Encephaloceles in Nigerian Igbos.
A survey of surgical specimens submitted to a central laboratory suggests that the Igbos of Nigeria are a further ethnic group in whom the anterior encephalocele is comparatively common.
Encephalocele↗
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Recurrent bacterial meningitis occurring five years after closed head injury and caused by an intranasal post-traumatic meningo-encephalocele.
A case of atypical presentation of a post-traumatic intranasal meningo-encephalocele is described in a patient with a history of recurrent bacterial meningitis occurring 5 years after closed head injury. The usefulness of the CT and MRI findings in diagnostic evaluation of this lesion is emphasized.
Adult↗
Skeletal morphology of anterior encephaloceles defined through the use of three-dimensional reconstruction of computed tomography.
We have used three-dimensional reconstruction of computed tomography to study the morphology of anterior encephaloceles. The findings based on living patients rather than cadaver specimens confirm previous findings suggesting 'blow out' defects which displace otherwise normal osseous structures.
Child↗
Functional and anatomic recovery in the monkey brain following excision of fetal encephalocele.
Teratogen-induced herniation of the occipital lobes (encephalocele) in monkey fetuses was repaired by amputation and cranioplasty in utero or postnatally. The in utero-treated animals had normal vision, CT scans indicated normal brain density, and autoradiographic visualization of the striate cortex by the [14C]-2-deoxyglucose mapping revealed almost complete functional and anatomic recovery. The animals treated postnatally were blind, and exhibited significant structural deficit in the operated area.
Age Factors↗
Occipital encephalocele associated with mediastinal enteric duplication cyst.
Enteric duplication is a rare developmental anomaly. This anomaly can be seen anywhere along the gastrointestinal tract, and less than 2% of the cases extend to the mediastinum. Developmental neuroectodermal and cutaneous ectodermal abnormalities, especially vertebral midline fusion abnormalities, are frequently associated with enteric duplications. We report a case of a newborn with occipital encephalocele, also having a mediastinal enteric duplication cyst. The clinical course of this case is described in detail to emphasize the occurrence of life-threatening complications of these associated developmental abnormalities during the treatment of the primary pathology in the neurosurgery practice. In the literature, we failed to demonstrate any co-existence of these two developmental abnormalities and review the embryogenesis of the association of these congenital malformations presented here.
Abnormalities, Multiple↗
Occipital encephalocele associated with a dermoid cyst.
The case of a neonate with a dermoid cyst originating within an occipital encephalocele is reported. The finding of the tumor was made during the routine repair of the meningoencephalocele. To our knowledge this is the first time that this association has been described.
Brain Neoplasms↗
Occipital encephalocele--a case of conjoined twinning?
A case of an occipital encephalocele is described. Morphological analysis of the surgical specimen suggests that the case represents an instance of incomplete conjoined twinning.
Brain Diseases↗
[Anatomical classification of anterior encephaloceles].
The authors present an anatomical classification of the anterior encephaloceles. The importance of a topographic knowledge of such anomalies in their diagnosis and treatment is emphasized in order to avoid complications during the investigation of a cranio-facial protrusion.
Encephalocele↗
Optic nerve compression from a basal encephalocele.
A woman developed headaches, transient visual obscurations, anosmia, and decreased visual acuity. Ocular examination showed bilateral pulsatile proptosis and disc edema with choroidal folds. Standardized ophthalmic echography showed absence of bony orbital roofs, prominent dural pulsations, direct apposition of brain parenchyma and orbital tissues, and echographic signs suggesting bilateral optic nerve compression. CT and MRI showed a large defect in the floor of the anterior cranial fossa. The cribriform plate, both orbital roofs, and sphenoid bones were displaced by a large basal encephalocele. Clinical improvement followed reconstruction of the anterior cranial fossa and decompression of both optic nerves.
Adult↗
Spontaneous cerebrospinal fluid rhinorrhea associated with dysplastic optic discs and a basal encephalocele: case report.
A 24-year-old man presented with a history of recurrent meningitis secondary to cerebrospinal fluid (CSF) rhinorrhea. The patient had bilateral optic disc dysplasia in association with a basal encephalocele, but had no midline facial anomalies. The syndrome is rare, and this is the first report of a patient with this disorder in the absence of facial or radiographic anomalies. The case serves to emphasize the importance of funduscopic examination in patients with spontaneous CSF rhinorrhea or recurrent meningitis, and stresses the value of Dextrostix examination of nasal secretions in the evaluation of suspected CSF rhinorrhea.
Adult↗
Nasal glioma and encephalocele: two separate entities. Report of two cases.
The term "nasal glioma" is a confusing misnomer as it implies a neoplastic condition with malignant potential, which it is not. Nasal glioma is a rare development abnormality and should be differentiated from glioma, which is a malignant tumor of the brain, and from a primary encephalocele, which is herniation of the cranial contents through a bone defect in the skull, through which it retains an intact connection with the central nervous system. Two cases of nasal glioma, one with and one without intracranial connections, are described and the literature is reviewed.
Child, Preschool↗
Isolated orbital roof fracture with traumatic encephalocele.
Several months after a fall down a flight of stairs, a one-year-old girl developed downward displacement and pulsation of the left eye. Vision and ocular motility were not impaired and the child remained neurologically normal. Computed tomography with metrizamide cisternography demonstrated herniation of brain tissue through a traumatic defect in the roof of the orbit. The orbital rim and other craniofacial bones were intact. Repair of the growing fracture found at operation restored normal appearance without disturbing function. To our knowledge, this is the first documented case of encephalocele associated with an isolated fracture of the orbital roof due to blunt head trauma.
Encephalocele↗
The role of sonography in the prenatal diagnosis and management of encephalocele.
Sonography has proved of great benefit in the diagnosis and management of certain fetal structural anomalies, including encephalocele. Active intervention may be guided by real-time sonography. It is considered that prenatal management will become increasingly common for a number of such anomalies.
Adolescent↗
A potential pitfall in the ultrasonographic diagnosis of fetal encephalocele.
Abnormalities of the fetal neural axis may be diagnosed prenatally utilizing ultrasonography. Attempts to increase the sensitivity of ultrasonography in the detection of these anomalies run the risk of increasing the false-positive diagnoses. Since the prenatal diagnosis of neural tube defects significantly affects the clinical management of the pregnancy, a false-positive diagnosis of neural tube anomaly must be avoided. The authors describe several cases of patients with polyhydramnios in which the fetal ear protruded into the amniotic fluid, simulating the appearance of an encephalocele on ultrasonography.
Adult↗
Atretic fronto-ethmoidal encephalocele.
This case report describes a 4 months old infant with a lump on forehead since birth. The lump turned out to be an atretic encephalocele. Herniation of brain matter through scalp suture lines during intrauterine life is a rare happening. Congenital and environmental factors have been implicated.
Biopsy, Needle↗
Dysgenesis of the internal carotid artery associated with transsphenoidal encephalocele: a neural crest syndrome?
We describe two original cases of internal carotid artery dysgenesis associated with a malformative spectrum, which includes transsphenoidal encephalocele, optic nerve coloboma, hypopituitarism, and hypertelorism. Cephalic neural crest cells migrate to various regions in the head and neck where they contribute to the development of structures as diverse as the anterior skull base, the walls of the craniofacial arteries, the forebrain, and the face. Data suggest that the link between these rare malformations is abnormal neural crest development.
Adult↗
Spontaneous cerebrospinal fluid leakage and middle ear encephalocele in seven patients.
Isolated cases of spontaneous cerebrospinal fluid (CSF) leakage with and without middle ear encephalocele have been reported. These leaks are usually accompanied by episodes of recurrent meningitis, hearing loss, or chronic headache. In this article, we report seven new cases of spontaneous CSF leakage. Six of these patients had conductive hearing loss and serous otitis media, and three had recurrent meningitis. Prior to a definitive diagnosis, six patients had received myringotomy tubes, which produced profuse clear otorrhea. Three patients had positive beta-2 transferrin assays. Computed tomography and magnetic resonance imaging confirmed a defect in the temporal bone tegmen. A combined transmastoid and middle fossa surgical approach with a three-layer closure was used to repair the tegmen defect. All patients had a lumbar drain placed prior to surgery. In addition to describing the seven new cases, we review the history of CSF leakage and discuss diagnostic methods, surgical findings, and our recommendations for management.
Adult↗
[The differential diagnosis of the Meckel syndrome and the Ellis-van-Creveld syndrome with encephalocele (author's transl)].
The differential diagnosis of Meckel's syndrome and Ellis-van-Creveld's syndrome with encephalocele is summarized utilizing two own observations. Radiographic criteria are of special importance. In EvCS typical signs of chondrodysplasia are present whereas in MS we found pelvic changes similar to Down's syndrome and striking rhomboid deformities of the tibiae. Clinically relevant is ectodermal dyplasia in EvCS, especially, when cystic kidneys in MS are not palpable.
Diagnosis, Differential↗