HYDROCEPHALUS
Rene Santin, M.D.
Texas Tech University Health Sciences Center
Department of Pediatrics
HYDROCEPHALUS
Rene Santin, M.D.
Texas Tech University Health Sciences Center
Department of Pediatrics
HYDROCEPHALUS
• DEFINITION:
DIVERSE GROUP OF
CONDITIONS WHICH RESULT
FROM IMPAIRED CIRCULATION
AND RESORPTION OF CSF.
CSF FORMATION
• CSF IS FORMED BY THE CHOROID
PLEXUS.
• NORMAL CSF PRODUCTION: 20
ml/h.
TYPES OF
HYDROCEPHALUS
• OBSTRUCTIVE OR NON-
COMMUNICATING
(OBSTRUCTION WITHIN
THE VENTRICULAR SYSTEM)
• NON OBSTRUCTIVE OR
COMMUNICATING
(MALFUNCTION OF
ARACHNOID VILLI)
CAUSES OF
HYDROCEPHALUS
• LESIONS OR MALFORMATIONS OF THE
POSTERIOR FOSSA
– CHIARI MALFORMATION
– DANDY- WALKER SYNDROME
– TUMORS
• IVH
• MENINGITIS: PNEUMOCOCCAL, TB
• INTRAUTERINE INFECTIONS
PATHOLOGY
• AQUEDUCTAL STENOSIS:
– ABNORMALLY NARROW AQUEDUCTUS
OF SYLVIUS.
• AQUEDUCTAL GLIOSIS:
– BRISK GLIAL RESPONSE OF EPENDIMAL
LINING
CHIARI MALFORMATION
•TYPE I:
– DISPLACEMENT OF CEREBELLAR
TONSILS INTO THE CERVICAL CANAL.
– GIVES SYMPTOMS IN ADOLESCENCE OR
ADULT LIFE. (HEADACHE, NECK PAIN)
– NO HYDROCEPHALUS
.
CHIARI MALFORMATION
•TYPE II :
– PROGRESSIVE HYDROCEPHALUS AND
MYELOMENINGOCELE.
– ELONGATION OF THE 4TH VENTRICLE.
– DISPLACEMENT OF INFERIOR VERMIS,
PONS, AND MEDULLA INTO CERVICAL
CANAL
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
IVH
• DEFINITION:
– BLEEDING IN SUBEPENDIMAL
GERMINAL MATRIX WITH/WITHOUT
EXTENSION INTO VENTRICLES AND
BRAIN PARENCHYMA
• INCIDENCE:
– IN PREMATURES 25 - 40 %
IVH
• PATHOLOGY:
– INTRAVASCULAR
– VASCULAR
– EXTRAVASCULAR
• COMPLICATIONS:
– HYDROCEPHALUS
• 20 % IN MODERATE BLEEDS
• 65-100 % IN LARGE BLEEDS.
PREVENTION OF IVH
• AVOID PROLONGUED LABOR OR
DIFFICULT VAGINAL DELIVERY
• AVOID PNEUMOTHORAX
• AVOID OF HYPOTENSION OR
HYPERTENSION IN THE NEONATE
• AVOID HYPOXIC ISCHEMIC INSULT
PREVENTION OF IVH
• INDOMETHACIN
• PROPHYLACTIC FFP??
• PROPHYLACTIC PLATELET
TRANSFUSION???
• PHENOBARBITAL???
• VITAMIN E ???
CLINICAL
MANIFESTATIONS
• SYMPTOMS:
– IRRITABILITY
– POOR FEED
– LETHARGY
– VOMITING
– IN OLDER PATIENTS:
• HEADACHE
• CHANGES IN PERSONALITY
• ACADEMIC DETERIORATION
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)
IMAGING STUDIES
• X-RAY PLAIN FILMS:
– SEPARATION OF SUTURES
– EROSION OF POSTERIOR CLINOIDS
– INCREASED CONVOLUTIONAL MARKINGS
(BEATEN SILVER APPEAREANCE)
• ULTRASOUND
• CT SCAN
• MRI
THERAPY
• MEDICAL:
– ACETAZOLAMIDE
– FUROSEMIDE
• SURGICAL:
– V-P SHUNT PLACEMENT
PROGNOSIS
• INCREASED RISK FOR
DEVELOPMENTAL DISABILITIES
• MEAN IQ IS REDUCED COMPARED
TO GENERAL POPULATION
• ABNORMALITIES IN MEMORY
• SOME PATIENTS SHOW AGGRESSIVE
OR DELINQUENT BEHAVIOR.
PROGNOSIS
• VISUAL PROBLEMS:
– STRABISMUS
– VISUOSPATIAL ABNORMALITIES
– DECREASED VISUAL ACUITY
– VISUAL FIELD DEFECTS
• PATIENTS REQUIRE LONG TERM
FOLLOW UP (MULTIDISCIPLINARY)