Clinical features of pediatric idiopathic intracranial hypertension.

Introduction: Adult idiopathic intracranial hypertension (IIH) is often linked to obesity, however, causes of IIH in children are not well understood. This project identifies potential risk factors and features of pediatric IIH. Methods: This study consisted of a retrospective chart review of patients ages 5-17 years who were seen at a tertiary care children's hospital and diagnosed with IIH. Diagnostic criteria included the presence of papilledema, normal neurological exam, normal neuroimaging, normal cerebrospinal fluid composition, and an opening pressure of a lumbar puncture >28 mmHg. Results: Of the 26 cases of IIH, 19 met all diagnostic criteria for this study, while seven patients were probable IIH, as they lacked papilledema. Intracranial pressure ranged from 28 to 66 mmHg, with a mean of 40.23 mmHg (±10.74). Overall, 50.0% (95% CI: 29.9-70.1%) of IIH patients were obese, with patients 12 years of age and younger exhibiting an overall obesity rate of 30.7% and patients 13 years of age and older having an obesity rate of 69.2%. The overall allergy rate in this IIH patient population was 46.2% (95% CI: 26.6-66.6%). Conclusion: Obesity appears to have no association with IIH in younger cases, but it is a more common feature in older children. An autoimmune component may play a role in pediatric IIH, given the high rate of atopy observed in this pediatric IIH patient cohort. Because a diagnosis of IIH can have an absence of optic nerve edema, taking a detailed history and performing a thorough examination are keys to diagnosing IIH in the pediatric population.

Comparison of the characteristics of retinal hemorrhages in abusive head trauma versus normal vaginal delivery.

BACKGROUND: Retinal hemorrhage (RH) is one of the hallmarks of abusive head trauma (AHT); however, RH is also encountered with normal vaginal deliveries (NVD) and thus presents the clinician with a diagnostic dilemma. The purpose of this study was to compare RHs in AHT with those of NVD. METHODS: Records of with AHT and NVD infants with RH evaluated from 2013 to 2015 were reviewed retrospectively. Pattern, size, extent, and severity were compared using RetCam images. Severities were calculated using the RH grading scale. RESULTS: A total of 20 patients with AHT and 200 NVD infants were included. RH size was significantly larger in AHT patients compared to the NVD group (3.1 ± 0.512 vs 0.96 ± 0.046 disk diameters, resp.). The AHT group also demonstrated a higher RH incidence involving all three retinal layers compared to the NVD group (60% vs 0.6%, resp. [P < 0.001]). Vitreous hemorrhages were more common in the AHT group compared to the NVD group (54.3% vs 1.5% [P < 0.001]). Also, the grading scale demonstrated higher scores in the AHT group than the NVD group (7.15 ± 0.948 vs 3.59 ± 0.274, resp.). CONCLUSIONS: AHT and NVD share similar retinal findings, but they also have unique differentiators. In our subjects, AHT presented with more severe retinal findings than NVD, including larger RH size, a higher percentage involving all three retinal layers, a higher percentage of vitreous hemorrhages, and higher RH grading scale scores. Also, NVD retinal hemorrhages resolved quickly, within 4 weeks of birth in 95% of the patients.

Critical Assessment of an Ocular Photoscreener.

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.].

Assessment of an Advanced Vision Screener in the Detection of Amblyopia in the Nebraska Pediatric Population.

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.].

Wrong-Site Surgery in Pediatric Ophthalmology.

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; 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.].

Neuroimaging and endocrine disorders in paediatric optic nerve hypoplasia.

PURPOSE: Optic nerve hypoplasia (ONH) is one of the leading causes of blindness among children. The purpose of this retrospective study is to determine the risk factors and association between brain MRI findings, pituitary abnormalities and endocrine disorders with the presence of ONH. METHODS: A retrospective review of patients seen at paediatric ophthalmology clinics from January 2006 to December 2016 at Children's Hospital and Medical Center and the University of Nebraska Medical Center was performed. All patients with a documented diagnosis of ONH or septo-optic dysplasia were identified. MRI and endocrinology results were analysed by masked examiners. RESULTS: Out of 77 patients, overall incidence of abnormal pituitary on MRI was 35.1% and the incidence of endocrine abnormalities was 37.7%. Of the 57 patients with bilateral ONH, 23 (40.4%) had an abnormal pituitary while 4 of the 20 patients (20.0%) with unilateral ONH had an abnormal pituitary on MRI. The sensitivity and specific of brain MRI as signs of endocrinopathy are 67.9% and 83.3%, respectively. CONCLUSION: This study has determined that abnormal MRI findings do not have the sensitivity to predict endocrinopathy, nor does a normal MRI rule out possible endocrine abnormalities. When patients with ONH present with normal neurological examinations, normal endocrine workup and normal developmental milestones, a MRI of the brain may be deferred until new indications arise. Regardless of the MRI status, children with ONH should have a comprehensive endocrine evaluation and continue to have routine endocrine follow-up.

Retinopathy of prematurity: preferred practice patterns among pediatric ophthalmologists.

PURPOSE: The treatment of retinopathy of prematurity (ROP) is not standardized and can vary significantly between providers. This study aims to determine preferred practices in treating ROP by globally surveying pediatric ophthalmologists. METHODS: Between January and February 2017, an international pediatric ophthalmology interest group was invited to complete an anonymous survey of 18 questions. The main objectives were to determine the preferred first line of treatment for ROP, the preferred dosage of intravitreal bevacizumab (IVB) used, and the outcome and possible complications following bevacizumab injection. RESULTS: Out of 101 pediatric ophthalmologists, 72 (71.8%) stated that they had direct involvement in the treatment of ROP. When presented with type 1 ROP which requires treatment, 69 ophthalmologists (68.3%) stated that they prefer laser treatment over bevacizumab, and 33 ophthalmologists (32.7%) stated they would recommend bevacizumab as a first choice. Ninety-three ophthalmologists (92.1%) reported the success of 1 laser treatment between 75% and 100%, and 35 ophthalmologists (34.7%) perceive bevacizumab to be 75%-100% successful. Half dose of adult-prescribed bevacizumab at 0.625 mg/0.05 mL was preferred by 47 of the ophthalmologists (46.5%). No cases of endophthalmitis were reported with intravitreal injection. CONCLUSION: Laser photoablation remains the preferred mode of treatment for ROP among surveyed ophthalmologists across the world. Though bevacizumab is currently being used, this form of treatment is not as common, primarily due to the unknown safety profile and potential long-term ramifications of the drug.

Clinical comparison of ocular and systemic findings in diagnosed cases of abusive and non-abusive head trauma.

PURPOSE: Child abuse is a leading cause of death in infants, which is often associated with abusive head trauma (AHT). The purpose of this retrospective analysis was to identify ocular and systemic findings in confirmed cases of AHT and compare them to a group of non-abusive head trauma (NAHT) patients. PATIENTS AND METHODS: A retrospective chart review of 165 patients with accidental and non-accidental trauma admitted between 2013 and 2015 to Children's Hospital and Medical Center in Omaha, NE, USA, was performed. Diagnosis of AHT was made after the analysis of ocular and systemic findings by various subspecialists. The NAHT group consisted of accidental trauma, abusive trauma without significant apparent head involvement on initial evaluation and unconfirmed AHT cases. RESULTS: Of the 165 presenting cases, 30 patients were diagnosed with AHT and 127 were diagnosed with NAHT. Ocular findings in AHT patients were significant for retinal hemorrhages (63%) and vitreous hemorrhages (37%), while NAHT patients had no ocular findings (p<0.001). Neuroimaging revealed subdural hemorrhages (SDHs) in 29 out of 30 AHT patients (97%) and in 27 out of 127 NAHT patients (21%). Seizures were present in 43% of AHT patients (n=13) and only in 8% of NAHT patients (n=10). CONCLUSION: AHT has statistically significant findings of retinal and vitreous hemorrhages. The absence of diffuse retinal hemorrhages, however, does not preclude the AHT diagnosis as more than one-third of AHT patients lacked retinal hemorrhages. SDHs, loss of consciousness and history of seizures also have high correlation with a diagnosis of AHT.

Myeloid Sarcoma in the Orbit.

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.].

Central toxic keratopathy: a case study and literature review.

BACKGROUND: Central toxic keratopathy (CTK) is a rare, non-inflammatory corneal opacity that can occur after corneal laser refractive surgery. It is characterized by the absence of inflammatory cells within the cornea or anterior chamber, central stromal necrosis, and corneal opacification, with an onset of 3 to 9 days after refractive surgery; CASE STUDY: This case report reviews the clinical findings, differential diagnosis, possible etiologies, and management of CTK; CONCLUSION: Though listed in the literature under numerous names, including diffuse lamellar keratitis (DLK) Stage IV, central lamellar keratitis (CLK), central flap necrosis (CFN), flap necrosis syndrome (FNS), and keratocyte-induced corneal microedema (KME), the conditions share characteristics with CTK, including clinical findings and treatment modalities. Treatment for CTK is controversial, though studies show best practices include close monitoring for possible corneal melt, avoiding topical corticosteroids, and possible enhancements for resultant hyperopia.

Malignant melanoma associated with lichen sclerosus in the vulva of a 10-year-old.

Malignant melanoma of the vulva in childhood is a rare neoplasm. Lichen sclerosus of the vulva in childhood is also a rare disease. The association of these two rare lesions in the vulva of young girls is extremely rare. We present a 10-year-old white girl with malignant melanoma associated with lichen sclerosus of the vulva. She had dark pigmentation of both the labia minora and posterior fourchette. The inner labia majora and fourchette showed whitish, glistening areas of skin. Histologic examination found mostly an in situ lentiginous/mucosal melanoma with focal invasion to a depth of 0.44 mm in the left upper labium majus. All specimens showed evidence of lichen sclerosus. Partial vulvectomy was performed, and no metastases were detected at the time of treatment. The patient has been disease free for the 12 months after treatment. It is critical for physicians to realize that melanoma can occur in children, and although rare, can occur in the vulva. We feel that lichen sclerosus in this instance may represent a pattern of host immune response to melanoma.