Congenital Clouding of the Cornea Follow-up

  • Author: Noah S Scheinfeld, MD, JD, FAAD; Chief Editor: Hampton Roy Sr, MD   more...
 
Updated: Jul 22, 2011
 

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.
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Inpatient & Outpatient Medications

  • Medications may be indicated for treatment after corneal transplantation.
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Complications

  • Complications of corneal transplantation are varied.
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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.
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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.
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Contributor Information and Disclosures
Author

Noah S Scheinfeld, MD, JD, FAAD  Assistant Clinical Professor, Department of Dermatology, Columbia University College of Physicians and Surgeons; Consulting Staff, Department of Dermatology, St Luke's Roosevelt Hospital Center, Beth Israel Medical Center, and 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.

Specialty Editor Board

Richard W Allinson, MD  Associate Professor, Department of Ophthalmology, Texas A&M University Health Science Center; Senior Staff Ophthalmologist, Scott and White Clinic

Richard W Allinson, MD, is a member of the following medical societies: American Academy of Ophthalmology, American Medical Association, and Texas Medical Association

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Christopher J Rapuano, MD  Professor, Department of Ophthalmology, Jefferson Medical College of Thomas Jefferson University; Director of the Cornea Service, Co-Director 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, Contact Lens Association of Ophthalmologists, Cornea Society, Eye Bank Association of America, International Society of Refractive Surgery, and Pan-American Association of Ophthalmology

Disclosure: Allergan Honoraria Speaking and teaching; Allergan Consulting fee Consulting; Alcon Honoraria Speaking and teaching; Inspire Honoraria Speaking and teaching; RPS Ownership interest Other; Vistakon Honoraria Speaking and teaching; EyeGate Pharma Consulting; Inspire Consulting fee Consulting; Bausch & Lomb Honoraria Speaking and teaching; Bausch & Lomb Consulting fee Consulting

Ralph Garzia, OD  Assistant Dean for Clinical and Academic Programs, Associate Professor, College 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.

References
  1. Bhat YR, Sanoj KM. Images in Clinical Practices. Sclerocornea. Indian Pediatrics. Mar 17 2005;42:42. [Full Text].

  2. EyeMDLink. Congenital hereditary endothelial dystrophy. Available at http://www.allaboutvision.com/conditions/.

  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. Desir J, Abramowicz M. Congenital hereditary endothelial dystrophy with progressive sensorineural deafness (Harboyan syndrome). Orphanet J Rare Dis. Oct 15 2008;3:28. [Medline].

  6. 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].

  7. Perry HD, Cameron JD. Congenital corneal opacities. Available at http://www.ophthalmic.hyperguides.com.

  8. Bower KS, Edwards JD, Wagner ME, Ward TP, Hidayat A. Novel corneal phenotype in a patient with alport syndrome. Cornea. 2009;28:599-606. [Medline].

  9. Murphy MJ, Polok BK, Schorderet DF, Cleary ML. Essential role for Pbx1 in corneal morphogenesis. Invest Ophthalmol Vis Sci. Feb 2010;51(2):795-803. [Medline]. [Full Text].

  10. Wang KJ, Wang BB, Zhang F, Zhao Y, Ma X, Zhu SQ. Novel beta-crystallin gene mutations in Chinese families with nuclear cataracts. Arch Ophthalmol. Mar 2011;3:337-43. [Medline].

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

  12. 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].

  13. 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].

  14. 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].

  15. Floyd MS, Kwon YH, Shah S, Benson C, Longmuir SQ. Unilateral congenital glaucoma in a child with optic nerve aplasia. J AAPOS. Apr 2011;2:200-2. [Medline].

  16. 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].

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

  18. 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].

  19. 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].

  20. 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].

  21. 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].

  22. Kottler U, Demir D, Schmidtmann I, Beck M, Pitz S. Central corneal thickness in mucopolysaccharidosis II and VI. Cornea. Mar 2010;29(3):260-2. [Medline].

  23. 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].

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

  25. 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].

  26. 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].

  27. 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].

  28. 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].

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

  30. 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].

  31. Cassidy L. Paediatric cataract. March 9 2001. optometrytoday. Available at http://www.optometry.co.uk/files/e8cf2c379b358b7d42e25bdfa54a9abf_cassidy20010309.pdf. Accessed March 12, 2011.

  32. 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].

  33. Ciralsky J, Colby K. Congenital corneal opacities: a review with a focus on genetics. Semin Ophthalmol. Oct-Dec 2007;22:241-6. [Medline].

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

  35. 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].

  36. 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].

  37. 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].

  38. Parthasarathy A, Naumann GO. Coexistent congenital hereditary endothelial dystrophy and congenital glaucoma. Cornea. Apr 2009;28(3):365; author reply 365-6. [Medline].

  39. Sherman MD. Dermoids, Limbal. eMedicine Journal [serial online]. 2010;Available at http://emedicine.medscape.com/article/1195334-overview.

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

  41. Tewfik TL. Manifestations of Craniofacial Syndromes. eMedicine Journal [serial online]. 2010;Available at http://emedicine.medscape.com/article/844209-overview.

  42. Thiagalingam S, Jakobiec FA, Chen T, Michaud N, Colby KA, Walton DS. Corneal anomalies in newborn primary congenital glaucoma. J Pediatr Ophthalmol Strabismus. Jul-Aug 2009;46(4):241-4. [Medline].

  43. 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].

  44. 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].

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Clouding of the cornea since childhood.
 
 
 
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