CHIARI

MALFORMATION
By
VIDA KWOFIE
ANGELINE MOTARI
ELLENOR CHANCE
LYLIANE MBAH

DEFINITION
Chiari Malformation (CM): A structural defect in
the cerebellum in which brain tissue extends into
the spinal canal (National Institute of Health (NIH), 2014)
CM may also develop when the bony space is
smaller than normal causing the cerebellum and
brain stem to be pushed downwards into the
foremen magnum and the upper spinal canal
(NIH, 2014)

PATHOPHYSIOLOGY

CAUSES
Congenital/ Primary
Structural defects in the brain & spinal cord during
fetal development.
Lack of proper vitamins and nutrients in maternal diet
Genetic mutation

Acquired/ Secondary
Injury
Exposure to harmful substances
Infection

RISK FACTORS
No known risk factors for Chiari malformation
Genetics and heredity may play a role
However these factors havent been confirmed by
research

(The Mount
Sinai Hospital, 2014)

CLASSIFICATION
CM is classified according to:
Severity of the disorder
Parts of the brain that protrude into spinal canal

There are 4 main types

(NIH, 2014)

CLASSIFICATION
Type I:
The base of the skull and upper spinal area are not
properly formed.
It is the most common and only type that is acquired.
It is asymptomatic at birth and childhood and becomes
noticeable in adolescence or adulthood
(Columbian Neurosurgeon, 2014)

CLASSIFICATION
Type II aka Arnold Chiari Malformation
Most common pediatric form of CM.
The cerebellar and the brain stem tissue extend into the
foramen magnum.
Usually associated with hydrocephalus and spinal
bifida.
Partial or complete paralysis of the area below the
spinal opening.
(NIH, 2014)

CLASSIFICATION

Type III: The back of the brain protrudes out of an opening

in the back of the skull area.
Most serious form of CM
Causes severe neurological defects

Type IV: cerebellar hypoplasia

The back of the brain fails to develop normally
Rare and most severe form
(Columbian Neurosurgeon, 2014)

SIGNS AND SYMPTOMS

General symptoms include:
neck pain, balance problems, muscle weakness,
numbness or other abnormal feelings in the arms
or legs, dizziness, vision problems, difficulty
swallowing, ringing or buzzing in the ears, hearing
loss, vomiting, insomnia, depression.
headache made worse by coughing or straining.

SIGNS AND SYMPTOMS

May also affect fine motor skills particularly hand
coordination.
Symptoms change in relation to CSF accumulation
and resulting pressure on nearby nerves and
tissues.

SIGNS AND SYMPTOMS

Adolescents with type I malformation maybe
asymptomatic initially but develop symptoms
later in life.
Infants may have symptoms from any type of
CM
difficulty swallowing, irritability when being fed,
excessive drooling, a weak cry, gagging or
vomiting, arm weakness, a stiff neck, breathing
problems, developmental delays, and FTT.

Associated Conditions
Hydrocephalus - excessive buildup of CSF in the
brain.
Spina bifida (myelomeningocele)-incomplete
development of the spinal cord and/or its protective
covering. Usually seen in Type II malformation.
Hydromyelia -cyst forms within the spinal cords
central canal destroying the center of the cord results
in pain, weakness, and stiffness in the back,
shoulders, and extremities.

Associated Conditions
Tethered cord syndrome -spinal cord attaches itself
to the spine which causing abnormal stretching of
the spinal cord results in permanent damage to the
muscles and nerves in the lower extremities.
Spinal curvature (Scoliosis and Kyphosis) is
common among Children with Type I
malformation.
hereditary syndromes associated with
neurological and skeletal abnormalities.

Diagnosis
Diagnosis
Physical exam
MRI **Used for diagnosis
X-ray & CT ** helpful to identify bone
abnormalities and fluid accumulations that are
often associated with CMs.

Complications
Syringomyelia is the development of a fluidfilled cyst or syrinx within the spinal cord
Myelopathy is the most serious
consequence of syringomyelia. Symptoms
include incoordination in the hands, a
heavy feeling in the legs, or numbness and
tingling in the legs.

Grade classification of disability r/t

myelopathy
According to the Modified Nurick Classification.
Grade 0 - No root signs or symptoms
Grade I - Root signs or symptoms; no evidence of cord
involvement
Grade II - Signs of cord involvement; normal gait
Grade III - Mild gait abnormality; able to be employed
Grade IV - Gait abnormality prevents employment
Grade V - Able to ambulate only with assistance
Grade VI - Chairbound or bedridden

Management/Treatment
Pain management/ muscle relaxer/soft collar
The goals of surgical treatment are decompression of the
syrinx and
Restoration of normal CSF flow
Surgical Procedures
suboccipital craniectomy,
cervical laminectomy,
duraplasty,
arachnoid dissection

Outcome/Prognosis Post surgery

Post surgical review with syringomyelia revealed that
the syrinx persists after surgery at an average rate of
6.7% (range, 0-22%).

Those with little or no neurological deficit or

primarily pain symptoms, can expect an excellent
outcome.
Those with severe and longstanding weakness
and muscle atrophy are less likely to improve.

QUESTIONS???
1. The most common pediatric form of chiari
malformation is:
A. Type I
B. Type II
C. Type III
D. Type IV

2. TRUE/ FALSE
Injury, exposure to harmful substances, or
infection can cause secondary/ acquired chiari
malformation

REFERENCE PAGE

Columbian Neurosurgeons: Chiari Malformation. Department of Neurosurgery.

Retrieved December 5,2014 from columbianneurosurgery.org
National Institute of Neurological Disorders and Stroke: Chiari Malformation Fact
Sheets. National Institute of health. Retrieved December 5,2014 from ninds.nih.gov
The Mount Sinai Hospital. (2014). Chiari malformation. Retrieved from
http://www.mountsinai.org/patient-care/service-areas/neurology/diseases-andconditions/chiari-malformation