eMedicine Specialties > Ophthalmology > Cornea

Posterior Polymorphous Corneal Dystrophy: Follow-up

Author: Dustin J Coupal, MD, FRCSC, Eye Specialist and Surgeon, Private Practice
Coauthor(s): W Keith Hamilton, MD, Clinical Assistant Professor, Department of Ophthalmology, Saskatoon City Hospital Eye Centre
Contributor Information and Disclosures

Updated: Feb 1, 2007

Follow-up

Further Outpatient Care

  • The frequency of follow-up care for patients with PPMD depends on the severity of disease.

Complications

  • Corneal erosion, secondary corneal ulcers, corneal scarring, graft rejection, endothelial failure, and other complications can occur and lead to corneal graft failure.
  • Glaucoma can be present prior to corneal transplantation or may result from corneal transplantation.

Prognosis

  • The prognosis is generally dependent on the severity of disease. In most cases, PPMD is a slowly progressive or nonprogressive disease. Patients with mild disease that is identified in adulthood have the best prognosis and are unlikely to require penetrating keratoplasty during their lifetime.

Miscellaneous

Medicolegal Pitfalls

  • Failure to have patients who have recently been diagnosed with PPMD undergo renal evaluation and hearing tests to assess for Alport syndrome, which has been associated with PPMD.
  • Patients need to be monitored for the development of glaucoma.

Special Concerns

  • Family members should be examined for evidence of asymptomatic corneal disease, which may help establish a diagnosis of PPMD.
 


More on Posterior Polymorphous Corneal Dystrophy

Overview: Posterior Polymorphous Corneal Dystrophy
Differential Diagnoses & Workup: Posterior Polymorphous Corneal Dystrophy
Treatment & Medication: Posterior Polymorphous Corneal Dystrophy
Follow-up: Posterior Polymorphous Corneal Dystrophy
Multimedia: Posterior Polymorphous Corneal Dystrophy
References
[ CLOSE WINDOW ]

References

  1. Anderson NJ, Badawi DY, Grossniklaus HE, Stulting RD. Posterior polymorphous membranous dystrophy with overlapping features of iridocorneal endothelial syndrome. Arch Ophthalmol. Apr 2001;119(4):624-5. [Medline].

  2. Bechara SJ, Grossniklaus HE, Waring GO, Wells JA 3rd. Keratoconus associated with posterior polymorphous dystrophy. Am J Ophthalmol. Dec 15 1991;112(6):729-31. [Medline].

  3. Boruchoff SA, Weiner MJ, Albert DM. Recurrence of posterior polymorphous corneal dystrophy after penetrating keratoplasty. Am J Ophthalmol. Mar 15 1990;109(3):323-8. [Medline].

  4. Brooks AM, Grant G, Gillies WE. Differentiation of posterior polymorphous dystrophy from other posterior corneal opacities by specular microscopy. Ophthalmology. Nov 1989;96(11):1639-45. [Medline].

  5. Chiou AG, Kaufman SC, Beuerman RW, et al. Confocal microscopy in posterior polymorphous corneal dystrophy. Ophthalmologica. 1999;213(4):211-3. [Medline].

  6. Colville DJ, Savige J. Alport syndrome. A review of the ocular manifestations. Ophthalmic Genet. Dec 1997;18(4):161-73. [Medline].

  7. Grupcheva CN, Chew GS, Edwards M, et al. Imaging posterior polymorphous corneal dystrophy by in vivo confocal microscopy. Clin Experiment Ophthalmol. Aug 2001;29(4):256-9. [Medline].

  8. Henriquez AS, Kenyon KR, Dohlman CH, et al. Morphologic characteristics of posterior polymorphous dystrophy. A study of nine corneas and review of the literature. Surv Ophthalmol. Sep-Oct 1984;29(2):139-47. [Medline].

  9. Klintworth GK. The molecular genetics of the corneal dystrophies--current status. Front Biosci. May 1 2003;8:d687-713. [Medline].

  10. Koeppe L. Klinische Beobachtungen mit der Nerstspaltlampe und dem Hornhautmikroskop. Albrecht von Graefe's Arch Klin Exp Ophthalmol. 1916;91:375-379.

  11. Krachmer JH. Posterior polymorphous corneal dystrophy: a disease characterized by epithelial-like endothelial cells which influence management and prognosis. Trans Am Ophthalmol Soc. 1985;83:413-75. [Medline].

  12. Laganowski HC, Sherrard ES, Muir MG, Buckley RJ. Distinguishing features of the iridocorneal endothelial syndrome and posterior polymorphous dystrophy: value of endothelial specular microscopy. Br J Ophthalmol. Apr 1991;75(4):212-6. [Medline].

  13. Moroi SE, Gokhale PA, Schteingart MT, et al. Clinicopathologic correlation and genetic analysis in a case of posterior polymorphous corneal dystrophy. Am J Ophthalmol. Apr 2003;135(4):461-70. [Medline].

  14. Presberg SE, Quigley HA, Forster RK, Green WR. Posterior polymorphous corneal dystrophy. Cornea. 1985-86;4(4):239-48. [Medline].

  15. Teekhasaenee C, Nimmanit S, Wutthiphan S, et al. Posterior polymorphous dystrophy and Alport syndrome. Ophthalmology. Aug 1991;98(8):1207-15. [Medline].

  16. Weissman BA, Ehrlich M, Levenson JE, Pettit TH. Four cases of keratoconus and posterior polymorphous corneal dystrophy. Optom Vis Sci. Apr 1989;66(4):243-6. [Medline].

[ CLOSE WINDOW ]

Further Reading

[ CLOSE WINDOW ]

Keywords

PPMD, posterior polymorphous dystrophy, posterior endothelial dystrophy, hereditary deep dystrophy, PPCD

[ CLOSE WINDOW ]

Contributor Information and Disclosures

Author

Dustin J Coupal, MD, FRCSC, Eye Specialist and Surgeon, Private Practice
Dustin J Coupal, MD, FRCSC is a member of the following medical societies: American Academy of Ophthalmology, Canadian Medical Association, Canadian Medical Protective Association, and Canadian Ophthalmological Society
Disclosure: Nothing to disclose.

Coauthor(s)

W Keith Hamilton, MD, Clinical Assistant Professor, Department of Ophthalmology, Saskatoon City Hospital Eye Centre
Disclosure: Nothing to disclose.

Medical Editor

Fernando H Murillo-Lopez, MD, Senior Surgeon, Unidad Privada de Oftalmologia CEMES
Fernando H Murillo-Lopez, MD is a member of the following medical societies: American Academy of Ophthalmology
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; Co-Chairman of the Cornea Service, Co-Chairman of Refractive Surgery Department, Wills Eye Hospital
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.