Fronto-Ethmoidal Meningoencephalocele : A Case Report

TAF. Benitaryani1, PD. Utomo2

1Medical Doctor, Faculty of Medicine, Universitas Kristen Indonesia 2Department of Radiology, Faculty of Medicine, Universitas Gadjah Mada, Sardjito Hospital

ABSTRACT : A 5-months-11-days-old girl is demonstrated who was born with a large swelling
(fronto-ethmoidal meningoencephalocele) at the root of the nose. Following radiographic investigations
of fronto-ethmoidal meningoencephalocele is made by Head Non-contrast MSCT Scan for confirmatory
diagnosis.
Keywords : Encephalocele, Frontoethmoidal Meningoencephalocele, MSCT Scan
RESULTS: Head Non-contrast multi-slice computed tomography scan (Non-contrast MSCT Scan) was
taken in axial, coronal, and sagittal planes. The result showed hypodense lesions in both anterior orbital
cavities, and on the anterior cranial fossa – especially on the left, with parenchymal components of the
brain forcing both ocular bulbs to their anterolateral sides, narrowing the left lateral ventricle and
causing a midline shift about 6.17 mm to the right anterior cranial fossa. A widened subarachnoid space
in the right anterior cranial fossa is communicated with the septum pellucidum cavity.

BACKGROUND: Encephalocele is herniation of intracranial contents through the neural tube defect.
These lesions are classified by their contents and location. Data on the prevalence of encephaloceles
range from 1 in 2500 to 1 in 25000 normal births. Meningocele is a cerebrospinal fluid (CSF)-filled
hemial sac, lined and covered by meninges. Meningoencephaloceles may be subdivided into occipital,
parietal, basal and syncipital. The syncipital group has been divided into three types which comprise
fronto-ethmoidal (subdivided by facial skeleton exit site into naso-frontal, naso-ethmoidal and naso-
orbital), interfrontal and those associated with craniofacial clefts. This paper reports a case of congenital
fronto-ethmoidal meningoencephalocele which is rare.
CASE REPORTS: A 5-months-11-days-old girl, with major complaint of frontal swelling between the
eyes since birth and progressively enlarged in size. The swelling inflated when crying or straining, and
sometimes deflated according to her intracranial pressure at the moment. When hospitalized, the
patient once vomited unexpectedly.
Figure 1. The smooth and rounded swelling seen over the nose of the patient
DISCUSSION: The category of congenital lesions includes the rare developmental midline nasal
masses in children.1 Such anomalies may be understood by studying their anatomy and embryologic
development. Potential intracranial extension or connection, if present, has implications for treatment
and prognosis. In patients with this type of lesion, magnetic resonance (MR) imaging is essential to
characterize and map the lesions. High-resolution computed tomography (CT) may be helpful in older
children, but a lack of ossification at the base of the skull in very young children may simulate defects.
Development of the nose and the nasal cavities occurs between 3 and 10 weeks of gestation.2,3 By the
10th week, the palatal shelves and the inferior septum fuse to form the secondary palate.2
Figure 4. Diagrams show normal development of the nasal cavities. Black and white areas indicate the
turbinate anlagen. n = nasal cavity, o = oral cavity, p = palatal shelves, s = septum, t = tongue. (a) At 9
weeks of gestation, the cartilaginous nasal septum directly overlies the buccal cavity. (b) A few days
later, the inferior nasal cavity widens and the palatal shelves assume a more horizontal orientation. (c)
Close to the 10th gestational week, the palatal shelves begin to approach each other, the primitive
turbinates start to take shape, and the nasal septum begins to descend. (d) At the end of the 10th week,
the palatal shelves fuse at the midline to form the secondary palate, which fuses superiorly with the nasal
septum. The oral and nasal cavities now are separated, and the turbinates are almost fully formed.
Figure 5. Diagrams show normal development of the frontonasal region. (a) Early in gestation, the frontal
bone (superior arrow) is separated from the nasal bone (inferior arrow) by a small fontanelle or fonticulus
frontalis (F). A small remnant of the prenasal space remains anterior and inferior to the intracranial
foramen cecum (C). Beneath these structures lies the cartilaginous nasal septum (S) or capsule. (b) With
continued growth, the frontal and nasal bones fuse, creating the frontonasal suture between them. The
foramen cecum (arrowhead) becomes shallower, and the crista galli (*) forms. The septum (S) grows
anteriorly and fuses with the nasal bones, obliterating the prenasal space.
Smith et al. states that cysts containing brain tissue, even if a link with the brain can not be
demonstrated, should be regarded as encephaloceles.5 As in the cases reported by various authours, our
patient had a naso-ethmoidal meningoencephalocele of the fronto-ethmoidal type because the hernial
opening was situated more laterally between the frontal and nasal bones.6,7 Because of their position
and size, fronto-ethmoidal meningoceles and meningoencephaloceles cause alterations and distortions
of the surrounding facial structures such as displacement of the medial orbital walls, the entire orbits,
telecanthus and hypertelorism.8 Patients with this malformation demonstrate swellings of varying size in
the glabella- nasal region. These swelling may be sessile or pedinculated. On palpation the mass may be
solid and firm or soft and cystic. The contents of the sac mostly consists of glial tissue, often infiltreted
with fibrous trabeculae. The skin over the mass may be normal in appearance, thin and shiny or thick
and wrinkled. Hyperpigmentation and hypertrichosis may be noted. Visual acuity may be decreased.
Strabismus and lacrimal obstructions, resulting in epiphora and/or dacryocytitis can be observed .9
Differential diagnosis should be made from traumatic encephalocele, ethmoid-frontal sinus mucocele,
neurinoma, hemangioma and glioma. Diagnosis or clinical recognition might not be easy if the cerebral
hernia is confined mainly within the nose. A common feature of encephaloceles that enter the nasal and
nasopharyngeal space is impairment of nasal airway. These lesions are easily mistaken clinically for nasal
polyps or tonsiller hyperplasia.
Confirmatory diagnosis of frontoethmoidal meningoencephalocele is made by Head Non-contrast MSCT
Scan. It helps to understand about bony abnormalities in the skull and soft-tissue components of the
brain.

CONCLUSION: We reported a case of Frontoethmoidal Meningoencephalocele. Head Non-contrast

Figure 2. Midsagittal Non-contrast MSCT Scan
image shows a nasoethmoidal encephalocele (E)
under the nasal bone. A stalk (*) connects the
encephalocele with the intracranial brain.
Figure 3. Head Non-contrast MSCT Scan showed hypodense
lesions in both anterior orbital cavities, and on the anterior
cranial fossa – especially on the left, with parenchymal
components of the brain forcing both ocular bulbs to their
anterolateral sides, narrowing the left lateral ventricle and
causing a midline shift about 6.17 mm to the right anterior
cranial fossa. A widened subarachnoid space in the right
anterior cranial fossa is communicated with the septum
pellucidum cavity.
The nasofrontal fontanelle, or fonticulus frontalis, temporarily separates the nasal and frontal bones (Fig
5a). Simultaneously, the transient prenasal space separates the nasal bones and the underlying
cartilaginous nasal capsule.4 A diverticulum of dura mater extends from the anterior cranial fossa
through a foramen into the prenasal space. This diverticulum briefly contacts the skin at the tip of the
nose before retracting back into the cranium. The nasal and frontal bones fuse, obliterating the
fonticulus frontalis and forming the frontonasal suture (Fig 5b). The prenasal space becomes smaller
with growth of the adjacent bone structures and eventually is reduced to a small blind canal (the
foramen cecum of frontal bone) anterior to the crista galli. Finally, the foramen cecum is filled by fibrous
tissue.
Encephaloceles have a direct connection to the intracranial cavity, and this connection must be
obliterated to avoid potential future meningitis and abscess formation. The neurodevelopmental
outcome depends on the state of the brain and the size of the encephalocele; larger lesions generally
are accompanied by more numerous intracranial anomalies and incur a worse prognosis.
MSCT Scan is an important imaging modality to help the radiologist and clinician determine a specific
diagnosis.
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