Acute Hydrops following PK • Baradaran-Rafiee et al Iranian Journal of Ophthalmology • Volume 20 • Number 4 • 2008

Acute Hydrops following Penetrating Keratoplasty in a Keratoconic Patient

Alireza Baradaran-Rafiee, MD1 • Manijeh Mahdavi, MD2 • Sepehr Feizi, MD3

Abstract

Purpose: To report a case with history of penetrating keratoplasty (PK) for that developed acute hydrops in the recipient and donor Methods: A 46-year-old man, with history of bilateral keratoconus, who had undergone corneal transplantation in his left eye, presented with complaints of sudden visual reduction, , redness and pain of the left eye. Results: Review of his clinical course, slit-lamp biomicroscopy, laboratory evaluations including confocal microscopy and ultrasound biomicroscopy revealed acute hydrops in the graft. Second corneal transplantation was done for his left eye and pathologic examination confirmed the diagnosis. Conclusion: Acute hydrops can occur after PK in patients with keratoconus. Although this condition is not common, it should be considered as a differential diagnosis of graft rejection.

Keywords: Acute Hydrops, Keratoconus, Corneal Transplantation

Iranian Journal of Ophthalmology 2008;20(4):49-53

Introduction Keratoconus is a degenerative, One of the complications of advanced non-inflammatory disease of the cornea, with keratoconus is acute hydrops. Affected onset generally at puberty. It is progressive in patients suffer from acute visual loss with pain 20% of cases and can be treated by lamellar and photophobia. On slit-lamp examination, or penetrating keratoplasty (PK). Its incidence conjunctival hyperemia and diffuse corneal in general population is reported to be about edema with intrastromal cystic spaces are 1/2000.1 Changes in corneal collagen visible. It is due to rupture of the endothelial structure, organization, and intercellular layer and Descemet’s membrane allowing matrix, and apoptosis and necrosis of aqueous to enter the stroma and producing keratocytes which Archiveprevalently or exclusively ofmarked stromalSID edema. Corneal edema may involve the central anterior stroma and the last weeks to months and gradual Bowman layer, are documented in the improvement is usually associated with literature. These findings that show reduction in pain and redness. Corneal scar structurally weakened corneal tissue, are formation is a late complication of acute typically seen in keratoconus.2,3 hydrops.4

1. Associate Professor of Ophthalmology, Labbafinejad Hospital, Shahid Correspondence to: Beheshti University of Medical Sciences Alireza Baradaran-Rafiee, MD Labbafinejad Hospital, Tehran 2. Assistant Professor of Ophthalmology, Yazd University of Medical Sciences Tel:+98 21 22585952 3. Assistant Professor of Ophthalmology, Ophthalmic Research Center, Email: [email protected] Labbafinejad Hospital, Shahid Beheshti University of Medical Sciences Received: January 16, 2007

Accepted: July 14, 2007

49 www.SID.ir Acute Hydrops following PK • Baradaran-Rafiee et al Iranian Journal of Ophthalmology • Volume 20 • Number 4 • 2008

Acute hydrops after corneal transplantation is rare, because cornea is usually harvested from a normal eye and extension of hydrops from recipient tissue to donor cornea is restricted by host-donor interface. Herein, we present a case of acute hydrops after PK in a keratoconic patient.

Case report A 46-year-old man, diagnosed with bilateral keratoconus, presented to us with acute visual loss, pain, redness and photophobia of his left eye. He had undergone PK for the left eye 20 years ago. During these years, he had no problem with his operated eye and his visual Figure 1. Epithelial and stromal edema of the recipient and donor cornea with inferior corneal vascularization acuity (VA) had been acceptable with glasses. One month before recent presentation, his problem had suddenly started and evolved Results within 10 to 12 hours. Asking his first surgeon, Confocal microscopy there had been no history of graft rejection Confocal microscopy (Confoscan 2, Nidek and he had not used any topical or systemic Technologies Srl, Vigouza, Italy) of the left medications. eye revealed significant intra- and intercellular At presentation, best corrected visual 20 corneal epithelial edema with diffuse and acuity (BCVA) of the right eye was /30 and of severe corneal stromal edema and multiple the left eye was counting finger at 1 m. On slit- intrastromal cystic spaces (Figure 2A). Also, lamp examination, Vogt’s striae and focal subepithelial hyperreflective areas Fleischer’s ring with corneal thinning and indicating fibrosis and stromal scar were ectasia were detected in the inferior reported (Figure 2B). A few paracentral area of the right eye indicating polymorphonuclear leukocytes were visible in keratoconus. The left eye showed diffuse the anterior stroma. No endothelial cell layer conjunctival congestion with severe corneal was detected. epithelial and stromal edema with cystic spaces that involved inferior part of the recipient cornea and a large part of the donor cornea. Also, there was superficial corneal vascularization inferiorly. There were no keratic precipitates (KPs). Because of severe and diffuse corneal stromal edema, it was not possible to evaluate anterior chamber in details (Figure 1). Intraocular pressure, Archive ofFigure 2. SIDA) Confocal microscopy shows intracellular measured by Tonopen XL instrument (Mentor, (arrowhead) and intercellular (arrow) edema with large Inc, Norwell, Massachusetts, USA), was intrastromal cleft of fluid (asterisk). B) A subepithelial 8 mmHg in the right eye and 10 mmHg in the hyper-reflective area (arrow) indicates subepithelial left eye. fibrosis. Acute hydrops involving both the recipient and donor cornea was diagnosed. The patient Ultrasound biomicroscopy received betamethasone 0.1% eye drop three A high frequency ultrasound biomicroscope times a day, homatropine 2% eye drop twice a (UBM) equipped with a 50 MHz transducer day and hypertonic sodium chloride 5% four (Paradigm UBM plus, Model p45; Paradigm times a day. Three months later, the corneal Medical Industries, Salt Lake City, Utah) edema significantly decreased, but because of clearly demonstrated separated and widened permanent corneal scar and low VA, repeated spaces within the stromal lamellae, a tear in corneal transplantation was done for the left Descemet’s membrane, and Descemet’s eye.

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Discussion membrane detachment (Figure 3). Keratoconus is the most important Subepithelial fibrotic scar and bullae were also degenerative corneal disorder that is visible. characterized by progressive corneal thinning and ectasia. Sporadic form is the most common, but positive family history has been reported in a small percentage of patients.1 It can be isolated or associated with other diseases such as Down syndrome,5 connective tissue disorders,6 mitral valve prolapse,7 atopia,8 vernal ,9 eye rubbing and use of contact lenses.1,10 Due to significant scar formation or contact intolerance, about 10% to 20% of patients need corneal transplantation.1 In spite of 5 year survival rate of 93% to 97% for corneal graft in these patients,11,12 there is a risk of graft rejection especially endothelial one. Acute hydrops is a complication of keratoconus that is caused by a tear in Descemet’s membrane and is manifested by severe and acute edema of the corneal

epithelium and stroma. Tear in Descemet’s Figure 3. Descemet’s membrane detachment (arrow) is membrane results in aqueous leakage into the explicitly shown in UBM. stroma.4 This complication, which occurs spontaneously or after trauma, is seen in 2% Histopathologic examination to 3% of the patients. The most common Histopathologic changes were focal disruption trauma is vigorous eye rubbing which is and fragmentation of Bowman's layer, which prevailing in patients with Down syndrome or was replaced in affected areas with severe ocular allergy.13,14 keratocytes and collagenous material. The Corneal edema secondary to acute epithelium itself was irregular in thickness and hydrops is usually self-limited and improves had an abnormal basement membrane in within 6 to 10 weeks leaving stromal scar. areas where Bowman's layer was destroyed Cycloplegics, topical steroids, or non-steroidal (Figure 4A). A fibrotic area and handful anti-inflammatory agents have been polymorphonuclear cells were clearly visible in suggested to treat acute hydrops. Hypertonic the central and anterior portion of the stroma sodium chloride eye drop or ointment is also (Figure 4B). In posterior stroma, another effective. Recalcitrant ones may last several fibrotic area was seen.Archive Descemet's membrane ofmonths andSID finally leave significant scar and was detached and retracted and the torn up to sixty percent of them will eventually edges rolled anteriorly (Figure 4C). need corneal transplantation. Sometimes, corneal scar is out of the visual axis and by flattening the corneal surface makes contact lens wearing more convenient.13,14 Herein, we introduced a keratoconic patient that had undergone corneal transplantation about 20 years ago and suffered from acute VA reduction secondary to acute hydrops.

Figure 4. A) Break in Bowman’s layer replaced with Confocal microscopy revealed cystic space keratocytes and collagenous material (H&E, ×250). B) that indicated intra- and intercellular epithelial Scattered polymorphonuclear infiltration within anterior and stromal edema. Since the patient was stroma and midstroma (H&E, ×100). C) Detached and referred to us after a while, some chronic retracted Descemet’s membrane with anteriorly rolled torn edges (PAS, ×10). changes including vascularization of the

51 www.SID.ir Acute Hydrops following PK • Baradaran-Rafiee et al Iranian Journal of Ophthalmology • Volume 20 • Number 4 • 2008 cornea, areas of stromal fibrosis and scar also simultaneously or immediately after tear in developed. Ultrasound biomicroscopy and Descemet’s membrane. In terms of resolving histopathologic examination explicitly edema, the presence of cleft between confirmed the detachment of Descemet’s Descemet’s membrane and corneal stroma membrane from the deep stroma. postpones membrane reattachment and Wickremasinghe et al15 reported two results in longstanding edema. Furthermore, keratoconic patients that had acute hydrops, intrastromal clefts expose large stromal 25 years after corneal transplantation. They surface to the anterior chamber and facilitate suffered from acute visual loss and corneal edema. photophobia. On slit-lamp examination, One interesting finding in our patient was stromal edema of the transplanted and corneal vascularization that apparently adjacent recipient cornea was observed. Tear developed after acute hydrops. Feder et al18 in Descemet’s membrane was not observed in pointed out the presence of the corneal these patients and they supposed that vascularization as a rare complication of acute Descemet’s membrane tear and detachment hydrops and considered hydrops adjacent to at the donor-host interface was responsible for the limbal area with intrastromal cleft as a risk edema. Also, hydrostatic pressure might lead factor for development of corneal to fluid penetration into the recipient stromal vascularization. cornea adjacent to the donor corneal stroma. Several studies19-21 reported recurrence of Thota et al16 studied on patients with acute keratoconus in transplanted cornea, even 22 hydrops secondary to keratoconus. In years after surgery. In one case of recurrent histopathologic examination, interlamellar keratoconus, acute hydrops occurred 20 years accumulation of fluid was more than after surgery.22 In our patient there was no intralamellar edema. Cornea responded to this history of recurrence of keratoconus and he fluid accumulation through formation of a had had good vision before hydrops. cellular layer around these cystic spaces. This reaction is actually the response of avascular Conclusion cornea to limit fluid dissemination and finally Although there are few reports of acute elimination of it. hydrops in transplanted cornea of keratoconic Using UBM, Nakagawa et al17 concluded patient, to the best of our knowledge, this is although the presence of tear in Descemet’s the first report of documented Descemet’s membrane is a main factor for acute hydrops membrane detachment using UBM. in keratoconic patients, it is not the single Differentiation between acute hydrops and cause and other factors including intrastromal graft rejection is very important because cleft formation are important. Intrastromal approach to these situations is completely clefts are confirmed in all patients with acute different. In acute hydrops, although stromal hydrops. They concluded that UBM is helpful scar may remain after resorption of the for detection of Descemet’s membrane tear edema, there is no place for high dose of and intrastromal clefts, especially when such steroids and the condition is self-limited. tears can not beArchive detected by slit-lamp of SID biomicroscopy. These clefts may happen

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