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PubMed2002

Anterior encephaloceles: a study of 92 cases.

Anterior encephalocele is a rare condition, and only a few large series have been published in the literature. Surprisingly, the incidence is much higher in Southeast Asian countries, including some parts of India. While the reported incidence in the West is between 1:35,000 and 1:40,000 live births, it is as high as 1:5,000 live births in Thailand. We present a series comprising 92 cases of anterior encephaloceles treated over a 30-year period (1971-2000). Frontoethmoidal encephaloceles are the commonest type, followed by the nasopharyngeal and orbital type. Among the frontoethmoidal encephaloceles, nasoethmoid is the commonest type, and these patients present with swelling over the bridge of the nose with significant hypertelorism and orbital deformities. The nasopharyngeal type remains occult and presents with nasal obstruction or CSF rhinorrhea. Rarely, the patient may present with meningitis. Since 1978, computed tomography (CT) scans have regularly been performed in our patients. CT scans delineate the skull defect and associated brain anomalies. There was associated hydrocephalus present in 12 patients and agenesis of the corpus callosum in 5 patients. In all patients, one-stage repair of the encephalocele and correction of bony anomalies by appropriate osteotomy was undertaken. Since 1988, in cases of frontoethmoidal encephalocele with significant hypertelorism, medial advancement of the medial half of the orbits on either sides was carried out, instead of a classical Tessier's operation. Postoperative morbidity included CSF leak in 20 patients, wound infection in 2 and chest infection in 3. There were 3 deaths in our study. The overall cosmetic outcome was good.

Adolescent↗
PubMed2003

Intrasphenoidal transsellar encephalocele repaired by endoscopic approach.

Spontaneous sphenoidal encephaloceles are rare entities. In the case of intrasphenoidal encephaloceles, most defects are temporosphenoidal and occur in the lateral wall of the sphenoidal sinus. There have been to our knowledge only 7 reports of medial, transsellar encephaloceles in the literature. We report a case of intrasphenoidal transsellar encephalocele that was successfully managed through an endoscopic approach with complementary lumboperitoneal shunting. This disorder presents a challenge in surgical management because of the involvement of opticochiasmatic structures, the hypothalamopituitary axis, and the delicate vasculature of the circle of Willis in and around the encephalocele, besides proximity to the cavernous sinus. Rhinologists should be aware of these malformations, since the optic nerve or chiasma may be totally exposed in the sphenoidal sinus in the course of this disease. This case illustrates the need for magnetic resonance imaging in case of sphenoidal abnormalities, as well as the possibility of endonasal repair without a transcranial approach for large intrasphenoidal encephaloceles.

Encephalocele↗
PubMed1991

Developmental anterobasal temporal encephalocele and temporal lobe epilepsy.

The authors describe the association between an antero-basal temporal lobe encephalocele and medically intractable temporal lobe epilepsy in three patients treated successfully by surgery. Two men and one woman, aged 26 to 37 years (mean 31 years), had onset of complex automatism and generalized seizures in their second and fourth decades (mean age 22.7 years). They had been epileptic for 6 to 14 years (mean 8.3 years) before surgery. Preoperative electroencephalograms localized ictal epileptic activity to the left mesial temporal lobe in all cases, and neuropsychological testing revealed dominant temporal lobe dysfunction. Magnetic resonance (MR) imaging demonstrated an anteromedial basal temporal encephalocele extending into the pterygopalatine fossa through a bone defect at the base of the greater sphenoid wing in the region of the foramen rotundum and pterygoid process, a discrete center of embryonal chondrification. At surgery, the encephaloceles were found in front of the uncus, and an area of gliosis extended from the encephalocele to the amygdalohippocampal region. All patients have been seizure-free following anterior temporal resection and amygdalohippocampectomy including the encephalocele. These three cases delineate a condition of disordered embryogenesis wherein a developmental anterobasal temporal encephalocele acts as the substrate for temporal lobe epilepsy. This lesion may be diagnosed preoperatively with MR imaging and should be considered in the differential diagnosis of late-onset temporal lobe epilepsy.

Adult↗
PubMed1987

[A case of holoprosencephaly with parietal encephalocele].

Holoprosencephaly, as described by DeMyer, is a cerebral malformation in which the embryonic prosencephalon fails to divide into cerebral hemispheres. According to the degree of division in the prosencephalon, he classified holoprosencephaly into three types; alobar, semilobar and lobar type. We have experienced a case of holoprosencephaly with parietal encephalocele. The patient was admitted to our hospital 4 hours after birth because of parietal encephalocele. A radical operation was carried out and encephalocele was diagnosed histologically. After discharge from our hospital, his psychosomatic development was remarkably delayed. On Oct. 20, 1985, he was re-admitted for further examination. CT-scan and cerebral angiography showed characteristic findings. The classification of holoprosencephaly and dorsal cyst malformation is discussed. On encephalocele with holoprosencephaly, it is said that there may be an extra-calvarial extension of the dorsal sac or ventricular system. But, Hutchinson stated in his experience that first, a closure disorder of the neural tube caused encephalocele. And later a disorder of diverticularization produced the holoprosencephalic abnormality. He suggested that there were two independent disorders of organogenesis which might rarely occur in the same patient. We also reported a similar case of holoprosencephaly with parietal encephalocele which has some controversial problems.

Abnormalities, Multiple↗
PubMed1981

[The prognosis of encephaloceles (author's transl)].

Encephaloceles were diagnosed in 39 patients over a 40-year interval from 1940. Thirty-three encephaloceles were located in the occipital region, 3 in parietal, 1 in glabellar, 1 in sphenomaxillar and 1 in intracranial. Nineteen patients were males and 20 were females. Eleven of 39 patients were dead. In 33 patients the encephalocele was excised and two died within a month after operation. Four patients expired within a year and other four died before the age of 4 years. Eleven of dead cases were meningoencephaloceles or meningoencephalocystoceles except one. In 6 of them the size of encephalocele was larger than 5 cm in diameter. Twenty-six patients survived 6 months fo 34 years. Six of them found to be severely retarded psychosomatically. Twenty others spend normal lives. Eldest patient whose occipital meningoencephalocele sized 4.5 cm in diameter was excised in 1945 works in a automobile dealer's shop as a car operator after graduation of a junior high school. He has two children, as well. Two patients are graduates of a high school, one a college and one a junior high school. Other patients of school age attend ordinary classes of each school with or without minimal handicaps such as paresis of one leg, unilateral visual loss, slight cerebellar ataxia, large head size and so on. Two of these 20 patients who are found to be in good state had encephaloceles larger than 5 cm in diameter, but other 18 smaller than 5 cm. Eleven of them were meningoceles. Four of 6 patients who had anterior or parietal encephalocele were found to have no neurological sequelae. Significant adverse prognostic factors were the presence of brain tissue within the sac of lesion and the size of it. However, hydrocephalus did not effect the quality of survival of our patients.

Adolescent↗
PubMed1993

Long-term outcome in surgically treated encephalocele.

This report describes long-term operative outcome of 24 cases with occipital encephalocele. The follow-up periods are between 4 and 20 years. Of the 24 patients with occipital encephalocele, two have died. Of the 22 living patients, 16 are living without neurological deficit, while six are disabled mentally and/or physically. Four cases of encephalocele developed hydrocephalus, and two of them died while the other two are still living with severe handicap. The presence of gross brain tissue in the sac of encephalocele and the size of the sac were also unfavorable factors for the prognosis. We conclude that the size and the content of the sac and associated hydrocephalus are the important factors that influence the long-term prognosis of occipital encephalocele.

Encephalocele↗
PubMedDate not supplied

Basal encephaloceles with morning glory syndrome, and progressive hormonal and visual disturbances: case report and review of the literature.

We report an 11-year-old girl with progressive hypopituitarism and visual loss of the right eye caused by trans-sphenoidal and sphenoethmoidal encephaloceles associated with morning glory syndrome. She was first seen at the age of 8 years, because of polydipsia and polyuria, and examination at that time revealed pituitary dwarfism and morning glory syndrome with visual disturbance of the right eye. Hormonal examinations revealed deficiency of growth hormone (GH) and anti-diuretic hormone (ADH). MR image showed trans-sphenoidal and sphenoethmoidal encephaloceles. At the age of 9 years, she was found at another institution to be blind in the right eye. Our examination of the patient at the age of 11 years revealed no change of the findings for the encephaloceles or optic system. Hormonal examination disclosed deficiencies of all hormones except for thyrotropin (TSH). This patient showed progressive hormonal and optic disturbances during the follow-up period. The natural course is still unclear, but our review of reported cases of trans-sphenoidal encephalocele with hormonal disturbance revealed that the most frequent findings were GH and ADH disturbance (over 60%), most patients (77.8%) showed progression of hormonal disturbance, and 40% of those with optic dysfunction showed progression. A patient with basal encephalocele with hormonal and / or optic disturbances requires careful long-term follow up.

Blindness↗
PubMed2006

Anterior encephalocele associated with subependymal nodular heterotopia, cortical dysplasia and epilepsy: case report and review of the literature.

The presence of subependymal nodular heterotopia and cortical dysgenesis has been infrequently reported in patients with encephalocele. The majority of these patients were found to have posterior encephaloceles. We report a case of a Hispanic female with a frontoethmoidal encephalocele who developed epilepsy at 15 years of age. Magnetic resonance imaging of the brain demonstrated left subependymal nodular heterotopia, partial agenesis of the corpus callosum and left fronto-temporal cortical dysplasia with polymicrogyria. This case illustrates the association of anterior encephalocele with subependymal nodular heterotopia, cortical dysplasia and epilepsy. It underscores the importance of screening for intracranial abnormalities in patients with anterior encephalocele.

Adolescent↗
PubMed2005

Spontaneous cerebrospinal fluid rhinorrhoea due to temporosphenoidal encephalocele.

A 57-year old man was referred to our hospital with spontaneous cerebrospinal fluid (CSF) rhinorrhoea of 6 years duration. He had an episode of meningitis 2 months previously. CT cisternography and cranial MRI revealed a defect in the lateral wall of the sphenoid sinus, with an anteromedial temporosphenoidal encephalocele. Surgery was performed transcranially through a pterional approach. The temporal encephalocele was amputated, the sphenoid sinus obliterated and the dural defect repaired. Lumbar drainage was used for 5 days after surgery. Spontaneous CSF rhinorrhoea is only infrequently due to temporal encephalocele. Anteromedial temporosphenoidal encephaloceles are the least common type of temporal encephalocele, with only 12 reported in the literature.

Cerebrospinal Fluid Rhinorrhea↗
PubMed2002

Traumatic encephalocele related to orbital roof fractures: report of six cases and literature review.

BACKGROUND: Orbital roof fractures after blunt injury are rare. Traumatic encephaloceles in the orbital cavity are even rarer, with only 15 cases published to date. METHODS: The clinical, radiological, and surgical findings of 6 cases of traumatic encephalocele treated at our institution from June 1998 to January 2000 are presented. They are also compared with previously published series. RESULTS: In contrast to other published cases, 5 out of 6 patients in our series were adults. The most common cause of trauma was road traffic accident. Ecchymosis and preoperative exophthalmos/proptosis were frequent. In all of our patients a coronal CT scan (3 mm increments with bone windows) was obtained. It demonstrated the extension of the orbital roof fractures and a possible encephalocele in 4 cases. Associated frontal brain contusions were seen in 5 cases. An MRI was performed in 3 patients (and only in 2 previously published cases); it showed the extension of the brain herniation into the orbital cavity. Surgical treatment via a fronto-basal approach with evacuation of the contused herniated brain tissue and orbital roof reconstruction was performed. The outcome at 6 months was good recovery in five patients with one patient still in a persistent vegetative state. Postoperatively the ocular disturbances improved in 5 cases. A review of the other published cases confirmed recovery of normal ocular function in the vast majority of the cases. CONCLUSIONS: Whenever orbital roof fractures associated with frontal contusions are identified in an acute brain injured patient, an orbital encephalocele should be suspected. In our opinion MRI is the investigation of choice in such patients. If the encephalocele is confirmed, a surgical approach via the subfrontal route is indicated with resection of herniated contused brain tissue, dural closure, and orbital roof reconstruction. Good results in regard to the orbital symptoms (mainly exophthalmos) can be expected.

Adult↗
PubMed2003

Survival of infants diagnosed with encephalocele in Atlanta, 1979-98.

This study aimed to evaluate the survival of a cohort of liveborn infants diagnosed with encephalocele during a 20-year period and the variation of such survival by selected demographic and clinical characteristics. We reviewed data from the Metropolitan Atlanta Congenital Defects Program (MACDP) to ascertain all live births diagnosed with encephalocele (n = 83) from 1979 to 1998. Of these, 66 (79%) had isolated defects. Among 70 liveborn infants with site of the defect specified, 50 were classified as having posterior and 20 with anterior defects. To identify their vital status, we used data from MACDP hospital records and vital records from the State of Georgia supplemented by linking registry data with the National Death Index from 1979 to 1999. Among children with encephalocele, 76.0% of the deaths (19/25) occurred during the first day of life. The survival probability to 1 year of age was 70.8%[95% confidence intervals (CI) 60.9, 80.7] and to 20 years of age was 67.3%[95% CI 55.7, 78.8]. In multivariable analysis, factors associated with increased mortality were low birthweight (<2500 g) [relative risk (RR) 5.18; 95% CI 2.13, 12.63], presence of multiple defects (RR 2.82; 95% CI 1.19, 6.69) and black race (RR 2.36; 95% CI 0.95, 5.85). Overall survival for infants with multiple defects (41.2%) was significantly poorer than survival among those with isolated defects (74.3%). A 70% decrease in risk of mortality was observed among infants born with encephalocele during 1989-98 compared with those born during 1979-88, but this decrease was evident only among cases with low birthweight (RR 0.29; 95% CI 0.01, 0.90). This study highlights the prognostic importance of multiple defects and low birthweight for infants with encephalocele and identifies a statistically significant difference in survival by race. This information is useful for clinicians and families who must plan for the long-term care of affected children.

Abnormalities, Multiple↗
PubMed1999

Subependymal nodular heterotopia in patients with encephalocele.

Only incidental mention has been made to date of the combined occurrence of subependymal heterotopia and posterior encephalocele. We evaluated the presence of disseminated nodular subependymal heterotopia in two series of patients with posterior encephalocele. The first series consisted of all six patients who were treated in our hospital for encephalocele during the last 11 years and who underwent magnetic resonance imaging (MRI). In three, subependymal nodular heterotopia was found by MRI. The second series consisted of eight autopsy cases with encephalocele, representing all cases of encephalocele that came to autopsy during a 10-year period on whom full microscopic examination could be performed. Nodular heterotopia was found in four. The combined occurrence of these two rare conditions may not be accidental.

Brain Diseases↗
PubMed2006

Management of cerebral vascular structures during endoscopic treatment of encephaloceles: a clinical report.

OBJECTIVES: Otolaryngologists are increasingly being called upon to treat patients with cerebrospinal fluid leak and encephaloceles. The endoscopic approach to the skull base through the nose and paranasal sinuses has proven effective and is well tolerated by patients. With its more widespread and frequent use, unusual cases and potential complications are becoming more apparent. METHODS: Treatment of two clinical cases in which a cerebral vascular structure was encountered during endoscopic treatment of an encephalocele is presented, and the condition is reviewed. RESULTS: Two patients presented after a skull base injury that occurred during endoscopic sinus surgery. In each case the initial treating surgeon attempted endoscopic repair of a cerebrospinal fluid leak, but the repair failed and the leak persisted. Upon referral to the author, in each case, a traumatic encephalocele with an active leak was apparent, and during repair a cerebral vessel was encountered. It appeared that the vessel had been "pulled down" into the skull base defect with the encephalocele's migration into the sinonasal cavity. CONCLUSIONS: This unusual clinical condition is discussed along with the potential complications that can result from it. Otolaryngologists who treat encephaloceles should be aware of the possibility of encountering a cerebral vessel and should understand the potential complications and management options.

Bone Transplantation↗
PubMed2006

Both nasal cerebral heterotopia and encephalocele in the same patient.

UNLABELLED: OBJECTIVE AND PATIENT: Both nasal cerebral heterotopia and encephalocele are rare congenital benign masses of neurogenic origin caused by an embryonic developmental abnormality. It is generally accepted that nasal heterotopia is a sequelae to encephalocele. This report presents an unusual case of nasal cerebral heterotopia and encephalocele arising in the same patient. The patient had a firm, solid mass measuring 1.5 x 1.0 cm on the bridge of the nose covered with normal skin and another mass in the nasal cavity obstructing the right nasal cavity. Computed tomography (CT) demonstrated that the nasal bone separated these masses. CT also showed a bony defect at the skull base. Surgery consisted of dividing the encephalocele and closure of the skull base fistulae, along with nasal subcutaneous mass enucleation. RESULTS: Intraoperative examination indicated the existence of a pit on the nasal bone where the pedicle of the nasal subcutaneous mass connected. Microscopic examination of the nasal cavity mass demonstrated meningoencephalocele, and examination of the nasal subcutaneous mass demonstrated nasal cerebral heterotopia, which was confirmed by immunohistochemical staining. After 10 months, complete removal of the subcutaneous nasal mass was recognized and there was no evidence of recurrence. CONCLUSION: Findings in this case suggest that the nasal cerebral heterotopias will result from encephalocele.

Brain↗
PubMed1989

Nasal cerebral heterotopia: the so-called nasal glioma or sequestered encephalocele and its variants.

Twenty two nasal cerebral heterotopias were compared with 11 nasal encephaloceles. No histological feature was found that would allow a communication with the brain to be confidently identified or excluded. Even laminated cerebral cortex with neurones and ependymal canals, suggestive of encephalocele, were found in heterotopias. Distinction required radiological and surgical evidence. However, CT scan could be misleading, in one infant suggesting a cribriform plate defect when none was found at craniotomy. Three children had multiple extracranial glial lesions, two with both heterotopia and encephalocele in the same patient. In a few older children it was extremely difficult to identify brain tissue because of marked replacement by fibrous tissue (up to 95%), leading to one misdiagnosis as fibroma, and considerable fibrosis occurred also in five of six recurrences and in a longstanding small encephalocele. In two heterotopias, cellularity in places approached that of low-grade neoplastic glioma. One nasopharyngeal heterotopia contained multiple mesenchymal tissues suggestive of teratoma. Two midline nasopharyngeal encephaloceles showed adjacent epithelium, possibly vestiges of Rathke's pouch.

Brain↗
PubMed2002

Endoscopic endonasal treatment of a spontaneous temporosphenoidal encephalocele with a detachable silicone balloon. Case report.

Temporosphenoidal encephaloceles are rare entities that occur when the temporal lobe herniates into the sphenoid sinus through a skull base defect of the temporal bone. Both an iatrogenic and a traumatic pathogenesis have been proposed. The authors describe a spontaneously occurring temporosphenoidal encephalocele in a 63-year-old woman who had a 4-year history of rhinorrhea. Spiral computerized tomography (CT) scanning revealed a bone defect located inside the ophthalmomaxillary triangle. The intrasphenoidal encephalocele had a heterogeneously hypointense signal compared with cerebrospinal fluid (CSF) on T1-weighted magnetic resonance (MR) images and a hyperintense signal compared with CSF on T2-weighted MR images. Two previous endonasal endoscopic procedures, performed by ear, nose, and throat surgeons, had been unsuccessful. The authors performed an endoscopic endonasal right nostril procedure by using 0 degrees and 45 degrees rigid-lens endoscopes that were 4 mm in diameter and 18 cm long. The encephalocele in the sphenoid sinus was partially removed. DuraGen and fat graft were positioned in the bone defect. Two No. 2 French detachable silicone balloons (1.5 cm3 volume) inflated with surgical glue were introduced into the skull defect and into the sphenoid sinus, respectively. The CSF leakage stopped immediately. No nasal packing or postoperative CSF lumbar drainage was necessary. The patient did well. Postoperative CT and MR imaging, obtained at 24 hours and at 3 months, demonstrated that the balloon and the fat graft filled the bone defect and the sphenoid sinus. Eight months postprocedure no CSF leakage was observed. This appears to be the first case reported in the literature of a temporosphenoidal encephalocele successfully treated by an endoscopic endonasal technique involving packing of the defect with inflated detachable balloons.

Bone Cements↗
PubMed2003

[Fronto-ethmoïdal encephaloceles in Dakar. Report of 9 cases].

A retrospective study of 9 cases of fronto-ethmoïdal encephaloceles is reported. Cases have been gathered from the files of Dakar University Neurosurgical Unit. Fronto-ethmoïdal encephaloceles were about 9.8% of all encephaloceles. In all the cases the exit hole from the anterior cranial fossa is at the site of the foramen caecum. The location of the tumor was fronto-nasal in seven patients and naso-ethmoïdal in two. The cranio-facial deformity consisted of increase of local volume and a lateral displacement of medial canthus in six cases, a down ward drift of the tip of the nose in two cases and one case of orbital hypertelorism. Eight cases were meningo-encephaloceles and the last a meningo-encephocystocele. Diola was the most ethnic group involved. All patients have been operated by a simple neurosurgical procedure without obstruction of the cranial defect nor bony displacement. In five cases mental and aesthetic results were good when operation was done during the first year of live. The authors stress early surgical treatment for fronto-ethmoïdal encephaloceles.

Child, Preschool↗
PubMed1978

[Craniolacunia in newborns with myelomeningocele and encephalocele (author's transl)].

Craniolacunia is characterized by clusters of deep pits with steep edges in the cranial vault of newborn infants. And also craniolacunia is anomaly of the skull of the newborn infants usually associated with spinal meningocele, myelomeningocele and occasionally with encephalocele. The purpose of this paper is to report the results of the skull roentgenologic study of 34 newborn infants with myelomeningocele and 11 newborns with encephalocele who were treated within 7 days after birth, and, to discuss the correlations with the head circumference, level of myelomeningocele, location of encephalocele, Arnold-Chiari malformation and hydrocephalus. Roentgenograms of the skull were obtained at the time of admission and were examined regarding the presence and the degree of craniolacunia. The radiographic changes were classified according to the extension of the craniolacunia, from grade 0 to grade 3. Among 34 cases of myelomeningocele, 28 cases (82%) were observed to have craniolacunia and among 11 cases of encephalocele, 4 cases (36%) had findings of craniolacunia. In our cases, the coincidence of craniolacunia with myelomeningocele was 82% and this is higher than in previous reports. But the coincidence of craniolacunia with encephalocele was same as in previous reports. There has been no report about the incidence of craniolacunia within 7 days of life.

Encephalocele↗