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Healed Corneal Ulcer with Keloid Formation

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Healed Corneal Ulcer with Keloid Formation Saudi Journal of Ophthalmology (2012) 26, 245–248 Case Report Healed corneal ulcer with keloid formation ⇑ Hind M. Alkatan, MD a, ; Khalid M. Al-Arfaj, MD c; Mohammed Hantera, MD d; Soliman Al-Kharashi, MD b Abstract We are reporting a 34-year-old Arabic white female patient who presented with a white mass covering her left cornea following multiple ocular surgeries and healed corneal ulcer. The lesion obscured further view of the iris, pupil and lens. The patient under- went penetrating keratoplasty and the histopathologic study of the left corneal button showed epithelial hyperplasia, absent Bow- man’s layer and subepithelial fibrovascular proliferation. The histopathologic appearance was suggestive of a corneal keloid which was supported by further ultrastructural study. The corneal graft remained clear 6 months after surgery and the patient was sat- isfied with the visual outcome. Penetrating keratoplasty may be an effective surgical option for corneal keloids in young adult patients. Keywords: Corneal mass, Histopathology, Keloid, Penetrating keratoplasty Ó 2012 Saudi Ophthalmological Society, King Saud University. All rights reserved. doi:10.1016/j.sjopt.2011.10.005 Introduction segment has been often unsuccessful.7 In extreme cases, the eyes were eventually enucleated due to spontaneous corneal Keloids and hypertrophic scars are fibrous tissue out- perforation or buphthalmos.8 We describe a case of corneal growths that result from a deviation from normal wound- keloid after healed corneal ulcer which was successfully man- healing process and were first described in 1865.1 Clinically, aged by penetrating keratoplasty. The clinical, histopatho- corneal keloids appear as gray–white elevated masses dif- logic, and ultrastructural findings are all presented. fusely involving the entire stroma or as localized solitary nod- ules.2–4 There have been approximately 76 documented cases of corneal keloid previously reported.5 Most reported Case report cases have occurred secondary to penetrating injury or sur- gery, and it is thought that inflammation causes a fibroblastic A white 34 year old female patient presented with a white reaction resulting in the keloid,4 others have developed after mass covering her left cornea, and was unable to close her small pox, rubella6 or can arise de-novo.5 left eye (Fig. 1a and b). Her ophthalmic history revealed com- Keloids are usually unilateral, but can be bilateral if associ- plicated cataract surgery (phacoemulsification with intraocu- ated with genetic syndromes. Surgical results of congenital lar lens implantation) 9 years prior to her presentation. Her corneal keloids have been very poor and in spite of repeated cataract was secondary to Fuchs uveitis. This was followed penetrating keratoplasties, the reconstruction of the anterior one year later, by intractable glaucoma which was controlled Received 31 August 2011; accepted 25 October 2011; available online 6 January 2012. a Pathology & Laboratory Medicine Department, King Khaled Eye Specialist Hospital, Riyadh, Saudi Arabia b Anterior Segment Division, King Khaled Eye Specialist Hospital, Riyadh, Saudi Arabia c Ophthalmology Department, Dammam University, Saudi Arabia d Albatal Center, Jeddah, Saudi Arabia ⇑ Corresponding author. Address: King Khaled Eye Specialist Hospital, P.O. Box 7191, Riyadh 11462, Saudi Arabia. Tel.: +966 14821234; fax: +966 14821234x3131. e-mail address: [email protected] (H.M. Alkatan). Peer review under responsibility Production and hosting by Elsevier of Saudi Ophthalmological Society, Access this article online: www.saudiophthaljournal.com King Saud University j www.sciencedirect.com 246 H.M. Alkatan et al. Figure 1. (a) The clinical appearance of the whitish lesion covering most of the left cornea. (b) Side view of the left corneal keloid. (c) Slitlamp photography showing clear corneal graft with no recurrence 6 months after surgery. (d) The post-operative clinical appearance showing peaked pupil secondary to peripheral anterior synechiae at 2 o’clock. by Trabeculectomy with Mitomycin C followed by fixation of a pars plana Ahmed valve. The patient then developed para- central sterile corneal ulcer measuring 3.00  2.50 mm involving the mid-stroma. The corneal ulcer healed after using conservative measures with residual left dense corneal scar. At the time of presentation her uncorrected visual acuity was hand motion in left eye and 20/30 in right eye. Slitlamp biomicroscopy showed large, dome shaped white non-vascu- larized mass measuring 8.00  7.50 mm, protruding from the lids with exposure keratopathy overlying the mass (fluores- cein positive staining). Applanation tonometry, anterior seg- ment examination, and dilated fundus examination were precluded by the corneal mass. The patient underwent penetrating keratoplasty in her left eye. In order to excise the entire corneal mass, large sized trephination was performed (with 9.00 mm corneal trephine) and the donor corneal graft measured 9.25 mm. This graft was secured with interrupted 10/0 sutures. The postoperative best spectacle corrected visual acuity in left eye was 20/60 with clear graft for a follow up period of 6 months (Fig. 1c). However, pupil distortion was noted due to peripheral ante- rior synechia (Fig. 1d). Half of the corneal button obtained during surgery was fixed in 3% formalin solution for light microscopy and the other half was fixed in 0.25 glutaralde- hyde for Electron Microscopy. Examination of the histologic sections showed acanthotic epithelial layer and localized sub- epithelial fibroblastic and myofibroblastic proliferation. At the base of the lesion, Bowman’s membrane was fragmented Figure 2. (a) Stromal neovascularization at the base of the lesion and the stroma showed extensive neovascularization and (periodic acid schiff, original magnification  100). (b) Vimentin stain scarring (Fig. 2a). An irregular thick Descemet’s membrane showing reactive fibroblasts (original magnification  100). Healed corneal ulcer with keloid formation 247 with normal endothelium was present. The proliferating spin- Smith1 ascribed it to inflammation, injury or surgery pro- dle cells representing fibroblasts were positively stained with ceeding such overgrowths of the cornea. From his observa- Vimentin (Fig. 2b). Few myofibroblasts showing positive tion of 27 cases, he concluded that although lesions staining with smooth muscle actin (SMA) were also present. resembling true tumors did originate from corneal tissue, it The Electron Microscopy showed mature myofibroblasts was very difficult to determine histologically whether these (Fig. 3a) displaying thin actin-like filaments under the plasma- lesions were to be classified as true fibrous tumors or merely lemma, scattered fusiform densities and focal basement as keloids. Others6 gave the term Juvenile fibromatosis’ to membrane (Fig. 2b). In addition, fibroblasts and inactive different varieties’ of fibrous tissue proliferation, which was fibrocytes were also identified (Fig. 3c). The fibroblasts found in patients below 15 years of age. In their view such tu- showed fibrillar cytoplasm (Fig. 3d). mors arose in sub-epithelial soft parts and at sites where scar tissue was known to have existed and they could hardly be Discussion differentiated from keloids. In our case, peripheral anterior synechiae at 2 o’clock was Corneal keloids and hypertrophic scars are relatively seen at time of penetrating keratoplasty (Fig. 1d), which can uncommon. They tend to occur more in children and young be explained by the occurence of silent sealed corneal perfo- adults.2,3,7,9 ration with iris prolapse complicating the corneal ulcer. This The clinical differential diagnosis includes sclerocornea, goes in line with the hypothesis by Pansons in 1904 and Fuchs dermoid, myxoma, Salzmann nodular degeneration, Peter in 1918 that corneal hypertrophic scars originate from the 10,11 anomaly, congenital hereditary endothelial dystrophy, con- stromal cells of the iris tissue. genital glaucoma, familial band-shaped keratopathy, sphe- However, others reported similar cases with no histologic roidal degeneration, squamous cell carcinoma, juvenile evidence of iris involvement thus suggesting that the patho- xanthogranuloma, fibrous histiocytoma, birth trauma, infec- genesis of such lesions is related to primary corneal repara- tion, and metabolic disease.5,9 tive activity and that it is actually derived from the corneal 12 The origin and nature of such tumors remain obscure. stroma. Most cases reported in the literature are described along Most reported cases have occurred secondary to pene- with fibrous tumors of cornea. Some researchers attributed trating injury or surgery, and it is thought that inflammation 4 them to undifferentiated hyperplasia of opaque corneal and causes a fibroblastic reaction resulting in the keloid. Mejia scleral tissue of developmental origin.6 in 2001 reported a patient with no history of previous trauma, Figure 3. (a) Electron Micrograph of mature myofibroblasts (original magnification  4900). (b) Electron Micrograph of myofibroblast showing numerous thin filaments and fusiform densities (original magnification  24,000). (c) Electron Micrograph of inactive fibrocyte (original magnification  3300). (d) Electron Micrograph of fibroblast with fibrillar cytoplasm (original magnification  18,500). 248 H.M. Alkatan et al. but had exacerbation in the growth of bilateral corneal keloid One of the drawbacks in the management of our case, is after superficial keratectomy.8 The present case reported
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