Literature Review
Chiari Malformation Type 1: A Systematic Review of Natural History and Conservative
Management
Benjamin Langridge1, Edward Phillips1, David Choi2
Key words
- Adult
- Arnold-Chiari
- Chiari
- Conservative
- Malformation
- Management
- Surgical
Abbreviations and Acronyms
CM-1: Chiari malformation type 1
From the 1University College London Medical School,
Bloomsbury, London; and 2National Hospital for Neurology
and Neurosurgery, London, United Kingdom
To whom correspondence should be addressed:
Benjamin Langridge, B.A.
[E-mail: benjamin.langridge.14@ucl.ac.uk]
Citation: World Neurosurg. (2017) 104:213-219.
http://dx.doi.org/10.1016/j.wneu.2017.04.082
Journal homepage: www.WORLDNEUROSURGERY.org
Available online: www.sciencedirect.com
1878-8750/$ - see front matter ª 2017 Elsevier Inc. All
rights reserved.
INTRODUCTION
The Arnold-Chiari malformation type 1,
which we refer to as Chiari malformation
type 1 (CM-1), was originally defined by
Hans Chiari as an “elongation of the ton-
sils and the medial parts of the inferior
lobes of the cerebellum into cone-shaped
projections which accompany the me-
dulla oblongata into the spinal canal.”1 A
common treatment for symptomatic CM-
1 is foramen magnum decompression,
and although there is debate around the
technical aspects of surgery, the outcomes
and risks of surgery are well documented.
However, little is known about the natural
history of CM-1 without surgery in symp-
tomatic and asymptomatic individuals,
and this information is necessary to decide
the merits of surgical management. CM-1
is uncommon in adults, and there is lit-
tle clear evidence to guide management.
We present, to our knowledge, the first
systematic review of the natural history
and conservative management of CM-1 in
adults to help surgeons and patients
decide when to perform surgery for CM-1.
WORLD NEUROSURGERY 104: 213-219, AUGUST 2017
- BACKGROUND: Chiari malformation type 1 (CM-1) is a variation of hindbrain
development that can sometimes occur in asymptomatic individuals. Conven-
tional treatment is surgical decompression, but little is known about the natural
history of patients who do not undergo surgical management. This information is
critical to determine how these patients should be managed. We conducted a
systematic literature review to determine the natural history of CM-1, particu-
larly in patients who did not undergo surgery and in asymptomatic individuals, to
help patients and physicians determine when surgery is likely to be beneficial.
- METHODS: The literature search was performed following Preferred
Reporting Items for Systematic Reviews and Meta-Analyses guidelines using the
electronic databases PubMed, Scopus, Cochrane Library, and Web of Science.
Inclusion and exclusion criteria were predefined.
- RESULTS: In symptomatic patients who did not undergo surgery, headaches
and nausea often improved, whereas ataxia and sensory disturbance tended not
to improve spontaneously. Of patients, 27%e47% had an improvement in
symptoms after 15 months, and 37%e40% with cough headache and 89% with
nausea who were managed nonoperatively improved at follow-up. Most
asymptomatic individuals with CM-1 remained asymptomatic (93.3%) even in the
presence of syringomyelia.
- CONCLUSIONS: The natural history of mild symptomatic and asymptomatic CM-
1 in adults is relatively benign and nonprogressive; the decision to perform surgical
decompression should be based on severity and duration of a patient’s symptoms at
presentation. It is reasonable to observe a patient with mild or asymptomatic
symptoms even in the presence of significant tonsillar descent or syringomyelia.
We reviewed published studies of CM-1 to No time limits were set during the search.
determine the following: 1) the presenting Indexes were accessed between February
features of CM-1 in adults, 2) common 2, 2016, and February 22, 2016. A search
indications for surgical intervention, 3) the strategy was created using the terms
natural history of CM-1 in adults, 4) the listed in Table 1. Results were reviewed to
natural history of asymptomatic incidental exclude duplicates, articles not published
CM-1, and 5) the natural history syringo- in the English language, and articles that
myelia with CM-1. were irrelevant in topic (Figure 1). The
full texts of the remaining articles were
reviewed in detail and outlined in this
MATERIALS AND METHODS
systematic review. The reference lists of
Data Sources and Search Strategy these full articles were hand-searched for
We performed a systematic literature re- any additional articles that were missed by
view in the format recommended by the the original search strategies.
Preferred Reporting Items for Systematic
Reviews and Meta-Analyses Guidelines.2 A Study Selection and Analysis
literature search was performed using the We included any article related to CM-1
electronic databases PubMed, Scopus, but excluded articles relating solely to
Cochrane Library, and Web of Science. pediatric patients (<18 years old) or
www.WORLDNEUROSURGERY.org 213

BENJAMIN LANGRIDGE ET AL.
Table 1. Search Terms Used to Identify
Relevant Publications* and Reasons for
Article Exclusion
Search Terms
1. Chiari malformation 1 conservative
2. Chiari malformation 1 nonoperative
3. Chiari malformation 1 natural history
4. Chiari malformation 1 nonsurgical
5. Chiari malformation 1 asymptomatic
Number
Reasons for Exclusion Excluded
Title and abstract review
Unrelated topic
Pediatric only
NoneEnglish language
Nonhuman
Chiari malformation type 2
Acquired malformation
Full-text review
Unrelated to question
Surgical interventions
Pediatric only
Not Chiari malformation 1
Congenital syndromes
Duplicates
Acquired malformations
*PubMed, Scopus, Cochrane Library, and Web of
Science.
patients with additional congenital syn-
dromes. Articles that included both pedi-
atric and adult patients were retained, and
the relevant adult data were included. Ar-
ticles were analyzed with a predetermined,
standardized appraisal pro forma.
RESULTS
Selected Articles
Our search strategy (Table 1) identified 344
articles, which were reduced to 198 after
excluding duplicates. After title and
abstract review, the number of relevant
articles was reduced to 44. Following
full-text review, a further 33 articles were
excluded, leaving 11 articles for detailed
inclusion in this systematic review.3-13
214 www.SCIENCEDIRECT.com
Four further articles were identified from
article bibliographies.14-17 Therefore, 15
articles were identified for inclusion in
this systematic review (Figure 1). Table 1
also shows the breakdown of reasons for
article exclusion, and Table 2 outlines the
characteristics of the reviewed articles.
Some heterogeneity exists in the
definition of CM-1 used in published ar-
ticles; Table 3 provides a summary of the
definitions used in the reviewed articles.
Presenting Features of CM-1 in Adults
CM-1 manifests in numerous ways.
Headaches, of either the cough or
migrainous type, and paresthesia are the
74 most common presenting symptoms.
65 Other common presenting symptoms
include nausea, dysphagia, apnea, clonus,
6
cerebellar symptoms, drop attacks, muscle
5 atrophy, and dysphonia (Table 4). Case
3 reports exist of more unusual
presentations, such as orthopnea,19
1
vertigo,20 and diplopia.21 Additionally,
many cases of CM-1 are found inciden-
12 tally on magnetic resonance imaging
6
scans.11
The literature search found 21 articles
5 with details on the presenting symptoms of
4
1 cases of CM-1.4-6,8-10,15,16,18,20-29 Of
3 these, 13 articles, most of which were
case reports, discussed <10 pa-
2 tients.6,8,10,15,16,19-22,24-27 Four articles
1 looked at between 10 and 40 patients,3,9,23,28
and 3 looked at >40 patients.4,5,18 One
article reviewed patients with syringomye-
lia, but it did not distinguish which patients
had CM-1 and thus was excluded.30
The 3 articles with >40 patients were by
Killeen et al.,5 Chavez et al.,4 and
Hayhurst et al.18 See Table 4 for their
presenting features. Killeen et al.5
reported 47 adult patients who were not
recommended for surgery; the 21
pediatric patients in the study were
excluded. Chavez et al.4 identified 68
conservatively treated patients and 109
surgically treated patients; 29% of the
patients were <18 years old, but it was
not possible to separate these patients
from the adult patients. Hayhurst et al.18
looked at 96 patients with Chiari
malformations; 83 had CM-1 and 13 had
Chiari malformation type 2. Patients with
Chiari malformation type 2 are only a
small subset in the study by Hayhurst
et al.; however, they were not
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LITERATURE REVIEW
CHIARI MALFORMATION TYPE 1
differentiated and thus limit interpretation
of the data.
There were also case reports of patients
presenting with rare symptoms. Although
sleep apnea is seen in a small proportion
of patients, Mangubat et al.19 recorded a
case of orthopnea secondary to CM-1.
Klein et al.20 reported a case of vertigo
and vestibular difficulties, and Furuya
et al.28 reported 6 cases of vertigo.
Patients can also present with visual
problems. Bindal et al.3 reported 27
patients and found that 7 had
nystagmus. Zainon and Mohamad21
reported 1 patient who had diplopia,
vocal cord prolapse, and giddiness. Decq
et al.27 reported 2 cases in which visual
problems were presenting features. One
patient had diplopia and on examination
had papilledema. The second patient had
a 1-hour episode of blindness that
resolved spontaneously.
Common Indications for Surgical
Intervention
Many articles were identified in the sys-
tematic review that specified presenting
symptoms of CM-1, but it was difficult to
ascertain the exact symptoms that precip-
itated the decision to operate. However, 5
articles did discuss indications for surgery
in various formats. Three groups of in-
vestigators mentioned the indications that
were used in their study or discussed the
indications they consider generally
accepted.4,5,12 Three groups assessed the
usefulness of various factors in indicating
surgery.3,4,30 Table 5 provides a summary
of the criteria used for surgical
intervention.
There was no consensus on the exact
indications for surgery in CM-1. According
to Chavez et al.,4 common indications
include “(1) cough-associated headaches
that impact quality of life, (2) large or
enlarging syrinx, and (3) objective
abnormal neurologic findings or myelop-
athy. CSF [cerebrospinal fluid] flow
abnormalities, tonsillar descent, or cervi-
comedullary crowding serve as adjunct
criteria.”
Ramón et al.12 stated that it is widely
agreed that surgery is not necessary in
asymptomatic patients, but in patients
with progressive posterior fossa or spinal
cord signs, hydrocephalus or
syringomyelia surgery is recommended.
However, several case reports identified
 LITERATURE REVIEW
BENJAMIN LANGRIDGE ET AL. CHIARI MALFORMATION TYPE 1

patients with syringomyelia who did not

undergo surgery.6 It is known that
syringomyelia can be found with a
significant prevalence in the general
population, and these cases rarely require
surgery.15 Ramón et al.12 also stated that
in patients presenting only with cough
headaches, surgery was performed in 25%
of cases. This demonstrates a degree of
variability in the indications for surgery.
This uncertainty has led to several
studies to try to identify the best in-
dications for surgery and to produce
evidence-based guidelines on when it is
best to operate. In a retrospective uncon-
trolled series, Chavez et al.4 compared
symptom improvement in patients who
either did or did not have surgery.
Surgery seemed to provide the greatest
benefit in cases of headache, with 95%
of patients with cough headache
improving after surgery but only 40%
improving with conservative treatment (P
Figure 1. Preferred Reporting Items for Systematic Reviews and < 0.05). Regarding other headache
Meta-Analyses literature search flow diagram.
types, 93% improved with surgery, and
61.5% improved with conservative

Table 2. Studies Included in Qualitative Synthesis

Number of Natural History Natural History Coexistent
Author, Year Article Type Patients (Asymptomatic) (Symptomatic) Syringomyelia

Bindal et al., 19953 Retrospective cohort study 27 þþ

Chavez et al., 20144 Cohort study 177 þþþ þ
Kalb et al., 2012 14
Retrospective cohort study 104 þ
Killeen et al., 20155 Retrospective cohort study 68 þþþ
Klekamp et al., 20016 Case report and review 1 þ
Leu, 2015 7
Review N/A þ
Massimi et al., 20118 Case series 3 þ
McDonnell et al., 20009 Cross-sectional study 76 þþ
Miele et al., 2012 10
Case report 1 þ
Morris et al., 200911 Meta-analysis N/A þ
Nishizawa et al., 200115 Case-control study 9 þþ þþ
Ramón et al., 201112 Review N/A þ
Santoro et al., 1993 16
Case series 2 þ
Schneider et al., 2013 17
Case report 1 þ
Schuster et al., 201313 Systematic review N/A þ

þ, þþ, and þþþ indicate the relative utility of each article in informing the respective results subsections: “Natural History of Asymptomatic Chiari Malformation 1,” “Natural History of
Symptomatic Chiari Malformation 1,” and “Coexistence of Syringomyelia with Chiari Malformation 1.”
N/A, not applicable.

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 LITERATURE REVIEW
BENJAMIN LANGRIDGE ET AL. CHIARI MALFORMATION TYPE 1

conservative management on the basis of

Table 3. Definition of Chiari Malformation Type 1 Used mild symptoms either improved or
Reference CM-1 Definition Used remained unchanged over time (average
follow-up 4.9 years  2.9), with 25.5%
Bindal et al.3 Herniation of the cerebellar tonsils below the plane of the foramen magnum reporting worsening of symptoms and 10.6%
Chavez et al. 4
Herniation of the cerebellar tonsils through the foramen magnum >5 mm reporting both improvement and worsening
of certain symptoms. Killeen et al. also re-
Kalb et al.14 Herniation of the cerebellar tonsils through the foramen magnum >5 mm
ported specifically on cough headache in
Killeen et al. 5
Herniation of the cerebellar tonsils through the foramen magnum >5 mm adults, a common indication for surgery, and
Klekamp et al. 6
Herniation of the cerebellar tonsils through the foramen magnum >5 mm found that 37% reported an improvement,
51.9% reported no change, and 11.1% re-
Leu7 Herniation of the cerebellar tonsils through the foramen magnum >5 mm
ported worsening of headaches; 28% of
8
Massimi et al. Not defined migrainous headache symptoms were also
McDonnell et al.9 Not defined reported to have improved, but little other
information was provided on migraine or
Miele et al.10
Herniation of the cerebellar tonsils through the foramen magnum >5 mm, posterior
fossa crowding, and restricted cerebrospinal fluid flow through the foramen
other symptoms in the adult cohort.
magnum Similarly, Chavez et al.4 found that
47.1% of adult patients improved (mean
Morris et al.11 Not defined
follow-up of 15.2 months, SD 23.5
Nishizawa et al.15 Not defined months), with the likelihood of improve-
Ramón et al. 12
Displacement of the cerebellar tonsils at least 3e5 mm below the foramen ment being affected by the presenting
magnum; the authors state that the criteria for Chiari 1 malformation should not be symptoms of the patient. This allowed the
considered absolute stratification of patients by their likelihood
Santoro et al.16 Not defined
of improvement with conservative man-
agement. Of patients presenting with
Schneider et al. 17
Herniation of the cerebellar tonsils through the foramen magnum >5 mm nausea, 88.9% improved at follow-up, and
13
Schuster et al. Tonsillar ectopia located below the foramen magnum the remaining 11.1% remained unchanged;
61.5% of migrainous headaches improved,
and 31.3% were unchanged. Although
treatment (P ¼ 0.09). Killeen et al.5 found syringomyelia stabilized or improved only other symptoms improved less frequently,
that the presence of cough headaches slightly. Bindal et al.3 suggested that paresthesia and ataxia either improved or
was a potentially negative indicator for symptoms of brainstem compression remained the same in 75.4% and 82.1% of
improvement. should be a stronger indication for patients, respectively. Although symptoms
Symptoms of ataxia improved in 21% of surgery compared with symptoms of of nausea improved less frequently with
conservatively treated patients but 87.5% syringomyelia. Ucar et al.30 suggested surgical management (85.7%) than with
of surgically treated patients (P < 0.05). using a novel imaging technique called conservative management, most symp-
This would seem to suggest that ataxia is a “Sampling Perfection with Application toms improved more frequently with sur-
strong indication for surgery. However, of optimized Contrast using different flip gical intervention, including cough
the 36 patients in the study with ataxia, angle Evolutions” to image tonsillar headaches (94.6%), migraine headaches
only 8 underwent surgery, suggesting that motion and use this information to (92.9%), and ataxia (87.5%).
ataxia is not commonly used as a strong determine suitability for surgery. Beyond these 2 studies, there are addi-
indication for surgery. Paresthesia was the tional infrequent case reports of sponta-
only other symptom that showed a differ- Natural History of Symptomatic CM-1 neous resolution of malformations,
ence in improvement between the surgical The natural history of symptomatic CM-1 including associated syringomyelia.6,10
and conservative groups; 77% of patients in adults is poorly documented, with Although many patients did well with
improved after surgery, whereas only 42% most studies investigating pediatric cases conservative management, some patients
improved with conservative management.5 or outcomes after surgery. Our literature reported deterioration of symptoms. Cha-
However, this was not a randomized review identified 7 articles4-9,16 relevant to vez et al.4 reported that 11.8% of patients
study, so it is difficult to say if this question, but most available evidence with conservative management developed
paresthesia is independently associated originates from the publications by Killeen new symptoms over the follow-up period,
with improvement after surgery. et al.5 and Chavez et al.4 These articles such as diffuse headaches, numbness, and
Bindal et al.3 produced a classification suggested that many patients selected for dysphagia. Killeen et al.5 reported that
system to predict outcomes and help conservative management of mild 25.5% of conservatively managed adult
determine the appropriate surgical symptoms either improve or do not patients reported worsening of symptoms.
management. Using 21 cases, they found deteriorate after long-term follow-up, There are some reports of severe and life-
that symptoms from brainstem with serious events being infrequent. threatening presentations in patients
compression were significantly improved Killeen et al.5 found that most (63.8%) found to have CM-1, such as acute respira-
after surgery, whereas symptoms from adult patients who were selected for tory failure8 and sleep-related breathing

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BENJAMIN LANGRIDGE ET AL.
Table 4. Incidence of Symptoms in 3 Key Articles
Killeen et al.5
Symptom (n [ 47)
Cough headache only 9 (19%)
Migraine headache only 14 (30%)
Both cough and migraine 18 (38%)
headache
Headache of any type 41 (87%)
Paresthesia 31 (70%)
Cerebellar symptoms 26 (55%)
Dysphagia 10 (21%)
Dysphagia or apnea
Nausea 7 (15%)
Drop attacks
Cranial nerve dysfunction
disorders.7 These severe presentations
appear to be rare, however, and are not
described in larger studies.
Specific subpopulations of patients may
have more adverse outcomes than ex-
pected. McDonnell et al.9 looked at CM-1 in
patients with spina bifida and reported that
23.7% of these patients reported new
symptoms over the previous 12 months.
Some evidence suggests that pediatric
and adult patients with CM-1 have
different outcomes. Compared with adult
patients, pediatric patients tended to be
less symptomatic at diagnosis and to have
a higher likelihood of improvement at
follow-up. In the study by Killeen et al.,5
pediatric patients reported improved or
stable symptoms in 95.2% of cases
compared with 63.8% of adult patients
(P < 0.05). Killeen et al. demonstrated
not only that this difference existed
between pediatric and adult groups but
also that it was age dependent, with
improvement of symptoms being
predicted by younger age at presentation.
The odds of improvement of symptoms
were 0.48 times lower for every 10-year
increase in age (95% confidence interval
0.28e0.815, P < 0.05). This has significant
implications for research that combines
both adult and pediatric patient groups
without separating the 2 populations.
Natural History of Asymptomatic CM-1
Despite a prevalence of 0.24%, the long-
term prognosis of asymptomatic CM-1
WORLD NEUROSURGERY 104: 213-219, AUGUST 2017
Chavez et al.4 Hayhurst et al.18
(n [ 177) (n [ 96) Combined
67 76/224 (34%)
34 48/224 (21%)
31 49/224 (22%)
132 63 (66%) 236/320 (74%)
66 14 (15%) 111/320 (35%)
36 13 (14%) 75/320 (23%)
10/47 (21%)
16 16/177 (9%)
23 30/224 (13%)
4 7 11/273 (4%)
14 (15%) 14/96 (15%)
has been poorly studied.11 In the
literature search, we initially identified 1
article, by Bindal et al.,3 which followed
27 patients over several years with CM-1.
Of these, there were only 5 patients who
were asymptomatic and who did not have
surgery. After a mean 2-year follow-up, all
5 patients remained asymptomatic.
We identified 2 additional articles that
reported asymptomatic patients with CM-1
over a period of several years. Nishizawa
et al.15 identified 9patients with
asymptomatic CM-1 and syringomyelia
who were monitored for >10 years. All
patients had presented incidentally during
brain “check-ups,” head injuries, tension
headaches, or paranasal sinusitis. In the
follow-up period of 11.2 years  0.7, 8
patients remained asymptomatic and
showed no neurologic change. One pa-
tient noticed clumsiness of fingers 7 years
after diagnosis and underwent surgery
with a successful outcome. Additionally,
there were no significant differences in
magnetic resonance imaging scan find-
ings over the 10-year period, including the
patient who had surgery up until the time
of the operation.
Santoro et al.16 also reported an
asymptomatic case of CM-1 and syringo-
myelia incidentally diagnosed in a preg-
nant woman. When presenting to the
hospital with pregnancy for the second
time 5 years later, the same patient un-
derwent magnetic resonance imaging,
which showed the presence of CM-1
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LITERATURE REVIEW
CHIARI MALFORMATION TYPE 1
without syringomyelia. The patient
remained asymptomatic throughout this
time.
Coexistence of Syringomyelia with CM-1
Syringomyelia commonly coexists with
CM-1, being present in approximately 50%
of cases.13 The presence of a syrinx has
been repeatedly advocated as an indicator
for surgical intervention,12,13 and Chavez
et al.4 found that patients treated
surgically were more likely to have a
coexistent syrinx (P < 0.0001). Limited
evidence substantiates this position,
particularly in light of the fact that
patients with coexistent syringomyelia
are rarely studied separately from
patients with CM-1 alone. However, the
presence of a coexistent syrinx has been
reported as a negative predictor of
improvement after surgical
decompression.13,14
Some evidence suggests that conserva-
tive management is appropriate in pa-
tients with coexistent syringomyelia who
are asymptomatic at diagnosis. Nishizawa
et al.15 found that most of these patients
showed no neurologic change over long-
term follow-up (mean 11.2 years), sug-
gesting that if patients are asymptomatic,
conservative management is appropriate.
However, only 9 patients were in this
group in their study; 1 patient exhibited
neurologic changes after 7 years of follow-
up and underwent surgical intervention.
As sudden deterioration has been
observed in patients with previously
asymptomatic syringomyelia,17 careful
long-term monitoring would be prudent.
DISCUSSION
There were no randomized controlled tri-
als of surgery versus conservative man-
agement of CM-1. The best published
evidence was level 2a and 2b cohort or
case comparison series. Therefore, it is
possible to suggest grade B recommen-
dations for the management of symp-
tomatic adult CM-1. Patients presenting
with significant headaches, ataxia, or
paresthesia are more likely to undergo
surgical treatment of CM-1, although the
decision to operate is a subjective one,
depending on the balance of severity of
symptoms, impact on quality of life, and
the potential for surgical complications. In
the absence of symptoms, there is little
217
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BENJAMIN LANGRIDGE ET AL. CHIARI MALFORMATION TYPE 1

nonspecific symptoms are less likely to

Table 5. Criteria Used for Surgical Intervention improve. Potential risks of surgery include
Reference Criteria Used for Surgical Intervention meningitis (4.8%), wound infection
(3.2%), stroke (0.7%),31 hydrocephalus
Bindal et al.3 Any patient with symptoms of brainstem compression or syringomyelia (3.0%), and cerebrospinal fluid fistula
underwent surgery. Patients with no symptoms of brainstem compression or (5.9%).32 These risks should be weighed
syringomyelia did not undergo surgery. against the potential benefits of surgery,
Chavez et al.4 Inclusion criteria were the presence of cough-associated headaches that impact which vary with operative technique33
quality of life, large or enlarging syrinx, and objective abnormal neurological and the severity of symptoms, accepting
findings or myelopathy; cerebrospinal fluid flow abnormalities, tonsillar descent, that most patients follow an indolent
or cervicomedullary crowding served as adjunct criteria. course and some patients with mild
Kalb et al.14 Symptomatic patients, with or without syringomyelia, were generally considered symptoms may improve.
for surgery.
Killeen et al.5 Patients with radiographic evidence of CM-1 and clinical symptoms of CM-1 Limitations of the Review
were generally recommended for surgery; however, if there was a lack of There are very few studies with large
clinical objective findings or only mildly symptomatic tussive headaches, surgery numbers of patients, and all are retro-
was generally not recommended. spective nonrandomized studies,
Klekamp et al.6 Not defined providing level 2 evidence at best. There-
7
fore, the strength of recommendation is
Leu Not defined
limited to grade B for the management of
Massimi et al.8 Not defined symptomatic adult patients with CM-1. For
McDonnell et al. 9
Not defined asymptomatic adult patients with CM-1,
although the available evidence portrays
Miele et al.10 Not defined
a fairly benign course over long-term
11
Morris et al. Not defined follow-up, the number of patients in
Nishizawa et al. 15
Not defined these studies limits recommendations to
grade C. There is also likely to be selection
Ramón et al.12 Decompressive surgery is not recommended in asymptomatic patients; in
bias when retrospectively comparing sur-
patients with progressive posterior fossa or spinal cord symptoms/signs,
hydrocephalus or syringomyelia surgery is recommended. gical and conservative cohorts in the
literature, as surgical patients may have
Santoro et al.16 Not defined more severe symptoms.
Schneider et al.17 Not defined
Schuster et al. 13
Not defined Recommendations
The key limiting factor for further
CM-1, Chiari malformation type 1. advancement of management recommen-
dations in asymptomatic and symptomatic
adult patients with CM-1 is the size and
evidence to suggest that surgery should be very few asymptomatic patients require retrospective nature of much of the
performed on the basis of radiologic surgery >10 years after diagnosis. The currently available evidence. The develop-
findings alone. presence of a large syrinx can sometimes ment of a registry for such patients,
If patients are mildly symptomatic, CM- be alarming at first presentation, and allowing aggregation and long-term
1 is not necessarily a progressive condi- many surgeons may advocate surgery in follow-up, would be of significant
tion, and mild symptoms may remain case of progression. However, patients benefit. We also recommend that a uni-
static or improve with time. Therefore, it commonly present with very few or mild form definition of CM-1 be adopted by
is reasonable to observe patients who symptoms despite a large syrinx, and future studies to aid review and meta-
present with mild symptoms, although again it is reasonable to observe such pa- analysis (Table 3).
there are rare case reports of sudden tients and offer surgery if the severity of
deterioration. It is impossible to deter- symptoms warrants surgical intervention
mine risk factors for sudden deterioration in the future or there is significant radio- CONCLUSIONS
from the evidence available, and this logic progression of the syrinx. However, The natural history of symptomatic and
should be placed into context when dis- owing to the low number of asymptomatic asymptomatic CM-1 in adults is relatively
cussing the management of CM-1 with patients in these studies, the recommen- benign and nonprogressive, and the deci-
patients in the clinic. Surgery should be dations for management of asymptomatic sion to perform a foramen magnum
offered if symptoms subsequently patients is weaker (grade C). decompression operation should be based
deteriorate. After surgery, symptoms of headache on the severity and duration of a patient’s
Most patients who present with and ataxia are most likely to improve, but symptoms at the time of presentation. It is
asymptomatic CM-1 are likely to remain other symptoms may not even within the reasonable to observe a patient with mild
asymptomatic. Larger studies show that same patient. Symptoms of nausea and or asymptomatic symptoms, even in the

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BENJAMIN LANGRIDGE ET AL.
presence of significant tonsillar descent or
syringomyelia. Although class 1 evidence
is lacking, there is sufficient evidence to
suggest that randomly assigning patients
into a surgical arm of a clinical trial would
not be reasonable if they were mildly
symptomatic or asymptomatic, and pa-
tients should be offered surgery depend-
ing on the severity of presenting
symptoms. Patients with CM-1 who are
either asymptomatic or mildly symptom-
atic at diagnosis may improve or remain
symptomatically stable with conservative
management. Although serious deteriora-
tion has been reported, this is rare and
must be balanced against the known risks
of surgical intervention.
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Conflict of interest statement: The authors declare that the
article content was composed in the absence of any
commercial or financial relationships that could be construed
as a potential conflict of interest.
Received 15 January 2017; accepted 12 April 2017
Citation: World Neurosurg. (2017) 104:213-219.
http://dx.doi.org/10.1016/j.wneu.2017.04.082
Journal homepage: www.WORLDNEUROSURGERY.org
Available online: www.sciencedirect.com
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