eMedicine Specialties > Ophthalmology > Cornea

Posterior Polymorphous Corneal Dystrophy: Treatment & Medication

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

Treatment

Medical Care

Management of PPMD varies widely based on the differences in severity of corneal decompensation. Many patients who are asymptomatic and show minimal signs of PPMD can be treated conservatively and do not require therapy.

  • Ruptured corneal bullae should be treated similar to a corneal abrasion.
  • Hyperosmotic saline drops and ointments may be used in cases of corneal failure with corneal edema.
  • Secondary glaucoma may require medical or surgical management.
  • A bandage soft contact lens may be used as a temporary measure to treat bullous keratopathy.

Surgical Care

  • Corneal transplantation is usually reserved for patients with substantially decreased visual acuity or when the disease is advanced and painful due to ruptured epithelial bullae. Penetrating keratoplasty (full thickness corneal transplantation) has been the transplant procedure of choice in the past. The newer techniques of posterior endothelial keratoplasty may have some advantages for some patients.
  • Rare cases of corneal graft failure due to recurrence of PPMD in the donor graft have been reported, but the most common problem following a corneal transplant is related to uncontrolled glaucoma.
  • To treat pain in eyes without good visual potential, other procedures, such as anterior stromal micropuncture, excimer laser phototherapeutic keratectomy, amniotic membrane transplantation, and conjunctival flap surgery, can also be considered.
  • When glaucoma medications no longer adequately control the intraocular pressure, glaucoma laser or incisional surgery may be required to prevent glaucomatous optic nerve damage.

Consultations

Depending on the severity of the condition, consultation with corneal and glaucoma subspecialists may be warranted.

Medication

The goals of pharmacotherapy are to reduce morbidity and to prevent complications.

Hypertonic agents

Sodium chloride hypertonic ophthalmic solutions are used to dehydrate the cornea.


Sodium chloride (Muro 128 Ophthalmic)

Used for temporary relief of corneal edema. Available as 2% and 5% ophthalmic solution concentrations and 5% ointment.

Adult

Solution: 1-2 gtt into affected eye(s) q3-4h
Ointment: Apply 0.5-inch ribbon into affected eye(s) q3-4h

Pediatric

Administer as in adults

Pregnancy

C - Safety for use during pregnancy has not been established.

Precautions

May cause temporary burning and irritation upon use; if pain, change in vision, continued redness, or irritation of the eye occurs or if the initial condition/problem worsens or persists, reevaluate therapy; do not use product if it changes color or becomes cloudy

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
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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].

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Further Reading

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Keywords

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

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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.

 
 
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