eMedicine Specialties > Ophthalmology > Cornea

Posterior Polymorphous Corneal Dystrophy: Differential Diagnoses & Workup

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

Differential Diagnoses

Dystrophy, Fuchs Endothelial
Glaucoma, Primary Congenital

Other Problems to Be Considered

Congenital hereditary endothelial dystrophy (CHED)
Iridocorneal endothelial (ICE) syndrome

Workup

Imaging Studies

  • Specular microscopy has proven to be valuable in identifying the characteristic vesicular changes of the Descemet membrane in patients with PPMD. Typically, the endothelial cell count is only mildly diminished with varying amounts of polymegethism and pleomorphism.
  • Confocal microscopy has also been used to identify the typical features of PPMD.
  • On most occasions, PPMD can be clinically diagnosed using slit lamp examination; however, mild cases may require further imaging studies to confirm the diagnosis.

Other Tests

  • Tonometry is used to measure intraocular pressure, which may be elevated in up to 40% of patients with PPMD.
  • The secondary open-angle glaucoma that occurs in some patients with PPMD is believed to be due to an abnormality of the trabecular endothelium.
  • Examination of family members may also be helpful in making the diagnosis.

Histologic Findings

Light microscopy typically demonstrates 4 types of cells: normal endothelial cells, degenerated endothelial cells, fibroblast-like cells, and epithelial-like cells. In 60% of cases, a multilayered endothelium can be identified that has many characteristics similar to that of the epithelium. Immunohistochemical studies have also shown positive staining of the endothelium using epithelial cell markers.

Scanning electron microscopy and transmission electron microscopy of corneal tissue show lamination of the Descemet membrane, fibroblast-like endothelial cells, and microvilli characteristic of epithelial transformation of the corneal endothelium.

Staging

No formal staging system has been described for 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
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References

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  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].

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  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].

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  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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