[Treatment and prognosis of idiopathic intracranial hypertension in children. Retrospective study (1995-2009) and literature review].

AIM

Idiopathic intracranial hypertension (IIH) may cause severe visual loss due to the optic nerve damage. Routine management involves mainly medical treatment. The aim of this study was to improve diagnosis and management of IIH in children.

METHODS

The medical records of all patients with definite IIH seen at the children's hospital of Toulouse between 1995 and 2009 were reviewed. Cases of secondary intracranial hypertension were included because they did not present any cerebral lesions and underwent a similar therapeutic approach. The clinical and ophthalmological data at the beginning and at the end of their treatment was collected.

RESULTS

Eighteen children were included in this study. The average age was 10 years and the sex-ratio was equal to 1. There were 3 cases of secondary idiopathic intracranial hypertension in this pediatric group. The main features encountered were headache (15 children) and diplopia (8 children). Abnormal neurological examination was found for 11 patients with abducens nerve paresis in 8 cases, rachialgia in 6 cases, and neurogenic pains (neuralgia, dysesthesia, paresthesia, hyperesthesia) in the other cases. Papilledema was noted in 16 patients. At the initial phase, loss of visual acuity was documented in 6 patients and altered visual field in nine patients. All patients had a medical treatment. When recurrence occurred, each new treatment was documented, for a total of 23 treatments analyzed. Lumbar puncture was the only treatment for 2 patients. In 16 cases, first-line treatment was acetazolamide and it was the second choice in 1 case, with an average dosage of 11.2mg/kg and a mean duration of 2.5 months (15 treatments could be analyzed). This treatment was effective in 11 cases out of 15. Steroids were the initial treatment in 4 cases and second-line treatment in 4 cases (after failed acetazolamide therapy). The dosage was 1.5-2mg/kg for a mean duration of 1.5 months (6 treatments could be analyzed). This treatment was effective in 5 patients out of 6. One patient had dual therapy. No surgical procedure was necessary in this pediatric cohort. Three patients presented relapses of IIH. The outcome was good with no residual visual impairment in the 13 patients analyzed. One patient was still under medication.

COMMENTS

Therapeutic management of IIH in a pediatric population is essentially medical, in some cases limited to lumbar puncture. The first-line treatment is acetazolamide, but this study shows that low doses and short duration are usually chosen. Doses must be increased and treatment prolonged to avoid the use of corticosteroids as a second-line treatment and prevent possible relapses that require close monitoring of visual function.

CONCLUSION

The visual prognosis is generally better for this age group compared to adults and no risk factors for visual sequelae were identified. A standardized protocol for management of IIH was proposed.