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At least 19 recordsLinked to original sources
PubMed2002

Endoscopic repair of acquired encephaloceles, meningoceles, and meningo-encephaloceles: predictors of success.

Meningoceles, encephaloceles, and meningo-encephaloceles may develop through defects associated with congenital anomalies or through defects produced by tumor, trauma, or surgery. Their initial management, surgical indications, and repair techniques are not universally accepted. We undertook this study to compare the contributions of different surgical techniques and materials to the success of endoscopic repairs of acquired meningo-encephaloceles. We also examined whether characteristics of the patient, the meningo-encephalocele, or the adjunctive treatment influenced the outcome of the repair.We retrospectively reviewed the clinical charts of all patients undergoing transnasal endoscopic repair of acquired meningo-encephaloceles at our academic hospitals. We encountered 17 patients with meningo-encephaloceles of the anterior fossa and parasellar area; 15 were repaired immediately using transnasal endoscopic techniques. Two persistent leaks associated with hydrocephalus were repaired during a second endoscopic attempt, which was rapidly followed by ventriculoperitoneal shunting. Location and size of the skull base defect, its etiology, and the technique and choice of material used for repair did not significantly affect surgical outcomes. However, the presence of hydrocephalus was significantly related to poor surgical outcomes.

Journal Article↗
PubMed2000

Nasal encephaloceles.

Nasal encephaloceles can be divided into frontoethmoidal and basal encephaloceles. Both conditions are very rare, but frontoethmoidal encephaloceles show a relatively high incidence (1:5,000) in Southeast Asia. The pathogenesis of encephaloceles may be explained by a disturbance in separation of surface ectoderm (epithelial layer) and neurectoderm (nervous tissue) in the midline just after closure of the neural folds. It should be regarded as a 'late' neurulation defect taking place during the 4th gestational week. Apoptosis appears to be related to this separation process. Frontoethmoidal encephaloceles can be recognized as a facial mass covered with normal skin, while basal encephaloceles may cause nasal obstruction or symptoms related to herniation of basal structures. Diagnostic CT or MR imaging delineates the anatomy of the herniated mass. Therapy for frontoethmoidal encephaloceles consists in excision of the cele, watertight closure of the dural defect and reconstruction of the skull defect. Basal encephaloceles may harbour vital herniated structures which should be saved. Hydrocephalus should be dealt with first, followed by elective single-stage reconstructive surgery. The prognosis appears to be better for patients with frontoethmoidal encephaloceles than for patients with occipital or parietal encephaloceles, and it depends largely on the presence of additional congenital anomalies of the brain. The differential diagnosis of a nasal mass must include nasal glioma, dermoid cyst, and nasal polyp.

Child↗
PubMed2006

Are encephaloceles neural tube defects?

OBJECTIVE: Encephalocele is classified as a neural tube defect, but questions have been raised regarding whether its epidemiological characteristics are similar to those of other neural tube defects. DESIGN: We compared characteristics of temporal trends in, and the impact of folic acid grain fortification on, the prevalence of encephalocele, spina bifida, and anencephaly using data from the Metropolitan Atlanta Congenital Defects Program, a population-based birth defects surveillance system. Prevalences of encephalocele, spina bifida, and anencephaly were compared by maternal age, gender, race, birth weight, ascertainment period (1968-1981, 1982-1993, or 1994-2002), and fortification period (1994-1996 [prefortification] and 1998-2002 [postfortification]) using prevalence ratios with 95% confidence intervals. Temporal trends were assessed using Poisson and negative binomial regression models. RESULTS: Prevalence rates of encephalocele (n = 167), spina bifida (n = 650), and anencephaly (n = 431) were 1.4, 5.5, and 3.7 per 10 000 live births, respectively. Encephalocele was similar to anencephaly in showing an increased prevalence among girls and multiple gestation pregnancies and to spina bifida and anencephaly in an annual prevalence decrease between 1968 and 2002 (-1.2% for encephalocele, -4.2% for spina bifida, and -3.6% for anencephaly). With fortification, prevalence decreased for spina bifida but not significantly for encephalocele or anencephaly. CONCLUSIONS: Encephalocele shows more similarities to spina bifida or anencephaly than it shows differences with respect to characteristics, temporal trend, and impact of fortification. Additional studies should be done to explore the etiologic heterogeneity of encephalocele using better markers of folate status and a wider range of risk factors.

Adolescent↗
PubMed2002

Sphenoid encephaloceles: disease management and identification of lesions within the lateral recess of the sphenoid sinus.

OBJECTIVES: Encephaloceles usually involve herniation of frontal lobe tissue through an anterior cranial fossa defect into the ethmoid sinus or nasal cavity. Encephaloceles can also result from temporal lobe herniation through a middle fossa defect into the sphenoid sinus. Within the sphenoid, encephaloceles are thought to occur most commonly in the central or midline aspect of the sinus. Lateral sphenoid encephaloceles, especially within the lateral aspect of the sphenoid sinus when the sphenoid sinus has pneumatized extensively into the pterygoid recess, are considered exceedingly rare. The objectives of the study were to review our experience with sphenoid encephaloceles to understand the relative frequency and the locations in which they occur within the sphenoid sinus and to report our experience in caring for patients with this condition. STUDY DESIGN: Retrospective review. METHODS: Retrospective review of patient records and operative reports from 1991 to 2000. RESULTS Twelve patients were treated for intrasphenoid encephaloceles during a 10-year period. Eight patients had lesions located in the lateral recess of the sphenoid sinus. Surgical repair was undertaken in all 12 cases using endoscopic techniques. In 11 of 12 cases, the repair was successful with follow-up times of 12 to 69 months (mean follow-up, 31.9 mo). CONCLUSIONS: Temporal lobe encephaloceles in the lateral sphenoid sinus have been reported rarely in the literature. Careful preoperative evaluation and localization of the sphenoid defect are critical for the selection of the optimal surgical approach for repair of the skull base defect. Our 10-year experience represents the largest group of patients treated endoscopically for intrasphenoid encephaloceles reported to date.

Adult↗
PubMed1986

Anterior basal encephalocele of the neonatal and infantile period.

Diagnosis of occult basal encephalocele is not difficult if the peculiar clinical and radiological signs of this anomaly are borne in mind. Recent surgery for transsphenoidal encephalocele has had better results than realized. However, high surgical risks may still be encountered in transsphenoidal encephalocele of the early infantile period, because the pituitary-hypothalamic structures are usually incorporated in the herniated encephalocele of this age group. Surgical indications for and operative approaches to transsphenoidal encephalocele in the infantile period are discussed on the basis of the authors' failure in transcranial repair, which resulted in early postoperative death due to hypothalamic dysfunction. Reviewing the reported cases of anterior basal encephalocele, a high correlation between transsphenoidal encephalocele, particularly in the pediatric age group, and allied malformations of the face, eye, and brain was disclosed. This characteristic malformation complex may be explained by a common pathogenetic mechanism operating in the embryonal period at about the stage of the anterior neuropore closure and occurring in the ventral surface of the cephalic end of the neural tube. Three cases of transsphenoidal encephalocele diagnosed in the neonatal period with progressive obstruction in the nasopharyngeal airway are also reported. A characteristic malformation complex consisting of median cleft face syndrome, optic nerve dysplasia, and agenesis of the corpus callosum was associated in two cases; the other patient had an extremely rare combination of septooptic dysplasia. Two patients died pre- and postoperatively, respectively; the other patient did not undergo operation because of grave multiple anomalies.

Abnormalities, Multiple↗
PubMedDate not supplied

Safety and efficacy of endoscopic repair of CSF leaks and encephaloceles: a survey of the members of the American Rhinologic Society.

The purpose of this article is to review the endoscopic management of cerebrospinal fluid (CSF) leaks and encephaloceles, with particular emphasis on safety and efficacy, by retrospective assessment utilizing the results of a mailed questionnaire. Surveys were mailed to members of the American Rhinologic Society with practices in both academic centers and/or private settings. Survey results were then assessed and tabulated. There were 635 mailings, with 197 responses (31%). Seventy-two (36% of respondents) indicated that they performed endoscopic management of CSF leaks and encephaloceles, while 125 (64% of respondents) did not. Respondents reported approximately 522 cases of CSF leaks and approximately 128 cases of encephaloceles managed by endoscopy. Success rates after a single procedure were estimated at 90% for CSF leaks and 93% for encephaloceles. Success rates after a secondary procedure were estimated at 86% and 97%, respectively; 29% of respondents have, at some point, made a referral to neurosurgery. A total of 13 complications related to endoscopic repairs were reported (2.5%). For CSF leak repair, complications included seizures, 0.2%; meningitis, 1.1%; and one reported case each of cavernous sinus thrombosis, temporary visual problems, sinusitis, and intracranial hypertension/bleed. There was only one reported death in the approximately 522 cases. Eleven complications following encephalocele repairs (8.5%) included seizures, 3.1%; meningitis, 2.3%; and one reported case each of brain abscess, sinusitis, false aneurysm of middle cerebral artery, and mild dizziness. No deaths following encephalocele repair were reported. The endoscopic management of CSF leaks and encephaloceles has become increasingly popular and has proven to have low morbidity and mortality with high success. Overall, our results confirm that in the hands of the skilled endoscopist, endoscopic management of CSF leaks and encephaloceles is highly efficacious and has a very low incidence of significant complication.

Bandages↗
PubMed2000

Can prognostic indicators be identified in a fetus with an encephalocele?

Encephaloceles, like other congenital malformations of the brain diagnosable in utero, can be either complicated (there being an associated chromosomal abnormality, abnormalities in the remainder of the central nervous system (CNS) and/or other organs), or isolated (no abnormalities in the chromosomes, the remainder of the CNS or other organs). Complicated cases invariably have a poor prognosis but amongst those with isolated lesions the outcome is variable with some affected children having poor mental and physical development but others who are only mildly or moderately disabled. To be able to make an informed decision about how to manage their pregnancy parents need to know what the prognosis is likely to be for their fetus with an encephalocele. To see if the necessary information could be reliably gathered by prenatal assessment of affected fetuses, a review was carried out of the medical records and ultrasound scans of 31 fetuses with encephaloceles referred to the Fetal Management Unit at St. Mary's Hospital in Manchester between January 1991 and December 1997. Eighteen of the cohort were classified as having a complicated encephalocele. Thirteen of the pregnancies were terminated; there were three intrauterine deaths, and one neonatal death. There is only one surviving child who is severely disabled. Thirteen fetuses were classified as having isolated encephaloceles, six had a mass of neural tissue in the encephalocele sac and were terminated, one died in utero and six had a cystic lesion or only a nubbin and have survived with few or no abnormalities. This study has shown that it is possible to identify fetuses with an encephalocele with a favourable outcome.

Abnormalities, Multiple↗
PubMed1994

The repair of frontal encephaloceles in Papua New Guinea.

Frontal encephaloceles are common in Papua New Guinea (PNG). Seventeen cases collected over 7 years are reported. Eleven frontal (sincipital) encephaloceles were repaired successfully via an extracranial approach. One of these patients with a frontonasal encephalocele developed a recurrence following the extracranial approach, which was subsequently repaired intracranially. Three patients with small naso-ethmoidal encephaloceles were repaired intracranially via an extradural approach. The other three cases have not yet had surgical correction. Complex craniofacial surgery which corrects hypertelorism as well as the encephalocele is unavailable in the developing world. For the general surgeon in the developing world, the extracranial approach is recommended for the frontonasal encephaloceles, and the intracranial approach for the naso-ethmoidal and naso-orbital encephaloceles. Hydrocephalus and epilepsy have not developed in the patients.

Child, Preschool↗
PubMed2003

Intraorbital encephalocele: an important complication of orbital roof fractures in pediatric patients.

Orbital roof fractures are uncommon, and traumatic intraorbital encephalocele formation is a very rare complication of this type of injury. We treated 43 pediatric patients with orbital roof fractures at our center over a 4-year period. The aim of this study was to retrospectively investigate conditions that may lead to intraorbital encephalocele formation in children with orbital roof fractures. Each case was reviewed, and the cause of injury, associated clinical and computerized tomography findings, the Glasgow Coma Scale score on admission, neurological status, other bodily injuries, hospitalization time and type and width of the orbital roof fracture were recorded. The findings in 6 patients who developed encephaloceles were compared to corresponding findings in the 37 patients who did not develop this complication. A total of 44 orbital roof fractures were diagnosed by axial and coronal computed tomography scanning. Six of the 43 children developed intraorbital encephaloceles in the first month after head trauma. In each of these cases, magnetic resonance imaging demonstrated the intraorbital cystic lesion in communication with the subarachnoid space. The width of each orbital roof fracture was measured on axial and coronal computed tomography slices and was confirmed by measurements during surgery. The width of the fractures in the encephalocele cases ranged from 2-4 mm. Duraplasty and orbitoplasty were performed in all the patients with encephalocele. Pediatric patients with orbital roof fractures that exhibit more than 2 mm diastasis and are associated with frontal cerebral contusion may be at greater risk for developing intraorbital encephalocele. All such cases should be monitored closely and investigated further with magnetic resonance imaging.

Adolescent↗
PubMed1995

Encephaloceles of the anterior cranial fossa.

From a total of 114 encephaloceles treated surgically at The Hospital for Sick Children in the 15 years to 1994, the case records of 17 patients with sincipital and 5 patients with basal defects were reviewed retrospectively. The condition was evident at birth in 64% of patients, while the remainder presented with either cerebrospinal fluid (CSF) rhinorrhea, nasal obstruction, or feeding difficulty. Hypertelorism affected 73% of patients. All encephaloceles were repaired transcranially, at a mean age of 2 years, usually by means of an intradural pericranial graft. Five children with gross hypertelorism underwent orbital translocation at the time of encephalocele repair. Of those not corrected, primary and secondary hypertelorism regressed in most instances where the encephalocele was treated before the age of 2 years. There were no deaths. The only case of CSF rhinorrhea occurred in a patient with a basal defect, in whom intradural repair was not possible because of adherence of diencephalic structures to the sac wall. Hypertelorism recurred in 1 patient after orbital translocation, requiring recorrection 2 years later. One patient with untreated secondary hypertelorism failed to regress after the encephalocele was excised at the age of 4 months. Developmental outcome was normal in 59% of children, whilst 18% have mild mental or physical disability, and 23% are severely impaired. A child with a sincipital or basal defect and mild hypertelorism should have the encephalocele treated in early childhood to allow the facial skeleton to remodel with growth. When an encephalocele is accompanied by gross hypertelorism or a facial cleft, one-stage correction can be undertaken safely in early childhood with minimal mortality and acceptable morbidity.

Encephalocele↗
PubMed1996

Postneurulation rapid brain growth represents a critical time for encephalocele formation: a chick model.

Current theory regarding the pathogenesis of encephaloceles suggests that the defect occurs after neurulation is completed in which brain tissue herniates through defective mesodermal elements necessary for skull modeling. To better delineate the mechanisms of encephalocele development, we performed a variety of microsurgical manipulations in the developing cranium of the chick embryo during the postneurulation period of early rapid brain growth. The results of the study revealed that encephaloceles could be induced in 78% of chick embryos manipulated at stage 26 that had evidence of marked decompression of the primitive ventricle. On the other hand, control embryos or embryos manipulated without ventricular decompression at stage 26 did not develop encephaloceles (0 of 32). Embryos with decompression of the primitive ventricle treated at earlier stages (20-24) showed only a 5% incidence of encephaloceles. These findings suggest that there is both a critical time point during postneurulation rapid brain growth when encephaloceles are prone to occur and a mesenchymal defect associated with decompression of the primitive ventricle that must be present to induce encephaloceles.

Age Factors↗
PubMed2006

Expansile cranioplasty for massive occipital encephalocele.

OBJECTIVE: This report highlights the management of infrequently encountered massive occipital encephaloceles with herniation of large amount of apparently functional cortex into the encephalocele sac. MATERIALS AND METHODS: Two children with giant occipital encephaloceles with herniation of significant brain parenchyma into the encephalocele sac were managed with expansile cranioplasty and reconstruction of the calvarial defect with autologus bone graft harvested from the adjacent parietal region. CONCLUSIONS: Giant occipital encephaloceles with significant brain parenchyma in the encephalocele sac can be considered for preservation of the herniated parenchyma and expansile cranioplasty.

Bone Transplantation↗
PubMedDate not supplied

Extramedullary hematopoiesis within a frontoethmoidal encephalocele in a newborn with holoprosencephaly.

We present the first report of extramedullary hematopoiesis (EMH) in an encephalocele. The patient was a new-born with semilobar holoprosencephaly, a frontoethmoidal encephalocele, and a large subdural hematoma. The encephalocele appeared as a hemorrhagic mass, protruding from the forehead to cover the right eye, without involvement of the sinuses or nasopharynx. Computerized tomography and magnetic resonance imaging studies ruled out other forms of holoprosencephaly and confirmed the continuity of the brain with the extruded mass. Immunohistochemistry confirmed the presence of an atrophic epithelium covering the mass. Histologic examination of the encephalocele revealed EMH both within and adjacent to malformed cerebral cortex, with a tendency for the hematopoietic cells to line up in columns within malformed cerebral cortex. We propose that a single event during the fourth week of gestation could both interrupt closure of the neural tube, giving rise to the encephalocele, and impair migration of the neural crest, leading to holoprosencephaly secondary to failure of neural crest derivatives to induce basomedial telencephalic differentiation. EMH may have been induced from hematopoietic stem cells in the richly vascular meningeal component of the encephalocele, in response to anemia and hypoxia.

Biomarkers↗
PubMed1999

Transcranial approach for transsphenoidal encephalocele: report of two cases.

BACKGROUND: Whereas the transcranial approach has been regarded as the therapy of choice for transethmoidal encephalocele, its feasibility for transsphenoidal encephalocele has remained controversial, particularly in neonates and infants. CASE REPORT: Two cases of transsphenoidal encephalocele operated transcranially are presented. In the first case, this 6-year-old boy underwent a transpalatal operation with repair of a cleft palate in another hospital before admission. Reoperation via the transcranial route was carried out because of postoperative recurrent meningitis. With partial resection of the anterior wall, the encephalocele could be separated from the underlying tissue, and the interspace was filled with the pericranial flap. He made an uneventful recovery and has been well for the past 3 years. The second was a 3-month-old baby with a large encephalocele filling the nasopharyngeal space. As the cleft palate was absent, the transcranial approach was employed. In this case, the herniated tissue was excised at the lowest level possible. Postoperatively, panhypopituitarism became manifest. Re-evaluation of the preoperative magnetic resonance imaging (MRI) disclosed a small mass far below the dorsum sellae, which turned out to be an anomalous pituitary gland on histologic examination. CONCLUSIONS: The transcranial approach is considered a valid alternative for the therapy of transsphenoidal encephalocele, particularly when the transpalatal approach is unfeasible. While the anterior wall of the herniated sac may be safely resected, the posterior wall should under no circumstances be sacrificed. The preoperative MRI is essential, as it may provide valuable information as to the location of vital structures within the herniated tissue.

Child↗
PubMed2000

Trans-sphenoidal and sphenoethmoidal encephalocele: report of two cases and review of the literature.

Basal encephaloceles are the least common form of encephalocele. Due to the critical position of the bony defect, visual and endocrinological abnormalities are frequently associated with basal encephaloceles. There is significant confusion in the classification of basal encephaloceles, particularly among trans-sphenoidal, sphenoethmoidal and intrasphenoidal subtypes. Two cases of basal encephaloceles are presented (one trans-sphenoidal and one sphenoethmoidal), along with a review of the literature. The relationship between the basal encephaloceles and endocrinological abnormalities is also emphasized.

Adolescent↗
PubMed2002

Trans-sellar trans-sphenoidal encephaloceles: report of two cases.

Trans-sphenoidal encephaloceles are rare entities. Only about 20 such cases have been reported in the world literature to date. Among these, the trans-sellar variety is rarest (five cases). We present two cases of trans-sellar trans-sphenoidal encephaloceles seen over the last 12 years. The first case was a 5 month old male infant presenting with nasal obstruction and a mass in the mouth. The second case was a 14 year old boy presenting with chronic nasal obstruction since early childhood. In the first case, the mass was coming to the mouth through the split palate. In the second case, the mass could be seen in the posterior half of the nose by rhinoscopy. The computed tomography scans including coronal and sagittal reconstructions had revealed trans-sellar trans-sphenoid encephaloceles in both the patients. The magnetic resonance imaging in the second case showed the cavity and the structures of the floor of the third ventricle forming the main fundal contents. The magnetic resonance angiography revealed dipping of the ectatic anterior cerebral arteries in the neck of the encephalocele sac in this case. Extracranial trans-palatal approach and the repair of encephalocele was done in the first case with the palatal reconstruction at the same sitting. In the second patient, a trans-nasal approach with bilateral maxillotomies was performed to repair the encephalocele, and the skull base defect was repaired by a split rib graft, which was fixed with mini-plate and screws posteriorly at the clivus and anteriorly at the anterior edge of the bony defect. The cleft lip and hypertelorism also were corrected at the same sitting. Our first case had CSF leak on the 10th postoperative day and developed fulminant meningitis, despite appropriate antibiotics, and succumbed. The second patient had a good postoperative recovery and a satisfactory follow up for 2 years.

Adolescent↗
PubMed2005

Repair of iatrogenic temporal lobe encephalocele after canal wall down mastoidectomy in the presence of active cholesteatoma.

OBJECTIVE: Although mastoid and middle ear obliteration provides the ultimate repair of an encephalocele, retained squamous epithelium may result in the occult recurrence of cholesteatoma. For most patients, a preferable technique is to perform a canal-wall-up mastoidectomy with middle fossa craniotomy. However, temporal lobe encephaloceles are occasionally found in patients with canal-wall-down cavities along with active cholesteatoma. We sought to describe our management strategy for this dilemma. STUDY DESIGN: Retrospective review. SETTING: Tertiary referral center. PATIENTS: We reviewed all patients with encephaloceles treated by the primary surgeon. Patients without active cholesteatoma and a canal-wall-down cavity were excluded. INTERVENTION: Surgical management of the encephalocele and cholesteatoma. MAIN OUTCOME MEASURE: Successful repair and a noninfected ear. RESULTS: Three patients met the inclusion criteria. All had previous canal-wall-down surgery for cholesteatoma by outside surgeons and presented with chronic otorrhea, large tegmen defects, and brain herniation into the mastoid cavity. All had incomplete removal of their posterior canal wall. Our management strategy involved completing the canal-wall-down mastoidectomy and repairing the temporal floor defect using a three-layer closure via a middle fossa craniotomy. This included suture repair of the dural defect with or without a graft, a temporalis muscle rotation flap, and a split-calvarial bone graft. All patients recovered from their surgery without evidence of further cerebrospinal fluid leak, encephalocele, or cholesteatoma with a minimum follow-up time of 6 months. CONCLUSIONS: A temporal lobe encephalocele can be safely repaired while maintaining a mastoid bowl. This may be the safest treatment option for patients with active cholesteatoma.

Adult↗