TECHNIQUE APPLICATION

TAILORED OPERATIVE TECHNIQUE FOR CHIARI TYPE I

MALFORMATION USING INTRAOPERATIVE COLOR
DOPPLER ULTRASONOGRAPHY
Thomas H. Milhorat, M.D.
Department of Neurosurgery, The
Chiari Institute, North Shore
University Hospital, Long Island
Jewish Health System,
Manhasset, New York
Paolo A. Bolognese, M.D.
Department of Neurosurgery, The
Chiari Institute, North Shore
University Hospital, Long Island
Jewish Health System,
Manhasset, New York
Reprint Requests:
Thomas H. Milhorat, M.D.,
Department of Neurosurgery, The
Chiari Institute, North Shore
University Hospital, Long Island
Jewish Health System, 300
Community Drive, Manhasset, NY
11030.
Email: Milhorat@nshs.edu
Received, December 18, 2002.
Accepted, June 4, 2003.
OBJECTIVE: We describe an operative technique for Chiari Type I malformation that
uses color Doppler ultrasonography as a guide for performing patient-specific posterior
fossa decompressions. The technique has been used since 1999 in more than 300
operations.
METHODS: On the basis of real-time anatomic and physiological measurements, the
following goals of surgery were monitored: 1) adequate decompression of the cervi-
comedullary junction; 2) creation of a retrocerebellar space of 8 to 10 cm3 volume;
and 3) establishment of optimal cerebrospinal fluid flow between the cranial and
spinal compartments.
RESULTS: The size of the craniectomy was tailored to conform to the area of cerebellar
impaction as demarcated by compressed subarachnoid spaces. A laminectomy was
not performed unless the cerebellar tonsils were herniated below C1. Before opening
the dura, color Doppler ultrasonography imaging was invaluable in planning operative
strategies. A simple duraplasty without additional steps was found to be appropriate
treatment in occasional patients with minimal tonsillar herniation (5–8 mm). In all
other cases, it was necessary to perform an internal decompression that included lysis
of the arachnoid and shrinkage of the cerebellar tonsils to achieve the goals of surgery.
Optimal cerebrospinal fluid flow through the foramen magnum in anesthetized, prone
patients was found to have the following characteristics: a peak velocity of 3 to 5 cm/s,
bidirectional movement, and a waveform exhibiting vascular and respiratory varia-
tions. The attainment of surgical goals was confirmed in most patients by postoperative
neuroimaging.
CONCLUSION: Color Doppler ultrasonography imaging is an important technological
advance that permits the neurosurgeon to tailor the steps of Chiari surgery according
to patient-specific variables. The success of this technique depends on the mastery of
a new and sophisticated monitoring modality.
KEY WORDS: Cerebrospinal fluid flow, Chiari Type I malformation, Color Doppler imaging, Intraoperative
ultrasound imaging, Posterior fossa decompression

Neurosurgery 53:899-906, 2003 DOI: 10.1227/01.NEU.0000083591.22113.CB www.neurosurgery-online.com

T
he surgical treatment of Chiari Type I
malformation (CMI) has not been stan-
dardized. Of the available operative
techniques, the most widely performed is a
posterior fossa decompression consisting of
suboccipital craniectomy, C1 laminectomy,
and duraplasty (1, 4, 9, 15). Controversy exists
concerning the extent of the bony decompres-
sion and the need for additional steps such as
shrinkage or resection of the cerebellar tonsils.
Although experienced neurosurgeons tend to
use a fixed operative technique, this approach
has limitations, given the great variability of
patient-specific findings.
Since 1999, we have used intraoperative
color Doppler ultrasonography (CDU) as a
guide to posterior fossa decompression in pa-
tients with CMI. The technology can be
adapted to measure cerebrospinal fluid (CSF)
flow and provides real-time information re-
garding neural displacements, vascular anat-
omy, and CSF circulation at the cervicomed-

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MILHORAT AND BOLOGNESE
ullary junction. Distinct advantages of CDU imaging include
complication avoidance and the ability to tailor operative
steps according to patient-specific variables. The methodology
of CDU and the details of the tailored operative technique are
presented.
METHODOLOGY OF CDU IMAGING
Historical Background
Modern techniques of intraoperative ultrasonography are
based on B-scan real-time imaging. The basic methodology
provides real-time bidimensional anatomic images and has
been used in neurosurgery since the early 1980s for the intra-
operative localization of a wide variety of intracerebral and
intramedullary lesions (3). First-generation ultrasound ma-
chines were easy to use and required limited training for
competence in data acquisition. With advances in machine
design, including the introduction of electronic probes, it was
possible to complement anatomic imaging with pulse-wave
Doppler and color Doppler coding. Specific advantages of
triplex CDU imaging include the high definition of vascular
structures and the ability to measure arterial and venous
blood flow patterns.
Beginning in 1983, one of us (PAB), working in conjunction
with Victor A. Fasano at the University of Turin, investigated
the uses of intraoperative ultrasonography in more than 1200
neurosurgical procedures. Included in this experience was the
in vivo testing of numerous probes, machines, and calibration
techniques. The results of these investigations led to the intro-
duction of CDU in aneurysm surgery (2). In recent years, we
have investigated the feasibility of using CDU for imaging
CSF flow during Chiari surgery.
Adaptations for Measuring CSF Flow
The imaging of CSF flow by means of color Doppler tech-
nology poses technical challenges. In contrast to blood, CSF
has the following characteristics: 1) a very low content of cells
and proteins, which limits the reflection of ultrasound waves;
2) a low-velocity circulation with nonhomogeneous flow
through a network of irregular spaces rather than vessels; and
3) a significant component of to-and-fro movement. To ad-
dress these technical issues, it was necessary to use high-
performance equipment that was capable of maximizing
Doppler sensitivity. Most of the results in the current study
were obtained with the Acuson Sequoia 512 system (Acuson
Corp., Mountain View, CA) equipped with standard high-
definition probes.
Because CDU machines are designed to measure blood
flow, the steps of data acquisition must be adapted according
to the unique features of CSF flow. This can be accomplished
by manually adjusting the controls for B-scan real-time pro-
cessing, pulse-wave Doppler imaging, and color Doppler im-
aging. Adjustments of machine default values include recali-
bration of the following commands: B-scan gain; pulse-wave
and color Doppler scales; pulse-wave Doppler gain; pulse-
900 | VOLUME 53 | NUMBER 4 | OCTOBER 2003
wave and color Doppler filters; time gain compensation; and
focusing of B-scan real time. Each of these commands must be
fine-tuned to achieve optimal images and Doppler informa-
tion. Experience is required to recognize artifacts and the
aliasing of color Doppler images. Acuson Corp. has reviewed
the measurements of CSF flow in this study and confirmed
their validity.
CSF Volume Measurements
The Acuson Sequoia 512 system has a preloaded software
package for calculating the volume of an area of interest,
which is obtained by measuring the area in three planes; two
scan orientations perpendicular to each other are obtained.
Cisterna magna volumes, for example, were calculated by
measuring the superoinferior (length) and anteroposterior
(depth) dimensions in the sagittal plane. The width of the
space was measured in the axial plane. Volume calculations
were made by means of the following formula:
Volume ⫽ 共D1 ⫻ D2 ⫻ D3兲␲/6
where D1, D2, and D3 are the three measurements of the
cisterna magna.
Surgical Database
Between November 1999 and November 2002, CDU was
used in 315 operations for CMI. All operations were per-
formed by the senior author (THM). There were 152 primary
operations and 163 reoperations for failed Chiari surgery.
Before surgery, patients underwent whole-neuraxis magnetic
resonance imaging (MRI), cine phase contrast MRI, and three-
dimensional computed tomography of the head and neck.
Optional studies included flexion-extension x-ray assessment
of the cervical spine, flexion-extension MRI, three-dimensional
computed tomographic angiography, and metrizamide my-
elography. MRI scans of the cervical spine and cine phase
contrast MRI were obtained 3 to 12 months after surgery.
TAILORED OPERATIVE TECHNIQUE
(see video at web site)
Surgical Indications
Radiographic Criteria
All of the patients in this series had demonstrable impaction
of the foramen magnum with MRI evidence of tonsillar her-
niation, obliteration of the cisterna magna, and reduced or
absent CSF flow at the cervicomedullary junction (as assessed
by cine phase contrast MRI). The vast majority of patients had
some osseous features of CMI (12), such as hypoplasia of the
posterior fossa, reduced length of the supraocciput, increased
slope of the tentorium, and reduced length of the clivus. Other
findings that were present in some patients included anterior
displacement of the cerebellum, kinking of the medulla, ret-
roflexion of the odontoid, basilar invagination, scoliosis,
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 OPERATIVE TECHNIQUE FOR CHIARI TYPE I MALFORMATION

Klippel-Feil anomaly, and hydrocephalus. Syringomyelia or a
presyrinx state was present in 182 patients. In 163 patients
undergoing reoperation for failed Chiari surgery, there was
radiographic evidence of one or more of the following find-
ings: underdecompression of the posterior fossa; overdecom-
pression of the posterior fossa with cerebellar ptosis; pseudo-
meningocele formation; surgical meningocele; cranial settling;
basilar invagination; and hydrocephalus.
Clinical Criteria
The definition of what constitutes a symptomatic CMI has
been addressed elsewhere (12). In the current series, the indi-
cations for operation and were limited to one or more of the
following criteria: 1) evidence of progressive neurological de-
terioration; 2) presence of intractable or disabling symptoms
with a Karnofsky Performance Scale score of 70 or less; and 3)
MRI evidence of syrinx propagation. The mean Karnofsky
Performance Scale score in this series was 60 (unable to per-
form normal activities; requires occasional assistance). The
indications for reoperation on patients with failed Chiari sur-
gery were the same as those for patients undergoing primary
operations.
Surgical Decision Making
The presence of clinically significant anterior compression
of the cervicomedullary junction by a retroflexed or invagi-
nated odontoid was regarded as a contraindication for pri-
mary decompression of the posterior fossa. Anterior compres-
sions were divided into two groups by cervical traction.
Irreducible compressions were managed by transoral odon-
toidectomy, then a craniovertebral fusion at the time of pos-
terior decompression. Reducible compressions were managed
by a one-stage posterior decompression and fusion.
Each of the operative steps of posterior fossa decompression
was guided by CDU using real-time anatomic and physiolog-
ical measurements. The goals of surgery were as follows: 1)
adequate decompression of the cervicomedullary junction; 2)
creation of a retrocerebellar space of 8 to 10 cm3 volume; and
3) establishment of optimal CSF flow between the cranial and
spinal compartments.
External Decompression
Posterior fossa decompressions were performed under so-
matosensory evoked potential monitoring with the patient in
the prone position and the head flexed in a Mayfield head-
holder. The suboccipital area was exposed through a midline
incision that extended from the inion to the second cervical
spine. When a pericranial graft was harvested for duraplasty,
the incision was extended two fingerbreadths above the inion.
The size of the bony decompression was guided by CDU.
After a small suboccipital opening was made, the atlantooc-
cipital membrane was excised to facilitate imaging. Thereafter,
the craniectomy was enlarged in a stepwise manner to expose
the dura overlying the area of cerebellar impaction, as demar-
cated by compressed or obliterated subarachnoid spaces. The
superior limit of the craniectomy was never above the prepy-
ramidal fissure. Laterally, the craniectomy was widened to
create a nearly circular bony opening. The final dimensions of
the craniectomy were generally in the range of 3.5 ⫻ 3.5 cm.
After completion of the craniectomy, a decision was made
regarding whether or not to perform a laminectomy. The
determining factors were the extent of tonsillar herniation and
the required length of the dural incision. CDU was used to
establish the true position of the tonsillar tips, which was
typically 3 to 6 mm lower than predicted by MRI. A laminec-
tomy was not performed for herniations above C1. In patients
with intermediate hernia-
tions (12–15 mm), the supe-
rior aspect of the C1 arch was
sometimes underbitten (Fig.
1). Herniations of more than
15 mm generally required a
standard C1 laminectomy.
Similar guidelines were ap-
plied to herniations at lower
levels. The relationship of the
cerebellar tonsils to bony
landmarks was altered by
anomalies such as assimila-
tion of the atlas.

CDU Imaging before

Opening of the Dura
FIGURE 1. CDU images of cervicomedullary junction in a 42-year-old woman with CMI and syringomyelia. A,
sagittal image showing a 13-mm tonsillar herniation that extends to within 0.38 cm (distance between asterisks) of C1. Before opening of the dura,
Trapezoid outlines color Doppler sample volume. B, sagittal image after underbiting superior arch of C1, which CDU was used for anatomic
increases space between C1 and tonsillar tips to 0.97 cm (distance between asterisks). D, dura; P, posteroinferior orientation and to establish
cerebellar artery; T, cerebellar tonsil; SC, spinal cord; tbv, tonsillar blood vessel. Dashed line demarcates shadow baseline measurements of CSF
artifact of bone. flow (Fig. 2). Structures that

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MILHORAT AND BOLOGNESE

were routinely imaged included the cerebellar tonsils, the uvula, Cisterna magna volumes of less than 0.5 cm3 and CSF flow
the medulla, the upper cervical spinal cord, both vertebral arter- velocities in the range of 0 to 0.8 cm/s anterior and posterior to
ies, both posteroinferior cerebellar arteries, the marginal sinuses, the cervicomedullary junction were typical baseline findings.
the parenchymal arteries and veins, and bridging vessels sus-
pended by the arachnoid. The identification of aberrant vascular Dural Opening
anatomy, asymmetrical herniations, and neural displacements The dura was opened with a Y-shaped incision across the mar-
helped reduce the risk of surgical error (Fig. 3A). In patients ginal sinuses unless CDU imaging suggested a better line of entry.
undergoing reoperation for failed Chiari surgery, information The arachnoid was left intact, and imaging was repeated in appro-
concerning the location and extent of meningocerebral scarring priate cases to analyze whether duraplasty alone might be sufficient
was invaluable in planning dissection strategies (Fig. 3B). treatment. Opening the dura invariably resulted in some reexpan-
CSF circulation at the cervicomedullary junction was assessed sion of the cisterna magna, but a significant improvement in CSF
immediately before opening the dura. The following measure- flow was rarely observed. A simple duraplasty without additional
ments were made and stored: 1) the size and volume of the steps was performed in occasional patients who met the following
cisterna magna; 2) the size and volume of the dorsal cervical criteria: cisterna magna volume of at least 4 cm3; CSF velocity/flow
theca between C1 and the tonsillar tips; 3) CSF velocity/flow in of at least 2 cm/s; and CSF tracings demonstrating bidirectional
the cisterna magna; 4) CSF velocity/flow in the dorsal cervical movement with vascular and respiratory variations.
theca; and 5) CSF velocity/flow in the premedullary cisterns.
Internal Decompression
The arachnoid was opened,
and CSF was allowed to drain
spontaneously. We used mag-
nification vision to visualize
the area. The arachnoid was
resected widely, and adhe-
sions to the cerebellar tonsils,
posteroinferior cerebellar ar-
teries, and spinal cord were
coagulated and divided. The
tonsils were mobilized, and
the posteroinferior cerebellar
arteries were protected with
moist cotton patties. On the
FIGURE 2. CDU images of cervicomedullary junction after external decompression and before opening the dura.
basis of direct inspection, a de-
A, sagittal image in a 9-year-old boy with CMI showing an 11-mm tonsillar herniation, intact C1, and regional
vascular anatomy. B, pulse-wave Doppler tracing (bottom) with sagittal target image (top) in a 38-year-old man
cision was made whether or
with CMI and syringomyelia showing no cisterna magna and minimal CSF flow caudal to cerebellar tonsils (cur- not to shrink the cerebellar
sor). D, dura; P, posteroinferior cerebellar artery; VA, vertebral artery; T, cerebellar tonsil; SC, spinal cord; av, tonsils. The tonsils were not
arachnoid vessel; aa, arachnoid adhesion. Dashed line demarcates shadow artifact of bone. shrunken if the obex area was
open and a pulsatile flow of
CSF could be observed to exit
from the fourth ventricle into
the dorsal cervical theca. In
most cases, the tonsils were re-
duced with low-voltage, bipo-
lar coagulation until the tonsil-
lar tips were positioned at or
slightly above the putative
level of the foramen magnum.

Expansile Duraplasty
The dura was closed with a
FIGURE 3. A, axial B-scan real-time image of cervicomedullary junction in a 47-year-old woman with CMI and
graft of autogenous pericra-
syringomyelia showing an 18-mm asymmetrical tonsillar herniation with spinal cord displacement. B, sagittal CDU nium or reconstituted cadav-
image of cervicomedullary junction in a 3-year-old girl undergoing reoperation for failed Chiari surgery showing eric dura. Cadaveric grafts,
meningocerebral cicatrix with dura adherent to posteroinferior cerebellar artery and tonsillar branches. D, dura; P, which most closely resemble
posterior inferior cerebellar artery; T, cerebellar tonsil; tbv, tonsillar blood vessel; SC, spinal cord. living dura, were discontin-

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 OPERATIVE TECHNIQUE FOR CHIARI TYPE I MALFORMATION

ued for general use in 2001 because of limited supply and con-
cerns related to transmissible prions. A graft of approximately 5
cm in length and 2.5 cm in width was usually sufficient to
produce a competent retrocerebellar space. The graft was an-
chored to the poles of the incision and sewn in place with
continuous locking sutures of 5-0 Gore-Tex (W.L. Gore & Asso-
ciates, Inc., Flagstaff, AZ). Before the last suture was tied down,
the retrocerebellar space was inflated with 30 to 40 ml of sterile
saline to expand the graft and to eliminate intradural air bubbles,
which can degrade CDU images. Valsalva maneuvers were per-
formed to ensure a watertight closure. CSF leaks were corrected
by oversewing the suture line.
CDU Imaging after Closing the Dura
After dural closure, CDU imaging was repeated to assess
the goals of surgery. Optimal CSF flow was found to have the
following characteristics: a peak velocity of 3 to 5 cm/s; bidi-
rectional movement; and a waveform exhibiting arterial, ve-
nous, and respiratory variations (Fig. 4). Figures 5 and 6 show
typical findings before and after lysis of the arachnoid, shrink-
age of the tonsils, and duraplasty. Postoperative neuroimag-
ing demonstrated a normal-sized cisterna magna and unre-
stricted CSF flow anteriorly and posteriorly through the
foramen magnum in most patients. Overly generous duraplas-
ties and iatrogenic meningoceles were associated with subop-
timal CSF flow velocities (⬍1 cm/s) (Fig. 7A). The problem
could usually be corrected by tightening the graft with rein-
forcing sutures or adding a restrictive graft (Fig. 7B). Excessive
CSF flow velocities (⬎8 cm/s) were most often encountered
during reoperations in which the dura was densely scarred
and thickened. Such observations are consistent with princi-
ples of fluid mechanics governing rates of flow through spaces
of varying compliance and cross sectional area.

Wound Closure
The suboccipital craniectomy
was covered with a titanium
mesh/acrylic cranioplasty to
protect the duraplasty and limit
FIGURE 4. Optimal CSF flow characteristics. Pulse-wave Doppler tracings in a 38-year-old woman with a 19-mm the extent of extradural scar-
tonsillar herniation after lysis of arachnoid, shrinkage of tonsils, and duraplasty show a peak CSF velocity of 4 ring. The paraspinal muscles
cm/s, bidirectional movement, arterial pulsations (A), and respiratory and venous variations (B). were sewn to the inferior border
of the plate. In patients at risk
for a CSF leak, a lumbar drain
was inserted after surgery.

DISCUSSION
The surgical treatment of
CMI requires familiarity with
a great number of technical
options, some of which are
controversial. Among the un-
settled questions are the fol-
lowing: the size of the crani-
FIGURE 5. CSF flow in a 39-year-old woman with a ectomy (8, 10, 16); the extent
10-mm tonsillar herniation before and after lysis of arach- of the laminectomy (10, 15);
noid, shrinkage of tonsils, and duraplasty. A, pulse-wave the need to preserve (13) or
Doppler tracing (bottom) with sagittal target image open (1) the arachnoid; the
(top) before opening the dura, showing no significant need for additional steps
CSF flow caudal to cerebellar tonsils (cursor). B, pulse- such as lysis of the arachnoid
wave Doppler tracing (bottom) with sagittal target im- (9, 15), shrinkage or resection
age (top) after internal decompression showing CSF ve- of the cerebellar tonsils (1, 7),
locity of 3 cm/s. Red signal on target image (cursor)
stenting of the fourth ventri-
represents CSF flow; multicolor blots represent aliasing
from vascular structures. C, sagittal CDU image after
cle (5), and plugging the obex
internal decompression showing CSF flow (red signal) (14); the need to close dura
through the cisterna magna and dorsal cervical theca. (15) or leave it open (19); the
Multicolor blots represent aliasing from vascular struc- size of the duraplasty (10, 16);
tures. D, dura; DP, duraplasty; P, posteroinferior cere- the optimal material for dura-
bellar artery; T, cerebellar tonsil; SC, spinal cord; CSF, plasty (1, 9, 10); and the need
cerebrospinal fluid. for a cranioplasty (17). There

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MILHORAT AND BOLOGNESE

The introduction of CDU as an intraoperative guide for

posterior fossa surgery is an important technological advance
that permits the neurosurgeon to tailor operative steps accord-
ing to patient-specific variables on the basis of real-time ana-
tomic and physiological measurements. Of its potential uses,
CDU is particularly well suited for Chiari surgery in which
technical decisions are based largely on preoperative neuro-
radiological findings. A potential disadvantage of CDU is its
sophisticated methodology. Special training is required for
competence in the acquisition and interpretation of data.
The development of strategies for tailoring the steps of
Chiari surgery by the use of CDU involved a number of
assumptions. The first of these was that the area of cerebellar
impaction conforms to the area of compressed or obliterated
subarachnoid spaces. Supporting this assumption is neurora-
diological evidence that effacement of the subarachnoid space
is one of the earliest signs of cerebral displacement and that
FIGURE 6. CSF flow in a 40-year-old man undergoing reoperation for extra-axial masses tend to produce a congruent area of cortical
failed Chiari surgery after lysis of arachnoid, shrinkage of tonsils, and sulcus compression (6). Another assumption was that the ideal
duraplasty. Sagittal CDU image shows jet stream flow of CSF (blue sig- volume of the reconstructed retrocerebellar space should be in
nal) out of the fourth ventricle (arrow) with a peak velocity of 4 cm/s as the range of 8 to 10 cm3. This was based on two pieces of
compared with no flow before internal decompression. Variations in color evidence: anatomic estimates of cisterna magna size (18), and
coding are a function of direction of flow. D, dura; T, cerebellar tonsil; SC, volumetric measurements of the posterior cranial fossa indi-
spinal cord. cating that total CSF volume (26.7 ⫾ 7.3 cm3) is reduced by a
mean of 10.8 ⫾ 7.1 cm3 in patients with CMI (12). No assump-
tions could be made concerning normal rates of CSF flow at
is no consensus on these matters and little in the way of the cervicomedullary junction. The measurements reported
scientifically controlled comparative data. here were obtained in anesthetized patients in the prone po-
Complicating the treatment of CMI is the wide range of sition before and after posterior fossa decompression. Under
patient-specific variables. These include the level of tonsillar the conditions of operation, an optimal rate of CSF flow was
descent, the degree of craniovertebral dysmorphism, the tight- assumed to have been achieved in patients with adequate
ness of the posterior fossa, the completeness of the CSF block, decompressions, visible evidence of an unrestricted and pul-
and the presence or absence of syringomyelia. It is doubtful satile flow of CSF from the fourth ventricle into the dorsal
that any operative technique with fixed steps and inalterable cervical theca, and a competent duraplasty. Mean peak veloc-
dimensions of decompression can deal effectively with all of ities after decompression ranged from 3 to 5 cm/s as com-
these variables. pared with 0 to 0.8 cm/s before decompression. Because nor-
mal CSF exhibits to-and-fro
movements with distinct vas-
cular and respiratory varia-
tions (11), the presence of
these findings on postdecom-
pression waveform tracings
suggests that the flow veloc-
ities achieved were in a phys-
iological range. Postopera-
tive cine phase contrast MRI
confirmed the presence of
unrestricted CSF flow anteri-
orly and posteriorly through
the foramen magnum in
most patients.
FIGURE 7. CDU images of cervicomedullary junction in a 49-year-old woman undergoing reoperation for failed
Chiari surgery and meningocele formation. A, pulse-wave Doppler tracing (bottom) with sagittal target image
The ability to adjust surgi-
(top) showing no CSF flow in the cisterna magna (cursor) before repair of the meningocele. Multicolor blots rep- cal strategies on the basis of
resent aliasing from vascular structures. B, pulse-wave Doppler tracing (bottom) with sagittal target image (top) real-time anatomic and phys-
showing CSF velocity of 3 cm/s in the cisterna magna (cursor) after repair of the meningocele with restrictive iological measurements is a
duraplasty. M, meningocele; T, cerebellar tonsil; DP, old duraplasty; RD, restrictive duraplasty. defining step in the evolution

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 OPERATIVE TECHNIQUE
of Chiari surgery. It remains to be analyzed which strategies
are optimal. Ultimately, the value of the current technique will
depend on the results of an ongoing longitudinal study that
correlates CDU-guided operative steps with pre- and postop-
erative MRI scans, pre- and postoperative cine phase contrast
MRI scans, symptoms and signs, and long-term patient
outcome.
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15. Rekate HL: Management of Chiari malformations, in Dickman CA, Spetzler
RF, Sonntag VKH (eds): Surgery of the Craniovertebral Junction. New York,
Thieme, 1998, pp 507–521.
16. Sahuquillo J, Rubio E, Poca MA, Rovira A, Rodriguez-Baeza A, Cervera C:
Posterior fossa reconstruction: A surgical technique for the treatment of
Chiari I malformation and Chiari I/syringomyelia complex—Preliminary
results and magnetic resonance imaging quantitative assessment of hind-
brain migration. Neurosurgery 35:874–884, 1994.
17. Sakamoto H, Nishikawa M, Hakuba A, Yasui T, Kitano S, Nakanishi N,
Inoue Y: Expansive suboccipital cranioplasty for the treatment of syringo-
myelia associated with Chiari malformation. Acta Neurochir (Wien) 123:
949–961, 1999.
18. Schaefer GB, Thompson JN, Bodensteiner JB, Gingold M, Wilson M, Wilson
D: Age-related changes in the relative growth of the posterior fossa. J Child
Neurol 6:15–19, 1991.
19. Williams B: Surgery for hindbrain related syringomyelia. Adv Tech Stand
Neurosurg 20:107–164, 1993.
NEUROSURGERY
FOR CHIARI TYPE I MALFORMATION
Acknowledgment
The authors received no financial support in conjunction with the conduct of
the study described in this article.
COMMENTS
T he authors present their extensive experience with the use
of intraoperative color Doppler ultrasonography (CDU) as
a guide in tailoring suboccipital decompressive surgery in
patients with Chiari Type I malformation (CMI). In their ex-
perience, overdecompression as well as underdecompression
may result in less than optimal neurological outcomes. Mea-
surement of cerebrospinal fluid (CSF) pulsation velocities and
calculation of the volume of the cisterna magna before and
after reconstruction may aid in tailoring the operation for each
patient. Use of the technique clearly requires dedication and
experience. As the authors point out, its validation depends on
longitudinal studies correlating CDU findings with measure-
ments on the basis of cine magnetic resonance imaging and
functional outcome.
Robert G. Grossman
Houston, Texas
I n general, excellent outcomes are expected in the treatment
of patients with hindbrain herniation or CMI. It is definitely
true, however, that this group of patients encompasses a large
number of different conditions and degrees of severity. It
makes a great deal of sense to tailor the operation to the
specific needs of the individual patient, and the use of CDU is
a logical way of distinguishing which patients need what type
of operation. The goal of creating a 10-cm3 volume for the
newly created cisterna magna likewise is logical and ensures
adequate transmission of CSF pulses from the cranial to the
cerebrospinal compartments and the mixing of CSF from ce-
rebrospinal to cortical subarachnoid spaces.
Logically, the authors decided the proper parameters of
treatment, and they performed CDU to verify that they
achieved those objectives. This study does not analyze
whether these parameters are superior to other proposed
treatments for the condition, such as more extensive bone
removal or scoring rather than patching of the dura (1, 2, 4).
“Shrinking” of the cerebellar tonsils is commonly performed
to ensure that there is sufficient room for the creation of a new,
more copious cisterna magna. My preference is to avoid this
maneuver, because the cerebellum tends to adhere to the dural
patch if the pial surface is violated. If more room is needed
than can be provided by the patch itself, I recommend reat-
taching the piece of cranium that has been removed from the
posterior fossa after it has been rotated to create an enlarged
posterior fossa. I then use a central dural tacking stitch to hold
the patch against the bone to ensure that there is a new
cisterna magna. Sakamoto et al. (3) used this technique suc-
cessfully. I think that performing CDU to document the cre-
ation of an adequate cisterna magna, thereby allowing the
VOLUME 53 | NUMBER 4 | OCTOBER 2003 | 905
MILHORAT AND BOLOGNESE
procedure to be tailored to the individual patient, holds
promise.
Harold L. Rekate
Phoenix, Arizona
1. Gambardella G, Caruso G, Caffo M, Germano A, La Rosa G, Tomasello F:
Transverse microincisions of the outer layer of the dura mater combined with
foramen magnum decompression as treatment for syringomyelia with Chiari
I malformation. Acta Neurochir (Wien) 140:134–139, 1998.
2. James HE, Brant A: Treatment of the Chiari malformation with bone decom-
pression without durotomy in children and young adults. Childs Nerv Syst
18:202–206, 2002.
3. Sakamoto H, Nishikawa M, Hakuba A, Yasui T, Kitano S, Nakanishi N, Inoue
Y: Expansive suboccipital cranioplasty for the treatment of syringomyelia
associated with Chiari malformation. Acta Neurochir (Wien) 141:949–961,
1999.
4. Sindou M, Chavez-Machuca J, Hashish H: Cranio-cervical decompression for
Chiari type I-malformation, adding extreme lateral foramen magnum open-
ing and expansile duroplasty with arachnoid preservation: Technique and
long-term functional results in 44 consecutive adult cases—Comparison with
literature data. Acta Neurochir (Wien) 144:1005–1019, 2002.
M ilhorat and Bolognese describe their observations in per-
forming intraoperative CDU. The use of this elegant
investigational technique has permitted them to gauge the
amount of bone that they wish to remove in a given situation
and has provided them with a demonstration of anatomy. In
addition, these studies have generated some interesting quan-
titative data regarding CSF flow and the size of the cisterna
magna before and after decompressive surgery.
From the practical point of view, approximate measure-
ments for optimal bone removal in an individual patient can
be made on the basis of preoperative magnetic resonance
imaging scans. It is clear on the basis of this study that the
large bony decompressions that were once widely used are
not necessary. The problem is really with the tonsils them-
selves, even though I think that the underlying anatomic
abnormality is smaller than the usual posterior fossa volume.
It is not necessary, however, to enlarge the entire posterior
fossa to achieve tonsillar decompression.
Because the authors noted a small cisterna magna before
performing tonsil reduction and duraplasty, and because the
establishment of a larger cisterna magna seems to correlate
with good outcome (2), it is difficult to understand their
reluctance to perform a complete C1 laminectomy in every
patient. If nothing else, performing this procedure permits one
to create a larger cisterna magna. I concur with the desirability
of reducing the mass of the cerebellar tonsils, which my col-
leagues and I have performed routinely for a number of years.
This can be accomplished by using bipolar current as de-
scribed (1), but occasionally when the tonsils are gliotic or
large, subpial resection should also be considered.
Ulrich Batzdorf
Los Angeles, California
1. Halamandaris G, Batzdorf U: Adult Chiari malformation. Contemp
Neurosurg 11(26), 1989.
2. Sahuquillo J, Rubio E, Poca MA, Rovira A, Rodriguez-Baeza A, Cervera C:
Posterior fossa reconstruction: A surgical technique for the treatment of
Chiari I malformation and Chiari I/syringomyelia complex—Preliminary
results and magnetic resonance imaging quantitative assessment of hindbrain
migration. Neurosurgery 35:874–885, 1994.
M ilhorat and Bolognese report an excellent technique ap-
plication. Dr. Milhorat is a member of the scientific
community who possibly performs more CMI operations than
any other surgeon in the United States. He shows that he
meticulously studies each patient and each approach so that
he may improve on what has been done previously. The
authors achieve the simple goal of convincing the reader that
CDU is a safe, effective, and efficient adjuvant in surgery
performed for hindbrain malformations. Clearly, this modal-
ity requires the right equipment and skill to be used and
interpreted correctly. However, it is presented as yet another
noninvasive technique (in addition to the cine phase contrast
magnetic resonance imaging) that does not contaminate the
entity it is attempting to evaluate, specifically the CSF and its
flow patterns. This is an enormous advantage for those sur-
geons who base their treatment on the correction of the ana-
tomic and thus the physiological conditions that exist in the
posterior fossa. The authors are enthusiastic about the possi-
bility that this technique may alter individual surgical ap-
proaches to CMI on the basis of specific, real-time anatomic
and physiological factors. This may well be true. First, how-
ever, the authors must show how this technique and the
subsequent tailored surgical approach correlate with patient
outcome in terms of symptoms and radiological criteria such
as magnetic resonance imaging. In the next several years, I
have little doubt that Milhorat and Bolognese will do precisely
that.
Richard G. Ellenbogen
Seattle, Washington