Retinoblastoma is the most common intraocular malignant tumor of childhood. Intra-arterial chemotherapy (IAC) is a recently popularized technique used for the treatment of retinoblastoma, to decrease mortality, increase preservation of the eye, and prevent blindness. Along with the extensive use of IAC, it is important to apply noninvasive examination methods to assess the activity of the tumor and the risk factors for disease dissemination without histopathological confirmation. There are few studies that have assessed the value of magnetic resonance imaging (MRI) in evaluating the efficacy and complications of IAC for retinoblastoma. We retrospectively analyzed the MRI features of 60 patients with unilateral retinoblastoma given the primary treatment of IAC from January 2014 to February 2016 in our hospital. Our study showed that MRI could well assess the decreased activity of the tumor after IAC, presenting with diminished tumor size, increased apparent diffusion coefficient (ADC) values (from 0.94 ± 0.24 × 10-3 mm2/s to 2.24 ± 0.40 × 10-3 mm2/s), and a reduced degree of enhancement of the tumor. Our study also showed that MRI can monitor the risk factors of abnormal enhancement of the postlaminar optic nerve, to avoid unnecessary enucleation. Meanwhile, the results showed that the main late complications after IAC included affected eyeball volume reduction, subretinal hemorrhage, vitreous hemorrhage, vitreous opacity, cataractous len, and choroidal vascular ischemia.

/pdf/the-value-of-mri-in-evaluating-the-efficacy-and-2mihn64v4f.pdf

The value of MRI in evaluating the efficacy and complications with the treatment of intra-arterial chemotherapy for retinoblastoma.

Retinoblastoma (RB) is the most common ocular cancer, and it typically presents before the age of 5 years in over 90% of cases. In high resource countries, RB patients tend to survive and retain their sight. This is not the case in low-resource countries because of late presentation and delayed intervention arising mostly from sociocultural and socioeconomic challenges. RB has no gender or racial predilection; the incidence is estimated as 1:16,000-1:18,000 live-births or 11/1 million children under 5 years. Most of the world's RB cases are found in Asia and Africa while most RB treatment centres are in America and Europe. RB is easy to detect by caregivers as a glowing white 'cat eye reflex' at night or when captured on camera. Health workers at primary care level can detect RB in early life if red reflex test and/or squint (Hirschberg) tests are deployed as part of wellness checks done especially during routine immunisation and well-baby clinics in the first 24 months of life. In most cases of RB, biopsies for histological confirmation are not required for diagnosis and treatment decisions to be made. Clinical information, ophthalmic evaluation and imaging modalities are typically used. There have been significant changes in the management of RB using various treatment modalities such as enucleation with orbital implant, use of chemotherapy delivered through intravenous, intravitreal, periocular and intra-arterial routes and targeted treatment with laser, cryotherapy and brachytherapy. Algorithm for management and development of the national RB program within the context of a low-resource country is presented from review of data extracted from Mendeley library, PubMed library, Google Scholar and One Network; full-text articles were mostly retrieved through the American Academy of Ophthalmology.

Contemporary management of retinoblastoma in the context of a low-resource country.

Preoperative MRI detection of post-laminar optic nerve invasion (PLONI) offers guidance in assessing the probability of total tumor resection, an estimation of the extent of surgery, and screening of candidates for eye-preserving therapies or neoadjuvant chemotherapies in the patients with retinoblastoma (RB). The purpose of this systematic review and meta-analysis was to evaluate the diagnostic performance of MRI for detecting PLONI in patients with RB and to demonstrate the factors that may influence the diagnostic performance. Ovid-MEDLINE and EMBASE databases were searched up to January 11, 2020, for studies identifying the diagnostic performance of MRI for detecting PLONI in patients with RB. The pooled sensitivity and specificity of all studies were calculated followed by meta-regression analysis. Twelve (1240 patients, 1255 enucleated globes) studies were included. The pooled sensitivity was 61%, and the pooled specificity was 88%. Higgins I2 statistic demonstrated moderate heterogeneity in the sensitivity (I2 = 72.23%) and specificity (I2 = 78.11%). Spearman correlation coefficient indicated the presence of a threshold effect. In the meta-regression, higher magnetic field strength (3 T than 1.5 T), performing fat suppression, and thinner slice thickness (< 3 mm) were factors causing heterogeneity and enhancing diagnostic power across the included studies. MR imaging was demonstrated to have acceptable diagnostic performance in detecting PLONI in patients with RB. The variation in the magnetic field strength and protocols was the main factor behind the heterogeneity across the included studies. Therefore, there is room for developing and optimizing the MR protocols for patients with RB.

Diagnostic performance of MRI of post-laminar optic nerve invasion detection in retinoblastoma: A systematic review and meta-analysis.

Retinoblastoma is the commonest intraocular pediatric neoplasm. With the recent scope of shifting to eye preserving therapies, preoperative MRI is becoming the primary noninvasive diagnostic method to confirm the diagnosis, assess the ocular coats and optic nerve, and detect extraocular tumor extension and intracranial primitive neuroectodermal tumor associated with retinoblastoma as funduscopy and ultrasound have limitations. Diffusion-weighted images (DWIs) detect abnormalities based on differences in tissue cellularity. Echoplanar-based (EP) DWI results in susceptibility artifacts and geometric distortions, which make DWI of the orbit a challenging technique. The purpose of our study is to evaluate preoperative magnetic resonance (MR) imaging diagnostic merits and indices in patients with retinoblastoma. Fifty-eight eyes were prospectively reviewed in 30 girls and 27 boys (mean age at diagnosis was 23 months ± 18.9) with retinoblastoma by two radiologists on T1-weighted images, fat saturated T2-weighted images, DWIs, ADC, and postcontrast T1-weighted MR images with and without fat suppression. All patients included were primarily treated by surgical enucleation; one girl had bilateral enucleation. MRI shows accuracy, sensitivity, specificity, NPV, and PPV; 86.2%, 95.3%, 60%, 81.8%, and 87.2% respectively for choroidal invasion; 75.8%, 58.8%, 82.9%, 83%, and 58.8% in prelaminar optic nerve invasion; and 86.2%, 85.2%, 87%, 87%, and 85% in postlaminar optic nerve invasion (PLONI) respectively. For sclera and extrascleral tumor invasion, MRI show accuracy, sensitivity, specificity, NPV, and PPV of 93%, 55.6%, 100%, 92.5%, 100%, and 98.3%, 100%, 98%,100%, 80% respectively. On DWIs, the vital solid tumor shows hyperintense signal compared to the vitreous. The difference between the mean ADC value of the solid enhancing tumor (0.3[0.12–0.8] × 10−3 s/mm2) and nonenhancing necrotic parts (1.4[1.0–1.8] × 10−3 s/mm2) was statistically significant (p < 0.001). No correlation was found between the ADC values and high risk features for metastasis. Abnormal anterior chamber enhancement shows no statistically significant correlation with main MRI and histopathology findings. Maximum tumor diameter in mm show statistically significant correlation with postlaminar optic nerve invasion (ρ = .002) and choroidal invasion (ρ = .007). Sensitivity and specificity of different tumor size cutoff values are calculated using the ROC analysis. High-resolution ocular MR imaging can reliably detect postlaminar optic nerve invasion, choroid, and extrascleral invasion; meanwhile, it shows low sensitivity in prelaminar optic nerve, ciliary body, and sclera invasion. Significantly lower ADC values in viable tumor than necrotic parts are recorded.

/pdf/high-resolution-mr-imaging-guidelines-in-retinoblastoma-3hub92bne9.pdf

High Resolution MR Imaging guidelines in retinoblastoma: prospective study correlated with histopathological results

Orbital disorders in children consist of varied pathologies affecting the orbits, orbital contents, visual pathway, and innervation of the extraocular or intraocular muscles. The underlying etiology of these disorders may be traumatic or nontraumatic. Presumed location of the lesion along with the additional findings, such as eye pain, swelling, exophthalmos/enophthalmos, erythema, conjunctival vascular dilatation, intraocular pressure, etc, help in determining if imaging is needed, modality of choice, and extent of coverage (orbits and/or head). Occasionally, clinical signs and symptoms may be nonspecific, and, in these cases, diagnostic imaging studies play a key role in depicting the nature and extent of the injury or disease. In this document, various clinical scenarios are discussed by which a child may present with an orbital or vision abnormality. Imaging studies that might be most appropriate (based on the best available evidence or expert consensus) in these clinical scenarios are also discussed. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision process support the systematic analysis of the medical literature from peer reviewed journals. Established methodology principles such as Grading of Recommendations Assessment, Development, and Evaluation or GRADE are adapted to evaluate the evidence. The RAND/UCLA Appropriateness Method User Manual provides the methodology to determine the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where peer reviewed literature is lacking or equivocal, experts may be the primary evidentiary source available to formulate a recommendation. 

ACR Appropriateness Criteria® Orbital Imaging and Vision Loss-Child

This study compared 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.

MRI of orbits and head in 28 retinoblastoma patients (28 eyes) treated with primary enucleation were evaluated. Iris neoangiogenesis, infiltrations of optic nerve, choroid, anterior segment and sclera suspected at MR and histology were compared. Abnormal anterior segment enhancement (AASE) was also correlated with histologically proven infiltrations. Brain images were also evaluated.

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). Odds ratios (OR) and accuracy were significant in scleral and prelaminar optic nerve infiltration. Brain examination was unremarkable in all cases.

High-resolution MRI may add important findings to clinical evaluation of advanced stage retinoblastomas.

MRI helps depict clinically undetectable risk factors in advanced stage retinoblastomas.

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MRI helps depict clinically undetectable risk factors in advanced stage retinoblastomas.