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Appropriateness Criteria

Reference Study Type Patients/Events Study Objective(Purpose of Study) Study Results Study Quality
1. Graves JS, Galetta SL. Acute visual loss and other neuro-ophthalmologic emergencies: management. Neurol Clin 2012;30:75-99, viii. Review/Other-Dx N/A To review the approach to acute visual loss, the abnormal optic disc, double vision, and the neuro-ophthalmologic signs of neurologic emergencies, including hydrocephalus, herniation syndromes, vascular lesions, and trauma. No results stated in the abstract. 4
2. Vachha BA, Robson CD. Imaging of Pediatric Orbital Diseases. Neuroimaging Clin N Am 2015;25:477-501. Review/Other-Dx N.A To review a variety of congenital and developmental disorders of the pediatric orbit with particular emphasis on ocular lesions, followed by a description of developmental and neoplastic orbital and ocular masses No results listed in abstract. 4
3. Goh PS, Gi MT, Charlton A, Tan C, Gangadhara Sundar JK, Amrith S. Review of orbital imaging. Eur J Radiol. 2008; 66(3):387-395. Review/Other-Dx N/A Review imaging of patients with suspected orbital disease. Article presents a diagnostic strategy based on a compartment model. In the diagnosis of patients with orbital disease, a systematic evaluation using an anatomical compartment strategy, evaluation of imaging features, and correlation with clinical presentation and patient age are recommended. 4
4. Acar U, Tok OY, Acar DE, Burcu A, Ornek F. A new ocular trauma score in pediatric penetrating eye injuries. Eye (Lond) 2011;25:370-4. Review/Other-Tx 29 patients (12 female, 17 male) To assess the prognostic value of a new ocular trauma score (OTS) in pediatric penetrating injuries. In total, 30 eyes in 29 patients (41.38% female, 58.62% male) with a mean age of 6.83±4.00 years (range: 1–15 years) were included in the study. Initial VA, which was evaluated in 22 patients, was as follows: no light perception (NLP) in 2 (9.09%) patients, light perception (LP) to hand motion (HM) in 8 (36.36%) patients, counting fingers in 6 (27.27%) patients, 0.1–0.5 in 4 (18.18%) patients, and =0.6 in 2 (9.09%) patients. Final VA, which was evaluated in 27 patients, was as follows: NLP in 3 (11.11%) patients, LP to HM in 3 (11.11%) patients, counting fingers in 2 (7.41%) patients, 0.1–0.5 in 11 (40.74%) patients, and =0.6 in 8 (29.63%) patients. The relationship between initial VA and final VA was statistically significant (P<0.001). 4
5. Stotland MA, Do NK. Pediatric orbital fractures. [Review]. J Craniofac Surg. 22(4):1230-5, 2011 Jul. Review/Other-Dx N/A To  review the epidemiology, anatomy, growth implications, pathomechanics, particular clinical features, assessment, and surgical management of pediatric orbital fractures. No results stated in the abstract. 4
6. Betts AM, O'Brien WT, Davies BW, Youssef OH. A systematic approach to CT evaluation of orbital trauma. [Review]. Emergency Radiology. 21(5):511-31, 2014 Oct.EMERG. RADIOL.. 21(5):511-31, 2014 Oct. Review/Other-Dx N/A To present a systematic approach to assist radiologists in the rapid evaluation of orbital trauma using the "BALPINE" mnemonic-bones, anterior chamber, lens, posterior globe structures, intraconal orbit, neurovascular structures, and extraocular muscles/extraconal orbit No results stated in abstract. 4
7. Hink EM, Wei LA, Durairaj VD. Clinical features and treatment of pediatric orbit fractures. Ophthal Plast Reconstr Surg. 30(2):124-31, 2014 Mar-Apr. Review/Other-Dx 312 To describe a series of orbital fractures and associated ophthalmic and craniofacial injuries in the pediatric population. Five hundred ninety-one fractures in 312 patients were evaluated. There were 192 boys (62%) and 120 girls (38%) with an average age of 7.3 years (range 4 months to 16 years). Orbit fractures associated with other craniofacial fractures were more common (62%) than isolated orbit fractures (internal fractures and fractures involving the orbital rim but without extension beyond the orbit) (38%). Roof and medial wall fractures were most common (30% and 28%, respectively), followed by orbital floor (24%) and lateral wall (18%) fractures. Orbital roof fractures are the most common fracture in patients <8 years old, whereas orbital floor fractures are the most common fracture in patients older than 8 years. Eighty-seven patients (28%) underwent surgical repair. There is an increasing incidence of surgery in older patients (p = 0.02). Associated neurologic injuries were more common (23%) than associated ophthalmic injuries (20%). 4
8. Barh A, Swaminathan M, Mukherjee B. Orbital fractures in children: clinical features and management outcomes. J AAPOS. 22(6):415.e1-415.e7, 2018 12. Review/Other-Dx 52 patients To report the clinical characteristics and management outcomes of orbital fractures in children. In our study cohort, orbital floor fracture was most common. The trapdoor type of fracture was seen in almost half of the patients, with diplopia being the most common presenting complaint. Early surgical intervention was associated with complete resolution of ocular motility limitation and diplopia.s. 4
9. Roth FS, Koshy JC, Goldberg JS, Soparkar CN. Pearls of orbital trauma management. Semin Plast Surg 2010;24:398-410. Review/Other-Dx N/A To provide an understanding of the essential subtleties of orbital injuries requires an appreciation for the orbital examination, appropriate management, and potential complications. No results stated in the abstract. 4
10. Li X, Zarbin MA, Bhagat N. Pediatric open globe injury: A review of the literature. J Emerg Trauma Shock 2015;8:216-23. Review/Other-Dx N/A The aim of this article is to review the epidemiology, diagnosis, management, and prognosis of this condition by conducting a systematic literature search with inclusion of all case series on pediatric open globe injury (OGI) published between 1996 and 2015. No results stated in the abstract. 4
11. Mashriqi F, Iwanaga J, Loukas M, D'Antoni AV, Tubbs RS. Penetrating Orbital Injuries: A Review. Cureus 2017;9:e1725. Review/Other-Dx N/A To discuss penetrating orbital injuries and present the complications associated with each orbital entry mode: superior, inferior, medial, lateral rims of the orbit, and extraorbital entry. No abstract available 4
12. Kubal WS. Imaging of orbital trauma. [Review] [25 refs]. Radiographics. 28(6):1729-39, 2008 Oct. Review/Other-Dx N/A To review the imaging of orbital trauma. No results stated in abstract. 4
13. Patel SN, Langer PD, Zarbin MA, Bhagat N. Diagnostic value of clinical examination and radiographic imaging in identification of intraocular foreign bodies in open globe injury. Eur J Ophthalmol. 22(2):259-68, 2012 Mar-Apr. Review/Other-Dx 527 patients To evaluate the diagnostic accuracy of clinical eye examination and radiographic imaging in the identification of intraocular foreign bodies (IOFBs) in open-globe traumatic injuries. A total of 527 patients with traumatic open globe injuries presented to UH, Newark, New Jersey, USA, between 1998 and 2008. Of these, 74 patients had surgically confirmed IOFBs. Mean age of patients with traumatic open globe injury and an IOFB was 33 years (range, 8-69 years); mean follow-up was 17.6 months (range, 1 day-90 months). Foreign bodies were identified as glass (13), metal (58), wood (1), plastic (0), and other (2). There were 24 anterior segment (AS) IOFBs, 45 posterior segment (PS) IOFBs, and 5 noted in both segments. Clinical eye examination at presentation identified an IOFB in 34 (45.6%) of 74 patients. B-scan echography revealed an IOFB in 14 (51.9%) of 27 cases. Computed tomography scan of the orbits identified IOFBs in 56 (94.9%) of 59 cases. Clinical eye examination was performed in all (100%) patients. B-scan was performed only when posterior segment pathology was suspected. Computed tomography scan was performed when an IOFB or orbital fracture was suspected. 4
14. Lescher S, Wickmann V, Hofstetter P, Porto L. Paediatric patients with sudden vision impairment - An overview of MRI findings. European Journal of Paediatric Neurology. 20(4):616-24, 2016 Jul. Review/Other-Dx 95 children (47 male, 48 female) The purpose of this study was first to evaluate non-traumatic (tumoural and non-tumoural) causes of acute vision impairment; and, second, to assess whether conventional cerebral and orbital magnetic resonance (MR) imaging is helpful for children with sudden visual impairment. Patients with acute visual impairment were usually older than 7 years. In 36% of the patients a correlation between the MRI findings and the clinical symptoms was found. The most common causes of visual impairment were: infectious diseases (16%), migraine (12%), autoimmune diseases (11%), optic nerve neuritis with unknown aetiology (8%), neoplasms (8%), idiopathic intracranial hypertension (5%) and orthostatic hypotension (4%). Still, in 23% of the patients the cause remained unclear. 4
15. Daniel MC, Coughtrey A, Heyman I, Dahlmann-Noor AH. Medically unexplained visual loss in children and young people: an observational single site study of incidence and outcomes. Eye (Lond) 2017;31:1068-73. Observational-Dx 85 patients To determine the incidence of medically unexplained visual loss (MUVL) in children in an open access children's eye casualty. We identified 85 cases of MUVL (54 females; median age: 9 years (IQR 7-12)). The median duration of follow-up was 1.2 months (IQR 0-4.3). The estimated annual incidence was 3.5% (95% confidence interval 2.9-4.4%). Thirty-three per cent of children had a history of psychiatric disorders, reported a stressful life event, or showed signs of psychiatric disorder at the time of first presentation. The recovery rate was 25%. Median improvement in best-corrected visual acuity from presentation to last appointment was 0.22 (IQR 0.06-0.43) logMAR. 3
16. Toldo I, Pinello L, Suppiej A, et al. Nonorganic (psychogenic) visual loss in children: a retrospective series. J Neuroophthalmol 2010;30:26-30. Review/Other-Dx 58 patients To investigate the clinical characteristics, outcome, and prognostic indicators in a large series of children who received this diagnosis, nonorganic (psychogenic) visual loss in children at a single institution. Visual deficits consisted mostly of reduced visual acuity (76%) and visual field defects (48%). The diagnosis of nonorganic visual loss could be reached with confidence by means of observing inconsistent performance on a wide array of visual function tests, and, in doubtful cases, by means of electrophysiologic investigations. The mean time from onset to diagnosis was 3.1 months. The mean duration of visual symptoms from reported onset to disappearance was 7.4 months. Complete resolution of all visual symptoms occurred in 93% of patients and did so within 12 months of diagnosis in 85% of patients. There was no correlation between the duration of visual symptoms and age at onset, sex, time to diagnosis, type of ocular symptoms, or presence of psychosocial or psychologic difficulties. 4
17. Filippi M, Rocca MA, Ciccarelli O, et al. MRI criteria for the diagnosis of multiple sclerosis: MAGNIMS consensus guidelines. [Review]. Lancet neurol.. 15(3):292-303, 2016 Mar. Review/Other-Dx N/A To provide consensus guidelines for the magnetic resonance imaging (MRI) criteria in the diagnosis of multiple sclerosis. No results stated in abstract. 4
18. Mushlin AI, Detsky AS, Phelps CE, et al. The accuracy of magnetic resonance imaging in patients with suspected multiple sclerosis. The Rochester-Toronto Magnetic Resonance Imaging Study Group. JAMA 1993;269:3146-51. Observational-Dx 303 patients To design and implement a methodologically rigorous study to examine the accuracy of magnetic resonance imaging (MRI) in a patient population clinically suspected of having multiple sclerosis (MS). Magnetic resonance imaging of the head was considerably more accurate than CT in diagnosing MS. The area under the receiver-operating characteristic curve for MS was 0.82 (compared with 0.52 for CT) indicating that MRI was a good but not definitively accurate test for MS. A "definite MS" reading on an MRI of the head was specific for MS (likelihood ratio, 24.9) and essentially established the diagnosis, especially in patients clinically designated as "probable MS" before testing. However, MRI of the head was negative for MS in 25% and equivocal in 40% of the patients considered to have MS by the diagnostic review committee (sensitivity, 58%). 2
19. Al Othman B, Raabe J, Kini A, Lee AG. Neuroradiology for ophthalmologists. Eye (Lond) 2020;34:1027-38. Review/Other-Dx N/A To review the best approaches to neuroimaging for specific ophthalmologic conditions and discuss characteristic radiographic findings. No results stated in abstract. 4
20. Kim HJ, Paul F, Lana-Peixoto MA, et al. MRI characteristics of neuromyelitis optica spectrum disorder: an international update. [Review]. Neurology. 84(11):1165-73, 2015 Mar 17.Neurology. 84(11):1165-73, 2015 Mar 17. Review/Other-Dx N/A To review the brain, optic nerve, and spinal cord MRI findings of NMO and its spectrum disorder (NMOSD). No results stated in the abstract. 4
21. Cotton F, Weiner HL, Jolesz FA, Guttmann CR. MRI contrast uptake in new lesions in relapsing-remitting MS followed at weekly intervals. Neurology 2003;60:640-6. Observational-Dx 26 patients To perform intervals of magnetic resonance imaging studies for patients with relapsing-remitting MS (RRMS) in a year-long longitudinal study. The average duration of Gd-DTPA enhancement in individual new lesions was 3.07 weeks (median, 2 weeks). Significant correlations were demonstrated between the duration of contrast enhancement or initial growth rates and lesion volumes. Different lesions in the same patient appeared to develop largely independent of each other and demonstrated a large range in the duration of enhancement during the acute phase of their evolution. 3
22. Tillema JM, Pirko I. Neuroradiological evaluation of demyelinating disease. Ther Adv Neurol Disord 2013;6:249-68. Review/Other-Dx N/A This review focuses on the imaging spectrum of acquired demyelinating disease. No results stated in the abstract. 4
23. Papageorgiou E, McLean RJ, Gottlob I. Nystagmus in childhood. Pediatr Neonatol 2014;55:341-51. Review/Other-Dx N/A To review nystagmus in childhood. No results stated in abstract. 4
24. Bertsch M, Floyd M, Kehoe T, Pfeifer W, Drack AV. The clinical evaluation of infantile nystagmus: What to do first and why. Ophthalmic Genet 2017;38:22-33. Review/Other-Dx 284 charts To determine the most common diagnoses in order to develop a testing algorithm. A total of 284 charts were identified; 202 met inclusion criteria. The three most common causes were Albinism (19%), Leber Congenital Amaurosis (LCA; 14%), and Non-LCA retinal dystrophy (13%). Anatomic retinal disorders comprised 10%, motor another 10%. The most common first test was MRI (74/202) with a diagnostic yield of 16%. For 28 MRI-first patients, nystagmus alone was the indication; for 46 MRI-first patients other neurologic signs were present. 0/28 nystagmus-only patients had a diagnostic MRI while 14/46 (30%) with neurologic signs did. The yield of ERG as first test was 56%, OCT 55%, and molecular genetic testing 47%. Overall, 90% of patients had an etiology identified. 4
25. Ehrt O. Infantile and acquired nystagmus in childhood. Eur J Paediatr Neurol 2012;16:567-72. Review/Other-Dx N/A To review infantile and acquired nystagmus in childhood. No results stated in abstract. 4
26. Lavery MA, O'Neill JF, Chu FC, Martyn LJ. Acquired nystagmus in early childhood: a presenting sign of intracranial tumor. Ophthalmology 1984;91:425-53. Review/Other-Dx 9 patients To analyze the medical histories of ten patients from seven medical centers. Nine patients presented before the age of 10 months. The nystagmus, primarily described as pendular and asymmetric, was difficult to differentiate from and therefore most often diagnosed as spasmus nutans . On average in the ten patients, the intracranial glioma was not recognized for 8.6 months after the onset of nystagmus. In the five diagnosed as spasmus nutans , the mean delay in recognizing the tumor was 14.5 months. Three associated clinical findings were present or developed in these patients to distinguish this entity from spasmus nutans : optic atrophy in all ten patients, poor feeding due to diencephalic syndrome in 5 of 10, and increased intracranial pressure with hydrocephalus in 3 of 10. The acquired nystagmus in these infants was evidence of a life-threatening chiasmal/ parachiasmal glioma. 4
27. Reginald AY, Tandon A, Donelyl J, Bartel U, Buncic R. Nystagmus as a presenting sign in optic nerve glioma: The last decade. J Am Assoc Pediatr Ophthalmol Strabismus 2010;14:e6. Review/Other-Dx 29 patients To present characteristics suspicious of a nonocular basis of nystagmus and suggested criteria for neuro-imaging are presented. 29 cases were identified with an average age of 7 months at onset of nystagmus and 9 months at diagnosis of OPG. OPG with unilateral nystagmus (n=17) presented earlier (average, 3 months) and showed significant conversion to bilateral nystagmus by 15 months. 7 cases had more typical horizontal (pendular or jerk) nystagmus, but rotary and multiplanar nystagmus was seen in 10 cases and atypical dysconjugate and seesaw nystagmus in 7 cases. The majority 20 cases had no other ocular involvement. Associated systemic signs were seen in 7 cases, neurofibromatosis in 4 cases. 4
28. Shaw FS, Kriss A, Russel-Eggitt I, Taylor D, Harris C. Diagnosing children presenting with asymmetric pendular nystagmus. Dev Med Child Neurol 2001;43:622-7. Review/Other-Dx 277 children To investigate the prevalence and diagnosis of patients presenting with asymmetric horizontal nystagmus to the Ophthalmology Department at Great Ormond Street Children's Hospital, London in order to assess the relative prevalence of serious intracranial pathology. Clinical records of 277children, presenting with congenital nystagmus over an 8-year period were reviewed. Nystagmus was asymmetric in 24 of 277 cases. Seven of these patients were diagnosed with spasmus nutans. This is a rare condition that is only diagnosed retrospectively based on the absence of any abnormal neuroimaging or electrophysiological findings. Twelve of 24 patients had intracranial pathology and all had abnormal visual evoked potentials (VEPs). Five patients were diagnosed with congenital sensory defect nystagmus including one with albinism, three with congenital cone dysfunction, and one with cone-rod dystrophy. This paper stresses that although neuroimaging is necessary in all patients presenting with asymmetric nystagmus, such nystagmus can also occur with retinal disease or albinism and indicates the importance of non-invasive VEP/ERG testing in all forms of nystagmus. 4
29. Batmanabane V, Heon E, Dai T, et al. The role of MR imaging in investigating isolated pediatric nystagmus. Pediatr Radiol. 46(12):1721-1727, 2016 Nov. Observational-Dx 148 children To assess the yield of MR imaging in isolated pediatric nystagmus and define a management algorithm to minimize avoidable MRI referrals and streamline MRI protocols. We included 85 boys and 63 girls (total 148, average age at MRI 4.24 ± 4.19 years). Twenty-three (15.5%) children had abnormal intracranial findings on MRI including abnormal signal lesions (4.1%; n=6), Chiari I malformations (3.4%; n=5) and optic pathway glioma (2.0%; n=3). The time of onset of nystagmus was not associated with an abnormal MRI (P=0.2). Seventy children underwent visual electrophysiology testing but this test could not predict abnormality at MRI, either (P=0.12). 3
30. Garone G, Suppiej A, Vanacore N, et al. Characteristics of Acute Nystagmus in the Pediatric Emergency Department. Pediatrics 2020;146. Observational-Dx 206 patients To describe the epidemiology, clinical features, underlying causes, and management of acute nystagmus (AN) in children. Our secondary aim was to identify clinical features associated with a higher risk of significant underlying neurologic abnormalities in children with AN to contribute to the improvement of its management in the emergency setting. A total of 206 patients with AN were included (male-to-female ratio: 1.01; mean age: 8 years 11 months). The most frequently associated symptoms were headache (43.2%) and vertigo (42.2%). Ataxia (17.5%) and strabismus (13.1%) were the most common neurologic signs. Migraine (25.7%) and vestibular disorders (14.1%) were the most common causes of AN. Idiopathic infantile nystagmus was the most common cause in infants <1 year of age. UCs accounted for 18.9% of all cases, mostly represented by brain tumors (8.3%). Accordant with the logistic model, cranial nerve deficits, ataxia, or strabismus were strongly associated with an underlying UC. Presence of vertigo or attribution of a nonurgent triage code was associated with a reduced risk of UCs. 3
31. Benson JC, Nascene D, Truwit C, McKinney AM. Septo-optic Dysplasia : Assessment of Associated Findings with Special Attention to the Olfactory Sulci and Tracts. Clin Neuroradiol. 29(3):505-513, 2019 Sep. Review/Other-Dx 33 patients To focus on olfactory sulcus and bulb-tract hypoplasia. Out of 41 septo-optic dysplasia patients 33 were included (mean age = 120.7 months), with 8 excluded due to isolated septum pellucidum absence (n = 5), isolated bilateral optic hypoplasia (n = 2), or inadequate imaging (n = 1). An olfactory sulcus was hypoplastic on one or both sides in 14/33 (42.4%). Olfactory bulb hypoplasia was noted in one or both tracts in 15/33 (45.4%). A significant correlation was found between degree of olfactory sulcal and bulb-tract hypoplasia (? = 0.528, p = 0.0009). Other anomalies were: anterior falx dysplasia (n = 16, 48.5%), incomplete hippocampal inversion (n = 14, 42.4%), polymicrogyria (n = 11, 33.3%), callosal complete or partial agenesis (n = 10, 30.3%), schizencephaly (n = 8, 24.2%), ectopic posterior pituitary (n = 6, 18.2%), and nodular heterotopia (n = 4, 12.1%). Of the age-matched control patients 10/33 (30.3%) had at least mild anterior falx hypoplasia, and 1 control patient was noted to have unilateral incomplete hippocampal inversion (IHI); none of the age-matched control patients had olfactory sulcus or bulb-tract hypoplasia. 4
32. Qian X, Fouzdar Jain S, Morgan LA, Kruse T, Cabrera M, Suh DW. Neuroimaging and endocrine disorders in paediatric optic nerve hypoplasia. Br J Ophthalmol. 102(7):906-910, 2018 07. Review/Other-Dx 77 patients To determine the risk factors and association between brain MRI findings, pituitary abnormalities and endocrine disorders with the presence of ONH. 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. 4
33. Ramakrishnaiah RH, Shelton JB, Glasier CM, Phillips PH. Reliability of magnetic resonance imaging for the detection of hypopituitarism in children with optic nerve hypoplasia. Ophthalmology. 121(1):387-391, 2014 Jan. Observational-Dx 101 children To determine the reliability of neurohypophyseal abnormalities on magnetic resonance imaging (MRI) for the detection of hypopituitarism in children with ONH. Neurohypophyseal abnormalities, including absent pituitary infundibulum, ectopic posterior pituitary bright spot, and absent posterior pituitary bright spot, occurred in 33 children. Magnetic resonance imaging disclosed neurohypophyseal abnormalities in 27 of the 28 children with hypopituitarism (sensitivity, 96%). A normal neurohypophysis occurred in 67 of 73 children with normal endocrinologic function (specificity, 92%). 2
34. Lenhart PD, Desai NK, Bruce BB, Hutchinson AK, Lambert SR. The role of magnetic resonance imaging in diagnosing optic nerve hypoplasia. Am J Ophthalmol. 158(6):1164-1171.e2, 2014 Dec. Observational-Dx 57 cases To establish objective lower limits of normal optic nerve (ON) size in children based on high-resolution orbital magnetic resonance imaging (MRI). Measurements were made in 26 cases of clinically confirmed ON hypoplasia and 31 controls (median age: ON hypoplasia, 1 year; controls, 5.5 years). Nine of 26 cases (35%) and 19 of 31 controls (61%) underwent high-resolution T2-weighted imaging of the orbits. Mean ON diameter was 1.36 mm (95% CI: 1.19-1.54; P < .001) smaller for clinically hypoplastic ONs than for controls. Optic nerve diameter increased by 0.05 mm per year of age (95% CI: 0.03-0.07; P < .001). A lower bound to the 95% prediction interval for normal optic nerves was (2.24 mm + 0.052 × [age in years]) mm and excluded all but 1 case. 2
35. Park ES, Park JB, Ra YS. Pediatric Glioma at the Optic Pathway and Thalamus. J Korean Neurosurg Soc 2018;61:352-62. Review/Other-Dx N/A To discuss the clinical features, treatment paradigms, and evolving concepts related to two types of pediatric gliomas affecting two main locations: the optic pathway and thalamus. No results listed in abstract. 4
36. Rasool N, Odel JG, Kazim M. Optic pathway glioma of childhood. Curr Opin Ophthalmol 2017;28:289-95. Review/Other-Dx N/A To review the epidemiology, clinical presentation, diagnosis, and management of these lesions in patients with and without neurofibromatosis type 1 (NF-1). Most commonly, patients diagnosed with OPG have NF-1 especially if the lesions are bilateral. Such lesions tend to have a relatively indolent course and at least 50% of patients have no evidence of visual loss. Rarely, children without NF-1 may sporadically develop OPG with such lesions often having a more aggressive nature and greater propensity for visual dysfunction. The gold standard for diagnosis and follow-up are thorough neuro-ophthalmic examinations with specific attention to visual acuity. Management must be individualized and may comprise conservative follow-up, chemotherapy, radiation and/or surgical intervention. 4
37. Cassina M, Frizziero L, Opocher E, et al. Optic Pathway Glioma in Type 1 Neurofibromatosis: Review of Its Pathogenesis, Diagnostic Assessment, and Treatment Recommendations. Cancers (Basel) 2019;11. Review/Other-Dx N/A To provide a comprehensive overview about the clinical management of NF1-associated OPG, focusing on the most recent advances from preclinical studies with genetically engineered models and the ongoing clinical trials. No results stated in abstract. 4
38. Maloney E, Stanescu AL, Perez FA, et al. Surveillance magnetic resonance imaging for isolated optic pathway gliomas: is gadolinium necessary? Pediatr Radiol 2018;48:1472-84. Observational-Dx 17 patients To determine surveillance Magnetic Resonance factors that motivate changes in tumor-directed therapies and extrapolate cost-efficacy of a non-contrast follow-up protocol. We included 17 neurofibromatosis type 1 (NF1) and 21 non-NF1 patients who underwent a median 16.9 and 24.3 cumulative contrast-enhanced MR exams over 7.7 years and 8.1 years of follow-up, respectively. Eight children (one with NF1) had intervention based on contrast-enhanced MR findings alone. For these eight, increased tumor size was the only common feature, and it was apparent on non-contrast T2 sequences. For the median patient, a non-contrast follow-up protocol could result in 15.9 (NF1) and 23.3 (non-NF1) fewer gadolinium-based contrast agent administrations, and a 39% lower yearly RVU burden. 3
39. Marsault P, Ducassou S, Menut F, Bessou P, Havez-Enjolras M, Chateil JF. Diagnostic performance of an unenhanced MRI exam for tumor follow-up of the optic pathway gliomas in children. Neuroradiology 2019;61:711-20. Observational-Dx 17 patients The aim of our work was to evaluate the diagnostic performance of an unenhanced magnetic resonance imaging (MRI) examination for the follow-up of optic pathway gliomas (OPG) in children. The mean age of patients was 5.4 ± 3.4 years and mean follow-up length 6.7 years. The mean number of MRI + C was 13.5 (SD 7.2). The sensitivity of unenhanced MRI for tumor follow-up was 84-88% (95% CI 63.9-97.5). The specificity was 91.3-100% (95% CI 72-100). The PPV was 91.7% for reader 1 and 100% for reader 2. The NVP was 87.5% for reader 1 and 85.2% for reader 2. There was an excellent inter-observer agreement regarding tumor progression: kappa coefficient of 0.87 (p < 0.001). Inter/intra-variability for percentage of tumor volume variation between two exams were good (correlation coefficients of 0.97 and 0.94). 2
40. Inger HE, Rogers DL, McGregor ML, Aylward SC, Reem RE. Diagnostic criteria in pediatric intracranial hypertension. Journal of Aapos: American Association for Pediatric Ophthalmology & Strabismus. 21(6):492-495.e2, 2017 Dec. Review/Other-Dx 50 patients T compare the diagnostic criteria for PTCS to the modified Dandy criteria and to identify limitations within both sets of criteria. Of the 50 patients, 31 (62%) met diagnostic criteria for definite PTCS, 10 (20%) met criteria for probable PTCS, and 9 patients (18%) failed to meet sufficient PTCS criteria for diagnosis. 4
41. Rook BS, Phillips PH. Pediatric pseudotumor cerebri. [Review]. Current Opinion in Ophthalmology. 27(5):416-9, 2016 Sep. Review/Other-Dx N/A To review the epidemiology, diagnosis, and treatment of pediatric pseudotumor cerebri. Revised diagnostic criteria define an opening cerebrospinal fluid pressure greater than 28 cm water as elevated in the pediatric population. Obesity is an important risk factor for primary and secondary PTC in post-pubertal children. Magnetic resonance imaging shows findings suggestive of elevated intracranial pressure in children with PTC, similar to those of adults with PTC. Diamox and weight loss are effective treatments for PTC patients with mild visual field loss. Severe papilledema, decreased vision, and optical coherence tomography measures at presentation identify patients at increased risk for subsequent visual loss. 4
42. Friedman DI, Liu GT, Digre KB. Revised diagnostic criteria for the pseudotumor cerebri syndrome in adults and children. Neurology. 81(13):1159-65, 2013 Sep 24. Review/Other-Dx N/A To provide diagnostic criteria for pseudotumor cerebri syndrome in adults and children. No results stated in abstract. 4
43. Gilbert AL, Heidary G. Update on the evaluation of pediatric idiopathic intracranial hypertension. [Review]. Curr Opin Ophthalmol. 27(6):493-497, 2016 Nov. Review/Other-Dx N/A To summarize relevant articles on the diagnostic tools used in evaluation and management of pediatric IIH. Studies suggest that characteristic pediatric IIH MRI findings include empty sella turcica, decreased pituitary gland size, optic nerve tortuosity, perioptic subarachnoid space enlargement, posterior globe flattering, and intraocular protrusion of the optic nerve head. On optical coherence tomography (OCT), increased retinal nerve fiber layer and macular thickness may be observed in children with IIH compared with controls. The retinal nerve fiber layer thickness seems to coincide with the severity of papilledema and may be more sensitive than funduscopy for detecting optic nerve head elevation. Research on ultrasound of the optic nerve shows increased size of the optic nerve sheath diameter in pediatric IIH patients, and this may correlate with increased opening pressure on lumbar puncture. 4
44. Rudloe TF, Harper MB, Prabhu SP, Rahbar R, Vanderveen D, Kimia AA. Acute periorbital infections: who needs emergent imaging?. Pediatrics. 125(4):e719-26, 2010 Apr. Observational-Dx 918 patients To identify predictors for intraorbital or intracranial abscess among children who present with signs or symptoms of periorbital infection. These predictors could be used to better target patients for emergent CT. Nine hundred eighteen patients were included; 298 underwent a CT scan, and of those, 111 were shown to have an abscess. Although proptosis, pain with external ocular movement, and ophthalmoplegia were associated with presence of an abscess, 56 (50.5%) patients with abscess did not experience these symptoms. Other variables associated with the presence of an abscess in multivariate analysis were a peripheral blood neutrophil count greater than 10,000/microL, absence of infectious conjunctivitis, periorbital edema, age greater than 3 years, and previous antibiotic therapy (P < .05 for all). Our recursive partitioning model identified all high-risk (44%) patients as well as a low-risk (0.4%-2%) group (Rsq = 0.27). 3
45. Suhaili DN, Goh BS, Gendeh BS. A ten year retrospective review of orbital complications secondary to acute sinusitis in children. Med J Malaysia. 65(1):49-52, 2010 Mar. Review/Other-Dx N/A To review the nature of orbital complication, clinical presentation and treatment modalities and outcome seen in children with acute sinusitis in a tertiary referral institute. No results stated in abstract. 4
46. Mathew AV, Craig E, Al-Mahmoud R, et al. Paediatric post-septal and pre-septal cellulitis: 10 years' experience at a tertiary-level children's hospital. Br J Radiol. 87(1033):20130503, 2014 Jan. Review/Other-Dx 125 patients To assess the incidence and complications of pre-septal (pre-SC) and post-septal (post-SC) cellulitis over 10 years. 71/125 (57%) patients had post-SC. 50/125 (40%) patients imaged for pre-SC/post-SC had orbital abscess; 44/50 (88%) of these involved the medial orbit. Patients can develop solely superior or inferior abscesses that are difficult to identify by axial imaging alone, hence coronal reformatted imaging is essential. 5/125 (4%) patients developed major complications (SOV/CST/empyema), hence imaging review of the head and cavernous sinus region is essential. A diagnosis of post-SC on CT should alert the radiologist because this diagnosis can be associated with an increased incidence (5/71, 7%) of complications. 4
47. Chawla B, Sharma S, Sen S, et al. Correlation between clinical features, magnetic resonance imaging, and histopathologic findings in retinoblastoma: a prospective study. Ophthalmology. 119(4):850-6, 2012 Apr. Observational-Dx 75 patients To correlate clinical features with histopathology findings in advanced intraocular retinoblastoma and to determine the diagnostic accuracy of magnetic resonance imaging (MRI) in detecting tumor invasion. A significant association was found between iris neovascularization and choroidal invasion (P = 0.032), intraocular pressure and optic nerve invasion (P = 0.034), and shallow anterior chamber and iris invasion (P = 0.021). Corneal diameter did not show any significant correlation with high-risk histopathology. On MRI, tumor volume showed a significant association with optic nerve invasion (P = 0.023). The accuracy of MRI in detecting choroidal invasion was 68% (sensitivity, 60%; specificity, 80%). Prelaminar invasion was correctly identified in 9 out of 15 eyes (accuracy, 84%; sensitivity, 60%; specificity, 90%), whereas the accuracy of MRI in detecting postlaminar invasion was 76% (sensitivity, 61.9%; specificity, 81.5%). Ciliary body invasion was correctly identified in 5 out of 7 eyes (accuracy, 93.3%; specificity, 95.6%) and scleral invasion in 5 out of 6 eyes (accuracy, 98.7%; specificity, 100%). 3
48. Galluzzi P, Hadjistilianou T, Cerase A, et al. MRI helps depict clinically undetectable risk factors in advanced stage retinoblastomas. Neuroradiol. j.. 28(1):53-61, 2015 Feb. Observational-Dx 28 patients To compare high-resolution MRI with histology in advanced stage retinoblastomas in which ophthalmoscopy and ultrasonography did not give an exhaustive depiction of the tumour and/or its extension. Significant values were obtained for: prelaminar optic nerve (ON) sensitivity (0.88), positive predictive value (PPV) (0.75) and negative predictive value (NPV) (0.71); post-laminar ON sensitivity (0.50), specificity (0.83), PPV (0.50) and NPV (0.83); overall choroid sensitivity (0.82), and massive choroid NPV (0.69); scleral specificity (1), and NPV (1). AASE correlated with iris neoangiogenesis in 14 out of 19 eyes, and showed significant values for: overall ON PPV (0.65), prelaminar ON sensitivity (0.65), and PPV (0.61), post-laminar ON NPV (0.64); overall choroid sensitivity (0.77), PPV (0.59) and NPV (0.73); scleral NPV (0.83); anterior segment sensitivity (1), and NPV (1). 2
49. Heran F, Berges O, Blustajn J, et al. Tumor pathology of the orbit. [Review]. Diagnostic and Interventional Imaging. 95(10):933-44, 2014 Oct. Review/Other-Dx N/A To review the tumor pathology of the orbit. No results stated in abstract. 4
50. Radhakrishnan V, Sharma S, Vishnubhatla S, Bakhshi S. MRI findings at baseline and after neoadjuvant chemotherapy in orbital retinoblastoma (IRSS stage III). Br J Ophthalmol. 97(1):52-8, 2013 Jan. Observational-Dx 28 patients To evaluate the role of MRI in International Retinoblastoma Staging System (IRSS) stage III retinoblastoma treated with NACT. The proposed staging at baseline and after NACT was able to predict event-free-survival (EFS) (p=0.005 and p <0.001, respectively) and overall survival (OS) (p=0.002 and p=0.001, respectively) using the log-rank test for trends. Patients with complete or partial response according to the proposed response evaluation criteria had significantly better EFS (p<0.001) and OS (p=0.024) than those who had stable or progressive disease. 3
51. American College of Radiology. ACR Appropriateness Criteria® Radiation Dose Assessment Introduction. Available at: https://www.acr.org/-/media/ACR/Files/Appropriateness-Criteria/RadiationDoseAssessmentIntro.pdf. Review/Other-Dx N/A To provide evidence-based guidelines on exposure of patients to ionizing radiation. No abstract available. 4
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