Hydrocephalus is an excessive accumulation of cerebrospinal fluid (CSF) within the ventricles of the brain that results in enlarged ventricles and increased intracranial pressure. It can be caused by obstruction of CSF flow, overproduction of CSF, or impaired absorption of CSF. Common symptoms include an enlarged head size, vomiting, and headaches. Treatment options include the use of shunts to divert CSF from the brain to the abdominal cavity or endoscopic procedures. Prognosis depends on severity but can include long-term neurological and developmental issues if not treated effectively.

1. HYDROCEPHALUS
IBRAHIM
ADAMU
UG15/MDMD/
1014
GOMBE STATE UNIVERSITY
COLLEGE OF MEDICAL SCIENCES
DEPARTMENT OF PAEDIATRICS
Moderator: Dr. Ahmed Bappa Bakura
MEDICAL STUDENTS’
PRESENTATION

2. OUTLINE
• Introduction
• Epidemiology
• Relevant anatomy & CSF Physiology
• Pathophysiology
• Classifications
• Aetiology
• Clinical features
• Special Types
• Investigations
• Treatment
• Complications
• Prognosis
• Conclusion

3. INTRODUCTION
• Derived from Greek-
Hydro- water
Kefale- head
• Literally means “water in the head”
• Hydrocephalus refers to the excessive accumulation of CSF within the
ventricular system leading to enlargement of the ventricles (with or without
increase CSF pressure)
• Hydrocephalus result from disturbance of CSF production, flow, or absorption
leading to an abnormal increase in volume of CSF within the CNS

4. INTRODUCTION
• It is a common complication of Tuberculous meningitis; upon presentation may
indicate a higher risk of symptomatic cerebral infarcts
• As a clinical entity, hydrocephalus dates back to antiquity.
• In 5th century, Hippocrates describes it as water in the head.
• Galen described relationship of choroid plexus
• 17th century AD, Willis- CSF physiology

5. NATURAL HISTORY
• Hydrocephalus is a fatal disease condition
• Fifty-five percent progresses to brain herniation, coma and eventually death
• However in up to 45%, there is arrest of ventricular enlargement; compensated
hydrocephalus
• Children with compensated hydrocephalus do not require CSF diversion,
however many factors; fever, infection, trauma can offset this balance causing
decompensation
• Decompensated hydrocephalus would require CSF diversion in about 70% of
cases

6. EPIDEMIOLOGY
• The global estimated annual incidence of pediatric hydrocephalus is 400,000
new cases each year
• Prevalence higher in Africa and Latin America 145 and 136 per 100,000 births
respectively and lowest in U.S about 68 per 100,000 births
• The incidence is about 0.9-1.8/1000 birth in Nigeria
• Slight male preponderance

7. RELEVANT ANATOMY

8. CSF PHYSIOLOGY
• Formation of CSF is by:
• 80% by choroid plexus
• 20% by ventricular ependymal cells and brain tissue
• Formation is energy dependent process requiring carbonic anhydrase, with total
daily output of 450mls, 0.3ml/min
• The normal total volume of CSF in the CNS is about 150ml in adult and 25mls in
neonates, 50mls in infants
• After formation, CSF passes via foramen of Monro to the 3rd ventricle then via
aqueduct of Sylvius to the 4th ventricle then via foramena of luscka & Magendi
into the cortical and spinal cord subarachnoid space

9. CSF PHYSIOLOGY
• CSF in subarachnoid space is absorbed by arachnoid villi to dural venous sinuses
• In contrast, absorption is energy independent, it is driven by pressure gradient
from subarachnoid space across the arachnoid villi into venous sinuses
• The normal ICP is 100 – 180 mmH20 (8-15mmHg)
• When ICP is <7mmHg, CSF is produced at a rate of 0.3ml/min but no absorption
occur.
• Other proposed pathway for CSF absorption includes the nerve root sleeve of
cranial nerves, paranasal sinuses and nasal mucosal

10. PATHOPHYSIOLOGY
• Obstruction of ventricular passage way, CSF over production or impaired
absorption causes ventricular dilatation and increased pressure
• Trans-ependymal CSF leakage into periventricular tissue results to subsequent
scarring, elevated ICP, brain herniaton, coma and death
Obstruction of CSF flow within the ventricular system
Obstructive (non communicating type)

11. PATHOPHYSIOLOGY
Dysfunction in absorption or CSF over secretion
Non obstructive (communicating type)

12. CLASSIFICATION
• Idiopathic or Secondary
• Acute or Chronic
• Congenital or Acquired
• Communicating or Non-communicating

13. AETIOLOGY
• OBSTRUCTIVE
a. Obstruction of aqueduct of sylvius
 Congenital atresia: - may be sex linked recessive or may be
associated with spina bifida
 Obstruction from outside by: brain tumors
 Obstruction from inside:- Post hemorrhagic (specially in premature),
Post infection (T.B., pneumocci, mumps)
b. Congenital atresia of:
 Foramen of Monro
 Foramina of Luscka & Magendi: with cystic dilatation of 4th
ventricle usually with agenesis of cerebellar vermis (Dandy Walker
malformation)

14. AETIOLOGY
c. Arnold Chiari malformation: Congenital downward displacement of
cerebellum, pons & medulla consequently elongation of the 4th ventricle
d. Congenital infection especially toxoplasmosis
NON-OBSTRUCTIVE
a. Defective CSF absorption
 Subarachnoid space adhesions; due to Post hemorrhagic (IVH) or
Post meningitis
 Leukemic infiltration
 Dural sinus thrombosis

15. AETIOLOGY
b. Excessive CSF secretion
 Choroid plexus papilloma
 Choroid plexus congestion as in meningitis
 Ependymoma

16. CLINICAL FEATURES
INFANT
• Large head with progressive increase in size (increasing
head circumference on serial measurement).
• Fontanels are widely opened & bulging.
• Sutures are widely separated.
• Dilated scalp veins.
• Eyes deviated downwards ~ sunset appearance
• Skull percussion ~ cracked pot sound (Macewen sign).
• Craniotabes
• Back of the skull:- Promeninet occiput esp. in Dandy
Walker.

17. • Mild vomiting may be present with irritability and poor feeding
• Delayed motor milestones and mental retardation in severe cases
OLDER CHILDREN
• Marked neurologic manifestations as the sutures are not easily separated with
subsequent marked increase intracranial tension
• Bursting headache (severe in the morning) and blurring of vision
• Projectile vomiting (unrelated to meals)
• Deterioration in school performance
• Bradycardia, irregular respiration & hypertension (Cushing triad)
CLINICAL FEATURES

18. SPECIAL TYPES
• Hydrocephalus ex-vacuo
This condition occurs when there is brain injury with subsequent shrinkage of the
brain substance with increase in CSF volume although the pressure may be normal
• Normal pressure hydrocephalus
The term Normal Pressure Hydrocephalus NPH describes a condition that rarely
occurs in younger patients with an enlarged ventricles and normal CSF pressure at
lumbar puncture
NB: Hydranencephaly should not be confused with hydrocephalus which is a rare
condition in which the brains cerebral hemispheres are absent and replaced by
sacs filled with CSF

19. DIAGNOSIS
Clinical picture
• History
• General examination
Investigation

20. INVESTIGATION
1. Cranial X-ray
• Before closure of sutures -
Wide fontanels, wide
separation of sutures.
Craniofacial disproportion
with large cranium.
• After closure of sutures
increase intra cranial
tension (beaten silver
appearance, wide sella)

21. Beaten Silver Appearance

22. Ultrasound

23. INVESTIGATION
• 5. Simultaneous lumbar & ventricular manometry:
-Normally, both are equal
-Ventricular pressure> spinal pressure in obstructive hydrocephalus
• 6. Lumbar Puncture and CSF examination: xanthochromia and increased
pressure

24. DIFFERENTIALS
• Constitutional
• Hydrancephaly
• Space occupying lesion e.g. tumor
• Subdural heamatoma

25. TREATMENT
The goal of treatment is to achieve optimum neurologic function and prevent or
reverse the neurologic damage caused by distortion of the brain from ventricular
dilatation
 Medical
 Surgical
MEDICAL TREATMENT
• Carbonic anhydrase inhibitors: acetazolamide
• Loop diuretics: Frosemide
• Osmotic diuretics: Mannitol
Draw backs: - Transient effect and electrolytes & pH disturbances.

26. TREATMENT
SURGICAL TREATMENT
• Surgical options depends on the type of the hydrocephalus
1. Shunting
Used for communicating hydrocephalus and some non
communicating hydrocephalus

28. TREATMENT
Sites
• Peritoneal cavity
• Atrium
• Pleural cavity
• Others- bladder, ureter
Complications
• Mechanical failure due to either Peritoneal end obstruction, Shunt
fracture or Shunt migration
• Shunt infection 5-15% of Shunts get infected by organism like
S.epidermidis, S. aureus, enterococcus, streptococcus
• Shunt overdrainage
• Hemorrhage

29. TREATMENT

30. TREATMENT
2. Endoscopic third ventriculostomy (ETV) - Option for non- communicating
hydrocephalus.
Others
i. Excision of cyst and tumour
ii. Choroid plexectomy or endoscopic diathermy coagulation of choroid plexus

31. PROGNOSIS
• Natural history is poor with 50% dying before 3 years
• Only 20 – 23% reach adult life and only 38% of these have normal intelligence
• About 50-55% of shunted hydrocephalus have IQ > 80
• Shunt complication further worsen the prognosis

32. CONCLUSION
• Hydrocephalus is a fatal disease condition
• It refers to the excessive accumulation of CSF within the ventricular system
leading to enlargement of the ventricles
• Early detection and treatment is key to preventing long term sequalae
• Prognosis is poor with 50% dying before 3 years

33. REFERENCES
• Nelson Textbook of Paediatrics 19th Edition
• Robert M. Kliegman, Bonita F. Stanton, Joseph W. St. Geme III, Nina F. Schor, &
Richard E. Behrman Pediatric Textbook
• https://www.thisdaylive.com/index.php/2017/04/09/hydrocephalus/
• Lecture note on Hydrocephalus by Dr Sani Adamu, Department of Surgery,
FTHG

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