Treatment of Pellucid Marginal Degeneration 1Abdelsattar N Farrag, 2Ahmed a Hussein, 3Shiji Ummar
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IJKECD Treatment10.5005/jp-journals-10025-1148 of Pellucid Marginal Degeneration REVIEW ARTICLE Treatment of Pellucid Marginal Degeneration 1Abdelsattar N Farrag, 2Ahmed A Hussein, 3Shiji Ummar ABSTRACT Although PMD classically has been affecting the infe- Purpose: To summarize the recent trends in the treatment rior cornea, superior PMD has also been reported, and we of pellucid marginal degeneration (PMD) based on available should consider it in the differential diagnosis of superior published data. corneal ectasia.7 The ectasia in PMD causes progressive Method and literature search: A PubMed search was con- diminution of both uncorrected and corrected visual ducted with combinations not limited to the following search acuity as a result of high against-the-rule astigmatism.1,2 terms: Pellucid marginal degeneration, Corneal ectasia, The condition is most commonly affecting males Corneal collagen cross-linking (CXL), Intracorneal ring seg- ments (ICRS), Contact lens, Keratoplasty in corneal ectasia. and usually presents between the 2nd and 5th decades 3,8 A review of the search results was performed and relevant of life. articles to the topic were included. The PMD can be diagnosed classically by slit-lamp Summary: Ophthalmologists have got a wide array of thera- examination, which shows a clear band of inferior corneal peutic modalities for the management of PMD. However, the key thinning extending from 4 to 8 o’clock. There is typically to optimal treatment is careful clinical assessment of patients a 1 to 2 mm of uninvolved normal cornea. The maximum and their visual requirements and tailoring the treatment to point of protrusion in PMD occurs in the area superior individual patients. to the thinning. This is considered one of the important Keywords: Corneal collagen cross-linking, Corneal ectasia, Intracorneal ring segment, Keratoplasty, Pellucid marginal points in differentiation with keratoconus, in which the 9-11 degeneration. maximum protrusion is within the area of thinning. Corneal topography with classical pattern of crab-claw How to cite this article: Farrag AN, Hussein AA, Ummar S. Treatment of Pellucid Marginal Degeneration. Int J Kerat Ect appearance is not diagnostic for PMD, as it can present Cor Dis 2017;6(2):87-91. with inferior keratoconus. So, it is recommended to Source of support: Nil perform more detailed analysis with slit-lamp micros- copy, topography, anterior and posterior elevation maps Conflict of interest: None by Scheimpflug imaging and full pachymetric maps that shows 12 mm of corneal coverage for improving the INTRODUCTION diagnostic accuracy for PMD.12 Pellucid marginal corneal degeneration is a bilateral, Management modalities of PMD can be broadly clas- noninflammatory, corneal ectasia, most frequently sified as: involving the inferior peripheral cornea in a crescentic • Corneal rehabilitation treatment which includes: pattern. The corneal thinning usually arises between the – Nonsurgical: spectacles, contact lenses 4 and 8 o’clock position and protrusion occurs as a result – Surgical: ICRS and CXL of this thinning.1-3 Schlaeppi4 was the first one who used • Toric intraocular lens (IOL) implantation the term “pellucid,” meaning clear, to signify the clarity • Corneal replacement surgery which includes: of the cornea despite the presence of corneal ectasia. It – Full thickness or penetrating keratoplasty (PKP): is usually bilateral and asymmetrical; nevertheless, uni- oversized central PKP/inferiorly decentered PKP, lateral cases are reported.5,6 lamellar keratoplasty (LK), simultaneous central PKP, and inferior peripheral LK, intrastromal LK, crescentic wedge resection, epikeratophakia, and 1Staff Registrar, 2Resident, 3Cornea Specialist thermokeratoplasty. The aim of this article is to review the current treat- 1Department of Ophthalmology, Singapore National Eye Center Singapore ment modalities of PMD. 2Department of Ophthalmology, Baladi Foundation, Aswan, Egypt NONSURGICAL CORNEAL REHABILITATION 3Department of Ophthalmology, Al Nahdha Hospital, Muscat Oman TREATMENT Corresponding Author: Shiji Ummar, Cornea Specialist Most patients with early and moderate PMD can be visu- Department of Ophthalmology, Al Nahdha Hospital, Muscat ally rehabilitated by spectacles or contact lenses.3,8 Oman, Phone: +96893504023, e-mail: dr.shijiparvez@yahoo. In the early stages of PMD, soft toric lenses can be an co.in option for improvement in visual acuity. International Journal of Keratoconus and Ectatic Corneal Diseases, July-December 2017;6(2):87-91 87 Abdelsattar N Farrag et al In moderate PMD, treatment usually requires the use forward displacement of the anterior surface at this area. of large-diameter rigid, gas-permeable lenses due to the This causes a peripheral steepening and a flattening of the development of irregular astigmatism and progression central part of the anterior cornea due to the morphologic of corneal irregularity.8,13 In addition, specialized contact structure of the corneal lamellae.24 The same principle lenses, such as reverse geometry lenses and “bi-toric” can be applied in cases of PMD resulting in decreasing lenses can be useful in the management of moderate and centralization of the peripheral corneal protrusion, PMD.14,15 inducing a significant change in corneal asymmetry and In advanced PMD, the scleral contact lens is a helpful refraction. nonsurgical treatment when the fitting with corneal The implantation of ICRS in PMD proved to be effec- lenses is extremely difficult because of the peripheral tive in the improvement of visual outcome either by location of the ectasia.16 manual or femtosecond surgery.25-31 Scleral lenses are large-diameter gas-permeable A case report showed improvement of visual acuity lenses that completely vault the cornea and rest on the after femtosecond implantation of a single ICRS in supe- sclera. Many studies reported the successful use of rior PMD. However, follow-up was only for 3 months.32 scleral lenses in the treatment of ocular surface disorders, The ICRS implantation may be associated with post- postkeratoplasty patients, and advanced corneal ectatic operative complications, which include segment extru- disorders.16-18 sion, corneal neovascularization, infectious keratitis, In a recent study evaluating the success of prosthetic channel deposits around the ring, ring segment migra- replacement of the ocular surface ecosystem (PROSE) tion, epithelial plug at the incision site, corneal melting, in PMD patients, a significant improvement in visual and night halos.33 acuity with PROSE (Boston Foundation for Sight, USA) Limited number of case reports or case series of ICRS is reported in all patients with PMD after failure of all implantation in PMD may be due to rare presentation in other nonsurgical options. However, we should be careful clinical practice and that ICRS implantation is limited to while prescribing these lenses at the advanced stage of the specific criteria, which may not be fit for most patients. disease as the risk of hydrops is high in these patients.19 Future studies are recommended with larger number of Also, another study reported success in the use of patients. scleral Misa1 lenses (Microlens Contact lens Techno logy, Arnhem, Netherlands) to improve visual acuity Collagen Cross-linking in long-term follow-up in patients with PMD, when any Wollensak et al34 demonstrated the effects of CXL on other optical modalities failed, and they recommend it the biomechanical properties of human and porcine for moderate advanced PMD when other types of contact cornea. Riboflavin (vitamin B2) is a photosensitizer lens cannot be fitted or in cases of poor operative prog- with an absorption peak for ultraviolet (UV) A at a nosis and for patients who refuse surgical intervention.20 wavelength of 370 nm. The molecule fluoresces and SURGICAL CORNEAL REHABILITATION excites to a triplet state when exposed to UV radiation of this wavelength, with subsequent release of singlet TREATMENT oxygen and superoxide radicals. In a cornea saturated Intracorneal Ring Segment with riboflavin, there will be a formation of new cova- Intracorneal ring segments are small pieces made of lent bonds between collagen molecules by oxidative polymethylmethacrylate, which are implanted in the deamination. It has proved its efficacy in arresting 35-37 deep corneal stroma, aiming for modifications of corneal progression of keratoconus. curvature and refractive changes. This notion was pro- A limited number of studies and case reports posed by Reynolds in 1978. The first implants were full have been published describing the effect of CXL in rings implanted into a circumferential corneal channel PMD. All showed improvement and/or stabilization 38-41 through a peripheral single corneal incision. Then to of visual acuity and keratometric parameters. The decrease the rate of incision-related complications and CXL was also combined with topography-guided tran- make it surgically easier, the rings were changed to the sepithelial surface ablation and showed improvement C-shape range, and they were renamed as ICRS.21-23 of visual outcome. However, the follow-up period was The ICRS is used in early-to-moderate PMD for 12 months.42 deferring corneal transplant and improving tolerance of Koller et al43 examined the peripheral effect of CXL contact lens. The principle of using this originated from by evaluation of the stromal demarcation line by OCT, what was demonstrated by the addition of extramaterial and they concluded that the targeted depth of CXL using at the normal