Spontaneous perforation and only unilateral involvement is rare in PMD. Topographic finding at 6 months postoperatively. The left eye shows no clinical or topographical evidence of PMD 2 years after the procedure. After suture removal, the patient obtained a CDVA of 6/9 (−2.75 D cylinder at 90 degrees), and topographically, there was a reduction in steep K (K2 = 42.8 D at 160 degrees) and regularization of the cornea as shown in Figures 5 and 6 at 6 months postoperatively. At the fourth-month postoperative period, alternate suture removal was performed and removed subsequently over a period of 1 month. Topical steroids (loteprednol 0.5%) were started on the second postoperative day and slowly tapered over 6 weeks. On postoperative day 1, the anterior chamber was formed, and sutures were intact and topical medications (moxifloxacin eye drops 0.5%, hydroxypropyl methylcellulose 0.3%, and homatropine 2%) were given. Six interrupted radial sutures were placed using 10-0 nylon (Ethilon) and a bandage contact lens was placed. The defect was surgically repaired with suturing (2 sutures as primary sutures at the perforation site) and iris repositioning with additional compression sutures along the crescentric area (2 sutures on either side of the primary sutures directed along the flat axis obtained from the topographic images) as shown in Figure 4. Topographic image of the left eye at presentation.įour months later, the patient presented with a sudden decrease in vision with acute pain in the right eye and was noted to have full-thickness perforation at the 6 o'clock to 7 o'clock position measuring approximately 2.5 mm with iris prolapse as shown in Figure 3. A diagnosis of PMD was made, and a scleral contact lens was prescribed (the patient did not use it and preferred spectacles). Figures 1 and 2 show the topographic images of the right and left eye, with right eye CDVA improving to 6/6 and left eye at presentation. The fellow eye was completely normal with normal topography and pachymetry. On topography (Pentacam, OCULUS Optikgeräte GmbH), the right eye showed the crab claw sign with against-the-rule astigmatism of −12.00 D at 65 degrees, and K1 (flat keratometry) and K2 (steep keratometry) were 37.90 D at 60 degrees and 51.10 D at 165 degrees, respectively. Fellow eye uncorrected distance visual acuity was 6/6 and normal on clinical examination. On examination, a crescentric arcuate irregular reflex 2.0 mm away from the limbus extending from the 4 o'clock to 8 o'clock position was noted. CASE REPORTĪ 64-year-old man presented to the clinic with blurring of vision in the right eye and was found to have an uncorrected distance visual acuity of 3/60 and a corrected distance visual acuity (CDVA) of 6/18 with a −12.00 diopter (D) cylinder axis at 65 degrees. 2 Also, only very few cases of hydrops and acute spontaneous perforation have been described in the literature. 1 Unlike keratoconus, the incidence of acute hydrops is rare, and no clear-cut modality of treatment has been described for the same. Pellucid marginal degeneration (PMD) is a rare corneal ectatic disorder that is common in men in the fourth to fifth decade, which presents with bilateral and often asymmetrical peripheral corneal thinning in a crescentic manner with high against-the-rule astigmatism.
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