Migratory Serpiginous Corneal Epitheliopathy

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Migratory Serpiginous Corneal Epitheliopathy is a unilateral corneal disease characterized by serpiginous or amoeboid-shaped elevations of the corneal epithelium. The differential diagnosis includes bacterial or viral serpiginous keratitis or ulcer. Thus, gram-stain with culture and sensitivity are essential diagnostic procedures. Management consists of lubricants and topical antibiotics, despite the absence of infectious organisms noted in the few cases reported. Corneal debridement with bandage contact lenses may be necessary if medical management fails to improve the corneal pathology.

Disease Entity

Migratory serpiginous corneal epitheliopathy (MSCE)

Disease

Migratory serpiginous corneal epitheliopathy (MSCE) was first described in a case series of four patients by Mohanty et al.[1] It is identifiable by a well-demarcated, raised epithelium taking a serpiginous or amoeboid form on the surface of the cornea. These unilateral lesions appear in various amoeboid-like shapes in an interpalpebral geographic pattern. Recurrence after debridement is common, although new lesions appear in various geographical interpalpebral locations and differ from the original lesion; thus, the term migratory.[1] Pathological samples from debrided epithelium in MSCE cases reveal no cellular atypia or dysplasia, and the conjunctiva is spared.

Etiology

Currently, the cause of MSCE is not well understood. The leading theory is that it is caused by exaggerated tissue response to trivial trauma, creating an abnormal reparative process. The serpiginous, amoeboid lesion and the accompanying corneal inflammation cause a common initial misdiagnosis of bacterial or viral serpiginous keratitis or ulcer, yet in MSCE, no infectious organism or causes have been identified. Despite the absence of infectious organisms, treatment with topical antibiotics is shown to be effective. No other possible stimuli have been identified.[1]

Risk Factors

Risk factors include young males and possibly low-impact trauma to the eye. No predisposing genetic or immunological factors have been identified thus far, including no nutritional deficiencies, ocular surgery, foreign body, contact lens wear, exposure to toxic substances, diabetes, ocular cicatricial pemphigoid, or allergic disorders.[1]

General Pathology

Histopathology of tissues obtained from debridement or corneal scrapings typically shows folded epithelial strips with full-thickness keratinizing squamous metaplasia with parakeratosis. Further findings include stomal edema dyskeratosis, intraepithelial edema with cytoplasmic ballooning, and degenerative changes. No microorganisms nor foreign bodies, atypia, or dysplasia are noted in histopathology.[1] (For histopathology photos, see Reference 1, Mohanty)

Pathophysiology

The epithelial defects seen in MSCE shown on histopathology demonstrated loose epithelial cell adherence and discontinuity between the epithelial basement membrane and Bowman’s membrane. This finding is likely due to dysfunction in cell to cell and cell to matrix adhesion. Additionally, the delayed healing and recurrent lesions signify that, in MSCE, there is likely a defect in epithelial cell migration and proliferation.[1][2]

Primary prevention

Trivial ocular trauma that initially goes unnoticed and unreported has been proposed as a potential causative event in cases of MSCE. If this is the case, primary prevention of MSCE would consist of using eye protection such as goggles or glasses to prevent ocular trauma.[1]

Diagnosis

MSCE is diagnosed clinically using a slit-lamp examination. Furthermore, MSCE is often considered a diagnosis of exclusion and requires the following information to complete the diagnosis.

History

This disease presents unilaterally in young males 14 to 31 years of age. These patients typically experience a gradual decrease in visual acuity over four to ten weeks. Sometimes patients have been treated with antiviral medications without any improvement in symptoms or changes in the visible lesion. In all reported cases, patients denied any environmental exposure and any restrictions or changes in diet. Furthermore, the patient could likely have uneventful trivial trauma to the eye that was not noticeable and went untreated directly after the event.[1]

Physical examination

In all patients described, a slit-lamp examination revealed a unilateral corneal lesion with greyish-white raised epithelium in irregular shapes and a well-demarcated border in an amoeboid pattern. Minimal subepithelial haze was noted within the border of each amoeboid pattern. Moderate conjunctival congestion and serous discharge are common signs. On slit-lamp examination, punctate fluorescein staining was noted in a serpiginous pattern in each case. It is important to note that no corneal edema, infiltrates, opacities or neovascularization was observed in the area surrounding the lesion.[1] (For clinical photos, see Reference 1, Mohanty)

Signs

Significant signs include conjunctival congestion, serous discharge, and greyish white superficially raised corneal epithelium. Signs contained to the central cornea with a clear surrounding area and no cystic changes, vascularization, epithelial or stromal edema.[1]

Symptoms

Patients present with progressive blurry vision for more than one month and as long as ten weeks prior to presentation. Other common symptoms include ocular irritation, redness, photophobia, foreign body sensation, and watering. These symptoms dissipate one to eight weeks after treatment with debridement, topical lubricants and antibacterial antibiotics, and a two-week bandage contact lens.[1]

Clinical diagnosis

The diagnosis of MSCE is guided by signs, symptoms, and diagnostic procedures, including slit-lamp examination, anterior segment ocular coherence tomography, and histopathological samples obtained from debridement. Since this is often a diagnosis of exclusion, corneal scrapings can be used for gram stain and cultures to rule out an infectious source. Furthermore, imaging is not essential for the diagnosis, but anterior segment optical coherence tomography of affected corneas has revealed elevated hyperreflective epithelial surface lesions and no stromal involvement.[1]

Diagnostic procedures

Since this is often a diagnosis of exclusion, corneal scrapings can be used for gram stain and cultures to rule out an infectious source. Furthermore, imaging is not essential for the diagnosis, but anterior segment optical coherence tomography of affected corneas has revealed elevated hyperreflective epithelial surface lesions and no stromal involvement.[1]

Laboratory test

Gram stain and culture/sensitivity are recommended to rule out possible infectious diseases.[1]

Differential diagnosis

Common corneal pathologies that could appear similar to MSCE include bacterial or viral serpiginous keratitis, bacterial or viral serpiginous corneal ulcer, advanced wavelike epitheliopathy (AWE), corneal dysplasia, carcinoma in situ, squamous cell carcinoma, hereditary benign intraepithelial dyskeratosis, corneal pannus, corneal epithelial keratinization, contact lens-induced keratopathy, superior limbic keratoconjunctivitis, or prominent epithelial basement membrane dystrophy.[3][4][5][6][7][8][9][10][11][12][13][14] Clinicians often mistake MSCE for Herpes Simplex epithelial keratitis due to the serpiginous pattern, corneal inflammation, and unfamiliarity with MSCE. This common misdiagnosis leads clinicians to treat empirically with topical or oral antivirals.[1]

Management

Management consists initially of a medical regimen of lubricants and antibiotics. Corneal debridement may be performed to remove excess tissue. The cornea must be monitored frequently and assessed whether additional corneal debridement is necessary.[1]

Medical therapy

Effective therapies for treating MSCE include topical lubricants and antibacterial antibiotics combined with corneal debridement and bandage contact lens for two or more weeks. Corneal debridement of the lesion removes the excess tissue. Following debridement, a bandage contact lens combined with lubricating drops aid in complete recovery.[1] Time to improvement ranges from one to eight weeks. Recurrence is common within one week to one month. Recurrent lesions are successfully treated with lubricants, leading to recovery without corneal scaring after 4-6 weeks. However, repeated epithelial debridement may be necessary. Of note, it has been proposed that topical antibacterial antibiotics are not indicated for most cases but may help treat stubborn recurrences. Lesions can dissipate after treatment with gatifloxacin 0.5% plus CMC 0.5%, but if no improvement is seen after two weeks, corneal debridement should be performed with the placement of a bandage contact lens. Loteprednol etabonate 0.3% for the first 15 days of treatment may also be added to decrease ocular surface inflammation.[1] Further clinical trials are needed to prove these effective empirical treatments.

Medical follow up

Recurrence following debridement and contact lens wearing is highly likely. Therefore, regular follow-up appointments each week for a month after debridement are required to monitor and treat recurrences. After one month since the last recurrence, patients can follow up with annual eye exams or as needed. [1]

Surgery

Corneal debridement is the indicated procedural therapy in patients with MSCE. Epithelial debridement promotes appropriate healing of the corneal epithelium with minimal scarring. Epithelial debridement is essential when lesions occur in the visual axis to prevent worsening long-term visual acuity deficits. A bandage contact lens should be placed after epithelial debridement to protect the healing epithelium from the microtrauma and friction resulting from blinking.[1][15][16]

Surgical follow up

After corneal debridement and bandage contact lens placement, the bandage contact lens should remain in place for two to three weeks. After this time period, bandage contact lens should be removed and the healing process observed.[1][15][16]

Complications

The known complications include corneal scarring and recurrent lesions despite aggressive treatment with corneal debridement, bandage contact lens placement, lubricant, and antibiotic eye drops.[1]

Prognosis

With treatment, the ameboid-shaped lesions commonly resolve within one month, with vision returning to baseline or 20/20 or close to it.[1]

References

  1. 1.00 1.01 1.02 1.03 1.04 1.05 1.06 1.07 1.08 1.09 1.10 1.11 1.12 1.13 1.14 1.15 1.16 1.17 1.18 1.19 1.20 1.21 1.22 Mohanty A, Mittal R, Khurana A, Chanda S, Priyadarshini S, Sahu SK. Migratory serpiginous corneal epitheliopathy (MSCE)- details of 4 cases. The Ocular Surface. 2020;18(4):742-747. doi:10.1016/J.JTOS.2020.08.002
  2. Vaidyanathan U, Hopping GC, Liu HY, et al. Persistent Corneal Epithelial Defects: A Review Article. Med Hypothesis Discov Innov Ophthalmol. 2019;8(3):163-176.
  3. D’Aversa G, Luchs JL, Fox MJ, Rosenbaum PS, Udell IJ. Advancing Wavelike Epitheliopathy. Ophthalmology. 2020;104(6):962-969. doi:10.1016/s0161-6420(97)30199-7
  4. Waring GO, Rodrigues MM, Laibson PR. Corneal dystrophies. I. Dystrophies of the epithelium, Bowman’s layer and stroma. Survey of Ophthalmology. 1978;23(2):71-122. doi:10.1016/0039-6257(78)90090-5
  5. Babu K, Narasimha Murthy KY, Ramachandra Murthy K. Wavelike epitheliopathy after phacoemulsification: Role of in vivo confocal microscopy. Cornea. 2007;26(6):747-748. doi:10.1097/ICO.0B013E31804F59F3
  6. Erdem U, Hurmeric V, Muftuoglu O, Kerimoglu H. Linear Corneal Epithelial Keratitis with a Horseshoe Pattern. http://dx.doi.org/101080/09273940701244210. 2009;15(2):131-134. doi:10.1080/09273940701244210
  7. Miller DD, Hasan SA, Simmons NL, Stewart MW. Recurrent corneal erosion: a comprehensive review. Clinical Ophthalmology (Auckland, NZ). 2019;13:325. doi:10.2147/OPTH.S157430
  8. Waring GO, Roth AM, Ekins MB. Clinical and Pathologic Description of 17 Cases of Corneal Intraepithelial Neoplasia. American Journal of Ophthalmology. 1984;97(5):547-559. doi:10.1016/0002-9394(84)90371-4
  9. Mas Tur V, AlMaazmi A, AlSaadi A, et al. Columnar keratopathy: An early manifestation of limbal stem cell deficiency. Journal of EuCornea. 2019;3(3-5):1-4. doi:10.1016/J.XJEC.2019.10.001
  10. Campbell RJ, Bourne WM. Unilateral Central Corneal Epithelial Dysplasia. Ophthalmology. 1981;88(12):1231-1238. doi:10.1016/S0161-6420(81)34867-2
  11. Reed JW, Cashwell LF, Klintworth GK. Corneal Manifestations of Hereditary Benign Intraepithelial Dyskeratosis. Archives of Ophthalmology. 1979;97(2):297-300. doi:10.1001/ARCHOPHT.1979.01020010149011
  12. Von Sallmann L, Paton D. Hereditary Benign Intraepithelial Dyskeratosis: I. Ocular Manifestations. AMA Archives of Ophthalmology. 1960;63(3):421-429. doi:10.1001/ARCHOPHT.1960.00950020423004
  13. Bloomfield SE, Jakobiec FA, Theodore FH. Contact Lens Induced Keratopathy: A Severe Complication Extending the Spectrum of Keratoconjunctivitis in Contact Lens Wearers. Ophthalmology. 1984;91(3):290-294. doi:10.1016/S0161-6420(84)34308-1
  14. Sendele DD, Kenyon KR, Mobilia EF, Rosenthal P, Steinert R, Hanninen LA. Superior Limbic Keratoconjunctivitis in Contact Lens Wearers. Ophthalmology. 1983;90(6):616-622. doi:10.1016/S0161-6420(83)34507-3
  15. 15.0 15.1 McGrath LA, Lee GA. Corneal epithelial debridement for diagnosis and therapy of ocular surface disease. Clin Exp Optom. 2015 Mar;98(2):155-9. doi: 10.1111/cxo.12213. Epub 2014 Oct 20. PMID: 25331183.
  16. 16.0 16.1 Vaidyanathan U, Hopping GC, Liu HY, et al. Persistent Corneal Epithelial Defects: A Review Article. Med Hypothesis Discov Innov Ophthalmol. 2019;8(3):163-176.
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