Hydrocephalus Flashcards
(147 cards)
What is the definition of hydrocephalus?
An abnormal accumulation of CSF within the ventricles
What is the prevalence of hydrocephalus?
1-1.5% overall and 1/1000 for congenital hydrocephalus
What are the congenital causes of hydrocephalus?
Chiari 1 and 2 Aqueductal stenosis Dandy Walker malformation X-linked inherited disorder
What are the acquired causes of hydrocephalus?
Infection SAH / IVH Neoplastic obstruction Post-op (iatrogenic)
What are the symptoms or raised ICP?
Headache Papilloedema N&V Gait ataxia 6th nerve palsy Up-gaze palsy
How does hydrocephalus present in children?
Enlarged OFC Irritiability / poor feeding / poor head control Bulging fontanelle Frontal bossing Engorged scalp veins Sun setting 6th nerve palsy Crack pot skull Splaying of the sutures
What is the normal OFC?
Same as crown to rump length
When should changes in OFC signify further investigation?
If crossing centiles (curves) on growth chart OFC >2 S.D. above normal Head circumference out of proportion to body length
Through which mechanisms can hydrocephalus cause blindness?
Papilloedema causing optic nerve damage PCA infarction from herniation 3rd ventricle dilatation compressing the optic chiasm
What are Lundberg waves?
Describe mean ICP patterns in patients
A = mean wave ICP >50 mmHg lasting between 5-20 minutes. Suggests ICP exceeding cerebral compliance
B = mean wave ICP 20-50 lasting < 5 minutes. Seen in sleep.
C = mean wave ICP < 20 occuring every 10 seconds. Due to oscillations in baroR and chemoR control.
What are the characteristics of pregeniculate blindness?
Marked optic nerve atrophy Loss of pupillary reflex
What does postgeniculate injury cause?
Cortical blindness. May be associated with Anton’s syndrome (denial of the deficit) and Ridoch’s phenomenon (appreciation of moving but no stationary objects)
What is the blood supply to the occipital pole?
MCA and PCA - hence why there is macular sparing with PCA infarcts
What imaging features are suggestive of hydrocephalus?
- Temporal horn diameter >2mm with barely visible sylvian fissure 2. Temporal horn diameter >2mm and FH/ID ratio >0.5 3. Ballooning of the 3rd ventricle 4. Periventricular oedema 5. Evan’s ratio 6. Thinning or bowing of the corpus callosum
What is Evan’s ratio?
FH diameter divided by biparietal diameter. If >0.3 then suggests hydrocephalus
What are the causes of pseudohydrocephalus?
Ex vacuo hydrocephalus - due to cerebral atrophy Agenesis of the CC Septo-optic dysplasia Hydranencephaly
What is external hydrocephalus?
Enlarged subarachnoid spaces and increased OFCs. Usually ventricles are also mildly dilated.
What are the features of chronic hydrocephalus?
Beaten copper cranium Empty sella Erosion of the dorsum sella Macrocrania Atrophy of corpus callosum Suture diastasis or delayed closure in infants
How can external hydrocephalus be distinguished from chronic subdural collections?
Cortical vein sign - these are seen with external hydrocephalus but not with chronic subdural collections
What is the pathophysiology of X-linked hydrocephalus?
X-linked so only affects males. Females are asymptomatic carriers. L1CAM mutation - integrin cell adhesion molecule important for axonal migration. Causes L1 syndromes.
What are L1 syndromes?
CRASH = corpus callosum hypoplasia, retardation, adducted thumbs, spastic paralysis and hydrocephalus due to aqueductal stenosis. Other features are aphasia, shuffling gait, vermis hypoplasia etc
What feature is pathognomonic of L1 syndrome?
Rippled ventricular wall after VP shunt insertion
What is arrested hydrocephalus?
Where there is no progression or deleterious sequelae of the hydrocephalus (may decompensate in the future). There should be near normal ventricle size, normal head growth curve and continued development.
What groups of patients are most likely to develop a trapped 4th ventricle?
Chronic lateral ventricle shunting, Fungal intracranial infection and Dandy walker malformation
