J Neurosurg Pediatrics 5:415–418,

5:000–000, 2010

Hydrocephalus associated with cystic dilation of the

foramina of Magendie and Luschka
Case report

Hirokazu Takami, M.D.,1 Masahiro Shin, M.D.,1 Masafumi Kuroiwa, M.D.,1

Ayako Isoo, M.D.,1 Kan Takahashi, M.D., 2 and Nobuhito Saito, M.D.1
Departments of 1Neurosurgery and 2Pediatrics, University of Tokyo Hospital, Tokyo, Japan

Cystic malformations in the posterior cranial fossa result from developmental failure in the paleocerebellum
and meninges. The authors present the case of an infant with hydrocephalus associated with cystic dilation of the
foramina of Magendie and Luschka.
This 7-month-old female infant presented with sudden onset of tonic-clonic seizures. Computed tomography
revealed tetraventricular hydrocephalus. Magnetic resonance imaging demonstrated a cyst communicating with the
fourth ventricle and projecting to the cisterna magna and the cerebellopontine cisterns through the foramina of Ma-
gendie and Luschka. A suboccipital craniotomy was performed for removal of the cyst wall, and the transparent
membrane covering the foramen of Magendie was removed under a microscope. After the surgery, the patient’s
hydrocephalus improved and a phase contrast cine MR imaging study showed evidence of normal CSF flow at the
level of the third and fourth ventricles. Three weeks later, however, the hydrocephalus recurred. An endoscopic third
ventriculocisternostomy was performed to address the possibility of stagnant CSF flow in the posterior cranial fossa,
but the hydrocephalus continued. Finally the patient underwent placement of a ventriculoperitoneal shunt, resulting
in improvement of her symptoms and resolution of the hydrocephalus.
On the basis of this experience and previously published reports, the authors speculate that the cystic malforma-
tion in their patient could be classified in a continuum of persistent Blake pouch cysts. Hydrocephalus was caused
by a combination of obstruction of CSF flow at the outlets of the fourth ventricle and disequilibrium between CSF
production and absorption capacity. (DOI: 10.3171/2009.10.PEDS09179)

Key Words      •      Blake pouch cyst      •      hydrocephalus      •     

ventriculoperitoneal shunt      •      foramen of Magendie      •      foramen of Luschka

C
ystic malformations in the posterior cranial fossa
include the Dandy-Walker malformation and its
variants as well as the persistent Blake pouch cyst,
the mega cisterna magna, and the arachnoid cyst. Except
for the arachnoid cyst, those malformations can be further
classified into 2 groups on the basis of their embryological
origin: anomalies of the area membranacea anterior or the
area membranacea posterior.2,4,8,12,13,15 Dandy-Walker mal-
formation and its variants are characterized by hypoplasia
of the cerebellar vermis and cystic dilation of the fourth
ventricle, which are due to failure of assimilation of the
area membranacea anterior.2,8,12,13,15 The Blake pouch is
Abbreviations used in this paper: BPC = Blake pouch cyst; VP =
ventriculoperitoneal.
defined as transient cyst formation during the embryonic
period at the area membranacea posterior, which becomes
the tela choroidea of the fourth ventricle, before the fora-
men of Magendie opens. An imperforate foramen of Ma-
gendie and associated lack of regression of this transient
Blake pouch results in the persistence of the cystic cav-
ity, the so-called Blake pouch cyst (BPC).2,8,12,13,15 Mor-
phologically, there are distinctions in the degree of cystic
dilation communicating with the fourth ventricle between
Dandy-Walker malformation and persistent BPC. In the
Dandy-Walker malformation, there is variable hypoplasia
and elevation of cerebellar vermis. In the persistent BPC,
the cerebellar vermis and the inferior medullary velum are
fully developed, and they are compressed by the cyst in the
posterior cranial fossa.10

J Neurosurg: Pediatrics / Volume 5 / April 2010 415


In this report, we present our experience with an infant
who had hydrocephalus associated with cystic dilation of
the foramina of Magendie and Luschka. In reference to the
previous literature, we discuss the CSF dynamics and the
mechanism of hydrocephalus in our patient.
Case Report
History and Presentation. This 7-month-old female
infant, born after an uneventful pregnancy with normal
development, was admitted to our hospital after present-
ing with sudden onset of tonic-clonic seizures.
Examination and Treatment Planning. Her head cir-
cumference was within the normal range and the anterior
fontanel was flat. At examination, the baby was slightly
fretful, but there was no neurological symptom except for
disturbance of upper gaze. Computed tomography scans
revealed tetraventricular hydrocephalus, and MR images
demonstrated a cyst communicating with the fourth ven-
tricle and projecting to the cisterna magna and the cer-
ebellopontine cisterns through the foramina of Magendie
and Luschka (Figs. 1 and 2). To release the CSF obstruc-
tion, surgical removal of the cyst membrane was planned.
Endoscopic third ventriculostomy (ETV) was also consid-
ered as a treatment option. However, CSF flow dynamics
in the infantile period is not fully understood, and ETV
before the age of 1 year has been reported to have a rela-
tively low success rate.6 Also, it was apparent from preop-
erative imaging examinations that there was obstruction
at the fourth ventricle outlets. Taking these factors into
consideration, we judged that surgical removal of the cyst
membrane was the best treatment choice.
First Operation. With the patient in a prone position,
the lateral ventricle was drained through the right poste-
rior horn and a suboccipital craniotomy was performed.
After dural opening, the anatomical structures around the
foramen of Magendie were observed under a surgical mi-
croscope. The cerebellar tonsils were relatively hypoplas-
tic bilaterally, and the foramen of Magendie was widely
enlarged and sealed with a clear transparent membrane.
The membranous structure was dissected and removed at
the margin of the foramen, which was found to contain
arachnoid mater and fragments of ependymal and glial
Fig. 1.  Preoperative MR images revealing enlargement of the fora-
men of Magendie and a cyst in the medial cerebellar hemisphere.  Left:
A T1-weighted Gd-enhanced sagittal image.  Right: A T2-weighted
axial image.
416
H. Takami et al.
Fig. 2.  Preoperative 3D FIESTA (fast imaging employing steady-
state acquisition) MR images showing the membranous structures (ar-
rows) communicating with the fourth ventricle through the foramina of
Magendie (A [sagittal section] and B [coronal section]) and Luschka (C
[axial section] and D [coronal section]).
tissue. Gently retracting the cerebellar hemisphere, the
interior of the fourth ventricle was inspected. There were
no abnormal structures or cysts in the fourth ventricle,
and the aqueduct was intact.
Postoperative Course. After the surgery, the ventric­
ular drain was removed, and the ventricle size became
gradually smaller on the CT scans. Phase contrast cine MR
imaging for CSF flow, performed after the surgery, sug-
gested patent CSF flow at the level of the aqueduct and
the foramen of Magendie. The immediate postoperative
course was uneventful, and the patient was discharged 2
weeks after the surgery.
Second Admission and Examination. A week later
the patient was brought back to our hospital with repeated
projectile vomiting. No neurological deficit was found, but
the anterior fontanel was tense and bulging. New CT scans
revealed recurrence of tetraventricular hydrocephalus.
Second Operation and Postoperative Course. Con-
sidering the possibility of insufficient release of CSF flow
in the posterior cranial fossa, a third ventriculocisternos-
tomy with placement of a CSF reservoir was urgently per-
formed. A 4-mm stoma was made at the tuber cinereum
via a flexible endoscope, but expected pulsatile movement
of the ventricle floor, confirming successful replacement
of the CSF pathway, was not observed, and a ventricu-
lar catheter connected to a subcutaneous reservoir was
placed in the right lateral ventricle. Postoperatively, hy-
drocephalus was not ameliorated, and the symptoms of
intracranial hypertension continued. Daily reservoir taps
were necessary to control the symptoms.
Third Operation and Postoperative Course. Three
J Neurosurg: Pediatrics / Volume 5 / April 2010
Blake pouch cyst
Fig. 3.  Postoperative CT scans obtained after VP shunt placement
showing improvement of tetraventricular hydrocephalus.
days later, a VP shunt was placed using a programmable
pressure valve set at 7 cm H2O. Following shunt place-
ment, the infant’s clinical course was uneventful, and fol-
low-up CT scans showed improvement of hydrocephalus
(Fig. 3).
Discussion
Hydrocephalus associated with obstruction of the
fourth ventricle outlets has been reported as a rare compli-
cation after severe meningitis (when adhesions block the
flow of CSF).7,11 Congenital cystic occlusion of the fourth
ventricle outlets is extremely rare and, in the published lit-
erature, we could find only a brief description of another
type of fourth ventricle cyst associated with occipital en-
cephalocele.3 Although persistent BPC has been defined
as membranous occlusion of the foramen of Magendie in
most publications, there has been little description of the
foramina of Luschka. It was assumed that the cyst at the fo-
ramen of Magendie compresses the cerebellum in the outer
directions causing stenosis of the foramina of Luschka,
leading to the disequilibrium of CSF at the fourth ventricle
outlets. Persistent BPC is congenital cystic dilation of the
foramen of Magendie. However, if obstruction of the fo-
ramina of Luschka were evident in patients with persistent
BPC, it could be an entity of the congenital “fourth ven-
tricular outlet occlusion (FVOO).”7 During embryological
development, the foramina of Luschka open subsequent to
opening of the foramen of Magendie. We speculate that, in
our patient, in the presence of an imperforate foramen of
Magendie, cystic dilation of the foramina of Luschka was
unable to achieve perforation of these foramina, leading to
failure of regression of the Blake pouch as well as the cystic
membranes of the lateral foramina. The maturation of CSF
flow dynamics in humans is not fully understood. Accord-
ing to the findings so far, arachnoid granulations appear
in the postnatal period or just before birth and increase in
complexity as development proceeds.5 Without the mature
CSF absorption route through arachnoid granulations, it
is speculated that, until infancy, CSF dynamics is mainly
maintained by absorption via the transependymal-intersti-
tial space to the perivascular subpial space, the perineural
space to the lymphatic system, and the capillaries.9 On the
basis of studies in animal models, it is thought that dur-
ing late infancy, the major absorption of CSF occurs at the
J Neurosurg: Pediatrics / Volume 5 / April 2010
parasagittal arachnoid villi, and the route of CSF absorp-
tion shifts from the extra–arachnoid villus sites to the ma-
jor absorption pathway.14 Considering the ontogenesis of
CSF dynamics in infants, in our patient the CSF dynamics
might have reached equilibrium between CSF production
and the minor absorption pathway through extra–arach-
noid villus sites before symptoms occurred. However, as
CSF production increased, the gradual shifts in the absorp-
tion pathway were disturbed because the CSF flow was ob-
structed at the outlets of the fourth ventricle, causing the
patient’s symptoms and cystic dilation of the foramina of
Magendie and Luschka. We speculate that at the infant’s
initial presentation her seizures were caused by enlarge-
ment of the ventricular systems. It is possible that the lack
of macrocephaly at that time was because the CSF dynam-
ics might have been still partially compensated by minor
absorption pathways and those changes might not yet have
reached levels that would cause intracranial hypertension.
After release of CSF obstruction, development of absorp-
tion function through major pathways could not immedi-
ately match CSF production, and this would explain the
delayed recurrence of hydrocephalus with the symptoms of
intracranial hypertension.
Radiographic findings suggested that the pathological
condition in our patient could be on the continuum of per-
sistent BPC. Persistent BPC is a relatively rare malforma-
tion in the posterior cranial fossa, and to the best of our
knowledge, only 9 cases have been reported to date;1,2,4,15,16
involvement of anomalies of the foramina of Luschka was
not described in any of these cases, and details of surgery
were described in only 2 cases.1,4 Arai and Sato1 treated a
17-month-old girl with BPC by a combination of cyst wall
resection, cyst-peritoneal shunt placement, and VP shunt
placement. Conti et al.4 reported on a 37-year-old woman
who had syringomyelia associated with hydrocephalus and
BPC and underwent a decompressive craniectomy, a C-1
laminectomy, and opening of the cysts. The postoperative
MR images indicated a reduction of the syringomyelia,
but her neurological condition deteriorated 20 days after
the first operation because of worsening of tetraventricular
hydrocephalus, which was resolved by insertion of a VP
shunt. In a radiological investigation of CSF flow patterns,
Yildiz et al.,16 evaluated 4 cases of untreated BPC using
phase contrast cine MR imaging. According to their report,
hyperdynamic flow at the level of the third and fourth ven-
tricles (characteristic of communicating hydrocephalus)
was observed in all 4 patients, and flow was not present
either within the cyst or between the cyst and the superior–
posterior cervical subarachnoid space.
The urgency of our patient’s situation did not allow
us to perform sufficient preoperative CSF flow studies.
The phase contrast cine MR imaging after removal of the
membranous occlusion from the foramina of Magendie
and Luschka transiently showed a normal CSF flow pat-
tern. Three weeks later, however, the patient presented
with hydrocephalus that had progressed relatively slowly.
Taking those findings into consideration, we conclude that
our patient’s hydrocephalus was caused by the combina-
tion of obstruction of CSF flow at the outlets of the fourth
ventricle and disequilibrium of CSF dynamics between
production and absorption capacity.
417

The pathophysiology and the treatment for these mal-
formations is still controversial. However, we hope that
our experience will contribute to elucidating the patho-
logical mechanism and the treatment of these rare anom-
alies in the posterior cranial fossa.
Disclosure
The authors report no conflict of interest concerning the mate-
rials or methods used in this study or the findings specified in this
paper.
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Manuscript submitted April 14, 2009.
Accepted October 20, 2009.
Address correspondence to: Hirokazu Takami, M.D., Department
of Neurosurgery, University of Tokyo Hospital, 7-3-1 Hongo, Bun­
kyo-ku, Tokyo, 113-8655, Japan. email: takami-tky@umin.ac.jp.
J Neurosurg: Pediatrics / Volume 5 / April 2010