Pellucid Marginal Degeneration 

  • Author: Karim Rasheed, MD, MSc; Chief Editor: Hampton Roy Sr, MD   more...
 
Updated: Feb 18, 2010
 

Background

Schalaeppi first coined the term pellucid marginal degeneration in 1957. This bilateral, noninflammatory, peripheral corneal thinning disorder is characterized by a peripheral band of thinning of the inferior cornea. The cornea in and adjacent to the thinned area is ectatic.

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Pathophysiology

The etiology of pellucid marginal degeneration has not been clearly established, but collagen abnormalities, as seen in keratoconus, have been reported. The thinned and presumably weakened cornea may protrude as a result of the positive intraocular pressure.

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Epidemiology

Frequency

United States

Pellucid marginal degeneration is rare, and the exact incidence and prevalence are unknown. However, the incidence may be considerably underestimated, as this condition is often misdiagnosed as keratoconus.

Mortality/Morbidity

  • Deterioration of visual function results from the irregular astigmatism induced by asymmetric distortion of the cornea. The deterioration in visual function is commonly severe.
  • Refractive surgery, such as photorefractive keratectomy (PRK), can cause severe corneal haze, and results with laser-assisted in situ keratomileusis (LASIK) may be unpredictable.

Race

No racial preponderance has been identified.

Sex

An equal distribution exists between the sexes.

Age

Patients usually are aged 20-40 years at the time of clinical presentation.

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Contributor Information and Disclosures
Author

Karim Rasheed, MD, MSc  Medical Director, Sani Eye Center

Karim Rasheed, MD, MSc is a member of the following medical societies: American Academy of Ophthalmology

Disclosure: Nothing to disclose.

Coauthor(s)

Yaron Rabinowitz, MD  Chairman, Division of Ophthalmology, Cedars-Sinai Medical Center; Clinical Associate Professor, Departments of Ophthalmology and Pediatrics, University of California at Los Angeles

Disclosure: Nothing to disclose.

Specialty Editor Board

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.

Simon K Law, MD, PharmD  Assistant Professor of Ophthalmology, Jules Stein Eye Institute; Chief of Section of Ophthalmology Surgical Services, Department of Veterans Affairs Healthcare Center, West Los Angeles

Simon K Law, MD, PharmD is a member of the following medical societies: American Academy of Ophthalmology, American Glaucoma Society, and Association for Research in Vision and Ophthalmology

Disclosure: Nothing to disclose.

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, 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

Lance L Brown, OD, MD  Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri

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. Oie Y, Maeda N, Kosaki R, Suzaki A, Hirohara Y, Mihashi T, et al. Characteristics of ocular higher-order aberrations in patients with pellucid marginal corneal degeneration. J Cataract Refract Surg. Nov 2008;34(11):1928-34. [Medline].

  2. Ertan A, Bahadir M. J. Intrastromal ring segment insertion using a femtosecondlaser to correct pellucid marginal corneal degeneration. J Cataract Refract Surg. Oct 2006;32(10):1710-6. [Medline].

  3. Ghajarnia M, Moshirfar M, Mifflin MD. Descemet detachment after femtosecond-laser-assisted placement of intrastromal ring segments in pellucid marginal degeneration. J Cataract Refract Surg. Dec 2008;34(12):2174-6. [Medline].

  4. Rasheed K, Rabinowitz YS. Results of combined lamellar and penetrating keratoplasty for pellucid marginal degeneration [abstr]. Ophthalmol Suppl. 1997;Oct:191.

  5. Javadi MA, Karimian F, Hosseinzadeh A, Noroozizadeh HM, Sa'eedifar MR, Rabie HM, et al. Lamellar crescentic resection for pellucid marginal corneal degeneration. J Refract Surg. Mar-Apr 2004;20(2):162-5. [Medline].

  6. Krachmer JH. Pellucid marginal corneal degeneration. Arch Ophthalmol. Jul 1978;96(7):1217-21. [Medline].

  7. Kymionis GD, Aslanides IM, Siganos CS, Pallikaris IG. Intacs for early pellucid marginal degeneration. J Cataract Refract Surg. Jan 2004;30(1):230-3. [Medline].

  8. Maguire LJ, Klyce SD, McDonald MB, Kaufman HE. Corneal topography of pellucid marginal degeneration. Ophthalmology. May 1987;94(5):519-24. [Medline].

  9. Rabinowitz YS. Keratoconus. Surv Ophthalmol. Jan-Feb 1998;42(4):297-319. [Medline].

  10. Schanzlin DJ, Sarno EM, Robin JB. Crescentic lamellar keratoplasty for pellucid marginal degeneration. Am J Ophthalmol. Aug 1983;96(2):253-4. [Medline].

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Slit lamp image of the inferior cornea in a patient with advanced pellucid marginal degeneration. Image illustrates inferior corneal thinning, a hallmark of this disease.
Corneal topography of early (right eye) and moderate (left eye) pellucid marginal degeneration.
Image shows simultaneous central penetrating keratoplasty and inferior peripheral lamellar keratoplasty performed to treat pellucid marginal degeneration.
 
 
 
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