{'fraction_non_alphanumeric': 0.029647630619684083, 'fraction_numerical': 0.015795868772782502, 'mean_word_length': 5.217522658610272, 'pattern_counts': {'":': 0, '<': 0, '<?xml version=': 0, '>': 0, 'https://': 0, 'lorem ipsum': 0, 'www.': 0, 'xml': 0}, 'pii_count': 0, 'substrings_counts': 0, 'word_list_counts': {'cursed_substrings.json': 0, 'profanity_word_list.json': 0, 'sexual_word_list.json': 0, 'zh_pornsignals.json': 0}}
Severe Chronic Progressive External Ophthalmoplegia-Associated Ptosis Successfully Treated With Scleral Lenses.

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