The effect of enucleation on orbital growth in patients with retinoblastoma.

PurposeTo measure orbital volume using serial magnetic resonance imaging (MRI) scans to determine the effect of enucleation on orbital growth over time.MethodsThe medical records of patients who underwent unilateral enucleation for retinoblastoma with a minimum of 2 MRIs were retrospectively reviewed. Orbital asymmetry was calculated using MRI measurements. Nonlinear and linear mixed effect regression models were used to predict the effect of age at time of enucleation on degree of orbital asymmetry.ResultsA total of 27 patients were included (mean age at enucleation, 2 years; range, 2.5 months to 5 years). Age at scan (P = 0.046) and age at enucleation (P = 0.0006) were found to have a significant effect on orbital asymmetry. Change in orbital asymmetry over time was more pronounced after enucleation in children enucleated at <1 (P < 0.0001) or <2 years of age (P = 0.0109). Younger age at enucleation was associated with a greater degree of asymmetry over time, although this effect was extinguished for patients enucleated after the age of 3 years.ConclusionsIn patients with retinoblastoma, enucleation with orbital implant before 3 years of age has a significant effect on orbital volume asymmetry. After the age of 3, this effect appears to be less detrimental to the degree of orbital asymmetry

Dark without pressure retinal changes in a paediatric age group

Background/objectivesWe aim to describe the clinical and imaging characteristics of young children with dark without pressure- a well-defined area of dark retina in the mid periphery associated with attenuated hyperreflective outer segment bands on spectral domain OCT.Subjects/methodsRetrospective medical chart review. We reviewed the charts and retinal images of children with dark without pressure (DWP) seen in the paediatric eye clinic that had undergone retinal imaging.ResultsWe identified 6 children (3 males, 3 females) ranging in age from 3 to 13 years with DWP lesions. These were an incidental finding in children referred for other disorders. The lesions spared the macula and were typically in the mid-peripheral retina. In those children who could cooperate with detailed imaging, the DWP lesions were hypoautofluorecent on fundus autofluorescence imaging and OCT imaging showed reduced reflectance of the outer segments and EZ line as has been described in older children and adults. In two of the six subjects a DWP lesion appeared in an area of previously normal fundus; in no subjects did the lesion resolve over the period of follow up.ConclusionsThe clinical and imaging characteristics of DWP lesions are similar in young children to those described in adults. The site of dysfunction appears to be at the level of the outer retina, but the precise cause is unknown. Recognition of this benign disorder will prevent patient anxiety and unnecessary further investigation

Congenital ciliary body cysts causing lens abnormalities and secondary angle closure glaucoma in a child

Purpose: To report a case of congenital ciliary body cysts causing microspherophakia, ectopia lentis, and secondary angle closure glaucoma in an infant. Observations: A 16-month-old male was found to have bilateral ciliary body cysts associated with zonular laxity or absence causing microspherophakia and ectopia lentis as demonstrated on multimodal imaging. Additionally, the patient had secondary angle closure glaucoma which was likely multi-factorial related to both lens abnormalities and anterior displacement of the iris from the cysts themselves. The patient underwent lensectomy and cyst removal which resulted in intraocular pressure stabilization and visual rehabilitation. Conclusions and Importance: Congenital ciliary body cysts are a rare cause of lens abnormalities and secondary angle closure glaucoma in children. Information regarding genetic underpinnings or systemic associations is limited

Congenital ciliary body cysts causing lens abnormalities and secondary angle closure glaucoma in a child

PurposeTo report a case of congenital ciliary body cysts causing microspherophakia, ectopia lentis, and secondary angle closure glaucoma in an infant.ObservationsA 16-month-old male was found to have bilateral ciliary body cysts associated with zonular laxity or absence causing microspherophakia and ectopia lentis as demonstrated on multimodal imaging. Additionally, the patient had secondary angle closure glaucoma which was likely multi-factorial related to both lens abnormalities and anterior displacement of the iris from the cysts themselves. The patient underwent lensectomy and cyst removal which resulted in intraocular pressure stabilization and visual rehabilitation.Conclusions and importanceCongenital ciliary body cysts are a rare cause of lens abnormalities and secondary angle closure glaucoma in children. Information regarding genetic underpinnings or systemic associations is limited