Rare Combination of Frontonasal and Bilateral Naso-orbital Encephaloceles

Abstract

Encephaloceles, while a common entity affecting 1:4000 live births, typically occur in the occipital region. Encephaloceles involving the frontal region comprise only 15% of all cases. Naso-orbital encephaloceles are rarely seen. Our case profiles a child born at term with an atrial septal defect (ASD), micrognathia, cleft lip, and frontonasal as well as bilateral naso-orbital encephaloceles. At birth the encephaloceles were undetected. During the cleft palate pre-operative preparation, the bilateral naso-orbital encephaloceles were diagnosed as dacrocystoceles for which the child underwent surgical repair. Misdiagnosis and loss to follow up lead to delayed surgical treatment until the child was almost two years of age; the right eye was near complete closure due to the increasing size of the encephalocele. This case highlights the importance of meticulous radiologic interpretation of midline nasal masses, as a correct diagnosis impacts clinical management and directs surgical repair.

CT Imaging in Pediatric Nasofrontal Cephaloceles

“…In the latter series, all patients were males and underwent surgery. In our study we found a unique case of complex nasofrontal and nasoorbital cephalocele, similar to the one reported by Alexander [16].…”

“…We have compiled observational data in a series of children with nasofrontal cephaloceles in Burkina Faso, in order to describe their CT features and associated facial dysmorphic appearances. Our series accounts among the rare reports of CT-facial correlations of craniocephalic malformations [4,[8][9][10][11][12][13][14][15][16][17]. We had the opportunity to perform such a study because of local conditions which include the lack of prenatal care and prevention as well as adequate surgery for malformation repair.…”

Intraorbital Encephalocele Presenting with Exophthalmos and Orbital Dystopia : CT and MRI Findings

“…The greater wing of the left sphenoidal bone was hypoplastic and the left superior orbital fissure was enlarged on 3D CT examination in our case, consistently with the congenital orbital encephaloceles which does not accompany with a few syndromes in literature, although it is quite frequent. As in our case, there was the right sphenoidal wing dysplasia on 3D CT in a 22-year-old patient described by Sharma et al 8) and the patient had progressive exopthalmus since 7 years of age and the right temporal lobe was herniated to orbita 1 , 3) . MRI provides definite data about the localization of the bone defect, the content of the encephalocele and surrounding tissues (5).…”

“…Encephaloceles are seen in the rate of one in every 4000 live births and defined as the protrusion of the intracranial structures 1 , 3) . Most of them occur sporadically and do not accompany to hereditary syndromes 6) .…”

“…Different to a meningocele, the bulging portion of which contains only cerebrospinal fluid and overlaying dura, an encephalocele is a cystic malformation of the brain tissue herniating through bone 6) . Encephaloceles occur at a rate of one per 4000 live births worldwide 1) . Encephaloceles are classified according to their localizations in the cranium.…”