Extensive intracranial calcifications and leukoencephalopathy are seen in both Coats plus and leukoencephalopathy with calcifications and cysts (LCC; Labrune syndrome). Coats plus syndrome is additionally characterized by the presence of bilateral retinal telangiectasia and exudates while LCC shows the progressive formation of parenchymal brain cysts. Despite these apparently distinguishing features, recent evidence suggests that Coats plus and LCC represent the same clinical entity with a common primary pathogenesis involving a small vessel obliterative microangiopathy. Here, we describe eight previously unreported cases, and present an update on one of the original Coats plus patients to highlight the emerging core clinical features of the "cerebroretinal microangiopathy with calcification and cysts" (CRMCC) phenotype.

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Those questions where consensus was not reached highlight areas of either poor evidence or contradicting evidence, and may help investigators identify possible research questions.

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Anterior segment imaging in the pediatric population using commercially available equipment is rewarding but can be challenging. Successful imaging requires familiarity with the imaging modality used, a positive attitude, and the ability to quickly develop rapport with children. In this review, we demonstrate how external and slitlamp photography, Scheimpflug imaging, handheld digital fundus camera, ultrasound biomicroscopy, and anterior segment optical coherence tomography can be valuable in the documentation, diagnosis, and management of pediatric anterior segment disease. Families understand their child's disease process when it is demonstrated photographically and feel more motivated and involved in their care. Compliance with treatment is often enhanced through this process.

We report on a 4-year-old boy who presented to the ophthalmology department for assessment of convergent strabismus. Ophthalmic examination showed a left morning glory optic disc anomaly and retinal detachment. Plain films obtained for investigation of short stature prior to ophthalmic examination revealed delayed bone age. Ophthalmological findings prompted CT and MRI imaging and angiographic investigations. Midline cranial defects and abnormal carotid circulation were identified. These findings may be associated with morning glory optic disc anomaly, and their association is often under-recognized. It is important that clinicians and radiologists be aware of this spectrum of disorders, as the vascular abnormalities may predispose the patient to transient ischemic attacks and strokes. Growth delay may result from hypopituitarism.

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