Hydrocephalus is characterized by abnormal accumulation of cerebrospinal fluid (CSF) within the brain's ventricles, leading to potentially harmful pressure on brain tissue. It has a prevalence of 1-1.5% and causes can be congenital or acquired, with various types classified as communicating or non-communicating. Treatment options include medical management with diuretics and surgical interventions such as shunting, with prognosis dependent on the underlying cause.

1. HYDROCEPHALUS
BY:
V.VIJAY ANAND
CRRI

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

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.

24. THANK YOU
VIJAY ANAND
CRRI