Peripheral Hypertrophic Subepithelial Corneal Degeneration

Gore, Daniel M.; Iovieno, Alfonso; Connell, Benjamin; Alexander, Robert; Meligonis, George; Dart, John K.G.

doi:10.1016/j.ophtha.2012.10.037

Cited by 27 publications

(15 citation statements)

References 10 publications

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“…It was first described in 2003 in 6 patients and they were all female patients 7 . Also, Gore and his co-authors described PHSCD ten years later in 22 patients with female predominance in 90% of the cases 2 . We think that SND is different than PHSCD or this new entity in our patients.…”

Section: Discussionmentioning

confidence: 61%

“…Symptoms of PHSCD were reported to be variable. Gore et al reported that the condition was asymptomatic in 3 (14%) patients, while symptomatic patients complained of ocular surface discomfort (OSD) in 10 (45%), reduced vision in 4 (18%) and both symptoms in 5 (23%) 2 . Another study reported reduced visual acuity in 12 of 14 patients (86%), OSD in 9 (64%) and 2 (14%) asymptomatic 1 .…”

Section: Discussionmentioning

confidence: 99%

“…Peripheral hypertrophic subepithelial corneal degeneration (PHSCD) is an idiopathic entity characterized by fibrosis between the epithelium and Bowman’s layer and causes flattening in the cornea which may lead to regular and irregular astigmatism. These patients have bilateral, fairly symmetric, peripheral, hypertrophic and subepithelial corneal opacification [1], [2]. The disorder has been rarely reported in the literature with unknown cause and share clinical and histopathological features of Salzmann’s nodular degeneration (SND) [3], [4]…”

Section: Introductionmentioning

confidence: 99%

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Successful Treatment of Corneal Opacification with Associated Thickened Epithelium by Simple Peeling: Acquired Corneal Subepithelial Hypertrophy (ACSH)

Al‐Rajhi¹,

Helmi

Alkatan

et al. 2019

Saudi Journal of Ophthalmology

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PurposeTo study clinical and histopathological findings of corneal opacification caused by thickened epithelium leading to reduced vision and topographical changes and to evaluate the outcome of its removal.MethodsTwelve patients (17 eyes) with central, paracentral or peripheral corneal opacification were reviewed to obtain their visual acuity, describe their slit lamp (SL) appearance (depth, extent and density) and document their topographic changes before and after peeling of the epithelium under SL or surgical removal under the microscope. Specimens of six cases were available for histopathological examination and immunohistochemical staining.ResultsMost of the eye opacifications were secondary to corneal procedures in 10 [Penetrating keratoplasty (PKP) in 7 for congenital glaucoma, keratoconus or adherent leukoma – usually over graft-host junction –, Photorefractive keratectomy (PRK) in 2 and Phototherapeutic keratectomy in one], chronic inflammation following trachoma or non-specific causes (3), and herpetic scar (1). Three cases were considered to be idiopathic. All cases presented with decreased vision, astigmatism or changes in topography or refraction. Their vision, clinical symptoms and topography improved after treatment. Histopathologically, all six cases shared findings that are similar to what have been described as peripheral hypertrophic subepithelial corneal degeneration (PHSCD) rather than Salzmann’s nodular degeneration. None of the cases showed inflammation or subepithelial pannus formation in the excised tissue. However, our cases did not fit into the diagnosis of PHSCD because of the location of the corneal opacification (being peripheral in 41% of the corneas, the presence of underlying primary etiologic factors in 82% of the eyes and the bilateral occurrence in 5 patients.ConclusionsMeticulous SL examination aided by corneal imaging may accurately diagnose and determine the depth of corneal opacification as a cause for reduced vision. Histopathologically, the removed tissue is similar to PHSCD, but cases differ in their clinical profile. Peeling the thickened epithelial/subepithelial tissue is curative in most patients, improves visual and clinical outcome and avoids unnecessary corneal grafting.

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Section: Discussionmentioning

confidence: 61%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Successful Treatment of Corneal Opacification with Associated Thickened Epithelium by Simple Peeling: Acquired Corneal Subepithelial Hypertrophy (ACSH)

Al‐Rajhi¹,

Helmi

Alkatan

et al. 2019

Saudi Journal of Ophthalmology

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“…Recently proposed diagnostic criteria for PHSD include perilimbal subepithelial fibrosis with thickening of the cornea, possible extension to the mid-periphery but no central cornea involvement 2,3. Superficial neovascularization up to the base of the fibrous tissue and two or more diopters of topographic astigmatism represent additional distinguishable features.…”

Section: Discussionmentioning

confidence: 99%

“…Peripheral hypertrophic subepithelial corneal degeneration (PHSD) is a clinical entity of unknown etiology, first reported in 2003,1 that more commonly needs to be differentiated from Salzmann’s nodular degeneration (SND). It is characterized by predominantly bilateral, gradually progressing, perilimbal, subepithelial corneal fibrosis with adjacent superficial neovascularization in the absence of concurrent or preceding clinically visible ocular surface inflammation 1–3. Terrien marginal degeneration and corneal intraepithelial neoplasia (CIN) can equally present with peripheral corneal opacification sharing many similarities with PHSD but otherwise exhibiting distinct clinicopathological properties.…”

Section: Introductionmentioning

confidence: 99%

<p>Peripheral hypertrophic subepithelial corneal degeneration: clinical aspects related to in vivo confocal microscopy and optical coherence tomography</p>

Gunzinger¹,

Voulgari²,

Petrović³

et al. 2019

IMCRJ

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Purpose To report the findings of anterior segment optical coherence tomography (AS-OCT) and in vivo confocal microscopy (IVCM) in two patients with peripheral hypertrophic subepithelial corneal degeneration (PHSD). Methods Case series by restrospective chart review and imaging analysis of AS-OCT and IVCM. Results Slit lamp examination of the two patients revealed a bilateral subepithelial-elevated fibrous tissue of the superior-nasal quadrant, as well as inferior-nasal in one of the patients. Best corrected visual acuity ranged from 20/25 to 20/15. AS-OCT showed continuous, homogenous, well-demarked hyperreflective subepithelial band associated with hyperreflectivity in the anterior stroma. IVCM demonstrated normal epithelial cell morphology and arrangement and a fibrous structure subepithelial and in the anterior stroma. Conclusion AS-OCT and IVCM can facilitate the diagnosis of PHSD and differentiate it from other corneal entities that present peripheral opacifications.

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Peripheral Hypertrophic Subepithelial Corneal Degeneration

2017

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A woman in her 40s with an unremarkable medical history presented with bilateral blurred vision. Visual acuity was 20/40 OU. Slitlamp examination revealed bilateral peripheral corneal fibrosis and nasal superficial vascularization resembling a pseudopterygium. Corneal topography identified irregular astigmatism (5.9 diopters [D] OD; 7.7 D OS), and optical coherence tomography showed focal hyperreflectivity as well as subepithelial nodules (Figure, A). Lesions were removed by superficial keratectomy plus intraoperative application of mitomycin C. Subsequent histopathologic evaluation demonstrated corneal changes without inflammation (Figure, B). At 3 months after surgery, the astigmatism had decreased (0.6 D OD; 1.8 D OS), while visual acuity had improved (20/20 OD; 20/25 OS).Peripheral hypertrophic subepithelial corneal degeneration is a typically bilateral, slowly progressive perilimbal disorder that, to our knowledge, has rarely been described in the literature and needs to be distinguished from Salzmann nodular degeneration. 1,2 The lack of histologic inflammation is pathognomonic, yet the cause of peripheral hypertrophic subepithelial corneal degeneration is still unknown. 3,4

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Peripheral Hypertrophic Subepithelial Corneal Degeneration

Cited by 27 publications

References 10 publications

Successful Treatment of Corneal Opacification with Associated Thickened Epithelium by Simple Peeling: Acquired Corneal Subepithelial Hypertrophy (ACSH)

Successful Treatment of Corneal Opacification with Associated Thickened Epithelium by Simple Peeling: Acquired Corneal Subepithelial Hypertrophy (ACSH)

<p>Peripheral hypertrophic subepithelial corneal degeneration: clinical aspects related to in vivo confocal microscopy and optical coherence tomography</p>

Peripheral Hypertrophic Subepithelial Corneal Degeneration

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