Ocular manifestations and management recommendations of lysosomal storage disorders I: mucopolysaccharidoses

Carlton R Fenzl,1 Kyla Teramoto,2 Majid Moshirfar3 1John A Moran Eye Center, University of Utah, Salt Lake City, UT, USA; 2John A Burns School of Medicine, University of Hawai’i, Honolulu, HI, USA; 3Cornea and Refractive Surgery Division, Department of Ophthalmology, Francis I. Proctor Foundation, University of California, San Francisco, CA, USA Abstract: The mucopolysaccharidoses (MPS) are a group of lysosomal storage disorders caused by inborn errors of glycosaminoglycan (GAG) metabolism. These diseases are classified by enzyme deficiency into seven groups: type I, II, III, IV, VI, VII, and IX. GAG accumulation leads to characteristic clinical features. Some ophthalmic findings that are characteristic of MPS diseases include corneal clouding, retinal degeneration, decreased electroretinogram wave amplitude, optic atrophy, papilledema, and glaucoma. Current treatments such as hematopoietic stem cell transplantation and enzyme replacement therapy have increased the life span of many MPS patients and created the need to improve management of ocular symptoms. This article aims to provide a comprehensive review of ocular manifestations and treatment options for the various types of MPS. Keywords: MPS, glycosaminoglycan, corneal cloudin

Use of infant donor tissue in endothelial keratoplasty

Majid Moshirfar,1 Yichieh Shiuey,2 Carlton Robert Fenzl3 1Department of Ophthalmology, Francis I Proctor Foundation, University of California San Francisco, San Francisco, CA, USA; 2Palo Alto Medical Foundation, Sunnyvale, CA, USA; 3John A Moran Eye Center, University of Utah, Salt Lake City, UT, USA We have read with great interest the recent article by Kobayashi et al describing endothelial keratoplasty using infant donor tissue.1 This is a fine case report describing the use of infant donor tissue as a viable source when performing non-Descemet stripping automated endothelial keratoplasty (nDSAEK). Descemet stripping automated endothelial keratoplasty (DSAEK) and nDSAEK seem to be the only currently accepted forms of keratoplasty for which donor tissue under the age of 2 years is acceptable. When used to perform penetrating keratoplasty, it is known that infantile grafts behave in an ectatic fashion.2,3 This is likely due to their more elastic properties. Tissue preparation in Descemet membrane endothelial keratoplasty (DMEK) proves to be too difficult given the strength of adhesion of Descemet membrane to the overlying stroma.1 Read the original article&nbsp

What we know about ocular manifestations of Ebola

Majid Moshirfar,1 Carlton R Fenzl,2 Zhan Li3 1Department of Ophthalmology, Francis I Proctor Foundation, University of California San Francisco, San Francisco, CA, USA; 2John A Moran Eye Center, University of Utah, Salt Lake City, UT, USA; 3University of Nevada School of Medicine, Reno, NV, USA Abstract: Ebola hemorrhagic fever is a deadly disease caused by several species of ebolavirus. The current outbreak of 2014 is unique in that it has affected a greater number of people than ever before. It also has an unusual geographic distribution. Nonspecific findings such as fever and generalized weakness have traditionally been very common early in the acute phase. Ophthalmic manifestations have also been reported in significant numbers. Conjunctival injection has been identified in both the acute and late phases. Subconjunctival hemorrhage and excessive lacrimation have also been reported. Various forms of uveitis have been associated with the convalescent phase of the disease. When identified in conjunction with other signs such as fever, acute findings such as conjunctivitis may contribute to the diagnosis of Ebola hemorrhagic fever. Ideally, serologic testing should be performed prior to isolation and treatment of these individuals. Considering the prevalence of the current outbreak and the threat of transcontinental spread, ophthalmic health professionals need to be aware of the ocular manifestations of Ebola hemorrhagic fever as well as the associated signs and symptoms in order to prevent further spread. Keywords: conjunctivitis, ebolavirus, subconjunctival hemorrhage, uveitis, Ebola hemorrhagic feve

Accuracy of corneal trephination depth using the Moria single-use adjustable depth vacuum trephine system

Carlton R Fenzl,1 Adam J Gess,2 Majid Moshirfar31John A Moran Eye Center, University of Utah, Salt Lake City, UT, 2Eye Doctors of Washington, Washington, DC, 3Cornea and Refractive Surgery Division, Department of Ophthalmology, Francis I Proctor Foundation, University of California San Francisco, San Francisco, CA, USABackground: The Moria single-use adjustable depth trephine is a device that allows a goal trephination depth to be set prior to the surgical procedure.Methods: Eleven fresh human cadaveric eyes were trephined using 8.0 mm Moria single-use adjustable vacuum trephines. Prior to trephination, the average corneal pachymetry in the peripheral 7–10 mm range was obtained using anterior segment optical coherence tomography. The trephination depth was set to 80% of that value. Light microscopy was used to image anteroposterior cross-sections of each corneal specimen. Digital protractor software was used to evaluate the trephination angle, depth, and length. All adequately processed specimens were included in the analysis. In addition, trephination angle data from a previous publication by Moshirfar et al were used as a comparison with those of this study.Results: Trephination analysis of depth compared with pachymetry revealed a mean of 83.7%±6.53% (95% confidence interval 79.8–87.6). Maximum and minimum trephined depths were 95.35% and 71.3%, respectively. Trephination depth compared with angular corneal thickness yielded a mean of 66.2%±4.79% (95% confidence interval 63.0–69.4). Maximum and minimum depths were 73.7% and 59.7%, respectively. Analysis of trephination angle yielded a mean of 130.2±3.57 degrees (95% confidence interval 127.8–132.61). Maximum and minimum angles were 135.5 degrees and 126 degrees, respectively. The standard deviation of the trephination angle of the Moria trephine was found to be significantly less than that of Hessburg-Barron and Hanna trephines calculated in the previous study.Conclusion: The Moria adjustable vacuum trephine is an accurate method of trephination when a specific depth is desired. Further investigation is needed to determine the relevance of this in relation to deep anterior lamellar keratoplasty.Keywords: trephine, Moria, deep anterior lamellar keratoplasty, adjustable depth, vacuu

Delayed manifestation of bilateral scleral thinning after I-BRITE® procedure and review of literature for cosmetic eye-whitening procedures

Majid Moshirfar,1 Michael V McCaughey,2 Carlton R Fenzl,3 Luis Santiago-Caban,4 Gregory D Kramer,3 Nick Mamalis3 1Department of Ophthalmology, Francis I Proctor Foundation, University of California San Francisco, San Francisco, CA, USA; 2University of New Mexico School of Medicine, Albuquerque, NM, USA; 3John A Moran Eye Center, University of Utah, Salt Lake City, UT, USA; 4Department of Ophthalmology, University of Puerto Rico School of Medicine, San Juan, PR, USA Purpose: To report a case of delayed-onset bilateral scleral thinning and calcium deposition following a cosmetic ocular-whitening procedure (I-BRITE®).Methods: A 33-year-old male patient with a history of right-sided ptosis repair and left-sided anterior uveitis had previously undergone bilateral I-BRITE treatment for chronic conjunctival hyperemia. Four years after the procedure, the patient was referred to our institution with bilateral scleral thinning and overlying calcific depositions. A literature review was performed through PubMed from 1980 through 2014 using the search terms ‘cosmetic’, ‘ocular’, ‘conjunctivectomy’, ‘regional conjunctivectomy’, ‘I-BRITE’, ‘eye-whitening’, ‘scleritis’, ‘necrotizing scleritis’, ‘anterior uveitis’, ‘mitomycin C’, ‘5-fluorouracil’, and ‘bevacizumab’, along with associated cross-referencing from relevant articles.Results: Examination of the patient revealed bilateral necrotizing scleritis within the nasal region of both eyes. Calcified plaques were also present within the areas of scleromalacia, along with epithelial defects demonstrated with fluorescein staining. Although evidence of previous intraocular inflammation was apparent within the left eye, there were no active signs of inflammation evident within either eye on initial presentation. Complication rates reported in the literature include: scleral thinning (1.8%), calcific plaque formation (2.9%), fibrovascular proliferation (13%), diplopia (1.2%), elevation of intraocular pressure (4.2%), and recurrence of conjunctival hyperemia (2.1%).Conclusion: Cosmetic ocular whitening procedures have an attendant high complication rate, and have been associated with several adverse postoperative complications, which have in turn generated several reservations regarding the veritable benefit of the procedure. Many postsurgical complications may demonstrate delayed apparition, varying from several months to several years after primary surgical intervention as in the case reported here. Keywords: necrotizing scleritis, scleromalacia, cosmetic ocular whitenin

Corneal biomechanical properties after laser-assisted in situ keratomileusis and photorefractive keratectomy

Eileen S Hwang,1 Brian C Stagg,1 Russell Swan,1 Carlton R Fenzl,1 Molly McFadden,2 Valliammai Muthappan,1 Luis Santiago-Caban,1 Mark D Mifflin,1 Majid Moshirfar1,3 1Department of Ophthalmology and Visual Sciences, John A. Moran Eye Center, 2Department of Internal Medicine, University of Utah, Salt Lake City, 3HDR Research Center, Hoopes Vision, Draper, UT, USA Background: The purpose of this study was to evaluate the effects of laser-assisted in situ keratomileusis (LASIK) and photorefractive keratectomy (PRK) on corneal biomechanical properties.Methods: We used the ocular response analyzer to measure corneal hysteresis (CH) and corneal resistance factor (CRF) before and after refractive surgery.Results: In all, 230 eyes underwent LASIK and 115 eyes underwent PRK without mitomycin C (MMC). Both procedures decreased CH and CRF from baseline. When MMC was used after PRK in 20 eyes, it resulted in lower corneal biomechanical properties at 3 months when compared to the other procedures, but all three procedures had similar values at 12 months.Conclusion: Significant but similar decreases in corneal biomechanical properties after LASIK, PRK without MMC, and PRK with MMC were noted. Keywords: corneal biomechanics, photorefractive keratectomy, laser-assisted in situ keratomileusis, corneal hysteresis, corneal resistance factor, mitomycin