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)