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PubMed1995

Repair of temporosphenoidal encephalocele with a vascularized split calvarial cranioplasty: technical case report.

A 54-year-old woman was referred for the evaluation and treatment of spontaneous cerebrospinal fluid rhinorrhea. Magnetic resonance imaging revealed a right anteromedial intrasphenoidal encephalocele with cisternography showing the flow of contrast into the sphenoid encephalocele. Transcranial surgical repair was performed. This included the use of a vascularized split-thickness calvarial graft fixated over the cranial base defect with a titanium microplate. The technical details of this repair are discussed. Ten cases of temporosphenoidal encephaloceles have been reported. The literature concerning this rare form of encephalocele is reviewed.

Cerebrospinal Fluid Rhinorrhea↗
PubMed2001

Endoscopic management of basal encephaloceles.

Encephaloceles have been traditionally treated by a transcranial or external approach. The advent of endoscopic sinus surgery has allowed a new intranasal approach for the treatment of basal encephaloceles, minimising patient morbidity. We discuss the classification and presentation of encephaloceles, and present our experience in the repair of six cases. We believe transnasal endoscopic repair is the method of choice in dealing with selected basal encephaloceles.

Adult↗
PubMed2005

Concordant occipital encephalocele in monoamniotic twins.

Anomalies occur with a greater frequency in twin gestations. Due to its multifactorial inheritance, twins are usually discordant for encephalocele. We present a case of monoamniotic twins concordant for occipital encephalocele and discordant for lung and cord anomalies. Ultrasonographic examination at 17 weeks' gestation revealed occipital encephalocele in both fetuses. The maternal serum level of alpha-fetoprotein was increased. Fetal autopsy revealed occipital encepaholocele in both twins and right pulmonary hypoplasia and one umbilical artery in one sibling. Monoamniotic twins concordant for encephalocele occur with extreme rarity. To the best of our knowledge, monoamniotic twins concordant for this neural tube defect have not been previously reported.

Adult↗
PubMed1990

The Morning Glory syndrome associated with sphenoethmoidal encephalocele.

A 10-month-old infant was referred to the ophthalmic department of the Royal Victoria Hospital for assessment. Pregnancy and delivery had been normal. At birth, cleft lip, cleft palate, hypertelorism and a soft tissue mass in the mouth were noted. At two days of age he developed meningitis, axial computerized tomography showed a sphenoethmoidal encephalocele, and agenesis of the corpus callosum. The basal encephalocele was surgically repaired. On examination in the ophthalmic department he was able to follow a light with the left eye. He perceived light with the right eye but did not follow. There was a manifest right divergent squint, measuring -10 degrees, and a right afferent nerve defect. Examination under anaesthetic was performed. The right fundus showed a Morning Glory syndrome. The disc was pink and deeply excavated, and surrounded by a ring of chorioretinal pigmentary disturbance. There was a central tuft of glial tissue. The left fundus was normal. The association of Morning Glory syndrome and basal encephalocele is rare; four previous cases have been reported. In all patients there were associated mid-facial congenital anomalies such as cleft lip and cleft palate. The presence of a basal encephalocele should be suspected if the Morning Glory syndrome occurs in association with mid-facial congenital anomalies.

Abnormalities, Multiple↗
PubMed2005

Spontaneous motor cortex encephalocele presenting with simple partial seizures and progressive hemiparesis. Case report and review of the literature.

Several cases of congenital or acquired temporal encephaloceles have been reported in the literature as the causative mechanism of simple and/or complex partial seizures. In this report the authors describe a rare case of spontaneous parietal encephalocele presenting with simple partial seizures and progressively increasing contralateral upper-extremity motor deficit. The unusual anatomical location of an encephalocele associated with seizures and the delayed seizure onset represent distinctive characteristics in this case. Preoperative imaging included surface electroencephalography, computerized tomography, and brain magnetic resonance imaging. Frameless neuronavigation and intraoperative cortical mapping were used to aid resection of the encephalocele, and the dural and bone defects were reconstructed. The surgical outcome in this case was excellent, and the patient has remained seizure free. The pertinent literature is reviewed in this report.

Disease Progression↗
PubMed1989

Subtorcular occipital encephaloceles. Anatomical considerations relevant to operative management.

Three cases of occipital encephalocele, one with associated myelomeningocele, are presented. All received preoperative evaluation with magnetic resonance imaging. Such studies provide optimal demonstration of the cerebral and hindbrain anatomy to guide operative treatment and formulate prognosis. Review of available radiographic, operative, and pathological information suggests that most, if not all, occipital encephaloceles are associated with an anomaly of the hindbrain, and the usual anomaly is a rhombic roof encephalocele. In such cases, the site of cranial herniation is caudal to the torcula, regardless of the presence or absence of occipital lobe tissue within the sac. Experimental and clinical analysis suggests that occipital encephaloceles most likely arise from abnormalities in the development of the skull base.

Brain Stem↗
PubMed1992

Tension pneumocephalus associated with rupture of a middle fossa encephalocele. Case report.

Acquired nontraumatic (spontaneous) encephaloceles of the middle fossa are not common. Rupture of an encephalocele frequently leads to a cerebrospinal fluid fistula. Tension pneumocephalus consequent to rupture of an encephalocele has not been previously reported, but conceivably occurs by means of a ball-valve mechanism in the ensuing fistulous tract. An unusual case is presented of an elderly man who suffered acute life-threatening neurological symptoms from a tension pneumocephalus that likely developed from rupture of an acquired nontraumatic encephalocele of the left middle fossa. The symptoms correlated with the location of the intracranial abnormalities. The literature is reviewed and the pathophysiology of the lesion is discussed.

Aged↗
PubMed1999

A rare case of upper airway obstruction in an infant caused by basal encephalocele complicating facial midline deformity.

A four-month-old male infant with basal encephalocele of the transsphenoidal type presented with upper airway obstruction and facial midline deformity, including cleft lip, cleft palate, hypertelorism and exophthalmos. Basal encephalocele is a rare disease, and usually not detectable from the outside. In this case, initially the cause of an upper airway obstruction was considered to be posterior rhinostenosis, and posterior rhinoplasty with inferior nasal conchectomy was scheduled. However, in preoperative examination, computed tomography (CT) and magnetic resonance imaging (MRI) revealed a bony defect in the sphenoidal bone and a cystic mass in communication with cerebrospinal fluid, herniating into the nasal cavity through the bony defect. The mass was diagnosed as a transsphenoidal encephalocele, the scheduled operation cancelled, and tracheostomy performed for airway management. The possibility of basal encephalocele should be considered in the case of upper airway obstruction with facial midline deformity.

Cleft Lip↗
PubMed1992

[A case of transethmoidal encephalocele].

A case of a 3-year-old boy with transethmoidal encephalocele is presented. The patient was found to have bacterial meningitis, which responded well to an intravenous antibiotics therapy. No physical anomaly was evident on examination but plain skull X-ray film showed cloudiness of the left nasal antrum. Coronal CT scan disclosed a defect in the left cribriform plate and soft tissue mass in the left nasal cavity. MRI showed an anterior basal encephalocele protruding into the nasal cavity. Hypothalamic-pituitary system and the optic nerves appeared normal in the sagittal image. CSF rhinorrhea was confirmed by RI cisternography. An operation was performed transcranially. After a left frontal craniotomy, a unilateral bony defect in the cribriform plate and protrusion of the brain was observed subfrontally. The crista galli was intact. The herniated brain substance was transected and partially removed and the bony defect plugged by temporal muscle and covered by lyofirized dura. Microscopic examination of the herniated brain mass revealed gliosis and capillary proliferation. The patient recovered well and there has been no recurrence of CSF rhinorrhea or meningitis. Basal encephalocele is a very rare congenital anomaly. It is reported to constitute 1 to 10% of all encephaloceles. Incidence is estimated as 1 in every 35,000 to 40,000 live births. The anomaly is classified into two subtypes; transethmoidal (TE) and transsphenoidal (TS).(ABSTRACT TRUNCATED AT 250 WORDS)

Child, Preschool↗
PubMed2005

Transalar encephalocele associated with Wegener granulomatosis and meningeal enhancement: case report.

Transalar encephaloceles are rare lesions that do not fit the standard classification of basal encephaloceles. Typically, these lesions present in adulthood, with nonspecific symptoms. We report here a case of a patient with Wegener disease in whom a large transalar encephalocele posterior to the sinus was noted when he was preoperative for left maxillary sinus surgery. The encephalocele demonstrated irregular peripheral enhancement along the margin--a very uncommon finding--as well as contrast enhancement of the basal meninges, which can be seen occasionally with Wegener granulomatosis.

Encephalocele↗
PubMedDate not supplied

[Dural bone defects and encephalocele associated with chronic otitis media or its surgery].

OBJECTIVES: We investigated the incidence of dural bone defects and encephalocele following radical mastoidectomy or open cavity tympanoplasty. PATIENTS AND METHODS: We retrospectively evaluated 190 operations of 182 patients (97 males, 85 females; mean age 30.6 years; range 9 to 70 years) who underwent radical mastoidectomy (n=89) or open cavity tympanoplasty (n=101). Follow-up examinations included otomicroscopy. Patients with documented dural bone defects on examination or operation records were further evaluated by axial and coronal computed tomography scans of the temporal bone. Those with suspected encephalocele were studied by magnetic resonance imaging. RESULTS: Dural bone defects were detected in 17 patients perioperatively. Of these, 14 defects were associated with chronic otitis media. Three iatrogenic bone defects were induced during obliteration of lateral sinus thrombosis. The mastoid tegmen was the most common site of defects (41%) and the most common etiologic factor was cholesteatoma (84%). Encephalocele was detected in one patient in whom radical mastoidectomy with lateral sinus obliteration was performed due to lateral sinus thrombophlebitis. Encephalomalasia was found nearby the defect area in two patients. CONCLUSION: Dural bone defects associated with chronic otitis media are not rare, the most common cause being cholesteatoma. Repair of dural lacerations requires watertight closure to prevent the occurrence of encephalocele.

Adolescent↗
PubMed1982

Basal encephalocele associated with suprasellar epidermoid cyst.

A 27-year-old woman suffered from multiple congenital defects, including transsphenoidal encephalocele. Recent progressive visual loss was at first attributed to this encephalocele, but was later proved to be caused by a suprasellar epidermoid cyst. Its removal was followed by improvement of vision. To the best of our knowledge, the association of basal encephalocele and epidermoid cyst has not been previously described.

Abnormalities, Multiple↗
PubMed1983

Intranasal encephalocele masking as a nasal polyp in an adult patient.

Intranasal encephaloceles are rarely seen in adult patients. These lesions may be mistaken for nasal polyps. A 36-year-old woman died after the removal of an intranasal encephalocele during a routine polypectomy. The possibility of an intranasal encephalocele must be considered when examining adult patients with nasal polyps.

Adult↗
PubMed1985

The induction of microphthalmia, encephalocele, and other head defects following hyperthermia during the gastrulation process in the rat.

The aim of this study was to ascertain whether there is a period during early embryonic development of the rat that is particularly sensitive to hyperthermia. Pregnant Sprague-Dawley rats were partially immersed in a water bath at 43.5 degrees C until their core temperatures, monitored by a rectal thermistor probe, were elevated to 43.5 degrees C. The procedure was repeated 6 hours later. The regimen of two heatings was performed over a range of development from early gastrulation (8 days 18 hours) to about the 12 somite stage (10 days 18 hours). The rats were killed on days 17-19 and the fetuses were examined. Each group contained a minimum of five litters. The main teratogenic effect of the hyperthermia was the induction of one or more head defects, notably microphthalmia, encephalocele (either a single, large, parietal encephalocele or multiple small protuberances), and maxillary hypoplasia. Microphthalmia was the most common defect with approximately 90% of surviving fetuses having small eyes when heating occurred between 9 days 6 hours and 10 days 0 hours (9.06 and 10.00). Encephaloceles were induced by heating between 9.00 and 10.00 with a peak sensitivity between 9.12 and 9.18 when 57% of surviving fetuses were affected. Maxillary hypoplasia resulted from heating between 9.06 and 10.06 with up to 20% of surviving fetuses being affected. Control rats were exposed to the same experimental procedure in a water bath at 38 degrees C on 9.12 and 9.18, the gestational time most sensitive to hyperthermia induced malformations. There were no abnormal fetuses in the controls. The critical period identified spans 9 days 6 hours to 10 days 0 hours gestational age. In developmental terms this includes a large proportion of the gastrulation process.

Animals↗
PubMed1992

Repair of giant occipital encephaloceles with microcephaly secondary to massive brain herniation.

Giant occipital encephaloceles rarely contain large amounts of neural tissue that cannot be replaced in the abnormally small calvarium. Resection of neural elements is therefore often necessary in order to accomplish a closure. A technique is described wherein an extracranial compartment is prepared utilizing fine tantalum mesh to enclose the neural contents. The mesh is attached to the periphery of the skull defect providing a rigid extracranial compartment for the encephalocele. As intracranial pressure increases, the calvarium is forced to expand. The tantalum mesh is gradually imbricated into the calvarium by daily digital compression. If ventriculomegaly occurs, an interval ventriculoperitoneal shunt is placed. The encephalocele repair is reopened and the tantalum is surgically imbricated at that time. This allows for a satisfactory cosmetic result with preservation of all neural elements.

Encephalocele↗
PubMed1981

A large convexity meningioma followed by liquorrhoea from a nasal encephalocele. A case report.

A 46-year-old woman was readmitted, two years after removal of a large convexity meningioma, because of cerebrospinal fluid rhinorrhoea from a nasal encephalocele eventually visualized by CT-scan. The encephalocele was extirpated via an intracranial approach, and its histological appearance is described. The aetiology of this extracranial brain tissue protrusion is discussed. It is suggested that the mechanisms behind the formation of this encephalocele are a combination of a congenital malformation and the long term increase of the intracranial pressure due to the meningioma.

Brain Neoplasms↗
PubMed2004

Surgical closure and reconstruction of a large occipital encephalocele without parenchymal excision.

INTRODUCTION: Encephalomeningocele is a herniation of the brain and meninges through a congenital bone defect resulting from the failure of normal midline fusion of the cranial neural tube. Various sites are possible; however, among Caucasians more than 70% of encephaloceles are located on the occipital/posterior part of the cranium. Usually they contain degenerative cerebral cortex, which is often excised during surgical closure of the defect. Techniques to preserve the herniated brain tissue have been described, but they focus on increasing the intracranial cavity by ventriculomegaly and retracting the encephalocele into new intracranial space in a second stage, as a result of hydrocephalic treatment. TECHNICAL REPORT: The authors report a well-documented newborn with a large encephalocystocele of the left occipital lobe. This encephalocele included the left lateral ventricle and a part of the cerebellum, and was covered with allopathic skin. Initially the cystic portion was removed so that a partial reduction of the encephalocystocele was achieved. Six months later, the surgical closure of the defect was performed, with preservation of the occipital and cerebellar parenchyma, by incising the tentorium and retracting the cortex to the newly created infratentorial space. The bony defect was covered with autologous osseous graft harvested from parietal bone and reconstructed. OUTCOME: In the long-term follow-up after 13 years, the child has preserved visual function.

Child↗
PubMedDate not supplied

Encephalocele in the ethmoid sinus presenting as a massive intracerebral hemorrhage after a "polypectomy": a case report.

The discovery of a congenital transethmoidal encephalocele in an adult patient is very rare and is sometimes misdiagnosed as a polyp of the ethmoid sinus. It is important to note that a congenital transethmoidal encephalocele presenting as a massive intracerebral hemorrhage in an adult patient has never been reported. This study's patient underwent endoscopic polypectomy and suffered from massive intracerebral hemorrhage because the encephalocele contained a frontobasal artery. Transcranial surgery was successfully performed to repair the frontal base. We report on the patient's condition and discuss the clinical implications of the present case.

Arteries↗