Papilloedema with Neurological Deterioration

Optic disc swelling from raised intracranial pressure in a patient who is worsening neurologically (headache, vomiting, drowsiness, new focal deficit, seizure, falling consciousness). It is a neurosurgical and medical emergency that requires imaging and pressure control within minutes to hours, and ophthalmic input is limited to confirmation and documentation.

• Causes:
  • Mass lesions with oedema or obstructive hydrocephalus: primary and metastatic tumours, abscess, subdural or intracerebral haematoma, cerebellar lesions.
  • Cerebral venous sinus thrombosis; subarachnoid haemorrhage; meningitis and encephalitis; cryptococcal meningitis in HIV.
  • Malignant hypertension and posterior reversible encephalopathy syndrome, eclampsia.
  • Idiopathic intracranial hypertension with fulminant course.
  • Traumatic brain injury, hepatic encephalopathy, and acute hydrocephalus (shunt malfunction).
• Danger signs of impending herniation: unilateral dilated pupil with ptosis (uncal herniation), decreasing Glasgow Coma Scale score, Cushing triad (hypertension, bradycardia, irregular breathing), and posturing.

• Severe headache (worse lying and in the morning), repeated vomiting without nausea, confusion, drowsiness, irritability, and seizures; neck stiffness and fever in infection.
• Bilateral disc swelling with blurred margins, obscured vessels, haemorrhages and cotton wool spots; transient visual obscurations, enlarged blind spot, constricted fields, and sixth nerve palsy (false localising sign); papilloedema may be absent in the first 24 to 48 hours of an acute rise in pressure.
• Neurological: focal weakness, speech disturbance, gait ataxia, upgaze palsy (hydrocephalus), pupil changes, and meningism.
• Severe papilloedema with haemorrhage and exudate can occur in malignant hypertension with retinopathy, with blood pressure above 180/120 mmHg.

• Resuscitation first: airway, breathing, circulation, Glasgow Coma Scale, pupils, blood glucose, and blood pressure.
• Immediate non-contrast CT head (mass, haemorrhage, hydrocephalus, oedema, midline shift, dense venous sinus) with CT venography; urgent MRI brain with contrast and MR venography when stable.
• Lumbar puncture only after imaging has excluded mass effect or obstructive hydrocephalus; measure opening pressure and send CSF for cells, protein, glucose, culture, polymerase chain reaction, India ink or cryptococcal antigen, and cytology.
• Blood tests: full count, urea and electrolytes, liver function, glucose, clotting, blood cultures, HIV, ammonia, toxicology, pregnancy test, and inflammatory markers.
• Ophthalmic documentation (when the patient is stable): dilated fundus photographs, optical coherence tomography, visual acuity, pupils, and fields; do not delay neurosurgical care for these tests.
• Blood pressure monitoring and, in neurocritical care, intracranial pressure monitoring.

• Resuscitate and protect the brain: elevate the head to 30 degrees, keep the neck neutral, secure the airway if Glasgow Coma Scale is 8 or below, maintain oxygenation and normocarbia (PaCO2 35 to 40 mmHg; brief hyperventilation to 30 to 35 mmHg only as a bridge to definitive treatment), and avoid hypotension and hypoglycaemia.
• Osmotic therapy for raised pressure or herniation: mannitol 20 percent 0.25 to 1 g/kg IV over 15 to 20 minutes (repeat every 4 to 6 hours, maintaining serum osmolality below 320 mOsm/kg), or hypertonic saline 3 percent 2 to 5 mL/kg over 10 to 20 minutes (23.4 percent 30 mL via central line in emergencies, sodium target 145 to 155 mmol/L).
• Dexamethasone 10 mg IV then 4 mg every 6 hours for vasogenic oedema from tumour or abscess (avoid in head injury; in bacterial meningitis give 10 mg every 6 hours for 4 days beginning before or with the first antibiotic dose).
• Seizures: levetiracetam 40 to 60 mg/kg IV loading (maximum 4500 mg) or lorazepam 4 mg IV for active seizures, then maintenance.
• Cause specific care:
  • Neurosurgery for external ventricular drain, shunt revision, tumour resection, haematoma evacuation, or decompressive craniectomy.
  • Bacterial meningitis: ceftriaxone 2 g IV every 12 hours, vancomycin 15 to 20 mg/kg every 8 to 12 hours, plus ampicillin 2 g every 4 hours when Listeria is possible.
  • Cryptococcal meningitis: liposomal amphotericin B 3 to 4 mg/kg daily with flucytosine 100 mg/kg/day in 4 divided doses, and therapeutic lumbar punctures to keep pressure below 20 cmH2O.
  • Cerebral venous sinus thrombosis: low-molecular-weight heparin (enoxaparin 1 mg/kg twice daily) or unfractionated heparin infusion, even with haemorrhagic infarction, then oral anticoagulation for 3 to 12 months.
  • Malignant hypertension and posterior reversible encephalopathy syndrome: lower mean arterial pressure by no more than 20 to 25 percent in the first hour with nicardipine 5 mg/hour (titrate by 2.5 mg/hour every 15 minutes to 15 mg/hour) or labetalol 20 mg IV bolus then infusion 0.5 to 2 mg/min; eclampsia: magnesium sulphate 4 g IV over 5 to 15 minutes then 1 g/hour, with delivery planning.
  • Fulminant idiopathic intracranial hypertension: intravenous acetazolamide 500 mg, short course methylprednisolone 1 g daily for 3 days, and urgent cerebrospinal fluid diversion or optic nerve sheath fenestration for threatened vision.
• Admit to a neurocritical or high dependency unit with hourly neurological observations.
• Ophthalmic follow up when stable: visual fields and optical coherence tomography every 1 to 2 weeks until papilloedema resolves, and rehabilitation of residual visual loss.