Macrocephaly is defined as a head circumference greater than 2 standard deviations above the mean for age and sex. It can be caused by increased skull size due to conditions like chronic anemia or increased brain size. Hydrocephalus, an excessive accumulation of cerebrospinal fluid in the brain ventricles, is another potential cause. Hydrocephalus can be obstructive due to blockages or non-obstructive if caused by increased fluid production or decreased absorption. Symptoms include irritability, poor feeding, lethargy and vomiting. Diagnosis involves medical history, physical exam, and brain imaging studies like CT or MRI scans. Treatment options include supportive care, medications to reduce fluid production, and surgical placement of a VP

1. MACROCEPHALY
ANU PRIYA

2. MACROCEPHALY
Head 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

3. Causes
Big skull-chronic anemia,osteogenesis
imperfecta,rickets
Big brain-megalencephaly(tay-sach),cerebral
gigantism
More CSF in ventricles-hydrocephalus
Abnormal accumulation-subdural effusion
Familial-commonest cause

4. HYDROCEPHALUS
(Greek-water in head)
DEFINITION:
Excessive accumulation of CSF in the ventricular system

5. TYPES OF HYDROCEPHALUS
OBSTRUCTIVE OR NON-COMMUNICATING
-at the level of aqueduct 3 mm long & 2 mm wide
NON OBSTRUCTIVE OR COMMUNICATING
-increased production
-decreased absorption

6. CAUSES OF HYDROCEPHALUS
COMMUNICATING HYDROCEPHALUS
Increased Production
• Tumors in choroid plexus
Decreased Absorption
Congenital-TORCH
Acquired-Meningitis,leukemia

7. CAUSES OF HYDROCEPHALUS
• OBSTRUCTIVE HYDROCEPHALUS
Congenital
Aqueductal stenosis
Arnold chiari syndrome
Dandy-walker syndrome
Acquired
Aqueductal gliosis-
meningitis,bleeding,mumps encephalitis
Posterior fossa tumors-medulloblastoma

8. CLINICAL MANIFESTATIONS
SYMPTOMS:
IRRITABILITY
POOR FEED
LETHARGY
VOMITING

9. CLINICAL MANIFESTATIONS
SIGNS:
Before AF closure
-BULGING ANTERIOR FONTANEL,
-INCREASED HEAD CIRC.
-DILATED SCALP VEINS
-SETTING SUN SIGN
-WEAKNESS OF LOWER LIMBS

10. • 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)

11. DIAGNOSIS
History
1.Prematurity
2.Intrauterine infections
3.Intracranial hemorrhage
4.Meningitis
5.Mumps encephalitis

12. On examination
1.Café-au-lait patches
2.Spinal dysmorphism
3.Wide AF,wide sutures,sunset eye sign
4.Lower limb weakness,cranial bruit
5.Chorioretinitis,papilloedema

13. IMAGING STUDIES
PLAIN X-RAY SKULL:
SEPARATION OF SUTURES
EROSION OF POSTERIOR CLINOIDS
INCREASED CONVOLUTIONAL MARKINGS
(SILVER BEATEN APPEAREANCE)
ULTRASOUND
CT SCAN
MRI

14. TREATMENT
SUPPORTIVE:(control of ICP)
a.head elevated to 30o
b.Control of temperature
c.Control of seizures
d.Maintain BP
e.Analgesia and sedation

15. TREATMENT
MEDICAL: (for decreasing the production)
_
MANNITOL/ORAL GLYCEROL
-ACETAZOLAMIDE
-FUROSEMIDE
SURGICAL:(for obstruction and decreased
absorption)
V-P SHUNT PLACEMENT

16. PROGNOSIS
INCREASED RISK FOR DEVELOPMENTAL
DISABILITIES
MEAN IQ IS REDUCED COMPARED TO GENERAL
POPULATION
ABNORMALITIES IN MEMORY
SOME PATIENTS SHOW AGGRESSIVE OR
DELINQUENT BEHAVIOR.

17. PROGNOSIS
VISUAL PROBLEMS:
STRABISMUS
VISUOSPATIAL ABNORMALITIES
DECREASED VISUAL ACUITY
VISUAL FIELD DEFECTS
PATIENTS REQUIRE LONG TERM FOLLOW UP