Managing idiopathic intracranial hypertension in the eye clinic

Abstract

Idiopathic intracranial hypertension (IIH) is a neuro-ophthalmological condition characterised by a raised intracranial pressure and papilloedema that causes disabling headaches. The main risk factors of female sex and living with obesity have been known for some time, however the knowledge of the underlying pathophysiology is evolving. Papilloedema can impact the visual function, and the majority of people are offered acetazolamide. Those with sight threatening disease need urgent management, though there is little high quality evidence to recommend any particular surgical intervention. Headache treatment is an unmet clinical need and simple medication overuse advice has the potential to reduce the chronification of migraine-like headaches. IIH is emerging as a systemic metabolic disease distinct from people living with obesity alone. While weight loss is the main stay of disease modifying therapy this is challenging to access and many healthcare professionals that manage the condition have no formal training or accessible pathways for weight management. The aim of this "how to do it" article is to present the latest advances in knowledge of IIH that we pragmatically included in routine clinical care for people living with the condition.

Fig. 1. This figure shows Case 1 with mild papilloedema.

A colour fundus image of the left eye; and B right eye. Humphrey 30-2 perimetry showed mild enlargement of the blind spot bilaterally with very mild peripheral changes; C grey scale left eye; and D right eye.

Fig. 2. This figure shows Case 2 with moderate papilledema that then has worsening of their visual function and in whom acetazolamide is not tolerated.

A colour fundus image of the right eye; and B left eye. C the pRNFL print out with a global RNFL of right eye (OD) 259microns and left eye (OS) 249microns.

Fig. 3. This composite figure shows Case 2 with moderate papilledema that then has worsening of their visual function and in whom acetazolamide is not tolerated.

A HVF 30-2 of the left eye at the first visit; and B HVF 30-2 of the right eye at first visit. Both have enlargement of the blind spots, worse on the right. C HVF 30-2 of the left eye at the sight threatening visit; and D HVF 30-2 of the right eye when fulminant sight threatening disease is evident with progression of the superior loss and peripheral field constriction.

Fig. 4. This is a composite figure of Case 3 with severe papilloedema.

A colour fundus image of the left eye; and B right eye. Humphrey 30-2 perimetry showed mild enlargement of the blind spot bilaterally with very mild peripheral changes; C grey scale left eye; and D right eye.