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A Morphological Classification of Sincipital Encephalomeningoceles

This document describes a morphological classification of sincipital encephalomeningoceles based on postmortem dissection findings from 12 patients. The classification divides the lesions into 3 main groups based on the location of the skull defect: 1) Fronto-ethmoidal encephalomeningocele, 2) Interfrontal encephalomeningocele, and 3) Craniofacial cleft. The fronto-ethmoidal group is further divided into 3 subgroups based on the location of the facial mass: nasofrontal, naso-ethmoidal, and naso-orbital. The classification aims to be pragmatic and useful for surgical management.
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0% found this document useful (0 votes)
59 views11 pages

A Morphological Classification of Sincipital Encephalomeningoceles

This document describes a morphological classification of sincipital encephalomeningoceles based on postmortem dissection findings from 12 patients. The classification divides the lesions into 3 main groups based on the location of the skull defect: 1) Fronto-ethmoidal encephalomeningocele, 2) Interfrontal encephalomeningocele, and 3) Craniofacial cleft. The fronto-ethmoidal group is further divided into 3 subgroups based on the location of the facial mass: nasofrontal, naso-ethmoidal, and naso-orbital. The classification aims to be pragmatic and useful for surgical management.
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© © All Rights Reserved
We take content rights seriously. If you suspect this is your content, claim it here.
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Download as PDF, TXT or read online on Scribd
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A morphological classification of

sincipital encephalomeningoceles

CHABAS SUWANWELA,M.D., AND NITAYA SUWANWELA,M.D.


Section of Neurological Surgery, Department o/Surgery, Chulalongkorn Hospital
and Faculty of Medicine Chulalongkorn University, Bangkok, Thailand

Cranial defects and cerebral abnormalities as revealed by postmortem dis-


section in 12 patients with sincipital encephalomeningocele are reported. The
various methods of classifying this lesion are discussed. A classification based
on the location of the defect in the cranium is outlined. The clinical application
of such a classification and its usefulness in the surgical management are em-
phasized.

KeY WoRDs sincipital encephalomeningocele

M ENINGOCELESin the anterior part of 2. Inteffrontal encephalomeningocele


the head are rare in Western Europe (Case 11 )
and America but appear to be com- 3. Craniofacial cleft (Case 12).
mon in Southeast Asia. We have found 100
cases in the past 8 years and have reported the Fronto-ethmoidal Encephalomeningoceles
clinical manifestations and treatment else- In 10 patients, the opening of the defect in
where? m~ We have done postmortem dissec- the skull as seen intracranially was located at
tion on 12 patients with the herniation in the the junction between the frontal and ethmoi-
region from the forehead to the nose; our find- dal bones. Nine had a single opening in the
ings, we believe, throw some light on the un- midline corresponding to the site of the fora-
settled classification of such lesions. This men cecum. The crista gaUi was situated at
communication is an attempt to make a prag- the posteroinferior rim of the defect. One
matic and useful classification based on the patient (Case 10) had two openings at the
location of the defect in the cranium. anterior end of the cribriform plate, one on
The results of postmortem dissection in each side. The crista galli was at the poste-
12 patients, indicating the site of the facial rior part of the bridge of the bone between
mass and the skull defect, are shown in Fig. the two openings. The patients in this group
1, and the findings in the patients are tabu- can be divided into three subgroups accord-
lated in Table 1. Based on the location of the ing to the site of the facial end of the orifice
defect in the skull, we have divided them (Fig. 2).
into three groups, as follows:
1. Fronto-ethmoidal encephalomeningocele Nasolrontal Type (Cases 1 through 6)
A. Nasofrontal (Cases 1-6) The skull defect as seen from the facial
B. Naso-ethmoidal (Cases 7-9) aspect was almost identical in these six pa-
C. Naso-orbital (Case 10) tients; it was round or ovoid at the bregmatic

I. Neurosurg. / Volume 36 / February, 1972 201


Charas Suwanwela and Nitaya Suwanwela
region between the deformed orbits (Fig. 3). when seen from the intracranial side and be-
The crista gaUi projected into the defect tween the frontal and nasal bones as seen
from its inferior rim. The anterior portion of from the outside. The two orifices were,
the medial orbital wall was displaced later- however, very close together.
ally. The ethmoidal bone forming the middle All patients in this subgroup had a mass
portion of the floor of the anterior cranial at the glabella or at the root of the nose
fossa was low in position compared with the (Fig. 4), the size and character of which
roof of the orbits. When seen intracranially, varied greatly. The size ranged from 2 cm in
the anterior cranial fossa was unusually deep diameter to one larger than the child's head.
in the middle. The nasal bones and the fron- Two patients had no skin covering and brain
tal processes of the maxillary bone, as well tissue presented in the form of two nodular
as the nasal cartilage, were in normal rela- masses separated by a median constriction,
tionship. The cranial defect was therefore corresponding to both frontal tips and the in-
between the frontal and ethmoidal bones tervening falx cerebri. The mass in the other

1 2 3 4 5 6

7 8 9
0Go10 11 12
FIG. I. Drawings to illustrate the findings in all 12 patients. The appearance and location of the mass
are shown in the upper row, the defects of the bones of the face in the middle row. The intracranial open-
ings in Cases 1 through 10 and the defects of the cranial vault in Cases 11 and 12 are shown in the lower
row,

202 1. Neurosurg. / Volume 36 / February, 1972


Sincipital encephalomeningoceles
TABLE 1
Details of findings on dissection of 12 encephalomeningoceles
Age Location Skin Covering Location & Size Amount of
Case at Sex of Size of & Wall of (cnt) of Herniated Brain
No. Death Mass Mass (cm) Sac Cranial Defect* Brain

8 days M glabella 4 X3 X2 bleeding, no skin FE 2X2.5 large holotelencephaly, micro-


covering, thin NF gyria & agyria
wall of sac

1 mo. M glabella =x2x5 thin & shiny skin FE 2.5X2.5 small (2 X2 X3 small frontal lobes, long
thin wall of sac NF cm) quaddgeminal plate &
aqueduct

1 mo. F l glabella 4 X3 X2 no skin covering, FE 2 X2 large left unilateral hydro-


infected, thin wall NF cephalus, elongated
of sac brain stem & hypo-
thalamus
I
prem. F glabella 4 X3 X2 no skin covering, F E 2 X2 moderate small frontal lobes, trac-
newbn. bleeding, no sac NF tion of cortex & anterior
wall communicating artery
forward

2 mos. F glabella 5X3X2 ulcerated, infec- FE 3X3 moderate right unilateral hydro-
ted, thin wall of NF (3 X4 X4 cm) cephalus, reverse direc-
sac tion of optic nerve &
internal carotid artery,
long brain stem, abnor-
mal position of falx
cerebri

2 mos, M glabella lOXl0Xl5 thin&shiny skin, FE 4 X2 about one-half fairly well preserved
(neck 5 X2) thin wall of sac NF cortical gyri, stretched
cortex & brain stem
I.
3 inos. F wide base of 0.5XI X l . 5 thin skin over FE 2 X2 small (1 X2X2 bilateral hydrocephalus,
nose with mass, shiny at NE cm) elongated brain stem
I right mass cyst

2 yrs F both sides of 2XI XI thin skin over FE 2 X3 moderate right unilateral hydro-
base of nose mass, shiny at NE (2 X2 X4 cm) cephalus, elongated
cysts brain stem, abnormal
position of falx cerebri

9 1 89yrs. M both sides of 4 X3 X2 normal Skin with FE 2 X4 moderate bilateral hydrocephalus,


wide base of scar, thin wall of NE (2 X4 X4 cm) elongated brain stem
nose sac

10 1 too. M both infra- 2 X I X0.5 normal skin, thick FE; NO minimal normal
orbital 1.5 XI XO.5 wall of sac O. 7 X0.7 left (olfactory bulb)
0.4X0.2 right
II 8 days F midforehead 7 X5 X2 normal skin, thick interfrontal moderate normal cortex, con-
wall of sac 6X7 (2 X6 X6 era) stdcted ring at frontal
lobes

12 newbn. F whole head 15XIOX8 thin & shiny, righl craniofacial more than half severe damage, partly
5X4X3 normal, left 5X6 macerated, two constric-
tions at cerebrum

* FE = fronto-ethmoidal, NF = nasofrontal, NE = naso-ethmoidal, NO = naso-orbital

two patients had a thin skin covering which from the size of a fingertip to almost half of
had ruptured and become infected. The last the brain (Fig. 6). When a large amount of
two patients had cystic masses covered with brain was herniated, the cortical gyri and
thin and shiny skin. sulci converged at the neck of the opening.
Upon dissection of the brain, one patient Certain areas of cortex were smooth with
(Case 1) had a single cerebrum without a faint or no convolutions. The cerebral blood
falx cerebri or corpus callosum, that is, a ho- vessels also followed the brain into the herni-
lotelencephaly (Fig. 5). The remaining five ation. In one patient, the anterior communi-
patients had herniation of both frontal tips cating artery was displaced to the level of the
and the intervening falx cerebri into the sac. crista galli (Fig. 7). The olfactory apparatus
The amount of the herniated brain varied was almost invariably herniated and the ol-

1. Neurosurg. / Volume 36 / February, 1972 203


Charas Suwanwela and Nitaya Suwanwela
[actory tract stretched. The brain stem was
affected to a variable degree. I n some, there
was an elongation of the quadrigeminal plate
and angulation of the aqueduct, which could
very well be the cause of hydrocephalus. In
others, the brain stem and hypothalamu~
were elongated to unrecognizable structures.
In severe cases, the optic nerves, after pass-
ing out of the optic canal, bent sharply for-
ward and took a forward course to the infe-
rior surface of the brain as far as 1 cm away.
The internal carotid arteries followed the
same course as the optic nerves. In one pa-
tient, the tip of the temporal lobe was seen to
herniate above the sphenoid ridge into the
anterior cranial fossa. The frontal tips were
not equally herniated, and there was some

FIG. 3. Case 1. Nasofrontal subgroup of fronto-


ethmoidal encephalomeningocele showing the de-
fect between the frontal bone on one side and the
ethmoidal and nasal bones on the other. The crista
galli is seen protruding into the defect. Note the
low position of the cribriform plates in relation to
the roof of the orbits. This patient is Case 5 in our
previous report. ~

Ft6. 2. Drawings to illustrate the location of the


bone defect in the three subgroups of the fronto-
ethmoidal encephalomeningocele. The anterior as-
pect and midsagittal plane of the cranium together
with the nasal cartilage are shown: A. Normal.
B. Nasofrontal. C. Naso-ethmoidal. D. Naso-
orbital. The medial wall of the orbit instead of the
midsagittal plane is shown in D. E = ethmoidal
bone, F = frontal bone, M = maxillary bone Fie. 4. Case 4. Nasofrontal subgroup of the
(shaded area), N = nasal bone (striped area), fronto-ethmoidal encephalomeningocele showing
nc = nasal cartilage, and S = sphenoid bone. the typical location of the mass in this subgroup.

204 I. Neurosurg. / Volume 36 / February, 1972


Sincipital encephalomeningoceles

FIG. 5. Case 1. Superior view (left) and coronal section (right) of the brain in an 8-day-old boy with a
nasofrontal encephalomeningocele showing holotelencephaly.

rotation of the brain. Unilateral hydrocepha- ally seen on top of the ethmoidal bone in the
lus was seen in two of these five patients. depths of the hole. The lateral wall, which at
The herniated portion of the brain under- times was membranous instead of bony, was
went variable degrees of change, from one formed by the medial wall of the orbit (Fig.
that was rather well preserved to one that 8).
had become a firm nodular mass of adherent In this subgroup, the neck of the encepha-
scar. lomeningocele was long. Its intracranial ori-
In one patient (Case 5) the falx cerebri fice was between the frontal and ethmoidal
attached to the tentorium cerebelli along the bones, and its outer end lay between the na-
left apex of the petrous pyramid instead of sal bones and the nasal cartilage. The dis-
the median portion. The right cerebral hemi- tance between the nasal bones and the nasal
sphere, which was hydrocephalic, was larger
than the left one and occupied the right half
as well as the posterior portion of the left
half of the cranial cavity. The superior sagit-
tal sinus also deviated to the left and lay
close to the point of attachment of the falx
cerebri.
Naso-ethmoidaI Type (Cases 7 through 9)
The cranial defect in three patients was
between the nasal bones and the nasal carti-
lage as seen from the face. The nasal bones
and a portion of the frontal process of the
maxillary bone were attached to the frontal
bone above the sac of the encephalomenin-
gocele, forming the anterosuperior wall of
the canal for the sac. The posteroinferior Fro. 6. Case 6. Brain showing constriction in the
middle corresponding to the neck of a nasofrontal
wall was formed by the nasal cartilage and encephalomeningocele. About one-half of the brain
nasal septum which attached superiorly to was outside the cranial cavity. Note the rather well-
the ethmoidal bone. The crista galli was usu- formed cortical gyri.

1. Neurosurg. / Volume 36 / February, 1972 205


Charas Suwanwela and Nitaya Suwanwela
group A. All three had a moderate degree of
hydrocephalus. In one (Case 8), the falx
cerebri was attached along the apex of the
left petrous pyramid (similar to Case 5). In
another, there was agenesis of the corpus
callosum, which had been replaced by an in-
terhemispheric cyst, measuring 6 X 3 X 1.5
cm, containing cerebrospinal fluid.

Naso-orbital Type (Case 10)


This patient had two masses, one on each
side, at the nasolabial fold between the nose
and lower eyelids. They were rather finn
with normal skin covering. Upon dissection
of the face, thick-walled cystic masses were
found, at the depth of which small nodules
of the herniated brain were located (Fig.
11). The frontal and nasal bones and carti-
lage were in normal relationship. The frontal
process of the maxillary bone was defective
in the middle, forming the anterior limits of
Fro. 7. Case 4. Undersurface of the brain showing
the herniated front tip. Note the elongation of the
anterior cerebral artery and the far-forward dis-
placement of the anterior communicating artery.
Smooth cortical surface is also seen.

cartilage varied. When they were in close


proximity, the sac of the encephalomeningo-
cele was localized within the widened bridge
of nose. In one patient, it extended laterally
and the lesion almost resembled the naso-
orbital type to be described.
The mass in these three patients was at
the root of the nose extending to the inner
canthus of the eyes. One of them had cystic
swellings on both sides of the root of the
nose (Fig. 9). Another patient had diffuse
swelling of the bridge of the nose, with nor-
mal skin covering. The mass was rather firm
in the middle and soft on both sides. In the
third patient, a shiny cystic mass was present
to the right of the midline on top of the wide
bridge of the nose.
On dissection of the face, it was found
that the cystic swellings and the soft areas
corresponded to the herniated frontal tips FIG. 8. Case 7. Naso-ethmoidal subgroup of
and an overlying space containing cerebro- fronto-ethmoidal encephalomeningoceles. Postmor-
spinal fluid. In the midline, there was a thick tem dissection showing the defect in the facial
fibrous septum which was a prolongation of bones. The nasal bones and the frontal processes of
the falx cerebri (Fig. 10). the maxillary bone are attached to the frontal bone
above the defect. The nasal cartilage adjoins the
The brains of these patients showed ethmoidal bone below the defect. The lateral wall
changes similar to those described in Sub- of the defect, being membranous, was removed.

206 J. Neurosurg. / Volume 36 / February, 1972


Sincipital encephalomeningoceles
inferior margin, a 1 cm metopic suture was
seen extending downward to join the fronto-
nasal suture. Posteriorly the defect merged
into the anterior fontanel. The sagittal and
coronal sutures were in normal positions.
The rest of the skull showed the so-called
"beaten-silver" appearance. Both of the
frontal lobes extended into and filled the
frontal swelling. A constriction ring could be
seen on the cerebral cortex corresponding to
the rim of the bone defect. The ventricles
were slightly enlarged but the brain was oth-
erwise normal.
Craniofacial Cleft
We have seen two Fatients with this mal-
formation of the head and face. One of them
(Case 12) was studied by postmortem dis-
FIG. 9. Case 9. Naso ethmoidal subgroup of section. The child had a small skull with a
fronto-ethmoidal encephalomeningocele showing big hole on the top. Two large cystic masses
the typical location of the mass or masses.
extended up from the area of the bone de-
fect. A complete cleft lip and palate was seen
on the left. The right nostril was displaced
the external orifice. The lacrimal bone and
rostrally and was situated close to the base
the lamina papyracea of the ethmoid consti-
tuted the posterior edge of the external ori-
fice. Intracranially, two holes were seen at
the foremost end of the cribriform plate just
in front of the crista galli. They measured
about 6 mm on the right and 4 mm on the
left. In this patient, the canal forming the
neck of the encephalomeningocele was very
long. The brain was globular in shape and
was within normal limits except for the her-
niation of the tips of both olfactory bulbs
and also, on the left side, the surrounding or-
bital cortex.
Interfrontal Encephalomeningocele
One patient (Case 11) was born with
multiple anomalies: globular swelling at the
midforehead, lumbosacral myelomeningo-
cele, flexion deformity of the metacarpopha-
langeal joints of both hands, claw toes, small
and deformed ears, and interatrial septal de-
fect (Fig. 12).
The mass at his forehead, which was
about one-fourth the size of the child's head,
was soft, cystic, and did not transilluminate.
It extended from the nasion to the anterior
fontanel. Bone edge with everting margins
was palpable at the base of the mass. The
FIG. 10. Case 9. Postmortem dissection showing
posterior fontanel was closed. The ovoid de- herniation of the tips of both frontal lobes through
fect in the skull was bounded on both sides the defect. The fibrous band in the middle is the
and anteriorly by frontal bones. At its antero- herniated falx cerebri.

!. Neurosurg. / Volume 36 / February, 1972 207


Charas Suwanwela and Nitaya Suwanwela

of the right cyst between the widely sepa-


rated eyes (Fig. 13).
Upon dissection of the skull, a large defect
was found extending from the ethmoidal to
the occipital bones. It was bounded by the
frontal and parietal bones on both sides. The
right orbit was smaller than the left; they
were far apart, separated by the rostraUy dis-
placed nasal structures. There was a cartilag-
inous spicule resembling a crista galli be-
tween the two nasal openings. Less than half
of the brain was within the cranial cavity.
The majority of the cerebral cortex was her-
niated into the left mass, from which a sec-
ondary hern;ation extended into the larger
right mass. The left mass was largely filled
with brain tissue, while that on the right one
was mostly cystic. The portion of the brain
tissue in the right mass was partially macer-
ated.
Fro. 12. Case 11. Interfrontal encephalomeningo-
Discussion cele in an 8-day-old girl who also had a lumbo-
sacral myelomeningocele, flexion deformity of the
Congenital encephalomeningocele is an metacarpophalangeal joints of both hands, claw
extracranial protrusion of the cerebral tissue toes, and an interatrial septal defect.
and meninges through a congenital defect in
the cranial bones. Classification is unsatis- cal, sincipital, basal, nasal, orbital, etc., is
factory based on the contents of the cranial useful in the initial description of the pa-
meningocele, encephalomeningocele, and tients. 2,',~,1~176 In certain areas,
encephalocystocele, whether they contain however, a single term is used with dif-
pure meninges, meninges and brain, or part ferent meanings, and the different groups
of the ventricle, because they can be classi- are often not clearly defined. The most use-
fied only after a detailed microscopic exami- ful classification appears to be based on the
nation. Classification based on the location location of the defect in the cranial bones.
of the mass, such as occipital, parietal, verti- Here, confusion still exists when one is deal-
ing with the lesion in the anterior and basal
part of the head, which is rare in western
countries.
According to Mustakallio, 29 Mesterton in
1855 classified the 19 sincipital hernias of
the brain collected by him into nasofrontal,
naso-ethmoidal, and naso-orbital subgroups,
according to the location of the external ori-
fice of the skull defect. In 1890, von Meyer ~-~
reported a case and published the same clas-
sification, which has since been widely
quoted2 ,~2,1~,19,-~7 In 1903, Stadfeldt, quoted
by Strandberg, 37 called all three forms of sin-
cipital encephalomeningocele by the general
name "fronto-ethmoidal" and gave the
following reason: "because it is generally
characteristic of all these groups that there is
Fro. I 1. Case 10. Naso-orbital subgroup of fronto- inside the cranium an internal orifice of the
ethmoidal encephalomeningocele. Postmortem dis-
section showing the thick-walled sacs. This patient hernia distinguishable between the frontal
is Case 11 in our previous reportY bone and the ethmoid."

208 1. Neurosurg. / Volume 36 / February, 1972


Sincipital encephalomeningoceles
former three subgroups. The sphenomaxil-
lary subgroup with a mass in the spheno-
maxillary fossa is a theoretical one, and we
have found no report of such an occurrence.
The encephalomeningocele in the posterior
part of the orbit with a defect in the sphe-
noid wings and orbital plate of the frontal
bone cannot, however, be placed in any of
the subgroups. T M It may be called a fronto-
sphenoidal or spheno-orbital subgroup.
Patients have been reported with more ex-
tensive herniation through a cleft involving
the anterior two-thirds of the cranial vault
and the upper f a c e , :~,4~ a s well as patients
with a combination of basal encephalome-
ningocele and cleft palate, cleft lip, or bifid
FiG. 13. Case 12. Anterior-cranial upper-facial nose. 17,2:~ An extensive occipitocervical de-
cleft. This newborn boy had two large cystic masses
overlying a large skull defect. He also had a com- fect constitutes the occipital counterpart of
plete cleft lip and palate on the left side. the craniofacial cleft. Acrania and anenceph-
aly can be considered to be the most ex-
Since that time, many reports on the sub- treme malformations in the same line.
ject have appeared, but the nomenclature A classification of encephalomeningoceles
and classification are still not uniform. The bared on the location of the defect in the
type characterized by a defect between the cranium is summarized below:
frontal and nasal bones with a mass at the
glabellar region (our Group 1, Subgroup A) Classification of Encephalomeningoceles
is called nasofrontal by most authors. 9,27,~5 It I. Occipital encephalomeningocele
has been also called frontonasal, 22 glabellar, 22 II. Encephalomeningocele of the cranial
nasal, 2~ and rhinoencephalocele? The de- vault
fect in Group 1, Subgroup B, has been called A. Interfrontal
naso-ethmoidal, 1,9,1~ frontonasal, ~1 as well B. Anterior fontanel
as a more general term such as nasal, ante- C. Interparietal
rior, or sincipital. ~6,29 Naso-orbital, 32 ante- D. Posterior fontanel
rior orbital, G,11 and nasolacrimal 3~ have been E. Temporal
used for Subgroup C. III. Fronto-ethmoidal encephalomeningocele
The interfrontal type, as in our Case 1 1 A. Nasofrontal
has been reported in a small number of pa- B. Naso-ethmoidal
tients. 2~,a4,38 The basal encephalomeningo- C. Naso-orbital
cele is rare and an intranasal mass from her- IV. Basal encephalomeningocele
niation through a defect in the cribriform A. Transethmoidal
plate is the most common form? ,a2,~*,x~,z~ B. Spheno-ethmoidal
Fenger 12 in 1895 quoted Heinecke (1882) C. Transsphenoidal
as the originator of its classification into D. Frontosphenoidal or spheno-orbital
sphenopharyngeal, spheno-orbital, and V. Cranioschisis
sphenomaxillary, depending on the tract of A. Cranial--upper facial cleft
the herniation. Transethmoidal, ~9 and intra- B. Basal---lower facial cleft
nasal 9,1~ were later added. Blumerffeld~ in C. Occipitocervical cleft
1965 suggested another classification in ac- D. Acrania and anencephaly.
cordance with the site where the herniation
leaves the cranial cavity, the subgroups being We have found this classification useful in
transethmoidal, sphenoethmoidal, trans- the selection of the operative approach in re-
sphenoidal, and sphenomaxillary. Most pairing the lesion. In the nasofrontal sub-
cases of basal encephalomeningocele re- group of the fronto-ethmoidal encephalo-
ported ~,~,8,~4,~8,~~ can be included in the meningocele, the mass usually presents ex-

1. Neurosurg. / Volume 36 / February, 1972 209


Charas Suwanwela and Nitaya Suwanwela
tracranially and the lesion has a short neck. 13. Finerman WB, Pick EI: Intranasal encephalo-
This type of lesion can therefore be repaired meningocele. Ann Otol 62:114-120, 1953
14. Fisher RG, Uihlein A, Keith HM: Spina bifida
by an extracranial approach similar to other and cranium bifida: study of 530 cases. Mayo
encephalomeningoceles of the cranial vault. Clin Proc 27:33-38, 1952
On the other hand, the sacs of the naso-eth- 15. Gisselsson L: Intranasal forms of encephalo-
moidal and naso-orbital types have long meningocele. Acta Otolar (Stockholm) 35:
519-531, 1947
necks; the distance between the internal and 16. GSrdiiren S: Orbital encephalocele associated
external orifice is considerable, and a good with acrocephaly. Brit J Ophthal 36:151-154,
dural closure at the internal orifice is not fea- 1952
sible if approached extracraniaUy. A craniot- 17. Ingraham FD, Matson DD: Spina bifida and
cranium bifidum. IV. An unusual nasopharyn-
omy with intracranial repair of the defect is geal encephalocele. New Eng J Med 228:815-
necessary even though it is a more formida- 820, 1943
ble operation; in fact more than one opera- 18. Ingraham FD, Swan H: Spina bifida and cra-
tion may be required. Plastic reconstruction nium bifidum. I. A survey of five hundred and
may be done at the same time as the extra- forty-six cases. New Eng J Med 228:559-563,
1943
cranial repair; this is not feasible in the in- 19. Josephy H: Stfrungen der Anlage (Missbil-
tracranial operation. dungen) des Gehirns, in Bumke O, Foerster O
(eds): Handbuch der Neurologie, vol 1. Berlin,
Acknowledgments Verlag Von Julius Springer, 1936, pp 6-7
20. Kahn EA, Crosby EC, Schneider RC, et al:
Completion of preparation for this paper was Correlative Neurosurgery. Springfield, Ill,
carried out at the Bowman G r a y School of Charles C Thomas, 1969, ed 2, pp 416-418
Medicine with the assistance of Courtland H. 21. Klein D: Genetic factors and classification of
Davis, Jr., M.D., John Moossy, M.D., and Ora craniofacial anomalies derived from a pertur-
Lee Payne. bation of the first branchial arch, in Longacre
JJ (ed): Craniofacial Anomalies, Pathogenesis
and Repair. Philadelphia, J B Lippincott, 1968,
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910 I. Neurosurg, / Volume 36 / February, 1972

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