PubMed Health⌕ Search

SEARCH · PubMed Health

Results for “ENCEPHALOCELE”

Explore indexed PubMed citations for clinical trials, systematic reviews and public health research. Read source abstracts and follow each citation to its original PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 523 records · Page 29Linked to original sources
PubMed1998

Management of encephaloceles and craniofacial problems in the neonatal period.

Although craniofacial problems are commonly detected during the neonatal period, only a selected subgroup of lesions require early intervention. Such lesions are characterized either by their potential for causing early neurologic impairment, infection, or other severe sequelae if treatment is delayed or by the fact that the therapeutic outcome is more favorable with early intervention. An overriding consideration in the decision to treat any of these problems during the neonatal period is the risk of major blood loss, which can pose a life-threatening problem. Accordingly, meticulous surgical technique and close monitoring of hemodynamic status are crucial to optimizing therapeutic outcome. Herein, we present therapeutic caveats and operative techniques for the neurosurgical management of craniofacial lesions commonly encountered during the neonatal period.

Birth Injuries↗
PubMedDate not supplied

[Fronto-ethmoidal meningo-encephaloceles and nasal gliomas (author's transl)].

Meningoencephalocoeles and nasal gliomas are congenital malformations rare in Europe but much better known in Morocco and in Thailand. These lesions have embryonic relations which are such that certain authors consider them to be only clinical varieties of the same malformation. They cause extra nasal, intra nasal or mixed tumours discovered most often at birth. Treatment is surgical either via a high intracranial approach or via a low facial approach (both approaches sometimes being used in association) and the prognosis is dominated by the problem of the dura mater if there are connections between the tumour mass and the subarachnoid spaces. When the problem is resolved by dural plasty, the later prognosis is excellent and, in particular, the psychomotor development of the children appears to be normal. Cosmetic problems, and in particular hypertelorism, may nevertheless require later surgery.

Female↗
PubMed2002

Management of multiple spontaneous nasal meningoencephaloceles.

OBJECTIVES/HYPOTHESIS: Multiple spontaneous nasal meningoencephaloceles in the same patient are rare lesions. Although many skull base defects occur after prior trauma or surgery, otolaryngologists must be aware of the potential for spontaneous encephaloceles. We present our experience with this unusual condition and discuss its pathophysiology and unique management issues. STUDY DESIGN: Retrospective. METHODS: Review of medical records, radiographic images, and cerebrospinal fluid pressures. RESULTS: We identified 5 patients with multiple, simultaneous, spontaneous encephaloceles: 4 patients with 2 encephaloceles and 1 patient with 3 encephaloceles (11 in all). Locations of the 11 encephaloceles were sphenoid lateral recess (6), frontal sinus with supraorbital ethmoid extension (2), ethmoid roof (1), frontal sinus (1), and central sphenoid (1). Three patients had bilateral sphenoid lateral recess encephaloceles, accounting for all six in that location. All four patients with available radiographic studies demonstrated empty sella turcica. Surgical approaches included endoscopic transpterygoid approach to the lateral sphenoid recess (3), endoscopic approach to ethmoid and central sphenoid (3), and osteoplastic flap with frontal sinus obliteration (2). We had 100% success at latest endoscopic follow-up (mean period, 17 mo). Three patients had postoperative lumbar punctures with mean cerebrospinal fluid pressure of 28.3 cm water (range, 19-34 cm; normal range, 0-15 cm). CONCLUSIONS: Multiple spontaneous encephaloceles can be managed safely and successfully using endoscopic and extracranial approaches. A high index of suspicion for this diagnosis must be maintained, especially in patients with radiographic evidence of laterally pneumatized sphenoid sinuses or empty sella. Spontaneous encephaloceles and cerebrospinal fluid leaks represent a form of intracranial hypertension.

Encephalocele↗
PubMed1990

Major congenital neurologic malformations. A 17-year survey.

We present characteristics of four major congenital neurologic malformations--anencephaly, spina bifida, encephaloceles, and hydrocephalus--from a population of 763, 364 live-born and still-born infants born from 1971 through 1987. During the 17-year study period, 275 infants had anencephaly (0.36 per 1000 total births), 526 had spina bifida (0.69 per 1000 total births), 112 had encephaloceles (0.15 per 1000 total births), and 370 had hydrocephalus (0.48 per 1000 total births). There was a female preponderance of infants with anencephaly, spina bifida, and encephaloceles, while males predominated among those with hydrocephalus. We found declining incidences of anencephaly, spina bifida, and encephaloceles only among white females. Black infants were significantly less likely than white infants or infants of other races to have spina bifida. Twenty percent of infants with anencephaly had congenital anomalies unrelated to the primary defect, as did 40% with encephaloceles, 37% with hydrocephalus, and 22% with spina bifida. Because the racial background of the patient population closely resembles that of the United States as a whole, the features of the malformations described may reflect those of the country.

Anencephaly↗
PubMed1982

Syndromes with cephaloceles.

Encephaloceles may occur as isolated malformations or together with other anomalies making up various syndromes or associations. With the possible exception of the aberrant tissue band syndrome and the Meckel syndrome, little attention has been paid to syndromes with encephaloceles as a group. This paper discusses syndromes of known genesis, including the aberrant tissue band syndrome, Chemke syndrome, cryptophthalmos syndrome, dyssegmental dwarfism, frontonasal dysplasia, Knobloch syndrome, Meckel syndrome, pseudo-Meckel syndrome, von Voss syndrome, and warfarin syndrome. Associations include a wide range of anomalies that may occur with encephalocele. Some abnormalities are secondary to encephalocele formation; others represent primary noncontiguous embryonic malformations. Associated anomalies include absent corpus callosum, orofacial clefting, craniostenosis, Dandy-Walker defect, Arnold-Chiari defect, ectrodactyly, hemifacial microsomia, hypothalamic-pituitary dysfunction, Klippel-Feil anomaly, iniencephaly, and myelomeningocele. Finally, several other conditions with encephalocele are discussed. Some may represent true low-frequency associations; others are spurious.

Agenesis of Corpus Callosum↗
PubMed2003

[Meningoencephalocele as rhinosurgical problem].

Cephalocele is a rare lesion mainly of congenital or traumatic origin. The lesion occurs as an extracranial hernia of dura mater that consists of cerebrospinal fluid only (meningocele) or cerebellar tissue (encephalocele). Some cephaloceles concern anterior cranial fossa and then are observed as hernias in nasal cavity or in paranasal sinuses. Three patients are presented. In 52 year woman, who was admitted due to idiopathic rhinorrhea (persisting 16 years) with periodical headache, the meningocele in right ethmoid sinus was diagnosed. The lesion was removed with the rhinosurgical approach and the defect in anterior cranial fossa was repaired with the free cartilaginous flap. 12 year boy was admitted due to the nasal tumor diagnosed by CT and MRI. The lesion was removed with the combined access: firstly the hernia sac was cut intracranially with the frontal craniotomy and then encephalocele was removed with lateral rhinotomy. Cranial fossa defect was repaired by the periosteum flap. In third case (8 year boy) encephalocele caused 5 incidences of purulent meningitis. CT and MRI showed the lesion in frontal sinus. Encephalocele was removed with rhinosurgical approach and the defect in posterior frontal wall was repaired with free cartilaginous and mucosal flaps. In all patients long term result of operations was good. Anatomic and pathologic conditions that influence on the choice of rhinosurgical or combined access to nasal and sinusal encephaloceles are presented and discussed.

Child↗
PubMed1998

Congenital anomalies in the teratological collection of Museum Vrolik in Amsterdam, The Netherlands. IV: closure defects of the neural tube.

The Museum Vrolik collection of the Department of Anatomy and Embryology of the University of Amsterdam, founded by Gerardus Vrolik (1775-1859) and his son Willem Vrolik (1801-1863), consists of more than 5,000 specimens of human and animal anatomy, embryology, pathology, and congenital anomalies. Recently, the collection of congenital anomalies, comprising 360 specimens, was recatalogued and redescribed according to contemporary morphogenetic opinions. The original descriptions, as far as preserved, were compared with the clinical and additional findings. Based on the classification by closure site, proposed by Van Allen et al. [1993], the following closure defects of the neural tube were diagnosed in 107 specimens: craniorachischisis, holoacranium with partial rachischisis, faciocranioschisis, holoacranium, meroacranium, occipital cranioschisis, frontal encephalocele, parietal encephalocele, occipital encephalocele, occipital encephalocele with rachischisis, occipito-cervical encephalocele, thoracolumbar spina bifida, lumbosacral spina bifida, occult sacral dysraphism, and sacrococcygeal dysgenesis. Furthermore, we diagnosed three conditions that did not appear in the aforementioned classification; we named these conditions craniorachischisis totalis, occipital craniorachischisis, and thoracolumbosacral spina bifida. The results of our study are in line with the assumption that particularly occipito-cervical neural tube defects are associated with concomitant anomalies and are more common in females. The results support the idea of multisite closure of the human neural tube.

Abnormalities, Multiple↗
PubMed1976

Etiologic heterogeneity of neural-tube defects.

We classified 106 stillborn and live-born infants with anencephaly, meningomyelocele, meningocele and encephalocele according to the recognized causes of these malformations. Six different causes were identified, including both genetic and nongenetic disorders; 12 per cent had nongenetic disorders, a chromosome abnormality, or an encephalocele as part of the autosomal recessive Meckel syndrome. Therefore, for this 12 per cent genetic counseling normally provided for isolated anencephaly, meningomyelocele or encephalocele would have been incorrect. If all infants were considered together regardless of cause, the precurrence and recurrence rates of similar malformations in the sibs were 5.2 and 1.7 per cent respectively. However, if infants with other disorders, especially the Meckel syndrome, were excluded, the precurrence and recurrence rates for isolated anencephaly, meningomyelocele and encephalocele among white infants were only 1.7 per cent and 0 per cent. These rates are much lower than the risk of 5 per cent currently being used in genetic counseling in the United States.

Abnormalities, Multiple↗
PubMed2006

Survival of infants with neural tube defects in the presence of folic acid fortification.

OBJECTIVE: Neural tube defects (NTDs) are preventable through preconceptional and periconceptional folic acid intake. Although decreases in the prevalence of NTDs have been reported since folic acid fortification of United States grain products began, it is not known whether folic acid plays a role in reducing the severity of occurring NTDs. Our aim was to determine whether survival among infants born with spina bifida and encephalocele has improved since folic acid fortification and to measure the effects of selected maternal, pregnancy, and birth characteristics on first-year (infant) survival rates. METHODS: A retrospective cohort study was conducted and included 2841 infants with spina bifida and 638 infants with encephalocele who were born between 1995 and 2001 and were registered in any of 16 participating birth defects monitoring programs in the United States. First-year survival rates for both spina bifida and encephalocele cohorts were measured with Kaplan-Meier estimation; factors associated with improved chances of first-year survival, including birth before or during folic acid fortification, were measured with Cox proportional-hazards regression analysis. RESULTS: Infants with spina bifida experienced a significantly improved first-year survival rate of 92.1% (adjusted hazard ratio: 0.68; 95% confidence interval: 0.50-0.91) during the period of mandatory folic acid fortification, compared with a 90.3% survival rate for those born before fortification. Infants with encephalocele had a statistically nonsignificant increase in survival rates, ie, 79.1% (adjusted hazard ratio: 0.76; 95% confidence interval: 0.51-1.13) with folic acid fortification, compared with 75.7% for earlier births. CONCLUSIONS: Folic acid may play a role in reducing the severity of NTDs in addition to preventing the occurrence of NTDs. This phenomenon contributes to our understanding of the efficacy of folic acid. Additionally, as survival of NTD-affected infants improves, health care, education, and family support must expand to meet their needs.

Dietary Supplements↗