Hydrocephalus

Approach to hydrocephalus

1. HYDROCEPHALUS Dr Kaushik Barot

2. Absorbed by the dural venous sinuses by the  arachnoid granulations . Total volume of CSF 125-150 ml (1.5-2ml/kg) in adults at any time. Ultrafiltration 500 ml per day, 25ml/hour Children-3ml/kg Infants-4ml/kg

3. Total volume of CSF 125-150 ml (1.5-2ml/kg) in adults,50ml in infants at any time. Ultrafiltration 500 ml per day, 25ml/hour Children-3ml/kg Infants-4ml/kg Absorbed by the dural venous sinuses by the  arachnoid granulations . LV 3V 4V 75 % csf secreted by choroid plexus 25% brain capillary endothelium ICP is pulsatile Newborn < 5mm of Hg Infants-6-15 mm Children-10-15 mm

4. WHAT IS HDROCEPHALUS ? Increased ventricular size due to increase in volume of CSF due to either Increased production Obstruction Impaired absorption

5. DON’T CONFUSE ! HYDROCEPHALUS HYDRANCEPHALY

6. TYPES OF HYDROCEPHALUS

7. NON-COMMUNICATING Or OBSTRUCTIVE ACQUIRED CONGENITAL 1) CONGENITAL ANOMALIES Aqueductal stenosis(x linked) Arnold Chiari malformation Dandy walker malformation Vein of Galen malformation Spina bifida AV malformation 2) CONGENITAL INFECTIONS Toxoplasmosis- aqueductal stenosis Zika virus INFECTIONS VENTRICULAR HEMORRHAGE Prematurity AV malformation Meningitis Mumps SPACE OCCUPYING LESION Intra‐ventricular tumor Posterior fossa tumor

8. COMMUNICATING or NON-OBSTRUCTIVE INFECTIONS OVERPRODUCTION UNDERABSORPTION CONGENITAL DEFICIENCY OF ARACHNOID GRANULATION POST HEMORRHAGIC INFLAMMATION as in meningitis CHOROID PLEXUS PAPILLOMA CHOROID PLEXUS CARCINOMA CHOROID PLEXUS INFLAMMATION HEMORRHAGE CONGENITAL INFECTIONS MENINGITIS (PYO OR TBME) SUBARACHNOID HEMORRHAGE TRAUMA Achondroplasia Normal pressure hydrocephalus Hydrocephalus ex vacuo OTHERS DEFECTS IN SUBAROCHNOID SPACE INFILTRATES

9. FOR PRACTICAL'S:- COMMON CAUSES

10. PATHOPHYSIOLOGY Obstruction to CSF flow Reversal of ventricular fluid into periventricular white matter Demyelination and progressive gliosis Damage to periventricular white matter and later gr ay matter

11. Risk of Herniation

12. CLINICAL FEATURES

13. SUN SETTING SIGN CRACKPOT (MACEWEN’S SIGN

14. TRANSILLUMINATION Torch with rubber rim in dark room. Rim of lucency >2-2.5 cm in frontal region >1cm in occipital region POSITIVE in 1) Hydrocephalus 2) Hydrancephaly 3) Porencephaly 4) Subdural effusion 5) Subudral hematoma

15. EXAMINATION Open squamoparietal suture beyond one month is an early sign Serial HC measurement/HC more than 2 std. deviation Papilledema Abducens palsy Pyramidal tract lesions ( lower extremities ) Crackpot sign positive

16. FEW MALFORMATIONS AHEAD !

17. POSTERIOR FOSSA CONTENTS CEREBELLUM BRAIN STEM OCCIPITAL LOBE 4 th VENTRICLE CHIARI MALFORMATION or previously k/a Arnold Chiari malformation CEREBELLAR TONSILS TYPE - I CEREBELLAR TONSILS VERMIS BRAIN STEM TYPE-II TYPE -III Per mont DOWNWARDS DISPLACMENT towards the foramen magnum OF THE………

18. POSTERIOR FOSSA CONTENTS CEREBELLUM BRAIN STEM OCCIPITAL LOBE 4 th VENTRICLE CHIARI MALFORMATION or previously k/a Arnold Chiari malformation CEREBELLAR TONSILS TYPE - I CEREBELLAR TONSILS VERMIS BRAIN STEM TYPE-II TYPE -III Per mont DOWNWARDS DISPLACMENT towards the foramen magnum OF THE………

19. CHIARI MALFORMATION TYPE 1 Seen in adolescence Not associated with Hydrocephalus Headache, Neck pain Progressive spasticity Associated with Syringomyelia TYPE 2 Seen in infants Progressive hydrocephalus Weak cry, stridor, apnea Abnormality of gait , Spasticity Incoordination Associated with Myelo ‐meningocele, Spina bifida

20. DANDY WALKER MALFORMATION Failure of development of roof of 4 th ventricle Cystic dilatation of 4 th ventricle ( Blake's pouch) Cerebellar hypoplasia Ass. Anomalies‐ absence of corpus callosum

21. DANDY WALKER MALFORMATION Clinical features Increasing head size Prominent occiput Cerebellar ataxia Delayed motor & cognitive development Trans‐illumination

22. VEIN OF GALEN ANEURYSMAL MALFORMATIONS VGAM consist of a tangled mass of dilated vessels supplied by an enlarged artery. Hydrocephalus, High output cardiac failure.

23. HOW TO REMEMBER !

24. NORMAL PRESURE HYDROCEPHALUS

25. HYDROCEPHALUS EX VACUO EX -out of Vacuo -space Enlargement of cerebral ventricles & subarachnoid spaces, caused by encephalic volume loss. NOT a true hydrocephalus I ncreases in CSF volume without increased CSF pressure

26. DO ALL HYDROCEPHALUS PROGRESS ?... NO ARRESTED HYDROCEPHALUS A large proportion of congenital and acquired hydrocephalus may undergo spontaneous arrest. No surgical intervention needed.

27. INVESTIGATIONS

28. X‐RAY SKULL

29. ULTRASOUND BRAIN

30. CT SCAN Useful when AF is closed WATCH FOR Ventricle size Cortical mantle Periventricular ooze Associated malformation EVANS ratio (lateral ventricular width: hemispheric width >33%-needs surgery)

31. CT SCAN – DILATED VENTRICLES Earliest sign is dilation of occipital horns and atria of lateral ventricles. Communicating-All ventricles dilated Non communicating:- Upstream ventricles dilated.

32. MRI BRAIN Dilated ventricles Useful when suspecting posterior fossa or white matter lesions.

33. TREATMENT Goals of therapy is to decrease ICT to same limits preserving brain tissue. Mild /Arrested cases managed medically. Severe cases with cortical mantle < 1 cm optic atrophy associated anomalies Won’t benefit from surgery. Moderate cases should be operated early.

34. MEDICAL TREATMENT Reduction of CSF production Acetazolamide 50-100mg/kg/day. w/f metabolic acidosis Oral glycerol 1ml/kg/dose 8 hourly Mannitol for emergency (5ml/kg f/b 2ml/kg qds x 2 days) Furosemide 0.5-2mg/kg/day

35. INDICATIONS FOR SURGERY Progressive thinning of cortical mantle despite medical therapy Cortical mantle less then 2-2.5 cm especially in infancy Papilledema on fundoscopy Periventricular ooze on CT scan Evans ratio > 33 %

36. SURGICAL TREATMENT Diversion of CSF to extra cranial sites using shunts Ventriculo peritoneal Ventriculo atrial Ventriculo azygous Theco peritoneal Endoscopic third ventriculostomy

37. VENTRICULOPERITONEAL SHUNT

38. TYPES OF VP SHUNT 1) Chabra’s 2) Pudenz – Hakin 3) Splitz – Holter valve

39. TREATMENT

40. EXTERNAL VENTRICULAR DRAIN DEVICE

41. COMPLICATIONS OF VP SHUNT

42. PROGNOSIS Untreated – 50 % mortality in severe hydrocephalus Medical management- 30 % improvement in ventriculomegaly VP shunt- 60 % improvement in ventriculomegaly 44 % in fundus 70% - neurologically impaired