aetiological diagnostic work up medication, including contraceptives? recent rapid weight gain?...
TRANSCRIPT
Aetiological diagnostic work up
Medication, including contraceptives?Recent rapid weight gain?Menstruational problems?
Current or recent infection?Any chronic illness?
HISTORY
Overweight? Fever? Signs of CNS-infection?
Focal neurological signs (suggestive for IIH: sixth nerve palsy)?
Reduced level of consciousness?High blood pressure?
Clinical examination
Laboratory
Na, Ca, Mg, phosphate, blood gases, BUN, glucose, AST, RBC, WBC, CRP,
T3, TSH
Associated or causative conditions
IMPORTANT NOTICE: Any prepubertal child and male adolescent has to be considered atypical and secondary intracranial hypertension has to be
suspected.
Medical disorders: Addison’s disease; Hypoparathyroidism; Hypo- and hyperthyroidism;
Vitamin D deficiency; Chronic obstructive pulmonary disease;
Right heart failure with pulmonary hypertension; Sleep apnoea; Renal
failure; severe iron deficiency anaemia; Thrombophilia
(Antiphospholipid-Syndrome); PCOS, SLE.
Medications: Tetracycline and related compounds; Nitrofuratoin; Chinolone; Vitamin A and related compounds; Anabolic steroids;
Corticosteroid withdrawal following prolonged administration
(including inhalative steroids); Growth hormone administration in deficient patients; Nalidixic acid, Lithium, Norplant_ levonorgestral
implant system
Obstruction to venous drainage:Cerebral venous sinus thrombosis;
Jugular vein thrombosis
(Post)infectious: Lyme-disease; post-varicella; ???
More comprehensive work-up is required if secondary intracranial hypertension is
suspected (see right column)
Establishing idiopathic intracranial hypertension
Exclude intracranial mass lesion, malformation, ventriculomegaly.
Exclude sinus venous thrombosis by MRV in every atypical case, poor treatment
response, relapse!
Brain imaging
Performed during the morning, in lateral decubitus position, calm child.
Sedation if required. Avoid ketamine and inhalitve
anaesthetics.
Check CSF opening pressure: >20 cm H2O is abnormal
Check for: Cell count, protein, glucose. Consider infectious work-up.
Consider repeated LP if presentation is suggestive for IIH but pressure is within normal limits, particularly in the young
child.
Lumbar puncture
Ophthalmology
Use age adjusted standardised visual field testing.
Use Papilledema Grading System Scale.Visus? Neuroophthalmology (VI palsy?).
Lit.: Pediatric Idiopathic Intracranial Hypertension. Surv Ophthalmol 52:597--617, 2007.
Therapy of idiopathic intracranial hypertension
Acetazolamid 15 (-100) mg/kg/day in 2 to 3 divided
doses
First choice
Contact neurosurgeon
No response
No response
More comprehensive work-up is required as secondary intracranial hypertension is
suspected.
Not tolerated
Replace by furosemide (0.3--0.6 mg/kg per day)
Consider corticosteroids
No response
Regular ophthalmological
follow-up.
Check for: visual acuity, colour vision,
visual field, papilledema
Regular ophthalmological
follow-up.
Check for: visual acuity, colour vision,
visual field, papilledema
No response/progression
No response/ progression
Side effects: GI upset; paresthesias involving the lips, fingers, and toes; anorexia; electrolyte imbalance (metabolic acidosis). Kidney stones are rare, aplastic anemia exceedingly rare
Acetazolamid + furosemide OR consider topiramate (1,5-3 mg/kg/d)
No response