HYDROCEPHALUS

for pediatrics UG teaching

1. HYDROCEPHALUS Dr M.Sanjeevappa M.D.( paeds ) Asst.Professor Dept. of Paediatrics Govt. Medical College Anantapur

2. From Greek: Hydro means Water Kephalos means Head Definition : An abnormal increase in the amount of CSF within the cranial cavity that is accompanied by expansion of the cerebral ventricles, enlargement of the skull and atrophy of the brain due to impaired circulation and /or absorption of CSF. DEFINITION

3. CSF PATHWAY Flows from lateral ventricles through foramina of Monro into third ventricle. Enters fourth ventricle through aqueduct of Sylvius . Enters subarachnoid space through foramina of Luschka and foramen of Magendie into the cisterns at the base of the brain. Resorbed by arachnoid villi at top of brain into sagittal sinus.

4. Balances the amount of blood in the head. Bathes and protects the brain and spinal cord. Carries nutrients between the brain and spinal cord while removing waste . CSF FUNCTIONS

5. Obstructive (non-communicating ) and non-obstructive (communicating). Congenital and acquired. Normal pressure hydrocephalus (NPH) and increasing pressure hydrocephalus. Acute and chronic hydrocephalus. TYPES OF HYDROCEPHALUS

6. Obstructive Non- obstructive

7. Obstructive hydrocephalus: Aqueductal branching or forking. Aqueductal stenosis - sex-linked recessive , neurofibromatosis. A vein of Galen malformation. Intrauterine viral infections. Malformations of the posterior fossa Arnold- Chiari malformation, Dandy-Walker syndrome. ETIOLOGY

8. mumps meningoencephalitis . Aqueductal gliosis - neonatal meningitis or SAH. Lesions of the posterior fossa Posterior fossa brain tumors. Hypervitaminosis A Ependimoma Continued….

9. Non-obstructive hydrocephalus: subarachnoid hemorrhage. Pneumococcal and tuberculous meningitis. intrauterine infections. Leukemic infiltrates. ETIOLOGY

10. Influenced by the following : Patient's age Cause Location of obstruction Duration Rapidity of onset CLINICAL FEATURES

11. Symptoms in infants: Poor feeding Irritability Reduced activity Vomiting CLINICAL FEATURES

12. Sign in infants: Head enlargement. Dysjunction of sutures. Dilated scalp veins. Tense fontanelle : The anterior fontanelle in infants may be excessively tense. Setting-sun sign. Increased limb tone. CLINICAL FEATURES

13. Symptoms in children : Slowing of mental capacity. Headache. Neck pain suggesting tonsillar herniation . Vomiting. Stunted growth . CLINICAL FEATURES

14. Blurred vision - Consequence of papilledema and later of optic atrophy . Double vision - Related to unilateral or bilateral sixth nerve palsy . Difficulty in walking secondary to spasticity. Drowsiness. Continued….

15. Signs in children: Papilledema. Failure of upward gaze. Macewen sign: A "cracked pot" sound is noted on percussion of the head. Unsteady gait. Large head. Unilateral or bilateral sixth nerve palsy. CLINICAL FEATURES

16. Cases history: Prematurity with intracranial hemorrhage Meningitis. Mumps encephalitis. Multiple café-au- lait spots. DIAGNOSIS

17. Examination : Occipitofrontal head circumference. Abnormal midline skin lesions. Cranial bruit. Transillumination of the skull. Fundus examination. DIAGNOSIS

18. Investigations: X-Ray skull – Silver beaten appearance. CT / MRI Brain. Neurosonogram . DIAGNOSIS

19. Chronic anemia. Rickets. Osteogenesis imperfecta. Epiphyseal dysplasia. Chronic subdural collections. Megalencephaly : lysosomal diseases eg : Tay -Sachs disease. aminoacidurias eg : maple syrup urine disease leukodystrophies eg : metachromatic leukodystrophy Cerebral gigantism eg : Sotos syndrome. DIFFERENTIAL DIAGNOSIS

20. The main goal is to minimize or prevent brain damage by improving CSF flow . Medical therapy: To decrease the ICP or reduce the production of CSF with drugs. Acetazolamide Furosemide TREATMENT

21. Treating the causes: searching for and direct removal of the obstruction. Shunt procedure: V-P shunt - the most common V-A shunt Third Ventriculostomy SURGICAL INTERVENTIONS

22. SHUNT PROCEDURE

23. Third Ventriculostomy

24. depends on the cause. increased risk for various developmental disabilities. The mean intelligence quotient is reduced. Vision problems are common, eg : strabismus, visual field defects, optic atrophy Accelerated pubertal development. PROGNOSIS

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