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At least 127 records · Page 7Linked to original sources
PubMed1997

Encephalocele: experimental model. Morphogenesis, pathogenesis and clinical correlations discussion.

RESEARCH OBJECT: This study intends to consider an encephalocele experimental model, obtained in embryonate eggs, treated in a post-neurulation phase with chemical teratogens. This study intends to point out possibility that pathogenic process, which have determined a malformation, is referable to an original defect of embryonic cranial coatings development and that neurulation defect is secondary. EXPERIMENTAL PLAN: Chick and duck embryonate eggs have been used. They have been inoculated in their development phase with known chemical teratogens, as Dintoina and Blue Trypan. A controlling group has been inoculated with physiological solution and, then, it has been followed till the hatching. The experimental group has been undergone to artificial hatching, according to prearranged conditions and it has been analysed during different phases of its development. MEASURES: Four cranioencephalic malformations have been obtained: three cases of encephalocele and one case of exencephalia. The individual pathological compounds have been studied under the morphological and histopathological profile. The skull base dimensions have been taken and then compared with the controlling group ones. CONCLUSIONS: In the light of experimental data, some considerations have been undertaken considering the pathogenetic hypothesis findable in medical literature, stressing the possibility that encephalocele may be arranged as a post-neurulation defect and that, for taxonomic aims, it has to fit in a different group within cranial disraphims.

Animals↗
PubMed1999

Unusual presentations of middle fossa encephaloceles: report of two cases.

Basal encephaloceles are rare occurrences with occult and often varied presentations. Frequently, the need for surgical treatment is not clear to the patient or the physician, leading to potentially fatal complications. We report the case of a 44-year-old woman with an 8-year history of nonspecific complaints who presented to us with trigeminal neuralgia. Also, an unsual case is presented of a 60-year-old man with bilateral middle fossa encephaloceles who suffered acutely from widespread pneumocephalus acting as a mess lesion. Both are cases of nontraomatic middle fossa encephaloceles, which are of perficular interest due to the age of the patients, their atypical presentations, and the asseciated complications.

Case Reports↗
PubMed1995

Atypical retinochoroidal coloboma in patients with dysplastic optic discs and transsphenoidal encephalocele.

OBJECTIVE: To evaluate the clinical significance of V- or tongue-shaped infrapapillary retinochoroidal depigmentation in association with dysplastic optic discs. METHODS: Clinical data from all patients with V- or tongue-shaped infrapapillary retinochoroidal depigmentation and dysplastic optic discs were evaluated retrospectively. RESULTS: Five patients with this atypical colobomatous anomaly had transsphenoidal encephalocele. A sixth patient had an ectopic midline pharyngeal mass with no skull-base defect. CONCLUSION: In patients with optic disc dysplasias, the finding of this V- or tongue-shaped retinochoroidal pigmentary anomaly should prompt neuroimaging to look for transsphenoidal encephalocele.

Adolescent↗
PubMed1984

Spontaneous encephalocele of the middle ear.

Mature glial tissue was seen in the middle ear of a patient with no previous pathologic precedents, and a clinical diagnosis of serous otitis media was made. That anomaly was detected, and its corresponding clinical, roentgenographic, and pathologic findings are described. The results led us to diagnose it as a congenital (spontaneous) encephalocele of the middle ear. To our knowledge, other case of pure congenital encephalocele of the middle ear has been reported.

Brain Neoplasms↗
PubMed1986

Spontaneous encephalocele of the temporal bone.

Postsurgical and posttraumatic encephaloceles of the tegmen tympani and tegmen mastoideum are well-recognized occurrences. Less frequently recognized, however, is the spontaneous occurrence of an encephalocele associated with cerebrospinal fluid otorrhea or rhinorrhea. This cerebral herniation into the middle ear and the mastoid cavity in adults is associated with a loss of both bony and dural support, and surgical management must deal with both the cerebrospinal fluid leak and herniation of the brain.

Adult↗
PubMed2003

Apparent encephalocele in twin fetus papyraceus with twin-reversal arterial perfusion.

An apparently iniencephalic or exencephalic monoamniotic monochorionic female twin fetus, delivered as a fetus papyraceus at 28 weeks of gestation, had severe anomalies of the central nervous system and spine, including occipital encephalocele with a defect of the occipital bone. The encephalocele contained a spherical mass of autolyzed brain tissue without identifiable cerebellum or vermis. The cervical canal was widely patent dorsally, there were severe bony anomalies, including agenesis and fusion of vertebrae in the cervicothoracic spine. The upper limbs were absent. All organs were hypoplastic and autolyzed. The hypoplastic heart had three chambers. The placenta was monochorionic and monoamniotic; barium injection showed a twin-reversal arterial perfusion and entanglement of the umbilical cords.

Abnormalities, Multiple↗
PubMed1985

The differential prenatal diagnosis of cystic hygromata and encephalocele by ultrasound examination.

The difficulty in distinguishing an encephalocele from a cystic hygroma has recently been highlighted by five patients referred to our unit with an incorrect diagnosis of cystic hygroma. A review of the literature revealed four other cases of misdiagnosis. Based on review of 46 patients with one or other diagnosis, we have attempted to establish guidelines to aid diagnosis. We have always been able to identify the bony defect in the fetal vault in cases of encephaloceles. The pattern of the septa is usually characteristic in cystic hygroma.

Diagnosis, Differential↗
PubMed1989

False-negative amniotic fluid acetylcholinesterase in a case of meningo-encephalocele.

We describe a patient with a significantly elevated serum alphafetoprotein (AFP) concentration at 17 weeks of gestation, who showed only a marginally increased amniotic fluid AFP and lacked the second rapidly migrating band of acetylcholinesterase electrophoresis. Ultrasound examination revealed an encephalocele and ventriculomegaly. Autopsy showed that the encephalocele was not covered by skin.

Acetylcholinesterase↗
PubMed2003

First-trimester diagnosis of osteogenesis imperfecta associated with encephalocele by conventional and three-dimensional ultrasound.

To illustrate the three-dimensional sonographic features of a rare genetic disorder, we report on prenatal diagnosis of osteogenesis imperfecta congenita associated with encephalocele at 13 weeks of gestation, using conventional and three-dimensional ultrasound. Because the parents were first-degree cousins and on the basis of the family history, a recessive autosomal inheritance was suspected. Of seven previous pregnancies, five were unaffected and two had been terminated in the second trimester owing to a similar abnormality (one affected boy and one affected girl). In the case we present, the diagnosis was made on the basis of two-dimensional ultrasound performed by physicians aware of the history; the quality of three-dimensional ultrasound imaging suggests that this technique might have contributed toward establishing a precise diagnosis in the absence of a positive family history. Besides, the global view provided by three-dimensional surface-rendering images made the parents more confident of the accuracy of the diagnosis. Although osteogenesis imperfecta congenita is generally considered as autosomal dominant, the case we report suggests that it may be inherited in a recessive autosomal fashion at least when associated with encephalocele. Three-dimensional ultrasound confirmed the conventional two-dimensional examination and was helpful in convincing the parents of the accuracy of the diagnosis.

Abnormalities, Multiple↗
PubMed1989

An unusual case of temporal encephalocele.

An unusual case of temporal encephalocele is presented in this report. A large temporal mass was detected prenatally by ultrasound and following delivery by caesarian section. It was determined to be a large temporal encephalocele with extensive invasion into the subtemporal, facial and cervical regions. This case is presented because of the unique pathology of this congenital malformation and the extensive surgery that was required. The management and the clinical and radiological features are discussed.

Encephalocele↗
PubMed1990

Management of anterior encephalocele.

The authors report their experience with anterior encephalocele, comprising 11 congenital and 11 traumatic cases. Unlike occipital encephaloceles, anterior lesions are usually associated with normal mental development. Surgery aims to restore normal anatomy with repair of the defective dura, bone and skin. Indications, surgical methods and outcome are discussed.

Child↗
PubMed1994

Anterior encephaloceles: a report of 30 cases.

Thirty cases of anterior encephalocele treated in our centre over an 18-year period (from 1973 to 1990) are presented. At the time of surgery over 60% of the patients were under the age of 2 years, and 40% were aged under 1 year. Only one child was over 10 years of age. Twenty-six patients had the fronto-ethmoidal type of defect, while two each had frontonasal- and naso-orbital-type lesions. Twenty-five children had varying degrees of hypertelorism. Four had an enlarged head and four microcephaly. In 26 patients one-stage repair of the encephalocele and reconstruction of the orbits was undertaken. A ventriculoperitoneal shunt was performed prior to definitive surgery in three patients with gross hydrocephalus. There was no postoperative mortality. Six patients had postoperative CSF rhinorrhoea, three of them requiring a lumboperitoneal shunt. This study highlights the role of one-stage repair of this defect.

Age Factors↗
PubMed1976

Acquired basal encephalocele.

The radiodiagnostic signs of congenital basal encephalocele are well known. The case history described here shows that a long existing, acquired, traumatic transethmoidal encephalocele can cause the same roentgen appearance. The differences between the congenital and recent posttraumatic forms are indicated.

Adolescent↗
PubMed2001

Craniofacial surgery for leaking encephalocele in a newborn baby.

A newborn baby with a leaking frontoethmoidal encephalocele is reported. The 1-day-old baby was referred to our center with a swelling on the nose leaking CSF. The child was the third baby of a consanguinous marriage. The baby had a swelling over the nose, 8 cm in diameter. The center of the swelling looked unhealthy, with a small raw area seeping CSF. CT scan showed a swelling containing tissue isodense with normal brain. The bone window and the 3D CT revealed a basal bone defect in front of the crista galii and an outer bone defect at the frontonasal junction over the nasion. The baby was operated on the 5th day after birth. One-stage repair of the encephalocele was performed, along with correction of hypertelorism and bone grafting over the forehead. The baby withstood the surgery well and was discharged from the hospital on the 10th postoperative day.

Cerebrospinal Fluid↗
PubMed1975

Cryptophthalmos syndrome with basal encephaloceles.

A 2,144-g white girl was born with absence of the right ear and eye, cleft lip and palate, two basal encephaloceles, tricuspid atresia, ventricualr and atrial septal defects, detransposition of the great vessels, right aortic arch, and aberrant right subclavian artery. Through an oval defect in the center of the sphenoid bone, soft tissue protruded into the right nasopharynx. The medial portions of the roof of both orbits and the cribriform plate were absent and soft tissue protruded through this bony defect. Basal tomography was required to demonstrate the encephaloceles, which should be suspected in any child with a median cleft syndrome, a flat broad nasal root, and hypertelorism.

Abnormalities, Multiple↗
PubMed1991

A primary encephalocele culture yields a pure population of human astrocytes.

In order to develop a reliable source of human astrocytes for in vitro studies, we established a primary explant culture of a human encephalocele. This culture yielded a population of cells which were karyotypically normal, morphologically resembled astrocytes, expressed glial fibrillary acid protein, and responded mitogenically to exogenous growth factors. We conclude that white matter derived from human encephaloceles can be used to generate pure populations of normal astrocytes.

Astrocytes↗
PubMed1983

Transsphenoidal encephaloceles.

Transsphenoidal encephaloceles are rare congenital anomalies that may be immediately apparent in infants that present with multiple cranial midline defects. They should also be suspected in patients presenting with cerebrospinal fluid rhinorrhea, an epipharyngeal soft tissue mass, a visual defect, or an endocrinologic disturbance, especially when associated with midfacial and optic nerve anomalies. Plain x-ray films of the skull may show absence of the sellar floor and a soft tissue mass; the diagnosis is confirmed by computed tomography scanning. Surgical therapy may be indicated for persistent cerebrospinal fluid rhinorrhea, symptomatic epipharyngeal respiratory distress, or progression of neurological deficits. Two cases of transsphenoidal encephalocele are reported.

Adult↗
PubMed1989

Tecto-cerebellar dysraphia with occipital encephalocele.

We report four cases with the rare syndrome of tecto-cerebellar dysraphia with occipital encephalocele. The clinical features seen in these patients included episodic tachypnea and irregular breathing, opsoclonus, ataxia, marked hypotonia of the limbs, coloboma, and polydactyly. All four patients had midline occipital encephalocele. The cranial computed tomography scan showed partial to total agenesis of the vermis with a large communication between cisterna magna and the fourth ventricle. The computed tomography scan also showed partial deficiency of the midbrain tectum. We discuss the clinical and radiological findings and review the literature.

Cerebellum↗