J Neurosurg: Pediatrics / Volume 6 / December 2010

J Neurosurg Pediatrics 6:000000, 2010

541
F
RONTOETHMOIDAL meningoencephaloceles are com-
mon in many Southeast Asian countries, occurring
in 1 in 5000 live births.
8,3840
These lesions often af-
fect poor, rural children in developing countries but their
etiology is still poorly understood.
32,36,39
In addition to
the obvious facial deformity of the fMEC itself, affected
children may also have telecanthus,
3,27
amblyopic eyes,
and epiphora.
5
Furthermore, some of the children have
neurological complications or associated brain anoma-
lies,
18,20
although most are mentally normal.
12,15,19
Several papers have been published on fMEC treat-
ment in countries with modern medical infrastructures,
15,18

such as Thailand.
2,21,29,37,38
However, fMECs are common
in many poorer and less-well-developed countries where
such facilities are not available. This puts a different em-
phasis on the development of effective fMEC treatment
programs. For example, in Cambodia the main focus of
fMEC management should be on the development of
low-cost but effective procedures by adapting treatment
programs to the countrys social, fnancial, and medical
J Neurosurg: Pediatrics / Volume 6 / December 2010
J Neurosurg Pediatrics 6:541549, 2010
Frontoethmoidal meningoencephalocele: appraisal of 200
operated cases
Clinical article
Ngiep OucheNg, M.D.,
1
FrDric Lauwers, M.D., ph.D.,
2, 3
JiM gOLLOgLy, M.D.,
1

LOuisa Draper, M.B.B.s., M.a.,
1
BruNO JOLy, M.D.,
2

aND FraNck-eMMaNueL rOux, M.D., ph.D.
2,4
1
Childrens Surgical Centre at Kien Khleang, Phnom Penh, Cambodia;
2
Mdecins du Monde (Opration
Sourire), Paris; and Departments of
3
Maxillofacial Surgery and
4
Neurosurgery, Hpital Purpan, Toulouse,
France
Object. Frontoethmoidal mengingoencephaloceles (fMECs) are frequently observed in Cambodia, especially
in poor families. The authors describe issues related to the surgical treatment of fMECs in Cambodia at the end of a
humanitarian program that provided surgery free of charge to patients and their families.
Methods. The authors reviewed 257 cases of fMEC involving patients who presented to their institution, the
Childrens Surgical Center in Phnom Penh, between 2004 and 2009. They treated 200 of these patients surgically
(108 males, 92 females; 89% younger than 18 years) using a low-cost management plan with no routine pre- or
postoperative investigations. Initially, surgery was performed by visiting foreign surgeons who taught the procedures
to resident surgeons. Patients were not charged for consultations or treatment and received at least 1 follow-up ex-
amination 6 months postoperatively.
Results. The nasoethmoidal type was the most frequent fMEC encountered (69%). Many patients had associ-
ated ophthalmological issues (46% of cases). Only 1 familial case was detected. Combined neurosurgical and facial
procedures were successfully standardized and learned by surgeons initially unfamiliar with fMEC management.
A neurosurgical approach avoided the need for a facial incision in 42 cases, improving cosmetic results. The most
common postoperative issues were a temporary CSF leak (24 cases [12%]) and/or infection (28 cases [14%]). There
were 3 deaths directly related to the operations. Cosmetic results were good in 145 cases, average in 27, poor in 7,
and worse than preoperative appearance in 6 patients. Fifteen patients were lost to follow-up. The parents of 87%
of the children were rice farmers. Questionnaire results confrmed that fMEC has important social and educational
consequences for the affected children and that these consequences can be partially improved by fMEC correction.
Conclusions. This experience in fMEC management demonstrates that local surgeons can treat these malforma-
tions with limited surgical materials and in a nonspecialized infrastructure after principles of treatment have been
learned and if they are carefully respected. Surgery for fMEC can thus be more accessible to a larger number of pa-
tients in developing countries. Moreover, local treatment facilitates better postoperative and follow-up care.
(DOI: 10.3171/2010.9.PEDS1043)
key wOrDs frontoethmoidalmeningoencephalocele Cambodia
craniofacialsurgery telecanthus
541
Abbreviation used in this paper: fMEC = frontoethmoidal menin-
goencephalocele.
This article contains some figures that are displayed in color
on line but in black and white in the print edition.
Click here to listen to the podcast featuring an
interview with the authors.
N. Oucheng et al.
542 J Neurosurg: Pediatrics / Volume 6 / December 2010
resources.
10,31
Cambodia is a developing country ranking
137th on the human poverty index, with more than 75% of
its population living on less than $2 a day (http://www.nis.
gov.kh/). There is a lack of local neurosurgery and plastic
surgery teams, and even where the necessary resources and
personnel are available, the costs of a craniofacial opera-
tion are prohibitive for most people.
We have published previously on this topic,
31,32
and
we report here on a series of 200 patients who were surgi-
cally treated for fMECs between 2004 and 2009 using
limited surgical materials and equipment. To our knowl-
edge, this is the largest series of fMEC cases published to
date. This study also represents the appraisal of a human-
itarian and teaching program. We discuss several issues
surrounding fMECs, focusing on the development of a
sustainable program of treatment and on surgical proce-
dures that can be adapted to local conditions.
Methods
Over a 68-month period (April 2004November
2009), we examined 257 patients with fMECs and treated
200 (78%) surgically at the Childrens Surgical Centre in
Phnom Penh (Cambodia). This is an international non-
governmental organization, without political or religious
affliation, which is registered in the US state of Alaska.
We work in collaboration with a staff of over 50 at the
National Rehabilitation Centre at Kien Khleang Hospi-
tal, Phnom Penh, providing free ophthalmic and recon-
structive surgical care for disabled people who could not
otherwise get the help they need. In 2008, we saw 6613
patients in the clinic and performed 2476 operations. We
have developed a program to encourage specialist sur-
geons from overseas countries to visit the center. This
serves 2 purposes: 1) to promote a continuing interest by
such specialists in supplying services to poor and disabled
people; and 2) to provide training for Khmer surgeons,
anesthesiologists, and support personnel. We also receive
support from Mdecins du Monde, a French nongov-
ernmental organization founded in 1980 that is involved
in medical, surgical, psychiatric, and rehabilitation pro-
grams worldwide. Mdecins du Monde provided 2 teams
of visiting surgeons (from France and Germany, includ-
ing a craniofacial surgeon, a neurosurgeon, an anesthe-
tist, and sometimes 1 or 2 nurses) who visited Cambodia
24 times per year over this period, staying for 12 weeks
each time to carry out clinical work. During these peri-
ods, the visiting teams provided instruction in all areas of
fMEC management, including patient selection for sur-
gery, operative technique, management of complications,
and organization of follow-up. The fMEC treatment
training program was divided into pre- and postoperative
patient evaluation; practical teaching of the surgical steps
of fMEC treatment; and theoretical teaching, including
published articles on fMEC that are not otherwise avail-
able in Cambodia. All operations were performed with
at least 1 Khmer surgeon working with the foreign team.
Patient recruitment was carried out by our institution,
and all treatment was free of charge from the beginning to
the last follow-up clinic. The parents or adult patients had
to sign a consent form that explained the aim of the surgery
and its possible complications. Operative conditions are
detailed elsewhere.
31
In brief, to make the program sustain-
able, we developed a low-cost strategy for management,
in which most patients received no pre- or postoperative
blood tests, chest radiographs, electrocardiograms, or CT
scans. Otherwise, standard operative methods and equip-
ment (including a cautery, drills to make bur holes, and, oc-
casionally, Leibinger plates and biological glue) were made
available for these operations.
Classifcation of fMECs
Frontoethmoidal myelomeningoceles were classi-
fed according to the system described by Suwanwela and
Suwanwela,
39
which is based on the work of Mesterton
24

and Von Meyer,
42
dividing the deformity into nasofrontal,
nasoethmoidal, and nasoorbital meningoencephalocoeles.
More refned classifcations based on CT fndings can also
be used.
15,24
However, when CT is not readily available, we
think that the anatomical classifcation of Suwanwela and
Suwanwela, although less accurate, is more practical.
Operative Procedure
A combined neurosurgical-craniofacial procedure
under general anesthesia was used to treat fMEC. A bi-
coronal incision was performed. Both supraorbital nerves
were dissected gently from their bony notch and pre-
served (in children, the notch is open and the supraorbital
nerve can usually be freed easily). Tissues on the glabella
were then elevated until the rim of the defect made by the
herniation was reached. A bone fap was then made by
means of frontal bur holes and removal of the medial or-
bital walls (Fig. 1A). Once the bone fap was elevated and
removed, the fMEC was exposed at its base and could be
manipulated to allow ligature of the base. This hernial
dissection is the most diffcult part of the neurosurgical
procedure because the dura in pediatric patients is often
thin in this area and adherent to the bone, and careful
dissection must be carried out to avoid dural tears (Fig.
1B). Dural tears are nevertheless frequent. Once ligated,
the hernia is removed by suction or cautery and the proxi-
mal part is gently pushed back. The dura of the hernia is
stitched if possible (Fig. 1C). A small frontal graft is then
taken to repair the base behind the coronal suture and po-
sitioned between the crista galli and the upper part of the
bone fap. An epicranial patch can also be used. This part
of the procedure is fundamental to prevent CSF leak (Fig.
1D). For the facial procedure, in cases of minimal soft tis-
sue expansion in the face, the fMEC remnants on the face
can be removed through the coronal incision alone. The
fMEC remnants can be totally or partially resected to
normalize facial contours (Fig. 1E). In cases with a large
herniation, covered by a large area of the facial skin, an
additional direct approach to the lump was performed by
performing incisions and resecting skin to obtain a mini-
mal residual scar designed to fall into the medial canthal
area extending down to the nasolabial fold or up to the
midline of the forehead (Fig. 1F). Afterward nasal and/or
orbital corrections were carried out. In most of the cases
a medial canthopexy was needed. The medial canthal
ligaments were exposed and moved into the right posi-
J Neurosurg: Pediatrics / Volume 6 / December 2010
Frontoethmoidal meningoencephalocele in Cambodia
543
tion with a transosseous fxation. This could be achieved
by a simple procedure when the osseous confguration
was near normal, by using a bone graft when the medial
wall of the orbits was dehiscent, or by replacement of the
medial orbital walls. In some cases, nasal reconstruction
was performed in association with the canthal correc-
tion technique by using a small part of the bone graft or
simply by reshaping the frontonasal bone fap to decrease
the intercanthal width and, in the sagittal plane, mold the
graft to conform to a normal nasofrontal angle (Fig. 1G).
At the end of the procedure, biological glue is used, if
available, on the lateral part of the repaired base and a
small plaster bandage is then used to hold the frontonasal
contours for a few days (Fig. 1H).
Follow-Up
Organizing the postoperative follow-up of patients in
Cambodia and other developing countries can be chal-
lenging.
11
Strict conditions for follow-up were established.
Each patient was entered into a database with name, ad-
dress, and a phone number by which he or she could be
reached. A majority of patients did not have a phone at
home and therefore gave the phone number of a relative or
the head of their village. Before each operation, pictures
of the patients face (frontal, lateral, and oblique views)
were taken and included in the database. Each patient was
called back 6 months after the operation for a follow-up
clinic appointment. At this point, new pictures were taken
for comparison with the preoperative photos.
Evaluation
To mitigate the inherent bias of a surgeon evaluating
his own surgical results, 4 categories were used to grade
the clinical results. Global aesthetic results were evalu-
ated by the surgeons as worse, poor, average, or good.
Worse result means that the postoperative facial
appearance of the patient got worse after surgery;
Poor result means that the postoperative facial ap-
pearance of the patient did not improve after surgery and
was still incompatible with a normal life;
Average result means that facial appearance was
still abnormal, although improved;
Good result means that facial appearance was al-
most normal, with perhaps a few scars visible on the pa-
tients face or very minimal facial scarring, so that no one
would guess that the patient had surgery for fMEC; a nor-
mal social life could be expected. Self-evaluation of cos-
metic results was based on the same 4 categories (worse,
poor, average, good). Children and parents were asked to
give their opinion about the fnal cosmetic results of the
fMEC operations. Finally, the social or educational im-
pact of surgery was evaluated by having the children and
parents answer a focused questionnaire before the opera-
tion and at the end of the follow-up period.
The questionnaire is shown in Table 1.
Results
Of the 257 patients with fMECs seen in our institu-
tion between 2004 and 2009, 200 (78%) were treated sur-
gically. With respect to the other 57 patients, intervention
was refused or postponed in 51 cases, 1 patient had the
opportunity to be treated abroad (in Singapore), and 5 pa-
tients were treated for various reasons by a small transfa-
cial approach. Of the 200 patients in whom we performed
surgery, 108 were male and 92 were female (male/female
ratio 1.17:1); 178 (89%) were younger than 18 years old (48
were 05 years old; 44, 610 years old; 63, 1115 years
old; and 23, 1618 years old) and 22 were older than 18
years. Patients came from all Cambodian provinces but
mostly from provinces surrounding the capital, where the
Childrens Surgical Centre is based. The mean duration
of follow-up was 11 months (range 229 months). Fifteen
patients were lost for follow-up.
Social and Educational Consequences
Unexpected social consequences of fMECs were
Fig. 1. Surgical technique used by our team to treat fMEC. A: First
step of the procedure: bicoronal incision and bone fap. The arrows indi-
cate the supraorbital nerves. B: Exposure of the fMEC and ligature of
the base (arrow). C: Ligature (arrow) and removal of the hernia. Inset:
The hernia is gently pushed back. D: Base reconstruction step. The
left inset shows the use of a small frontoparietal graft (arrowhead). The
right inset shows the use of an epicranial patch. E: Facial procedure:
In this case, the fMEC remains on the face were removed through the
coronal incision alone (arrow). F: Removing of the fMEC remains. In-
set: Additional direct facial approach was used in this case of a large
fMEC. G: Nasal and/or orbital correction, with decrease of the inter-
canthal width (arrows). H: End of the procedure: A small plaster ban-
dage is then used to hold the frontonasal contours.
N. Oucheng et al.
544 J Neurosurg: Pediatrics / Volume 6 / December 2010
found, including name-calling and severe ostracism of the
children. Indeed, 28 patients (14%) had a given name related
to their facial appearance: Pork, Poy, or Pong, mean-
ing bump; Domrey, meaning elephant; Chrouk,
meaning pig; or Koy, meaning corncob. The impact
on education was evaluated in the 96 children with fMECs
who were of school age. Of these children, 17 (18%) re-
fused to go to school due to perceived shame related to the
malformation, and 28 (29%) attended school but said that
were mocked by other children.
Socioeconomic Status
The parents occupation was documented in 184 cas-
es: in 160 cases (87%), the childrens parents were rice
farmers. Others were industrial or offce workers, traders,
or policeman. Almost all patients seen in our institution
were poor, with a family income of less than US $2 per
day.
Classifcation of fMECs
Most fMECs were nasoethmoidal (138 cases [69%]);
nasofrontal fMECs were seen in 54 cases (27%) and na-
soorbital in 8 cases (4%). Although most cases were sim-
ple, combined cases were also seen as detailed in Table 2.
Personal and Family History and fMEC-Associated
Conditions
Familial history of fMEC was only found in 2 pa-
tients (1%) who each had a cousin affected by the mal-
formation. The parents of some of the patients (21 cases
[10%]) said that the malformation grew with time. Twen-
ty-six patients (13%) had previously undergone surgery
via a transfacial approach to treat their fMEC in another
center. Overall, 92 patients (46%) had at least 1 ophthal-
mological problem (epiphora, visual feld defect, or stra-
bismus); these ophthalmological problems were the main
associated issues (Table 3). In 3 cases, we think that spon-
taneous closure of the fMEC occurred because we saw
a sequestered fMEC with no cranial base defect. A der-
moid cyst was also found associated with the fMEC in 2
cases. We found no other neural tube defects.
Surgical Procedures
The mean operative time was 2 hours 35 minutes
(range 45 minutes5 hours). Accurate blood losses were
not documented; nevertheless, only 2 patients (1%) need-
ed postoperative blood transfusions. Mean hospitaliza-
tion time was 20 days (range 1060 days). Our combined
neurosurgical/facial procedures were relatively standard-
ized and are illustrated in Fig. 1.
TABLE 1: Patient questionnaire: impact of surgery on social life and education
Before the operation:
Do you go to school? Y N
Are you happy to go to school? Y N
Do other children tease you now? Y N
Parents: What are your feelings about the disease of your child? open answer
After the operation (6 12 mos):
Social activities:
Are you? outgoing/confdent average shier than before the operation?
Do you have more friends than before the operation? Y N
Overall, are social life or activities after the operation: improved remained the same got worse
School:
Do you go to school? Y N
Are you happy to go now to school? Y N
Do other children tease you now? Y N
Overall, what is your opinion about the results of the operation:
Child (when possible): worse poor average good
Parent: worse poor average good
TABLE 2: Classifcation of fMECs in 200 surgically treated cases*
fMEC Type No. of Cases
NE 138
isolated NE 116
bilat 68
unilat 48
NE + NO 22
NE bilat + NO rt 5
NE bilat + NO lt 5
NE unilat + NO rt 4
NE unilat + NO lt 7
NE rt + NO lt 1
NF 54
NF isolated 47
NF + NO 7
NF + NO rt 3
NF + NO lt 4
NO isolated 8
* NE = nasoethmoidal; NF = nasofrontal; NO = nasoorbital.
J Neurosurg: Pediatrics / Volume 6 / December 2010
Frontoethmoidal meningoencephalocele in Cambodia
545
Immediate Postoperative Complications
The main postoperative complications were CSF leaks
and wound infections. Postoperative CSF leak under the
skin or through the facial scar was observed in 24 patients
(12%). These leaks were treated by reclosure of the facial
scar (5 cases), lumbar puncture and compressive head
dressing (8 cases), or reopening of the bicoronal incision
and direct reclosure of the fMEC (1 case). Other CSF leaks
resolved spontaneously within 15 days, but 1 was observed
that lasted 1 month. Skin wound infections were seen in 18
patients (9%), osteomyelitis in 4 cases (2%), and meningitis
in 6 cases (3%). These infectious complications were treat-
ed by appropriate treatments including antibiotics, removal
of infected bone, or skin reclosure. Other postoperative
complications observed were high hyperthermia (2 cases),
seizures (2 cases), hydrocephalus (4 cases), acute psychosis
(2 cases), and vision loss (1 case).
Perioperative Deaths
Three deaths (in patients aged 2, 6, and 11 years)
were directly related to the surgical procedure, with
breathing problems and aspiration within 17 hours after
surgery. Another 14-year-old boy died because of malaria
with major hyperthermia, seizures, and coma 2 days after
operation. Finally, after a trouble-free early postoperative
period, a 12-year-old girl died 2 months after the opera-
tion because of acute meningitis.
Complications Observed at Follow-Up
Unilateral or bilateral hypesthesia of the frontal scalp
was observed in 5 cases (2.5%). As of the most recent fol-
low-up examination, epiphora persisted in 14 patients of
67 patients who had it preoperatively, improved in 4, and
resolved in the remaining 49. New epiphora was observed
in 3 patients. Delayed recurrence of fMEC due to a new
subcutaneous CSF leak was detected in 14 patients (7%)
for various reasons including pseudarthrosis of the bone
fap (3 cases) and face skin problems (2 cases). Additional
surgery was performed in 6 of these 14 patients.
Cosmetic and Social Results
Overall, using our subjective criteria as defned in
Methods, cosmetic results were judged by the surgeons as
worse in 6 cases, poor in 7, average in 27, and good in 145
cases. Examples of pre- and postoperative photographs
are shown in Fig. 2. Patients and parents tended to have
a better overall better opinion about surgical results than
surgeons. Of the 42 of patients in whom no facial incision
was needed, 38 had good results. The aesthetic results are
summarized in Table 4.
Responses to the questionnaire items about social life
were received from 182 patients and/or parents and re-
sponses to the items concerning education were received
from 96. (In 18 cases, either the patients had no opinion
or were lost to follow-up.) Surgery on the fMEC improved
the social life of these patients in 45 cases (25%) according
to patients or parents evaluations. In the other cases, the
patients or their parents either reported that their was no
change in the patients social life or had no opinion. One
patient said that his social life got worse. Education was
better in 22 patients (23%) of the 96 who were of school
age.
Overall Treatment Cost
All operations were free of charge for all patients.
The overall cost of each operation was estimated to be
US $380: $280 paid by Mdecins du Monde to bring
the foreign surgical team to Cambodia for each particu-
lar operation and the $100 cost to the Childrens Surgical
Centre for each operation.
Discussion
We think that one way to make fMEC surgery more
accessible to a larger number of patients in developing
countries is to train local surgeons to perform the opera-
tion (thereby reducing costs and increasing the number
of staff members able to provide treatment). Our experi-
ence in fMEC management is that local surgeons previ-
ously unfamiliar with this feld of work can treat these
malformations with limited surgical materials and non-
specialized infrastructure once principles of treatment
(indications, surgical technique, and follow-up) have been
learned and as long as they are carefully respected. We
therefore strongly recommend local treatment of children
affected with fMECs. Transfer of these patients abroad
for surgery is very expensive, and for the same amount of
money one could train local surgeons and arrange treat-
ment and follow-up for a much greater number of chil-
dren. Local treatment also facilitates better postoperative
and follow-up care.
Geographical Distribution and Etiology
Frontoethmoidal meningoencephalocele has a re-
markably specifc geographical distribution in many
Southeast Asian countries, such as Burma,
11,40
Thai-
land,
2,3,8,21,3739
Cambodia,
7,10,31,43
Malaysia,
1
Indonesia,
13,33

and Papua New Guinea.
14
The condition is also found in
India
19
and Australia in the aboriginal population.
35
This
specifc and unique geographical distribution of fMEC
TABLE 3: Associated conditions observed in our 200 surgically
treated patients
Condition No. of Cases
epiphora (bilat or unilat) 67
visual feld defect 40
canthal dystopia 31
strabism 18
seizures 17
microphthalmia (bilat or unilat) 12
cognitive delay 5
clinical hydrocephalus 4
corneal scar 2
blindness 2
microcephaly 2
other: hemiparesis, delayed walking, obesity, lower limb
hypotonia, club foot, congenital scars, syndactyly
9
N. Oucheng et al.
546 J Neurosurg: Pediatrics / Volume 6 / December 2010
may provide clues in the investigation of its etiology,
with respect to genetic/racial or environmental factors,
although the matter is complicated by the occasional dis-
covery of fMECs in ethnically diverse groupsfor ex-
ample, in various African countries,
6,17
Mexico,
9
and Tur-
key.
41
On the other hand, fMECs are rare in Western Eu-
rope, Japan, and North America, even in immigrants from
Southeast Asia despite their presence in large minority
groups. Thus, the preferential localization of fMECs in
Southeast Asia must only be a part of the solution in fnd-
ing the causes of this craniofacial malformation.
Genetic predisposition, higher paternal age, parental
consanguinity, vitamin B defciency (due to malnutrition),
drugs, and chemicals used in Cambodia during the wars
in the 1970s have all been suggested by various authors
in the etiology of fMEC
5,8,36,39
but refuted by others.
40
A
slight preponderance in males and preferential occur-
rence of the disease in poor, rural people have often been
observed, as in this series.
11,31,32,40,41
However, these fnd-
ings could be biased by the fact that boys are more likely
to be referred for treatment than girls and by the social
structure of Southeast Asian countries (overwhelmingly
rural). Nevertheless, like our study, many large studies do
suggest that this disease is linked to poverty,
36,40
which
could also explain the very low prevalence of fMECs in
more developed Western countries.
It may be that a combination of environmental fac-
tors with a genetic predisposition could explain the patho-
genesis and geographical distribution of these malforma-
tions.
8,33,36
Familial cases are very rare among previously
Fig. 2. Preoperative and postoperative photographs of representative patients. A: Four of the 42 patients in whom no facial
incision was necessary. B: Example of types of facial incision used in this series. The bicoronal approach allowed fMEC remov-
al in most cases and was used unless the herniated mass was large or lay very low on the face or a facial scar already existed.
J Neurosurg: Pediatrics / Volume 6 / December 2010
Frontoethmoidal meningoencephalocele in Cambodia
547
published series
1,31,33,38,40
and in our own experience. From
our own series of cases, we have demonstrated that the
date of conception, with predominance for the wet sea-
son, may play a role.
31
This was also shown in a case se-
ries in Burma 25 years ago.
40
Among environmental fac-
tors, fungal and teratogenic agents such as afatoxin or
ochratoxin, found in moldy rice during the wet season,
could be involved.
2,43
But it must be acknowledged that
currently the etiology of fMEC is largely unknown.
Pathogenesis and Natural History
The pathogenesis and natural history of fMEC are
2 further points that remain unresolved. The condition
is not classifed in the category of neural tube defects,
18

and is not associated with other neural tube defects
4
as
seen in this series. Studies using immunohistochemical
morphological analysis with neuron-specifc enolase in
autopsy material or surgical material showed that there
was no sign of dysraphism in the underlying brain.
25
One
possible mechanism involves a local defciency of the me-
soderm (which forms the skull of the embryo) in com-
bination with abnormal adhesion of the neuroectoderm
to the surface ectoderm (for example, through defcient
apoptosis).
13,23,33
The natural history of this malformation is variable
among patients.
22
Neurological complications and con-
genital brain anomalies are uncommon in fMEC,
28,38
and
a majority of affected children are mentally normal.
15

Some of our parents reported that their childs malfor-
mation did not grow at all with time, whereas others (as
reported by other authors) noted that their childs mal-
formation became progressively larger.
9,38
Some authors
therefore recommend early surgical correction to mini-
mize the pressure effect of the mass on facial growth.
3,5

It is important, however, to take into consideration local
resources and experience with respect to anesthesia, as
smaller children and babies will be those at highest risk
of anesthetic complications. The prognosis of untreated
fMEC has also been debated. Historically, some authors
claimed that only a minority of children who do not un-
dergo surgery could survive to adulthood.
16,34
However,
there is no recent scientifc evidence to argue for or
against a signifcantly reduced life expectancy in patients
with fMEC. In developing countries such as Cambodia,
child mortality is high (http://www.nis.gov.kh/), and chil-
dren with fMEC may die for many reasons unrelated to
the malformation. In our institution, we saw and treated
many teenagers and even adults with fMECs. In addition,
many adult cases of fMEC have been described in the
modern medical literature.
3,11,40
However, our data are
collected from clinic consultations and therefore will not
include all patients with the deformity. In Cambodia, as
in many developing countries,
19
patient are often referred
late. Some patients may die before they are referred for
treatment, and such cases would not be included in our
series. We did not see any neonates or very young babies
with fMECs in our clinic, so we have little direct expe-
rience with patients in this age group. We hypothesize,
however, that large fMECs in very young children could
affect life expectancy,
38
although a majority of patients
with moderate or small fMECs could reach adult age.
Therefore, with the exception of neonates or young babies
with very large fMECs, we believe that surgery can usu-
ally be electively planned in fMECs,
12
since the malfor-
mation is covered by normal skin or at least by an epider-
mal layer, and delaying surgery until after infancy could
reduce the risk associated with anesthesia. It is worth
mentioning that life quality and life expectancy of these
patients can also be indirectly reduced by inappropriate
traditional treatment or postoperative morbidity and
mortality, which is not nil in many series.
1,3,19,27,31
Consid-
ering that the malformation is probably not fatal, we must
take extra care when selecting cases for surgical treat-
ment and ensure that we provide comprehensive informa-
tion for the patients and their parents about the condition
and the risks and benefts of different treatment options.
Surgical Issues
Most authors agree that fMEC should be treated as
soon as possible to avoid deleterious effects on facial
growth (for example, the development of telecanthus).
2,9

This principle must be adapted to local situations to re-
duce the risks from anaesthesia (for example, blood loss,
hypothermia) and from the operation itself (such as CSF
leaks or infection), which can be more common or more
severe in young babies.
18
Delaying surgical treatment to
810 months of age may minimize these complications.
19

In developing countries, one should take the weight and
size of the child into consideration, as the prevalence of
malnutrition often means that young children are smaller
than in more developed countries. In our series, we avoid-
ed treating children who were younger than 1 year of age
or weighed less than 810 kg. We also avoided perform-
ing surgery in cases involving children who had been
surgically treated by other teams and for whom no chart
or operation reports were available as well as in children
with intranasal meningoencephalocele (located posterior
to the crista galli). We were also cautious about reoperat-
ing in cases in which the aesthetic result was considered
good but not excellent. In follow-up, we performed sim-
ple aesthetic corrections (revision of scars, for instance),
avoiding new neurosurgical operations in the absence of
complications.
The lack of a CT scanner meant that we could not
adequately assess or treat hydrocephalus preoperatively.
However, we did not fnd that hydrocephalus was an im-
portant issue in the treatment of fMECs in our patients:
only 4 out of 200 children treated required shunts in the
postoperative period. In concordance with other authors,
we agree that the presence of hydrocephalus or ventricu-
TABLE 4: Esthetic results in 200 surgically treated cases
No. of Cases
Result Surgeon Evaluation Patient or Parent Evaluation
good 145 158
average 27 17
poor 7 8
worse 6 2
unknown 15 15
N. Oucheng et al.
548 J Neurosurg: Pediatrics / Volume 6 / December 2010
lar enlargement does not necessarily cause problems or
require treatment.
5
However, we did observe many oph-
thalmological problems, including epiphora, amblyopia,
and microphthalmia. Lacrimal dysfunction was especial-
ly frequent.
The choice of the best surgical treatment for fMEC
is debated. Most authors consider that the best method is
a combined procedure: bicoronal insicion, nasofrontal
bone fap, and facial reconstruction.
2,5,15,18,20
Others used
either bicoronal, transfacial approaches, or combined pro-
cedures according to the patient malformation pattern.
9

Some claim that fMEC can be corrected only by an extra-
cranial procedure;
11,14,30
this could be more appropriate in
some developing countries where neurosurgical expertise
is lacking.
11,13,14
Each method has its own value in certain
situations, but we prefer a combined procedure (Fig. 1)
which has, in our opinion, 3 major advantages: 1) it al-
lows a better fMEC closure, 2) a better telecanthus cor-
rection can be achieved, and 3) it can reduce and some-
times avoid facial scars. To achieve these aims, we used
the modifed Chula technique,
21,26,28
trying to avoid large
facial scars (Fig. 2).
Although sometimes debated,
5,41
most authors agree
that fMEC including the prolapsed brain (which is
thought to be glial or gliotic on the base of pathological
examinations) can be resected without causing neurologi-
cal sequelae.
11,13,18
However, we found that the prolapsed
brain that needed to be resected was not always gliotic
but sometimes contained normal brain, especially close
to the neck of the defect. In most cases, it is the anterior
part of the gyrus rectus that is resected. Nevertheless, we
believe that children are usually not cognitively affected
although formal neuropsychological testing is rarely per-
formed, due to local limitations
19
or loss to follow-up. Ac-
curate neuropsychological examinations could be useful
to confrm this presumed fact, especially when prolapsed
brain is resected.
Postoperative Issues
Postoperative morbidity in fMEC treatment is usu-
ally due to infection and CSF leaks.
11,19,21,31
Based on our
experience, CSF leaks were mostly observed during the
frst postoperative year. Two factors can explain CSF
leaks: the transfacial approach, and inadequate base re-
construction with no watertight dura closure. We strongly
believe that base reconstruction is fundamental to fMEC
correction and that it can be achieved with a bone graft,
pericranial patch, and surgical glue when available. The
dura of the fMEC is thin, and even with meticulous dis-
section dural tears are commonplace. Although a certain
number of leaks resolve spontaneously,
21,31
others do not,
which then predisposes to infection. The risk of CSF leak
can be reduced by avoiding a transfacial approach where
possible (for example, when the fMEC is small and the
facial skin of good quality).
29
Problems with scar healing and delayed infections
were also observed in our case series. In a developing
country like Cambodia, there are a variety of reasons
why patients may not attend follow-up clinics, even when
a complication arises. Follow-up must therefore be care-
fully planned with patients. In our series, the cost of travel
from the province to the capital (about $10) for follow-up
was prohibitive for many patients. This charge was paid
in advance in some cases to help to ensure follow-up.
We believe that without organizing follow-up, we would
have missed some complications and the reported post-
operative morbidity and mortality rates would have been
much lower. Our experience underscores the fundamental
importance of follow-up in fMEC management. On the
other hand, once patients had passed the frst few postop-
erative years, delayed complications were very rare.
Conclusions
This experience in fMEC management demonstrates
that local surgeons can treat these malformations with
limited surgical materials and in nonspecialized infra-
structure after principles of treatment have been learned
and if they are carefully respected. We think that surgery
for fMEC can thus be more accessible to a larger number
of patients in developing countries. Moreover, local treat-
ment facilitates better postoperative and follow-up care.
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.
Author contributions to the study and manuscript preparation
include the following. Conception and design: Roux. Acquisition of
data: Oucheng, Lauwers, Joly. Analysis and interpretation of data:
Roux, Oucheng, Lauwers, Joly. Drafting the article: Roux, Oucheng,
Draper. Reviewed final version of the manuscript and approved it for
submission: all authors. Administrative/technical/material support:
Roux, Draper. Study supervision: Roux, Lauwers, Gollogly.
Acknowledgments
The authors thank Denise Gbl for supplying the bibliography
for this work and Catherine Raux for data analysis. The authors also
thank visiting surgeons Thomas Pinzer and Gnter Lauer for help-
ing them during their humanitarian mission. The authors are also
indebted to Paulette Fauch, Florence Giroussens, Sylvie Simonnet,
and Julie Lembeye for assistance during this humanitarian program.
The authors also thank LOral Fondation.
References
1. Arshad AR, Selvapragasam T: Frontoethmoidal encephalocele:
treatment and outcome. JCraniofacSurg 19:175183, 2008
2. Boonvisut S, Ladpli S, Sujatanond M, Tandhavadhana C, Tisa-
vipat N, Luxsuwong M, et al: Morphologic study of 120 skull
base defects in frontoethmoidal encephalomeningoceles.
PlastReconstrSurg 101:17841795, 1998
3. Boonvisut S, Ladpli S, Sujatanond M, Tisavipat N, Luxsuwong
M, Nunta-aree S, et al: A new technique for the repair and
reconstruction of frontoethmoidal encephalomeningoceles by
medial orbital composite-unit translocation. BrJPlastSurg
54:93101, 2001
4. David DJ, Proudman TW: Cephaloceles: classifcation, pathol-
ogy, and management. WorldJSurg 13:349357, 1989
5. David DJ, Sheffeld L, Simpson DA, White J: Fronto-ethmoid-
al meningoencephaloceles: morphology and treatment. Br J
PlastSurg 37:271284, 1984
6. De Klerk DJJ, De Villiers JC: Frontal encephaloceles. SAfr
Med J 47:13501355, 1973
7. De Vries HR, Maxwell SM, Hendrickse RG: Foetal and neo-
natal exposure to afatoxins. Acta Paediatr Scand 78:373
378, 1989
J Neurosurg: Pediatrics / Volume 6 / December 2010
Frontoethmoidal meningoencephalocele in Cambodia
549
8. Flatz G, Sukthomya C: Fronto-ethmoidal encephalomeningo-
celes in the population of northern Thailand. Humangenetik
11:18, 1970
9. Fuente del Campo A, Escanero Salazar A, Baldizon Recio N,
Dimopulos A: Transfacial surgical treatment and anthropo-
metric considerations of frontoethmoidal meningoencephalo-
celes. AnnPlastSurg 23:377389, 1989
10. Gollogly J, Oucheng N, Lauer G, Pinzer T, Lauwers F, Roux
FE, et al: Frontoethmoidal meningoencephalocoele repair in
Cambodia: outcomes and cost comparisons. Trop Doct 38:
167170, 2008
11. Holm C, Thu M, Hans A, Martina M, Silvia GS, Moritz S,
et al: Extracranial correction of frontoethmoidal meningoen-
cephaloceles: feasibility and outcome in 52 consecutive cases.
PlastReconstrSurg 121:386e395e, 2008
12. Hoving EW: Nasal encephaloceles. ChildsNervSyst 16:702
706, 2000
13. Hoving EW, Vermeij-Keers C: Frontoethmoidal encephalo-
celes, a study of their pathogenesis. Pediatr Neurosurg 27:
246256, 1997
14. Jacob OJ, Rosenfeld JV, Watters DA: The repair of frontal
encephaloceles in Papua New Guinea. AustNZJSurg 64:
856860, 1994
15. Kumar A, Helling E, Guenther D, Crabtree T, Wexler AW, Brad-
ley JP: Correction of frontonasoethmoidal encephalocele: the
HULA procedure. PlastReconstrSurg 123:661669, 2009
16. Lampert FM: Pathogenesis and treatment of so-called con-
genital cerebral herniae. Surg Gynecol Obstet 38:159162,
1924
17. Lintilhac JP, Acquaviva R, Cochain JP: [Anterior meningo-
encephalocele of the base of the skull. Contribution of plastic
surgery.] Maroc Med 45:316324, 1966 (Fr)
18. Macfarlane R, Rutka JT, Armstrong D, Phillips J, Posnick J,
Forte V, et al: Encephaloceles of the anterior cranial fossa.
Pediatr Neurosurg 23:148158, 1995
19. Mahapatra AK, Agrawal D: Anterior encephaloceles: a series
of 103 cases over 32 years. JClinNeurosci 13:536539, 2006
20. Mahatumarat C, Rojvachiranonda N, Taecholarn C: Fronto-
ethmoidal encephalomeningocele: surgical correction by the
Chula technique. PlastReconstrSurg 111:556567, 2003
21. Mahatumarat C, Taecholarn C, Charoonsmith T: One-stage
extracranial repair and reconstruction for frontoethmoidal
encephalomeningocele: a new simple technique. JCraniofac
Surg 2:127134, 1991
22. Mahatumarat C, Taecholarn C, Rojvachiranonda N: Sponta-
neous closure of bony defect in a frontoethmoidal encepha-
lomeningocele patient. JCraniofacSurg 10:149154, 1999
23. Marin-Padilla M: Morphogenesis of experimental encepha-
locele (Cranioschisis occulta). JNeurolSci 46:8399, 1980
24. Mesterton CB: Ommedfdthjrnbrck[dissertation]. Up-
sala: Upsala University, 1855
25. Oi S, Matsumoto S: Morphological evaluation for neuronal
maturation in anencephaly and encephalocele in human neo-
nates. A proposal of reclassifcation of cephalic dysraphism.
ChildsNervSyst 6:350355, 1990
26. Pinzer T, Gollogly J, Krishnan KG, Schackert G, Lauer G:
Telecanthus and hypertelorism in frontoethmoidal meningo-
encephaloceles and the surgical correction of these condi-
tions: Part 1. An orbital anthropomorphometric evaluation of
the Khmer subpopulation of Cambodia. JCraniofacSurg 19:
137147, 2008
27. Pinzer T, Lauer G, Gollogly J, Schackert G: A complex ther-
apy for treatment of frontoethmoidal meningoencephalocele
in a developing third world country: neurosurgical aspects. J
Neurosurg 104 (5 Suppl):326331, 2006
28. Rojvachiranonda N, David DJ, Moore MH, Cole J: Frontoeth-
moidal encephalomeningocele: new morphological fndings
and a new classifcation. JCraniofacSurg 14:847858, 2003
29. Rojvachiranonda N, Mahatumarat C, Taecholarn C: Correc-
tion of the frontoethmoidal encephalomeningocele with mini-
mal facial incision: modifed Chula technique. J Craniofac
Surg 17:353357, 2006
30. Rosenfeld JV, Watters DAK: Surgery in developing countries.
J Neurosurg Pediatr 1:108109, 2008 (Letter)
31. Roux FE, Lauwers F, Oucheng N, Say B, Joly B, Gollogly J:
Treatment of frontoethmoidal meningoencephalocele in Cam-
bodia: a low-cost procedure for developing countries. J Neu-
rosurg 107 (1 Suppl):1121, 2007
32. Roux FE, Oucheng N, Lauwers-Cances V, Draper L, Cristini
C, Collogly J, et al: Seasonal variations in frontoethmoidal
meningoencephalocele births in Cambodia. Clinical article. J
Neurosurg Pediatr 4:553556, 2009
33. Sadewa AH, Sutomo R, Istiadjid M, Nishiyama K, Shirakawa
T, Matsuo M, et al: C677T mutation in the MTHFR gene was
not found in patients with frontoethmoidal encephalocele in
East Java, Indonesia. PediatrInt 46:409414, 2004
34. Schatz: Zur Therapie der Cephalocelen und der Spina Bifda.
BerlinKlinWschr 22:441, 1885
35. Simpson DA, David DJ, White J: Cephaloceles: treatment, out-
come, and antenatal diagnosis. Neurosurgery 15:1421, 1984
36. Suphapeetiporn K, Mahatumarat C, Rojvachiranonda N, Taech-
olarn C, Siriwan P, Srivuthana S, et al: Risk factors associated
with the occurrence of frontoethmoidal encephalomeningocele.
EurJPaediatrNeurol 12:102107, 2008
37. Suwanwela C: Geographical distribution of fronto-ethmoidal
encephalomeningocele. BrJPrevSocMed 26:193198, 1972
38. Suwanwela C, Sukabote C, Suwanwela N: Frontoethmoidal en -
cephalomeningocele. Surgery 69:617625, 1971
39. Suwanwela C, Suwanwela N: A morphological classifcation
of sincipital encephalomeningoceles. J Neurosurg 36:201
211, 1972
40. Thu A, Kyu H: Epidemiology of frontoethmoidal encephalo-
meningocoele in Burma. JEpidemiolCommunityHealth 38:
8998, 1984
41. Turgut M, Ozcan OE, Benli K, Ozgen T, Gray O, Salam S,
et al: Congenital nasal encephalocele: a review of 35 cases. J
CraniomaxillofacSurg 23:15, 1995
42. Von Meyer E: ber eine basale Hirnhernie in der Gegend der
Lamina cribrosa. Virchows Arch Path Anat Physiol Klin
Med 120:309320, 1890
43. Wangikar PB, Dwivedi P, Sharma AK, Sinha N: Effect in rats
of simultaneous prenatal exposure to ochratoxin A and afa-
toxin B1. II. Histopathological features of teratological anom-
alies induced in fetuses. Birth Defects Res B Dev Reprod
Toxicol 71:352358, 2004
Manuscript submitted February 2, 2010.
Accepted September 22, 2010.
Address correspondence to: Franck-Emmanuel Roux, M.D.,
Ph.D., Service de Neurochirurgie, Hpital Purpan, F-31059 Tou-
louse, France. email: franck_emmanuel.roux@yahoo.fr.