Severe Chronic Progressive External Ophthalmoplegia-Associated Ptosis Successfully Treated With Scleral Lenses

Clinical Correspondence Section Editors: Robert Avery, DO Karl C. Golnik, MD Caroline Froment, MD, PhD An-Gour Wang, MD Severe Chronic Progressive External Ophthalmoplegia– Associated Ptosis Successfully Treated With Scleral Lenses Christina Cherny, BS, Suzanne W. Sherman, OD, Lora R. Dagi Glass, MD C hronic progressive external ophthalmoplegia (CPEO) is a progressive neuromyopathic disorder marked by bilateral weakness of the levator and extraocular muscles, resulting in blepharoptosis and impaired ocular motility (1,2). CPEO plus refers to a syndrome of CPEO associated with systemic clinical manifestations, including myopathies and neuropathies (3). Treatment of ptosis related to the CPEO spectrum is challenging due to the progressive nature of the disorder, poor levator function, and high risk of exposure keratopathy. For this reason, surgical treatment is delayed as long as reasonably possible, and patient expectations are tempered. Medical treatment has typically included observation and ptosis crutches, with some reports of scleral lens use in moderate cases (4–6). We present a case of severe bilateral ptosis due to CPEO plus successfully treated with scleral lenses, allowing for both corneal protection and deferred surgical treatment. A 28-year-old woman presented with a history of bilateral ptosis for 17 years secondary to CPEO plus—specifically, Kearns–Sayre syndrome—for which she attested to constant brow elevation and chin lift for subjective visual field improvement. Ophthalmic history was significant for constant dry eye sensation and intolerance of ptosis crutches due to secondary keratopathy. The patient’s medical history was significant for Kearns–Sayre syndrome, dysautonomia, gastrointestinal pathology including esophageal spasms and gastroparesis, complex regional pain syndrome, and erythromelalgia. The patient also reported a confirmed allergy to adhesives, including acrylic polymers. On presenting oculoplastic examination, visual acuity was 20/25 and 20/20 (right eye and left eye, respectively). External examination was notable for complete pupillary reflex coverage bilaterally (both eyes), with the upper lid falling 1.5 mm below the pupillary reflex in both eyes at best with concentrated brow lifting. The interpalpebral fissure (IPF) was 3.5 mm in the right eye and 4 mm in the left eye when the brows were relaxed (Fig. 1). Levator function of 2 mm up to 3 mm with significant effort was noted in both eyes, and there was no lagophthalmos in both eyes. Extraocular movements demonstrated inability to move in any field of gaze, and no Bell phenomenon was present. Upon lifting of the lids, confrontational visual fields were full, although the patient did note diplopia. Intraocular pressure was 19 mm Hg in both eyes. Slit-lamp examination was notable for decreased tear breakup time. A dilated fundus examination indicated a salt and pepper fundus with retinal nerve fiber layer thinning in both eyes. The patient was not an ideal surgical candidate, given her inability to tolerate ptosis crutches due to keratopathy, diplopia with lid lifting, and young age with a significant likelihood of multiple ptosis procedures in the future. For this reason, on discussion with the optometric specialty lens service, a decision to trial large-diameter scleral lenses was undertaken. Given the patient’s acrylic polymer allergy (from which all rigid gas-permeable lenses are currently crafted), glass contact lenses were considered. However, these were ultimately rejected due to low oxygen permeability in a patient with chronic needs. BostonSight Scleral (BSS) lenses, which are made with a derivative of acrylic polymers known as fluorosilicone acrylate, were considered next due to their high oxygen permeability of 100 and wetting angle of 6. BSS lenses were chosen for an initial fit, with parameters of +0.25 D, 18.5 mm diameter, 8.00 mm base curve, 0.40 mm center thickness, standard FSE1 B-R85-5e1 in the right eye, and +0.25 D, 18.5 mm diameter, 8.00 mm base curve, 0.41 mm center thickness, standard FSE1 B-L85-5e1 in the left eye. The patient was offered the option to incorporate her vision correction into the scleral lens parameters; however, she preferred to continue wearing her spectacles due to habit and cosmetic concerns. Full State University of New York College of Optometry (CC), New York, New York; and Department of Ophthalmology (SWS, LRDG), Columbia University Irving Medical Center, New York, New York. The Department of Ophthalmology at Columbia University Irving Medical Center is funded by a nonrestricted grant from Research to Prevent Blindness. The authors report no conflicts of interest. Address correspondence to Lora R. Dagi Glass, MD, Edward S. Harkness Eye Institute, 635 W. 165th Street, PO Box 77, New York, NY 10033; E-mail: [e-mail] Cherny et al: J Neuro-Ophthalmol 2021; 41: e189-e191 FIG. 1. Patient without scleral lenses in place. e189 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Clinical Correspondence examination. She has remained comfortable with 5 months of follow-up. Although surgical correction is available for both the cosmetic and functional aspects of bilateral ptosis, surgical procedures are more challenging in patients with poor levator function, such as in the case of CPEO, with preoperative discussions and postoperative follow-up revolving around exposure keratopathy and often imperfect cosmesis (4,5). Scleral lenses, both with and without “shelf” modifications for lid positioning known as ptosis props, have previously been suggested as an alternative to ptosis surgery due to their ability to provide mechanical eyelid elevation, protect the ocular surface, and correct vision (6–10). Katsoulos et al (6) reported trying high-vault scleral lenses in a pediatric patient with similar clinical findings secondary to Kearns–Sayre syndrome, albeit with a smaller scleral lens diameter and a less severe ptosis, to determine efficacy in eyelid elevation. Increased upper eyelid support and a slightly widened palpebral aperture were noted, with the patient reporting subjectively better vision due to increased eyelid lifting (6). However, due to the medical team’s dissatisfaction with the outcome and the patient’s difficulty with lens manipulation, the patient and her family opted for surgical correction instead (6). Lindsay et al (7) reported successful use of larger-diameter scleral lenses in a patient with progressive bilateral ptosis FIG. 2. Patient with scleral lenses in place. insurance coverage was obtained due to her diagnosis of dry eye. Adequate fit was observed in each eye; edges showed a flush landing with no signs of compression and 300 mm of central clearance was noted, which contributed to the vault of the lens and thus aided with eyelid elevation. Using the scleral lenses, her margin reflex distance (MRD1) was noted to be 0.5 mm in both eyes, and IPF increased to 6.5 mm in both eyes (Fig. 2). A decision was made to continue with BSS lenses and to monitor closely for evidence of allergic signs and symptoms. In subsequent follow-up visits, the patient denied experiencing any allergic symptoms, despite 8–9 hours of daily use. She noted significant improvement in dryness and day-to-day sensitivity, overall confidence, and quality of life. Visual acuity remains excellent at 20/25 in both eyes and there is no evidence of papillae or hyperemia on slit-lamp TABLE 1. Summary of reported scleral lens usage for ptosis secondary to progressive external ophthalmoplegia Outcomes of Scleral Lens Usage for Ptosis Secondary to CPEO Age Sex History of prior eyelid surgery Scleral lens used Report 1 Report 26 Report 37 Report 48 Report 58 Report 69 28 F No 10 F No 43 F Yes 41 F Yes 42 F Yes 51 M Undisclosed BostonSight Scleral 18.5 Epsilon V3 Boston Equalens II with ptosis prop 21.0 PROSE PROSE Undisclosed Scleral lens diameter (mm) Length of successful lens 4 mo wear at publication Baseline IPF (mm) 3.5 OD, 4 OS Post-lens IPF (mm) Improvement in IPF (mm) Baseline MRD1 (mm) Post-lens MRD1 (mm) Improvement in MRD1 (mm) 6.5 OD, 6.5 OS 3.0 OD, 2.5 OS 21.5 OD, 21.5 OS 0.5 OD, 0.5 OS 2.0 OD, 2.0 OS 16.5 Lens wear discontinued 5.0 OD, 4.0 OS 6.0 OD, 5.0 OS 2.0 OD, 1.0 OS 0.5 OD, 0.0 OS 1.5 OD, 1.0 OS 1.0 OD, 1.0 OS .2 wk Undisclosed Undisclosed Undisclosed parameters parameters parameters 3 yr 1.5 yr Undisclosed ,5 OD, ,5 OS Undisclosed Undisclosed Undisclosed Undisclosed Undisclosed Undisclosed Undisclosed Undisclosed Undisclosed Undisclosed Undisclosed Undisclosed Undisclosed Undisclosed Undisclosed Undisclosed Undisclosed Undisclosed Undisclosed Undisclosed 1.0 OD, 1.0 OS 1.5 OD, 0.5 OS Undisclosed The present case is termed “Report 1.” CPEO, chronic progressive external ophthalmoplegia; IPF, interpalpebral fissure; MRD1, margin reflex distance; OD, right eye; OS, left eye. e190 Cherny et al: J Neuro-Ophthalmol 2021; 41: e189-e191 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Clinical Correspondence secondary to ocular myopathy; however, their report differed from the present case in their incorporation of ptosis props into the scleral lenses and in the patient’s extensive history of eyelid surgery; it is unclear if this is specifically a case of CPEO. Similarly, Scofield-Kaplan et al reported on 2 cases of bilateral ptosis secondary to CPEO with histories of surgical eyelid repair where treatment with PROSE scleral lenses was sufficient to obviate further surgical procedures (Table 1) (8). To date, there have been no reports of sustained, successful use of large-diameter scleral lenses in cases of severe bilateral ptosis secondary to CPEO without preceding history of surgical eyelid repairs. Our case report indicates that the use of large-diameter scleral lenses with increased vault may be a viable option for patients with CPEO and severe ptosis who are unable to tolerate ptosis crutches, particularly when surgical correction is hoping to be deferred, despite known acrylic polymer allergy. STATEMENT OF AUTHORSHIP Category 1: a. Conception and design: S. W. Sherman; b. Acquisition of data: S. W. Sherman and L.R. Dagi Glass; c. Analysis and interpretation of data: S. W. Sherman, L.R. Dagi Glass, and C. Cherny. Category 2: a. Drafting the manuscript: C. Cherny, L.R. Dagi Glass, and S. W. Sherman; b. Revising it for intellectual content: C. Cherny, L.R. Dagi Glass, and S. W. Sherman. Category 3: a. Final approval of the completed manuscript: C. Cherny, L.R. Dagi Glass, and S. W. Sherman. Cherny et al: J Neuro-Ophthalmol 2021; 41: e189-e191 REFERENCES 1. Bucelli RC, Lee MS, McClelland CM. Chronic progressive external ophthalmoplegia in the absence of ptosis. J Neuroophthalmol. 2016;36:270–274. 2. Soejima K, Sakurai H, Nozaki M, Fujiwara O, Masuda M, Yamada H, Shimizu J. Surgical treatment of blepharoptosis caused by chronic progressive external ophthalmoplegia. Ann Plast Surg. 2006;56:439–442. 3. Sundaram C, Meena AK, Uppin MS, Govindaraj P, Vanniarajan A, Thangaraj K, Kaul S, Kekunnaya R, Murthy JMK. Contribution of muscle biopsy and genetics to the diagnosis of chronic progressive external ophthalmoplegia of mitochondrial origin. J Clin Neurosci. 2011;18:535–538. 4. Finsterer J. Ptosis: causes, presentation, and management. Aesth Plast Surg. 2003;27:193–204. 5. Kwitko GM, Badri T, Patel BC. Blepharoplasty ptosis surgery. [StatPearls web site]. March 10, 2019. Available at: https:// www.ncbi.nlm.nih.gov/books/NBK482296/. Accessed October 2, 2019. 6. Katsoulos K, Livir Rallatos G, Mavrikakis I. Scleral contact lenses for the management of complicated ptosis. Orbit. 2018;37:201–207. 7. Lindsay RG, Ezekiel DF. Ptosis prop gas permeable scleral lens fitting for a patient with ocular myopathy. Clin Exp Optom. 1997;80:123–126. 8. Scofield-Kaplan SM, Dunbar KE, Campbell AA, Kazim M. Utility of PROSE device in the management of complex oculoplastic pathology. Ophthalmic Plast Reconstr Surg. 2018;34:242– 245. 9. Lane CM, Collin JRO. Treatment of ptosis in chronic progressive external ophthalmoplegia. Br J Ophthalmol. 1987;71:290–294. 10. Shah-Desai SD, Aslam SA, Pullum K, Beaconsfield M, Rose GE. Scleral contact lens usage in patients with complex blepharoptosis. Ophthal Plast Reconstr Surg. 2011;27:95–98. e191 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited.