Papilloedema and optic nerve disorders: Case

Framing the case

This twelve-year-old girl has the classic presentation of the idiopathic intracranial hypertension with the morning headache, the transient visual obscurations, the diplopia and the bilateral disc swelling. The framework that organises the case is the urgent neuroimaging, the Friedman criteria and the vision surveillance, and the first priority is the magnetic resonance imaging with the venography before the lumbar puncture, because the bilateral disc swelling is the raised intracranial pressure until proven otherwise. [1][2]

Confirming the diagnosis

The magnetic resonance imaging with the venography is performed urgently to exclude the tumour, the hydrocephalus and the venous sinus thrombosis. The lumbar puncture follows the normal neuroimaging, in the lateral decubitus position with the child relaxed and the legs extended, and the opening pressure is measured with the manometer before the fluid is removed. The opening pressure above two hundred and eighty millimetres of cerebrospinal fluid in the child confirms the raised pressure, and the normal composition excludes the meningitis and the inflammation. The Friedman criteria are fulfilled, and the idiopathic intracranial hypertension is the diagnosis after the secondary cause is excluded. [1][3]

Grading the papilloedema

The Frisén grading scale is applied to the disc swelling, and it drives the urgency and the surveillance. The grade one is the C-shaped halo that spares the temporal margin, the grade two is the complete halo, the grade three is the elevation with the halo and the early vessel deflection, the grade four is the partial obscuration of the major vessels, and the grade five is the complete obscuration of all the vessels. The visual acuity, the colour vision and the visual fields are assessed, with the optical coherence tomography of the retinal nerve fibre layer, and the enlarged blind spot and the field constriction are the early defects. [1][6]

Treatment

The treatment builds on the weight loss, the acetazolamide and the surveillance. The weight loss of the five to ten percent in the obese adolescent may resolve the papilloedema, and the dietetic and the lifestyle support are engaged. The acetazolamide, the carbonic anhydrase inhibitor, is given at approximately fifteen to twenty-five milligrams per kilogram per day in the divided doses, with the gradual titration and the monitoring for the paraesthesia, the taste disturbance and the metabolic acidosis. The topiramate is the alternative. The vision surveillance is non-negotiable, because the field loss may appear without the symptom. The optic nerve sheath fenestration and the cerebrospinal fluid shunt are reserved for the progressive vision loss despite the medical therapy. [2][3]

Contrasting the optic neuritis

The examiner asks the candidate to contrast this case with an eight-year-old boy who presents with the acute painful visual loss in the right eye. The optic neuritis presents with the acute visual loss, the pain on the eye movement, the dyschromatopsia and the relative afferent pupillary defect on the swinging-flashlight test, and it is the inflammation of the optic nerve rather than the raised pressure. The antibody testing for the myelin oligodendrocyte glycoprotein and the aquaporin-four and the magnetic resonance imaging of the brain and the orbits define the cause, and the intravenous methylprednisolone at twenty to thirty milligrams per kilogram per day to a maximum of one gram for three to five days is the first-line treatment. The myelin oligodendrocyte glycoprotein antibody disease recovers well but relapses, and the aquaporin-four antibody neuromyelitis optica spectrum disorder carries the poor recovery and demands the early maintenance immunotherapy. [8][9]

Communication and the family

The family is counselled honestly and with the realistic information about the diagnosis and the prognosis. The candidate names the idiopathic intracranial hypertension, explains that the weight loss and the acetazolamide resolve the papilloedema in the majority, and that the surveillance of the vision is the non-negotiable part of the care because the field loss may appear without the symptom. The parents are introduced to the multidisciplinary team, given a written plan, supported by the dietetic and the mental-health service, and taught the warning signs of the vision loss. The school and the educational liaison are engaged for the support. [2][3]