HYDROCEPHALUS

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HYDROCEPHALUS

• Rene Santin, M.D.
• Texas Tech University Health Sciences Center
• Department of Pediatrics

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HYDROCEPHALUS

• DEFINITION
• DIVERSE GROUP OF CONDITIONS WHICH RESULT FROM
  IMPAIRED CIRCULATION AND RESORPTION OF CSF.

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CSF FORMATION

• CSF IS FORMED BY THE CHOROID PLEXUS.
• NORMAL CSF PRODUCTION 20 ml/h.

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TYPES OF HYDROCEPHALUS

• OBSTRUCTIVE OR NON-COMMUNICATING (OBSTRUCTION
  WITHIN THE VENTRICULAR SYSTEM)
• NON OBSTRUCTIVE OR COMMUNICATING (MALFUNCTION OF
  ARACHNOID VILLI)

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CAUSES OF HYDROCEPHALUS

• LESIONS OR MALFORMATIONS OF THE POSTERIOR FOSSA
• CHIARI MALFORMATION
• DANDY- WALKER SYNDROME
• TUMORS
• IVH
• MENINGITIS PNEUMOCOCCAL, TB
• INTRAUTERINE INFECTIONS

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PATHOLOGY

• AQUEDUCTAL STENOSIS
• ABNORMALLY NARROW AQUEDUCTUS OF SYLVIUS.
• AQUEDUCTAL GLIOSIS
• BRISK GLIAL RESPONSE OF EPENDIMAL LINING

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CHIARI MALFORMATION

• TYPE I
• DISPLACEMENT OF CEREBELLAR TONSILS INTO THE
  CERVICAL CANAL.
• GIVES SYMPTOMS IN ADOLESCENCE OR ADULT LIFE.
  (HEADACHE, NECK PAIN)
• NO HYDROCEPHALUS.

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CHIARI MALFORMATION

• TYPE II
• PROGRESSIVE HYDROCEPHALUS AND MYELOMENINGOCELE.
• ELONGATION OF THE 4TH VENTRICLE.
• DISPLACEMENT OF INFERIOR VERMIS, PONS, AND
  MEDULLA INTO CERVICAL CANAL

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DANDY-WALKER SYNDROME

• CYSTIC EXPANSION OF THE 4TH VENTRICLE IN THE
  POSTERIOR FOSSA.
• DEVELOPMENTAL FAILURE OF ROOF OF 4TH VENTRICLE
  DURING EMBRYOGENESIS.
• 90 HAVE HYDROCEPHALUS
• PROMINENT OCCIPUT

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IVH

• DEFINITION
• BLEEDING IN SUBEPENDIMAL GERMINAL MATRIX
  WITH/WITHOUT EXTENSION INTO VENTRICLES AND BRAIN
  PARENCHYMA
• INCIDENCE
• IN PREMATURES 25 - 40

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IVH

• PATHOLOGY
• INTRAVASCULAR
• VASCULAR
• EXTRAVASCULAR
• COMPLICATIONS
• HYDROCEPHALUS
• 20 IN MODERATE BLEEDS
• 65-100 IN LARGE BLEEDS.

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PREVENTION OF IVH

• AVOID PROLONGUED LABOR OR DIFFICULT VAGINAL
  DELIVERY
• AVOID PNEUMOTHORAX
• AVOID OF HYPOTENSION OR HYPERTENSION IN THE
  NEONATE
• AVOID HYPOXIC ISCHEMIC INSULT

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PREVENTION OF IVH

• INDOMETHACIN
• PROPHYLACTIC FFP??
• PROPHYLACTIC PLATELET TRANSFUSION???
• PHENOBARBITAL???
• VITAMIN E ???

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CLINICAL MANIFESTATIONS

• SYMPTOMS
• IRRITABILITY
• POOR FEED
• LETHARGY
• VOMITING
• IN OLDER PATIENTS
• HEADACHE
• CHANGES IN PERSONALITY
• ACADEMIC DETERIORATION

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CLINICAL MANIFESTATIONS

• SIGNS
• ANTERIOR FONTANEL WIDE OPEN AND BULGING,
  INCREASED HEAD CIRC.
• DILATED SCALP VEINS
• SETTING SUN SIGN
• BRISK TENDON REFLEXES, SPASTICITY
• CLONUS, BABINSKY
• MACEWEN SIGN CRACKED POT
• PROMINENT OCCIPUT (DANDY-WALKER)

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IMAGING STUDIES

• X-RAY PLAIN FILMS
• SEPARATION OF SUTURES
• EROSION OF POSTERIOR CLINOIDS
• INCREASED CONVOLUTIONAL MARKINGS (BEATEN SILVER
  APPEAREANCE)
• ULTRASOUND
• CT SCAN
• MRI

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THERAPY

• MEDICAL
• ACETAZOLAMIDE
• FUROSEMIDE
• SURGICAL
• V-P SHUNT PLACEMENT

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PROGNOSIS

• INCREASED RISK FOR DEVELOPMENTAL DISABILITIES
• MEAN IQ IS REDUCED COMPARED TO GENERAL POPULATION
• ABNORMALITIES IN MEMORY
• SOME PATIENTS SHOW AGGRESSIVE OR DELINQUENT
  BEHAVIOR.

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PROGNOSIS

• VISUAL PROBLEMS
• STRABISMUS
• VISUOSPATIAL ABNORMALITIES
• DECREASED VISUAL ACUITY
• VISUAL FIELD DEFECTS
• PATIENTS REQUIRE LONG TERM FOLLOW UP
  (MULTIDISCIPLINARY)