eMedicine Specialties > Ophthalmology > Cornea

Congenital Clouding of the Cornea: Follow-up

Author: Noah S Scheinfeld, MD, JD, FAAD, Assistant Clinical Professor, Department of Dermatology, Columbia University; Consulting Staff, Department of Dermatology, St Luke's Roosevelt Hospital Center, Beth Israel Medical Center, New York Eye and Ear Infirmary; Private Practice
Coauthor(s): Benjamin D Freilich, MD, FACS, Assistant Clinical Professor, Department of Ophthalmology, Mount Sinai School of Medicine; Director of Retina Service, Bronx Veterans Administration Medical Center; Jonathan Freilich, MD, FACS, Clinical Instructor, Department of Ophthalmology, Mount Sinai School of Medicine; Consulting Staff, Department of Ophthalmology, Mount Sinai Hospital, St Luke's Roosevelt Hospital Center
Contributor Information and Disclosures

Updated: Jun 10, 2008

Follow-up

Further Outpatient Care

  • An ophthalmologist should provide regular follow-up care for patients with graft rejection.
  • In addition, a pediatrician should monitor patients for other congenital anomalies.
  • Patients should receive visual rehabilitation as needed.
  • A pediatric contact lens specialist should fit patients with aphakic contact lenses.

Inpatient & Outpatient Medications

  • Medications may be indicated for treatment after corneal transplantation.

Complications

  • Complications of corneal transplantation are varied.

Prognosis

  • The visual prognosis is guarded.
  • The earlier keratoplasty is performed (generally prior to 3-6 months of age), the better the likelihood of preventing deprivation amblyopia.
    • In most series, visual acuity in patients after keratoplasty was 20/80 or worse. Some investigators reported visual acuity of 20/40 in patients.
    • Also, in most series, the likelihood that patients maintain a clear graft was 30-50% at 10 years.
  • Patients with glaucoma and cataract had a worsened prognosis.
  • The prognosis for life depends on other systemic anomalies.

Patient Education

  • Children with Peters anomaly and other genetic syndromes associated with corneal opacities require special educational assistance depending on their visual outcome. A low-vision specialist should evaluate these children.
  • Patients may need loupes and binoculars depending on their visual potential.

Miscellaneous

Medicolegal Pitfalls

  • Failure to perform an evaluation under anesthesia in a timely fashion if an adequate examination cannot be performed in the office
  • Failure to differentiate Peters anomaly from other conditions that mimic it
  • Failure to refer the patient to other specialists to rule out systemic anomalies
  • Failure to provide regular follow-up care for children with graft rejection or glaucoma
  • Failure to offer visual rehabilitation

Special Concerns

  • Cases of congenital corneal clouding and Peters anomaly have been reported to occur with fetal alcohol syndrome.
  • Drinking during pregnancy should be discouraged.
 


More on Congenital Clouding of the Cornea

Overview: Congenital Clouding of the Cornea
Differential Diagnoses & Workup: Congenital Clouding of the Cornea
Treatment & Medication: Congenital Clouding of the Cornea
Follow-up: Congenital Clouding of the Cornea
Multimedia: Congenital Clouding of the Cornea
References

References

  1. Bhat YR, Sanoj KM. Images in Clinical Practices. Sclerocornea. Available at: http://www.indianpediatrics.net/mar2005/277.pdf. Indian Pediatrics. Mar 17 2005;42:42. [Full Text].

  2. EyeMDLink. Congenital hereditary endothelial dystrophy. Available at: http://www.eyemdlink.com/Condition.asp?ConditionID=485. September 16,. 2001;[Full Text].

  3. Hand CK, Harmon DL, Kennedy SM, FitzSimon JS, Collum LM, Parfrey NA. Localization of the gene for autosomal recessive congenital hereditary endothelial dystrophy (CHED2) to chromosome 20 by homozygosity mapping. Genomics. Oct 1 1999;61(1):1-4. [Medline].

  4. Miller MM, Butrus S, Hidayat A, Wei LL, Pontigo M. Corneoscleral transplantation in congenital corneal staphyloma and Peters' anomaly. Ophthalmic Genet. Mar 2003;24(1):59-63. [Medline].

  5. Waizenegger UR, Kohnen T, Weidle EG, Schütte E. [Congenital familial cornea plana with ptosis, peripheral sclerocornea and conjunctival xerosis]. Klin Monatsbl Augenheilkd. Aug 1995;207(2):111-6. [Medline].

  6. Perry HD, Cameron JD. Congenital corneal opacities. Available at: http://www.ophthalmic.hyperguides.com/default.asp?section=body.asp. [Full Text].

  7. Kao WW, Liu CY. Roles of lumican and keratocan on corneal transparency. Glycoconj J. May-Jun 2002;19(4-5):275-85. [Medline].

  8. Rezende RA, Uchoa UB, Uchoa R, Rapuano CJ, Laibson PR, Cohen EJ. Congenital corneal opacities in a cornea referral practice. Cornea. Aug 2004;23(6):565-70. [Medline].

  9. Bermejo E, Martínez-Frías ML. Congenital eye malformations: clinical-epidemiological analysis of 1,124,654 consecutive births in Spain. Am J Med Genet. Feb 17 1998;75(5):497-504. [Medline].

  10. el-Gilany AH, el-Fedawy S, Tharwat M. Causes of blindness and needs of the blind in Mansoura, Egypt. East Mediterr Health J. Jan 2002;8(1):6-17. [Medline].

  11. Hwang JM, Chung DC, Traboulsi EI. A new syndrome of hereditary congenital corneal opacities, cornea guttata, and corectopia. Arch Ophthalmol. Jul 2003;121(7):1053-4. [Medline].

  12. Cibis GW. Congenital glaucoma. J Am Optom Assoc. Sep 1987;58(9):728-33. [Medline].

  13. Edward DP, Li J, Sawaguchi S, Sugar J, Yue BY, Tso MO. Diffuse corneal clouding in siblings with fetal alcohol syndrome. Am J Ophthalmol. Apr 15 1993;115(4):484-93. [Medline].

  14. Aldave AJ, Eagle RC Jr, Streeten BW, Qi J, Raber IM. Congenital corneal opacification in De Barsy syndrome. Arch Ophthalmol. Feb 2001;119(2):285-8. [Medline].

  15. O'Neill JF. The ocular manifestations of congenital infection: a study of the early effect and long-term outcome of maternally transmitted rubella and toxoplasmosis. Trans Am Ophthalmol Soc. 1998;96:813-79. [Medline].

  16. Lin SC, Hu FR, Hou JW, Yao YT, Wang TR, Hung PT. Corneal opacity and congenital glaucoma associated with massive heparan sulfaturia: report of one case. Acta Paediatr Taiwan. Jan-Feb 1999;40(1):46-9. [Medline].

  17. Kim T, Cohen EJ, Schnall BM, Affel EL, Eagle RC Jr. Ultrasound biomicroscopy and histopathology of sclerocornea. Cornea. Jul 1998;17(4):443-5. [Medline].

  18. Reidy JJ. Penetrating keratoplasty in infancy and early childhood. Curr Opin Ophthalmol. Aug 2001;12(4):258-61. [Medline].

  19. Michaeli A, Markovich A, Rootman DS. Corneal transplants for the treatment of congenital corneal opacities. J Pediatr Ophthalmol Strabismus. Jan-Feb 2005;42(1):34-44. [Medline].

  20. Al-Torbak AA. Outcome of combined Ahmed glaucoma valve implant and penetrating keratoplasty in refractory congenital glaucoma with corneal opacity. Cornea. Aug 2004;23(6):554-9. [Medline].

  21. Miller MM, Butrus S, Hidayat A, Wei LL, Pontigo M. Corneoscleral transplantation in congenital corneal staphyloma and Peters' anomaly. Ophthalmic Genet. Mar 2003;24(1):59-63. [Medline].

  22. Mandal AK, Gothwal VK, Bagga H, Nutheti R, Mansoori T. Outcome of surgery on infants younger than 1 month with congenital glaucoma. Ophthalmology. Oct 2003;110(10):1909-15. [Medline].

  23. Frueh BE, Brown SI. Transplantation of congenitally opaque corneas. Br J Ophthalmol. Dec 1997;81(12):1064-9. [Medline].

  24. Bredrup C, Knappskog PM, Majewski J, Rodahl E, Boman H. Congenital stromal dystrophy of the cornea caused by a mutation in the decorin gene. Invest Ophthalmol Vis Sci. Feb 2005;46(2):420-6. [Medline].

  25. Cassidy L. Paediatric cataract. Available at: http://www.optometry.co.uk/files/e8cf2c379b358b7d42e25bdfa54a9abf_cassidy20010309.pdf. March 9, 2004. [Full Text].

  26. Cibis GW, Waeltermann J, Harris DJ. Peters' anomaly in association with ring 21 chromosomal abnormality. Am J Ophthalmol. Nov 15 1985;100(5):733-4. [Medline].

  27. Duke-Elder S. Congenital deformities. In: System of Ophthalmology. Vol III. St Louis: Mosby; 1965.

  28. Hansen L, Eiberg H, Rosenberg T. Novel MAF mutation in a family with congenital cataract-microcornea syndrome. Mol Vis. Oct 18 2007;13:2019-22. [Medline].

  29. Hansen L, Yao W, Eiberg H, Kjaer KW, Baggesen K, Hejtmancik JF, et al. Genetic heterogeneity in microcornea-cataract: five novel mutations in CRYAA, CRYGD, and GJA8. Invest Ophthalmol Vis Sci. Sep 2007;48(9):3937-44. [Medline].

  30. Holmberg J, Liu CY, Hjalt TA. PITX2 gain-of-function in Rieger syndrome eye model. Am J Pathol. Nov 2004;165(5):1633-41. [Medline].

  31. Sherman MD. Dermoids, Limbal. eMedicine Journal [serial online]. 2007;Available at http://www.emedicine.com/oph/topic210.htm.

  32. Stone DU, Siatkowski RM. Congenital retinal dystrophy and corneal opacity in trisomy 8 mosaicism. J AAPOS. Jun 2005;9(3):290-1. [Medline].

  33. Tewfik TL. Manifestations of Craniofacial Syndromes. eMedicine Journal [serial online]. 2006;Available at http://www.emedicine.com/ent/topic319.htm.

  34. Wimplinger I, Shaw GM, Kutsche K. HCCS loss-of-function missense mutation in a female with bilateral microphthalmia and sclerocornea: a novel gene for severe ocular malformations?. Mol Vis. Aug 27 2007;13:1475-82. [Medline].

  35. Zaidman GW. The use of CT scanning in the diagnosis of protuberant congenital corneal opacities. J Pediatr Ophthalmol Strabismus. Mar-Apr 1999;36(2):101-2. [Medline].

Further Reading

Keywords

Peters' anomaly, Peter anomaly, sclerocornea, congenital central corneal leukoma, primary congenital glaucoma, PCG, congenital hereditary endothelial dystrophy, CHED, posterior polymorphous dystrophy, PPMD, mucopolysaccharidosis, MPS

Contributor Information and Disclosures

Author

Noah S Scheinfeld, MD, JD, FAAD, Assistant Clinical Professor, Department of Dermatology, Columbia University; Consulting Staff, Department of Dermatology, St Luke's Roosevelt Hospital Center, Beth Israel Medical Center, New York Eye and Ear Infirmary; Private Practice
Noah S Scheinfeld, MD, JD, FAAD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Optigenex Consulting fee Independent contractor

Coauthor(s)

Benjamin D Freilich, MD, FACS, Assistant Clinical Professor, Department of Ophthalmology, Mount Sinai School of Medicine; Director of Retina Service, Bronx Veterans Administration Medical Center
Disclosure: Nothing to disclose.

Jonathan Freilich, MD, FACS, Clinical Instructor, Department of Ophthalmology, Mount Sinai School of Medicine; Consulting Staff, Department of Ophthalmology, Mount Sinai Hospital, St Luke's Roosevelt Hospital Center
Disclosure: Nothing to disclose.

Medical Editor

Richard W Allinson, MD, Associate Professor, Department of Ophthalmology, Texas A&M University Health Science Center, Scott and White Clinic
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Christopher J Rapuano, MD, Professor, Department of Ophthalmology, Jefferson Medical College of Thomas Jefferson University; Co-Chairman of the Cornea Service, Co-Chairman of Refractive Surgery Department, Wills Eye Institute
Christopher J Rapuano, MD is a member of the following medical societies: American Academy of Ophthalmology, American Society of Cataract and Refractive Surgery, Eye Bank Association of America, Pennsylvania Medical Society, and Philadelphia County Medical Society
Disclosure: Allergan Honoraria Speaking and teaching; Allergan Consulting fee Consulting; Alcon Honoraria Speaking and teaching; Inspire Honoraria Speaking and teaching; RPS Ownership interest Other

CME Editor

Ralph Garzia, OD, Assistant Dean for Clinical Programs, Associate Professor, School of Optometry, University of Missouri at St Louis
Ralph Garzia, OD is a member of the following medical societies: American Academy of Optometry and American Optometric Association
Disclosure: Nothing to disclose.

Chief Editor

Hampton Roy Sr, MD, Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences
Hampton Roy Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, and Pan-American Association of Ophthalmology
Disclosure: Nothing to disclose.

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.