Accuracy of Computed Tomography and Magnetic Resonance Imaging for Detection of Pathologic Risk Factors in Patients Diagnosed with Retinoblastoma.

Aim: To assess and compare the diagnostic accuracy of magnetic resonance imaging (MRI) and computed tomography (CT) for detecting the extent of tumor invasion in eyes with advanced retinoblastoma prior to enucleation using histopathologic analysis as a reference.Methods: A total of 56 patients (68 eyes) enucleated for retinoblastoma were reviewed. Eyes with complete data (CT 28, MRI 16) were included for analysis. Imaging data were reviewed by a pediatric radiologist, blinded to histopathology results. Four high-risk factors which included scleral, choroidal, anterior eye segment invasion, and postlaminar optic nerve invasion were assessed.Results: For CT, the diagnostic odds ratio (DOR), sensitivity, and specificity for detecting postlaminar optic nerve invasion were 1.7%, 25%, and 83.3%. Choroidal invasion was correctly identified in only 2 of 10 eyes (DOR 0.5, sensitivity 20%, specificity 66.7%). For scleral invasion, CT showed a diagnostic ratio of 1.5%, sensitivity 40%, specificity 69.6% whereas for anterior segment invasion DOR was 9.2%, sensitivity 100%, and specificity 65.4%. MRI showed a higher DOR for detecting postlaminar optic nerve invasion of 39%, sensitivity 77.8%, and specificity 100%. Choroidal and scleral invasion on MRI had a DOR of 6.5% (sensitivity 25%, specificity 100%) and 6% (sensitivity 33.3%, specificity 92.3%) respectively while DOR for anterior segment invasion was 1.3% (sensitivity 50%, specificity 57.1%).Conclusion: CT showed a poor diagnostic accuracy for all four high-risk factors in advanced retinoblastoma and therefore may not be a useful tool for assessment of tumor extension. For MRI, detection of postlaminar optic nerve invasion was moderately accurate although less accurate for detection of other risk factors. Decision-making for each child should not be based on any single parameter but rather on consideration of clinical factors in combination with radiologic findings.

Association between severe retinopathy of prematurity and postnatal weight gain in very low-birthweight infants at Chiang Mai University Hospital, Thailand.

Background: Poor postnatal weight gain has been associated with low serum IGF-1, a key factor in the pathogenesis of retinopathy of prematurity (ROP).Aim: To investigate an association between relative weight gain (RWG) and severe ROP in very low-birthweight (VLBW) Thai infants.Methods: The medical records of VLBW infants who were admitted to the neonatal intensive care unit in Chiang Mai University Hospital from June 2014 to December 2016 and screened for ROP were reviewed. RWG and total calorie intake (TCI) in the 2nd, 4rth and 6th week of age were calculated and those with no ROP/mild ROP and severe ROP requiring laser treatment were compared.Results: The study included 139 VLBW infants, 24 (17.3%) of whom had ROP requiring laser treatment. Infants with severe ROP requiring laser treatment had a lower median birthweight (840 vs 1,195 g, p < 0.001) and median gestational age (GA) (27 vs 30 wk, p < 0.001) than those with no ROP/mild ROP. When RWG and TCI were compared, the infants with severe ROP requiring laser treatment had a lower RWG at the 2nd (p < 0.01) and 4th weeks of age (p < 0.05) and had a lower TCI at the 2nd week of age (p < 0.001) than those with no ROP/mild ROP. Multivariate logistic analysis demonstrated that GA <29.5 w (p < 0.01), hypotension (p < 0.05), RWG <2.9 g/kg/d (p < 0.05) and TCI <98.5 kcal/kg/day (p < 0.001) at the 2nd week of age were independent risk factors for severe ROP requiring laser treatment.Conclusions: Poor weight gain and low calorie intake at the 2nd week of age were associated with severe ROP requiring laser treatment in VLBW infants. Monitoring weight gain and calorie intake during this period are essential and may improve the outcome of ROP.Abbreviations: BPD, bronchopulmonary dysplasia; IVH, intraventricular haemorrhage; NEC, necrotising enterocolitis; PDA, patent ductus arteriosus; PRC, packed red cells; PVL, periventricular leucomalacia; RDS, respiratory distress syndrome; ROP, retinopathy of prematurity; RWG, relative weight gain; SGA, small for gestational age; TCI, total calorie intake; VLBW, very low birthweight.

Cutis laxa with pulmonary emphysema, conjunctivochalasis, nasolacrimal duct obstruction, abnormal hair, and a novel FBLN5 mutation.

We report on a 4-year-old girl with autosomal recessive cutis laxa, type IA, or pulmonary emphysema type (ARCL1A; OMIM #219100), with loose and wrinkled skin, mitral and tricuspid valve prolapse, conjunctivochalasis, obstructed nasolacrimal ducts, hypoplastic maxilla, and early childhood-onset pulmonary emphysema. Mutation analysis of FBLN5 showed a homozygous c.432C>G missense mutation, and heterozygosity in the parents. This is predicted to cause amino acid substitution p.Cys144Trp. Conjunctivochalasis or redundant folds of conjunctiva and obstructed nasolacrimal ducts have not been reported to be associated with FBLN5 mutations. Histopathological study of the conjunctival biopsy showed that most blood vessels had normal elastic fibers. The gingiva appeared normal, but histologically elastic fibers were defective. Scanning electron micrography of scalp hair demonstrated hypoplastic hair follicles. The cuticles appear intact underneath the filamentous meshwork.

A survey of pterygium surgery in Thailand 2010.

OBJECTIVE: To survey and investigate the pterygium surgery among Thai ophthalmologists in 2010. MATERIAL AND METHOD: Questionnaires were distributed to 930 ophthalmologists who were the member of the Ophthalmological Society and Royal Colleges of Ophthalmologists of Thailand. Data received from 414 respondents were assessed and analyzed RESULTS: The majority of respondents were male (53.1%) while 46.9 % were female. Visual disturbance (57.6%) was the most common indication for surgery. Most of the respondents (41.1%) preferred pterygium excision combined with conjunctival autograft transplantation. Bare scleral technique was the second most common preferential procedure. Recurrence was the main postoperative complication. CONCLUSION: Although there was a great variation pterygium surgical pattern, excision with conjunctival autograft transplantation was the major preferential practice. Recurrent pterygium was the most common complication.

Eye movement control and its disorders.

We review the supranuclear control centers and pathways leading to individual cranial nerve nuclei in the brainstem. We discuss horizontal and vertical gaze and their abnormalities, and review the cranial nerves which subserve eye movements, III, IV, VI, including their clinical testing. We highlight the sites at which these nerves are clinically affected, which often result in characteristic associated features with neurological localizing value. Differential diagnoses of cranial nerve palsies including Duane and Möbius syndromes are also described. Lastly, we discuss the nature of childhood neuromuscular junction disorders such as myasthenia gravis, as well as disorders of the muscle itself (chronic progressive external ophthalmoplegia (CPEO), thyroid orbitopathy).

The optic nerve and visual pathways.

We review the clinical examination of the optic disc and assessment of the integrity of the visual pathways: assessment of vision in the infant and child, assessment of disc size and configuration, detection of abnormalities such as cupping or disc swelling and their significance, visual field examination in the child, and the various field defects which result from pathology affecting the visual pathway anywhere along its course. Congenital anomalies of the optic disc, their systemic associations and significance are discussed. We also review the presentation of the visually impaired child, the significance of nystagmus in this context, the differential diagnosis and investigation.

The effect of changing oxygen saturation protocols on the incidence of laser treatment for retinopathy of prematurity.

OBJECTIVE: To compare the incidence of retinopathy of prematurity (ROP) requiring laser treatment among infants with birth weights of ≤ 1250 g before and after the implementation of a new oxygen saturation protocol in the neonatal intensive care unit of British Columbia Children's Hospital. DESIGN: Retrospective, comparative review of medical records. PARTICIPANTS: Premature infants with birth weights of ≤ 1250 g who were screened for retinopathy of prematurity from November 2003 to October 2005 and February 2006 to January 2008 (269 patients). METHODS: The hospital's ROP database was reviewed, and the patients were divided into 2 groups. The preprotocol group (November 2003-October 2005) consisted of patients who were screened before the new oxygen monitoring protocol was instituted and the postprotocol group (February 2006-January 2008) consisted of patients screened after its implementation. RESULTS: A total of 141 patients were in the preprotocol group and 128 patients in the postprotocol group. The mean birth weight and gestational age of the 2 groups were comparable (p = 0.81, 0.71, respectively). There was a 30% reduction in the incidence of laser treatment in the postprotocol group (preprotocol group = 22/141 [15.6%] vs postprotocol group = 13/128 [10.2%]; p = 0.19). CONCLUSIONS: We observed a decrease in the rate of severe ROP requiring laser treatment after the implementation of the new oxygen monitoring protocol. While not statistically significant, this is consistent with what has been found at other centres.