Purpose To describe an ophthalmic phenotype in children at relatively early stages of Wolfram syndrome. Methods Quantitative ophthalmic testing of visual acuity, color vision, automated visual field sensitivity, optic nerve pallor and cupping, and retinal nerve fiber layer (RNFL) thickness assessed by optical coherence tomography (OCT) was performed in 18 subjects 5–25 years of age. Subjects were also examined for presence or absence of afferent pupillary defects, cataracts, nystagmus, and strabismus. Results Subnormal visual acuity was detected in 89% of subjects, color vision deficits in 94%, visual field defects in 100%, optic disk pallor in 94%, abnormally large optic nerve cup:disk ratio in 33%, thinned RNFL in 100%, afferent pupillary defects in 61%, cataracts in 22%, nystagmus in 39%, and strabismus in 39% of subjects. RNFL thinning (P < 0.001), afferent pupillary defects (P = 0.01), strabismus (P = 0.04), and nystagmus (P = 0.04) were associated with more severe disease using the Wolfram United Rating Scale. Conclusions Children and adolescents with Wolfram syndrome have multiple ophthalmic markers that correlate with overall disease severity. RNFL thickness measured by OCT may be the most reliable early marker.

Allergic eyelid dermatitis is commonly related to eye drops and topical cosmetics or skin care products. Identification and elimination of causative agents is the mainstay of management. Topical corticosteroids often facilitate resolution of the associated inflammation. Therapy resistant patients may benefit from formal allergy testing.

Purpose: Timing of surgery in children with congenital ptosis is a critical component of care, and anisometropia is frequently cited as an indication for early intervention. The purpose of this study is to evaluate the change in refractive error following surgery for congenital ptosis to better inform decisions regarding the timing of surgery. Methods: A retrospective review of clinical records was performed on patients who underwent surgical correction of congenital ptosis in an academic oculoplastic surgery practice from 2002 to 2017. Patients with complete preoperative and postoperative refractive data were included in the study. Changes in refractive error following surgery were analyzed. Results: Among 184 pediatric patients who underwent ptosis surgery during the study period, 56 patients (71 eyes) met inclusion criteria. The mean age at surgery was 5.1 years. Mean refractive error change in all the operated eyes was a 0.82 D decrease in spherical equivalent (p = 0.1920) and a 0.40 D increase in cylinder (p = 0.0255). There were no statistically significant changes in spherical equivalent or cylinder in the control eyes. Conclusions: The authors data did not show movement toward normalization of refractive error following ptosis surgery. In fact, it showed a statistically significant worsening of astigmatism following surgery. Because refractive error does not improve following surgery, anisometropia should not be the sole indication for early surgery in congenital ptosis.