Aust.

Paediatric Journal
(1966) 2 : 20

CRANIUM BIFIDUM.
INVESTIGATION, PROGNOSIS AND MANAGEMENT*
N. BARROW1 and D. A. SIMPSOW
From the Adelaide Children’s Hospital
SYNOPSIS
The term cranium bifidum includes the encephaloceles and the cranial meningoceles.
Though rarer than spina bifida, these conditions are not uncommon, and their man-
agement can be difficult. A series of 14 patients is presented to illustrate the prob-
lems, and to show that treatment can produce good results where there are no crippling
associated cerebral dysplasias. The value of contrast radiological investigations to demon-
strate these dysplasias is emphasized. Some interesting and unexplained variations in the
geographical incidence of this condition are discussed.

Although there is widespread interest in the
surgical treatment of congenital malformations
of the central nervous system, attention has
been directed chiefly to the management of
spina bifida, and there has been little given
to the rarer conditions of cranium bifidum
and encephalocele.
Cranium bifidum is a congenital defect in
the bones of the skull, usually situated in the
midline. Almost always this defect is accom-
panied by protrusion of meninges, containing
fluid or neural tissue; the former is usually
known as a cranial meningocele, and the latter
as an encephalocele. An encephalocele is
such a gross malformation that it has always
been noted in studies of neonatal malforma-
tions. However, the therapeutic problems in-
volved have received little attention until
comparatively recently. Even today, there are
few easily available articles offering guidance
to the surgeon confronted by one of these
lesions.
CLINICAL MATERIAL
Our material consists of 14 patients who
presented at the Adelaide Children’s Hospital
during their infancy between the years 1955-
1964. Although small, the series has the merit
that the progress in all the patients has been
assessed recently.
Received on October 28, 1965.
*Supported by a grant from the Neurological Research Foun-
dation of South Australia.
1. Research Assistant.
2. Hon. Assistant Neurosurgeon.
The series consists of 10 females and 4
males. One was born of recently settled
German immigrants, 3 of aboriginal parents,
and the other 10 were born of Australian
parents, presumably of European descent.
In the period in which these patients were
seen, there were 140 patients with spina
bifida, a ratio of 1:lO.
The majority of the patients presented very
early in life, 4 were less than a day old, 5
were aged less than one month, and the re-
maining 5 were aged 2 to 7 months.
The lesions occurred predominantly in the
occipital region as shown in Table I in which
the series of Ingraham and Matson (1954)
is included for comparison.
In 5 the lesion in the occipital region was
a meningocele, one of which was associated
with a cervical spinal meningocele and a
lumbar spina bifida occulta; in each of the
others there was an encephalocele of varied
size.
TABLE I
Site of Cranium Bifxdum: 14 Cases
Ingraham
Present & Matson
Location series (1954)
~~~~ -
Occipital .................... 11 139
Parietal . . . . . . . . . . . . . . . . . . . . 1 27
Frontal .................... 2 11
Nasal and Naso-
pharyngeal ................ - 10
 ?;. EARXOW
AND D. A . SIMPSON 21

Associated external cranial abnormalities

were observed in 7 of the patients at the time
of first admission; 4 patients had severe micro-
cephaly with a large occipital encephalocele,
2 patients with an occipital meningocele had
associated clinical hydrocephalus, and one
patient with a naso-frontal encephalocele had
unilateral microphthalmia. Some details of
the clinical histories are given in Table IV.
DIFFERENTIAL DIAGNOSIS
Cranium bifidum should be differentiated
from dermoid cysts which occasionally occur
in very similar situations, and from intra- Fig. 2. Ventriculogram (Case VIII, Table IV): air
nasal ‘gliomas’ which have a notorious reputa- delineates a comparatively normal right lateral ven-
tion for simulating intranasal encephaloceles. tricle, The t e d ventricle is also filled, and air has
entered the saculav encephalocele. The left ventricle
The diagnosis can be made by delineation of is not filled, nor are the aqueduct and fourth ven-
the anterior cranial fossa by subdural air tricle visible. Some difficulty was experienced in posi-
(Jefferson and Lewtas, 1963). tioning, because of the large occipital mass, and the
s&nificance of the ventriculogram was not
In the present series air studies were per- appreciated.
formed in 9 patients, 4 of them before opera-
tion. As a rule the abnormalities were easily
diagnosed, but in one child the ventriculogram
was misinterpreted as normal, when a single
normal lateral ventricle was delineated (Figs. 1
and 2). Vertebral angiography (Fig. 3) cor-
rected this error by demonstrating a highly
abnormal posterior cerebral artery which sup-
plied the contents of an encephalocele. Of
the 5 patients who had no air studies per-
formed, 3 had gross lesions and obvious severe
microcephaly. In the remaining 2 a major
cerebral abnormality was excluded on clinical
assessment alone.
Fig. 3. Vertebral angiogram (Case VIII, Table IV):
the emephalocele receives a liberal blood supply from
the basilar artery, presumably via the left posterior
cerebra[ artery.

Ventriculograms are also important in de-

termining the presence and severity of hydro-
cephalus, a common associated finding in these
patients. Air studies confirmed the presence
of hydrocephalus in 3 of our patients, and
showed this t o be due to aqueduct stenosis in
each case.

Fig. 1. Occipital encephalocele (Case VIII, Table
IV), in an aboriginal infant aged 5 weeks. The large
globular mass wae attached immediately below the
external occipital protruberance by a narrow pedicle.
TREATMENT
Ten patients underwent operation; 6 cranial
meningoceles, and 2 encephaloceles were re-
moved from the occipital region, 2 encephalo-
celes from the frontal region.
 CRANIUM BIFIDUM-INVESTIGATION
The operative procedures were usually not
difficult for the protrusions were nearly all
pedunculated and at least partially covered
by skin. The sac was mobilized by a circum-
ferential incision at its base; it was then opened
and inspected for neuraI elements, and when
none were found, it was amputated at its base.
Neural tissue was found within the sac in
4 cases; in 3 the neural elements were ob-
viously non-functional, and degenerate, and
so were amputated with the sac; in one the
neural tissue was well formed and was there-
fore replaced within the cranium.
The defects in the bone did not need im-
mediate repair except in 2 patients with a fron-
tal encephalocele, one of which was closed
with tantalum mesh and the other with a free
bone graft. Closure of the scalp was easy
in all except one case, in which excision of a
frontal encephalocele left a large defect which
was closed with a rotation skin flap.
Four of the 10 patients operated on de-
veloped hydrocephalus of various degrees of
severity, but only one-an infant with an oc-
cipital meningocele-required operative relief.
Air studies in this patient demonstrated a
block at the aqueduct and a ventriculo-cister-
nal shunt was performed at the age of 6
months.
RESULTS
Ten of the 14 patients are alive; 6 are able
to lead normal lives and are of average, or
above average mentality; of these 3 are nor-
mal in all respects, 2 have mild arrested
hydrocephalus with a normal head circum-
ference; the sixth child has congenital uni-
lateral microphthalmia with normal vision in
the other eye. A seventh infant appears to be
normal at the age of 4 months, but is too
young for definite assessment.
Three children are retarded both physic-
ally and mentally; in one the retardation is
moderate, but in the other 2 it is gross, and
2 of these 3 patients reside permanently in an
institution (Table 11).
Psychometric assessment has been carried
out in 5 of the normal children and in one
with moderate retardation (Table IV) . The
values are not absolute for some of the
children are still very young: the eldest 9
PROGNOSIS AND MANAGEMENT
TABLE I1
Outcome in 14 Cases Regarding Survival and
Mental Development
Normal Retarded Dead
Operated patients .... .... 7 2 1
Non-operated patients .... 0 1 3
years and the youngest 2V2 years. It was
felt unnecessary to subject the 2 grossly re-
tarded patients to these procedures.
Hydrocephalus was evident in 4 children;
one died subsequently from an unrelated ill-
ness and in 2 of the 3 living children, the
hydrocephalus has been arrested, spon-
taneously in the second year of life in one, and
in the other, it was controlled by a ventriculo-
cisternal shunt. Both these children have a
normal mentality, but in the third, hydro-
cephalus is still progressive and associated
with large porencephalic cysts which have ap-
parently followed an acquired block at the
aqueduct which was not evident in air studies
at the age of 2 months. The frontal cortex is
only 1-2 mm. thick. This gross cerebral mal-
formation, and evidence of developmental re-
tardation apparent even at the age of 6
months have contraindicated a ventriculo-
atrial shunt.
Four patients have died, and an autopsy has
been performed in 3 of them. One infant died
of Pseudomoms pyocyanea gastro-enteritis
one month after operation and at autopsy
the brain showed a severe degree of mal-
formation (Case VIII in Table IV). Figure
4 shows anomalies in the brainstem of this
infant. The other 3 children, all with marked
microcephaly , died of pneumonia.
Associated cerebral dysplasias were found
in 9 patients. The findings are based on an
autopsy in 3 patients, one of whom had been
investigated by means of air studies; 7 other
patients had air studies and an operation, and
one patient air studies alone. Cerebral mal-
formations were more numerous in the patients
with the grosser lesions and abnormalities out-
side the central nervous system were unex-
pectedly rare (Table 111).
In both the patients with a frontal en-
cephalocele and in those with a gross oc-
cipital lesion there were associated malfor-
mations of one or both cerebral hemispheres.
 N. BARROWAND D. A. SIMPSON 23

Radio-
Case No. Lesion graphic Associated Cerebral Treatment Result
Investiga- Abnormalities
tions
I Occipital L&V Hydrocephalus Excision Normal
meningocele Chiari malfor- V-C Shunt IQ 106
mation
Aqueduct stenosis
I1 Occipital Excision Normal
-
menineocele
I11 Occipital
meningocele
- - Excision Normal
IQ 105
IV Occipital L Chiari Type I11 Excision Retarded
meningocele DQ 60
Cervical spinal (Institu-
meningocele t ion )

V Occipital V Nil found Excision Normal

meningocele IQ 123
VI Occipital L Nil found Excision Apparently
meningocele normal
(recent
case)
VII Occipital V Hydrocephalus Excision Normal
encephalocele Aqueduct stenosis D Q 100
Large massa
intermedia
VIII Occipital L&V Microcephaly Excision Died
encephalocele Verfebral Hydrocepha!us P.M.
angiogrm Cerebellar dysplasia
Chiari malforma-
tion
IX Occipital L Microcephaly - Gross
encephalocele Cerebellar dysplasia mental re-
tardation
(Institu-
tion)
X Occipital - Microcephaly - Died
encephalocele Cerebellar dysplasia P.M.

I - -1
~~

XI Occipital Microcephaly
encephalocele Cerebellar agenesis
Cortical dysplasia
XI1 Parietal - Microcephaly - Died
encephalocele Cortical dysplasia
XI11 Frontal L&V Microcephaly Excision Retarded
encephalocele Hydrocephalus Repair by
Porencephaly bone graft
Aqueduct stenosis
-
XIV Frontal L Unilateral frontal Replaced Normal
encephalocele atrophy Repair by
Rudimentary optic tantalum
nerve mesh
 94 CRANIUM BIFIDUM-INVESTIGATION PROGNOSIS AND MANAGEMENT

TABLE 111 and we agree with Voris (1948) that each

Associated Anomalies in 14 Patients with Cranium case should be managed individually.
Bifidum
Central Nervous System
The first step is to form a correct assess-
Hydrocephalus . . . . . . . ment and from this to obtain as far as pos-
6
Dysplasia of telencephalon ( I with porenceph- sible, a prognosis as to future development,
I
which can be difficult. Outspoken abnor-
aly) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
Cerebellar dysplasias ( 3 with Arnold-Chiari malities such as microcephaly, can be inter-
malformation) . . . . . . . . . . . . . . . . . . . . . . . . . . . 7
preted confidently, but lesser degrees of cere-
Thalamic abnormalities (large massa inter-
bral hypoplasia may be clinically undetectable
media) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2
Eyes in the neonatal period. This is understand-
Rudimentary optic nerve and microphthalmia able, for the structures most likely to be in-
1
Skull volved in an encephalocele are immature at
1
birth. On the other hand, we found air
Craniolacunia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
Craniosynostosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1
studies to be most helpful in arriving at a
Hypertelorism . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1
prognosis, and as others have found (Ingra-
Extracranial abnormalities
ham and Matson, 1954; Voris, 1948), ventri-
Bilateral congenital dislocation of hips . . . . . . . 1
culography or encephalography should be
Left hydrocele . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . I
Cervical spinal rneningocele and lumbar spina performed at the earliest convenient time to
exclude major cerebral malformations, and
bifida occulta . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1
especially to determine whether hydrocephalus
All but one showed mental retardation clinic- is present. Lorber (1961) reported that of
ally or on psychometric testing. 16 patients with an encephalocele who had
Malformations were also encountered in systematic air studies, 11 had hydrocephalus
cerebral tissue in the 3 excised encephaloceles. of some degree and in most of these, the size
In 2 this tissue was poorly diffeientiated and of the head was within normal limits. Angio-
presumably non-functioning, and in the third graphy in selected cases may also be very
the tissue was well organized cerebral cortex useful (Fig. 3 ) . On the basis of these in-
but deficient in white fibre connections, and
vestigations surgical treatment was not under-
probably not of real value.
taken in 4 of our 14 patients and we have not
yet had occasion to regret the policy adopted.
Air studies may also be of negative value in
the diagnosis of cranial dermoid cysts, nasal
‘gliomas’, and other conditions in which the
suspicion of cranium bifidum is entertained.
Of the 8 patients with occipital lesions who
were investigated, 6 had abnormalities in the
hind-brain, notably some form of the Arnold-
Chiari malformation, some combined with a
stricture of the aqueduct or a cisternal sub-
....... . .
arachnoid block. The malformations were
Fig. 4. Sagittal section of brainstem, stained by Weil’s often of great complexity (Table IV).
method (Case VIII, Table IV). The dorsal part of
the cerebellum was removed and the cerebellar cor- The 4 patients with hydrocephalus all had a
tex lies anterior to the pons (X). The medulla stricture of the aqueduct of varying severity
oblongata shows a kink (Y) at its junction with the
spinal cord, and the tectum has a typical upward as determined by air studies in 3, and at
beak (Z). autopsy in one. In the latter, it was difficult
to decide whether the block at the aqueduct
DISCUSSION was due to congenital forking, or to compres-
Cranium bifidum is a most variable con- sion by incarceration of the hind-brain in a
dition both clinically and pathologically, small posterior fossa.
 N. BARROWAND D. A. SIMPSON 25

Deliberate subdural insufflation may be par- since, have found that occipital lesions carry
ticularly helpful here. In infants, air can the worst prognosis because of the frequency
usually be introduced into the subdural space of associated hydrocephalus, but it seems
at the conclusion of a pneumo-encephalogram likely that this opinion needs some qualifica-
by inverting the baby from prone position and tion now that the operation of ventriculo-
gently shaking; when the head is dependent atrial shunt permits efficient treatment in most
and the buttocks elevated, air will usually cases of hydrocephalus. Ingraham and Swan
enter the subdural space through the original reported that 20 of 59 surviving patients were
puncture. When this does not occur, a burr- mentally and physically normal, and Fisher,
hole is needed. Uihlein, and Keith (1952) that 25 of 59
The excision of the encephalocele or men- 'cases were 'in good condition'. Seven of the
ingocele is in most cases a minor procedure 10 patients we have operated upon are either
and can be performed in the first few weeks normal or suffering from physical disabilities
of life. Leakage of cerebrospinal fluid or not incompatible with a useful and happy life.
ulceration of the investing membrane are in- All but one have had a psychometric assess-
dications for urgent operation. However, a ment.
fronto-nasal encephalocele requires a com- The incidence of associated congenital ab-
paratively elaborate repair, and this may be normalities is also reported to be higher
easier if operation is deferred (Ingraham and in cranium bifidum than in spina bifida
Matson 1954). No special difficulties were (Schwidde, 1952; Cohn and Hamby, 1953),
encountered in the repair of a large fronto- but in our series, although associated con-
nasal encephalocele at the age of 3 months. genital abnormalities of the brain are numer-
It may be wise, where possible, to operate ous, extracranial abnormalities were unex-
before the paranasal air cells have become pectedly rare.
pneumatised. It may also be prudent, as It is interesting to note that a definite geo-
Chaturaporn (1964) has suggested, to leave graphical variation in the frequency of
an intranasal encephalocele in situ, only cranium bifidum as compared with spina
transecting its base, and repairing the dural bifida exists in the literature. In America,
defect, for removal of intranasal cerebral tis- Ingraham and Swan (1943) reported a ratio
sue may introduce the risk of meningitis. of 15.5, and Fisher, et al., (-1952) a ratio of
Progressive hydrocephalus complicating 1:8. In Germany, Benger and Schonbauer
cranium bifidum merits treatment if there are (1958) and Hemmer (1962) report an in-
no other contra-indications, and we now re- cidence of 1:l and 1:2 respectively. It seems
commend the universally applicable procedure likely that cranium bifidum is more common
of ventriculo-atrial shunt for hydrocephalus in eastern Europe generally. The location of
occurring in the first year of life. In older the lesions in the cranium also differs in dif-
children with a block at the aqueduct, a ven- ferent parts of the world. In America occi-
triculo-cisternal or ventriculo-spinal shunt is pital lesions are more numerous than frontal
preferable. A preliminary lumbar air en- lesions in the proportion of 63:21 (Ingraham
cephalogram, as well as a ventriculogram, and Swan, 1943), and the same occurs in
should be performed to establish the patency Germany where Hemmer (1962) gives a
of the external subarachnoid cisterns. This ratio of 20:l; but in Russia, it seems the re-
was in fact done in the one patient who re- verse is found: Sokolov (1954) gives a ratio
quired such surgery, and the result has been of 26 frontal lesions to 4 occipital, and Zverev
satisfactory. (1956) gives an even bigger ratio of 193:14.
Many reported series leave the reader in Chaturaporn (1964) indicated that frontal
doubt about the mentality of the survivors, lesions are more common throughout eastern
but it is clear that there is a high incidence Asia. In conformity with the American and
of severe mental disability, certainly higher German reports, occipital lesions were more
than with spina bifida (Schwidde, 1952). common in our series (Table I ) . Females
Ingraham and Swan (1943), and others were also more commonly affected in our
26 CRANIUM BIFIDUM-INVESTIGATION
PROGNOSIS
series than in the Russian literature: for ex-
ample, Sokolov (1954) gives an incidence of
17 males to 13 females, while the ratio of
males to females given by Poleshchyk (1956)
is 16:7.
CONCLUSIONS
The term cranium bifidum covers a wide
range of cranial and cerebral malformations,
often very complex and clinically formidable.
Obstructive hydrocephalus is common. Each
case needs thorough assessment, in which air
encephalography is invaluable. Surgical
treatment is often simple, and the majority of
cases treated have a normal mentality.
Modern methods of treatment of hydro-
cephalus have increased the scope of surgical
intervention; nevertheless some cases are un-
treatable, and these should be identified as
early as possible.
ACKNOWLEDGEMENTS
We wish to thank the Board of Management of
the Adelaide Children’s Hospital for permission to
report the cases published in this article. We also
thank Dr. M. Fowler for assistance in the patho-
logical studies, Mrs. H. Thiede for histo-pathological
work and Mr. R. Boyd for photography.
REFERENCES
Benzer, H. and Schonbauer, L. (1958). Report on 49
brain and spinal cord ruptures. Miinch. med
Wschr., 100, 565.
AND MANAGEMENT
Chaturaporn, Hongraprabhas ( 1964). Personal Com-
munication.
Cohn, G. A. and Hamby, W. B. (1953). Surgery
of cranium bifidum and spina bifida; a follow-up
report of 64 cases. J. Neurosurg., 10, 297.
Fisher, R. G., Uihlein, A., and Keith, H. M. (1952).
Spina bifida and cranium bifidum; a study of 530
cases. Proc. Staff Meet., Mayo Clin., 27, 33.
Hemmer, R. (1962). About the treatment and prog-
nosis of meningoceles, myeloceles and encephalo-
celes with reference to modern procedures of treat-
ment of hydrocephalus. Munch. med Wschr., 104,
2404.
Ingraham, F. D. and Matson, D. D. (1954). Neuro-
surgery of infancy and childhood. C. C. Thomas,
Springfield, 111.
Ingraham, F. D. and Swan, H. (1943). Spina bifida
and cranium bifidum; a survey of 546 cases. New
Engl. J . Med., 228. 559.
Jefferson, A. and Lewtas, N. (1963). Value of tomog-
raphy and subdural pneumography in subfrontal
fractures. Acfu rudiol. [Diagn] (Stockh.) 1, 118.
Lorber, J. ( 1961). Systematic ventriculographic stud-
ies in infants born with meningomyelocele and en-
cephalocele. The incidence and development of
hydrocephalus. Arch. Dis. Childh., 36, 381.
Poleshchuk, I. S. (1956). Our experience in surgical
treatment of congenital cerebral hernia. V o p .
Neirokhir, 20, 39.
Schwidde, J. T. (1952). Spina bifida; survey of 225
encephaloceles, meningoceles and myelomeningo-
celes. Amer. J. Dis. Childh., 84, 35.
Sokolov, N. I. (1954). Congenital encephalocele.
Khirurgiya (Mosk.), 9, 37.
Voris, H. C. (1948). Spina bifida and cranium bi-
fidum. J. int. Coll. Surg., ll, 634.
Zverev, A. F. (1956). Evaluation of various meth-
ods of operating on congenital cerebral hernia.
Khirugiya ( M o s k . ) 32, 60.
Adelaide Children’s Hospital,
Adelaide, South Australia.