Hydrocéphalie | Clinique Omicron Québec
Pathophysiology, classification and etiologies
- Physiology of the CSF and mechanisms of hydrocephalus : LCS production: choroid plexuses of lateral ventricles (70-80 %) + ependyma + cerebral capillaries → flow rate: 0.35 mL/min → 500 mL/d → total LCS volume: 150 mL (of which 25 mL intracranial + 75 mL in subarachnoid spaces + 50 mL intrachidial) → complete renewal of LCS: 3-4 times per day → composition: water + proteins (0.15-0.45 g/L) + glucose (2/3 of blood glucose) + rare cells (<5 elements/mm³) → reabsorption: arachnoid granulations of Pacchioni (in superior longitudinal and transverse sinuses) → passive mechanism via pressure gradient between ICP and venous pressure → accessory pathways: olfactory nerve sheaths (nasal lymphatics) + spinal root sheaths + glymphatic system (Iliff 2012 - Science Translational Medicine : glymphatic system - peri-arterial pathway - plays a role in clearance of CSF and brain proteins, notably amyloid + glymphatic system dysfunction → link with HPN + Alzheimer's disease); hydrocephalus classification: obstructive (non-communicating) hydrocephalus: obstacle inside ventricular system → dilatation upstream of obstacle → frequent sites: aqueduct of Sylvius (narrowest - aqueduct stenosis = most frequent cause of congenital hydrocephalus) + foramen of Monro + 4th ventricle (tumor + malformation) + communicating hydrocephalus: obstruction outside ventricular system → in subarachnoid spaces or arachnoid granulations → CSF flows freely into ventricles → causes: meningitis (fibrosis of granulations) + SAH (blood in subarachnoid spaces → obstruction) + meningeal carcinomatosis → normal pressure hydrocephalus (NPH): variant of communicating hydrocephalus → normal or intermittent ICP → exact mechanism debated (reduced cerebral compliance + glymphatic dysfunction) → ex-vacuo hydrocephalus: cerebral atrophy → passive expansion of ventricles → normal ICP → not true hydrocephalus (no active accumulation of CSF) → important to distinguish from true hydrocephalus → no surgical treatment; Monro-Kellie's law: constant intracranial volume (rigid bone box in adults) → increase in CSF → initial compensation by reduction in cerebral blood volume → then increase in ICP → in children (open fontanelles + unwelded sutures): skull expansion possible → macrocephaly → less acute presentation → bulging fontanelle
- Aetiologies by age - pediatric, young adult and older adult : congenital and pediatric hydrocephalus (prevalence: 1-2/1,000 births): stenosis of the aqueduct of Sylvius: most frequent cause of congenital hydrocephalus (43 % - Rekate 2011 - Journal of Neurosurgery Pediatrics) → X-linked genetic forms (L1CAM mutation - chromosome Xq28) → sporadic forms + Chiari type II malformation (Arnold-Chiari): herniation of cerebellar tonsils + brain stem through foramen magnum → often associated with myelomeningocele (spina bifida aperta) → obstruction of 4th ventricle → hydrocephalus in 90 % of cases + Dandy-Walker malformation: absence or hypoplasia of the cerebellar vermis + posterior fossa cyst + dilatation of the 4th ventricle → hydrocephalus + perinatal infections: CMV + toxoplasmosis + rubella → fetal meningoencephalitis → arachnoid fibrosis + intraventricular hemorrhage (HIV) of the premature baby: HIV grade III-IV according to Papile → post-hemorrhagic fibrosis → post-hemorrhagic hydrocephalus → severe complication of prematurity → acute presentation within 2-4 weeks post-IVD + prematurity <32 weeks: leading acquired pediatric cause in Canada; young adult hydrocephalus - acquired causes: intracranial tumors: medulloblastoma (posterior fossa ++) + ependymoma + trunk gliomas → 4th ventricle or aqueduct obstruction + bacterial or tuberculous meningitis → arachnoid fibrosis → communicating hydrocephalus + subarachnoid hemorrhage (SAH): blood in subarachnoid spaces → granulation obstruction → 20-30 % of SAH survivors develop chronic hydrocephalus + intraventricular hemorrhage (IVH) → direct obstruction + fibrosis + colloid cyst of the 3rd ventricle: benign tumor at the junction of the two foramens of Monro → intermittent acute obstruction → «Monro's foramen syndrome» → positional headache + sudden posture-dependent loss of consciousness → urgent neurosurgery + granulomatosis (sarcoidosis + meningeal tuberculosis) → communicating hydrocephalus; normal pressure hydrocephalus (NPH) of the elderly adult: prevalence: 5-11 % of dementias after age 65 (Jaraj 2014 - Neurology) → primary (idiopathic - iNPH): the majority of cases → mechanism: dysfunction of the glymphatic system + impaired venous compliance + deficient reabsorption of aging arachnoid granulations → secondary (sNPH): old SAH + head trauma + meningitis + intracranial surgery → Hakim-Adams triad: gait disorders (first + most important) + urinary incontinence + cognitive decline (in this order of onset and severity) + worldwide prevalence: idiopathic HPN: 14/100,000/year → underdiagnosed as mimicking dementia or Parkinson's disease
- Clinical presentation according to age and acuity : acute hydrocephalus (neurosurgical emergency): intracranial hypertension syndrome (ICHT): helmet headache + jet vomiting (not preceded by nausea) + visual disorders (diplopia - bilateral VI paralysis of false localization + visual blur + postural visual eclipses) + disorders of consciousness (somnolence → stupor → coma) + Parinaud's sign (damage to mesencephalic tegmentum by pressure exerted by dilated 3rd ventricle → paralysis of gaze elevation + palpebral retraction + sunset sign) → Cushing's sign (bradycardia + hypertension + Cheyne-Stokes) → imminent cerebral involvement → ABSOLUTE EMERGENCY → in infants: bulging fontanel + diastasis of cranial sutures + progressive macrocephaly + sunset eyes + acute crying + irritability + hypotonia → measure PC at every consultation from 0 to 2 years; chronic hydrocephalus - HPN: Hakim-Adams triad: 1. Gait disorders: apraxia of walking + walking with small steps + magnetic feet (difficulty taking feet off the ground) + widening of the sustentation polygon + instability + risk of repeated falls → anterior to dementia and incontinence in 80 % of cases → walking test: ask patient to walk 10 m → time → count steps → assess falls → 2. Urinary incontinence: first urgenturia + nocturia → then true incontinence + hyperactive micturition reflex → unstable bladder (on damage to frontal continence centers) → 3. Cognitive decline: frontal syndrome + psychomotor slowness + attention disorders + working memory → resembles subcortical dementia → distinguish from Alzheimer's and FTD → cognitive decline in HPN is potentially reversible unlike degenerative dementia → associated signs: frontal archaic reflexes (grasping + palmomental) + ideomotor apraxia + mild extrapyramidal syndrome + brain MRI: ventricular dilatation disproportionate to cortical atrophy (Evans index - ratio of maximum diameter of frontal horns / internal diameter of skull at same level - >0.3 = ventricular dilatation) + Sylvius sign (dilatation of perisylvian cortical sulci) + narrowing of sulci at vertex (tight high convexity) = MRI sign characteristic of HPN (Mori 2012 - Neurosurgery)
Diagnosis and surgical treatment
| Situation / treatment | Diagnosis and surgical strategy | Complications and follow-up |
|---|---|---|
| Diagnostic workup for hydrocephalus - imaging and functional tests CT - MRI - lumbar puncture - drainage test - Evans index |
First-line imaging: injection-free cerebral CT: emergency clearing examination → visible ventricular dilatation + approximate level of obstruction + eliminates acute hemorrhage + edema → CT indices: Evans index (>0.3 = significant dilatation) + ballooning of temporal horns of lateral ventricles + effacement of sulci at vertex + transependymal edema (coronal periventricular hypodensity) → sign of acute hypertension; cerebral MRI: reference examination → key sequences: T1 (anatomy) + T2/FLAIR (periventricular edema) + sagittal T2: status of the aqueduct of Sylvius (void flow = fast flow = permeable → absence of void flow = obstruction or stenosis) + axial T2: dilatation of temporal horns (very sensitive) + PC flow sequence (phase contrast): quantification of flow through aqueduct → HPN MRI: ventricular dilatation + Evans index >0.3 + tight high convexity (narrowing of vertex sulci) + dilatation of sylvian fissures + permeable aqueduct (void flow present) → HPN vs Alzheimer's: hippocampal atrophy predominant in Alzheimer → less proportional ventricular dilatation; functional tests for HPN - predictors of response to shunting: discharge lumbar puncture (LP) (tap test): withdrawal of 30-50 mL of CSF → gait assessment before and 1-4h after LP → gait improvement of >10-20 % → positive predictor of response to shunting (sensitivity 26-61 % - Marmarou 2005 - Neurosurgery: meta-analysis) → frequent false negatives → a negative LP does not exclude response to shunting + prolonged lumbar drainage (DLE - external lumbar drainage): 3-5 days of continuous drainage (10 mL/h) → gait monitoring + daily cognition → more sensitive than single LP (sensitivity 80-100 % depending on series - Walchenbach 2002 - Journal of Neurology Neurosurgery and Psychiatry) → functional gold standard for surgical selection in HPN → invasive method (risk of meningeal infection + hypodrainage) + LCS manometry (measurement of opening pressure): normal or slightly increased pressure in HPN (25 cmH₂O; selection of HPN patients for shunting: positive criteria for good response to shunting: complete triad present + predominance of gait disorders + positive DLE + absence of neurodegenerative comorbidities (Alzheimer's + advanced Parkinson's) + MRI: typical HPN signs + no marked hippocampal atrophy + short duration of symptoms (<3 years) → poor prognostic factors: isolated severe dementia in the foreground + comorbid Alzheimer's (frequent - 50 % of HPN have an associated degenerative pathology - Golomb 1994 - JAMA) + very advanced age + surgical frailty | CSF biomarkers in HPN and differential diagnosis: standard CSF (PL) in HPN: normal or slightly elevated proteins + normal glucose + normal cells → no diagnostic value for HPN itself → Alzheimer biomarkers in CSF: A-beta 42 + total tau + phospho-tau → if amyloid low + tau high → Alzheimer comorbidity likely → predicts poorer response to shunting (Hamilton 2010 - Neurology: low A-beta in CSF of HPN = Alzheimer comorbid → reduced response to shunting) + differential diagnosis of HPN: Parkinson's disease (resting tremor + bradykinesia + rigidity + good response to L-DOPA → HPN: no tremor + resistance to L-DOPA + normal or slightly reduced DATscan in HPN) → multisystem atrophy (MSA) + progressive supranuclear palsy (PSP) + Alzheimer's + vascular dementia → MRI + biomarkers + DATscan → standardized neuropsychological assessment: MMSE + MoCA + Dubois 10-word test + D-KEFS Trail Making Test → gait assessment: TUG (Timed Up and Go) + 10-meter test (step counting) → before and after tap test |
| Ventriculoperitoneal bypass (DVP) - reference treatment Ventricular catheter - valve - peritoneal catheter - programmable - complications |
Ventriculoperitoneal diversion (DVP) - technique and components: principle: divert CSF from the ventricles into the peritoneal cavity (where it is reabsorbed by the peritoneum) → components: proximal ventricular catheter (introduced into the frontal or occipital horn of the lateral ventricle) + pressure-regulating valve (subcutaneous in the mastoid or retroauricular region) + distal catheter (tunneled subcutaneously → thorax → abdomen → peritoneal cavity) → total length: 60-80 cm → 60-90 minute surgery under general anesthesia → guidance by stereotactic neurosurgery or neuronavigation → 24h post-operative CT scan: verification of proximal catheter position + start of ventricular decompression; DVP valves - types and settings: fixed-pressure valves: low pressure (valve opens at 10 cmH₂O) → choice according to preoperative ICP + programmable variable-pressure valves (Strata Medtronic + Certas Integra + Polaris B. Braun): non-invasive opening pressure adjustment by external magnetic field → major advantage: post-operative pressure adjustment without re-intervention → risk: accidental reprogramming by MRI or certain magnetic devices → check setting value after any MRI → anti-siphon valves (Codman Hakim + Diamond valve): prevent excessive drainage in standing position (orthostatic hypodrainage) + reservoir chamber valves (Ommaya reservoir): allows reservoir puncture for CSF dosing or drug injection; DVP efficacy in HPN: improvements observed: walking → improves first + best → in 70-90 % of cases → incontinence: improvement in 50-70 % + cognition: improvement in 40-60 % → improvement most uncertain → Hebb 2012 - Neurosurgery: meta-analysis DVP for HPN → overall improvement 60-80 % → long-term maintenance in 50-60 % at 2 years → possible recurrence due to valve dysfunction → risk of age-related surgical complications in treated population. | DVP complications - frequencies and management: proximal catheter obstruction (most common - 20-30 % at 2 years): fibrosis + astrocytes + choroid plexuses + clots → presentation: recurrence of hydrocephalus symptoms + ventricular dilatation on follow-up CT → valve test: reservoir chamber puncture → if normal pressure + CSF flows well → distal obstruction → if high pressure + little CSF → proximal obstruction → CT or MRI with flow sequences → surgical revision + shunt infection (5-10 % within 6 months): Staphylococcus epidermidis ++ + aureus + Propionibacterium acnes → subacute picture: fever + headache + meningismus + inflammatory signs along subcutaneous path → treatment: shunt explantation + external ventricular drainage (EVD) + antibiotic therapy IV 2-3 weeks + reimplantation after sterilization + overdrainage (15-30 %) : overdrainage → ventricular collapse → bilateral subdural hematoma (classically in elderly HPN) → orthostatic headache (worst in standing position → relieved lying down) → CT scan: ventricles small + subdural hematomas → increase valve set pressure or replace with anti-siphon valve → underdrag: maintain ventricular dilatation despite DVP → lower valve pressure + postoperative seizure + catheter disconnection or fracture + abdominal externalization + bowel dysmotility (rare) + intraperitoneal migration + LCS ascites + abdominal orifice stenosis |
| Endoscopic ventriculostomy of the 3rd ventricle (VCS - ETV) - alternative to DVP Obstructive hydrocephalus - neorifice - valveless - complications - ETV-CPC |
Endoscopic ventriculostomy of the 3rd ventricle (ETV - Endoscopic Third Ventriculostomy): principle: creation of an orifice in the floor of the 3rd ventricle → communication between the 3rd ventricle and the pre-mesencephalic cisterns → bypasses aqueduct obstruction → CSF shunted into subarachnoid spaces → reabsorbed normally → no implanted hardware → no valve → major advantage: no infectious risk from equipment + no valve obstruction → risk of stoma re-obstruction possible; ETV indications: non-communicating obstructive hydrocephalus (aqueduct stenosis + obstruction by posterior fossa tumor) → 1st-line treatment in these indications in adults + obstruction secondary to pineal tumor + astrocytoma of the 3rd ventricle → relieves HTIC while awaiting tumor treatment + post-HSA hydrocephalus + post-meningitis if the obstruction is in the aqueduct → contraindications: communicating hydrocephalus (ETV ineffective - reabsorption is deficient downstream → creating an orifice does not solve the problem) + HPN (communicating by definition → ETV not indicated) + floor of 3rd ventricle anatomically not feasible (thick + vascular) + infants <6 months (poorer response); ETV-SS score (Kulkarni 2009 - Journal of Neurosurgery): predictor of ETV success → age (infant 6 months = 30 pts) + etiology (MMC = 0 pts → aqueduct stenosis = 20 pts) + previous shunt status → score 0-90 → score 80 = excellent candidate; ETV combined with choroid plexus coagulation (ETV-CPC): special pediatric technique → in addition to ETV: choroid plexus coagulation to reduce CSF production → used in resource-limited countries and in some pediatric centers in North America → indicated in infants whose ETV-SS score is too low for ETV alone → Warf 2008 - Journal of Neurosurgery Pediatrics: ETV-CPC superior to DVP in sub-Saharan Africa for infantile hydrocephalus → 2-year success rate: 55-60 % vs DVP 45 % in this setting; endoscopic technique: ventricular trocar + rigid endoscope + entry through foramen of Monro → visualization of floor of 3rd ventricle → perforation + balloon opening (Fogarty ballooning) → CSF pulsation check + hemostatic control → no local anesthesia (GA) → complications: basilar artery bleeding (catastrophic but rare <1 %) + meningitis + early stoma failure (10-15 % in 1st year) | External ventricular drainage (EVD) - management of acute hydrocephalus: principle: temporary ventricular catheter → subcutaneous tunnel → external drainage system (graduated bags at ear height + 10-15 cmH₂O above external auditory meatus) → enables flow-controlled LCS drainage → ICP monitoring → daily LCS analysis (proteins + cells + cultures) → indication: acute, life-threatening hydrocephalus (post-HSA + post-HIV + post-meningitis + HIV grade IV prematurity) → while waiting for a definitive solution (DVP or ETV) or definitive treatment of a HSA (clip + coïl) → duration: 7-14 days (risk of ventriculitis increases after 14 days) → DVE infection rate: 5-10 % at 14 days → prophylactic antibiotic therapy under debate → DVE impregnated with antibiotics (Bactiseal - codman): reduction of infection → recommended by SNIS (Society of NeuroInterventional Surgery) + DVE reservoir (Ommaya reservoir): in premature babies with HIV → repeated punctures to drain CSF pending periventricular healing + stabilization → then DVP vs ETV evaluation according to evolution + ventriculo-atrial shunt (VAS): alternative to DVP if peritoneal cavity contraindicated (multiple abdominal surgeries + adhesions + ascites) → distal catheter in right atrium (internal jugular vein) → specific complications: endocarditis + pulmonary embolism (LCS aggregates + thrombus) + arrhythmias + lumbo-peritoneal shunt (DLP): alternative for HPN + benign intracranial HSI (pseudotumor cerebri) → catheter in lumbar space → drainage to peritoneum → risk of lumbar syphilization (iatrogenic disc herniation) → less used in pediatrics |
| Preterm and pediatric hydrocephalus - special features HIV grade III-IV - meningomyelocele - Chiari II - Dandy-Walker - Ommaya reservoir |
Post-hemorrhagic hydrocephalus of prematurity (PHVH): HIV grade III (hemorrhage with ventricular dilatation) and IV (parenchymal hemorrhage) of Papile classification → post-hemorrhagic hydrocephalus in 25-35 % of HIV grade III + 50-75 % of HIV grade IV → pathophysiology: blood in CSF → fibrin + degradation products → obstruction of arachnoid granulations + meningeal inflammatory reaction → chronic fibrosis → monitoring: transfontanellar ultrasound (ETF) → ventricular index measurements → biweekly monitoring → temporary treatment before definitive DVP (generally possible from 2-3 kg and 36-40 corrected weeks): Ommaya reservoir (subcutaneous) → repeated transparietal punctures (3-10 mL) → 2-3×/week → relieves pressure → wait for stabilization and growth → definitive DVP if progressive dilatation despite punctures + acetazolamide + furosemide: formerly used → DRIFT study (Kennedy 2007 - Lancet) and meta-analyses → no demonstrated benefit → abandoned + ongoing or active hemorrhage: contraindication to DVP (bloody CSF → immediate valve obstruction) → wait for CSF clarification (protein <1-2 g/L); meningomyelocele (MMC) and Chiari II: 90 % of MMC have associated hydrocephalus → spinal sac closure first (within 24-48h of birth or in utero - MOMS trial - Adzick 2011 - NEJM : fetal MMC surgery in utero at 19-25 weeks → reduced need for DVP from 82 % to 40 % at 12 months + improved neuromotor development) → secondary DVP if progressive hydrocephalus despite closure + Chiari II: hernia reduction → may improve hydrocephalus in some patients (indirect effect); developmental follow-up of child with hydrocephalus and DVP: regular neuropsychological assessment → IQ + language + executive functions + coordinations + behavioral disorders + adapted schooling → annual MRI (or more frequent if symptoms) → PC growth monitoring → parental education on signs of shunt dysfunction | Shunt dysfunction - warning signs for patient and family to recognize: shunt obstruction or infection = neurosurgical emergency → signs of shunt obstruction in children: progressive headaches + vomiting + drowsiness + irritability + sundowning eyes + bulging fontanel (if still open) + meningismus if infection + signs of obstruction in adults and HPN: recurrence of gait disturbances + incontinence + headaches + confusion + nausea + decreased vision → signs of overdrainage: orthostatic headaches (standing → worse → lying → better) + bilateral subdural hematomas → to be distinguished from obstruction → urgent CT → if obstruction → dilated ventricles → if overdrainage → small ventricles + subdural collections → DO NOT OPERATE RESERVOIR WITHOUT NEUROCHIRGIAN'S AGREEMENT if known patient with shunt → valve test (compression of reservoir chamber): if chamber quickly refills after compression → valve permeable downstream → if does not refill → distal obstruction → if chamber does not compress → proximal obstruction → these are assessed by neurosurgeon or specialist nurse; relative contraindications and precautions with DVP carriers: MRI: programmable valves can be deprogrammed by MRI → CHECK AND REPROGRAM VALVE AFTER EACH MRI → valve cards to be carried → some valves are MRI compatible (Codman Hakim Precision) → some require mandatory post-MRI verification + contact sport: not recommended if subcutaneous valve apparent + air travel: cabin pressure variation → little impact on modern valves → advise noting symptoms + physical activities: no major restrictions in modern DVPs → swimming pool (avoid pool water in ears if DVE in progress) |
Explosive headache + jet vomiting + altered consciousness + Parinaud's sign + Cushing's sign (bradycardia + hypertension + Cheyne-Stokes respiration) in a patient with known or suspected hydrocephalus → acute intracranial hypertension + threat of cerebral involvement → 911 call + urgent cerebral CT → emergency neurosurgery (DVE or valve puncture) → do not perform lumbar puncture (risk of involvement by abrupt decompression of posterior fossa).
Infant with bulging fontanel + rapidly progressing macrocephaly (increasing PC >2 standard deviations in 4 weeks) + sunset eyes + irritability + high-pitched crying → progressive hydrocephalus → pediatric emergencies → ETF + CT → pediatric neurosurgery → temporary DVE or Ommaya reservoir awaiting definitive DVP.
DVP patient with recurrence of hydrocephalus symptoms (headache + vomiting + visual disturbances + somnolence) + fever >38.5°C + meningismus → obstruction + shunt infection likely → emergencies → brain CT → reservoir chamber puncture by neurosurgeon → CSF culture → shunt explantation + DVE + inpatient antibiotic therapy.
Sudden positional headache + posture-dependent transient loss of consciousness + MRI showing a mass at the junction of the two foramens of Monro → colloid cyst of 3rd ventricle → intermittent obstruction of both foramens of Monro → possible sudden death → urgent neurosurgical surgery (endoscopy + excision or bypass).
Consult at Clinique Omicron
Clinique Omicron's physicians provide the initial assessment of a patient presenting with progressive headaches, gait disturbances or cognitive decline suggestive of hydrocephalus, coordinate the appropriate imaging tests and refer to neurosurgery depending on the results. Long-term monitoring of patients with shunts - clinical surveillance, recognition of signs of dysfunction - is provided at several points of service in Quebec. To book an appointment, visit cliniqueomicron.ca.
The contents of this page are provided for information purposes only and do not replace the advice of a physician or neurosurgeon. Any recurrence of symptoms in a patient with a ventriculoperitoneal shunt should be urgently evaluated.
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