Evaluation of All Causes of Visual Function Loss in Children With Congenital Blepharoptosis.

PURPOSE: To evaluate the overall frequency of visual function loss in pediatric patients with congenital blepharoptosis. METHODS: This retrospective study was conducted in a tertiary center. The clinical records of patients younger than 18 years who were diagnosed as having congenital blephroptosis, underwent surgery, and had at least 1 year of postoperative follow-up were evaluated. Visual acuity results, presence of structural eye abnormalities, presence and type of amblyopia and strabismus, and refractive errors were recorded. The Mann-Whitney U test, chi-square test, and stepwise regression analysis were used for statistical analyses. RESULTS: The mean final visual acuity was 0.11 ± 0.23 logarithm of the minimum angle of resolution (logMAR) (range: 0.0 to 1.0 logMAR) at the final follow-up visit for 143 eyes of 123 patients (65 male and 58 female). In this cohort, 30 patients (24.4%) had amblyopia and 5 patients (4.1%) had visual loss related to structural eye pathology, amounting to a total of 35 patients (28.5%) with visual function loss. The mean final visual acuities of patients without amblyopia, patients with amblyopia, and patients with organic eye disorders were 0.01 ± 0.03, 0.29 ± 0.28, and 0.55 ± 0.42 logMAR, respectively. Severe blepharoptosis (≥ 4 mm) was present in 25 patients (83.3%) with amblyopia. Deprivational amblyopia was detected in 25 of 36 eyes (69.4%) with amblyopia. Independent risk factors associated with final visual acuity were presence of amblyopia at presentation (correlation coefficient [ß] ± standard error [SE] = -0.29 ± 0.04; P < .001), anisometropia (ß ± SE = -0.27 ± 0.06; P < .001), and ptosis severity (ß ± SE = -0.09 ± 0.04; P = .012). Visual impairment (visual acuity < 20/40) persisted in 11.9% of eyes at the final follow-up. CONCLUSIONS: Visual function loss was present in one-third of pediatric patients with congenital blepharoptosis. Both amblyopia and structural eye disorders contribute to visual impairment in this patient population. Structural eye pathology contributes independently to 4.1% of visual loss in this clinical setting. [J Pediatr Ophthalmol Strabismus. 2020;57(2):97-102.].

The Value of the Frontalis Suspension Procedure as a Repeat Intervention in Congenital Blepharoptosis.

PURPOSE: To evaluate the therapeutic benefits of frontalis suspension as a repeat intervention in congenital blepharoptosis. METHODS: Pediatric patients diagnosed as having congenital ptosis who had at least 2 years of postoperative follow-up were included in this retrospective study. A successful outcome was defined as a postoperative margin-reflex distance of 3 mm or greater. The chi-square, Student's t, and Mann-Whitney U tests were used in comparisons. RESULTS: Eighty-four eyes of 77 patients with a follow-up period of 8.4 ± 0.7 years were included. The initial surgery was levator resection in 29 (34.5%) eyes and frontalis suspension in 55 (65.5%) eyes. Frontalis suspension was performed for all repeat interventions (n = 20). Surgical success was achieved in 61.9% of patients with single surgery (75.9% for levator resection vs 54.5% for frontalis suspension; P = .06) and in 77.4% of patients following repeated surgeries (93.1% vs 69.1% for patients who initially underwent levator resection vs frontalis suspension, respectively; P = .012). A higher success rate was associated with better preoperative levator function (P = .01) and a higher margin-reflex distance (P = .004), and was inversely proportional to ptosis severity (P = .04). CONCLUSIONS: Frontalis suspension as a repeat intervention for congenital blepharoptosis is associated with a further increase in long-term anatomic success rates and should be considered when initial procedures fail or remain inadequate. [J Pediatr Ophthalmol Strabismus. 2017;54(5):320-323.].

The utility of margin-reflex distance in determining the type of surgical intervention for congenital blepharoptosis.

AIMS: To evaluate the utility of margin-reflex distance (MRD) as an alternative to levator function (LF) in choosing the appropriate surgical procedure for congenital blepharoptosis. SETTINGS AND DESIGN: This was a retrospective, observational study. SUBJECTS AND METHODS: Records of patients with simple (dystrophic) congenital ptosis who were operated and followed for ≥6 months postoperatively and whose outcomes were deemed as successful were evaluated in the study. Success was defined as a MRD at the last postoperative visit of ≥3 mm. In all cases, levator resection was performed when LF was >4 mm and frontalis suspension when LF was ≤4 mm. STATISTICAL ANALYSIS USED: For statistical evaluations, LF was accepted as the gold standard parameter for deciding on the surgical intervention, and the optimum cutoff point for initial MRD was determined as the point at which sensitivity and specificity was highest at the receiving operating curve for the selection of surgical procedure. RESULTS: Of one hundred and three eyes of ninety patients (44 female/46 male), levator resection was used in 44.7% and frontalis suspension in 55.3%. When the optimum cutoff point for MRD was determined as 0.5 mm, the sensitivity was 71%, specificity was 86%, and the area under the curve that represented the discriminative power of this parameter was found to be 0.826. CONCLUSION: The MRD at the cutoff point of 0.5 mm may be used as an alternative to LF to determine the type of surgical intervention in patients with congenital blepharoptosis whose LF cannot be reliably obtained in clinical evaluations.

Strabismus surgery in congenital fibrosis of the extraocular muscles: a paradigm.

BACKGROUND: Congenital fibrosis of extraocular muscles (CFEOM) is a rare group of disorders with variable phenotypes that result from aberrant innervation to the EOMs leading to synergistic vertical and/or horizontal deviations. We report our experience with the surgical management of patients with CFEOM. MATERIALS AND METHODS: We reviewed the clinical findings, the surgical management, and outcomes of 52 consecutive CFEOM patients operated by one surgeon at a university hospital setting between 1993 and 2014. Patients were divided into CFEOM1, 2, or 3 based on clinical and/or molecular genetic findings. RESULTS: Thirty-seven (71.2%) cases were bilateral and 15 (28.8%) were unilateral. Six of the bilateral cases had CFEOM2, and the rest of the patients had either CFEOM1 or CFEOM3. The median age at the first surgery was 10 (1-43) years. Twenty-five were females and 27 were males. Nineteen patients had previous strabismus and/or ptosis surgeries elsewhere. The mean number of operations at our center was 1.6 ± 0.7 (1-4). A temporary stay suture was used in eight patients and permanently in seven. Of the 40 patients with abnormal head position, 18 achieved excellent, 15 good, and seven poor outcomes and ocular alignment in primary position following the latest surgery was excellent in 19, good in 18, and poor in 14 of the patients, as defined in the "Methods" section of the paper. CONCLUSIONS: Although patients with CFEOM present significant strabismus surgical challenges because of EOM dysinnervation, fibrosis, and/or heterotopia, satisfactory alignment and improvement of the head posture can be attained in a significant proportion of patients using an individually tailored surgical approach.