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October consultation #6
CONSULTATION SECTION: CORNEA
be considered because Fuchs endothelial dystrophy usually presents at an earlier age and predominantly in women; this is especially important given this patient’s negative family ocular history. Therefore, other etiologies that present with corneal guttata, such as uveitis (chronic and silent) and infections such as herpes keratouveitis, must be considered. In addition, the patient was using lisinopril, and corneal guttata, endotheliitis, corneal erosion, as well as scarring have been reported as rare side effects associated with use of this angiotensin-converting-enzyme inhibitor antihypertensive. The next point I would consider is whether the pattern of the keratic precipitates in the endothelial rejection episodes were atypical, diffuse, or absent during different episodes of rejection (all which favor herpes keratouveitis rather than a simple endothelial rejection). I would also note that despite the lower risk for endothelial rejection in DSAEK and DMEK, this reaction has occurred repeatedly, leading to endothelial destruction and failure in the left eye. These events indicate that other factors should be considered as the probable underlying cause. Corneal perforation caused by Fuchs endothelial dystrophy, which occurred late, is rare even in a case of bacterial superinfection, which might occur in the setting of a decompensated cornea. Moreover, a nummular stromal opacity with scattered keratic precipitates can be seen in Figure 1, and the presence of a large coin-shaped precipitate and scattered keratic precipitates can be seen in Figure 2; and also a pattern of iris stromal atrophy is seen in both figures. These findings indicate the probable herpetic nature of the disease process. Corneal perforation, which occurred in the final stage, can also be explained by herpetic exacerbation leading to necrotizing keratitis. The overall diagnosis is the presence of HSV in the left eye concurrent with Fuchs endothelial dystrophy or medication-induced guttata. Herpes simplex virus has caused repeated attacks of exacerbation leading to uveitis, endotheliitis, rejection episodes, failure of DSAEK and DMEK, corneal decompensation, and finally perforation. At present, the corneal perforation, which should be managed immediately, depends on the size of the perforation and configuration of anterior chamber depth; however, this is beyond the scope of this discussion. If the perforation is small, it can be managed with tissue adhesive and a bandage contact lens. After this urgent situation is managed and the cornea becomes stable, optical PKP can be performed after 6 months. To confirm the diagnosis, I recommend performing PCR of the aqueous fluid for HSV. Oral acyclovir 400 mg 5 times per day (as a therapeutic dose) should be started. Starting the therapeutic dose of systemic acyclovir during the early postoperative period after keratoplasty is recommended to prevent acute exacerbation of disease. Also, in cases with recurrent HSV, prophylactic doses of acyclovir (400 mg twice daily) for up to 1 year is recommended.
1361
Valli Muthappan, MD Pittsburgh, Pennsylvania, USA This is a fascinating case of an elderly man who has a history of continued rejection after multiple endothelial keratoplasties in one eye. Despite escalating therapy, increasing inflammation has progressed to perforation. At this point, a wide differential diagnosis beyond immunologic rejection should be considered. Corneal melts can be associated with infectious processes, autoimmune disease, and neurotrophic causes. Regarding treatment, I would first address the urgent issue of perforation. Using cyanoacrylate glue in the office is a good temporizing measure until the underlying cause of the melt can be identified and treated. I would try to avoid an emergency PKP in this inflamed eye. Before gluing, I would swab the cornea and obtain an aqueous sample to send for bacterial cultures as well as HSV, VZV, and CMV PCRs. The patient should be screened for symptoms of an autoimmune process. He should also be checked for decreased corneal sensation and evidence of exposure keratopathy. Once a diagnosis is obtained, the underlying disease has been treated, and after the inflammation has been controlled, an optical PKP can be attempted.
Gene Kim, MD Houston, Texas, USA Looking at each episode of inflammation and corneal edema in the left eye, the differential diagnosis could be an immunologic corneal graft rejection or infectious HSV keratouveitis. Immunologic graft rejection tends to present with linear keratic precipitates (Khodadoust lines) and sectoral edema. Although the first episode of corneal edema could be attributed to an immunologic rejection, all later presentations are atypical for a corneal graft rejection. Subsequent episodes of corneal edema and inflammation occur with keratic precipitates and high IOPda combination that would point more toward the diagnosis of HSV endotheliitis and trabeculitis. The unilateral presentation of the inflammation, clustered pattern of keratic precipitates, and the large size of the keratic precipitatedindicating a granulomatous inflammationdfurther support the diagnosis of HSV keratitis. Each bout of keratouveitis causes increased endothelial cell loss and progressive corneal neuropathy, which eventually results in repeat endothelial grafts and a persistent epithelial defect that leads to corneal perforation. A test for decreased corneal sensation and PCR testing of the aqueous fluid for HSV could confirm the diagnosis. At the point of cornea perforation, the first problem that should be treated is the open globe. If the opening is small,
Volume 43 Issue 10 October 2017
be considered because Fuchs endothelial dystrophy usually presents at an earlier age and predominantly in women; this is especially important given this patient’s negative family ocular history. Therefore, other etiologies that present with corneal guttata, such as uveitis (chronic and silent) and infections such as herpes keratouveitis, must be considered. In addition, the patient was using lisinopril, and corneal guttata, endotheliitis, corneal erosion, as well as scarring have been reported as rare side effects associated with use of this angiotensin-converting-enzyme inhibitor antihypertensive. The next point I would consider is whether the pattern of the keratic precipitates in the endothelial rejection episodes were atypical, diffuse, or absent during different episodes of rejection (all which favor herpes keratouveitis rather than a simple endothelial rejection). I would also note that despite the lower risk for endothelial rejection in DSAEK and DMEK, this reaction has occurred repeatedly, leading to endothelial destruction and failure in the left eye. These events indicate that other factors should be considered as the probable underlying cause. Corneal perforation caused by Fuchs endothelial dystrophy, which occurred late, is rare even in a case of bacterial superinfection, which might occur in the setting of a decompensated cornea. Moreover, a nummular stromal opacity with scattered keratic precipitates can be seen in Figure 1, and the presence of a large coin-shaped precipitate and scattered keratic precipitates can be seen in Figure 2; and also a pattern of iris stromal atrophy is seen in both figures. These findings indicate the probable herpetic nature of the disease process. Corneal perforation, which occurred in the final stage, can also be explained by herpetic exacerbation leading to necrotizing keratitis. The overall diagnosis is the presence of HSV in the left eye concurrent with Fuchs endothelial dystrophy or medication-induced guttata. Herpes simplex virus has caused repeated attacks of exacerbation leading to uveitis, endotheliitis, rejection episodes, failure of DSAEK and DMEK, corneal decompensation, and finally perforation. At present, the corneal perforation, which should be managed immediately, depends on the size of the perforation and configuration of anterior chamber depth; however, this is beyond the scope of this discussion. If the perforation is small, it can be managed with tissue adhesive and a bandage contact lens. After this urgent situation is managed and the cornea becomes stable, optical PKP can be performed after 6 months. To confirm the diagnosis, I recommend performing PCR of the aqueous fluid for HSV. Oral acyclovir 400 mg 5 times per day (as a therapeutic dose) should be started. Starting the therapeutic dose of systemic acyclovir during the early postoperative period after keratoplasty is recommended to prevent acute exacerbation of disease. Also, in cases with recurrent HSV, prophylactic doses of acyclovir (400 mg twice daily) for up to 1 year is recommended.
1361
Valli Muthappan, MD Pittsburgh, Pennsylvania, USA This is a fascinating case of an elderly man who has a history of continued rejection after multiple endothelial keratoplasties in one eye. Despite escalating therapy, increasing inflammation has progressed to perforation. At this point, a wide differential diagnosis beyond immunologic rejection should be considered. Corneal melts can be associated with infectious processes, autoimmune disease, and neurotrophic causes. Regarding treatment, I would first address the urgent issue of perforation. Using cyanoacrylate glue in the office is a good temporizing measure until the underlying cause of the melt can be identified and treated. I would try to avoid an emergency PKP in this inflamed eye. Before gluing, I would swab the cornea and obtain an aqueous sample to send for bacterial cultures as well as HSV, VZV, and CMV PCRs. The patient should be screened for symptoms of an autoimmune process. He should also be checked for decreased corneal sensation and evidence of exposure keratopathy. Once a diagnosis is obtained, the underlying disease has been treated, and after the inflammation has been controlled, an optical PKP can be attempted.
Gene Kim, MD Houston, Texas, USA Looking at each episode of inflammation and corneal edema in the left eye, the differential diagnosis could be an immunologic corneal graft rejection or infectious HSV keratouveitis. Immunologic graft rejection tends to present with linear keratic precipitates (Khodadoust lines) and sectoral edema. Although the first episode of corneal edema could be attributed to an immunologic rejection, all later presentations are atypical for a corneal graft rejection. Subsequent episodes of corneal edema and inflammation occur with keratic precipitates and high IOPda combination that would point more toward the diagnosis of HSV endotheliitis and trabeculitis. The unilateral presentation of the inflammation, clustered pattern of keratic precipitates, and the large size of the keratic precipitatedindicating a granulomatous inflammationdfurther support the diagnosis of HSV keratitis. Each bout of keratouveitis causes increased endothelial cell loss and progressive corneal neuropathy, which eventually results in repeat endothelial grafts and a persistent epithelial defect that leads to corneal perforation. A test for decreased corneal sensation and PCR testing of the aqueous fluid for HSV could confirm the diagnosis. At the point of cornea perforation, the first problem that should be treated is the open globe. If the opening is small,
Volume 43 Issue 10 October 2017