Pseudotumour cerebri (PTC), or idiopathic intracranial hypertension (IIH), is a condition of increased intracranial pressure (ICP) without clinical, laboratory or radiological evidence of intracranial pathology. IIH is deemed a diagnosis of exclusion. The original diagnostic criteria were formulated in 1937 by Walter Dandy, 1 who described benign intracranial hypertension as the presence of increased ICP, normal cerebrospinal fluid (CSF) findings and no sign of a brain tumour on ventriculography. Corbett and Thompson 2 later removed the adjective benign and substituted idiopathic in light of the potential for devastating loss of vision.The name idiopathic intracranial hypertension indicates a condition with unknown aetiology. Over the years, a multitude of IIH cases and coinciding conditions and medications have been reported, many of which may have arisen by chance. [3][4][5][6][7] Hormone use has been implicated in many of these reports. Several studies have sought to evaluate the existence of these proposed associations; however, apart from obesity, no convincing evidence exists. CASE SUMMARYA 23-year-old trans-gendered male presented with a persistent headache for the past two months. The headache was worse in the morning, non-localised and exacerbated by coughing, yawning and supine positioning. In addition, for the past two weeks he had noted black spots in his vision as well as bilateral transient visual loss. The visual symptoms intensified with positional change, lasted no more than one minute, were more prominent in the peripheral visual field and were not accompanied by any photopsia. The patient denied having any numbness, extremity weakness, diplopia, nausea or vomiting. Furthermore, he reported no recent antibiotic use or changes in weight. Our patient presented with a body mass index (BMI) of 27.05, which is above the ideal BMI range of 18.5 to 25. He was currently taking testosterone cypionate intramuscularly on a weekly basis, prescribed by his endocrinologist as part of gender reassignment management. Past medical history was significant for a bilateral mastectomy.Visual acuity was 6/6 in the right eye and 6/6 in the left eye. Pupils were equal and reactive to light with no afferent pupillary defect. Colour vision, ocular motility, and Humphrey visual field testing were normal. Neurological screening revealed cranial nerves II through XII to be grossly intact. Intraocular pressures taken by Goldmann tonometry were 15 mmHg and 17 mmHg in the right and left eyes, respectively. The dilated fundus examination revealed florid bilateral disc oedema with flame-shaped haemorrhages and hard exudates (Figure 1). Optical coherence tomography (OCT) further supported the diagnosis of bilateral optic disc oedema (Figure 2).The patient was immediately referred for magnetic resonance imaging (MRI) and scheduled for neurological consultation. The MRI revealed a small Chiari I malformation as demonstrated by the cerebellar tonsils projecting below the level of the foramen magnum. Because no mass or lesion was fo...

SIGNIFICANCE Assessing and managing glaucoma are a complicated process in which experience plays a key role in decision making. Although advanced glaucoma is more easily diagnosed, patients with early glaucoma or who present with suspicious findings are more complicated. A need to aid clinicians in the decision-making process exists. PURPOSE The purpose of this study was to assess the impact of en face ocular coherence tomography images to clinical decision making when added to standard presentations of circumpapillary retinal nerve fiber layer thickness and automated perimetry. METHODS Thirty participants from two centers presenting either as a glaucoma suspect or for an initial glaucoma evaluation were enrolled. Six masked investigators were given standard presentations of circumpapillary retinal nerve fiber layer thickness and perimetry. They were asked if glaucomatous damage was present as well as a recommended plan of management on 5-point Likert scales. They were then given en face images of the retinal nerve fiber layer in three different presentations coupled with the standard presentation, and the questions were repeated. An intraclass correlation coefficient (ICC) was generated. RESULTS The masked investigators had moderate agreement from the standard presentation for assessment (ICC = 0.67 [95% confidence interval {CI}, 0.54 to 0.80] and ICC = 0.69 [95% CI, 0.52 to 0.80], respectively), as well as with the addition of the en face images (ICC = 0.69; 95% CI, 0.56 to 0.81). The en face images tended to change decisions in both assessment and plan toward likely to have glaucoma and likely to start treatment. CONCLUSIONS The addition of en face images to a standard presentation has an impact on clinical decision making. Although en face images seem to influence the decision toward likely to have glaucoma and likely to treat, it is unclear if this leads to a more accurate decision. Further investigations seem warranted to assess sensitivity and specificity of this approach.

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In this case, the patient presented with longstanding ocular surface disease known to be associated with this patient's inherited ectoderm disorder. In addition, this patient revealed a retinal tear, raising the possibility that patients with inherited congenital ectodermal dysplasia could be at risk for damaged structures originating from the neural ectoderm. In this heterogeneous disease, we are contributing to the existing literature a case of ectodermal dysplasia syndrome with obvious ectodermal complications that also had retinal findings leading us to speculate question if neural ectoderm could also be involved in this inherited disease.

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