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HYDROCEPHALUS
BY:
V.VIJAY ANAND
CRRI
GREEK – HYDRO {WATER} & KEPHALE {HEAD}
DEFINITION :
ABNORMAL EXCESSIVE ACCUMULATION OF CSF WITHIN
VENTRICLES/CAVITIES IN BRAIN
ABNORMAL DILATATION OF THE SPACES
POTENTIALLY HARMFUL PRESSURE ON THE TISSUE OF
THE BRAIN
Prevalance: 1-1.5%
Incidence: 0.3-3.5%
Upto 20% after SAH
1% after meningitis
CSF:
TOTAL VOLUME :
NEONATES : 50ml
ADULT : 150ml
{50%- CRANIAL CAVITY
50%- SPINAL CAVITY}
RATE :
NEWBORN -20ml/day
ADULT- 500ml/day
TURNOVER – 3TIMES/DAY
PRESSURE:
ADULT – 90-180mm H20
NEWBORN- 10-100mm H2O
RESPONSE OF INCREASED CSF:
 ABSORPTION - TRANSVENTRICULAR &
NERVE ROOT SLEEVES
 DILATATION – FRONTAL & TEMPORAL HORNS
 ELEVATION – CORPUS CALLOSUM
 THINNING – CEREBRAL MANTLE
 STRETCHING/PERFORATION – SEPTUM PELLUCIDUM
 ENLARGEMENT – THIRD VENTRICLE DOWNWARDS
PATHOGENESIS :
INCREASED PRODUCTION- CHOROID PLEXUS PAPPILOMA
OBSTRUCTION- INTERVENTRICULAR FORAMINA
(EXUDATES/ TUMOURS/ BLOOD CLOT)
- CONGENITAL
(STENOSIS/ ATRESIA)
- SECONDARY
(TUMOURS/ HAEMORRHAGE / INFECTIONS )
DECREASED ABSORPTION- OUTFLOW OBSTRUCTION
CAUSES :
CONGENITAL:
 INTRAUTERINE INFECTIONS
{TORCH}
 ARNOLD CHIARI MALFORMATION
 DANDY WALKER SYNDROME
 AGENESIS / ATRESIA / STENOSIS
 AV MALFORMATION
 BICKERS ADAMS SYNDROME
 CRANIAL DEFECTS
{ACHONDROPLASIA/ PLATYBASIA/ CRANIOSTENOSIS}
ACQUIRED :
 INFECTIONS
{ MENINGITIS/ MENINGOENCEPHALITIS/ CYSTICERCOSIS}
 MASS LESIONS
{NEOPLASM- ASTROCYTOMA/ MEDULLOBLASTOMA}
 INFLAMMATION {BRAIN ABSCESS}
 POST HAEMORRHAGIC{IVH/SAH/ INJURY}
 CHOROID PLEXUS PAPILLOMA
 SAGITTAL SINUS THROMBOSIS
 HYPERVITAMINOSIS A
 IDIOPATHIC
CLASSIFICATION :
1)COMMUNICATING TYPE :
 EXTERNAL / NON OBSTRUCTIVE TYPE
 OBSTRUCTION OUTSIDE THE VENTRICULAR SYSTEM
(IN BASAL CISTERNS/ARACHNOID VILLI)
 RESORPTION IMPAIRED DUE TO SCARRING/ FIBROSIS
2) NON COMMUNICATING TYPE :
 BLOCK WITHIN THE VENTRICULAR SYSTEM AT VARIOUS SITES
 MOST COMMON IN THIRD VENTRICLE AND AQUEDUCT
 VENTRICLES – DILATED
 EPENDYMA OF VENTRICLES MAY BE DISRUPTED
 CORTICAL ATROPHY (MORE ICP)
NORMAL PRESSURE HYDROCEPHALUS :
Post traumatic, post meningitic , SAH , deficieny of arachnoid granulations.
Intermittent raise of ICP
Enlarged ventricles ,normal CSF pressure ,cortical atrophy ,absence of papilledema.
Hakim triad - gait apraxia, incontinence, and dementia.
Headache is NOT a typical symptom in NPH, no sensory loss.
Magnetic gait, personality changes,
DTR increased, BABINSKI positive, release reflexes.
CLINICAL FEATURES :
Signs :
Macrocephaly
Dysjunction of sutures
Dilated scalp veins
Skin over scalp thin and shiny
MACEWEN SIGN
Tense/bulging fontanelle
SETTING-SUN SIGN
Pyramidal signs
Lateral rectus palsy
Papilledema
Bradycardia
Altered respiration
Cushing triad
Symptoms :
Poor feeding
High pitched cry
Irritability
Reduced activity
Vomiting
Headache
Bladder incontinence
Blurred vision
Drowsiness
Horizontal diplopia
PARINAUD SYNDROME
DIFFERENTIAL DIAGNOSIS :
 SUBDURAL EFFUSION
 CHRONIC SUBDURAL HAEMATOMA
 FAMILIAL MEGALENCEPHALY
 HYDRANENCEPHALY
 PSEUDOHYDROCEPHALUS
 PEUDOTUMOUR CEREBRI
ARRESTED HYDROCEPHALUS /
COMPENSATED HYDROCEPHALUS
no progression or deleterious sequelae requiring CSF shunting
- Near normal ventricular size, head growth curve
- Continued psychomotor development
SPECIAL FORMS :
“HYDROCEPHALUS EX-VACUO”
dilatation of the ventricles
CSF pressure is normal
Shrinkage of brain substance
Damage to the brain
Stroke or other form of injury
Chronic neuro degeneration
“EXTERNAL HYDROCEPHALUS /
BENIGN ENLARGEMENT OF THE EXTRA-AXIAL SPACES”
Excessive CSF-subarachnoid spaces
Ventricles -not enlarged significantly
Infant and early children.
Associated with familial macrocephaly
resolution within 1 year
ARRESTED HYDROCEPHALUS /
COMPENSATED HYDROCEPHALUS
no progression requiring CSF shunting
- Near normal ventricular size, head growth curve
Continued psychomotor development
INVESTIGATIONS :
 TORCH SCREENING
 CSF ANALYSIS , LP OPENING PRESSURE (OP)
 XRAY
 CT
 MRI
 USG FONTANELLE
 VENTRICULOGRAPHY
 PNEUMOENCEPHALOGRAPHY
CT/MRI FINDINGS:
 Temporal Horns > 2mm
 EVANS ratio
 Upward bowing of corpus callosum
 Erosion of sella turcica
 Ballooning of the Frontal Horns &
3rd Ventricles (Mickey Mouse sign)
TREATMENT :
MEDICAL:
DIURETICS:
Acetazolamide (Diamox) 50mg /kg/day
Furosemide (Lasix) 1mg/kg/day
Watch for electrolyte imbalance and
acetazolamide side effects:
Lethargy - tachypnea
diarrhoea - paresthesias
Perform weekly CT scan.
maintain therapy for a 6 month trial, then taper
dosage over 2-4 weeks
SURGICAL :
 Choroid Plexectomy
 Choroid plexus coagulation
 3rd Ventriculostomy
 Endoscopic fenestration of floor of third
ventricle
 Cerebral aqueductoplasty
 TAPPING: LP performed only in
communicating hydrocephalus.
 Surgical correction of the tumour/cysts
 Shunts
SHUNTING :
 VENTRICULO-
PERITONEAL SHUNTING
(VP SHUNTING) - growing children
 VENTRICULO-ATRIAL SHUNTING
(VA SHUNTING)
 LUMBO-PERITONEAL SHUNTING
(LP SHUNTING)
 VENTRICULO PLEURAL
SHUNTING
 VEBTRICULO CAVAL SHUNT
COMPLICATIONS OF SHUNTING:
 INFECTIONS
STAPH. EPIDERMIS AND STAPH. AUREUS.
 OBSTRUCTION OF THE CATHETER
 INTRACEREBRAL /SUBDURAL HEMORRHAGE
 OVER SHUNTING (VP SHUNTS)
 MISPLACEMENT
 SEIZURES
 CSF ASCITES
FOLLOW UP:
 HC MONITORING
 SIGNS OF INFECTION/BLOCK
 ANTIBIOTIC PROPHYLAXIS
 RE-EVALUATION
PROGNOSIS:
depends on primary cause
Large IVH- permanent hydrocephalus
NPH- responds to shunt
gait and incontinence respond to shunting
but dementia responds less frequently.
THANK YOU
VIJAY ANAND
CRRI
Hydrocephalus

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Hydrocephalus

  • 2. GREEK – HYDRO {WATER} & KEPHALE {HEAD} DEFINITION : ABNORMAL EXCESSIVE ACCUMULATION OF CSF WITHIN VENTRICLES/CAVITIES IN BRAIN ABNORMAL DILATATION OF THE SPACES POTENTIALLY HARMFUL PRESSURE ON THE TISSUE OF THE BRAIN
  • 3. Prevalance: 1-1.5% Incidence: 0.3-3.5% Upto 20% after SAH 1% after meningitis CSF: TOTAL VOLUME : NEONATES : 50ml ADULT : 150ml {50%- CRANIAL CAVITY 50%- SPINAL CAVITY} RATE : NEWBORN -20ml/day ADULT- 500ml/day TURNOVER – 3TIMES/DAY PRESSURE: ADULT – 90-180mm H20 NEWBORN- 10-100mm H2O
  • 4.
  • 5. RESPONSE OF INCREASED CSF:  ABSORPTION - TRANSVENTRICULAR & NERVE ROOT SLEEVES  DILATATION – FRONTAL & TEMPORAL HORNS  ELEVATION – CORPUS CALLOSUM  THINNING – CEREBRAL MANTLE  STRETCHING/PERFORATION – SEPTUM PELLUCIDUM  ENLARGEMENT – THIRD VENTRICLE DOWNWARDS
  • 6. PATHOGENESIS : INCREASED PRODUCTION- CHOROID PLEXUS PAPPILOMA OBSTRUCTION- INTERVENTRICULAR FORAMINA (EXUDATES/ TUMOURS/ BLOOD CLOT) - CONGENITAL (STENOSIS/ ATRESIA) - SECONDARY (TUMOURS/ HAEMORRHAGE / INFECTIONS ) DECREASED ABSORPTION- OUTFLOW OBSTRUCTION
  • 7. CAUSES : CONGENITAL:  INTRAUTERINE INFECTIONS {TORCH}  ARNOLD CHIARI MALFORMATION  DANDY WALKER SYNDROME  AGENESIS / ATRESIA / STENOSIS  AV MALFORMATION  BICKERS ADAMS SYNDROME  CRANIAL DEFECTS {ACHONDROPLASIA/ PLATYBASIA/ CRANIOSTENOSIS}
  • 8. ACQUIRED :  INFECTIONS { MENINGITIS/ MENINGOENCEPHALITIS/ CYSTICERCOSIS}  MASS LESIONS {NEOPLASM- ASTROCYTOMA/ MEDULLOBLASTOMA}  INFLAMMATION {BRAIN ABSCESS}  POST HAEMORRHAGIC{IVH/SAH/ INJURY}  CHOROID PLEXUS PAPILLOMA  SAGITTAL SINUS THROMBOSIS  HYPERVITAMINOSIS A  IDIOPATHIC
  • 9. CLASSIFICATION : 1)COMMUNICATING TYPE :  EXTERNAL / NON OBSTRUCTIVE TYPE  OBSTRUCTION OUTSIDE THE VENTRICULAR SYSTEM (IN BASAL CISTERNS/ARACHNOID VILLI)  RESORPTION IMPAIRED DUE TO SCARRING/ FIBROSIS
  • 10. 2) NON COMMUNICATING TYPE :  BLOCK WITHIN THE VENTRICULAR SYSTEM AT VARIOUS SITES  MOST COMMON IN THIRD VENTRICLE AND AQUEDUCT  VENTRICLES – DILATED  EPENDYMA OF VENTRICLES MAY BE DISRUPTED  CORTICAL ATROPHY (MORE ICP)
  • 11. NORMAL PRESSURE HYDROCEPHALUS : Post traumatic, post meningitic , SAH , deficieny of arachnoid granulations. Intermittent raise of ICP Enlarged ventricles ,normal CSF pressure ,cortical atrophy ,absence of papilledema. Hakim triad - gait apraxia, incontinence, and dementia. Headache is NOT a typical symptom in NPH, no sensory loss. Magnetic gait, personality changes, DTR increased, BABINSKI positive, release reflexes.
  • 12. CLINICAL FEATURES : Signs : Macrocephaly Dysjunction of sutures Dilated scalp veins Skin over scalp thin and shiny MACEWEN SIGN Tense/bulging fontanelle SETTING-SUN SIGN Pyramidal signs Lateral rectus palsy Papilledema Bradycardia Altered respiration Cushing triad Symptoms : Poor feeding High pitched cry Irritability Reduced activity Vomiting Headache Bladder incontinence Blurred vision Drowsiness Horizontal diplopia PARINAUD SYNDROME
  • 13. DIFFERENTIAL DIAGNOSIS :  SUBDURAL EFFUSION  CHRONIC SUBDURAL HAEMATOMA  FAMILIAL MEGALENCEPHALY  HYDRANENCEPHALY  PSEUDOHYDROCEPHALUS  PEUDOTUMOUR CEREBRI
  • 14. ARRESTED HYDROCEPHALUS / COMPENSATED HYDROCEPHALUS no progression or deleterious sequelae requiring CSF shunting - Near normal ventricular size, head growth curve - Continued psychomotor development
  • 15. SPECIAL FORMS : “HYDROCEPHALUS EX-VACUO” dilatation of the ventricles CSF pressure is normal Shrinkage of brain substance Damage to the brain Stroke or other form of injury Chronic neuro degeneration
  • 16. “EXTERNAL HYDROCEPHALUS / BENIGN ENLARGEMENT OF THE EXTRA-AXIAL SPACES” Excessive CSF-subarachnoid spaces Ventricles -not enlarged significantly Infant and early children. Associated with familial macrocephaly resolution within 1 year ARRESTED HYDROCEPHALUS / COMPENSATED HYDROCEPHALUS no progression requiring CSF shunting - Near normal ventricular size, head growth curve Continued psychomotor development
  • 17. INVESTIGATIONS :  TORCH SCREENING  CSF ANALYSIS , LP OPENING PRESSURE (OP)  XRAY  CT  MRI  USG FONTANELLE  VENTRICULOGRAPHY  PNEUMOENCEPHALOGRAPHY
  • 18. CT/MRI FINDINGS:  Temporal Horns > 2mm  EVANS ratio  Upward bowing of corpus callosum  Erosion of sella turcica  Ballooning of the Frontal Horns & 3rd Ventricles (Mickey Mouse sign)
  • 19. TREATMENT : MEDICAL: DIURETICS: Acetazolamide (Diamox) 50mg /kg/day Furosemide (Lasix) 1mg/kg/day Watch for electrolyte imbalance and acetazolamide side effects: Lethargy - tachypnea diarrhoea - paresthesias Perform weekly CT scan. maintain therapy for a 6 month trial, then taper dosage over 2-4 weeks
  • 20. SURGICAL :  Choroid Plexectomy  Choroid plexus coagulation  3rd Ventriculostomy  Endoscopic fenestration of floor of third ventricle  Cerebral aqueductoplasty  TAPPING: LP performed only in communicating hydrocephalus.  Surgical correction of the tumour/cysts  Shunts
  • 21. SHUNTING :  VENTRICULO- PERITONEAL SHUNTING (VP SHUNTING) - growing children  VENTRICULO-ATRIAL SHUNTING (VA SHUNTING)  LUMBO-PERITONEAL SHUNTING (LP SHUNTING)  VENTRICULO PLEURAL SHUNTING  VEBTRICULO CAVAL SHUNT
  • 22. COMPLICATIONS OF SHUNTING:  INFECTIONS STAPH. EPIDERMIS AND STAPH. AUREUS.  OBSTRUCTION OF THE CATHETER  INTRACEREBRAL /SUBDURAL HEMORRHAGE  OVER SHUNTING (VP SHUNTS)  MISPLACEMENT  SEIZURES  CSF ASCITES
  • 23. FOLLOW UP:  HC MONITORING  SIGNS OF INFECTION/BLOCK  ANTIBIOTIC PROPHYLAXIS  RE-EVALUATION PROGNOSIS: depends on primary cause Large IVH- permanent hydrocephalus NPH- responds to shunt gait and incontinence respond to shunting but dementia responds less frequently.