Orbital and Eyelid Characteristics, Strabismus, and Intracranial Pressure Control in Apert Children Treated by Endoscopic Strip Craniectomy versus Fronto-Orbital Advancement.

Apert syndrome is characterized by eyelid dysmorphology, V-pattern strabismus, extraocular muscle excyclorotation, and elevated intracranial pressure (ICP). We compare eyelid characteristics, severity of V-pattern strabismus, rectus muscle excyclorotation, and ICP control in Apert syndrome patients initially treated by endoscopic strip craniectomy (ESC) at about 4 months of age versus fronto-orbital advancement (FOA) performed about 1 year of age. Methods: Twenty-five patients treated at Boston Children's Hospital met inclusion criteria for this retrospective cohort study. Primary outcomes were magnitude of palpebral fissure downslanting at 1, 3, and 5 years of age, severity of V-pattern strabismus, rectus muscle excyclorotation, and interventions to control ICP. Results: Before craniofacial repair and through 1 year of age, none of the studied parameters differed for FOA versus ESC treated patients. Palpebral fissure downslanting became statistically greater for those treated by FOA by 3 (P < 0.001) and 5 years of age (P = 0.001). Likewise, severity of palpebral fissure downslanting correlated with severity of V-pattern strabismus at 3 (P = 0.004) and 5 (P = 0.002) years of age. Palpebral fissure downslanting and rectus muscle excyclorotation were typically coexistent (P = 0.053). Secondary interventions to control ICP were required in four of 14 patients treated by ESC (primarily FOA) and in two of 11 patients initially treated by FOA (primarily third ventriculostomy) (P = 0.661). Conclusions: Apert patients initially treated by ESC had less severe palpebral fissure downslanting and V-pattern strabismus, normalizing their appearance. Thirty percent initially treated by ESC required secondary FOA to control ICP.

Impact of Adding Augmented Superior Rectus Transpositions to Medial Rectus Muscle Recessions When Treating Esotropic Moebius Syndrome.

PURPOSE: To describe outcomes after treatment of Moebius syndrome (MBS) esotropia by adjustable bilateral medial rectus recession (BMR) with and without augmented superior rectus transposition (SRT). DESIGN: Retrospective case series. METHODS: Patients meeting 2014 diagnostic criteria for MBS and treated at Boston Children's Hospital between 2003 and 2019 were identified via billing records and chart review. Visual acuity, sensorimotor evaluations, strabismus procedures, and other clinical features were recorded. Surgical outcomes for patients treated with strabismus surgery (excluding those with prior surgery elsewhere) were evaluated. The primary outcome measure was postoperative alignment comparing treatment by adjustable BMR vs adjustable BMR+SRT. RESULTS: A total of 20 patients had MBS, and 12 of these (60%) were male. Fifteen patients (75%) had primary position esotropia, and all had bilateral abduction deficit. Eight of 20 patients met inclusion criteria for primary strabismus surgery outcome. Five had undergone adjustable BMR ranging from 4.5 to 6.5 mm. Three had undergone adjustable BMR+SRT, all with 4-mm medial rectus muscle recessions. Mean preoperative esotropia before treatment by BMR was 39.5 PD (± 15 PD) with mean postoperative esotropia 9 PD (± 7.9 PD) at 6 months. Mean preoperative esotropia before treatment by BMR+SRT was 70.8 PD (± 5.9 PD) with mean postoperative esotropia 2.5 PD (± 3.5 PD) at 6 months. Significantly greater reduction in esotropia resulted from BMR+SRT than from BMR (P = .036). CONCLUSIONS: BMR proved sufficient to treat esotropia <50 PD and BMR+SRT for greater esotropia in patients with MBS-associated abduction limitation.

The Tribal Odisha Eye Disease Study (TOES) 1: prevalence and causes of visual impairment among tribal children in an urban school in Eastern India.

PURPOSE: To estimate the prevalence and causes of visual impairment and other ocular comorbidities among tribal children in an urban school population in eastern India. METHOD: In this cross-sectional study, vision screening tests were administered to tribal school children. Demographic data, including name, age, sex, home district, height, and weight of each child, and examination data, including unaided and pinhole visual acuity, external eye examination with a flashlight, slit-lamp examination, intraocular pressure (IOP) measurement, and undilated fundus photography, were collected. Children with visual acuity of less than 20/20, abnormal anterior or posterior segment findings, and IOP of >21 mm Hg were referred for further evaluation. RESULTS: Of 10,038 children (5,840 males [58.2%]) screened, 335 (median age, 9 years; range, 6-17 years) were referred. Refractive error was the most common cause of visual impairment (59.52%; 95% CI, 51.97-66.65) followed by amblyopia (17.2%; 95% CI, 12.3-23.6) and posterior segment anomaly (14.88%; 95% CI, 10.2-21.0). The prevalence of best-corrected visual acuity of 20/40 was 0.13%. The prevalence of blindness was 0.03%. CONCLUSIONS: Visual impairment among tribal children in this residential school is an uncommon but important disability.

Optical coherence tomography findings and retinal changes after vitrectomy for optic disc pit maculopathy.

PURPOSE: To study the optical coherence tomography (OCT) patterns in optic disc pit maculopathy and retinal changes after vitreous surgery. MATERIALS AND METHODS: Retrospective review of consecutive cases with optic disc pit maculopathy seen at two tertiary eye institutes from January 2005 to June 2009. RESULTS: Twenty-four eyes of 23 patients are included. The presenting visual acuity ranged from 20/400 to 20/20 (median:20/80). The median age at presentation was 24 years (range, 6-57 years). Optical coherence tomography demonstrated a combination of retinoschisis and outer layer detachment (OLD) in 19 (79.17%) eyes, OLD only in 3 (12.5%) eyes and retinoschisis only in 2 (8.33%) eyes. An obvious communication (outer layer hole) between the schisis and OLD was seen in 14 (73.68%) of the 19 eyes with both features. Of the 21 eyes with retinoschisis, schisis was present in multiple layers in 15 (71.43%) and single layer in 6 (28.57%) eyes. Eleven eyes underwent pars plana vitrectomy including creation of posterior vitreous detachment (PVD), fluid-air exchange, low intensity laser photocoagulation at the temporal edge of the optic disc pit and non-expansile perfluoropropane gas (14%) injection. Five (45.45%) of 11 eyes undergoing vitrectomy had complete resolution and 4 (36.36%) eyes had partial resolution of maculopathy. Visual acuity improved in 8 (72.72%) of 11 eyes. CONCLUSION: Optical coherence tomography demonstrates multiple layer schisis and outer layer detachment as main features of optic disc pit maculopathy. Vitrectomy with PVD induction, laser photocoagulation and gas tamponade results in anatomical and visual improvement in most cases with optic disc pit maculopathy.