Outcomes of Strabismus Surgery Following Teprotumumab Therapy.

PURPOSE: To investigate the results of patients undergoing surgical treatment for strabismic diplopia in thyroid eye disease (TED) following teprotumumab. DESIGN: Multicenter, retrospective, case series. METHODS: We report 28 patients who underwent extraocular muscle surgery for strabismic diplopia after treatment with teprotumumab at 7 different academic centers. Elapsed time from last teprotumumab dose to the date of surgery, previous orbital decompression, primary preoperative horizontal and vertical deviation, surgical procedure, and 2-month postoperative results were collected from the patient records. RESULTS: Sixteen (57%) patients were diplopia-free after 1 surgery. Three (11%) chose prism spectacles to correct residual diplopia, 2 (7%) used compensatory head posture to resolve diplopia, and 1 (4%) had intermittent diplopia and was functionally improved (choosing no prisms or further surgery). These were considered treatment successes. Three (11%) patients required reoperation, and all were diplopia-free after their second procedure. CONCLUSIONS: Most patients requiring surgery for strabismic diplopia following teprotumumab achieve good outcomes with success rates comparable to series published before the availability of teprotumumab.

Ocular presentation of Mpox in a healthy child without known exposure.

Ocular complications can occur from Mpox infection and are increasingly being reported, with the rise in worldwide cases. There are few reports of Mpox outside of endemic areas in healthy children. We describe a healthy girl with Mpox who presented with ocular symptoms after experiencing trauma to the eye; this case highlights a pediatric case of Mpox localized to the eye and periorbital region. In the absence of a prodromal phase, the ocular signs and symptoms were initially thought to represent more common, benign etiologies. This case underscores the importance of considering Mpox, even in the absence of known exposures or in the setting of an atypical presentation.

Peripapillary hyperreflective ovoid masslike structures in a pediatric population referred for suspected papilledema.

BACKGROUND: Peripapillary hyperreflective ovoid masslike structures (PHOMS) are now considered a common cause of pseudopapilledema in the pediatric population. METHODS: The medical records of all patients ≤18 years of age with PHOMS, confirmed on optical coherence tomography, seen at single pediatric neuro-ophthalmology clinic between 2017 and 2021 were reviewed retrospectively. RESULTS: A total of 47 patients (35 females), with median age of 12.7 years at initial visit, were included. Of these, 24 (51%) of patients were optometry referrals. Headache was a common symptom. PHOMS were present bilaterally in all patients and were 360° around the disk in 57 of 94 eyes (61%). Patients with elevated intracranial pressure were more likely to have a reduction in the size of PHOMS at follow-up (P = 0.0133). Of the 47, 40 (85%) had diagnostic neuroimaging as a part of their papilledema workup. The retinal nerve fiber layer was thicker in patients with concomitant papilledema rather than optic disk drusen (P = 0.002) and compared with patients with PHOMS alone (P = 0.00051). CONCLUSIONS: Pediatric patients with PHOMS may be referred because their clinical appearance can be suggestive of papilledema. In our study cohort, PHOMS were bilateral and frequently occurred concurrently with other ophthalmic conditions.

Segmental Imaging of the Trochlear Nerve: Anatomic and Pathologic Considerations.

BACKGROUND: The trochlear nerve (the fourth cranial nerve) is the only cranial nerve that arises from the dorsal aspect of the midbrain. The nerve has a lengthy course making it highly susceptible to injury. It is also the smallest cranial nerve and is often difficult to identify on neuroimaging. EVIDENCE ACQUISITION: High-resolution 3-dimensional skull base MRI allows for submillimeter isotropic acquisition and is optimal for cranial nerve evaluation. In this text, the detailed anatomy of the fourth cranial nerve applicable to imaging will be reviewed. RESULTS: Detailed anatomic knowledge of each segment of the trochlear nerve is necessary in patients with trochlear nerve palsy. A systematic approach to identification and assessment of each trochlear nerve segment is essential. Pathologic cases are provided for each segment. CONCLUSIONS: A segmental approach to high-resolution 3-dimensional MRI for the study of the trochlear nerve is suggested.

Establishing a surgical outreach program in the developing world: pediatric strabismus surgery in Guatemala City, Guatemala.

PURPOSE: To report our experince in establishing a sustainable pediatric surgical outreach mission to an underserved population in Guatemala for treatment of strabismic disorders. METHODS: A pediatric ophthalmic surgical outreach mission was established. Children were evaluated for surgical intervention by 3 pediatric ophthalmologists and 2 orthoptists. Surgical care was provided at the Moore Pediatric Surgery Center, Guatemala City, over 4 days. Postoperative care was facilitated by Guatemalan physicians during the second year. RESULTS: In year 1, patients 1-17 years of age were referred by local healthcare providers. In year 2, more than 60% of patients were prescreened by a local pediatric ophthalmologist. We screened 47% more patients in year 2 (132 vs 90). Diagnoses included congenital and acquired esotropia, consecutive and acquired exotropia, congenital nystagmus, Duane syndrome, Brown syndrome, cranial nerve palsy, dissociated vertical deviation, and oblique muscle dysfunction. Overall, 42% of the patients who were screened underwent surgery. We performed 21 more surgeries in our second year (58 vs 37), a 57% increase. There were no significant intra- or postoperative complications. CONCLUSIONS: Surgical outreach programs for children with strabismic disorders in the developing world can be established through international cooperation, a multidisciplinary team of healthcare providers, and medical equipment allocations. Coordinating care with local pediatric ophthalmologists and medical directors facilitates best practice management for sustainability.

Traumatic avulsion of the oculomotor nerve documented by high-resolution magnetic resonance imaging.

Autopsy studies have described definitive traumatic avulsion of the oculomotor nerve from the brainstem; however, detailed characterization of mechanisms and localization of traumatic nerve injury has yet to be definitively described in vivo. We report the case of a 13-year-old girl in whom high-resolution magnetic resonance imaging confirmed irreversible injury to the left oculomotor nerve after trauma.

Blue cone monochromacy: visual function and efficacy outcome measures for clinical trials.

BACKGROUND: Blue Cone Monochromacy (BCM) is an X-linked retinopathy caused by mutations in the OPN1LW / OPN1MW gene cluster, encoding long (L)- and middle (M)-wavelength sensitive cone opsins. Recent evidence shows sufficient structural integrity of cone photoreceptors in BCM to warrant consideration of a gene therapy approach to the disease. In the present study, the vision in BCM is examined, specifically seeking clinically-feasible outcomes for a future clinical trial. METHODS: BCM patients (n = 25, ages 5-72) were studied with kinetic and static chromatic perimetry, full-field sensitivity testing, and eye movement recordings. Vision at the fovea and parafovea was probed with chromatic microperimetry. RESULTS: Kinetic fields with a Goldmann size V target were generally full. Short-wavelength (S-) sensitive cone function was normal or near normal in most patients. Light-adapted perimetry results on conventional background lights were abnormally reduced; 600-nm stimuli were seen by rods whereas white stimuli were seen by both rods and S-cones. Under dark-adapted conditions, 500-nm stimuli were seen by rods in both BCM and normals. Spectral sensitivity functions in the superior retina showed retained rod and S-cone functions in BCM under dark-adapted and light-adapted conditions. In the fovea, normal subjects showed L/M-cone mediation using a 650-nm stimulus under dark-adapted conditions, whereas BCM patients had reduced sensitivity driven by rod vision. Full-field red stimuli on bright blue backgrounds were seen by L/M-cones in normal subjects whereas BCM patients had abnormally reduced and rod-mediated sensitivities. Fixation location could vary from fovea to parafovea. Chromatic microperimetry demonstrated a large loss of sensitivity to red stimuli presented on a cyan adapting background at the anatomical fovea and surrounding parafovea. CONCLUSIONS: BCM rods continue to signal vision under conditions normally associated with daylight vision. Localized and retina-wide outcome measures were examined to evaluate possible improvement of L/M-cone-based vision in a clinical trial.

Global challenges in the management of congenital cataract: proceedings of the 4th International Congenital Cataract Symposium held on March 7, 2014, New York, New York.

Childhood cataracts have become a leading cause of preventable childhood blindness in many areas of the world. Here we summarize regional focus group discussions from the 4th Annual International Congenital Cataract Symposium on the current situation, challenges, and recommendations for the management of congenital cataracts in sub-Saharan Africa, the Middle East and North Africa, South Asia, Central America, South America, and developed nations. Strategies for managing congenital cataracts must be adapted and developed according to regional conditions. A basic framework for acceptable outcomes must focus on developing systems to address the critical components of education, access, quality care, and good follow-up.

Validating a nonhuman primate model of super-selective intraophthalmic artery chemotherapy: comparing ophthalmic artery diameters.

PURPOSE: Superselective intraophthalmic artery chemotherapy (SSIOAC) is being used for treatment of retinoblastoma; however, the hemodynamic consequences and toxicities are not fully known. We developed a nonhuman primate (NHP) model of SSIOAC and reported our clinical observations. For validation, we compared ophthalmic artery (OA) diameters between NHPs and children (<6 years). METHODS: Endovascular cannulation of the right OA was performed three times each in six adult male Rhesus macaques. Angiographic OA images were obtained and measured, and postmortem OAs were histologically sectioned and measured. Retrospectively, computed tomography (CT) and magnetic resonance (MR) angiography images of the head in children and adolescents (as an adult reference) were used to measure the OA luminal diameter at its origin. RESULTS: The median angiographic diameter of treated NHP OA origins (n = 6) was 1.06 mm (range 0.94-1.56). Histologic measurements (8 of 12 NHP OAs) gave a median diameter of 1.09 mm (range 0.95-1.41). In 98 children (from 169 consecutive CT and MR angiography studies; median age 1.01 years, range 0.01-5.74), 186 OAs were measurable at the origin (median luminal diameter 1.28 mm, range 0.82-2.00; P = 0.16 for the angiographic NHP diameters versus pediatric cohort). Angiographic measurements of 34 OAs (of 20 consecutive studies of adolescents; median age 16.55 years, range 14.40-18.18) gave a median luminal diameter of 1.45 mm (origin, range 1.13-1.66; P < 0.0001, adolescent versus pediatric). CONCLUSIONS: Measurements of the OA luminal diameter at its origin were similar between our NHP and pediatric cohort, validating our NHP model for testing both the hemodynamic consequences and toxicities of SSIOAC.

Chondromyxoid fibroma of the orbit.

A 51-year-old woman with a history of migraine headaches was found to have an incidental right orbital mass on MRI during neurologic evaluation for headaches. The orbital mass was a well-defined, lobulated, intraosseous soft tissue lesion with circumscribed margins. Clinically, there was noted proptosis, tenderness to palpation, and slight limitation to right abduction. An orbitotomy with incisional biopsy revealed a lesion arising within the lateral orbital rim extending to the subperiosteal space. Intraoperative frozen sections indicated a low grade sarcoma, possibly metastatic. The extraosseous component was excised, and the bone was curetted until all visible tumor was removed. A diagnosis of chondromyxoid fibroma was made. The patient did well until 5 months postoperatively, when right-sided proptosis returned due to recurrent tumor. Repeat surgical resection with removal of the lateral orbital rim was performed. Histopathology was consistent with recurrent chondromyxoid fibroma.

Outcomes in 15 patients with conjunctival melanoma treated with adjuvant topical mitomycin C: complications and recurrences.

PURPOSE: To report the long-term complications and rate of local recurrence in a cohort of patients with histopathologically confirmed conjunctival melanoma (CM) treated with adjuvant topical mitomycin C (MMC). DESIGN: Retrospective, nonrandomized interventional study. PARTICIPANTS: Fifteen patients with histopathologically confirmed diagnosis of CM treated with topical MMC. METHODS: We identified all patients with histopathologically confirmed diagnosis of CM treated with topical MMC between January 1999 and March 2010. Data extracted from the patients' medical records included demographic, clinical, and histopathologic findings; treatments; long-term complications (>6 months) of MMC therapy; recurrent and metastatic disease; and mortality. MAIN OUTCOME MEASURES: Prevalence of long-term complications of MMC and development of recurrent disease were assessed. RESULTS: Fifteen patients (12 female) received topical MMC a median of 2.8 months (0.37-110.9 months) after the diagnosis of CM. Median age at diagnosis was 62 years (29-82 years), and median length of follow-up was 23.8 months (2.2-130.8 months). Most common complications included injection (n=13), tearing (n=10), irritation (n=9), pain (n=9), and limbal stem cell deficiency with keratopathy (n=4). Twelve patients (80%) experienced at least 1 long-term complication, with failure of resolution of symptoms in 7 of these patients. Local recurrence was associated with longer delay to MMC initiation (2 ±8.0 vs. 30.8 ±11 months, P=0.06). Three patients developed metastases. Recurrence was associated with metastatic disease (P=0.001). Exenteration was required in 2 patients, 1 of whom developed metastatic disease and died. CONCLUSIONS: Careful consideration should be given to the use of adjuvant MMC for the treatment of residual intraepithelial disease after the diagnosis of CM given the risk of potential long-term ocular surface toxicities.