Hydrocephalus

HYDROCEPHALUS 1

HYDROCEPHALUS 1
PATHOGENESIS 2
1. INCREASED CSF PRODUCTION 2
2. CSF FLOW OBSTRUCTION 2
3. DECREASED CSF ABSORPTION 2
CAUSES 2
1. DEVELOPMENTAL ANOMALIES 2
2. POST-INFECTION 2
3. TUMORS 2
4. INTRACRANIAL HEMORRHAGE 2
5. TRAUMATIC BRAIN INJURY 2
6. CHROMOSOMAL ANOMALIES 2
INFANTS & YOUNG CHILDREN CLINICAL FEATURE 2
ADOLESCENT CLINICAL FEATURES 2
HYDROCEPHALUS WITH HIGH ICP 3
HYDROCEPHALUS WITH NORMAL ICP 3
TYPE 3
INVESTIGATIONS 4
1. PLAIN X-RAY 4
2. U/S HEAD 4
3. CT BRAIN 4
4. MRI BRAIN 4
5. ICP MONITORING (LP, EVD) 5
TREATMENT 5
O CSF SHUNTING PROCEDURES 5
HYDROCEPHALUS EX-VACUO 5
O CAUSES 5

Hydrocephalus
o Increased amount of CSF
o Ventriculomegaly
o ± Elevated ICP
▪ Normal Pressure (adults): 5 – 15 mmHg
▪ Normal Pressure (adults): 20 cm-water

Done by: Norah AlRohaimi (Pediatric Resident - NGH)

Source: UptoDate + Nelson
 Pathogenesis
1. Increased CSF production o Choroid papilloma

2. CSF flow obstruction o Tumors

o Congenital anomalies
▪ Occurs mostly at aqueduct of Sylvius, foramen of
Monroe, 3rd & 4th ventricles

3. Decreased CSF absorption o Post-meningitis

o Post-SAH

Causes
1. Developmental Anomalies:
o Aqueduct anomalies
o Dandy-walker malformation
o Chiari II malformation
o Myelomeningocele
2. Post-Infection: Meningitis
3. Tumors: Posterior fossa tumors
4. Intracranial hemorrhage: Sub-arachnoid
5. Traumatic brain injury:
6. Chromosomal Anomalies: Trisomy 13 & 18

Infants & young children Clinical Feature Adolescent Clinical Features

o Macrocephalus: increased head Elevated ICP

circumference Headache
o Bulging anterior fontanelle: widened N/V
cranial sutures Visual changes
o McEwen's cracked pot sound Papilledema
o Scalp vein dilation: increased collateral Decreased LOC
venous drainage Seizures
o Sunset Sign: Forced downward deviation of
the eyes
o Cushing Triad:
1. Episodic bradycardia
2. Apnea
3. Hypertension

Done by: Norah AlRohaimi (Pediatric Resident - NGH)

Source: UptoDate + Nelson
 Hydrocephalus with high ICP o Most symptomatic hydrocephalus
o Signs of elevated ICP

Hydrocephalus with normal o May be asymptomatic

ICP o Causes:
1. Idiopathic
2. Secondary: SAH, meningitis, trauma, radiation-induced
o Triad: wet, wobbly, wacky
1. Urinary incontinence
2. Gait disturbance: magnetic gait
3. Cognitive dysfunction/dementia: pressure on frontal lobes
(sometimes reversible)
• Only reversible cause of dementia

Type Communicating Non-communicating

Definition o Obstruction distal to ventricular o Obstructed CSF flow within ventricular system
system (extra-ventricular)
o Decreased CSF absorption by
arachnoid granulations

Etiology 1. Post-infection Congenital Acquired

▪ Meningitis
▪ Abscess 1. Primary Aqueduct 1. Aqueduct stenosis:
2. Post-hemorrhagic: stenosis o Adhesions post-
▪ SAH 2. Dandy-walker infection
▪ IVH malformation o Hemorrhage
3. Leptomeningeal carcinomatosis 3. Arnold-Chiari o Gliosis
4. Choroid plexus papilloma malformation o Tumor
5. Idiopathic normal pressure 4. Myelomeningocele 2. Intra-ventricular lesions
hydrocephalus 5. Encephalocele o tumors
3. Other:
o Neuro-sarcoidosis
o Abscess
o Granuloma
o Arachnoid cyst

MRI All ventricles dilated Only parts of ventricular system are dilated (proximal to the
obstruction)

Done by: Norah AlRohaimi (Pediatric Resident - NGH)

Source: UptoDate + Nelson
Investigations
1. Plain X-ray:
o Long-standing indirect signs of increased ICP
Separated skull sutures Silver beaten appearance

2. U/S Head:
o Through anterior fontanelles in infants
o Ventriculomegaly, size & location of lesions
3. CT Brain:
o Allows direct visualization of ventricular system
o Shows acute & chronic ventricular enlargement
o Often shows site & cause of ventricular obstruction
o Method of choice for emergency
4. MRI Brain:
o Better visualization of obstructive lesions & associated brain anomalies
o Signs:
▪ Trans-ependymal permeation: CSF herniates through ependyma

Done by: Norah AlRohaimi (Pediatric Resident - NGH)

Source: UptoDate + Nelson
 ▪ Sulci & gyri not seen
▪ Subarachnoid space obliterated
▪ Dilated lateral ventricles

5. ICP monitoring (LP, EVD):

o Investigate NPH & test response to shunting (lumbar tap test)

Treatment
o Surgical removal of obstructive lesion (if possible)
o Extra-ventricular Drain (EVD)
o CSF shunting procedures:
▪ Ventriculo-peritoneal (VP) shunt (MC)
▪ Ventriculo-atrial (VA) shunt
▪ Ventriculo-pleural shunt
▪ Lumbo-peritoneal shunt: for communicating hydrocephalus
o Endoscopic 3rd Ventriculostomy
▪ For non-communicating hydrocephalus

Hydrocephalus Ex-Vacuo
o Decreased brain volume - brain atrophy
▪ Enlarged ventricles - appear as increased CSF on imaging
o Normal ICP
o No NPH triad
o Causes:
▪ Advanced HIV
▪ Pick disease

Done by: Norah AlRohaimi (Pediatric Resident - NGH)

Source: UptoDate + Nelson