Childs Nerv Syst (2011) 27:167–174

DOI 10.1007/s00381-010-1154-6

CASE REPORT

Giant occipital meningocele in an 8-year-old child

with Dandy–Walker malformation
Giuseppe Talamonti & Marco Picano &
Alberto Debernardi & Moreno Bolzon & Mario Teruzzi &
Giuseppe D’Aliberti

Received: 27 March 2010 / Accepted: 8 April 2010 / Published online: 19 May 2010
# Springer-Verlag 2010

Abstract may be reached and the cyst may become calcified.

Introduction The possibility of an association between Surgical repair may warrant favorable outcome.
Dandy–Walker malformation and occipital meningocele is
well-known. However, just an overall number of about 40 Keywords Cerebrospinal fluid shunting .
cases have been previously reported. Giant occipital Cranial dysraphism . Dandy–Walker syndrome .
meningocele has been described only in three newborns. Encephalocele . Meningocele
Incidence, pathology, clinical presentation, and proper
management of this association are still poorly defined.
Report of the case An 8-year-old boy with Dandy–Walker Introduction
malformation and giant (25 cm in diameter) occipital
meningocele is presented. This boy was born without any The Dandy–Walker malformation (DWM) is a well-known
apparent occipital mass and harbored no other significant pathological condition of the posterior cranial fossa. It
malformations including hydrocephalus. On admission, he classically consists of cerebellar vermian hypoplasia,
was neurologically intact and the giant occipital mass posterior fossa cyst communicating with the 4th ventricle,
presented partially calcified cyst walls. Treatment consisted and hydrocephalus. The DWM may be associated with a
of the excision of the occipital malformation, cranioplasty, number of other malformations: cleft palate, microphthal-
and cysto-peritoneal shunt. Outcome was excellent. mia, facial hemangioma, ceplalocele, polymicrogiria, agen-
Conclusions To the best of our knowledge, among the few esis of the corpus callosum, finger anomalies, cardiac
reported patients with Dandy–Walker malformation associ- malformations, urogenital abnormalities, and so on [1, 2,
ated to occipital meningocele, this is the oldest one and the 19]. These associations probably mean that the condition
one with the largest occipital meningocele; he is unique depends on a complex developmental disorder related to
with calcified walls of the occipital meningocele and the genetic aberrations [3, 7]. Anyway, etiology and pathogen-
only one who survived the repair of the giant malformation. esis are still not completely understood [1, 3, 9, 13, 14, 18,
In Dandy–Walker malformation, occipital meningocele may 19]. The DWM may be responsible of more or less severe
develop and grow regardless of hydrocephalus. Giant size syndromes, and the term Dandy–Walker variant has been
coined just to indicate less severe malformations [7].
G. Talamonti (*) : M. Picano : A. Debernardi : G. D’Aliberti Typically, the patients complain of hydrocephalus, and the
Department of Neurosurgery, Niguarda Ca’Granda Hospital, DWM is usually diagnosed during the assessments for the
Piazza Ospedale Maggiore 3,
enlarged ventricles [9].
20162 Milan, Italy
e-mail: giuseppe.talamonti@ospedaleniguarda.it Cephalocele is a generic term which includes both cranial
meningocele (MC) and encephalocele (EC) [7, 8]. These are
M. Bolzon : M. Teruzzi the rarest neural tube defect [5, 6, 8]. They consist of the
Department of Neurointensive Care Unit,
herniation of dura mater without or with cerebral tissue
Niguarda Ca’Granda Hospital,
Piazza Ospedale Maggiore 3, through a skull defect [8]. Usually, the overlaying skin is
20162 Milan, Italy intact. Generally, this defect is quite evident since birth.
168 Childs Nerv Syst (2011) 27:167–174

In the vast majority of cases, both the DWM and

cephalocele are recognized in infancy and often in very
young babies. The association of DWM with cephalocele
was first described by Sutton in 1887 [15] but has been
subsequently reported just in about 40 cases [1–5, 10, 11,
13, 14, 16, 18, 19]. Giant and large occipital MCs (OMC)
associated with DWM are exceptional and have been
reported only in nine newborns [2, 5, 10, 18].
In this paper, we report the first case of DWM with giant
OMC in an 8-year-old boy. This patient was quite unique
because of his advanced age, the progressive development
of the OMC, the really giant size, and the partial
ossification of the OMC, as well as the modalities and
favorable results of the surgical treatment.

Case report

History and initial examinations

Fig. 1 Computer axial tomography obtained in 2008 showing
This boy was born in 2002, in Congo, through a non- features of Dandy–Walker malformation associated with a large
occipital meningocele
complicated natural delivery. At birth, no abnormality was
described apart from macrocephaly without any neurolog-
ical complaint. During the subsequent years, the psycho- hydrocephalus and documented the further increase of the
physical development was normal; the boy complained of OMC. The posterior fossa was enlarged; the torcular and
malaria and ascaridiasis which were treated by the local the transverse sinuses were upwardly displaced and did not
doctor. When he was about 3 years old, a small occipital enter the OMC. Apart from the DWM, no other cerebral
mass was first noticed, but no specific assessment was malformations were evident. The DWM freely communi-
performed. During the following years, this mass progres- cated with the OMC through a large occipital bone defect.
sively enlarged. Notwithstanding that he remained neuro- The occipital bone appeared outward folded, the cyst walls
logically asymptomatic and continued his common daily were somewhat ossified, and there were incomplete septae
activities just hiding the mass with large hats. When the within, which gave a multiloculated aspect to the OMC sac.
malformation could be no longer covered up, the boy
encountered the hostility of his social community, and his Treatment
relatives put him under the care of a missionary. Finally, in
2008, he underwent a plain computer tomography (CT) Surgical treatment was planned in a single stage. First,
scan which revealed a DWM with large OMC (Fig. 1). In external drainage was placed inside the posterior fossa cyst
2009, he was referred to us through a non-profit organiza- and the OMC dome was decompressed. Then, a linear
tion for international cooperation. incision was performed over the occipital mass. The dura
On admission, this 8-year-old boy presented with a giant mater appeared intact at the level of both the “dome” and
occipital mass which had maximum diameter of 25 cm. The the “neck” and could be easily dissected from the bone of
skin covering the mass was normal, and palpation was the sac wall. Then, a circular craniotomy was performed all
completely painless. The mass had a sac-like shape and around, so that the dural plane was completely exposed
resulted of bony consistence close to its origin from the from its origin at the “neck” up to the “dome”. Finally, the
skull (the “neck”), while it was elastic and pulsating on dura was incised and the posterior fossa cyst entered. The
the “dome”. The neurological examination was normal with unroofed 4th ventricle resulted exposed from the aqueduct
the exception of very mild ataxia. to the central ependymal canal. The so-called “membrane”
General examination revealed systolic murmur and was excised, and wide communication was created between
protuberant abdomen with mild hepatomegaly. Blood tests the 4th ventricle and the perimedullary space. The
were normal. Ultrasonography did not reveal major abdom- redundant dura was removed, and the dural sac was
inal problems, while mild tricuspid regurgitation was shown. reconstructed by water-tight suture and fibrin glue. Finally,
A repeated CT scan (Fig. 2) and magnetic resonance a cranioplasty was performed to repair a residual skull
imaging (MRI) (Fig. 3) confirmed the DWM without defect with diameter of about 8 cm. The bone fragments,
Childs Nerv Syst (2011) 27:167–174 169

Fig. 2 a,b Computer axial tomography obtained on admission to our malformed 4th ventricle and the occipital meningocele (b). The
department (2009). The Dandy–Walker malformation was confirmed. occipital bone appeared outwardly folded, and there were calcifica-
The occipital meningocele appeared increased (a). No hydrocephalus tions on the “neck” of the meningocele
was present and a clear communication existed between the

which covered the “neck”, were assembled to form a sort of Postoperative course
cranial operculum and fixed to the skull using reabsorbable
(polylactic) microplates and screws. By this way, a normal The patient was admitted in the neurointensive care unit.
and smoothed cranial contour was obtained. The redundant The following day, he was fully alert and without
skin was removed, and the wound was normally closed in neurological focality. The postoperative course was charac-
layers. terized by untreatable vomit, sialorrhea, bradycardia,
arterial hypertension, and headache. The hemodynamic
profile normalized in a few days, but scialorrea, vomit,
and headache persisted despite adequate analgesic and
antiemetic therapy. On the one hand, severe metabolic
alkalosis had to be faced; on the other hand, ketosis and
ketonuria had to be corrected. Prolonged parenteral nutri-
tion was necessary. General examination remained normal.
On the 7th postoperative day, the external cyst drainage
was removed to prevent from infections. Subsequently,
large subcutaneous cerebrospinal fluid (CSF) collections
repeatedly formed despite serial taps. Three weeks after
OMC repair, a cysto-peritoneal shunt was placed: the
wound completely healed and vomit and sialorrhea soon
resolved. Metabolic alkalosis and ketonuria normalized. On
the 30th day, the patient was discharged without any
neurological deficit.

Follow-up
Fig. 3 Magnetic resonance imaging showing enlarged posterior fossa
containing a huge cyst freely communicating with giant occipital Three months later, the boy was neurologically intact but an
meningocele. No other cerebral malformations were evident inward depression was evident at the occipital level. The
170 Childs Nerv Syst (2011) 27:167–174

skin was intact. Mild occipital trauma was reported. A new ultrasonography [2, 6, 7], it is possible that a lot of
CT scan showed unchanged brain situation, but depressed pregnancies are terminated following the diagnosis [2, 6–8],
fracture was evident at the level of the reconstructed so that the real incidence of DWM + OMC may be
operculum. underestimated.
The patient was reoperated on, the fractured operculum Since the first description by Sutton [15] in 1887, an
was re-assembled and re-placed using titanium microplates overall number of about 40 patients have been reported [1–
and screws. Normal smoothed skull contour was again 5, 10, 11, 13, 14, 16, 18, 19]. Despite racial incidence is
reconstructed. The dural plane and the cysto-peritoneal unknown, it seems that a lot of reports are concern with
shunt were left untouched. not-Caucasian patients [2–6, 10, 11, 14, 16–19]. A
This second postoperative course was smooth, and the condition of autosomal dominant DW with occipital
patient was discharged in a few days. cephalocele (named ADDWOC) has been recently identi-
One month later, the boy came back to his country. Six fied in two families, respectively of Vietnamese and
months later, the local doctor described him as neurolog- Brazilian origins [3]. Cases of DWM + ECs have been
ically intact. A new CT scan showed that the cyst was described within the Meckel–Gruber and other genetic
unchanged, the operculum had remained in place, and the syndromes [5, 8].
head was maintaining normal contour and shape (Fig. 4). The presence of OMC, as well as the early development
of hydrocephalus, usually brings the patients to neurosur-
gical attention immediately after birth [1, 2, 5, 11, 18]. In
Discussion fact, all the reported DWM + OMC were newborns or small
babies (Table 1) [1, 2, 5, 11, 18, 19]. In most cases, the
The association between DWM and OMC OMC were relatively small or even atresic [1, 11, 14, 19].
We could find only nine patients with DWM and OMC
DWM is a complex malformation of the posterior cranial larger than 5 cm [2, 5, 10, 18], but giant occipital mass
fossa which is often associated with other malformations of (more than 9 cm) were reported just in three cases [2, 10,
the central nervous system, face, palate, eyes, heart, fingers, 18]. Anyway, in all these cases, the cysts were huge since
and so on [1, 7, 11, 13, 14]. In most cases, the prognosis of birth. Apart from a few cases [1, 16, 19], in which the
DWM is conditioned by the severity of the associated OMCs grew following shunt malfunctions, we could not
malformations [1, 7, 9, 12, 18]. find any case of progressive OMC development or
The OMC consists of an exophytic mass, which contains enlargement. Since the vast majority of patients with
cerebrospinal fluid, protrudes through a skull defect, and is DWM + OMC were operated on in early infancy, it is
usually covered by intact skin [8]. Generally, the OMC is possible that such early surgery plays a role in determining
evident since birth or even the prenatal period [2, 5, 6, 10]. both the small size and the absence of progression of the
Giant OMCs (as large as or larger than the head size) are OMCs. The progressive increase of the cephalocele was not
rare and occasional [5]. Surgical repair is considered even mentioned in paper concerning specifically with
relatively urgent due to nursing care problems and is cephaloceles [5, 6, 8].
usually performed within a few days of birth [5, 8]. Hydrocephalus and shunting were reported in almost all
In 1991, Bindal et al. [1] reviewed a personal series of patients with DWM + OCM [1, 2, 5, 11, 16, 18, 19] and
50 DWM and found eight cases associated with OMC; just a couple of patients without hydrocephalus have been
these authors found just 11 other cases from the world described [5, 14]. Indeed, there were also babies in whom
literature but calculated that this association would occur in the hydrocephalus was not initially evident but soon
16% of DWM. On the other hand, in large cephalocele developed after OMC closure [1, 16]. This confirms that
series [5, 6, 8], the presence of DWM is very uncommon. the development of hydrocephalus and OMC is indepen-
Indeed, the association DWM + cephalocele continues to be dent [14]. On the other hand, it was speculated that the
considered extremely rare [2, 7, 11, 18]. Mohanty et al. [9] OMC may compensate for the increased intracranial
found no OMC in a series of 72 DWM. Through the pressure [5, 16]. While the pathogenesis of DWM still
Medline, we were able to find just 29 cases during the last remains poorly understood [1, 13, 18, 19], the association
20 years (Table 1). In 2006, Long et al. [7] reported a DWM + OMC is even more obscure [4]. The presence of
population-based study from a British Survey (NorCAS), the OMC probably means that the DWM is not an early
which collects all major malformations in fetuses, still- embryonic mal-development, but an event occurring much
births, and live-born infants. These authors mentioned no later than the closure of the neural tube [18].
case of DWM + OMC among all the posterior fossa For what concerns the treatment, Bindal et al. [1]
malformations that occurred over a period of 18 years in a advocated shunting as the first therapeutic approach in all
three million population. Owing to the diffusion of prenatal patients with DWM + OMC. Nevertheless, in their series,
Childs Nerv Syst (2011) 27:167–174 171

Fig. 4 a,b,c Computer axial tomography obtained 6 months after surgery. The features of Dandy–Walker malformations were still evident. No
hydrocephalus developed. The cyst appeared well-drained. The cranioplasty warranted adequate skull contour

six out of eight patients required OMC repair following Therefore, most patients with DWM + OMC must undergo
shunting. During the last 20 years, shunting alone has been OMC excision and repair [1, 2, 5, 10, 18, 19].
reported effective just in four of these patients (Table 1). While relatively good results may nowadays be obtained
Anyway, OMC decrease and even disappearance are in the treatment of isolated DWM or OMC [1, 6, 8, 19], the
possible after shunt placement [1, 5, 14, 18, 19]. This association of DWM + OMC seems more dangerous.
means that one can expect the skull defect to ossify so that Indeed, in the series of Bindal et al. [1], the presence of
surgical repair of the OMC might not be necessary. On the OMC seemed to be not as important as other anomalies in
other hand, shunting may favor the development of determining the poor outcome. However, this probably
decubital ulcers where the edges of occipital bone protrude does not apply to large and giant OMC [5, 10, 18]: among
and the rate of CSF infections is high [2, 10, 11, 17, 18]. the three reported patients with giant lesions, two died after
Furthermore, a treatment consisting of only shunt may be surgery [10, 18], and in the 3rd case the outcome was not
attempted just in small babies with small cysts [18]. specified [2]; five of the six patients with large OMC died

Table 1 DWM associated with OMC in the last 20 years

No Newborns or small babies Only shunt Shunt and repair Large OMCa Giant OMCb Mortality

Nishimaki et al. [11] 1 1 – 1 – – NR

Bindal et al. [1] 8 8c 2 6 – – No
Todo et al. [18] 1 1 – 1 – 1 1
Shuto et al. [14] 1 1 1 – – – No
Yuceer et al. [19] 7 7 – 7 – – No
Kotil et al. [5] 7 7 1 6 6 – 5
Jalali et al. [3] 2 2 NR NR – – NR
Cakmak et al. [2] 1 1 NR NR – 1 NR
Muzumdar and Goel [10] 1 1 – 1 – 1 1
Total 29 29 4 22 6 3 7
a
Large OMC=5 to 9 cm
b
Giant OMC>9 cm
c
Indeed, one of these patients underwent OMC repair at age of 34 months, but all these patients were initially managed by shunt and/or repair within a few
weeks of life.
NR not reported
172 Childs Nerv Syst (2011) 27:167–174

other case of DWM with an OMC which progressively

developed after birth and even became giant. Unfortunately,
we have no precise medical report about the birth of this
child. However, if an atresic or “true” OMC had been
present since birth, this should have been so small that it
was initially not noticed. We cannot document the
progressive increase of the OMC till the age of 7 years.
However, the CT scan we obtained on admission showed a
clear volume increase of the OMC in comparison with the
CT scan performed 1 year before in Congo (Figs. 1 and 2).
Anyway, the fact this child survived in a developing
country harboring a giant OMC is per se an exceptional
fact.
In spite of a clear macrocephaly, he had no hydroceph-
alus and was neurologically intact. This has been already
reported [5, 14] but just in very few cases. The absence of
hydrocephalus confirmed that the OMC developed inde-
pendently. Anyway, the progressive cyst increase, the
enlarged posterior cranial fossa, and the presence of
outwardly bended bone at the origin of the OMC gave
the impression that the cyst had been pulled out. Maybe,
the DWM was responsible of local hypertension that
triggered the formation and the progression of the OMC
which in turn compensated for hydrocephalus. The elastic-
ity and the compliance of the OMC sac probably acted like
a vent for CSF thus preventing dangerous elevation of the
intracranial pressure [5, 10]. These mechanisms probably
started during the embryonic life, which accounts for the
macrocephaly, and continued after birth, which could
explain the progressive development of the OMC. The
presence of incomplete septae inside the cyst could have
Fig. 5 a,b Preoperative and postoperative pictures caused a “trap door” effect with consequent further
expansion of the sac [10].
The relatively advanced age and the progressive chronic
after repair and one survived a bout of meningitis [5]. Such OMC formation probably correlate with the extended
results are summarized in Table 1. It is possible that ossification of the OMC walls in our case. We have not
surgical repair triggers abrupt changes in CSF pressure and found any other case of ossified OMC associated with
dynamics at the level of the posterior fossa. The consequent DWM. Anyway, this ossification influenced our therapeutic
metabolic and vegetative storm may influence the survival approach. In our case, the bony walls of the cyst prevented
of newborns with these lesions. from any chance of OMC resolution by shunt alone and the
indication for surgical repair was sharp.
Analysis of the present case We opted for contextual temporary cyst drainage and
OMC repair. This allowed easy reconstruction. When the
The case we are reporting presented some peculiarities. drainage was removed, no hydrocephalus developed that
This patient was the oldest patient with DWM + OMC in means the OMC was no longer having significant buffering
literature [1, 2, 5, 10, 11, 14, 18, 19]. He was the first effect at this age. Indeed, an alteration of local CSF
patient with DWM and giant OMC who was not a newborn dynamics with local hypertension did exist since the
[2, 5, 10, 18]. His OMC was the largest ever reported in repeated formation of subcutaneous CSF collections and
association with DWM [2, 5, 10, 18]. the prolonged irritation of the floor of the 4th ventricle with
Unlike all the other reported cases [1, 2, 5, 10, 11, 14, untreatable vomit, sialorrhea, and bradycardia. The untreat-
18, 19], our patient was described without any evident able vomit was undoubtedly responsible for metabolic
exophytic cranial mass at birth. We do not know of any alkalosis. Meanwhile, the consequent restricted food intake,
Childs Nerv Syst (2011) 27:167–174 173

together with the stress of surgery, surely played a role in more advanced age improved his chances of favorable
prolonged ketosis. Indeed, a thoroughly complete preoper- surgical outcome.
ative evaluation of the nutritional state of this patient had
been not obtained, and we can now hypothesize that his
nutritional reserves were marginal. It is possible that faster Conclusions
postoperative recovery would have been obtained through a
more careful or “proactive” nutritional approach in the This very rare case of DWM associated with OMC
perioperative period. demonstrates that the OMC may develop irrespectively of
Eventually, we had to place a cysto-peritoneal shunt, but hydrocephalus; it may progressively grow till very large
this could be safely done 3 weeks after surgical repair thus size and may become extensively ossified; the patient may
abating the risks from shunt infection/malfunction due to survive for a number of years; the CM may be safely
CSF contamination. excised with excellent results.
During the procedure for OMC removal, we also
performed the “membrane excision” to create wide artificial
outlet from the 4th ventricle. This was the treatment of Acknowledgement The authors wish to thank Father Hugo.
choice for DWM in the past, but it has been now Unfortunately, this world hasn't got enough men like him. He is a
missionary doctor who takes care and looks after hundreds of children
supplanted by shunting owing to relatively high rates of
in Congo. When we think what he does every day, immediately, what
complications [9, 19]. We create this communication we do becomes a drop in the ocean.
between the 4th ventricle and the perimedullary spaces just
because the membrane had become exposed during the Conflict of interest The authors declare that they have no conflict of
interest.
OMC removal, and its fenestration did not appear to add
further risks. The aforementioned persisting local alteration
of CSF dynamics probably means that this maneuver was
not effective. Following OMC removal and water-tight References
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