Chiari Malformation

Four types of Chiari malformations types I, II,

III, and IV

Chiari 1 malformation

Important features
Small posterior cranial fossa
herniation of the cerebellar tonsils through
the foramen magnum into the cervical
spinal canal
Distention CSF of the central canal of
spinal cord (ie, hydromyelia) or paracentral
cavities (ie, syringomyelia) in 25%
not directly associated with other
congenital brain malformations

Pathophysiology

mesodermal disorder
Underdevelopment of the occipital somites of
the para-axial mesoderm produces
overcrowded (PCF).
Tonsillar herniation occurs secondarily as a
result of mechanical factors.

Causes

May have genetic basis its association with

known genetic disorders such as
achondroplasia and Klippel-Feil syndrome
some families suggest an AD
Acquired CMI is reported to develop after
lumboperitoneal or ventriculoperitoneal shunt
placement,

CSF flow

During systole: increase cerebral flow

craniocaudal displacement.
during diastole: elastic recoil
reversal

remain asymptomatic for prolonged periods

aged 10-30 years
arachnoidal adhesions may increase the
compression of the hindbrain and spinal cord
and further interfere with CSF flow at the
foramen magnum and thus cause symptoms

Normal anatomy
Normally adult 5 mm
Infant upto 6 mm

Intracrainial findings

CT FINDINGS:
Obliterated cisterna magna
Hydrocephalus
Flattened spinal cord
Tonsillar ectopia
Peglike cerebellar tonsils
Normally positioned fourth ventricle

MRI FINDINGS
Displacement of cerebellar tonsils
below the level of the foramen
magnum
Pointed and/or peglike tonsils
Narrow PCF
Elongation of the fourth ventricle,
which remains in the normal position
Hindbrain abnormalities
Obstructive hydrocephalus
syringomyelia

EXTRA CRANIAL FINDINGS

Platybasia, basilar invagination (25-50%)

Atlantooccipital assimilation (1-5%)
Klippel-Feil syndrome (5-10%)
Incomplete ossification of C1 ring (5%)
spina bifida at the C1 level
Retroflexed odontoid process (26%)
Scoliosis (42%)
Kyphosis
Increased cervical lordosis
Cervical ribs
Fused thoracic ribs

Platybasia, basilar invagination

abnormal flattening of the skull base

When platybasia is associated with basilar
invagination, or the inward and upward
migration of the cervical spine through the
foramen magnum, signs and symptoms of
compression of the brainstem and upper
cervical cord can result (5).

Atlantooccipital assimilation

The union of the atlas with the occipital bone

constitutes the anomaly. There may be partial
or complete union

Klippel feil syndrome

They described patients who had a short,

webbed neck; decreased range of motion
(ROM) in the cervical spine; and a low hairline.
classified the syndrome into 3 categories:

Chiari II malformation

THEORIES

HYDRODYNAMIC THEORY: posterior fossa

herniation was related to supratentorial
hydrocephalus
TRACTION THEORY:tethered spinal cord near
the myelomeningocele may pull the
cerebellum and medulla into the cervical
canal

complex deformity of the calvarium, dura, and

hindbrain, and it is almost always associated
with myelomeningocele

LACUNAR SKULL

CONCAVE CLIVUS

Hypoplastic fenestrated falx

Cerebellar peg: Protrusion of vermis and

hemispheres through the foramen magnum
(90%) results in craniocaudal elongation of
cerebellum behind the spinal cord

Medullary kink: The medulla is kinked

inferiorly (75%) and lies dorsal to the spinal
cord, which is unable to descend because of
competent dentate ligaments

Towering cerebellum or vermian pseudotumor:

The cerebellar hemispheres and vermis also
extend above the incisura of the tentorium

Tubelike elongated fourth ventricle: The fourth

ventricle is elongated craniocaudally,
narrowed transversely, and decreased in
anteroposterior diameter
The cerebellopontine cistern and the cisterna
magna are obliterated.

Beaked tectum: Variable degrees of fusion of

the colliculi and tectum result in prominent
beaking and inferior displacement of the
tectal plat
Hydrocephalus
Colpocephaly: The occipital horns and atria
are often mildly enlarged because of
maldeveloped occipital lobes

Prominent massa intermedia: A prominent

massa intermedia, herniation of the third
ventricle into the suprasellar cistern, and an
enlarged suprapineal recess areoften seen

Associated anomalies
Myelomeningocele (88-100%)
Dysgenesis of corpus callosum (80-90%)
Obstructive hydrocephalus following closure of
myelomeningocele (50-98%)
Syringohydromyelia (50-90%)
Aqueductal stenosis (70%)
Absence of septum pellucidum (40%)
Contracted, narrow gyri (stenogyria; 50%)

HeterotopiasDiastematomyeliaSegmentation
anomalies (<10%),
incomplete C1 archMalrotation of the
posterior arches of C1 and C2

;Colpocephaly(1)
;beaked tectum) 2 (
cascade of an inferiorly) 3 (
displaced vermis behind the
;medulla
elongated, tubelike fourth) 4 (
; ventricle
; low-lying torcular herophili) 5(
cerebellar hemispheres) 6(
wrapping around the brainstem
;anteriorly
; concave clivus) 7 (
medullary spur; and) 8(
.medullary kink) 9(

Chiari III malformation

Incompatible with life

This condition always comprises of high
cervical and low occipital encephalocele
Encephaloceles contain varying amounts of
brain i.e. cerebellum, occipital lobes,
ventricles and sometimes pons and medulla
Herniated lesion may be strikingly abnormal
and often nonfunctioning because of necrosis,
gliosis, fibrosis and the presence of
heterotropias

Associated anomalies include petrous and

clivus scalloping, over/undergrown cerebellar
hemisphere, tonsillar herniation, deformed
midbrain, hydrocephalus, dysgenesis of
corpus callosum, posterior cervical vertebral
agenesis, spinal cord syrinx [