Acta Scientific Ophthalmology 4.10 (2021): 03-06.

Copyright

Copyright: © 2021 Kalpana Sharma., et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.




Metrics

Acceptance rate35%
Acceptance to publication20-30 days
ISI- IF1.042
JCR- IF0.24

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