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Purpose:To evaluate the validity of remote telemedicine screening for retinopathy of prematurity (ROP) in a population of at-risk preterm infants in Iowa and South Dakota.Methods:The medical records for all preterm infants screened for ROP at neonatal intensive care units (NICUs) in Sioux City, Iowa, and Sioux Falls, South Dakota, from September 1, 2017, to July 31, 2018, were retrospectively reviewed. The RetCam Shuttle (Natus Medical Inc., Pleasanton, CA) was used to capture retinal images, which were posted on a secure server for evaluation by a pediatric ophthalmologist. Infants with suspected ROP approaching the criteria for treatment with anti-vascular endothelial growth factor (VEGF) medications were transferred to the Children's Hospital and Medical Center NICU in Omaha, Nebraska, where a comprehensive examination was performed and treatment was administered when indicated. The remaining infants received an outpatient comprehensive examination by one of two pediatric ophthalmologists within 2 weeks of discharge.Results:A total of 124 telemedicine examinations were performed on 35 infants during the study period. Remote telemedicine screening for referral-warranted ROP using the RetCam Shuttle had a sensitivity of 100%, specificity of 97%, positive predictive value of 66.7%, and negative predictive value of 100%. Of the three infants transferred for referral-warranted ROP, two required treatment with anti-VEGF medications. Good outcomes were noted in all cases, and no patients progressed beyond stage 3 ROP.Conclusions:Telemedicine screening reliably detected referral-warranted ROP in at-risk premature infants at two remote sites, with no poor outcomes during the 11-month period. These results demonstrate the validity and utility of remote telemedicine screening for ROP.[[J Pediatr Ophthalmol Strabismus. 2019;56(3):157–161.]

Purpose:To determine the prevalence of pediatric ophthalmologists who have performed wrong-site surgery, propose risk factors leading to these errors, and assess the effectiveness of the Universal Protocol in preventing them.Methods:Approximately 1,000 pediatric ophthalmology surgeons were invited to complete an anonymous 10-question survey. Respondents were divided into two groups: those who performed or attempted wrong-site surgery (wrong-site surgery group) and those who had never performed a wrong-site surgery (intended surgical site group). The risk factors (ie, marking procedure, years in practice, surgical experience, adherence to the Universal Protocol time-out, and operating room factors) were compared between groups.Results:Of the 156 respondents, 56.4% never performed, 9% attempted, and 34.6% performed a wrong-site surgery. The use of any procedure to mark the eye decreased the likelihood of a wrong-site surgery by 61% (odds ratio [OR] = 0.39; Of the 156 respondents, 56.4% never performed, 9% attempted, and 34.6% performed a wrong-site surgery. The use of any procedure to mark the eye decreased the likelihood of a wrong-site surgery by 61% (odds ratio [OR] = 0.39; P= .069). A lower likelihood of error occurred when a single individual led the time-out and multiple individuals participated in checking the accuracy of the time-out. Surgeons in practice for less than 15 years had a lower likelihood of performing a wrong-site surgery (OR = 0.37; 95% confidence interval [CI] = 0.19 to 0.72; P= .003). Factors not significantly associated with wrong-site surgeries were the number of surgeries performed per year (OR = 0.66; 95% CI = 0.35 to 1.24; P= .20) and the number of operating rooms used.Conclusions:In concordance with previous reports of other surgical specialties, self-reported error in pediatric ophthalmology is not uncommon. This study highlighted important practices that can be easily adopted by surgeons to decrease the likelihood of wrong-site surgeries. First, marking the surgical site must be part of the preoperative preparation. Second, a single designated individual should lead the time-out and the surgeon should be directly involved in all steps of the time-out process. Third, surgeons who have been in practice for more than 15 years may require additional safeguards to ensure that the correct surgery is performed and to monitor their complacency.[[J Pediatr Ophthalmol Strabismus. 2018;55(3):152–158.]

Purpose:To assess the ease of use of the D-EYE digital ophthalmoscope (D-EYE Srl, Padova, Italy) in retinal screening against the conventional direct ophthalmoscope. The digital ophthalmoscope used comprised a smartphone equipped with a D-EYE lens that produces digital retinal images.Methods:Twenty-five medical students were given 30 minutes of instruction regarding how to use a direct ophthalmoscope and D-EYE digital ophthalmoscope by a pediatric ophthalmologist. Afterwards, they used two methods to view the fundus under dim light on two undilated volunteer participants under supervision of the pediatric ophthalmologist. Each student had to describe their findings and show the video taken from the smartphone to the pediatric ophthalmologist. Students also completed a survey rating their experience using each method.Results:Ninety-two percent of the medical students preferred the D-EYE digital ophthalmoscope to the direct ophthalmoscope. Students were also able to identify the optic nerve and macula in a shorter amount of time and review the images to confirm their findings. Overall, the medical students showed a strong preference for the D-EYE digital ophthalmoscope that was statistically significant (Ninety-two percent of the medical students preferred the D-EYE digital ophthalmoscope to the direct ophthalmoscope. Students were also able to identify the optic nerve and macula in a shorter amount of time and review the images to confirm their findings. Overall, the medical students showed a strong preference for the D-EYE digital ophthalmoscope that was statistically significant (P< .001).Conclusions:The D-EYE digital ophthalmoscope is a practical device that could be incorporated into medical education and clinical practice. Survey results revealed that most students preferred the D-EYE digital ophthalmoscope due to the recording features and larger image of the fundus.[[J Pediatr Ophthalmol Strabismus. 2018;55(3):201–206.]

Purpose:To determine the validity of the OPTEC 5500 vision screener (Stereo Optical Co., Inc., Chicago, IL) in assessing visual acuity and amblyopia in pediatric patients between the ages of 3 and 17 years by comparing it statistically to gold standard comprehensive ophthalmic examinations.Methods:A cross-sectional study was conducted of 64 patients between the ages of 3 and 17 years who underwent a vision screening test at a pediatric ophthalmology office using the OPTEC 5500 vision screener, followed by traditional visual acuity testing via the Snellen or Lea optotypes. After data were collected, the results of the OPTEC 5500 vision screener were compared to the Snellen and Lea visual acuity tests and statistical analysis was subsequently performed for the right and left eyes separately. Patients were considered to have risk factors for amblyopia based on the American Association for Pediatric Ophthalmology and Strabismus referral criteria guidelines.Results:The results of the OPTEC 5500 vision screener for the right eye of participants of all ages were a sensitivity of 77.4%, specificity of 100.0%, positive predictive value of 100.0%, negative predictive value of 50.0%, and accuracy of 81.5%. Results for the left eye were a sensitivity of 81.0%, specificity of 87.0%, positive predictive value of 91.9%, negative predictive value of 71.4%, and accuracy of 83.1%.Conclusions:Although the specificity and positive predictive value were acceptable, the sensitivity and negative predictive value of the OPTEC 5500 vision screener were below average when compared to other available devices, exhibiting some of the weaknesses of the device. Additional studies of the OPTEC 5500 vision screener with a larger population are necessary to assess the device in the general pediatric population, such as in general pediatric clinics and public schools. Additionally, other options for pediatric vision screening devices should be explored.[[J Pediatr Ophthalmol Strabismus. 2018;55(3):189–193.]

Purpose:To determine the accuracy of the PlusoptiX A12 photoscreener (PlusoptiX, Inc., Atlanta, GA) in detecting amblyopia or ambylogenic risk factors in pediatric patients in Nebraska.Methods:Using the PlusoptiX A12 photoscreener, data were collected from pediatric patients seen at a single pediatric ophthalmology practice. Each patient was screened using the device and also received a comprehensive ophthalmic examination. The results of the PlusoptiX A12 photoscreener were compared to the gold standard, comprehensive ophthalmic examination findings. The assessment of amblyopia or amblyogenic risk factors in the patients was based on the updated American Association for Pediatric Ophthalmology and Strabismus (AAPOS) referral criteria guidelines.Results:Data were collected from 219 consecutive pediatric patients (438 eyes) during the 3-month study period. Among the patients, 87 (40%) children were determined to have amblyopia or ambylogenic risk factors after the comprehensive pediatric ophthalmology examination based on the AAPOS guidelines. The PlusoptiX A12 photoscreener was found to have a sensitivity of 93.02%, specificity of 84.96%, false-positive rate of 9.13%, false-negative rate of 2.74%, positive predictive value of 80.00%, and negative predictive value of 94.96%.Conclusions:The PlusoptiX A12 photoscreener is viable and comparable to various commercially available devices in the detection of refractive amblyogenic risk factors based on the Nebraska pediatric patient population. Future studies may show increased sensitivity by combining the use of the PlusoptiX A12 photoscreener with an alternate cover test.[[J Pediatr Ophthalmol Strabismus. 2018;55(3):194–199.]

The authors describe a case of myeloid sarcoma of the orbit in a pediatric patient. An 8-month-old male infant presented to the ophthalmology clinic with a left orbital mass, which had been increasing in size over the previous 2 months. The mass was initially diagnosed at another clinic as an infantile hemangioma, and had been treated with a topical formulation of timolol. In the ophthalmology clinic, orbital magnetic resonance imaging showed a solid enhancing mass. A biopsy was performed, and histopathology revealed myeloid sarcoma. The disease responded well to a standard chemotherapy regimen. Myeloid sarcoma is a rare, extra-medullary presentation that can occur as an isolated tumor, concurrently with or at relapse of acute myeloid leukemia. Because few cases of myeloid sarcoma in the orbit have been reported, this case report aids in the management of myeloid sarcoma in pediatric patients. The report describes an 8-month-old male infant, the youngest patient to develop myeloid sarcoma without preexisting acute myeloid leukemia. The authors describe a case of myeloid sarcoma of the orbit in a pediatric patient. An 8-month-old male infant presented to the ophthalmology clinic with a left orbital mass, which had been increasing in size over the previous 2 months. The mass was initially diagnosed at another clinic as an infantile hemangioma, and had been treated with a topical formulation of timolol. In the ophthalmology clinic, orbital magnetic resonance imaging showed a solid enhancing mass. A biopsy was performed, and histopathology revealed myeloid sarcoma. The disease responded well to a standard chemotherapy regimen. Myeloid sarcoma is a rare, extra-medullary presentation that can occur as an isolated tumor, concurrently with or at relapse of acute myeloid leukemia. Because few cases of myeloid sarcoma in the orbit have been reported, this case report aids in the management of myeloid sarcoma in pediatric patients. The report describes an 8-month-old male infant, the youngest patient to develop myeloid sarcoma without preexisting acute myeloid leukemia. [[J Pediatr Ophthalmol Strabismus. 2016;53:e64–e68.]

Purpose:To study the magnitude of anisocoria in pediatric patients using the plusoptiX A08 (plusoptiX GmbH, Nuremberg, Germany) photoscreener as compared to a literature review of pediatric patients with known Horner syndrome to determine if anisocoria alone should raise suspicion for the diagnosis.Methods:The medical records of 592 consecutive patients, neonates to 9 years old, were collected and analyzed. All patients had complete ophthalmic examinations that included photoscreening with the plusoptiX A08. Data included age, pupil sizes, and anisocoria. A complete literature search of documented pupillary size in pediatric patients with the diagnosis of Horner syndrome was performed. This was then compared to the normative pediatric pupil data from the study.Results:Of the 592 children without Horner syndrome, 372 had an anisocoria of 0.1 to 0.5 mm (62.84%), 167 had an anisocoria of 0.6 to 1.2 mm (28.16%), and 21 had an anisocoria of 1.3 mm or greater (3.70%). There was no correlation between increasing age and severity of anisocoria (Of the 592 children without Horner syndrome, 372 had an anisocoria of 0.1 to 0.5 mm (62.84%), 167 had an anisocoria of 0.6 to 1.2 mm (28.16%), and 21 had an anisocoria of 1.3 mm or greater (3.70%). There was no correlation between increasing age and severity of anisocoria (P= .55). For pediatric patients with a diagnosis of Horner syndrome, the average level of anisocoria was 1.37 mm in room light and 2 mm in darkness. In room light, three children had anisocoria of 0.1 to 0.5 mm (9.4%), 14 had anisocoria of 0.6 to 1.2 mm (43.8%), and 15 had anisocoria of 1.3 mm or greater (46.9%). In darkness, the level of anisocoria increased in 19 patients, causing the first category, 0.1 to 0.5 mm, to include 1 patient (3.1%), the second group to include 5 patients (15.6%), and the last group to include 26 patients (81.3%). Other associated signs/symptoms included ptosis (100%), heterochromia (28.1%), anhidrosis (9.4%), straight hair on affected/curly on unaffected side (9.4%), and neck mass (6.3%). In 37.5% of cases, imaging results were negative and no specific etiology was determined.Conclusions:In a study of 592 children without Horner syndrome, the average pupillary size increased with age, but the degree of anisocoria remained stable with increasing age. Over half of the children studied had anisocoria up to 0.5 mm (62.84%), but rarely had anisocoria greater than 1.3 mm (3.70%). In children with a diagnosis of Horner syndrome, the majority had anisocoria greater than 1.3 mm, with the discrepancy in pupil size becoming more apparent in low levels of light intensity. Anisocoria greater than 1.3 mm is unlikely to be physiologic in a child; therefore, he or she should be carefully evaluated for other localizing signs, such as ptosis, anhidrosis, and neck mass.[[J Pediatr Ophthalmol Strabismus. 2016;53(3):186–189.]