CLINICAL STUDIES

OUTCOME OF CHIARI-ASSOCIATED SYRINGOMYELIA

AFTER HINDBRAIN DECOMPRESSION IN CHILDREN:
ANALYSIS OF 49 CONSECUTIVE CASES
Frank J. Attenello, M.S.
Department of Neurosurgery,
The Johns Hopkins School of Medicine,
Baltimore, Maryland

Matthew J. McGirt, M.D.

Department of Neurosurgery,
The Johns Hopkins School of Medicine,
Baltimore, Maryland

Muraya Gathinji, M.S.

Department of Neurosurgery,
The Johns Hopkins School of Medicine,
Baltimore, Maryland

Ghazala Datoo, B.S.

Department of Neurosurgery,
The Johns Hopkins School of Medicine,
Baltimore, Maryland

April Atiba, B.S.

Department of Neurosurgery,
The Johns Hopkins School of Medicine,
Baltimore, Maryland

Jon Weingart, M.D.

Department of Neurosurgery,
The Johns Hopkins School of Medicine,
Baltimore, Maryland

Benjamin Carson, M.D.

Department of Neurosurgery,
The Johns Hopkins School of Medicine,
Baltimore, Maryland

OBJECTIVE: Chiari I malformation is complicated by syringomyelia in many cases.

Hindbrain decompression remains first-line surgical treatment; however, the incidence,
time course, and predictors of syrinx resolution remain unclear. We set out to determine predictors of syrinx improvement after hindbrain decompression for Chiari Iassociated syringomyelia.
METHODS: Forty-nine consecutive pediatric patients undergoing posterior fossa decompression for Chiari I-associated syringomyelia were followed with serial magnetic resonance imaging evaluations postoperatively. Clinical, radiological, and operative variables were assessed as predictors of syrinx improvement as a function of time using
Kaplan-Meier plots and log-rank analysis.
RESULTS: Mean patient age was 11 5 years. Syringomyelia was symptomatic in 39
(80%) and asymptomatic in 10 (20%) cases. Twenty-one (54%) patients experienced
symptom resolution (median, 4 mo postoperatively). Twenty-seven (55%) patients
experienced radiographic improvement in syringomyelia (median, 14 mo postoperatively). After hindbrain decompression, motor symptoms were associated with a 2.35
increased hazard ratio for symptom improvement (P 0.031) versus all other symptoms. Among patients with sensory deficits, dysesthesia was associated with a 3.12
increased hazard ratio for symptom improvement (P 0.032) versus symptoms of
paresthesia or anesthesia.
CONCLUSION: In our experience, just more than one-half of patients with Chiariassociated syringomyelia demonstrated clinical and radiographic improvement after
hindbrain decompression. Median time to radiographic improvement lagged behind
clinical improvement by 10 months. Motor symptoms were more likely to improve with
hindbrain decompression. Paresthesia or anesthesia symptoms were less likely to improve
with hindbrain decompression. These findings may help guide surgical decision making and aid in patient education.
KEY WORDS: Chiari malformation, Outcome, Risk factors, Syringomyelia
Neurosurgery 62:13071313, 2008

DOI: 10.1227/01.NEU.0000316848.55059.51

www.neurosurgery-online.com

George I. Jallo, M.D.

Department of Neurosurgery,
The Johns Hopkins School of Medicine,
Baltimore, Maryland
Reprint requests:
Matthew J. McGirt, M.D.,
3553 Newland Road,
Baltimore, MD 21218.
Email: mmcgirt1@jhmi.edu
Received, August 11, 2007.
Accepted, March 25, 2008.

NEUROSURGERY

hiari I malformation (CM1) is classically

defined as a caudal displacement of 5
mm or more of the cerebellar tonsils past
the foramen magnum and into the spinal canal
(37). It most commonly presents in young
adults but is also common in the pediatric population (17, 37). Presentation ranges from
asymptomatic herniation or light headache to
symptoms of acute brainstem or spinal cord
compression (19, 35). Multiple comorbidities
occur with CM1, including syringomyelia, scoliosis, and hydrocephalus.

Syringomyelia is associated with many posterior fossa pathologies and has been reported
in as many as 35 to 75% of patients with CM1
(5, 12, 25, 26). Current theories regarding the
pathophysiology of syringomyelia include
Gardeners (water-hammer) theory and the
Williams craniospinal pressure dissociation
hypothesis (4). The fact that CM1 and syringomyelia co-occur so frequently suggests a
common cause (24). Several series of Chiariassociated syringomyelia have shown a significant association between the presence of these

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ATTENELLO ET AL

conditions and altered cerebrospinal fluid (CSF) flow (2, 13,

26). Common symptoms attributed to syringomyelia include
paresthesia, anesthesia, dysesthesia, weakness, gait and balance instability, bladder incontinence, and scoliosis (13).
Although debated, first-line surgical treatment for Chiari-associated syringomyelia remains suboccipital decompression. The
procedure has a variable success rate, with 44 to 100% of
syringomyelia resolving after surgery (10, 14, 16, 19, 21, 22, 29,
31, 32). Hence, predictors of syrinx response are needed to better select patient subgroups most likely to benefit from hindbrain compression. We set out to determine the incidence of
syrinx improvement both radiographically and symptomatically as well as the time course of this improvement. In addition, we sought to determine whether certain patient subgroups were more or less likely to respond to decompression
and to identify factors that affect the rate at which patients
achieve resolution of symptoms.

PATIENTS AND METHODS

We retrospectively reviewed 49 consecutive pediatric patients undergoing posterior fossa decompression for CM1-associated syringomyelia
at The Johns Hopkins Hospital between 1995 and 2005. Presenting
symptoms, neurological deficits, demographics, comorbidities, preand postoperative radiological studies, operative records, operative
technique, and follow-up clinical records were reviewed in all cases. An
electronic database was recorded by cataloging patient demographics,
presenting symptoms, and degree of tonsil herniation. Additionally,
the presence of scoliosis, hydrocephalus, basilar invagination, fused
cervical vertebrae, platybasia, atlanto-occipital assimilation, or cervicomedullary kinking on magnetic resonance imaging (MRI) were also
identified and recorded. Patient signs and symptoms were recorded,
including syrinx-specific symptoms of dysesthesia, paresthesia, gait
abnormalities, incontinence, weakness, and scoliosis. Appropriate otolaryngology, pulmonary, and ophthalmological consultations were
obtained to determine a central cause of apnea, cranial nerve, and ophthalmological findings. Headache was regarded as a symptom of
Chiari-associated syringomyelia based strictly on tussive nature and
reproducibility in the clinic. Perioperative complications, including
wound breakdown, pseudomeningocele, and aseptic meningitis were
recorded as well.
All patients in the reviewed study underwent preoperative MRI as
well as 3-month postoperative MRI. Additional follow-up MRI varied
but was usually repeated 12 to 18 months after surgery. Postoperative
MRI studies were retrospectively reviewed and compared with preoperative studies. Syringomyelia was classified as resolved if there was no
evidence of residual syrinx, decreased if the syrinx diameter was
decreased by at least 20%, and no change if the syrinx diameter was
decreased by less than 20% compared with the preoperative diameter.
Postoperative records were specifically evaluated for persistence of
syrinx-specific symptoms, including dysesthesia, paresthesia, gait
abnormalities, incontinence, and weakness. Resolution of hindbrain,
cranial nerve, or headache symptoms alone was not regarded as a resolution of syrinx-specific symptoms.

Operative Technique
All patients in this series were offered surgical decompression if
they presented with symptoms consistent with CM1 (tussive
headache, cervical pain, central apnea, dysphagia, aspiration, vertigo,

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vocal cord paralysis, motor/sensory deficits, nystagmus, ataxia, and

incoordination) and demonstrated cerebellar tonsil herniation below
the foramen magnum in addition to syringomyelia. The surgical technique for a craniectomy and duraplasty was standardized for all
reviewed patients performed by three surgeons (JW, BC, GJ). All
patients underwent surgery in the prone position with the head fixed
in a Mayfield or a horseshoe headholder. A small suboccipital craniectomy and a C1 laminectomy were performed. The craniectomy usually
extended from the foramen magnum upward, including the inferior
nuchal line of the occipital bone, covering the insertion area of the rectus capitis posterior minor muscle, and the medial insertion area of the
rectus capitis posterior major muscle. This typically measured 3 cm in
width and 3 cm in height. The surgeons ensured an adequate decompression of the rim of the foramen. The tonsils were visualized using
intraoperative ultrasonography. If the tip of the tonsils could not be
sufficiently exposed, a C2 laminectomy was additionally performed.
When performing duraplasty, the dura was opened in a Y-shaped
fashion spanning the majority of the 3 3 cm craniectomy. The dura
was closed with a triangular synthetic dural graft (Dura-Guard; BioVascular, Inc., St. Paul, MN), pericranium, or Gore-Tex (WL Gore &
Associates, Inc., Newark, DE), ensuring the reconstruction of the open
CSF space at the craniospinal junction. The preoperative surgical plan
for all cases was to perform duraplasty unless evidence of adequate
bony hindbrain decompression was evident on an intraoperative ultrasound scan (Prosound SSD 1000; Aloka Inc., San Diego, CA). The intraoperative decision not to perform duraplasty was subjectively based
on interpretation of the intraoperative ultrasound scan findings. Only
patients thought to have evidence of physiological systolic tonsilar
pulsations and decompression of the subarachnoid spaces ventral and
dorsal to the tonsils after bony decompression did not undergo
duraplasty.

Statistical Analysis
Radiographic and symptomatic improvement as a function of time
after surgery was expressed using the Kaplan-Meier method (23).
The univariate association of preoperative variables with radiographic and symptomatic improvement were assessed by log-rank
analysis for stratified covariates and proportional hazards regression
(8) for continuous variables to generate P values. For variables
demonstrating a significant association with survival (P 0.05), hazard ratios were generated using proportional hazards analysis. The
sample size of this study was underpowered to support multivariate
modeling; hence, multivariate proportional hazards regression analysis (Cox model) was not performed.

RESULTS
Patient Population
Forty-nine children underwent surgery for Chiari I
associated syringomyelia over the reviewed time period. Mean
SD age at time of surgery was 11 5 years and 19 (39%)
patients were male. Forty-three (88%) cases were primary surgery, whereas six (12%) cases were revision decompressions.
Syringomyelia was symptomatic in 39 (80%) cases and asymptomatic with regard to syrinx-specific symptoms in 10 (20%).
Symptoms attributed to syringomyelia included weakness in
eight (21%), paresthesia/anesthesia in 19 (49%), dysesthesia in
11 (28%), incontinence in six (15%), gait instability in three (8%),
and scoliosis in 17 (41%) patients. Eleven (22%) patients had

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CHIARI-ASSOCIATED SYRINGOMYELIA AFTER HINDBRAIN DECOMPRESSION IN CHILDREN

brainstem or cranial nerve symptoms, and 28 (57%) had occipital headache related to posterior fossa crowding. Five (10%)
patients had shunted hydrocephalus at the time of surgery.
Tonsil herniation was between more than 5 mm below the
foramen magnum and C1 in 2 (4%) patients, between C1 and C2
in 40 (81%) patients, and below C2 in seven (15%) patients.
Syringomyelia involved the cervical spinal cord in 46 (94%)
cases, the thoracic spinal cord in 25 (51%) cases, and the conus
in seven (14%) cases. The syrinx spanned a median of 6.5 spinal
levels and involved the holocord in six (12%) cases. The operative microscope was used for duraplasty in 28 (57%) cases. C2
laminectomy was performed in nine (18%) cases and tonsil
coagulation in five (10%) cases. Duraplasty was performed in 46
(94%) patients. Duraplasty was not performed in three patients.
Aseptic meningitis was seen in two (4%) patients, wound
breakdown was seen in two (4%) patients, and pseudomeningocele was seen in one (2%) patient. However, these perioperative complications did not occur in any patients who experienced syrinx-specific symptom persistence. Furthermore,
none of these perioperative complications required reoperation for treatment. No patients showed evidence of craniovertebral bony abnormalities, including cranial settling, platybasia,
Klippel-Feil, or basilar invagination.

FIGURE 1. Kaplan-Meier plot demonstrating the proportion of patients experiencing radiographic improvement in syringomyelia (resolution or decreased in axial
diameter of at least 20%) as a function of time after
hindbrain decompression for pediatric Chiari I malformation. Median time to improvement was 14 months.
By 6 months, 25% had improvement on magnetic resonance imaging.

Patient Follow-up
Patients were followed for a mean of 41 33 months postoperatively. Only two patients were followed for less than
6 months (4 mo each). Thirty-nine (80%) patients were followed
beyond 18 months after surgery, 34 (70%) patients were followed beyond 2 years, and 24 (49%) patients were followed
beyond 3 years.

Response of Syrinx to Cervicomedullary Decompression

Twenty-seven (55%) patients experienced radiographic
improvement in syringomyelia (decreased size or resolution)
during the follow-up period (Fig. 1). Twenty-one (42%) patients
demonstrated decreased syrinx size and six (12%) demonstrated resolved syrinx. The median time to radiographic
improvement was 14 months after surgery. Twenty-one (42%)
patients demonstrated no radiographic change in their syrinx
by the last follow-up evaluation (mean standard deviation;
24 17 mo after surgery). One patient demonstrated an
increased syrinx size 5 months postoperatively, requiring reoperation.
Of the 39 patients with symptomatic syringomyelia, 21 (54%)
experienced symptomatic improvement. The median time to
symptom improvement was 4 months after surgery (Fig. 2).
Similar to the overall study population, 16 (41%) patients with
symptomatic syringomyelia demonstrated no change in their
syrinx size by the time of the last follow-up evaluation,
whereas one (3%) patient showed increased syrinx size at the
time of follow-up. A summary of this subgroup of symptomatic patients is provided in Table 1.
Among three patients who did not undergo duraplasty, two
(66%) showed no radiographic syrinx improvement. Both
required subsequent surgery (one revision hindbrain decom-

NEUROSURGERY

FIGURE 2. Kaplan-Meier plot demonstrating the proportion of patients experiencing clinical improvement
in syrinx-related symptoms as a function of time after
hindbrain decompression for pediatric Chiari I malformation. Symptoms include dysesthesia, paresthesia/
anesthesia, weakness, gait/balance problems, incontinence, and scoliosis. Median time to symptom
improvement was 4 months and 61% demonstrated
symptom improvement at 1 year postoperatively.

pression for persistent symptomatic syrinx and one for progressive syrinx-associated scoliosis).
Of 18 patients showing persistent symptoms, revision decompression was pursued in five cases (one with a syringostomy
tube), showing calcification and scarring in each case. Each of
these five patients demonstrated abnormal hindbrain CSF flow
on cine-MRI. Of these, four (80%) showed improvement after
revision decompression. In eight cases, bracing or fusion was
pursued for scoliosis progression, halting curve progression in
all eight cases. Five patients had minor symptom recurrence
and were followed conservatively. Of these, four (80%) showed
partial radiographic improvement of syrinx, whereas one
patient showed a stable syrinx but declined reoperation.

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ATTENELLO ET AL

TABLE 1. Summary of patient population and presenting signs

and symptoms among 39 patients with Chiari I malformation
and symptomatic syringomyeliaa
Age, yr

11 4

Male

14 (36%)

Pseudomeningocele

Duraplasty

36 (92%)

Wound breakdown

1 (3%)

Syrinx spanned
levels

11 5

Aseptic meningitis

2 (5%)

Dysesthesia

11 (28%)

Improved dysesthesia

8 (73%)

Parasthesia/
anesthesia

19 (49%)

Improved parasthesia/
anesthesia

8 (42%)

2 (5%)

Gait instability

3 (8%)

Improved gait instability

3 (100%)

Weakness

8 (21%)

Improved weakness

6 (75%)

Incontinence

6 (15%)

Improved incontinence

3 (50%)

Improved scoliosis

8 (47%)

Scoliosis

17 (41%)

Values are presented as mean standard deviation and number (%) symptomatic patients.

Predictors of Symptomatic Improvement

In patients with symptomatic syringomyelia, motor symptoms versus other symptoms were associated with a 2.35
increased hazard ratio for symptom improvement (hazard
ratio, 2.35; 95% confidence interval, 1.1211.49; P 0.031) (Fig.
3A). Among patients with sensory deficits, dysesthesia versus
symptoms of parasthesia or anesthesia were associated with a
3.12 increased hazard ratio for symptom improvement (hazard
ratio, 3.12; 95% confidence interval, 1.1314.35; P 0.032) (Fig.
3B). Age (P 0.63), sex (P 0.10), urinary incontinence (P
0.37), scoliosis (P 0.65), gait abnormality (P 0.40), duration
of syrinx symptoms (P 0.19), location of syrinx (cervical versus thoracic) (P 0.33), increasing spinal levels of syrinx (P
0.89), and performance of tonsilar coagulation (P 0.14) or
duraplasty (P 0.63) were not associated with symptomatic
outcome.

DISCUSSION
Suboccipital decompression with or without duraplasty is
the most frequently used surgical intervention to treat Chiariassociated syringomyelia (1, 32). Our study showed that 55% of
patients who underwent hindbrain decompression experienced
radiographic improvement by a median of 14 months after surgery. Success rates in multiple series have shown variable outcomes, with incidences of radiographic improvement ranging
from 44 to 100% (10, 14, 16, 19, 21, 22, 29, 32). However, the incidence of radiographic improvement is largely affected by the
degree of change defined as improvement, which is variable in
previous studies. Although 55% of our series experienced at
least a 20% reduction in syrinx diameter, 73% of the patients
demonstrated some degree of decreased syrinx diameter. A
meta-analysis by Galarza et al. (15) found that 76% of patients
treated with decompression with or without duraplasty
demonstrated resolution of syrinx. Depreitere et al. (9), in an
adult series of 22 patients, saw radiographic improvement in

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FIGURE 3. Kaplan-Meier plot demonstrating the proportion of patients experiencing clinical improvement
in syrinx-related symptoms as a function of time after
hindbrain decompression for pediatric Chiari I malformation. Dysesthesia versus paresthesia/anesthesia
(P 0.032, log-rank test) (A) and motor symptoms
versus all other symptoms (P 0.031, log-rank test)
(B) demonstrated greater hazard for improvement after
hindbrain decompression.

80% (16 of 20) of patients. A radiographic success rate of as

many as 94% (32 of 34) of patients with Chiari-associated
syringomyelia has been reported, although that particular
series used shunts in addition to decompressive surgery (1).
Resolution of clinical symptoms in patients with Chiariassociated syrinx ranges from to 35 to 100% (9, 14, 19, 22, 27, 29,
34). In our series, improvement in clinical symptoms of
syringomyelia was seen in 54% of patients with symptomatic
syringomyelia by a median of 4 months after surgery. This,
however, is a much lower response rate than that found in a
few previously published studies, some of which report radiographic improvement in 95% of patients (31). In our study, the
rate of response was greatest within the first 6 months with the
majority of patients showing symptom improvement by 12
months. Only a single patient with persistent symptoms 1 year
after surgery went on to experience symptom relief at a later
time. Galarza et al. (15) achieved similar results in their cohort
of 60 patients: 64% of their CM1-syrinx patients who underwent foramen magnum decompression with or without

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CHIARI-ASSOCIATED SYRINGOMYELIA AFTER HINDBRAIN DECOMPRESSION IN CHILDREN

C
FIGURE 4. Preoperative T2weighted magnetic resonance
imaging scans (MRI) of Chiariassociated syringomyelia showing
tonsilar herniation to C1 (A), cervicothoracic syrinx from C3 to
T10 (B), and right thoracic scoliosis (C).

duraplasty achieved some form of symptomatic improvement

by the 21-month follow-up period. An example of a patient
with Chiari-associated syrinx and scoliosis is shown in Figure 4.
The same patient showed adequate hindbrain decompression,
persistent syrinx, and only partially resolved symptoms by 6
months postoperatively (Fig. 5). Because symptomatic
improvement remains likely within the first year postoperatively, patients are conservatively followed for symptom
improvement from postoperative months 6 through 12, at
which point reoperation is considered. However, case reports
have shown symptom resolution occurring as late as 3 years
postoperatively (11).
Among patients showing persistent symptoms after decompression, progressive scoliosis was the most common contributing factor. All of these cases were then successfully treated with
subsequent bracing or fusion. Incidence of progressive scoliosis after hindbrain decompression ranges from 40 to 75%, consistent with our observations (30, 33, 38). Our findings highlight the need for close follow-up and potentially early bracing
of patients with syrinx-associated scoliosis. Persistent hindbrain crowding contributed to treatment failure in more than
one-fourth of patients with persistent symptoms and was a
direct result of postoperative hindbrain scarring and adhesions
(28). In all of these cases, hindbrain CSF flow remained
obstructed on cine-MRI studies. CSF flow at the craniocervical
junction is shown to be decreased in patients with Chiariassociated syringomyelia, with CSF flow pathology resolving
in parallel fashion with clinical symptoms (18, 36). Persistent

NEUROSURGERY

FIGURE 5. Postoperative T2- C

weighted MRI scans of the patient
shown in Figure 4 at 6 months
postoperatively, showing adequate
decompression of the cervicomedullary junction (A), with no
change in syrinx on sagittal (B)
and axial (C) imaging, and no
change in scoliosis curvature. In
this patient, partially resolved
symptoms continue to be conservatively monitored. The majority of our
patients show symptom resolution at 1 year postoperatively, at which
point reoperation may be considered for patients continuing to experience
syrinx-related symptoms.

CSF flow abnormalities after hindbrain decompression may

serve as an early marker for patients who may not experience
symptomatic syrinx improvement. Despite the 46% rate of
symptom persistence in our series, posterior fossa decompression remains the procedure of choice for patients with symptomatic Chiari-related syringomyelia.
Although the median time to symptom improvement was 4
months after surgery, the median time to radiographic
improvement was 14 months. Bogdanov and Mendelevich (6)
found no correlation in their series between specific syrinx size
and clinical condition. This suggests, as seen in our series, that
clinical improvement does not necessitate concurrent radiographic improvement. Lack of change in syrinx on MRI is
often not clinically significant, suggesting that postoperative
MRI surveillance may only be useful in patients failing to
symptomatically improve by 1 year postoperatively. This trend
of clinical resolution before radiographic resolution is very
commonly seen in clinical conditions such as resolving
encephalomyelitis (20) and pneumonia (7).
We found dysesthesia to be the most responsive sensory
symptom and motor dysfunction to be more responsive than
all other symptoms after hindbrain decompression. Few studies have assessed predictors of syrinx symptom resolution
after posterior fossa decompression. Attal et al. (3) found the
duration of sensory deficits to correlate with resolution of sensory symptoms after decompression. Our series showed no
significant correlation between the length of symptoms and
symptom resolution, even when considering subgroups with
sensory symptoms alone or without scoliosis. In a study considering multiple radiological variables, including maximal

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ATTENELLO ET AL

tonsilar descent, odontoid retroflexion, and maximal axial

width of the fourth ventricle in their series, Tubbs et al. (35)
found no preoperative factors associated with subsequent
radiological treatment failure.
Although we have drawn several significant correlations in
this series, our conclusions are nevertheless limited by the retrospective design of the study. The data analysis is subject to
treatment bias, and all potential confounding factors cannot be
accounted for. Furthermore, these risk factors of outcome cannot be assumed as causal but merely associated observations.
Nevertheless, we demonstrate that Chiari-associated syringomyelia can be successfully treated by hindbrain decompression in the majority of patients and should remain the first line
of treatment in these patients.

CONCLUSION
In our experience, just more than one-half of patients with
Chiari-associated syringomyelia will demonstrate clinical and
radiographic improvement after hindbrain decompression. The
median time to radiographic improvement lagged behind clinical improvement by 10 months. Motor symptoms were more
likely to improve with hindbrain decompression. Paresthesia or
anesthesia symptoms were less likely to improve with hindbrain decompression. These findings may help guide surgical
decision making and aid in patient education.

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www.neurosurgery-online.com

CHIARI-ASSOCIATED SYRINGOMYELIA AFTER HINDBRAIN DECOMPRESSION IN CHILDREN

Acknowledgments
We thank Benjamin Brooke, M.D., Ph.D., for his biostatistical contributions to
this study. Matthew McGirt, M.D., is supported by the American Syringomyelia
Alliance Project (ASAP) Monkton Institute CNS Fellowship.

This article joins an increasing number of reports on the size of the

syrinx after posterior fossa decompression with the Chiari I malformation.
Arnold H. Menezes
Iowa City, Iowa

COMMENTS

he authors have reviewed the experience of three faculty neurosurgeons with posterior fossa decompression for the hindbrain herniation syndrome (Chiari I malformation). Of the 49 patients, 39 were felt
to be symptomatic from the syringomyelia, whereas in 10, the syrinx
was felt to be asymptomatic. The operative procedure was standardized with posterior fossa decompression and a C1 laminectomy. If the
dura was opened, a dural graft was made with synthetic dura.
After symptomatic patients, syrinx improvement occurred in 54%
(21 of 29), and one patient showed an increase in the size of the syrinx
during the follow-up period. A revision decompression was pursued in
five patients, and the authors report calcification and scarring in each
individual. Only six patients had resolution of the syrinx. Overall, 27
children experienced radiographic improvement in the syrinx size at
the time of the 1-year follow-up evaluation. Unfortunately, no mention
is made regarding the amount of arachnoidal adhesions or the presence
of veil over the fourth ventricle exit. This would be critical especially in patients in whom the syrinx failed to change.

n this study, the authors present six patients with sphenoidal

encephaloceles, five of whom were treated with an extradural
approach, three transorally, and two transnasally. They describe their
operative approach and compare their technique with the intradural
and endoscopic approaches based on a literature review. The authors
make some important points. First, the natural history of these lesions
is poorly defined, although the few patients with recognized sphenoidal encephaloceles managed expectantly deteriorated over time.
Second, the encephalocele sac often contains critical nervous tissue and
cannot be sacrificed. The tissue contents are best defined by magnetic
resonance imaging. Third, computed tomographic scanning with threedimensional imaging can be helpful in defining the bony defect. Lastly,
many of these patients have clinically significant defects in hypothalamic-pituitary axis function. Patients should have an endocrinological
evaluation preoperatively.
Corey Raffel
Rochester, Minnesota

Transgenic method for combinatorial expression of fluorescent proteins. Magnified view of fluorescent protein expression in mouse hippocampus. From, Livet J, Weissman TA, Kang H, Draft RW, Lu J, Bennis RA, Sanes JR, Lichtman JW: Transgenic strategies for combinatorial
expression of fluorescent proteins in the nervous system. Nature 450:5662, 2007. See Elder, pp 13581359.

NEUROSURGERY

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