Papilloedema and optic nerve disorders: SAQ

This girl has the classic presentation of the idiopathic intracranial hypertension with the daily headache, the transient visual obscurations, the diplopia and the bilateral disc swelling, confirmed by the normal neuroimaging and the opening pressure of three hundred and forty millimetres of cerebrospinal fluid, and the task is to outline the diagnosis, the management, the surveillance and the surgical options, contrasting the idiopathic form with the optic neuritis of the myelin oligodendrocyte glycoprotein antibody disease. ^[1][2]

Question 1 (10 marks)

Outline the diagnosis and the initial management of this twelve-year-old girl with the papilloedema. ^[1]

A full-mark answer covers the diagnosis, the Friedman criteria, the visual assessment and the medical management. ^[2]

The diagnosis and the Friedman criteria (3 marks). The diagnosis is the idiopathic intracranial hypertension, formerly the pseudotumour cerebri, defined by the Friedman criteria of the bilateral papilloedema, the normal neurological examination except for the cranial-nerve palsy, the normal neuroimaging that excludes the tumour, the hydrocephalus and the venous cause, the normal cerebrospinal fluid composition, and the opening pressure above two hundred and eighty millimetres of cerebrospinal fluid in the child. The opening pressure of three hundred and forty millimetres confirms the raised pressure, and the normal composition excludes the meningitis and the inflammation, and the idiopathic label is given after the secondary cause is excluded. ^[1]

The visual assessment and the urgency (3 marks). The vision is the outcome that drives the urgency, and the visual acuity, the colour vision, the visual fields and the optical coherence tomography are performed at the baseline and at each visit. The transient visual obscurations and the diplopia of the sixth-nerve palsy are the classic symptoms, and the enlarged blind spot and the field constriction are the early defects. The surveillance is non-negotiable, because the field loss may appear without the symptom, and the secondary optic atrophy is the complication of the chronic untreated papilloedema. ^[2][3]

The medical management (4 marks). The weight loss is the cornerstone of the management in the obese adolescent, and the modest reduction of the five to ten percent may resolve the papilloedema. The acetazolamide is the carbonic anhydrase inhibitor that reduces the cerebrospinal fluid production, given at approximately fifteen to twenty-five milligrams per kilogram per day in the divided doses in the child, with the gradual titration and the monitoring for the paraesthesia, the taste disturbance and the metabolic acidosis. The topiramate is the alternative, with the additional weight-loss and the migraine benefit. The family is counselled about the diagnosis, the surveillance and the prognosis, and the school and the dietetic support are engaged. ^[2][3]