Surgery and reconstruction of a frontoethmoidal encephalomeningocele

CASE REPORT

One-Stage Operation for A Frontoethmoidal Encephalomeningocele

Associated with Ventriculomegaly — A Case Report

1
Sheng-Tzung Tsai, Chien-Hsing Wang , Shinn-Zong Lin, Tsung-Lang Chiu

1
Department of Neurosurgery, Plastic Surgery , Buddhist Tzu Chi General Hospital, Hualien, Taiwan

ABSTRACT
Encephalomeningoceles, especially in the frontoethmoidal region, are a form of neural tube defect which affect patients in South-
east Asia more commonly than those in Western countries. We performed a 1-stage reconstruction and ventriculoperitoneal (V-P)
shunt placement for a frontoethmoidal encephalomeningocele in an 18-year-old male. We used the 3D reformation computed
tomography (CT) and magnetic resonance imaging (MRI) to delineate the skull defect and the exit pathway of the herniated brain
tissue and to determine the surgical strategy. The frontoethmoidal encephalomeningocele was completely resected via a 1-stage
operation. Neither hydrocephalus nor cerebrospinal fluid (CSF) rhinorrhea was noted after the operation. However, Klebsiella
pneumoniae meningitis was diagnosed 2 weeks after the operation. The infection was cured by the use of meropenem. A good
aesthetic result was achieved. A 1-stage operation is necessary for large frontoethmoidal encephalomeningoceles and should be
performed as early as possible in order to resect the mass, to reconstruct the defect, and to restore the aesthetic appearance with
assistance of 3D reformation CT and MRI. A V-P shunt or extraventricular drainage (EVD) catheter placement needs to be consid-
ered during the operation for large encephalomeningoceles coexisting with hydrocephalus. (Tzu Chi Med J 2006; 18:39-44)

Key words: frontoethmoidal encephalomeningocele, ventriculomegaly, craniofacial anomaly, one stage surgery

hydrocephalus. An operation including mass resection,

INTRODUCTION
Encephalomeningoceles are congenital malforma-
tions characterized by protrusion of the meninges and
brain tissue due to a skull defect. It is 1 form of a neural
tube defect as are anencephaly and spina bifida [1]. There
are 2 main types of encephalomeningocele,
frontoethmoidal and occipital, according to the location
of the defect. The frontoethmoidal type defect, which is
located in the area of the frontal and ethmoidal bones, is
exclusively common in Southeast Asia [2]. There has
been no consensus about the etiology, prognosis-related
classification, or surgical strategy for encephalomenin-
goceles. We present a young man who had a large
frontoethmoidal encephalomeningocele associated with
defect reconstruction, or even V-P shunt placement can
be performed in a single stage. We also review the medi-
cal literature and discuss the etiology, morphologically
oriented classification, encephalomeningocele-related
hydrocephalus, and surgical techniques.
CASE REPORT
An 18-year-old male was admitted to our hospital
due to a protruding soft, non-movable bulging mass with
a wide base of 4.0 × 5.2 × 5.4 cm between the eyes (Fig.
1). According to his father's description, he had been
born with the facial mass. It had slowly enlarged as he
grew up. The physical examination revealed interorbital

Received: April 15, 2005, Revised: May 3, 2005, Accepted: June 1, 2005
Address reprint requests and correspondence to: Dr. Tsung-Lang Chiu, Department of Neurosurgery, Buddhist Tzu Chi
General Hospital, 707, Section 3, Chung Yang Road, Hualien, Taiwan

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S. T. Tsai, C. H. Wang, S. Z. Lin, et al

hypertelorism, long-nose deformity, and inferior can-

thal dystopia. The neurological status demonstrated mild
mental retardation. No congenital anomalies were men-
tioned in his siblings.
The head CT scan and T2-weighted magnetic reso-
nance image (MRI) showed a large unilateral ventricu-
lar dilatation of the left lateral ventricle with a mass ef-
fect (Fig. 2). 3D reformation head skull CT showed a
huge defect in the medial wall of the left orbit. The de-
fect directly communicated with the anterior cranial
fossa. Another defect located on the medial wall of the
right orbit consisted of cystic components (Fig. 3A, 3B).
MRI demonstrated a 4.0 × 5.2 × 5.4 cm midline fa-
cial tumor that had multiple cystic central components Fig. 1. Preoperative photo showing the asymmetrical
and which was pressing the nose to the right. The defec- encephalomeningocele, interorbital hypertelorism,
long-nose deformity, and inferior canthal dystopia.
tive bilateral anterior ethmoidal sinuses communicated

2A 2B

2C 2D
Fig. 2. Preoperative CT image (A) and T2-weighted MRI (B) showing dilated left lateral ventricle and porencephaly. (C,D) The
large unilateral ventricular dilatation also showing a mass effect with anterior displacement of the left thalamus.

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 Surgery and reconstruction of a frontoethmoidal encephalomeningocele

with the content of the facial tumor and with the ante- sion down to the anterior of the tragus. A paranasal skin
rior cranial fossa (Fig. 4). incision was made in a reversed Y-shape because of the
We first placed a V-P shunt (OSVII) through the bilateral herniated masses in order to remove any re-
left Keen's point. Then, we made a bicoronal scalp inci- dundant skin. A typical bifrontal craniotomy and a T-
shaped frontonasal osteotomy were developed to obtain
adequate exposure to the encephalomeningoceles. The
skull defects over the inner wall of the orbit and ethmoi-
dal sinus were located, and the herniated dura sac and

the degenerative brain tissue it contained were resected.

The dura defect was closed with an autologous tensor



fascia lata. The right orbital wall was reconstructed. Its

Fig. 3. (A) 3D CT reconstruction viewed from the left infero-

oblique view demonstrating the external bone defects:
the bilateral nasoethmoid-nasoorbital type (white
arrow), the interorbital hypertelorism, the raised or-
bital roof, and the depressed nasal complex.

Fig. 4. Axial T1-weighted MRI demonstrating a 4.0 × 5.2 ×

5.4 cm midline facial tumor with multiple cystic-like
central components.

Fig. 3. (B) 3D CT reconstruction viewed from the left

superio-postero-oblique view showing the bilateral
internal skull defect (white arrows). Fig. 5. Skull bone graft used to augment the saddle nose.

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S. T. Tsai, C. H. Wang, S. Z. Lin, et al

inferior and medial walls were fashioned from the inner gocele is substantially greater in tropical latitudes, par-
layer of the formal skull bone flap. All areas were fixed ticularly in parts of Southeast Asia. In contrast, the ratio
with microplates. One piece of skull bone graft was also is lower in North America, Australia, and Europe, rang-
used to augment the saddle nose (Fig. 5). We found that ing from 1:2.5 to 1:15 [3].
the brain was quite swollen after the reconstruction. Despite the higher incidence of this congenital de-
Another extraventricular drainage (EVD) tube was in- fect in Southeast Asia, little is known about its etiology
serted into the right lateral ventricle via the right Kocher's and pathogenesis. Some evidence from previous stud-
point for intracranial pressure (ICP) monitoring. ies suggests that environmental factors may be poten-
The intracranial pressure consistently remained at tial causes [1,4,5]. So far, only aflatoxin has been pro-
15 mmHg from initiation of ICP monitoring through the posed as a teratogenic agent for this anomaly [4]. Most
third postoperative day. Brain CT showed shrinkage of cases occur among the lower socioeconomic classes [6].
the dilated left lateral ventricle (Fig. 6). There was no Frontoethmoidal encephalomeningoceles are closely
CSF leakage during this hospitalization. However, 2
weeks after the operation, the patient presented with
Klebsiella pneumoniae meningitis that was cured by in-
travenous meropenem trihydrate (1000 mg, q8h) for 1
month. The postoperative correction successfully elimi-
nated the frontoethmoidal encephalomeningocele, inter-
orbital hypertelorism, and the canthal dystopia. A good
cosmetic result had been achieved 3 months after the
operation (Fig. 7A, 7B).

DISCUSSION

The frontoethmoidal and sincipital regions are the

most-frequent site of meningoceles and encephalomenin-
goceles. Suwanwela and co-workers reported an inci-
dence of 1:5000 [2]. The ratio of the frontoethmoidal
encephalomeningocele to the occipital encephalomenin-

7A

7B
Fig. 7. (A) 3D CT reconstruction delineating the frontoeth-
moidal defect repair with skull bone grafts and nasal
augmentation postoperatively. (B) Postoperative re-
sult showing the paranasal skin incision and how it
Fig. 6. Post-shunting CT image showing shrinkage of the
had healed 3 months later.
dilated left lateral ventricle.

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 Surgery and reconstruction of a frontoethmoidal encephalomeningocele
related to the anomaly, spina bifida, which also results
from neural tube defects. This possibly suggests a role
of folate deficiency in the etiology of encephalomenin-
goceles. Although the reviewed studies did not docu-
ment a relationship between maternal folate levels and
the incidence of the encephalomeningoceles, some evi-
dence suggests different underlying mechanisms be-
tween these 2 forms of neural tube defects [1]. Environ-
mental factors especially nutritional deficiencies may
play a major role in the pathogenesis. This assumption
deserves clarification by additional case control studies.
Frontoethmoidal encephalomeningoceles are known
to have 1 or more skull defects beginning internally at
the junction between the frontal and ethmoidal bones
and ending externally at different locations on the facial
bones. Most patients present with a single perinasal mass
which produces a secondary pressure effect on the sur-
rounding facial bones. Anomalies such as interorbital
hypertelorism, a long nose, and epiphora are all com-
mon secondary sequelae.
Our patient had a bilateral combined nasoethmoid-
nasoorbital external bone defect, which is the most-com-
mon subtype of frontoethmoidal encephalomeningocele.
Rojvachiranonda and colleagues proposed a new classi-
fication for frontoethmoidal encephalomeningoceles by
additionally demonstrating the severity corresponding
to the outcome of this disease [7]. The exit pathway of
brain herniation in the new classification is a blowout
type, which is commonly associated with medium- and
large-sized facial masses. Facial deformities frequently
occur when the diameter of the herniated mass stalk ex-
ceeds 30 mm, as the 40 mm diameter in the present case.
On the other hand, the deforming encephalocele may
disturb growth. Psychological problems related to
encephaloceles can be severe when patients are of school
age. Although the timing of surgery is often beyond the
control of the craniofacial unit, it is recommended that
reconstruction be carried out as early as possible in a
child born with the progressive and blowout type of
encephalomeningocele [8].
Brain malformations are considered manifestations
of the disease. Malformations, including hydrocephalus
(12.0%-28.0%) and porencephaly (4.4%), were men-
tioned in previous studies [7,9,10]. These were also di-
agnosed in our patient.
Obviously, if the encephalomeningocele is large,
there is a higher risk postoperatively of hydrocephalus
and increased ICP, and careful monitoring should be
instituted. The mechanism for the above conditions is
unclear. The encephalocele may in part play the role of
CSF absorption and may have some pressure-buffering
effect. Although numerous studies have discussed post-
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operative hydrocephalus, it is unclear what phenomena
are involved in the possibility of postoperative CSF di-
version [11,12]. The CT of our patient showed a large
encephalomeningocele accompanied by porencephaly
and hydrocephalus before surgery. We, therefore, simul-
taneously performed the VP shunt procedure with exci-
sion of the mass. However, brain swelling was noted
after we reconstructed the anterior skull base. Another
EVD catheter was also put into place. Shrinkage of the
enlarged left occipital horn of the lateral ventricle was
revealed, and our patient did not show increased ICP
signs after the operation.
Instead of the traditional 2-stage correction by pre-
liminary intracranial disconnection and subsequent ex-
tracranial correction of the facial deformity [13], a 1-
stage operation has become the standard treatment [14,
15]. Most craniofacial surgeons prefer the combined
nasal-coronal approach with a frontal craniotomy be-
cause of the wide exposure [14]. On the other hand, the
frontal bone flap can also be remodeled to eliminate the
trigonocephalic bulge [8], repair any external skull
defects, and restore an esthetic appearance such as with
nasal augmentation.
One large series by Mahatumarat proposed another
procedure, the Chula technique, which substitutes a T-
shaped osteotomy of the frontonaso-orbital bone only
for the formal frontal craniotomy [16]. Compared with
previous techniques, this one provides a lower risk of
CSF leakage (2.8%) and meningitis (2.8%). The resected
middle portion of the T-shaped osteotomized bone can
also be used to augment the nose and decrease the dis-
tance between the medial orbital walls. However, our
patient had a large bilateral external facial bone defect
of the combined nasoethmoid-nasoorbital type. The floor
of the anterior cranial fossa was steep, and there was a
relatively long canal from the skull to the face so that
transcranial repair was necessary. We performed a typi-
cal bifrontal craniotomy with a T-shaped osteotomy for
1-stage reconstruction and obtained adequate exposure
in order to perform dura repair and the encephalome-
ningocele resection.
Klebsiella pneumoniae meningitis occurred 2 weeks
after we performed the operation. It is 1 of the most-
common pathogens causing postoperative meningitis.
A cranial fracture with cerebrospinal fluid leakage, a
craniotomy, systemic infections, and ventricular cathe-
ter irrigation all predispose patients to the development
of ventriculostomy-related infections (VRIs). An ex-
tended duration of catheterization is correlated with an
increasing risk of cerebrospinal fluid infections during
the first 10 days of catheterization [17]. There was no
CSF leakage, and we removed the EVD catheter 5 days
QP
S. T. Tsai, C. H. Wang, S. Z. Lin, et al
postoperatively. Meningitis probably resulted from
placement of the EVD or from the operation itself.
CONCLUSIONS
This case belonged to the frontoethmoidal type of
encephalomeningocele whose combined nasoethmoid-
nasoorbital defect is most-frequently observed. The pro-
gressively enlarging blow-out mass requires both a fron-
tal craniotomy and a T-shaped osteotomy as early as
possible to eliminate the mass and repair the skull defect,
according to the new classification proposed by
Rojvachiranonda and colleagues. The associated brain
anomalies of porencephaly and hydrocephalus require
further V-P shunt placement or at least ventricular drain-
age in case of postoperative increased intracranial
pressure.
REFERENCES
1. Botto LD, Moore CA, Khoury MJ, Erickson JD: Neural-
tube defects. N Engl J Med 1999; 341:1509-1519.
2. Suwanwela C, Suwanwela N: A morphological classifi-
cation of sincipital encephalomeningoceles. J Neurosurg
1972; 36:201-211.
3. Humphreys RP: Encephalocele and dermal sinuses. In:
Cheek WR, Marlin AE, eds. Pediatric neurosurgery:
Surgery of the developing nervous system. 3rd ed.
Philadelphia: WB Saunders, 1994, pp 96-103.
4. Aung Thu, Hta Kyu: Epidemiology of frontoethmoidal
encephalomeningocele in Burma. J Epidemiol Commu-
nity Health 1984; 38:89-98.
5. Richards CG: Frontoethmoidal meningoencephalocele:
A common and severe congenital abnormality in South
East Asia. Arch Dis Child 1992; 67:717-719.
QQ
6. Agthong S, Wiwanitkit V: Encephalomeningocele cases
over 10 years in Thailand: A case series. BMC Neurol-
ogy 2002; 2:3.
7. Rojvachiranonda N, David DJ, Moore MH, Cole J:
Frontoethmoidal encephalomeningocele: New morpho-
logical findings and a new classification. J Craniofac
Surg 2003; 14:847-858.
8. Holmes AD, Meara JG, Kolker AR, Rosenfeld JV, Klug
GL: Frontoethmoidal encephaloceles: Reconstruction
and refinements. J Craniofac Surg 2001; 12: 6-18.
9. Songur E, Mutluer S, Gurler T, et al: Management of
frontoethmoidal (sincipital) encephalocele. J Craniofac
Surg 1999; 10:135-139.
10. Mahapatra AK, Tandon PN, Dhawan IK, Khazanchi RK:
Anterior encephaloceles: A report of 30 cases. Childs
Nerv Syst 1994; 10:501-504.
11. Simpson DA, David DJ, White J: Cephaloceles:
Treatment, outcome, and antenatal diagnosis. Neuro-
surgery 1984; 15:14-21.
12. Fuente del Campo A, Escanero Salazar A, Baldizon
Recio N, Dimopulos A: Transfacial surgical treatment
and anthropometric considerations of frontoethmoidal
meningoencephaloceles. Ann Plast Surg 1989; 23:377-
389.
13. Tandon PN: Meningoencephalocoeles. Acta Neurol
Scand 1970; 46:369.
14. David DJ: Cephaloceles: Classification, pathology, and
management-A review. J Craniofac Surg 1993; 4:192-
202.
15. Forcada M, Montandon D, Rilliet B: Frontoethmoidal
cephaloceles: Transcranial and transfacial surgical
treatment. J Craniofac Surg 1993; 4:203-209.
16. Mahatumarat C, Rojvachiranonda N, Taecholam C:
Frontoethmoidal encephalomeningocele: Surgical cor-
rection by the Chula technique. Plast Reconstr Surg
2003; 111:556-565.
17. Lozier E, Sander Jr: Ventriculostomy-related infections:
A Critical review of the literature. Neurosurgery 2002;
51:170-182.
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