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)