The role of ICP monitoring in paediatric IIH.

INTRODUCTION: Diagnosis of idiopathic intracranial hypertension (IIH) in children is an extrapolation of the guidelines suggested for adult population. Lumbar puncture (LP) plays a crucial role in the diagnosis. The diagnosis of IIH at times is solely dependent on the interpretation of the opening pressure (OP). Unfortunately, LP-OP can vary due circumstantial parameters and therefore may be an unreliable form of intracranial pressure (ICP) measurement. Confirming the diagnosis based (as suggested by guidelines) on LP-OP in a doubtful clinical situation would be inappropriate. The aim of our study was to analyse the reliability of LP-OP and importance of ICP monitoring in situations where diagnosis of IIH was questionable. METHODS: Retrospective review of all children with diagnosis of IIH over a 10-year period was conducted. Children who underwent ICP monitoring (ICPM) were selected. We considered 2 LP-OP values-last LP (lLP) and the mean LP (mLP) for analysis. ICPM and LP-OP were compared. Follow-up till last clinic visit was also considered for long-term outcome. RESULTS: Eleven children (male 3; female 8) were included in the study. Mean delay between LP and ICPM was 112.8 days (17-257 days). There was lack of correlation between LP-OP and ICP in 9 children. ICP monitoring refuted the diagnosis of IIH in 80% of children and prevented exposure to unnecessary medical and surgical intervention. There was 1complication with ICPM. In 90% of children, there was no progression of symptoms following a decision based on ICP monitoring on long-term follow-up (mean, 36.5 months). CONCLUSION: When the diagnosis of IIH is in doubt, LP may be unreliable and formal ICP monitoring is advised.

Retinal astrocytoma regression in tuberous sclerosis patients treated with everolimus.

Everolimus is an inhibitor of the mammalian target of rapamycin (mTOR) that has been approved by the US Food and Drug Administration for the treatment of subependymal giant cell astrocytoma (SEGA) in patients with tuberous sclerosis complex (TSC). Retinal hamartomas, which are one of the major diagnostic features of TSC, tend to remain stable or gradually progress in the natural history of the disease. We report 2 patients with TSC treated with everolimus for SEGA in whom fundus photographs and spectral domain optical coherence tomography demonstrated regression of previously documented multiple retinal hamartomas in all 4 eyes.

Postural stability and visual impairment: Assessing balance in children with strabismus and amblyopia.

BACKGROUND: Vision plays an important role in controlling posture and balance in children. Reduced postural control has been reported in children with strabismus, but little has been reported specifically in amblyopia. OBJECTIVE: To investigate whether children with amblyopia have reduced balance compared to both children with strabismus without amblyopia and healthy controls. STUDY DESIGN AND METHODS: In this cross-sectional study, a total of 56 patients and healthy controls were recruited from the Ophthalmology and Otolaryngology Clinics at The Hospital for Sick Children, Toronto. Participants were divided into three groups: (1) 18 with unilateral amblyopia (strabismic amblyopia or mixed mechanism); (2) 16 with strabismus only without amblyopia; and (3) 22 visually-normal controls. The primary outcome was the balance performance as measured by the balance subtest of the Bruininks-Oseretsky Test of Motor Proficiency 2 [BOT2]. RESULTS: The age and gender-adjusted BOT2 balance scores were significantly reduced in the amblyopia group (mean score 9.0 ± 3.1 SD) and the strabismus without amblyopia group (mean score 8.6 ± 2.4 SD) compared to visually normal controls (mean score 18.9 ± 4.2) (p<0.0001), but no statistical difference was demonstrated between the two patient groups (p = 0.907). Further subgroup analysis of the strabismus only group did not reveal a statistically significant difference in performance on BOT2 balance score between strabismus only patients with good stereopsis 60 sec or better (BOT2 mean score 9.8±3.0 SD) to patients with 3000 sec or no stereopsis (BOT2 mean score 7.9±1.7) (p = 0.144). CONCLUSION: Our findings suggest that normal vision plays an important role in the development and maintenance of balance control. When normal binocular vision is disrupted in childhood in strabismus and/or amblyopia, not only is the vision affected, but balance is also reduced. Our results indicate that the presence of even mild binocular discordance/dysfunction (patients with intermittent strabismus and good stereopsis) may lead to postural instability.