J Neurosurg 71:375-38 I, 1989

Subtorcular occipital encephaloceles

Anatomical considerations relevant to operative management

PAUl. H. CHAPMAN, M.D., BROOKE SWEARINGEN, M.D., AND

VERNE S. CAVINESS, M.D., PH.D.
Divisions of Neurosurger.v and Pediatric Neurology, Massachusetts General Hospital, Harvard Medical
School, Boston, Massachusetts

v- Three cases of occipital encephalocele, one with associated myelomeningocele, are presented. All received
preoperative evaluation with magnetic resonance imaging. Such studies provide optimal demonstration of the
cerebral and hindbrain anatomy to guide operative treatment and formulate prognosis. Review of available
radiographic, operative, and pathological information suggests that most, if not all, occipital encephaloceles
are associated with an anomaly of the hindbrain, and the usual anomaly is a rhombic roof encephalocele. In
such cases, the site of cranial herniation is caudal to the torcula, regardless of the presence or absence of
occipital lobe tissue within the sac. Experimental and clinical analysis suggests that occipital encephaloceles
most likely arise from abnormalities in the development of the skull base.

KEY WORDS 9 occipital encephalocele 9 magnetic resonance imaging

Chiari malformation 9 cerebellum 9 congenital malformation 9
subtorcular encephalocele

O
CCIPITAL encephalocele occurs at an estimated
rate of one every 3000 to 10,000 live births.
The disorder is part of a spectrum of congenital
dysraphic syndromes which includes other types of
encephaloceles as well as myelodysplasia. 2'3~ It repre-
sents roughly 8% to 15% of this group of malforma-
tions. Most encephaloceles (66% to 89%) occur in the
occipital area. The term "occipital encephalocele" refers
to a variety of anatomical configurations. The cyst may
contain cerebellum, brain stem, occipital lobes, vascular
structures, or only cerebrospinal fluid (CSF) and glial
remnants. Herniation can occur through a bone de-
fect either above or below the tentorium in an infant
who is neurologically normal or variably retarded with
multiple abnormalities. The anatomical and clinical
diversity of these anomalies has led to a number of
pathogenetic theories and has made both a systemat-
ic surgical approach and estimates of long-term prog-
nosis difficult; although it is evident from early series
that the best single prognostic indicator is the amount
and type of neural tissue present within the enceph-
alocele s a c . 8"12"z5"21"22
Recent advances in neurodiagnostic imaging allow
much greater versatility in the preoperative assessment
of these lesions than heretofore. In particular, magnetic
resonance (MR) imaging has been of benefit in defining
not only the contents of the encephalocele but also any
associated intracranial anomalies. The anatomical in-
formation provided by such images is clearly helpful in
planning one's operative approach.
Three cases illustrating these concepts are presented.
We wish to emphasize the frequency with which the
site of herniation is below the torcula, in which case
the fundamental anomaly is of posterior fossa struc-
tures. A coherent anatomical schema is proposed for
these subtorcular occipital encephaloceles.
Case Reports
Case 1
This baby was the product of a 39-week gestation,
delivered by repeat Caesarean section to a 25-year-old
mother. The pregnancy was complicated only by ma-
ternal use of phenobarbital for a seizure disorder. Birth
weight was 5 lb 10 oz, and Apgar scores were 9 and 10.
An occipital encephalocele, 5 cm in diameter, was
noted. It arose from a bone defect continuous with the
posterior aspect of the foramen magnum. The neuro-

J. Neurosurg. / Volume 71/September, 1989 375

 P. H. Chapman, B. Swearingen, and V. S. Caviness

FIG. 1. Magnetic resonance imaging in Case 1. Left: Sagittal view suggesting herniation of the occipital
lobe and cerebellar tissue into the cephalocele sac. The cleft between the structures is indicated by an arrow.
Right: Midline sagittal view showing the brain stem to be relatively undistorted, despite the presence of a
hindbrain anomaly. There is an unusually large massa intermedia (arrow).

logical examination was unremarkable. Head circum-
ference was 31.5 cm (2nd percentile). Computerized
tomography (CT) and M R studies showed that the mass
contained soft tissue as well as fluid of CSF density
(Fig. 1). The base of the encephalocele was subtorcular.
The relative contribution of cerebellum and/or occipi-
tal lobes to the hernia could not be determined, and
there was no ventricular enlargement.
At operation, the sac was found to contain tissue
from both occipital lobes, separated by a midline cleft.
A substantial volume of malformed cerebellar tissue
and choroid plexus was herniated just caudal to this
cleft (Fig. 2). Because the herniated tissue appeared
markedly distorted and gliotic, it was excised to facili-
tate repair of the encephalocele.
slightly less than that of normal neonatal cerebellum,
neither hemispheric fissures nor vermis could be iden-
tified. Symmetrical fronds of choroid plexus emerged
from the inferior surface of the cerebellum and ex-
tended over its posterior aspect on either side of the
midline. On retracting the cerebellum upward, the
fourth ventricular floor and obex could be seen. The
rostral fourth ventricle appeared to be obliterated by
fusion of the brain stem and cerebellum. There was no
discernible aqueduct. The fluid cavity of the encephal-
ocele communicated directly with the distorted fourth
ventricle. Microscopically, the inner lining of the sac
consisted of glial elements alternating with a vascular

Case 2
This 1420-gm baby girl was delivered by Caesarean
section at 34 weeks to a 31-year-old mother. The preg-
nancy was complicated by preeclampsia, and an ultra-
sound study documented a posterior encephalocele.
Apgar scores were 1 and 7. Pulse and respiration im-
proved dramatically when the infant was repositioned
to relieve pressure on the 11 x 9-cm encephalocele sac.
Head circumference was 28 cm (5th percentile). Neu-
rologically, the infant appeared to have normal brain-
stem function. An MR evaluation demonstrated a large
cystic mass containing prolapsed cerebellum as well as
the apex of a markedly kinked brain stem (Fig. 3). The
posterior fossa was small, and there was no ventricu-
lomegaly.
At operation, the base of the sac was found to be
occupied by a symmetrical mass of herniated cerebel- FIG. 2. Diagrammatic reconstruction of the anomaly in
lum and the apex of the brain-stem kink (Fig. 4). Case 1, based on the magnetic resonance and operative find-
Although the volume of this tissue appeared only ings.

376 J. Neurosurg. / Volume 71/September, 1989

Subtorcular occipital encephaloceles
FIG. 3. Case 2. Magnetic resonance image, sagittal view,
showing a markedly distorted brain stem with its apex in the
base of the cephalocele. The hernia is located below the torcula
(double arrows). Note the nubbin of cerebellar tissue (single
arrow) situated ventral to the brain stem.
stroma covered by cuboidal epithelium. Because the
soft-tissue hernia represented not only a large volume
of cerebellum but also the apex of the brain stem, no
attempt was made to resect this mass in repairing the
encephalocele.
Case 3
This baby girl was the product of a 4 l-week gestation
and was delivered vaginally. This was the third child of
FIG. 4. Diagrammatic reconstruction of the findings in
Case 2. See text for details. There was no occipital lobe tissue
within the hernia.
J. Neurosurg. / Volume 71/September, 1989
a healthy 29-year-old mother with four pregnancies.
Apgar scores were 8 and 9. Multiple congenital abnor-
malities were noted, including a large epithelialized
occipital encephalocele and a sessile myelomeningocele
at T10-L5. There was no ventricular enlargement. A
lower thoracic sensorimotor level was noted. The infant
was initially managed nonoperatively. During the 1st
year of life, her large myelomeningocele epithelialized
without complication and she remained generally
healthy. At the age of 1 year, she was admitted for
operative repair of the encephalocele, which had grad-
ually enlarged and was now almost the size of the
infant's head. Magnetic resonance imaging of the en-
cephalocele demonstrated herniation of both occipital
lobes, as well as markedly distorted cerebellar tissue
(Figs. 5 and 6).
At operation, there was wide communication be-
tween the left occipital horn and the large fluid cavity.
Just caudal to this, a separate, smaller fluid space proved
to be an eventration of the fourth ventricle with asso-
ciated remnants of cerebellum. There was no tento-
rium. Given the volume and relatively well-formed
appearance of the prolapsed occipital lobe tissue, no
attempt was made to resect it at the time of encephal-
ocele repair.
Discussion
The morphology of the malformations of brain, men-
inges, and skull associated with occipital encephaloceles
is remarkably variable in quality and degree. This prob-
ably reflects the stochastic interaction of multiple path-
ophysiological determinants. Previously published an-
atomical studies illustrate the variability of this disor-
der. Karch and Urich ~' described five postmortem
cases: all involved posterior fossa brain structures and
the hernia arose beneath the torcula. Two of the five
had no cerebellum; in the others, the cerebellum was
rudimentary and lacked a vermis. When present, the
vermis protruded into the hernia sac. In three instances,
the brain stem was also within the sac. The brain stem
was severely distorted in all five cases, usually with an
S-shaped kink. The posterior fossa was uniformly small,
with a bone defect either in the squamous portion of
the occipital bone or contiguous with the foramen
magnum. The spinal cords in all five cases were hydro-
myelic and there were cleft-like fissures extending into
the brain stem from the floor of the fourth ventricle.
Leong and Shaw 2~ reported five cases of occipital
encephaloceles with midline defects of the occipital
bone. One hernia contained only choroid plexus con-
nected to the diencephalic tela choroidea. In that case,
the only brain abnormalities were a disordered cerebel-
lar nodulus and a thin posterior medullary velum. A
second case showed herniation of the entire telenceph-
alon posterior to the rolandic fissure, with a poorly
developed brain stem and "inverted" cerebellum. In the
third case, both occipital lobes and the entire cerebellum
were included within the sac. The fourth patient had
377
 P. H. Chapman, B. Swearingen, and V. S. Caviness
FIG. 5. Magnetic resonance imaging in Case 3. Left: Sagittal view indicating prolapse of hindbrain
elements as well as of the occipital lobe into the cephalocele sac. The brain stem is relatively undistorted.
Right: Note the small size of the posterior fossa, associated with caudal displacement of the occipital and
temporal lobes.
FIG. 6. Axial magnetic resonance imaging in Case 3.
Left: A midline cerebellar cleft is identified by the arrow.
Right: At a higher level, herniation of both occipital lobes
into the sac is evident.
an atrophic cerebellum with a herniation of the fight
temporal lobe and a pedicle of mesencephalon and roof
of the diencephalon. The fifth patient exhibited multi-
ple extracranial abnormalities with a skull defect con-
tiguous with the foramen m a g n u m through which had
herniated a 374-gm microgyric brain without cerebel-
lum, connected to a split brain stem.
Caviness and Evrard 2-~' have presented a detailed
pathological analysis of two cases with occipital en-
cephaloceles, both originating from the fourth ventri-
378
cle. In one, the fourth ventriculocele was bound to an
occipital lobe encephalocele which derived from the left
lateral ventricle through a microgyric cortical defect.
The ventriculocele passed through the occipital squama
to the left of midline. Only gliotic cerebellar remnants
were present and the lower brain stem was sharply
kinked along the axis of the ventriculocele with its apex
outside the bone defect. In their second case, the hernia
arose from an expansion of the velum medullare in the
lateral recess of the fourth ventricule. The cerebellum
was small, especially on the side of the diverticulum,
with cytoarchitectural abnormalities suggesting changes
that had occurred considerably later than, and possibly
as a consequence of, the massive ventriculocele. The
occipital lobe was not involved in this case.
A subset of occipital encephaloceles includes the
"inverse cerebellum" of Padget and Lindenberg 3~ and
Smith and Huntington, 33 as well as the "tecto-cerebellar
dysraphia with occipital encephalocele" described by
Friede? Dysgenesis of the tectum and cerebellar vermis
is characteristic of both of these entities. The cerebellar
hemispheres are rotated ventrally around the pons, and
the brain stem itself is kinked and displaced posteriorly.
The fourth ventricular roof is deficient, so that the
fourth ventricle communicates with the hernia, consti-
tuting a typical ventriculocele. The brain-stem tectum
may either be absent or extend extracranially into the
encephalocele cavity. When it is deficient, the aqueduct
remains open dorsally as part of the fourth ventriculo-
cele. Other abnormalities such as aplasia of the corpus
callosum, fusion of the lobes of the thalamus, and
herniation of the occipital lobes are variably present.
Padget and coworkerts "~2~ emphasized that the cases of
inverse cerebellum h~ve structural affinities with both
the Chiari and Dandy-Walker malformations and sug-
J. Neurosurg. / Volume 71/September, 1989
Subtorcular occipital encephaloceles
FIG. 7. Diagrammatic representation of various anatomical
features of subtorcular occipital encephaloceles.
gested that they may represent an intermediate form of
these entities. Kinking of the brain stem, a small pos-
terior fossa, and hydromyelia are c o m m o n to both the
Chiari malformation and inverse cerebellum. Although
the typical caudal displacement of cerebellum seen in
Chiari malformation is not found with an inverse cer-
ebellum, ventral protrusion of the cerebellar hemi-
spheres occurs with both entities.
Both the inverse cerebellum and the Dandy-Walker
malformation exhibit agenesis of the vermis, and the
occipital encephalocele may be homologous to the pos-
terior fossa cyst. Kojima and colleagues j~ described the
CT and operative findings in two infants diagnosed as
having Dandy-Walker cysts associated with occipital
meningocele. In both instances, the posterior fossa was
large with elevation of the torcula. There was agenesis
of the vermis with direct communication between
the posterior fossa cyst, the fourth ventricle, and the ex-
tracranial meningocele. Subsequently, Fenstermaker
and colleagues: presented four cases with rhombic roof
ventriculoceles extending extracranially as occipital en-
cephaloceles. At least two of these patients had other-
wise typical Dandy-Walker anomalies with a large pos-
terior fossa cyst and elevation of the tentorium and
dural venous sinuses.
In reviewing the available literature and our own
experience, we are impressed by the frequency with
which occipital encephaloceles represent an anomaly of
posterior fossa structures (Fig. 7). This may be the case
even when there is a substantial occipital lobe compo-
nent to the hernia. Despite the morphological diversity
of such lesions, certain anatomical features can regu-
larly be identified. Whenever a hindbrain anomaly is
J. Neurosurg. / Volume 71 /September. 1989
present, herniation occurs below the torcula, regardless
of the extent to which supratentorial structures are
involved. In such cases, the fluid-filled sac communi-
cates with the fourth ventricle as a ventriculocele. When
the ventriculocele is midline, it is usually caudal to the
developing cerebellum, and variable degrees of cerebel-
lar and tectal dysplasia are the rule. In extreme in-
stances, there may be no identifiable cerebellum. If the
evagination arises from the aqueduct, posterior aque-
ductal recess, or intervening isthmic region, the degree
of deformation and reduction in size of mesencephalic
and rhombencephalic structures may be extreme. The
term "meningocele" is occasionally used to describe
encephaloceles that contain no identifiable brain tissue.
This term implies a simple herniation of meninges
without involvement of brain. In the case of occipital
encephaloceles, evidence available from postmortem
specimens as well as from MR images argues that these
are uniformly evaginations of the ventricular system of
the brain. It would appear that the term has been
invoked when the ventriculocele consists of an ex-
tremely attenuated tectal remnant and there is no brain
tissue otherwise within the hernia.
Three principal hypotheses have been offered to ac-
count for the primary morphogenetic events that lead
to the formation of occipital encephaloceles. ~-~~~-~7'~
One theory, advanced by Marin-Padilla, 2~':7 holds that
there is a mismatch between growth of the basal skull
379
 P. H. Chapman, B. Swearingen, and V. S. Caviness
(which is reduced) and of the posterior fossa neural
structures (which are initially normal). This view is
supported by substantial experimental and clinical
pathological evidence. The experimental support for
this hypothesis has been observed in hamsters. 23 Mal-
formations which closely approximate the occipital en-
cephalocele encountered in man can be induced by
administration of three different teratogens: sodium
arsenate, clofibrate, and vitamin A. -~42535 It is critical
that these substances be introduced into the pregnant
dam when the hamster embryos are at the primitive
streak stage of development. Following exposure to one
of these teratogens, there is decrease in the rate and
ultimate degree of growth of the basal bones of the
skull, in particular the basioccipital component of the
sphenoid bone (clivus). In consequence, the posterior
fossa is small. Initially, closure and subsequent regional
segmentation of the neural tube proceeds normally,
and the contour of the ventricular system is likewise
unremarkable. Eventually, growth of the brain stem
and cerebellum outstrip the capacity of the posterior
fossa. Neural structures become crowded and progres-
sively entrapped. There is resulting sharp angulation of
the cervical flexure: the neural structures are driven
upward against the overlying anlagen of the occipital
squamae, still at a membranous stage of development.
In some cases, ventricular enlargement may supervene.
When this occurs, there is progressive cystic expansion
of the aqueductal or fourth ventricular roof. If the
process is of moderate degree and the rate of expansion
is slow, allowing the integument to remain intact over
the cyst, the result is a typical occipital encephalocele.
If the conditions of expansion are more extreme and
associated with breech of the cutaneous and mesenchy-
mal covering, neuroectodermal tissue is exposed to the
amniotic fluid and dissolution of the neural elements
occurs. The resulting severest degree of experimental
malformation closely approximates anencephaly en-
countered in humans.
The Dreher mutation in mice gives rise to a complex
malformation of the central nervous system which in-
cludes occipital encephalocele, approximating closely
the encephalocele induced by teratogens in hamsters. 3~
In both instances, the neurological disorder appears to
be secondary to impaired development of basal bones
of the skull with entrapment of the mesencephalon and
rhombencephalon within a posterior fossa of reduced
size (Wahlsten and VS Caviness, unpublished data).
Importantly, correlative studies of human skulls with
anencephaly and the spectrum of posterior fossa mal-
formations (including occipital encephalocele) confirm
a consistent and substantial reduction in the size of the
basioccipital bone. 2('-2vIn other words, the osseous ab-
normalities observed in these human malformations
are essentially identical to those encountered in the
experimental animal homologues induced by adminis-
tration of teratogens and in the Dreher mutant mouse.
The second morphogenetic hypothesis relating to
encephaloceles to be mentioned here was set forth
380
cogently by Chiari ~4 in describing a range of posterior
fossa malformations which bear his name. The present
series of occipital encephaloceles best corresponds to
the Chiari malformation, type III. Chiari observed that
essentially all specimens in his series were associated
with hydrocephalus. He inferred that these deformities,
including encephaloceles, arose secondary to a hydro-
cephalic process. Subsequent experience has established
that hydrocephalus is a frequent but not invariable
accompaniment to the full range of Chiari malforma-
tions, although it is essentially invariable in the type III
group. It is reasonable to conclude that hydrocephalus
is a factor contributing to deformation with entrap-
ment, compression, and displacement of posterior fossa
structures. As considered above, however, observations
in experimental animals and mutant models of the
human malformation suggest that the hydrocephalic
process probably arises secondary to entrapment and is
not the primary mechanism of tissue distortion.
A third morphogenetic hypothesis was set forth orig-
inally by von Recklinghausen 3a to account for the spec-
trum of developmental disorders associated with rach-
ischisis. This view holds that all such anomalies reflect
a failure of neural tube closure. The evidence cited from
experimental animals and genetic models tends to re-
fute this. Neural tube closure occurs normally and
deformation is a later event.
Operative correction offers the only reasonable alter-
native for long-term management of infants with oc-
cipital encephalocele. Even with operation, the neuro-
logical morbidity rate is high. The early series, which
antedated modern shunting techniques, stressed that
the absence of neural tissue within the sac was the best
prognostic indicator and microcephaly the worst. ~'~
More recent operative series have shown little improve-
ment on these earlier reports. ~221~2~32 The operative
approach for dealing with the herniated tissue has varied
from series to series. Most surgeons resect the obviously
abnormal tissue. A limiting factor with this approach
may be the presence of functional neural elements and
important vasculature within the hernia? Another tech-
nical problem can be a small cranium and/or posterior
fossa which will not accommodate the herniated brain.
One may attempt to enlarge the posterior fossa or
decompress it by ventriculostomy in order to protect
herniated tissue, although this is usually difficult. A
conservative alternative is simply to close the soft tissues
over functional, herniated brain once the excess tissues
have been excised. Staged revision can then be carried
out at a later time as necessary. This approach was
taken in our Cases 2 and 3, and has proved quite
satisfactory.
In the past, preoperative planning has been hampered
by uncertainty regarding the contents of the sac and,
especially, the possible association of brain-stem struc-
tures with the anomaly. The images available with MR
investigations now provide a superb tool for preopera-
tive assessment. Another diagnostic tool relevant to
these lesions is fetal ultrasonography. 13~4 This allows
J. Neurosurg. / Volume 71/September, 1989
Subtorcular occipital encephaloceles

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J. Neurosurg. / Volume 71/September, 1989 381