Non-Operative Management of Benign Intracranial Hypertension Presenting with Complete Visual Loss and Deafness

Abstract

Benign intracranial hypertension (BIH) may lead to blindness and rarely deafness. We describe the case of a rapidly deteriorating 14-year-old African girl who presented with headaches associated with complete visual and hearing loss due to BIH. This was managed non-operatively with lumbar cerebrospinal fluid tap, weight reduction, nicotinic acid and acetazolamide. Response to treatment was quite dramatic with resolution of severe headaches and regaining of light perception 8 days after commencing treatment. By 3 months hearing recovered to normal and there was resolution of vision. This to the best of our knowledge is the first reported case of complete visual and hearing loss occurring in a patient with BIH, which was managed successfully non-operatively. When indicated, non-operative management is an effective treatment option even in malignant BIH.

A 24-year-old woman with rapidly progressing vision loss

“…5,6 Fulminant IIH is an acutely severe and rapidly progressive form of IIH, with resultant permanent visual sequelae. 3,[7][8][9][10][11][12][13] It is defined as acute onset of signs and symptoms of intracranial hypertension; (<4 weeks between onset of initial symptoms and severe visual loss), rapid worsening of vision loss over several days, and a normal MRI and MRV (or CT venogram). 3 There is limited literature on fulminant IIH.…”

“…3 There is limited literature on fulminant IIH. [8][9][10][11][12] The pathophysiology of IIH remains unknown. Vision loss in papilledema is thought to be secondary to elevated CSF pressure transmitted to the anterior optic nerve sheath, resulting in axoplasmic flow stasis and subsequent intraneuronal ischemia.…”

Pseudotumor cerebri in pediatric age: role of obesity in the management of neurological impairments

“…12,16 In the case of visual loss and papilledema, surgical treatment is required if medical treatment is not immediately effective and neurological defects are not improving. In view of the benefits of the lumbar cerebrospinal fluid tap, we decided to adopt a lumbar catheter for constant, continuous lumbar cerebrospinal fluid drainage, with a CSF flow of 10 ml/h.…”

“…15 The need for immediate treatment to lower intracranial hypertension in children with severe papilledema and visual loss, while avoiding major surgery, has only been sporadically reported in literature. 12,16 We decided to adopt a protocol of extensive external lumbar cerebrospinal fluid drainage in order to test the efficacy of this technique not only in emergent situations but also in definitive treatment of this rare, still unclear pathology.…”

Management of pediatric patients with pseudotumor cerebri

“…Decreased visual acuity and visual field occurs in 5-20% of patients at initial presentation, and the course is usually insidious [4,15,18,20]. Acute loss of vision is rare but constitutes a true medical emergency [3,[10][11][12][13]15] ( Table 2). In these isolated cases, the age of patients ranged between 11 and 26 years.…”