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DR. KAELKE BIO 276

Arnold Chiari
Malformation
With Syringomyelia

Leslie Manley
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Introduction to Chiari Malformation

In 1827, Charles Prosper Olivier d’ Angers published an article about

disorders of the spinal cord. While working with cadavers he discovered cavities in

the some of the specimens; since the cavities had no name he put two Greek words

together, syrinx-meaning pipe and myelus-meaning marrow and came up with

syringomyelia[ CITATION Oro03 \l 1033 ]. In 1883, Dr. Cleland was the first to describe

posterior-fossa abnormalities. In 1891, Dr. Hans Von Chiari described the anomaly

as a hind brain deformity and described two types: type 1 anomaly, which was

described as elongation of the cerebellar tonsils, and type 2, which depicted

lengthening of the cerebellum and as well as an abnormality in the fourth ventricle.

In 1907, students of Arnold described four more cases and tagged Arnolds name

to the Chiari malformation. The current classification has evolved to four distinct

types of Chiari Malformation [ CITATION Mue \l 1033 ] [ CITATION His05 \l 1033 ].

I picked Chiari Malformation (CM) with Syringomyelia (SM), because

it has been a part of my life for the last seventeen years; my husband, Bill was

diagnosed with CM with SM. What does this all-mean one author describes CM as

brain stem conundrum[ CITATION Mue \l 1033 ]. In non-technical language, it means the

brain has fallen out of the skull and being pinched by the vertebrae in the neck,

with a cyst in the spinal cord. This is how CM was explained to us in the beginning.
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His CM was acute, the Neurosurgeon told him that if he did not have it done right

away the onset of his CM would disable him permanently, and that he may not walk

within three weeks. He had it done right away and was back to work in a week.

Anatomy and Physiology

Anatomy

The skull for protection surrounds the brain. The bottom of the skull

has an opening called the foramen magnum, which lets blood vessels and nerves

pass through to the brain from the spinal area. The cerebellum, brainstem and the

fourth ventricle lie within the posterior fossa, the base bone of the skull. The

brainstem connects the brain and the spinal cord through the foramen magnum; the

vertebrae protect the spinal cord from injury. The top two vertebrae also called C1

the atlas that the skull articulates with and C2 the axis that helps us move our

head from side to side[ CITATION Oro03 \l 1033 ].

Chiari malformation is divided and described into four different types.

Chiari I Malformation

Chiari I Malformation has traditionally been defined as a downward

herniation of the cerebellar tonsils through the foramen magnum, about three to

five millimeters in length below the posterior occipital bone. This is commonly
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called adult Chiari malformation, presents in patient’s twenties or thirties [ CITATION

Mue \l 1033 ].

Chiari II Malformation

Chiari II malformation, commonly known as pediatric Chiari

malformation and is present at birth, consists of being a downward herniation of

the lower cerebellum and the medulla into the spinal canal. Chiari II is associated

with myelomeningocele and brainstem dysfunction can be linked to mental

retardation in some cases[ CITATION Ali03 \l 1033 ][ CITATION Mic09 \l 1033 ].

Chiari III Malformation

Chiari III malformation is also present at birth, as a cerebellar

herniation into a cervical encephalocele, which is a protrusion of brain tissue

through an abnormal hole in the skull or fissure in the skull [ CITATION Tho99 \l 1033 ]

Chiari IV Malformation

Chiari IV malformation is considered an under developed brain known

as cerebellar hypoplasia, this form is also present at birth. Today they believe that

Chiari IV malformation is different from the other three and most specialists no

longer consider it a type of Chiari malformation[ CITATION Oro03 \l 1033 ].

Syringomyelia

Syringomyelia is an obstruction at the foramen magnum and cerebral

spinal fluid cannot flow normally. This will cause CSF to build up inside of the spinal

cord, this forms a syrinx (cavity). Can be caused by a spinal cord injury, tumor and

damage from inflammation around spinal cord[ CITATION May09 \l 1033 ].

Physiology

Chiari malformation is an unusual congenital neuroskeletal deformity,

the exact pathogenesis and incidences of the disorder are unknown. Theories

suggest that the malformation develops in the embryonic stage [ CITATION Oro03 \l

1033 ]. Many researchers and specialists believe that Chiari malformation may result

from an underdeveloped posterior occipital bone in the skull. The posterior fossa is

too small and does not provide room for the cerebellum and lower brain stem.

[ CITATION Mue \l 1033 ]

Signs and Symptoms

Many patients with Chiari malformation have no signs or symptoms and

do not need to be treatment. Their condition is only detected when other tests are

preformed for unrelated disorders[ CITATION May08 \l 1033 ]. Many times, it is

misdiagnosed because the symptoms are so common with other disorders. There
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are no measurable signs for Chiari malformation to help with diagnosis. X-ray can

measure the length of the syrinx in the syringomyelia.

Symptoms can include:

Headache

Headaches being the most common symptom described by most

patients these differ from normal headaches. Chiari headaches start in the

occipital region of the head radiating behind one eye region or both, then to the

temporal region. Pain is described more like a pressure inside the head than a

traditional pain. These pains are amplified when coughing or sneezing. [ CITATION Dia \l

1033 ]

Dizziness-Dysequilibrium-Weakness

The second most common symptom of Chiari is the feeling of

lightheadedness and being dizzy, intermittently. Many suffer from dysequilibrium

to the point of having unsteady gait and not being able to walk without falling

frequently. Some being unstable enough to be wheel chair bound. This can tie in

with weakness of the lower extremities, which patients also suffer from and many

have weakness in their upper extremities too limiting activity. They often complain

of tingling in their extremities as well [ CITATION Dia \l 1033 ].

Pain

The most common pain noted was neck pain, with lack of range of

motion described as stiffness, dull aching, to the extreme of stabbing. Some

patients feel burning or stabbing pain in their upper extremities and in the

thoracic region, this is most often associated with the syringomyelia. Patients that

have extreme pain also had complaints of nausea [ CITATION Dia \l 1033 ].

Neuro-Ophthalomologic Symptoms

Blurred vision was reported in patients ranging from inability to read

small print or having the vision to drive. Chiari II effects eye movements because

of the effects that it has on the cerebellum; the vermis expands when compressed

and spares some ocular motor functions. The ones without the expanded vermis

experience abnormal eye movements. This was discovered while doing vision testing

among a few other tests like IQ, and cognitive functions[ CITATION Mic09 \l 1033 ].

Depression

Reports of patients having mild to severe depression and occurs in

half of the patients with Chiari malformation. Many patients depression can be

cured by antidepressants. Some patients experience severe enough depression to

want to commit suicide or attempt it. Some Chiari patients just have major anxiety.
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Neurological Problems

Chiari patients often suffer from neurological problems, varying from

tingling in extremities to more serious such as seizures. Many experience radiating

pain from different areas of the body mainly from their nerve tracts or

unexplained itching. Some have memory loss as mild as forgetfulness to more

serious problems such as forgetting where they live or knowing who they are. Facial

numbness is problem for biting the tongue and not knowing how bad it is. Many

Chiari patients have loss of pain receptors, which can be very dangerous. They have

problems swallowing and some wake up in the middle of the night choking with

unknown reason.

Diagnosis and Prognosis

When assessing a patient with possible Chiari malformation, a

thorough medical history and physical exam with emphasis on the neurological

system such as Romberg, finger to nose, heel to shin, gait, rapid movements, hot,

cold, light touch and pinprick sensations. They also may go through cranial nerve

testing also.[ CITATION Mue \l 1033 ]

In the past, the only diagnosis was the physical, and made diagnosing

Chiari malformation difficult. Today, diagnosing Chiari has become much easier
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with more up to date imaging devices, for example x-rays are clearer. Most

hospitals and clinics have computerized tomography (CT), and magnetic resonance

imaging (MRI) being the most popular choice for diagnosing CM. MRI gives the

physician or specialist the most clear image, where they can measure syrinxes, how

far distended the cerebellum is and what classification the patients Chari

malformation, with or without syringomyelia.

Prognosis is usually good in CM patients, depending on the type and

severity of the CM they have. Most patients, where there is neurological damage,

usually recover their loss of feeling, and can go back to their life the way it was

before CM set in. In very severe cases, patients do not recover well, some of the

complications include hydrocephalous or paralysis. In Chiari IV, death is usually the

prognosis or severe mental retardation due to under developed brain.

Treatments

Treatment I

The most common treatment for Chiari malformation is decompression

surgery of the posterior fossa. It is also the treatment for most neurosurgeons to

perform. Decompression surgery has a few different ways it can be done; with or

without stabilization of neck and whether to use a duraplasty product, cadaver

tissue or bovine mater. The objective of the surgery is to relieve the pressure on

the cerebellar tonsils, brainstem and possible fourth ventricle, to enlarge the

posterior fossa space and to normalize the flow of CSF.

The patient is prone position with a halo screwed into the head, to

stabilize the head during surgery. A midline incision is made from the middle of

the occipital bone to the base of C2 or C3, so that the surgeon has plenty of visual

area of the skull. A craniectomy is preformed to expose the brain, cerebellum and

brain stem. Lamination of C1 and C2 is done to give more visualization during

surgery, which also helps when they need to shunt or sent to drain the spinal cord.

The dura mater is carefully cut away, and replaced with a duraplasty graph or

cadaver tissue that covers the cerebellar tonsils, the brainstem and the fourth

ventricle to hold them in place. Some surgeons use electrocautery to shrink the

tonsils. The graph also helps in regulation of the CSF flow [ CITATION Fra09 \l 1033 ].

The main complications of this surgery are CSF leakage, infection,

septic meningitis, apnea and hydrocephalus. [ CITATION ZQZ08 \l 1033 ]. Earlier patients

that did not have a stabilization done or the fusion of C1 and C2; have complaints

about the instability that they are experiencing. In some cases, the instability is so

great they have to undergo tracheostomy ventilation, so that they can

breathe[ CITATION Fre09 \l 1033 ].

Surgical procedure II for patient with a diagnosis of Chiari I

malformation, it is a fairly new procedure, transoral odontectomy and suboccipital

craniectomy, 2008, being done for treatment. This minimally evasive surgery is

done through the open mouth. The incision extends from the inferior third of the

clivus to the top of C3. This facilitates the decompression of the cerebellar

tonsils, the lower brainstem and the spinal cord. Therefore, there is minimal

removal of the atlas arch and occipital bone, which makes reconstructive surgery

less complicated, since most of the bones are there for them to work with. There

construction makes for greater cranial movement after surgery [ CITATION Ste08 \l 1033

Treatment II

Drug therapies, patients that present with headaches from physical

strain or the classic cough headache from Chiari. The first drug was

Acetazolamide; clients taking this had rapid response to this drug and were

relieved of their headaches. The medication relieves the inner cranial pressure and

depresses the excitability of the neurons. The second drug Indomethacin had very

similar results.

My opinion of the treatments I have found, my first opinion would

have been to go with the decompression, craniectomy surgery with stabilization for
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someone that also has syringomyelia. After reading about the transoral

odontiodectomy and suboccipital craniectomy, I believe this would be my

recommendation for someone with Chiari malformation without syringomyelia. It is

less evasive than the traditional surgery, and the patient’s quality of life would be

much better because they have more cervical movement with the transoral

odontiodectomy.

I would not recommend the drug treatment programs because, in the

end who knows what can happen. I would think from learning what I have about

drugs that the body would become immune to these, because they alter, the

neurons and blood flow so they would have the same effect as other drugs they

quit working and the body needs more to function.

Conclusion

Dr. Hans Von Chiari discovered these deformities over a century ago,

and to think how far technology has progressed in diagnosing diseases, going from

examining cadavers in hiding to the imaging devices we have today. He may not have

known how to fix his discoveries, but his name was still given to the deformity.

Although, even today people go without treatment for Chiari malformation, because

it can present itself in many ways often being misdiagnosed, the MRI is the only
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true way to diagnose Chiari. I believe that after being diagnosed that surgery be

done as soon as possible, there is no time frame for Chiari it can strike at any time

in life and the onset can be acute or very slow over time.

References
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Attenello, F., McGirt, M., Garces-Ambrssi, G., Chaichana, K., Carson, B., & Jallo, G. (2009).

Suboccipital decompression for Chiari 1 Malformation:outcome comparison of

duraplasty with expanded polytetrafluoroethylene dural subsitute versus

pericranial autograft. Child’s Nervous System, 25(2), 183-190. Retrieved from

Ebscohost database.

Green, A. (2006, July/‌August). Update on Chiari Malformation: Clinical Manifestations,

Diagnosis, and Treatments. Pediatric Nursing, 29(4). Retrieved from Ebscohost

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Hwang, S., Heilman, C., Riesenburger, R., & Kryzanski, J. (2008). C1-C2 arthodesis after

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compression in Chiari 1 patients. European Spine Journal, 17, 1211-1217. Retrieved

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http://www.mayoclinic.com/‌health/‌syringomyelia/‌DS01127
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