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At least 847 records · Page 47Linked to original sources
PubMed1982

Triamcinolone-induced alteration of the notochordal-basichondrocranial relationship in the rhesus monkey.

Triamcinolone-induced alteration of the notochordal-basichondrocranial relationship in the rhesus monkey. Timed-mated pregnant rhesus monkeys received triamcinolone acetonide (10 mg/kg, IM) during early organogenesis. Treated and control embryos and fetuses were removed by hysterotomy on days 35, 42, 50, 60, and 70. All treated cases 42 days or older exhibited craniofacial and brain defects. Microscopic examination showed abnormal notochordal folding and a shortened cranial base in the 35-day embryos prior to any observed CNS involvement. Bends, deviations, focal proliferation, and persistence of the notochord and altered cranial base were observed in older cases. This study supports the hypothesis that encephalocele and related CNS disorders could be secondary to a primary paraxial mesodermal insufficiency.

Animals↗
PubMed1981

[Prenatal diagnosis of Meckel-Gruber's syndrome by ultrasound (author's transl(].

The article reports on the ultrasonographic diagnosis of a Meckel-Gruber's syndrome in the 17th week of pregnancy. This malformation syndrome is characterized by the triad: encephalocele, polycystic kidneys, and polydactyly. This congenital disease is compared with malformation syndromes which are distinguishable by differential diagnosis, such as Potter's renofacial syndrome. Thorough ultrasonic examination will be able to detect the severe renal changes as well as the neural tube defects associated with Meckel-Gruber's syndrome. Further diagnostic aids for detecting neural tube defects are, in the first place, the alpha-fetoprotein values obtained from the serum of the mother and/or the amniotic fluid, as well as the determination of acetyl cholinesterase.

Adult↗
PubMed1981

Is Bendectin a teratogen?

We studied the first-trimester exposure to Bendectin for several major categories of birth defects among infants ascertained through the Metropolitan Atlanta Congenital Defects Program from 1968 to 1978. No associations were found between any of these defect categories and Bendectin exposure. An association, however, was found for the amniotic bands cases--a subgroup of limb reductions. We stratified our data to reflect the 1976 change in formulation when one of the three ingredients was removed. An association was found between esophageal atresia and the three-ingredient product and another between encephalocele and the two ingredient product. These statistical associations do not establish a causal relationship between Bendectin and the birth defects we studied. Furthermore, even if the associations are causal, the implied risk is extremely low, approximately one in 1,000 births.

Abnormalities, Drug-Induced↗
PubMedDate not supplied

Unusual cranial aspects of the Apert syndrome.

Frank cloverleaf skull is found in approximately 4% of Apert-syndrome infants. However, the usual Apert skull and its cloverleaf form are spectral in nature. In all patients, the temporal bones are obliquely situated, and the degree to which this is so determines whether no, mild, moderate, or severe cloverleafing will occur. True encephalocele rarely is found in the Apert syndrome, but pseudoencephalocele in the frontal region sometimes is confused with it. Because the Apert calvaria at birth is characterized by a widely gaping midline defect, the midfrontal region of the brain is not covered by bone during early infancy. However, with time, closure of the midline defect occurs by coalescence of bony islands, thus covering the pseudoencephalic region. Also discussed in this paper are (a) atypical calvarial development with patency of the coronal sutures in one case, (b) size and position of the temporal muscles, and (c) size and position of the superficial temporal fat pads.

Abnormalities, Multiple↗
PubMed1993

Cephaloceles: classification, pathology, and management--a review.

The spectrum of diseases that give rise to cephaloceles is reviewed with particular reference to conditions encountered by craniofacial teams. The broad term cephalocele contains the more focused term meningoencephalocele, which is most commonly used by craniofacial surgeons. The interesting pathology of frontoethmoidal meningoencephaloceles is described with reference to the experience of the Australian Craniofacial Unit from 1975 to 1993. Further observations supporting the uniqueness of this entity are made. Although the meningoencephalocele associated with craniofacial clefts does not in itself affect treatment, the management of frontoethmoidal meningoencephaloceles is dependent on a knowledge of their unique natural history. Long-term follow-up has allowed a number of conclusions to be reached in the light of treatment. Basal and posttraumatic encephaloceles are described with respect to their place in the classification system as well as the principles of treatment.

Encephalocele↗
PubMed1993

[Cysts of the lacrimal tracts in the newborn infant].

At birth, a thin membrane often remains at the distal end of the nasolacrimal duct in the inferior meatus. The rupture is spontaneous during the first month of life. Sometimes this impatency can lead to a symptomatology usually limited to an uni or bilateral dacryocystitis. Exceptionally a cyst can develop in the nasolacrimal passageways. The authors describe three cases of intranasal occurrences, two of which brought about an obstructive dyspnea. Twelve cases have been described in the literature. This affliction predominantly occurs in young girls. Whenever the nasal obstruction is isolated, it is important to eliminate any other cause of intranasal tumor, especially an encephalocele, before any surgical intervention. Computerized tomographic scan is indispensable in order to eliminate any connection with the intracranial space. Magnetic resonance imaging appears promising. For the authors, the treatment must be surgical and must be kept simple. It consists in a cyst excision.

Cysts↗
PubMed1993

Goldston syndrome reconsidered.

We describe two siblings with the combination of central nervous system malformations, renal dysplasia and hepatic ductal plate malformation. Based on the occurrence of a cerebellar Dandy-Walker malformation (variant), the diagnosis of Goldston syndrome was proposed in the first case. The second sibling however, showed a typical Meckel syndrome with occipital encephalocele. Both cases had a cranium bifidum with two defects in the squamous part of the occipital bone. This observation confirms that Goldston syndrome is not a distinct entity but merely a variant of Meckel syndrome, and that a cerebellar Dandy-Walker malformation can be a component of Meckel syndrome.

Abnormalities, Multiple↗
PubMed1997

Brain herniation into the middle ear and mastoid: concepts in diagnosis and surgical management.

OBJECTIVE: To review the occurrence characteristics of and clinical repair experience with brain herniation in to the middle ear and mastoid from 1970-1995. STUDY DESIGN: Retrospective chart/case review. SETTING: Private Otology/Neurotology referral practice. PATIENTS: Thirty-five patients with temporal bone brain herniation diagnosed and treated from 1970-1995. INTERVENTION: Diagnosis confirmed by CT and/or MRI. Treatment was surgical. MAIN OUTCOME MEASURES: Success of surgical repair of the problem in a large experience with follow-up of up to 180 months (mean, 48.7 months). RESULTS: Diagnosis is most effectively made by both (computed tomography (CT) and magnetic resonance imaging (MRI). In this series diagnosis was accurate in 89% with MRI. Primary repair was successful in all but three patients, two of whom required a second repair. One was unreconstructable. CONCLUSIONS: Temporal bone encephaloceles occur after ear surgery and in chronic otitis media. Prompt and effective surgical repair is successful and integral to complication avoidance.

Adult↗
PubMed1997

[Meckel syndrome. Update on a recurrent case].

Meckel's syndrome is an autosomal recessive disorder classically defined by occipital encephalocele, multicystic kidneys and polydactyly. However, a very wide phenotypic spectrum is characteristic of Meckel's syndrome. The prenatal diagnosis of this lethal syndrome is approached with ultrasound examination. The karyotype has to be done in order to make the differential diagnosis, especially with Trisomy 13 or 18. The rate of the Meckel's syndrome's gene is 1/400. The isolation of the specific gene will be soon helpful to define the Meckel's syndrome precisely.

Abnormalities, Multiple↗
PubMed1996

[An estimate of recurrence risk for neural tube defects in China].

A retrospective survey was made for estimating the recurrence risk of neural tube defects (NTD) in 233 hospitals of China during the period from July 1990 to June 1993. A total of 2159 women who born a baby with NTD in this pregnancy were investigated. Among them, 674 had one or more previous births (total in 889 births) before this pregnancy. Of the 889 children, 61 had NTD. Thus the overall estimate recurrence risk of NTD is 6.86%. The estimate recurrence risk of NTD is correlated with the prevalence. The estimate recurrence risks are 3.70%, 4.50% and 8.32% in the low-, average and high-prevalence areas respectively. There is a high recurrence risk for the same defect (anencephaly of spina bifida or encephalocele) of the index case.

Anencephaly↗
PubMed1998

Potential pitfalls in the work-up and diagnosis of choanal atresia.

To increase an awareness of the developmental anatomy of the nasal cavity as it applies to the radiologic work-up of choanal atresia and frontoethmoidal cephaloceles, we report two cases that demonstrate potentially serious imaging pitfalls. Two neonates with nasopharyngeal obstruction were imaged with CT and MR. Both patients had surgically proved bilateral bony choanal atresia. In addition to choanal atresia, CT showed a radiolucent, or nonossified cribriform plate and mucoid secretions within the nasal fossa, adjacent to the cribriform plate, which approximated the attenuation of brain parenchyma. In one of the patients, a preoperative diagnosis of nasopharyngeal encephalocele resulted in surgical exploration. At surgery, however, the cartilaginous cribriform plate was found to be intact.

Choanal Atresia↗
PubMed1997

[Studies on teratogenic effects of aluminum on intra uterine fetal development in mice].

Effects of oral administration of different doses of aluminum during pregnancy on intra uterine fetal development in mice were studied with experimental teratological methods. Results showed that over-intake of aluminum could lead obvious teratogenic and toxic effects on fetal development in mice. It could cause formation of encephalocele and dysostosis, intra uterine fetal growth retardation, and increase of embryo death rate, with a obvious dose-dependent relation. It suggests that excessive ingestion of aluminum may be one of the risk factors contributing to congenital neural tube defects, intra uterine fetal growth retardation and perinatal deaths.

Aluminum↗
PubMed1976

[Hypopituitarism with hypoplasia of the optic nerve (Kaplan, Grumbach and Hoyt syndrome].

Two new cases of the syndrome described by Kaplan, Grumbach and Hoyt are reported. The two children were hypopituitary dwarfs, with congenital nystagmus and small optic discs with double border. Pneumoencephalography showed malformations of the median structures, with trans-sphenoidal encephalocele in one case. The definition of this syndrome and its relation to De Morsier's septooptical dysplasia are briefly discussed.

Child, Preschool↗
PubMed1980

Cerebellar heterotopia in the orbit.

An infant with slowly progressive proptosis was found to have heterotopic cerebellum in her left orbit. Although cerebellar cell rests are common, to our knowledge none has been found previously in the orbit. It probably arose either from abnormal germ cell migration or from aberrant germ cell differentiation. Orbital heterotopia probably can be classified in the larger category of orbital encephaloceles because of similarities in presentation, clinical course, and treatment, although there can be differences in the embryologic mechanisms of their development.

Cerebellum↗
PubMed1987

Heterotopic brain tissue in the orbit.

A 3-month-old female infant with Turner's syndrome was noted to have progressive swelling of the left upper lid at the age of 1 month. A computed tomogram demonstrated a well-circumscribed, low-density, homogeneous orbital mass. Initially, the main clinical differential diagnosis included dermoid cyst, hemangioma, and encephalocele. Medial orbitotomy followed by histopathologic examination disclosed a cystic choristomatous malformation containing brain tissue. No choroid plexuses within the mass were demonstrated on microscopic examination. Aspiration of fluid from the cystic mass and subsequent biochemical studies unequivocally demonstrated that the mass was filled with cerebrospinal fluid. Despite five surgical procedures, including two frontal craniotomies, and specialized roentgenographic studies, no communication could be demonstrated between the orbital mass and the central nervous system. We postulate that the cerebrospinal fluid was produced within the orbital mass, which represented ectopic brain tissue. Problems in diagnosis and management of this unusual orbital malformation are discussed.

Brain↗
PubMed1987

Cerebro-ocular dysplasia-muscular dystrophy syndrome. Report of two cases.

Cerebro-ocular dysplasia-muscular dystrophy (COD-MD) syndrome is a rare disorder encompassing a triad of brain, eye, and muscle abnormalities. The principal central nervous system features are cerebral and cerebellar agyria-micropolygyria, cortical disorganization, glial-mesodermal proliferation within the leptomeninges, neuronal heterotopias, hypoplasia of nerve tracts, hydrocephalus, and, occasionally, encephalocele. Ocular abnormalities include microphthalmia, cataract, immature anterior chamber angle, retinal dysplasia with or without retinal detachment, persistent hyperplastic primary vitreous, optic nerve hypoplasia, and coloboma. Skeletal muscle findings include fiber splitting, variable fiber size, and endomysial fibrosis. Recent evidence has shown that COD-MD syndrome may be identical to the Walker-Warburg (also known as Warburg) syndrome. Fukuyama congenital muscular dystrophy is similar to the COD-MD and Walker-Warburg syndromes, although the ocular manifestations are less severe. We report the histopathologic findings in two siblings with multiple features of COD-MD syndrome.

Brain↗
PubMed1981

Heterotopic pharyngeal brain.

We report a case of heterotopic brain tissue in the oropharynx without connection to the CNS. We attempt to differentiate this developmental anomaly from encephaloceles and nasal gliomas, in which a communication with the CNS may lead to the development of postoperative meningitis.

Brain↗