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Idiopathic intracranial hypertension and congenital ocular melanocytosis: a new association
  1. K Ziahosseini,
  2. D Mathews,
  3. S Biswas,
  4. I C Lloyd
  1. Paediatric Ophthalmology Department, Manchester Royal Eye Hospital, Oxford Road, Manchester, UK
  1. Miss K Ziahosseini, Flat 5, 86a Great Bridgewater Street, Manchester M1 5JG, UK; kim.z{at}

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Congenital ocular melanocytosis is a rare anomaly that requires regular follow-up, since it carries an increased risk of glaucoma and uveal malignant melanoma. Idiopathic intracranial hypertension (IIH) has several identifiable risk factors in children. We report a previously unrecognised association between the two conditions in a child.


A 5-year-old Caucasian girl was under regular follow-up for left congenital ocular melanocytosis since 1 year of age. The left eye showed iris hyperchromia, patches of grey subconjunctival pigmentation on the bulbar conjunctiva and choroidal hyperpigmentation (fig 1A).

Figure 1 (A) Left iris hyperchromia. (B) Bilateral optic disc oedema.

She had mild left anisometropic amblyopia that improved with patching and refractive error correction. Intraocular pressures (IOP) and optic discs were normal. She was healthy and …

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  • Competing interests: None.

  • Patient consent: Obtained.