Content uploaded by Takato Morioka
Author content
All content in this area was uploaded by Takato Morioka
Content may be subject to copyright.
Neurol Med Chir （Tokyo） 50， 870 一一876， 2010
Neurosurgicα1 Mαmgeme皿意of Pα重ien意s
With Lumbosacral Myeloschisis
Takato MORIOKA， Kimiaki HASHIGUCHI＊， Nobutaka MUKAE，
Tetsuro SAYAMA， and Tomio SASAKI“
Department of Neurosurgery， Kyushu Rosai Hospital， Kitakyushu， Fukuoka；
’Department of Neurosurgery， Graduate School of Medical Sciences， Kyushu University， Fukuoka
Abstract
Myeloschisis is the most serious and complex congenital anomaly in spina bifida manifesta （cystica）．
However， with improvements in medical care and increased understanding of its pathophysiology， the
associated long－term morbidity and mortality rates have been significantly reduced． This article rev－
iews various issues associated with the neurosurgical management of patients with myeloschisis， such
as perinatal management， repair surgery for myeloschisis， neurosurgical management of hydrocepha－
lus， Chiari malformation type II， tethered cord syndrome and epilepsy， and intrauterine fetal surgery．
Key words： myeloschisis， myelomeningocele， hydrocephalus， Chiari malformation type ll，
tethered cord syndrome
Introduction
Spina bifida manifesta （cystica） causes visible skin
lesions such as meningocele， myelomeningocele and
myeloschisis， which occur when the neural tube
fails to fold normally during postovulatory days 21
to 27．33） Myeloschisis is the most serious and com－
plex congenital anomaly in spina bifida manifesta．
However， with improvements in medical care and
increased understanding of its pathophysiology， the
associated long－term morbidity and mortality rates
have been significantly reduced． ln this article， vari－
ous issues in the neurosurgical management of
patients with myeloschisis are reviewed．
Terminology
1
㌧醗
r
7剣 、鵡へ 一．繊3、
Fig．1 Various manifestations of myelomeningocele
and myeloschisis． （A） A placode is seen in the my－
elorneningocele． （B） ln the myelomeningocele， the ex－
posed placode is myeloschisis． （C） Myeloschisis， con－
sisting of a collapsed myelomeningocele， is a cleft spinal
cord arising from the failure of neural tube closure．
In clinical practice， it is difficult to distinguish
differences between myelomeningocele and my－
eloschisis （Fig． 1）． Myeloschisis is a cleft spinal cord
arising from a failure of neural tube closure （Fig．
IC）．i9・24・6e） The placode is defined as a plate of em－
bryonic epithelial cells constituting a primordial cell
group from which the spinal cord arisesi9・24） and is
often seen in a myelomeningocele （Fig． IA）． ln a my－
elomeningocele， an exposed placode is often a my－
eloschisis （Fig． IB）． ln other words， a myeloschisis is
a collapsed myelomeningocele （Fig． IC）． When the
placode in the myelomeningocele sac is a cleft spinal
cord， this anomaly is classified as myeloschisis
rather than myelomeningocele．i9・24・60）
Perinatal Management
Recent advances in morphological examinations
such as ultrasonography3） and prenatal magnetic
resonance （MR） imaging3・iO・36・37） have made it possi－
ble to make a correct diagnosis of spina bifida
manifesta in the prenatal period． ln particular， half－
Fourier acquisition single－shot turbo spin－echo T2－
weighted images， which provide useful diagnostic
images for structural abnormalities related to the
870
Presented by Medical*Online
Management of Myeloschisis 871
黙．
Fig． 2 （A） Prenatal half－Fourier acquisition single－shot
turbo spin－echo T2－weighted image at 29 weeks and 1
day of gestation showing ventricular enlargement and
myelomeningocele． A neural placode is noted in the my－
elomeningocele． （B） Constructive interference in
steady－state magnetic resonance image on the day of
birth clearly demonstrating a detailed anatomical
relationship between the neural placode and the ventral
roots． （C） Photograph of the baby at birth revealing a
large head and myeloschisis at the thoracolumbar area．
cerebrospinal fluid （CSF） space such as spina bifida
manifesta， hydrocephalus and Chiari malformation
type II （CM II） in a reasonable time， are useful ad－
juncts to ultrasonography （Fig， 2A）．，36，42）
When the diagnosis is made prenatally， intensive
perinatal management is mandatory． The baby is
usually delivered by planned cesarean section to
prevent rupture of the sac and subsequent ascending
infection， although there is no evidence for these
problems，29・55） Optimally， the cesarean delivery will
be scheduled in a center that employs neurosur－
geons and pediatricians who are specialized in ne－
onatal intensive care． Once a child is born with my－
eloschisis， thorough systemic physical and radiolog－
ical examinations must be conducted to look for as－
sociated conditions， such as pulmonary or cardiac
abnormalities． Neurological examinations are also
performed to evaluate the level of diagnosis of spinal
dysfunction．
Neuroradiological examinations are important to
determine whether hydrocephalus and CM II are
present and to evaluate the morphology of the
myeloschisis．i9） However， it is thought that spinal
MR imaging is not always essential， because surgi－
cal repair is considered to be a priority and must be
performed as soon as possible．52） Furthermore， with
the recent advances in prenatal high－resolution MR
imaging， no additional information can be obtained
by postnatal MR imaging．i） However， in our institu－
tion， we perform both spinal and cranial MR imag一
ing．24・25・36） ln particular， three－dimensional heavily
T2－weighted images such as constructive interfer－
ence in steady－state images， which deliver excellent
contrast between the CSF and solid structures such
as the placode and spinal roots with a minimum
slice thickness of O．7 mm in any specified linear or
curved plane，i7，i8・35，38－40・47－49，56｝ clearly demonstrate
the detailed anatomical relationship between the
placode and spinal roots （Fig． 2B）．i9） Three－dimen－
sional computed tomography is also performed to
evaluate the extent of the spina bifida and associated
bony anomalies such as kyphosis or bony septum in
diastematomyelia．24）
When CSF leakage or a thin sac is noted （Fig．
2C）， the baby is optimally operated on soon after
birth．9・i3，52） However， no relationship was demon－
strated between the timing of surgical intervention
and the eventual outcome．4） lt is generally accepted
that the operation can be postponed for up to 48 or
72 hours without any increase in meningitis． This
delay is particularly important for comprehensive
discussions， counseling and emotional support for
the upset parents in need of a decision－making
process before establishing consent for or against
surgical management of their newborn child．“） Prior
to the surgical intervention， the exposed myeloschi－
sis should be gently covered with sterile gauze． The
infant should be positioned prone or laterally to
avoid pressure on the placode．
Repair Surgery for Myeloschisis
Regarding intraoperative neurophysiological moni－
toring， both motor and sensory responses can be
recorded．‘4） To monitor the motor system， the pla－
code or roots in the operative field are stimulated，
and the evoked muscle responses （compound muscle
action potentials： CMAPs） from the external anal
sphincter and leg muscles such as the gastro－
cnemius， biceps femoris and tibialis anterior are
recorded （Fig． 3A－C）．i9） To monitor the sensory sys－
tem， the posterior tibial nerve at the ankle is stimu－
lated， and the sensory responses （somatosensory
evoked potentials： SEPs） from the placode in the
operative field are recorded （Fig． 3D， E）． ln many
patients with myeloschisis， stimulation of the pla－
code or roots evokes CMAPs of the legs （Fig． 3A， C）．
In addition， we can record polyphasic SEPs on the
placode following stimulation of the posterior tibial
nerve （Fig． 3D）． These findings indicate that the pla－
code is often functioning．“） Thus， the most im－
portant part of the surgery is preservation of the
function of the placode． Consequently， careful
dissection under an operating microscope and
neurophysiological monitoring are essential．
Neurol Med Chir （Tokyo） 50， September， 2010
Presented by Medical*Online
872 T． Morjoka et al．
．』一‘ @ 了禰面副s置目・
．一黶D
り ぬ の
．＿＿へ．＿＿．．
Tlb㎏lisロ1鳳じriし
．一㌃＿
GaStiVL↑neniれ」
@
@
@［
殴
ノ
一1
ヘー．I
I
一～＿一
：
コ
．一．．．＿一一． ｲ廻贋
：：t i一一
製
蒐．
／“1
冶
評 一一一一一一
ほ れ ゆぬビ
悔一ノ面面、
；一一＼／∀（二曲・，，
律st附￠ncm田；
階調：
賦tt
L」一盾窒窒w．一／
Vdiey－mv一一N一一
一一一一vu一一一一一
“N
L．
B） h剛
鱒
⑳
籔F
e
v⊂ブ
姻》
トノ．
療畷
． 総
）Gf・？・・
L ’
Fig． 3 （A， C） lntraoperative evoked compound muscle
action potentials （CMAPs） of the external anal sphincter
and leg muscles such as the biceps femoris， tibialis an－
terior and gastrocnemius following electrical stimula－
tion of the placode． After stimulation of the upper and
middle parts of the placode， CMAPs of the leg muscle
can be recorded． （B） The stimulation sites are indicated
on a photograph of the placode． （D， E） Somatosensory
evoked potentials （SEPs） recorded from the placode in
the operative field following stimulation of the left （D）
and right （E｝ posterior tibial nerves of the ankle． Poly－
phasic SEPs are recorded following stimulation of the
left posterior tibial nerve （D， arrow）． （F， G） Schematic
drawings of reconstructive surgery for myeloschisis．
（H， 1） Operative photographs show careful dissection of
the placode under an operative microscope （H） and ana－
tomical reconstruction of the spinal cord into a tubular
structure （1）．
Another important issue in the surgery is anatomical
reconstruction of the spinal cord， so as to retubulate
the placode． Pia－arachnoid to pia－arachnoid closure
of the placode into a tubular structure and suspen－
sion of the “re－made” cord in the CSF compartment
can establish a more normal environment （Fig． 3F，
G）．i2・3i・52） Therefore， the surgery consists of （i） careful
dissection of the placode and complete untethering，
（ii） reconstructive surgery for the myeloschisis to
retubulate the placode， （iii） dural plasty or complete
dural closure， （iv） prevention of CSF leakage with a
paravertebral muscle or fascia flap， and （v） skin
closure．i2）
The infant should be placed in the prone position
on lateral rolls to prevent pressure on the abdomen．
The entire back， including the myeloschisis， is then
prepared with 10 × diluted lsodine． Under a surgical
microscope， the placode is separated from the inter－
mediate zone． All nerve roots， which usually
originate from the ventral side of the placode， should
be carefully preserved． The goals of separation of the
placode are to reduce the possibility of future tether－
ing and to enable subsequent reconstruction of a
normal neural tube．52） Thus， the caudal end of the
placode or terminal filum， if identified， can be
sectioned based on the results of electrical stimula－
tion．i2・52） Once the placode has been separated from
the intermediate zone， it should be carefully inspect－
ed so as to remove any potential tissue that could
form a dermoid at a later date （Fig． 3H）．i2） The pla－
code can then be “reconstructed” into a tubular
structure by pia－arachnoid to pia－arachnoid closure．
This technique was originally described by McLone
in 1980．3i） Closure of the central canal and suspen－
sion of neural tissue in a pia－arachnoid－lined CSF
compartment establish a more normal microen－
vironment that is expected to prevent adhesion of
neural elements to the closure and subsequent
tethering of the spinal cord （Fig． 31）．i2）
Once the placode has been reconstructed， atten－
tion is turned to separating the dura from the
epidural space． Once the dura has been separated， it
should be reconstructed in a watertight fashion．
When a dural defect is noted， a pedunculated flap of
the fascia and muscle is used． Use of artificial
materials such as Gore－Tex is not recommended．
The dural surface can be covered with fibrin glue．
The closure should be tested by having the
anesthesiologist induce a Valsalva maneuver． A
watertight dural closure will reduce the risk of
postoperative CSF leakage and consequently reduce
the incidence of cord tethering．i2） The next layer to
be addressed is the muscle closure．’2） lf the lesion is
small， adequate muscle can often be obtained by
making lateral releases in the paravertebral muscles．
For larger defects， a pedunculated muscle flap can
be moved from the paravertebral muscles above the
Ievel of the lesion． Once the muscle has been closed，
the skin is examined to allow a primary closure． Skin
without subcutaneous tissue should be excised from
the skin margin． ln most cases， the skin can be
closed in a simple midline closure． ln some cases， a
flap mobilization is required． ln such cases， under－
lying muscle closure is important to obtain good
wound healing．52） There are many reports of plastic
surgical closures．26・27・4i，so）
Neurol Med Chir （Tokyo） 50， September， 2010
Presented by Medical*Online
Management of Myeloschisis 873
Neurosurgical Management
of Hydrocephalus
The malority of children with myeloschisis have as－
sociated hydrocephalus．30， The pathophysiological
mechanism of the associated hydrocephalus is un－
clear． With CM II， the posterior fossa is small and
the intradural contents of the upPer cervical canal
are tight． The hydrocephalus may be related to an a－
queductal stenosis or caused by blockade of the out－
lets of the fourth ventricle．6〕In addition venous ab．
り
normalities resulting from a small posterior fossa
have been suggested as a mechanism for the
hydrocephalus．45〕Regardless of the cause， when
progressive hydrocephalus is present it should be
treated．30〕
Frequently， hydrocephalus is obvious during the
prenatal period〔：Fig．2A〕or at birth〔Fig．2：B， C〕． If
signs of increas．ed intracranial pressure are present，
shunt placement is performed at the same time as
the primary surgery for the myeloschisis．34〕
However， if the repair is delayed for morle than 36
hours， there is an increased incidence of shunt in費
fection．11）When signs of meningitis are present in
CSF obtained at the repair surgery or the baby has a
low birth weight， placement of an Ommaya CSF
reservoir and repeated CSF tap or external ventricu－
Iar drainage are recommended． There is increasing
evidence suggesting that the failure rate of en－
doscopic third ventriculolstomy〔ETV〕is high in in－
fants and that ETV should be performed when the
child is older．30・52・54〕
Although ventricular enlargement is evident in
the vast majority of newborns with myeloschisis，
treatment of hydrocephalus is required in around
80％of patients．52〕It is interesting to note that shunt
treatment is usually required within the first month
of life， and not after 6 months．45・52〕
Once a shunt has been placed， a more difficult
problem is the management of its malfunction．30）In
the majority of cases， the symptoms and signs are
the classical ones associated with increased sym－
ptoms such as headaches， vomiting and disturbance
of consciousness． In patients with myeloschisis，
blockage of the outlets of the fourth ventricle to the
central canal account for the common association of
syringomyelia． With the development of syrin－
gomyelia， compensation of the increased intraven－
tricular pressure can occur，7・16〕and the symptoms
and signs can be quite subtle． When the physiologi－
cal mechanisms for compensation have been ex－
hausted， sudden respiratory or cardiac arrest can oc－
cur．30・46）
At the time of shunt revision， ETV is a considera－
tion．30〕An 80％success rate for managing shunt mal．
function by ETV was reported in children with my－
elo．meningocele and the following criteria identified
for predicting the effectiveness of the pro：cedure：ol－
der age， triventricular hydrocephalus， and scarcely
represented subarachnoid space．54〕However， recent
authors30・52｝have provided arguments against ETV．
Specifically， a 20－30％failure rate and difficulties
were associated with assessing ETV failure even us－
ing MR imaging to assess stoma patency alld cine－
MR imaging to evaluate flow．30〕Although ETV may
eliminate the need for a mechanical device， it does
not always eliminate the need for a ventricular
shunt．30〕At the time of ETV， ventricular drainage
may be necessary to measure intracranial pressure
and for safety．30）Furthermore， if the ETV closes， the
symptoms and signs may again be quite subtle．5〕
Once the mechanical device has been removed the
ラ
family may think that there is no longer any need for
ashunt． Education in this regard is important be－
cause it can prevent unnec．essary death from ETV
failure．ユ：5βo〕
Recognition of shunt independence is another
problem．52〕Among 850 children affected by non－
tumoral hydrocephalus， obvious shunt indepen－
dence could be demonstrated in 3．2％of patients and
that 25％had a myelomeningocele．22）On the other
hand， the erroneous concept of shunt independence
in spina bifida patients was described and sudden
respiratory and cardiac arrest was caused by ex－
haustion of the physiological mechanisms for com－
pensation．46）In a more recent paper， strict monitor－
ing of patients who presented with imagin．g－
documented shunt anomalies found that patients ol－
der than 40r 5 years with very low cord lesions who
underwent shunt treatment within a few weeks of
birth and without a recent history of shunt revision
see．med to have higher chances of becoming in－
dependent of the shunt．52〕
Neurosurgical Management of CM II
Although most patients with myeloschisis have ana－
tomical CM II， only 10－30010 become symptomatic
and require surgery．i3） However， symptomatic CM II
is the most common cause of death in patients with
myelomeningocele or myeloschisis who are younger
than 2 years of age．32） The first step in managing a
symptomatic CM II is ensuring that a shunt， if
present， is working optimally or treating previously
untreated hydrocephalus．5i，52） A shunt malfunction
or untreated hydrocephalus can turn a radiographi－
cally evident C M II into a symptomatic CM II by in－
creasing the intracranial pressure with subsequent
downward herniation of an already caudally dis－
placed brainstem and cerebellum． Expeditious and
Neurol Med Chir （Tokyo） 50， Septernber， 2010
Presented by Medical*Online
874 T． Morioka et al．
knowledgeable evaluation and prompt surgical
decompression of the hindbrain can prevent serious
morbidity and mortality in such patients， especially
those younger than 2 years of age．5i） Patients with
CM II whose conditions are not cured by a shunt
treatment must undergo a decompressive proce－
dure． A symptomatic CM II in an older child often
presents with more subtle findings but rarely in
aCute crisis．43，51，58）
In decompressive surgery for CM II， suboccipital
craniectomy is extended from the foramen magnum
as far as the torcular herophili， since the posterior
fossa is usually small， and associated with a laminec－
tomy that is adequate to expose the lower level of the
cerebellar tonsil and vermis．5i） The dural sac is wi－
dened as much as possible using a dural patch， but
the arachnoid layer and neural structures are left un－
touched．52）
dren with myelomeningocele or myeloschisis should
be followed， ideally in a multidisciplinary clinic， by
neurosurgeons， orthopedic surgeons， and urologists
who are aware of this condition．2i）
Management of Epilepsy
Epilepsies in patients with myeloschisis are recog－
nized as relatively common occurrences and their
relationships with associated hydrocephalus and
ventricular shunting have well been documented．53）
We have described that the epileptogenesis in
patients with myeloschisis seems to be correlated
with coexisting cerebral abnormalities such as poly－
microgyria （or stenogyria）23） rather than a ven－
triculoperitoneal shunt．60） Since the epilepsies in
these patients can be well－controlled with medica－
tion， epilepsy surgery is not indicated．60）
Neurosurgical Management of
Tethered Cord Syndrome （TCS）
TCS is a stretch－induced functional disorder of the
spinal cord． The mechanical cause of TCS is an in－
elastic structure anchoring the caudal end of the spi－
nal cord that prevents cephalad movement of the
lumbosacral cord． Stretching of the spinal cord oc－
curs in patients when either the spinal column
grows faster than the spinal cord or the spinal cord
undergoes forcible flexion and extension．59）
Approximately 10－30010 of children will develop
TCS following repair of a myelomeningocele or my－
eloschisis． Since essentially all children with repair
surgery will have a tethered cord， as demonstrated
on MR images．， the diagnosis of TCS is made based
on clinical criteria．52） The six common clinical
presentations of TCS are increased weakness （550／o），
worsening gait （540／o｝， scoliosis （510／o）， pain （32010）， or－
thopedic deformity （1101o）， and urological dysfunc－
tion （60／o）．2i） The average age for TCS has been
reported to be 6－8 years，20・52） although the possibility
that even adults may require detethering is increas－
ingly reported in recent papers．i4・28）
The primary goal of surgery is to detach the spinal
cord where it is adherent to the thecal sac， thereby
relieving the stretch on the terminal portion of the
cord．2i） Early diagnosis and prompt surgical release
of the tethered cord results in stabilization or im－
provement in most cases．2i） After detethering of the
cord， pain relief and motor improvement are fre－
quently obtained． However， it is difficult to obtain
recovery of urinary dysfunction， scoliosis and ortho－
pedic signs， although these symptoms and signs
cease to progress．52） Since TCS may present with or－
thopedic and／or urological signs or symptoms， chi1一
Intrauterine Fetal Surgery
Intrauterine fetal surgery for myelomeningocele or
myeloschisis merits future investigations．8） Clinical
trials have already been carried out at selected in－
stitutes in the United States， but have not been per－
formed in Japan because of socio－ethical problems．
Intrauterine surgery decreases the incidence of hin－
dbrain Chiari malformation and shunt－dependent
hydrocephalus， but increases the incidence of
premature delivery．2） Fetal surgery reduces shunt－
dependent hydrocephalus in patients with lesions
below L3 before 25 weeks of gestation．57） However，
prospective parents should be cautioned ．not to ex－
pect any improvement in leg function as the result of
such fetal surgery． The potential benefits of fetal sur－
gery must be carefully weighed against the potential
risks of premature birth．8）
References
1） Blaicher W， Bernaschek G， Deutinger J， Mes－
serschmidt A， Sahindler E， Prayer D： Fetal and early
postnatal magnetic resonance imaging－s there a
difference？ J Perinat Med 32： 53－57， 2004
2） Bruner JP， Tulipan N， Paschall RL， Boehm FH， Walsh
WF， Silva SR， Hernanz－Schulman M， Lowe田， Reed
GW： Fetal surgery for myelomeningocele and the in－
cidence of shunt－dependent hydrocephalus． JAMA
282： 1819－1825， 1999
3） Cameron M， Moran P： Prenatal screening and diag－
nosis of neural tube defects． Prenat Diagn 29：
402－411， 2009
4） Charney EB， Weller SC， Sutton LN， Bruce DA， Schut
LB： Management of the newborn with myelomenin－
gocele： time for a decision－making process． PediatTics
75： 58－64， 1985
Neurol Med Chir （Tokyo） 50， September， 2010
Presented by Medical*Online
Management of Myeloschisis 875
）
5
）
6
）
7
〕
8
〕
9
ユ。〕
ユ1〕
12）
13）
14）
15）
ユ6〕
17）
18）
19）
Cinalli G， Sainte－Rose C， Chumas P， Zerah M，
Brunelle F， Lot G， Pierre－Kahan A， RenieT D： Failure
of third ventriculostomy in the treatment of aqueduc－
tal stenosis in children． J Neurosurg 90： 448－454， 1999
Cohen AR， Robinson S： Early management of my－
elomeningocele， in McLone DG〔ed〕：Pediαtric Nθu－
rosurgery： Surgery of the Developing Nervous System，
ed 4． Philadelphia， WB Saunders， 2001， pp 241－260
Epstein F， Marlin AE， Hochwald G， Ransohoff J： My－
elomeningocele： a progressive intra－uterine disease，
Dev Med Child Neurol Suppl 37： 12－15， 1976
Fichter MA， Dornseifer U， Henke J， Schneider KTM，
Kovacs L， Biemer E， Bruner J， Adzick NS， Harrison
MR， Papadopulos NA： Fetal spina bifida repair－cur－
rent trends and prospects of intrauterine neurosur－
gery． Fetal Diagn Ther 23： 271－286， 2008
Fukui K， Morioka T， Kawamura T， Yoshida F， Nishio
S， Hikino S， Yamamoto J， Kira R， Kusuhara K：
［Delayed plastic repair of lumbosacral myeloschisis］．
No Shinkei Geka Sokuho 12： 429－433， 2002 （Japanese）
Fukui K， Morioka T， Nishio S， Mihara F， Nakayama
H， Tsukimori K， Fukui M： Fetal germinal matrix and
intraventricular hemorrhage diagnosed by magnetic
resonance imaging． Neuroradiology 43： 68－72， 2001
Gamache FW Jr： Treatment of hydrocephalus in
patients with meningomyelocele or encephalocele： a
recent series． Childs Nerv Syst 11： 487－488， 1995
Gaskill SJ： Primary closure of open myelomenin－
gocele． Neurosurg Focus 16（2）： E3， 2004
Gaskill SJ， Hudgins RJ： Myelomeningocele． Neu－
rosurg Focus 16（2）： lntroduction， 2004
George TM， Fagan LH： Adult tethered cord syn－
drome in patients with postrepair myelomenin－
gocele： an evidence－based outcome study． J Neu－
rosllrg 102（2 Suppl）： 150－156， 2005
Hader WJ， Drake J， Cochrane D， Sparrow O， lohnson
ES， Kestle J： Death after late failure of third ven－
triculostomy in children． Report of three cases． J Neu－
rosurg 97： 211－215， 2002
Hall P， Lindseth R， Campbell R， Kalsbeck JE， Desousa
A： Scoliosis and hydrocephalus in myelocele
patients． The effects of ventricular shunting， J Neu－
rosurg 50： 174－178， 1979
Hashiguchi K， Morioka T， Fukui K， Miyagi Y， Mihara
F， Yoshiura T， Nagata S， Sasaki T’： Usefulness of con－
structive interference in steady－state magnetic
resonance imaging in the presurgical examination
for lumbosacral lipoma． J Neurosurg 103（6 Suppl）：
537－543， 2005
Hashiguchi K， Morioka T， Samura K， Yoshida F，
Miyagi Y， Nagata S， Kokubo T， Yoshiura T， Sasaki T：
Holocord hydrosyringomyelia with terminal my－
elocystocele revealed by constructive interference in
steady－state MR imaging． Pediatr Neurosurg 44：
509－512， 2008
Hashiguchi K， Morioka T， Yoshida F， Miyagi Y， Mi－
hara F， Yoshiura T， Nagata S， Sasaki T： Feasibility
and limitation of constructive interference in steady－
state （CISS） MR imaging in neonates with lum一
bosacral myeloschisis． Neuroradiology 49： 579－585，
2007
20） Herman JM， McLone DG， Storrs BB， Dauser RC：
Analysis of 15 patients with myelomeningocele or
spinal lipoma reoperated upon for tethered cord．
Presentation， management and outcome． Pediatr
Neurosurg 19： 243－249， 1993
21） Hudgins RI， Gilreath CL： Tethered spinal cord fol－
lowing repair of myelomeningocele． Neurosurg Focus
16（2）： E7， 2004
22） lannelli A， Rea G， Di Rocco C： CSF shunt removal in
children with hydrocephalus． Acta Neurochir （Wien）
147： 503－507， 2005
23） Kawamura T， Morioka T， Nishio S， Fukui M：
［Thoracic split cord malformation with lumbosacral
myeloschisis． A case report］． No Shinkei Geka Sokuho
10： 701－704， 2000 （Japanese）
24） Kawamura T， Morioka T， Nishio S， Mihara F， Fukui
M： Cerebral abnormalities in lumbosacral neural
tube closure defect： MR imaging evaluation． Childs
Nerv Syst 17： 405－410， 2001
25） Kawamura T， Nishio S， Morioka T， Fukui K： Callosal
anomalies in patients with spinal dysraphism： CQrre－
lat’ion of clinical and neuroimaging features with
hemispheric abnormalities． Neurol Res 24： 463－467，
2002
26） Kitazawa T， lwasawa M， Shinohara H： Extended
latissimus dorsi musculocutaneous flap for closure of
a meningomyelocele in a 5－hour－old neonate． Ann
PIast Surg 47：’ 215－216， 2001
27） Lapid O， Rosenberg L， Cohen A： Meningomyelocele・
reconstruction with bilobed flaps． Br J Plast Surg 54：
570－572， 2001
28） Lee GY， Paradiso G， Tator CH， Gentili F， Massicotte
EM， Fehlings MG： Surgical management of tethered
cord syndrome in adults： indications， techniques，
and long－term outcomes in 60 patients． J Neurosurg 4：
123－131， 2006
29） Lewis D， Tolosa JE， Kaufmann M， Goodman M， Far－
rell C， Berghella V： Elective cesarean delivery and
long－term motor function or ambulation status in in－
fants with meningomyelocele． Obstet Gynecol 103：
469－473， 2004
30） Marlin AE： Management of hydrocephalus in the
patients with myelomeningocele： an argument
against third ventriculostomy． Neurosurg Focus 16（2）：
E4， 2004
31） McLone DG： Technique for closure of myelomenin－
gocele． Childs Brain 6： 65－73， 1980
32） McLone DG； Continuing concepts in the manage－
ment of spina bifida． Pediatr Neurosurg 18： 254－256，
1992
33） McLone DG， Dias MS： Normal and abnormal early
development of the nervous system， in Cheek WR
（ed｝： Pediatric Neurosurgery： Surgery of the Develop－
ing Nervous Sys施m， ed 3． Philadelphia， WB Saun－
ders， 1994， pp 3－39
34） Miller PD， Pollack IF， Pang D， Albright AL： Compari－
son of simultaneous versus delayed ven一
Neurol Med Chir （Tokyo） 50， Septem ber， 2010
Presented by Medical*Online
876 T． Morioka et al．
35）
36）
37）
38）
39）
40｝
41）
42）
43）
44）
45）
46）
47）
triculoperitoneal shunt insertion in children under－
going myelomeningocele repair． J Child Neurol 11：
370－372， 1996
Miyagi Y， Morioka T， Yoshida F， Hashiguchi K， ln－
oue D， Mizoguchi M， Shono T， Nagata S， Sasaki T：
Unusual MR presentation of occult spinal dys－
raphism with lumbosacral lipoma and dermoid cyst：
A case report． Childs Nerv Syst 23： 109－112， 2007
Morioka T， Hashiguchi K， Kawamura T， Mihara F，
Hikino S， Nagata H， lwaki T， Sasaki T： ［Fetal magnet－
ic resonance imaging in prenatal diagnosis of central
nervous system abnormalities］． Shoni No No－Shinkei
30： 17－24， 2005 Uapanese）
Morioka T， Hashiguchi K， Nagata S， Miyagi Y， Mi－
hara F， Hikino S， Tsukimori K， Sasaki T： Fetal germi－
nal matrix and intraventricular hemorrhage． Pediatr
Neurosurg 42： 354－361， 2006
Morioka T， Hashiguchi K， Samura K， Yoshida F，
Miyagi Y， Yoshiura T， Suzuki SO， Sasaki T： Detailed
anatomy of intracranial venous anomalies associated
with atretic parietal cephaloceles revealed by high－
resolution 3D－CISS and high－field T2－weighted
reversed MR images． Childs Nerv Syst 25： 309－315，
2009
Morioka T， Hashiguchi K， Yoshida F， Matsumoto K，
Miyagi Y， Nagata S， Yoshiura T， Matsumoto K，
Taguchi T， Sasaki T： Neurosurgical management of
occult spinal dysraphism associated with OEIS com－
plex． Childs Nerv Syst 24： 723－729， 2008
Morioka T， Hashiguchi K， Yoshida F， Nagata S，
Miyagi Y， Mihara F， Sasaki T：Dyna血ic morphologi・
cal changes in lumbosacral lipoma during the first
months of life revealed by constructive interference
in steady－state （CISS） MR imaging． Childs Nerv Syst
23： 415－420， 2007
Munro IR， Neu BR， Humphreys RP， Lauritzen CG：
Limberg－latissimus dorsi myocutaneous flap for
closure of myelomeningocele． Chjlds Brain 10：
381－386， 1983
0i S， Honda Y， Hidaka M， Sato O， Matsumoto S： ln－
trauterine high－resolution magnetic resonance imag－
ing in fetal hydrocephalus and prenatal estimation of
postnatal outcomes with “perspective classifica－
tion．” J Neurosurg 88： 685－694， 1998
Pollack IF， Pang D， Albright AL， Krieger D： Outcome
following hindbrain decompression of symptomatic
Chiari malformations in children previously treated
with myelomeningocele closure and shunts． J Neu－
rosurg 77： 881－888， 1992
Reiges DH， Dallmann DE， Scarff TB， Woodford J： ln－
tra－operative evoked potential studies of newborn in－
fants with myelomeningocele． Dev Med Child Neurol
Suppl 37： 42－49， 1976
Rekate HL： To shunt or not to shunt： hydrocephalus
and dysraphism． Clin Neurosurg 32： 593－607， 1985
Rekate HL： Management of hydrocephalus and the
erroneous concept of shunt independence in spina
bifida patients． BNI Q 4： 17－20， 1988
Samura K， Morioka T， Hashiguchi K， Yoshida F，
48）
49）
50）
51）
52）
53）
54）
55）
56）
57）
58）
59）
60）
Hokama M， Yamaguchi S， Nagata S， Suzuki SO，
Yoshiura T， Sasaki T： Bursal cyst （bursitis） of the coc－
cygeal region clinically mimics sacrococcygeal
meningocele， Childs Nerv Syst 24： 533－535， 2008
Samura K， Morioka T， Hashiguchi K， Yoshida F，
Miyagi Y， Yoshiura T， Suzuki SO， Sasaki T： Coexis－
tence of a human tail and congenital dermal sinus as－
sociated with lumbosacral lipoma． Chjlds Nerv Syst
25： 137－141， 2009
Samura K， Morioka T， Miyagi Y， Nagata S，
Mizoguchi M， Mihara F， Sasaki T： Surgical strategy
for multiple huge spinal extradural meningeal cysts．
Case report． J Neurosurg 107（4 Suppl）： 297－302， 2007
Sarifakioglu N， Bingul F， Terzioglu A， Ates L， Asian
G： Bilateral split latissimus dorsi V－Y flaps for closure
of large thoracolumbar meningomyelocele defects．
Br J PIast Surg 56； 303－306， 2003
Stevenson KL： Chiari Type II malformation： past，
present， and future． Neurosurg Focus 16（2）： E5， 2004
Talamonti G， D’Aliberti G， Collice M： Myelomenin－
gocele： long－term neurosurgical treatment and fol－
low－up in 202 patients， J Neurosurg 107（5 Suppl）：
368－386， 2007
Talwar’ D， Baldwin MA， Horbatt CI： Epilepsy in chil－
dren with meningomyelocele． Pediatr Neurol 13：
29－32， 1995
Teo C， lones R： Management of hydrocephalus by en－
doscopic third ventriculostomy in patients with my－
elomeningocele． Pediatr Neurosurg 25； 57－63， 1996
Thompson DN： Postnatal management and outcome
for neural tube defects including spina bifida and en－
cephaloceles． Prenatal Diagn 29： 412－419， 2009
Tokunaga S， Morioka T， Hashiguchi K， Samura K，，
Yoshida F， Miyagi Y， Yoshiura T， Yamanouchi T，
Sasaki T： Double lumbosacral lipomas of the dorsal
and filar types associated with OEIS complex． Neurol
Med Chir （Tokyo） 49： 487－490， 2009
Tulipan N， Sutton LN， Bruner JP， Cohen BM， Joh－
nso：n M， Adzick NC：The effect of intrauterin．e皿y－
elomeningocele repair on the incidence of shunt－
dependent hydrocephalus， Pediatr Neurosurg 38：
27・一33， 2004
Vandertop WP， Asai A， Hoffman HJ， Drake JM， Hum－
phrey RP， Rutka JT， Becker LE： Surgical decompres－
sion for symptomatic Chiari II malformation in ne－
onates with myelomeningocele． J Neurosurg 77：
541－544， 1992
Yamada／ S， Won DJ， Yamada SM： Pathophysiology of
tethered cord syndrome： correlation with sym－
ptomatology． Neurosurg Focus 16（2）： E6， 2004
Yoshida F， Morioka T， Hashiguchi K， Kawamura T，
Miyagi Y， Nagata S， Mihara F， lzumi H， Sasaki T：
Epilepsy in patients with spina bifida at lumbosacral
region． Neurosurg Rev 29： 327－332， 2006
Address reprint requests to： Takato Morioka， M．D．， Ph．D．，
Department of Neurosurgery， Kyushu Rosai
Hospital， 1－3－1 Kuzuharataka皿atsu， Kokura－
mina血i－ku， Kitakyushu， Fukuoka 800－0296， Japan．
e－mail： takato＠med．kyushu－u．ac．jp
Neurol Med Chir （Tokyo） 50， September， 2010
Presented by Medical*Online
