Clinical Review & Education
JAMA Ophthalmology Clinical Challenge
Man With Blurry Vision Lauren R. Schneider, MD; Ellen Shorter, OD; Maria S. Cortina, MD
A
B
Figure. A, Slitlamp photograph of the right eye demonstrating a paracentral neurotrophic ulcer with anterior uveitis and a hypopyon. B, Slitlamp photograph of the left eye demonstrating fluorescein staining of the corneal
persistent epithelial defect. Of note, the left eye also has reactive anterior chamber uveitis with a hypopyon.
A 53-year-old man presented with progressively worsening blurry vision of the left eye. The patient reported redness in his eye for 2 days. He denied pain, itchiness, or recent ocular trauma. He had a distant history of contact lens wear but terminated use owing to recurrent corneal abrasions. His medical history was significant for uncontrolled type 2 diabetes mellitus, leading Quiz at to bilateral below-the-knee amputations, and osteomyjamaophthalmology.com elitis. The patient was unemployed and denied cigarette smoking, alcohol use, or drug use. Current medications included insulin, nasal spray, and aspirin. Review of systems was positive for stiff joints of the hands and back pain. The patient reported that results from a workup for Crohn disease were negative. On examination, uncorrected visual acuity was 20/40 in the right eye and 20/100 in the left eye. Findings from Schirmer testing without anesthesia for 2 minutes were 20 mm OD and 15 mm OS. Corneal sensation was bilaterally decreased to a level of 2/4. There were inferior corneal epithelial defects, with significant corneal thinning bilaterally, and an inferonasal Descemetocele of the right eye. The anterior chambers possessed 3 to 4+ cells, with bilateral hypopyons (Figure). Serology was sent owing to concern regarding the patient’s joint pain, need for a Crohn disease workup, and presence of bilateral corneal thinning with anterior uveitis. Significant results included an elevated rheumatoid factor of 27 IU/mL (normal, 0-14 IU/mL), while all other laboratory results were within normal limits. Results from corneal cultures were positive for pansensitive coagulase-negative Staphylococcus, which was likely a contaminant from the eyelids.
jamaophthalmology.com
WHAT WOULD YOU DO NEXT?
A. Prescribe oral prednisone B. Place bandage contact lenses C. Perform tarsorrhaphy D. Prescribelubricationwithautologous serum tears
JAMA Ophthalmology June 2014 Volume 132, Number 6
Copyright 2014 American Medical Association. All rights reserved.
Downloaded From: http://archopht.jamanetwork.com/ by a University of Otago Library User on 11/28/2016
771
Clinical Review & Education JAMA Ophthalmology Clinical Challenge
Diagnosis Bilateral neurotrophic ulcers with keratolysis and reactive uveitis
What To Do Next B. Place bandage contact lenses Suspicion for peripheral ulcerative keratitis was low, primarily based on the nonlimbal location of the corneal thinning. The pathology findings at the lower third of the cornea suggested that the defects were related to neurotrophic and exposure risk factors. Attention was devoted primarily to the persistent epithelial defects. Soft bandage contact lenses were placed in both eyes and punctal plugs were inserted to support wetting of the ocular surface. The patient was prescribed artificial tears and polytrim for antibiotic prophylaxis because he was instructed to use the contact lenses on a continual basis. Within a week, both corneas had failed to fully epithelialize. One week after removal of the bandage contact lenses, the epithelial defects reverted to their original sizes. Doxycycline and serum tears were given; a Prokera was placed in each eye; and prednisolone acetate, 1%, 6 times daily was prescribed. The hypopyons resolved 1 week later but the corneal epithelial defects persisted. A tarsorrhaphy was considered, but the patient had relatively small palpebral fissures and the visual limitations imparted by bilateral lid closure was not a practical option for long-term corneal rehabilitation. The patient was then fit with a Prosthetic Replacement of the Ocular Surface Ecosystem (PROSE) sclera lens, which led to complete resolution of the epithelial defects within hours, as well as quiescence of the residual anterior chamber inflammation. This case represents an example of diabetic neurotrophic corneal ulcers with reactive anterior uveitis that responded rapidly to treatment with the PROSE lens.
ing, but also led to ineffective adherence of the epithelium to the underlying basement membrane. The combination of these insults led to persistent epithelial defects with robust anterior chamber reactive inflammation. Treatment was focused on promoting unhindered re-epithelialization and stabilization of the ocular surface. A reasonable initial treatment often consists of copious lubrication with artificial tears and ointments. Another approach is continuouswear contact lenses, yet standard soft contact lenses move with blinking, thus transmitting frictional forces that work against wound healing. The PROSE is a fluid-ventilated gas-permeable prosthetic device that vaults over the corneal surface.1 The lens provides constant and ample lubrication, while also protecting the cornea from repeated mechanical trauma caused by the lids during blinking.1 While patching would minimally stress fragile epithelium, oxygenation is far less superior than with the PROSE.2 Furthermore, lids possessing unfavorable characteristics, such as keratinization, may still pose a threat to the corneal surface despite a tarsorrhaphy. Amniotic membrane encouraged some clinical improvement in this patient; however, once the amnion had dissolved, there was no continued protection of the ocular surface and it subsequently broke down. It has been reported that autologous serum replaces essential tear components that promote re-epithelialization of persistent epithelial defects.3 The contribution of serum tears was still insufficient stimulization for epithelial healing in this patient. The PROSE is a successful long-term treatment approach that supports resolution of and prevention of recurrent epithelial erosions. In the setting of severe, persistent neurotrophic ulcers, which often pose a clinical challenge, the PROSE played a vital role in evading corneal perforation and also rehabilitating vision that had been compromised by corneal irregularity.
Discussion In a normal eye, epithelium will grow over a corneal abrasion with relative ease. In this patient, the presence of diabetic corneal neuropathy not only caused significant impedance to epithelial heal-
The patient continues to wear PROSE lenses daily and has maintained an intact corneal epithelium for 5 months.
ARTICLE INFORMATION
REFERENCES
Author Affiliations: Department of Ophthalmology, University of Illinois at Chicago.
1. Ling JD, Gire A, Pflugfelder SC. PROSE therapy used to minimize corneal trauma in patients with corneal epithelial defects. Am J Ophthalmol. 2013;155(4):615-619, e1-e2.
Corresponding Author: Lauren R. Schneider, MD, Department of Ophthalmology, University of Illinois at Chicago, 1855 W Taylor St, Chicago, IL 60612 ([email protected]). Conflict of Interest Disclosures: None reported.
772
Patient Outcome
3. Tsubota K, Goto E, Shimmura S, Shimazaki J. Treatment of persistent corneal epithelial defect by autologous serum application. Ophthalmology. 1999;106(10):1984-1989.
2. Rosenthal P, Cotter JM, Baum J. Treatment of persistent corneal epithelial defect with extended wear of a fluid-ventilated gas-permeable scleral contact lens. Am J Ophthalmol. 2000;130(1):33-41.
JAMA Ophthalmology June 2014 Volume 132, Number 6
Copyright 2014 American Medical Association. All rights reserved.
Downloaded From: http://archopht.jamanetwork.com/ by a University of Otago Library User on 11/28/2016
jamaophthalmology.com
JAMA Ophthalmology Clinical Challenge
Man With Blurry Vision Lauren R. Schneider, MD; Ellen Shorter, OD; Maria S. Cortina, MD
A
B
Figure. A, Slitlamp photograph of the right eye demonstrating a paracentral neurotrophic ulcer with anterior uveitis and a hypopyon. B, Slitlamp photograph of the left eye demonstrating fluorescein staining of the corneal
persistent epithelial defect. Of note, the left eye also has reactive anterior chamber uveitis with a hypopyon.
A 53-year-old man presented with progressively worsening blurry vision of the left eye. The patient reported redness in his eye for 2 days. He denied pain, itchiness, or recent ocular trauma. He had a distant history of contact lens wear but terminated use owing to recurrent corneal abrasions. His medical history was significant for uncontrolled type 2 diabetes mellitus, leading Quiz at to bilateral below-the-knee amputations, and osteomyjamaophthalmology.com elitis. The patient was unemployed and denied cigarette smoking, alcohol use, or drug use. Current medications included insulin, nasal spray, and aspirin. Review of systems was positive for stiff joints of the hands and back pain. The patient reported that results from a workup for Crohn disease were negative. On examination, uncorrected visual acuity was 20/40 in the right eye and 20/100 in the left eye. Findings from Schirmer testing without anesthesia for 2 minutes were 20 mm OD and 15 mm OS. Corneal sensation was bilaterally decreased to a level of 2/4. There were inferior corneal epithelial defects, with significant corneal thinning bilaterally, and an inferonasal Descemetocele of the right eye. The anterior chambers possessed 3 to 4+ cells, with bilateral hypopyons (Figure). Serology was sent owing to concern regarding the patient’s joint pain, need for a Crohn disease workup, and presence of bilateral corneal thinning with anterior uveitis. Significant results included an elevated rheumatoid factor of 27 IU/mL (normal, 0-14 IU/mL), while all other laboratory results were within normal limits. Results from corneal cultures were positive for pansensitive coagulase-negative Staphylococcus, which was likely a contaminant from the eyelids.
jamaophthalmology.com
WHAT WOULD YOU DO NEXT?
A. Prescribe oral prednisone B. Place bandage contact lenses C. Perform tarsorrhaphy D. Prescribelubricationwithautologous serum tears
JAMA Ophthalmology June 2014 Volume 132, Number 6
Copyright 2014 American Medical Association. All rights reserved.
Downloaded From: http://archopht.jamanetwork.com/ by a University of Otago Library User on 11/28/2016
771
Clinical Review & Education JAMA Ophthalmology Clinical Challenge
Diagnosis Bilateral neurotrophic ulcers with keratolysis and reactive uveitis
What To Do Next B. Place bandage contact lenses Suspicion for peripheral ulcerative keratitis was low, primarily based on the nonlimbal location of the corneal thinning. The pathology findings at the lower third of the cornea suggested that the defects were related to neurotrophic and exposure risk factors. Attention was devoted primarily to the persistent epithelial defects. Soft bandage contact lenses were placed in both eyes and punctal plugs were inserted to support wetting of the ocular surface. The patient was prescribed artificial tears and polytrim for antibiotic prophylaxis because he was instructed to use the contact lenses on a continual basis. Within a week, both corneas had failed to fully epithelialize. One week after removal of the bandage contact lenses, the epithelial defects reverted to their original sizes. Doxycycline and serum tears were given; a Prokera was placed in each eye; and prednisolone acetate, 1%, 6 times daily was prescribed. The hypopyons resolved 1 week later but the corneal epithelial defects persisted. A tarsorrhaphy was considered, but the patient had relatively small palpebral fissures and the visual limitations imparted by bilateral lid closure was not a practical option for long-term corneal rehabilitation. The patient was then fit with a Prosthetic Replacement of the Ocular Surface Ecosystem (PROSE) sclera lens, which led to complete resolution of the epithelial defects within hours, as well as quiescence of the residual anterior chamber inflammation. This case represents an example of diabetic neurotrophic corneal ulcers with reactive anterior uveitis that responded rapidly to treatment with the PROSE lens.
ing, but also led to ineffective adherence of the epithelium to the underlying basement membrane. The combination of these insults led to persistent epithelial defects with robust anterior chamber reactive inflammation. Treatment was focused on promoting unhindered re-epithelialization and stabilization of the ocular surface. A reasonable initial treatment often consists of copious lubrication with artificial tears and ointments. Another approach is continuouswear contact lenses, yet standard soft contact lenses move with blinking, thus transmitting frictional forces that work against wound healing. The PROSE is a fluid-ventilated gas-permeable prosthetic device that vaults over the corneal surface.1 The lens provides constant and ample lubrication, while also protecting the cornea from repeated mechanical trauma caused by the lids during blinking.1 While patching would minimally stress fragile epithelium, oxygenation is far less superior than with the PROSE.2 Furthermore, lids possessing unfavorable characteristics, such as keratinization, may still pose a threat to the corneal surface despite a tarsorrhaphy. Amniotic membrane encouraged some clinical improvement in this patient; however, once the amnion had dissolved, there was no continued protection of the ocular surface and it subsequently broke down. It has been reported that autologous serum replaces essential tear components that promote re-epithelialization of persistent epithelial defects.3 The contribution of serum tears was still insufficient stimulization for epithelial healing in this patient. The PROSE is a successful long-term treatment approach that supports resolution of and prevention of recurrent epithelial erosions. In the setting of severe, persistent neurotrophic ulcers, which often pose a clinical challenge, the PROSE played a vital role in evading corneal perforation and also rehabilitating vision that had been compromised by corneal irregularity.
Discussion In a normal eye, epithelium will grow over a corneal abrasion with relative ease. In this patient, the presence of diabetic corneal neuropathy not only caused significant impedance to epithelial heal-
The patient continues to wear PROSE lenses daily and has maintained an intact corneal epithelium for 5 months.
ARTICLE INFORMATION
REFERENCES
Author Affiliations: Department of Ophthalmology, University of Illinois at Chicago.
1. Ling JD, Gire A, Pflugfelder SC. PROSE therapy used to minimize corneal trauma in patients with corneal epithelial defects. Am J Ophthalmol. 2013;155(4):615-619, e1-e2.
Corresponding Author: Lauren R. Schneider, MD, Department of Ophthalmology, University of Illinois at Chicago, 1855 W Taylor St, Chicago, IL 60612 ([email protected]). Conflict of Interest Disclosures: None reported.
772
Patient Outcome
3. Tsubota K, Goto E, Shimmura S, Shimazaki J. Treatment of persistent corneal epithelial defect by autologous serum application. Ophthalmology. 1999;106(10):1984-1989.
2. Rosenthal P, Cotter JM, Baum J. Treatment of persistent corneal epithelial defect with extended wear of a fluid-ventilated gas-permeable scleral contact lens. Am J Ophthalmol. 2000;130(1):33-41.
JAMA Ophthalmology June 2014 Volume 132, Number 6
Copyright 2014 American Medical Association. All rights reserved.
Downloaded From: http://archopht.jamanetwork.com/ by a University of Otago Library User on 11/28/2016
jamaophthalmology.com
