Idiopathic Intracranial Hypertension (IIH) is a term commonly used to describe the association of intracranial pressure without clinical laboratory or radiological evidence of an intracranial, space occupying lesion. The incidence of IIH is variable and it ranges from 0.57 to 2/100.000 in the general population. IHH is a rare entity in pediatric patients.

Known as benign intracranial hypertension or pseudotumor cerebri, IIH is a diagnosis of exclusion made in the presence of papilledema, normal neuroimaging and cerebrospinal fluid analysis, but with an elevated cerebrospinal fluid pressure. The diagnosis of IIH is suspected on the basis of the patient’s and family history, and the clinical examination. To correctly diagnose IIH it is necessary to perform: neurologic examination, ophthalmoscopy, visual field assessment, ocular motility examination and brain MRI with venography. Previously, the modified Dandy criteria were used for this diagnosis in both adults and children (Table 1,2). However, Raangwala and Liu proposed new criteria to aid diagnosing for pseudotumor cerebri in prepuberal children, as presentation in this population can be quite different compared to adolescents and adults.

The pathophysiology of IIH is not clear: there are many hypotheses on the development of this condition, including alterations in liquor absorption and production, cerebral edema, abnormalities in cerebral vasomotor control and cerebral blood flow, and venus obstruction. Risk factors for the development of IHH are: obesity, adolescent age, female sex, intake of high dose Vitamin A derivatives (e.g. isotretinoin for acne), long-term tetracicline use, steroid and hormonal contraceptives. The most common presenting symptom is a severe headache, worsening in the morning and being exacerbated by the Valsalva maneuver. The other manifestations are: exotropia, horizontal diplopia, caused by cranial nerve VI palsy, decreased visual acuity or blurring central vision caused by papilledema, nausea and vomiting.   The most significant physical finding of IIH is bilateral disc edema secondary to the increased ICP.

The pseudotumor cerebri is usually a self limited condition but a delay in treatment can lead to optic atrophy and blindness. Medical treatment with acetazolamide in association with corticosteroids has been applied with success. It is rarely necessary to perform serial lumbar punctures, lumboperitoneal or ventriculoperitoneal shunt, subtemporal decompression or optic nerve sheath fenestration.

In conclusion, our case can be considered atypical as it involved a young child without any risk factors for IHH. This demonstrates that when presented with a case of severe and recurring headache, even in children, it is necessary to follow an accurate diagnostic protocol, in order to exclude any cerebral tumors, encephalithis, hydrocephalus and venous sinus trombosis. The diagnosis involves several professionals: the neuropediatrician, ophtalomologist and neuroradiologist. Regarding the therapy, our patient required a combined regimen with acetazolamide and steroids. Once we suspended the treatment with steroids, the headache recurred, without any other associated symptoms. A relapse was confirmed, and steroids were not prescribed again.

After 12 months the patient presented again with episodes of severe headache with ophtalmoscopy and neuroimaging positive for IIH. Since in this case medical therapy was ineffective in the first place, surgical treatment was deemed necessary. The long term follow-up confirmed that the correct therapeutic approach included surgical treatment, leading to a complete recovery as well as a prevention from severe and permanent complications such as progressive blindness from postpapilledema and optic atrophy.