Good Morning All! 

1. Good Morning All! Morning Report: Tuesday, March 6th

2. Pediatric Idiopathic Intracranial Hypertension AKA: PseudotumorCerebri

3. Definition • Elevated ICP without any evidence of neurologic disease

4. Epidemiology Adults Children • Female predilection • Rare in adults older than 45 (most common ages 20-44) • Strong association with obesity • Female predilection after puberty • Rare before age 10 (before puberty) • Association with obesity increases with age *No racial predisposition or genetic locus

5. Pathogenesis • Elusive!! • Absence of an increase in ventricular size despite increased ICP also puzzling • Vision loss • Transmission of high ICP to optic nerve head axoplasmic stasis and microvascular compromise

6. Differential Diagnosis • Diagnosis of EXCLUSION!! • No other identifiable neurologic disease • Numerous “associations” with IIH • Nomenclature dictates that identifiable causative factors be excluded from the diagnosis of IIH and be referred to as “secondary causes of intracranial HTN.”

8. Clinical Evaluations • History • HA • Worse in AM • Awaken patient from sleep • Increase with Valsalva • Nausea/ vomiting • Ophthalmic symptoms • Decreased/ blurred vision • Diplopia • Transient visual obscurations • Other: ataxia, dizziness, neck/shoulder/back pain, stiff neck, facial or limb paresthesias, facial nerve palsy, pulsatile tinnitus

9. Clinical Evaluations • Physical Exam • Ophthalmologic exam • Papilledema*

10. Clinical Evaluations • Physical Exam • Ophthalmologic exam (con’t) • Afferent pupillary defect • Color vision defecit • Loss of visual acuity • Uni-or bilateral sixth nerve palsy • Third or fourth nerve paresis • Neurologic exam • Excluding ophthalmic findings, exam should be NORMAL!

11. Investigations • MRI/MRV • Imaging studies of choice for IIH • Exclude the possibility of herniation prior to LP (older children and adults) • Identify secondary causes of increased ICP • Sinus or venous thrombosis • Malignancy • Meningeal abnormalities • Gliomatosiscerebri • Should be normal except for signs of increased ICP • Ventricles should be of normal to small size

12. Investigations • CSF studies • Elevated opening pressure (>180-200 mm H20) • Normal cell count, protein and glucose • Absence of infection • Ancillary studies • Lots of ophtho specific testing that I have NEVER heard of….AND • Visual field testing • More sensitive than visual acuity and contrast sensitivity testing in the detection of worsening disease

13. Management • Medical • Acetazolamide • Carbonic anhydrase inhibitor that reduces CSF production • 25-100 mg/kg/d, max 2g/d • Contraindicated in sulfa allergy and in significant renal or liver dz • Furosemide • Can be used in combination with or instead of acetazolamide • Effect on CSF production weaker than acetazolamide • Corticosteroids • Should be administered with caution • Used in conjunction with diuretics to treat children who’s response to diuretics was poor

14. Management • Surgical • Optic nerve sheath decompression • CSF shunting • Weight management • +/- bariatric surgery

15. Prognosis and Follow-up • *Vision loss can be permanent!* • Predictors of vision loss in IIH • Recent weight gain • Subretinal hemorrhage • Significant visual field loss at presentation • HTN • High-grade papilledema • Disc appearance cannot be used to predict final outcome • CLOSE follow-up • 1 month intervals for 6-12 mos after the disease has stabilized, then less frequently for a minimum of 5 years

16. Thanks for your attention! Noon Conference: Mead Johnson Webinar, Pediatric Mental Health (LUNCH PROVIDED!!!)