The n e w e ng l a n d j o u r na l of m e dic i n e

Review Article

Allan H. Ropper, M.D., Editor

Congenital and Acquired Chiari Syndrome

Robert M. Friedlander, M.D.​​

C
hiari malformation, an anomaly of the posterior cranial From the Department of Neurological
fossa, also known as Arnold–Chiari malformation, was described in autop- Surgery, University of Pittsburgh School
of Medicine, University of Pittsburgh
sies by Hans Chiari, an Austrian pathologist, in 1891. He defined four Medical Center, Pittsburgh. Dr. Fried-
distinct anatomical configurations in which the cerebellar tonsils (the most caudal lander can be contacted at f­ riedlanderr@​
part of the cerebellum) protruded below the lower margin of the foramen magnum.1 ­upmc​.­edu or at the Department of Neu-
rological Surgery, University of Pitts-
Several subtypes have subsequently been described, often in association with other burgh School of Medicine, University of
anomalies, but the essential feature remains the descent of the cerebellar tonsils Pittsburgh Medical Center Presbyterian
below their normal position just above the rim of the posterior fossa. Hospital, 200 Lothrop St., Ste. B-449,
Pittsburgh, PA 15213.

N Engl J Med 2024;390:2191-8.

Cl a ssific at ion DOI: 10.1056/NEJMra2308055
Copyright © 2024 Massachusetts Medical Society.
Although many characteristics of Chiari malformation are similar in children and
adults, this review focuses on the most common type, adult Chiari malformation CME
type 1 (CM1), in which only the cerebellar tonsils descend below the foramen
magnum. This malformation can be due to a congenitally small posterior fossa,
or it can be acquired as a result of high pressure from above the cerebellum or low
pressure below the cerebellum that displaces the tonsils. The next most common
Chiari malformation is type 2, which is characterized by descent of the cerebellar
vermis and fourth ventricle below the foramen magnum, usually accompanied by an
open myelomeningocele. This malformation was recently reviewed in the Journal.2
Chiari malformation types 3 (occipital encephalocele), 4 (cerebellar hypoplasia),
and 5 (cerebellar hypoplasia and occipital-lobe ectopia into the posterior fossa) are
seen in children and newborns and are more rare and more severe than CM1.
Types 2, 3, 4, and 5, as well as a type that is associated with craniosynostosis
(premature closure of cranial sutures), are congenital syndromes in children.3-5
Congenital CM1 occurs in patients with a constitutionally small posterior fossa.6,7
In adults, symptoms from CM1 most commonly develop before the age of 45 years
(mean age, 40 years), with an approximate female:male ratio of 3:1, whereas in pe-
diatric CM1, the mean age at presentation is 8 years, with an approximately equal
female:male ratio.3,8-12
As the cerebellar tonsils protrude below the foramen magnum, obstruction of
the foramina of Luschka and Magendie of the lower medulla and compression of
the upper cervical spine may occur, impeding the flow of cerebrospinal fluid (CSF)
from the fourth ventricle to the upper spinal subarachnoid space. In some in-
stances, this is accompanied by a syrinx (cystic cavity), which is most commonly
located in the cervical cord but can involve any part of the spinal cord and rarely
the brainstem, as described below. Herniation of the cerebellar tonsils to at least
5 mm below the foramen magnum is the usual definition of CM1 (Fig. 1). How-
ever, some symptomatic patients have smaller herniations, and some asymptomatic
patients have larger herniations.9,13 A study involving 2000 asymptomatic adults
showed a prevalence of 0.9% for tonsillar descent that was at least 5 mm below the
foramen magnum on magnetic resonance imaging (MRI), a finding that is consis-

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 The n e w e ng l a n d j o u r na l of m e dic i n e

B
the malformation (see below) had failed in many
A
of the patients in this series, creating a potential
referral bias that may have led to overestimation
of the prevalence because the rate of surgical
failure is increased among patients with connec-
M tive-tissue disorders.20

Pathoph ysiol o gy
As mentioned above, low-lying cerebellar tonsils
can result in obstruction of normal CSF outflow
C D
of the fourth ventricle.21,22 It has been suggested
that this obstruction leads to dissociation of nor-
mal CSF pulsatile flow between the cranial and
spinal compartments, which is likely to play a
role in the headache that often accompanies the
M
malformation and in the formation of a CSF-filled
cavity (syrinx). The other main symptoms of CM1
T T are due more directly to downward tonsillar her-
niation through the foramen magnum and pres-
sure on the lower medulla and upper cervical
spinal cord, as described below. In some cases,
Figure 1. Features of CM1 on Magnetic Resonance Imaging. particularly those with less than 5 mm of tonsil-
A normal sagittal T1 image (Panel A) shows that the cerebellar tonsils are lar descent, partial obstruction of CSF flow occurs
above the plane of the foramen magnum. A normal axial T2-weighted im- posteriorly at the fourth ventricular CSF outlets
age (Panel B) shows the foramen magnum (outlined by blue dots) and the as a result of arachnoid webs, tumors, or an
lower medulla (M). Ample space for cerebrospinal fluid (CSF; white signal
surrounding the medulla) is present. A sagittal T1-weighted image of a
ectatic posterior inferior cerebellar artery.
typical patient with Chiari malformation type 1 (CM1) (Panel C) shows cer- Although a congenitally small posterior fossa
ebellar tonsillar herniation below the foramen magnum, as indicated by is the most common cause of cerebellar tonsillar
the red line (McRae’s line). The blue line, perpendicular to McRae’s line herniation, acquired cerebellar descent can result
and extending to the tip of the tonsil, is used to determine the magnitude from pressure gradients created by a pulling ef-
of tonsillar herniation. An axial T2-weighted image of a patient with CM1
(Panel D) shows the tonsillar tissue (T) within the foramen magnum (out-
fect from below or a pushing effect from above.
lined by blue dots) and compression of the lower medulla (M). Obliterated Pull from below the foramen magnum is the result
CSF space at the foramen magnum is indicated by the virtual absence of of spinal CSF leakage, a CSF–venous fistula, or a
white signal. tethered cord.23,24 Push from above the foramen
magnum can result from hydrocephalus, subdural
collections, a brain tumor, or an arachnoid cyst,
tent with the anatomical definition of CM1.14 with a tumor or cyst particularly implicated when
The term Chiari syndrome refers to the manifes- it is large.9,13,17,25,26
tation of symptoms attributable to the malfor-
mation (Table 1).9,16,17 Signs a nd S ymp t oms
Although most often CM1 occurs as a spo- of Chi a r i M a l for m at ion
radic condition, with a low familial prevalence,
tentative evidence of a genetic cause has been Symptoms attributable to descent of the cerebel-
reported in a few cases, with many genes impli- lar tonsils into the foramen magnum vary. The
cated — none strongly — and no candidate gene most common symptom is headache, which is
offering a unifying explanation for the malfor- most likely due to transiently raised intracranial
mation.16,18 An association has also been found pressure from partial blockage of CSF flow. The
between CM1 and connective-tissue disorders such headache is usually suboccipital and dull or
as the Ehlers–Danlos syndrome.19 In one study, the throbbing, but it can be located anywhere in the
prevalence of connective-tissue disorders with cranium and have other characteristics, includ-
CM1 was 12.7%. However, surgical treatment of ing migraine and tensionlike discomfort. Chiari

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 Congenital and Acquired Chiari Syndrome

headaches are characteristically exacerbated by Table 1. Imaging Features of CM1 and Signs and Symptoms of the Chiari
Valsalva-like activities such as coughing, laughing, Syndrome (Symptomatic CM1).*
sneezing, straining, lifting heavy objects, and
changing body or head positions. Imaging features of CM1
An additional symptom, which is independent Cerebellar tonsillar herniation below the foramen magnum; ≥5 mm, or 3–5
mm in the presence of a syrinx or peglike tonsillar shape (<3 mm consid-
of but often associated with headaches, is pares- ered normal)
thesia in one or both hands, often involving all
Signs and symptoms of the Chiari syndrome
the fingers (without a radicular distribution). This
Headache (typically occipital or suboccipital), Valsalva-associated headache
symptom is probably related to compression of
the dorsal medulla or dorsal columns of the up- Numbness or weakness in hands, feet, or both
per cervical cord. Similar symptoms can occur in Oscillopsia (oscillating objects or blurry vision)
the feet, although this location is less common. Hearing problems (ear pressure, hyperacusia, or hypoacusia), tinnitus
Other, less common but characteristic signs and Balance problems (ataxia or dizziness)
symptoms are sleep apnea; tinnitus, which may Nystagmus, particularly downbeating
be pulsatile; hypoacusis or hyperacusis; difficulty
Sleep apnea
swallowing; several forms of nystagmus, includ-
ing downbeat nystagmus (caused by only a few Swallowing difficulty and various cranial-nerve palsies
other disorders, such as lithium toxicity and tu- * Information adapted from Massimi et al.,5 Ciaramitaro et al.,9 and Kumar et
mors at the cervicomedullary junction); upgaze al.15 CM1 denotes Chiari malformation type 1.
paresis; oscillopsia; blurry or double vision; and
dizziness.8,9,27 Neurocognitive and psychiatric
symptoms have been detected by means of formal particularly sagittal images, and with cervical
testing in patients with CM1, although a direct spine MRI to detect a syrinx. As mentioned above,
cause and effect have not been identified.28,29 It is
a cerebellar tonsillar descent of at least 5 mm
unclear, for example, whether cognitive and psy- below the foramen magnum is required to meet
chiatric symptoms result from chronic pain, but imaging criteria for CM1.13,17,27 The tonsils are
some investigators consider the symptoms to be often also pointed (i.e., peglike), instead of nor-
due to impaired cerebellar circuitry involved in mally rounded. Although this finding is not re-
cognition.30 Chronic fatigue syndrome has been quired for the diagnosis, it indicates tonsillar
proposed as a CM1 symptom, although the as- compression and shaping by the dorsal bony
sociation has not been convincingly shown.31 structures of the posterior fossa. In one consen-
The spinal cord syrinx may be symptomatic sus report that broadly reflects clinical practice,
or asymptomatic, with a large syrinx more likely cerebellar tonsillar herniation to between 3 and
to be symptomatic. Patients with a syrinx may 5 mm below the foramen magnum has also been
present with complex symptoms that are inde- classified as CM1 in the presence of a syrinx or
pendent of those directly caused by the tonsillarpeglike shape of the tonsils.9 This measurement
herniation — typically numbness or decreased is obtained on a midsagittal MRI scan by draw-
pain or temperature sensitivity in the hands and ing a line from basion to opisthion (the anterior
over the shoulders, hand weakness and atrophy, and posterior bone limits, respectively, of the
and arm, leg, neck, or back pain, as well as blad-
foramen magnum, McRea’s line) and drawing a
der or bowel incontinence. In patients with po- perpendicular line down to the lowest aspect of
sitional headaches that are more severe when the the cerebellar tonsils, the length of the latter
patients are standing and less severe when they representing the magnitude of descent of the
are lying down, the diagnosis of spinal CSF leak tonsils. An additional consideration in affirming
and intracranial hypotension as an acquired causethe diagnosis is the degree of “crowding” in the
of tonsillar herniation should be considered. posterior fossa (reduction in the space for CSF to
flow) at the level of the foramen magnum on a
T2-weighted axial image (Fig. 1).
Im aging
Cardiac-gated CSF flow imaging (cine flow
Although tonsillar descent can be identified by MRI), a technique used to evaluate CSF flow at
computed tomographic (CT) scanning, Chiari mal- the level of the fourth ventricle and the foramen
formations are better visualized with brain MRI, magnum, normally shows bidirectional CSF flow

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 The n e w e ng l a n d j o u r na l of m e dic i n e

with systole and diastole. Compromised CSF stem compression), platybasia (abnormal angu-
dynamics on cine flow MRI is characteristic of lation of the skull base and spine), and cranio-
symptomatic CM1 and has been predictive of cervical instability.
abatement of symptoms after surgery.22 An ab- As noted above, acquired cerebellar tonsillar
normal cine flow study is not required for di- herniation can also result from a spinal CSF leak
agnosis but provides supportive evidence of as the contents of the posterior fossa are pulled
symptomatic CM1, with 76% sensitivity and 62% down into the upper spinal canal because of the
specificity in one series.32 Increased cardiac cycle– pressure gradient created by the leak. In such
associated motion of the tonsils has also been cases, cranial and spinal MRI often shows dural
shown on MRI imaging in patients with symp- enhancement after the administration of gado-
tomatic CM1, but its role in diagnosis has not linium, a finding consistent with intracranial
been established. This abnormal type of tonsil- hypotension.24,38 A spinal MRI is performed to
lar motion is reduced after surgical decompres- identify the presence and site of the CSF leak,
sion of the posterior fossa.33 but both are challenging to detect, and more
An imaging feature often found in patients elaborate studies, such as injection of nonionic
with CM1 is compression of the pituitary gland iodinated contrast medium into the CSF space,
from above, resulting in CSF within the sella may be needed. Another cause of reduced CSF
turcica, referred to as empty sella. This finding pressure is a direct CSF–venous fistula (drainage
is consistent with increased intracranial pres- of spinal CSF into a vein), which results in sec-
sure. It is more commonly associated with idio- ondary downward tonsillar herniation.23,24
pathic intracranial hypertension (pseudotumor
cerebri), and its presence can aid in determining T r e atmen t
that the Chiari malformation is likely to be ac-
quired from raised intracranial pressure rather The twofold goal of treatment is to relieve com-
than congenital.34 pression of the cervicomedullary junction and
A syrinx develops within the upper spinal normalize CSF flow at the foramen magnum.
cord in 27 to 66% of patients with CM1. The Validated medical treatments that will accom-
syrinx is located in the middle of the spinal cord plish this are lacking. Asymptomatic patients, in
within the central canal (hydromyelia) and is whom CM1 may have been detected incidentally
lined by normal ependymal cells or is located on imaging performed for various reasons, do
eccentrically in the spinal cord (syringomyelia) not require surgery if there is no syrinx. The
and is not lined by ependyma.10,35 A syrinx in the Congress of Neurological Surgeons has issued
cervical spinal cord can extend caudally through- guidelines generated from a systematic review
out the cord or rostrally into the brain stem. that concluded that decompression of the poste-
Some patients have compression of the ven- rior fossa leads to a decrease in symptoms of
tral lower brain stem or kinking of the brain Chiari malformation.39 However, the consensus
stem as a result of the downward displacement guidelines also indicated that evidence to support
of the cerebellum (classified as Chiari malforma- any specific surgical approach was insufficient.9
tion type 1.5), which is addressed with a complex The management of asymptomatic CM1 in
diagnostic approach (dynamic plain films and a patients with a syrinx is controversial. Guidelines
CT scan) and special surgical procedures. Depend- suggest close follow-up of such patients, with
ing on the degree of ventral brain-stem compres- surgery recommended if the syrinx expands or
sion and occipital–cervical mechanical instability, associated symptoms appear.5,9
ventral odontoid decompression and occipital–cer- Surgical procedures to decompress the poste-
vical fusion may be performed in addition to stan- rior fossa vary in complexity and extent and in-
dard decompression of the posterior fossa.9,36,37 clude removing the lower aspect of the occipital
The proximate causes of ventral compression in- bone in the posterior fossa; widening the open-
clude basilar invagination (superior translation of ing of the foramen magnum and removing the
the top of the C2 vertebra into the foramen mag- posterior arch of C1; opening the dura and then
num), a retroflexed odontoid process (posterior incising the arachnoid, which can tether the
angulation of the odontoid process causing brain- tonsils inferiorly, to release the tissue; resecting

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 Congenital and Acquired Chiari Syndrome

Normal CSF flow Neural compression and impaired CSF flow

Compromised
CSF flow

Arachnoid
granulation
Area of bone
Choroid to be removed
plexus
Compressed
spinal cord
Herniation of
and central
cerebellar
canal
tonsils

Compromised
CSF flow

Cerebellum
Unidirectional
CSF flow
Posterior fossa decompression and
CSF flow reestablishment
Central
canal
Base of Unidirectional
C1 Foramen of skull CSF flow
C1 Magendie
Spinal
cord
C2
C2
Bidirectional Duraplasty
CSF flow
C3 Bidirectional
C3
CSF flow

Figure 2. Surgical Goal of Posterior Fossa Decompression and Reestablishment of CSF Flow.
In normal CSF flow (left panel), CSF is produced by the choroid plexus (located in the lateral, third, and fourth ventricles) and flows es-
sentially unidirectionally through the ventricles. In the caudal aspect of the fourth ventricle, CSF exits from the foramen of Magendie
(midline) into the cisterna magna and from the foramina of Luschka (lateral) into the pontine cistern, where through bidirectional flow,
CSF distributes down the thecal sac, and back up around the cerebral hemispheres and is absorbed by the arachnoid granulations. In
CM1, the cerebellar tonsils herniate through the foramen magnum (upper right panel), which compromises CSF flow out from the
fourth ventricle and rostral flow through the cisterna magna. In addition, tonsillar herniation results in pressure on the lower medulla
and upper cervical cord. The lower aspect of the occipital bone and medial aspect of the posterior arch of C1 (in red) are removed dur-
ing decompression of the posterior fossa. Following bone decompression and expansile duraplasty, normal CSF flow is reestablished
(lower right panel), and pressure on the medulla and cervical cord is eliminated.

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 The n e w e ng l a n d j o u r na l of m e dic i n e

arachnoid membranes at the level of the fourth cranial hypertension.44 In patients with persistent
ventricular outlet (which can obstruct CSF flow); or recurrent symptoms after surgery, a lumbar
and reducing the volume of the tonsils (by elec- puncture can help distinguish chemical menin-
trocautery) or performing a complete tonsillar gitis from infection and increased intracranial
resection. When the dura is opened, expansion of pressure.
the subarachnoid space is achieved with a dura- Chemical meningitis can be treated with glu-
plasty graft and closure of the dura in a water- cocorticoids; infection can be treated with ap-
tight fashion (Fig. 2). As mentioned above, no propriate antibiotic agents and, when indicated,
surgical procedure has been validated as supe- surgical irrigation and débridement; and elevated
rior to others, and practice varies across institu- CSF pressures can be treated with a diuretic agent
tions and surgeons. The use of duraplasty is (i.e., acetazolamide) or may require a ventriculo-
supported by some systematic reviews, especially peritoneal shunt. Patients with a syrinx who have
for patients with CM1 and syringomyelia.35,40 Pre- undergone decompression may have abatement
liminary results from a cluster randomized trial of associated symptoms, and regression of the
comparing posterior fossa decompression with syrinx occurs in approximately 78% of patients.3
and without duraplasty in children and young The consensus guidelines indicate that alleviation
adults (ClinicalTrials.gov number, NCT02669836) of symptoms does not correlate with resolution of
suggest similar rates of surgical complications the syrinx, and it is not clear why some patients,
and similar clinical outcomes.41 The overall extent despite having undergone proper decompression,
of the intradural procedure is also controversial.9,40 do not have decreases in syrinx-related symptoms
Acquired tonsillar herniation due to a CSF or improvements in the findings on imaging
leak or a mass is treated by addressing the un- studies.
derlying leak or lesion.24 Lumbar punctures are A meta-analysis of surgical outcomes has pro-
relatively contraindicated in patients with congeni- vided support for greater relief from symptoms
tal CM1 who have not undergone surgery, given after bone decompression and duraplasty (with or
the risk that lowered spinal CSF pressure may without tonsillar shrinkage or resection) than
cause further tonsillar herniation. after bone-only decompression. The differential
improvement is more evident for patients with
syringomyelia. More extensive surgery is associ-
Surgic a l Ou t c ome s
ated with higher complication rates.35,40 A meta-
Patients often experience immediate relief of analysis comparing bone decompression and
limb paresthesia and tinnitus after surgical de- duraplasty with the more extensive procedure of
compression of CM1. Most of the other symp- bone decompression, duraplasty and subarach-
toms are diminished or eliminated within sev- noid dissection, and tonsillar shrinkage showed
eral weeks to months. For example, abatement similar alleviation of symptoms with the two
of headaches and other symptoms after surgery procedures, but complication rates were higher
has been reported in 80% or more of patients in with the latter procedure.45
several series.3,8,29,35,42 The two most common According to one study involving an interna-
postoperative complications with posterior fossa tional database,12 6.8% of patients underwent
decompression are foreign-body (chemical) asep- reoperation because of symptoms that had re-
tic meningitis from a dural graft and CSF leak, lapsed or had not been alleviated. These circum-
which occur, respectively, in up to 32% and 21% stances occur for two main reasons: insufficient
of patients who have undergone surgery.12,35,42 The decompression from the initial procedure or the
risk of chemical meningitis may be associated development of intradural scarring. Cine flow
with the graft type. Rates of chemical meningitis MRI can be useful in evaluating these problems.
are lower with autografts or allografts than with Abnormal cine flow in patients who never had
artificial or bovine grafts.35,43 CSF leak results improvement suggests insufficient decompres-
from CSF seeping around the graft suture line sion. Abnormal cine flow in patients who initially
and is often exaggerated by a coexisting increase had improvement and thereafter had symptom-
in intracranial pressure. After surgery, some pa- atic relapse suggests subarachnoid scarring from
tients have a transient or permanent increase in the surgery. Patients who undergo bone-only de-
intracranial pressure, including idiopathic intra- compression without duraplasty appear to have a

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 Congenital and Acquired Chiari Syndrome

higher rate of symptomatic relapse or lack of have atypical symptoms or borderline imaging
improvement leading to reoperation.46 findings. For these patients, cine flow MRI can
be informative.22 Research is required in order to
identify patients who would benefit from surgi-
C onclusions
cal decompression, delineate appropriate surgi-
With the wide availability of cerebral imaging, cal techniques, and improve our understanding
recognition of Chiari malformations has become of the association of CM1 with cognitive and
common, and determining which patients have psychiatric symptoms.
symptoms associated with this anatomical ab- Disclosure forms provided by the author are available with the
normality can be challenging. Many patients full text of this article at NEJM.org.

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