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MACROCEPHALYANU PRIYA
MACROCEPHALYHead circumference >2 S.D./>2.5cm above the mean for the age and sex
Head circumference >97th percentile for the age and sex
Expected HC for a particular child by comparing with standardized charts
CausesBig skull-chronic anemia,osteogenesis imperfecta,rickets
Big brain-megalencephaly(tay-sach),cerebral gigantism
More CSF in ventricles-hydrocephalusAbnormal accumulation-subdural effusion
Familial-commonest cause
HYDROCEPHALUS(Greek-water in head)
DEFINITION: Excessive accumulation of CSF in the ventricular system
TYPES OF HYDROCEPHALUSOBSTRUCTIVE OR NON-COMMUNICATING -at the level of aqueduct 3 mm long & 2 mm wide
NON OBSTRUCTIVE OR COMMUNICATING -increased production -decreased absorption
CAUSES OF HYDROCEPHALUSCOMMUNICATING HYDROCEPHALUS
Increased Production•Tumors in choroid plexus
Decreased AbsorptionCongenital-TORCHAcquired-Meningitis,leukemia
CAUSES OF HYDROCEPHALUS•OBSTRUCTIVE HYDROCEPHALUSCongenitalAqueductal stenosisArnold chiari syndromeDandy-walker syndromeAcquiredAqueductal gliosis-meningitis,bleeding,mumps encephalitis
Posterior fossa tumors-medulloblastoma
CLINICAL MANIFESTATIONSSYMPTOMS:
IRRITABILITYPOOR FEEDLETHARGYVOMITING
CLINICAL MANIFESTATIONSSIGNS:Before AF closure
-BULGING ANTERIOR FONTANEL, -INCREASED HEAD CIRC. -DILATED SCALP VEINS -SETTING SUN SIGN -WEAKNESS OF LOWER LIMBS
•After AF closure-Headache,vomiting-Blurring of vision-Bradycardia,increased Bp-6th CN Palsy-Papilloedema-Transillumination Test-Macewen Sign “Cracked Pot”-Prominent Occiput (Dandy-walker)
DIAGNOSISHistory1.Prematurity2.Intrauterine infections3.Intracranial hemorrhage4.Meningitis5.Mumps encephalitis
On examination1.Café-au-lait patches2.Spinal dysmorphism3.Wide AF,wide sutures,sunset eye sign 4.Lower limb weakness,cranial bruit5.Chorioretinitis,papilloedema
IMAGING STUDIESPLAIN X-RAY SKULL:
SEPARATION OF SUTURESEROSION OF POSTERIOR CLINOIDSINCREASED CONVOLUTIONAL MARKINGS (SILVER BEATEN APPEAREANCE)
ULTRASOUNDCT SCANMRI
TREATMENTSUPPORTIVE:(control of ICP)a.head elevated to 30o b.Control of temperaturec.Control of seizuresd.Maintain BPe.Analgesia and sedation
TREATMENTMEDICAL: (for decreasing the production) _ MANNITOL/ORAL GLYCEROL
-ACETAZOLAMIDE -FUROSEMIDE
SURGICAL:(for obstruction and decreased absorption)V-P SHUNT PLACEMENT
PROGNOSISINCREASED RISK FOR DEVELOPMENTAL DISABILITIES
MEAN IQ IS REDUCED COMPARED TO GENERAL POPULATION
ABNORMALITIES IN MEMORY SOME PATIENTS SHOW AGGRESSIVE OR DELINQUENT BEHAVIOR.
PROGNOSISVISUAL PROBLEMS:
STRABISMUSVISUOSPATIAL ABNORMALITIESDECREASED VISUAL ACUITYVISUAL FIELD DEFECTS
PATIENTS REQUIRE LONG TERM FOLLOW UP