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