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Pellucid Marginal Degeneration


Presentation

  • We present a report of a spontaneous corneal perforation in a patient with pellucid marginal corneal degeneration.[ncbi.nlm.nih.gov]
  • Transplantation of the entire thickness of the cornea (penetrating keratoplasty) may be performed if there is enough normal tissue present. However, if there is not enough normal tissue present, then attaching the graft is difficult.[en.wikipedia.org]
Amyloidosis
  • Secondary corneal amyloidosis is a corneal response to chronic injury and irritation that is not commonly reported and often overlooked.[ncbi.nlm.nih.gov]
Hypotension
  • Acute hydropsAcute hydrops • Risk factor:- Ocular allergy,Eye rubbing,Down syndrome, • C/O:- blurred vision, irritation, pain, watering, photophobia • Mx:- conservative • Hypertonic agents, steroids, antibiotics, cycloplegics, hypotensive agents • Bandage[slideshare.net]
Corneal Infiltrate
  • Unlike keratoconus, PMD usually does not show signs of Fleischer’s ring, corneal infiltration, vascularisation, scarring or lipid deposition.[nhp.gov.in]
Chemosis
  • Ocular adenexa were healthy without any congestion or chemosis.[ijo.in]
Rabies
  • 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. 2004 Mar-Apr. 20(2):162-5. [Medline].[emedicine.medscape.com]
  • Javadi MA, Karimian F, Hosseinzadeh A, Noroozizadeh HM, Sa’eedifar MR, Rabie HM. Lamellar crescentic resection for pellucid marginal corneal degeneration. J Refract Surg 2004;20:162–165. PubMed Google Scholar 13. Fleming JR, Reynolds AI, Kilmer L.[link.springer.com]

Treatment

  • Intracameral injection of C(3)F(8) may possibly be a viable treatment option for acute hydrops in a case of corneal ectasia.[ncbi.nlm.nih.gov]
  • Videokeratography Maps in the Left Eye Before Treatment. The Topographic Pattern Highlights the “crab claw” Appearance, with 7.80-D Against-The-Rule Astigmatism.[doi.org]

Prognosis

  • Prognosis Prognosis of PMD is quite good. Vision restoration with our special contact lenses results in normal vision allowing legal driving with minimal visual distortions. Many eyes can be be restored to normal vision even in severe cases.[lensdesign.ca]
  • Prognosis The affected cornea maintains its clarity. However, patients experience progressive deterioration of vision due to irregular astigmatism. [6] The prognosis of pellucid marginal degeneration is clinical. Krachmer JH.[emedicine.medscape.com]
  • Often times this condition is confused with Keratoconus and shares many of its characteristics and prognosis.[precisionfamilyeyecare.com]

Etiology

  • CONCLUSIONS: Although little is understood about the etiology, pathophysiology, epidemiology, and genetics of PMD, new treatments are improving visual outcomes and reducing complications.[ncbi.nlm.nih.gov]
  • June 2013 Volume 54, Issue 15 Free ARVO Annual Meeting Abstract June 2013 Familial Corneal Patterns in Pellucid Marginal Degeneration (PMD): Uncovering a Possible Etiology Author Affiliations & Notes Hajirah Saeed Ophthalmology, Loyola University Medical[iovs.arvojournals.org]
  • Etiology The etiology of PMD remains unknown. Risk Factors PMD, while rare, is the second most common noninflammatory corneal thinning disorder behind keratoconus.[eyewiki.aao.org]

Epidemiology

  • CONCLUSIONS: Although little is understood about the etiology, pathophysiology, epidemiology, and genetics of PMD, new treatments are improving visual outcomes and reducing complications.[ncbi.nlm.nih.gov]
  • 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.[emedicine.medscape.com]
  • […] techniques allow thickening of the inferior peripheral cornea and steepening of the vertical meridien. [15] [16] Due to the thinning of the cornea, people with PMD are poor candidates for procedures such as LASIK and photorefractive keratectomy. [17] Epidemiology[en.wikipedia.org]
Sex distribution
Age distribution

Pathophysiology

  • CONCLUSIONS: Although little is understood about the etiology, pathophysiology, epidemiology, and genetics of PMD, new treatments are improving visual outcomes and reducing complications.[ncbi.nlm.nih.gov]
  • Currently, many corneal specialists view keratoconus and corneal pellucid marginal degeneration as the same basic pathophysiology of the cornea but with a different primary focus (ie, inferocentral for keratoconus; generally inferior for corneal pellucid[crstoday.com]
  • Pathophysiology The exact pathophysiology of PMD has not been ascertained, but it is thought to be secondary to collagen abnormalities, similar to keratoconus.[eyewiki.aao.org]

Prevention

  • Primary prevention No preventative strategies exist for PMD. Given the corneal thinning and the potential association of eye rubbing with keratoconus and its relationship to PMD, it could be considered to advise patients not to rub their eyes.[eyewiki.aao.org]

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