__________________________________ Idiopathic Intracranial Hypertension in Children: A Review of Eight Cases Hissah Al Abdulsalam1, Abdulrahman Albakr1, Saeed Hassan2, Muddathir H Hamad2 , Ikhlass Altweijri1, Fahad A Bashiri2, Daniah A. Al-Showaeir3 __________________________________ 1- Division of Neurosurgery, Department of Surgery, College of Medicine, King Saud University, Riyadh, Saudi Arabia 2- Division of Neurology, Departments of Pediatrics, King Khalid University Hospital, College of Medicine, King Saud University, Riyadh, Saudi Arabia 3- Neuro-ophthalmology division, Ophthalmology department, College of Medicine, King Saud University, Riyadh, Saudi Arabia

Introduction Idiopathic intracranial hypertension (IIH) is rarely encountered in children. It is characterized by increased intracranial pressure (ICP) without any evidence of underlying brain pathology, structural abnormalities, hydrocephalus, or any abnormal meningeal enhancement. * * Friedman DI, et al. Neurology. 2002.

Introduction In the United States, the annual incidence of IIH in children is 0.9 per 100,000 *, 0.6 per 100,000 in Nova Scotia and Prince Edward Island in Eastern Canada **, and 0.5 per 100,000 in Germany. *** *Schexnayder LK, et al. Current Paediatrics. 2006. **Bursztyn LLCD, et al. Can J Ophthalmol. 2014 ***Tibussek D, et al. Klin Padiatr. 2013

Introduction In a study that was done in Oman, the incidence of IIH in children below 15 years of age was 1.9 per 100,000; with an incidence of 2.96 per 100,000 in female children.* To date, no similar epidemiological study has been done to estimate the incidence of IIH in pediatrics in Saudi Arabia. *Idiculla T, et al. Oman J Ophthalmol. 2013.

Methodology This study aimed to review the clinical presentation, etiology, diagnosis, management and outcomes of children with IIH at our institute. We retrospectively reviewed and analyzed the charts of all pediatric patients diagnosed with IIH from 2010 to 2016. 8 cases were identified. We defined idiopathic intracranial hypertension based on the modified Dandy criteria.* We classified the patients into prepubertal vs pubertal. *Smith JL. J Clin Neuroophthalmol. 1985.

Results The mean age at presentation of these patients was approximately 10 years, ranging from 3 to 17 years. The male-to-female ratio for the total group was equal. In the prepubertal group, the male-to-female ratio was equal. However, In the pubertal group, 66.67% (n=2) were females. It is known

Results The most common presenting clinical symptoms were headache (6/8) and visual symptoms (6/8). Visual symptoms included: photophobia in 3 patients, diplopia in 2 patients, and blurred vision in 2 patient. Other symptoms such as vomiting, was reported in 3 patients.

Table 1. Summary of Clinical Picture, Associated conditions, Treatment and Follow-Up. Papilledema was found in (7/8) patients. Neurologic examination was unremarkable in all patients, except two patients who had 6th cranial nerve palsy. Abbreviations. M male; F female; FU follow- up; LP Lumbar puncture; OP opening pressure; AZ Acetazolamide; ND no data.

Table 1. Summary of Clinical Picture, Associated conditions, Treatment and Follow-Up. Lumbar puncture was done in all patients. Opening pressure ranged from 260 to 500 mm H2O. Abbreviations. M male; F female; FU follow- up; LP Lumbar puncture; OP opening pressure; AZ Acetazolamide; ND no data.

Results 7/8 were treated with acetazolamide. Five patients responded to acetazolamide alone, which was evident by resolution of papilledema and improved clinical picture. Two cases were treated by Topiramate.

Results Only 1 patient received corticosteroids, after failure of acetazolamide alone. The same patient subsequently had deterioration of vision despite full medical treatment and undergone recurrent therapeutic lumbar punctures. The length of follow-up ranged from 1 month to 3 years with a mean follow-up duration of 17 months.

Discussion Unlike IIH in adults, in pre-pubertal children, IIH is less commonly associated with obesity, and has no female predominance. * However, IIH in post-pubertal children is usually similar to the adult disease. ** *Balcer LJ, et al. Neurology. 1999. *Wall M, et al. JAMA Neurol. 2014. ** Rangwala LM, et al. Surv Ophthalmol. 2007.

Discussion Medical treatment is indicated initially which include carbonic anhydrase inhibitors. * The most commonly used carbonic anhydrase inhibitor is Acetazolamide and is usually used first. * Topiramate is also being used for treatment of IIH due to its analgesic effect and its effect on weight loss.** Friedman DI, et al. Neurology. 2002. ** Ko MW, et al. Horm Res Paediatr. 2010.

Discussion Surgical treatment in the form of optic nerve sheath fenestration (ONSF) and CSF diversion is indicated when there is failure of lowering ICP despite maximum medical treatment or if the visual function is deteriorating.* None of our patients in this series required surgical treatment . *Ko MW, Liu GT. Horm Res Paediatr. 2010.

Discussion The recurrence rate of IIH in children is reported in the literature to be reaching up to 22%. * During the follow-up period, only 1 patient (16%) had recurrence. *Kesler A, et al. J Child Neurol. 2002.

Conclusion IIH in children requires long-term treatment and follow-up but with early diagnosis and treatment, most children will have good prognosis with complete resolution of their symptoms. Our study adds to the body of evidence by reporting our institution’s experience in IIH in pediatrics.

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