Unidad Obstetrica Quirurgica, Hospital General de la S. S. A. de Acapulco, Guerrero, Mexico. Instituto Mexicano de Diagnostico por Imagen (I. M. D. I.). Facultad de Medicina, Division de Posgrado de la Universidada Autonoma de Guerrero, Mexico.
Definition
A cephalocele is a herniation of brain contents through a defect in the skull. The anterior cephalocele refers to a midline frontonasal herniation of brain and meninges throughout the skull defect.
Prevalence
0.5:10,000 live births.
Etiology
Sporadic, familiar or associated with other malformations.
Pathophysiology
Failure of closure of the rostral neuropore
Associated anomalies
Ocular hypertelorism, nasal widening, complex facial malformations as cleft palate, lip and median nasal fissure, spina bifida, agenesis of the corpus callosum, ocular malformations and ventriculomegaly, deformity of the frontal bones, microcephaly, hydrocephaly.
Differential diagnosis
Teratomas, gliomas, dermal sinus cysts, facial hemangioma, lacrimal duct cyst, orbital duplication and proboscis.
Prognosis
Depending on the contents of the cephalocele sac.
Sonographic findings
The frontal encephalocele recognizable as a mass of the fetal brain protruded fetal brain placed between the bregma and the anterior part of the ethmoid bone. The majority extents into the root of the nose. The appearance on ultrasound examination and mass, a solid mass with components. We can also recognize the skull defect. Associated anomalies include .
Case report
A 22-year-old-woman (G3P2) with unremarkable history, referred to our department at 40 weeks for routine ultrasound. Our investigation revealed irregularly shaped skull with solid brain mass protruding anteriorly to the forehead - anterior cephalocele. No other malformations were seen. Postnatal investigation confirmed our investigation. Here are some of the ultrasound images we obtained.
Images 1, 2. 40 weeks of pregnancy - transverse sections through the skull showing frontal encephalocele (a mass of brain tissue protruding anteriorly).