Morning Report: Tuesday, March 6th

Good Morning All!

Pediatric Idiopathic Intracranial HypertensionAKA: Pseudotumor Cerebri

Elevated ICP without any evidence of neurologic disease

Definition

AdultsFemale predilectionRare 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

EpidemiologyChildren

*No racial predisposition or genetic locus

Elusive!!Absence of an increase in ventricular size despite increased ICP also puzzling

Vision lossTransmission of high ICP to optic nerve head axoplasmic stasis and microvascular compromise

Pathogenesis

Diagnosis of EXCLUSION!!No other identifiable neurologic disease

Numerous “associations” with IIHNomenclature dictates that identifiable

causative factors be excluded from the diagnosis of IIH and be referred to as “secondary causes of intracranial HTN.”

Differential Diagnosis

HistoryHA

Worse in AMAwaken patient from sleepIncrease with Valsalva

Nausea/ vomitingOphthalmic symptoms

Decreased/ blurred visionDiplopiaTransient visual obscurations

Other: ataxia, dizziness, neck/shoulder/back pain, stiff neck, facial or limb paresthesias, facial nerve palsy, pulsatile tinnitus

Clinical Evaluations

Physical ExamOphthalmologic exam

Papilledema*

Clinical Evaluations

Physical ExamOphthalmologic exam (con’t)

Afferent pupillary defectColor vision defecitLoss of visual acuityUni-or bilateral sixth nerve palsyThird or fourth nerve paresis

Neurologic examExcluding ophthalmic findings, exam should be

NORMAL!

Clinical Evaluations

MRI/MRVImaging studies of choice for IIH

Exclude the possibility of herniation prior to LP (older children and adults)

Identify secondary causes of increased ICPSinus or venous thrombosisMalignancyMeningeal abnormalitiesGliomatosis cerebri

Should be normal except for signs of increased ICPVentricles should be of normal to small size

Investigations

CSF studiesElevated opening pressure (>180-200 mm

H20) Normal cell count, protein and glucoseAbsence of infection

Ancillary studiesLots of ophtho specific testing that I have

NEVER heard of….ANDVisual field testing

More sensitive than visual acuity and contrast sensitivity testing in the detection of worsening disease

Investigations

MedicalAcetazolamide

Carbonic anhydrase inhibitor that reduces CSF production25-100 mg/kg/d, max 2g/dContraindicated in sulfa allergy and in significant renal or

liver dzFurosemide

Can be used in combination with or instead of acetazolamide

Effect on CSF production weaker than acetazolamideCorticosteroids

Should be administered with caution Used in conjunction with diuretics to treat children who’s

response to diuretics was poor

Management

SurgicalOptic nerve sheath decompressionCSF shunting

Weight management+/- bariatric surgery

Management

*Vision loss can be permanent!*Predictors of vision loss in IIH

Recent weight gainSubretinal hemorrhageSignificant visual field loss at presentation HTNHigh-grade papilledema

Disc appearance cannot be used to predict final outcome

CLOSE follow-up1 month intervals for 6-12 mos after the

disease has stabilized, then less frequently for a minimum of 5 years

Prognosis and Follow-up

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