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PubMed1975

Optic nerve anomalies in basal encephalocele.

Basal encephalocele should be suspected in child when hypertelorism, defect of the bridge of the nose, and other midfacial anomalies are present. Optic nerve anomalies may occur with basal encephalocele. Those previously described are pale discs, coloboma of the optic nerve head, and pit of the optic disc. Two additional disc anomalies that are associated with basal encephalocele are megalopapilla and optic nerve dysplasia. A mass in the nose or nasopharynx under these circumstances is most likely an encephalocele and biopsy is contraindicated.

Abnormalities, Multiple↗
PubMed1987

Intrasphenoidal encephaloceles--a clinical entity.

In the current classification basal encephaloceles are grouped together with real transsphenoidal encephaloceles. But those encephaloceles extending only into but not through the sphenoid sinus seem to represent a specific clinical entity and therefore should be regarded as a rare subgroup of sphenoidal encephaloceles. One personal case and six cases from the literature are reviewed, the own case being associated with an empty sella turcica. The initial sign is rhinorrhea, almost invariably. The association with other intracranial anomalies is uncommon. The extradural transsphenoidal or transethmoidal midline approach accompanied by a shunting procedure today is the most suitable method of surgical treatment.

Adult↗
PubMedDate not supplied

Anterior encephaloceles.

Sixty five children with anterior encephaloceles were retrospectively studied for clinical presentation, radiological findings, surgery and outcome. They were sampled over a 22 years period (Jan 1973-Dec 1994). Only 6 children were above 10 years of age. Naso-ethmoid type was the most frequent type, encountered in 45 patients. Surprisingly, nasopharyngeal type was observed in 6 children. Swelling over the nose and hypertelorism were usual findings, recorded in 53 and 50 patients respectively. Ten patients, each, had CSF rhinorrhoea and proptosis. In fifty four patients CT scans were performed and bony defect could be demonstrated in 45 of them. Three each had agenesis of corpus callosum and porencphalic cysts. Six patients with hydrocephalus underwent ventricul operitoneal shunt, prior to surgical repair of the encephaloceles. In fifty nine patients, one stage repair of encephalocele and hpertelorism was carried out. There was one postoperative mortality. Ten patients developed postoperative CSF rhinorrhoea, among them 6 required percutaneous lumbo-peritoneal shunt. This study brings out the rare cases of nasopharyngeal encephaloceles and highlights the need for one stage repair in these patients.

Adolescent↗
PubMed2002

Occipital encephalocele, lipomeningomyelocele, and Chiari I malformation: case report and review of the literature.

CASE REPORT: We report a pediatric patient with encephalocele, lipomeningomyelocele, and Chiari I malformation. DISCUSSION: We also review the extant medical literature regarding associations between these three entities. We propose that the combination of these three pathologies, which is reported here for the first time, is not serendipitous but rather a low-frequency association. CONCLUSIONS: After a literature review, we speculate that encephalocele and lipomeningomyelocele most probably occur by mechanisms similar to those that produce encephalocele and meningomyelocele and that the tonsillar ectopia in our patient is due to an abnormally small posterior cranial fossa resulting from the lack of neural tissue within the cranium at critical times during development or has its genesis, as some encephaloceles may, in inappropriate paraxial mesoderm formation.

Arnold-Chiari Malformation↗
PubMed2003

Morning glory syndrome and basal encephalocele.

BACKGROUND: Morning glory syndrome (MGS) is a congenital optic disc dysplasia often associated with craniofacial anomalies, especially basal encephalocele. Clinical presentations are varied and often occult. CASE REPORT: We describe a case of bilateral MGS associated with basal encephalocele that was detected by chance when treatment was sought for respiratory distress. CONCLUSIONS: MGS and basal encephalocele should always be suspected in cases of midline deficiencies, particularly when ophthalmic signs of strabismus or poor vision are present. CT and MRI should be performed to delineate the extent of the lesion and a complete hormone screening should be carried out to exclude pituitary deficiency. The pathogenesis of MGS and basal encephalocele are unknown; however, it is thought to occur during the 5th week of embryonic development.

Encephalocele↗
PubMed1993

A ten-year review of encephalocele in a teaching hospital.

A 10-year review of infants with encephalocele was carried out in a multicentered teaching hospital. Out of 23,438 infants seen within the period, only 12 cases of encephalocele were seen, giving an incidence of 0.5/1000 births. Nine (75%) of patients had occipital encephalocele, two (16.7%) occipitoparietal and one (8.3%) fronto-nasal. Two patients had spina bifida as an associated neural tube defect while ocular abnormalities were seen in another two. None had neurological defects at birth but some showed evidence of hydrocephalus and delayed milestones after surgery. Ultrasonic diagnosis was made in one case only. The etiologic, prognostic and descriptive characteristics, in addition to obstetric management of encephalocele are discussed.

Encephalocele↗
PubMed1990

Intractable complex partial seizures associated with occult temporal lobe encephalocele and meningoangiomatosis: a case report.

Occult congenital temporal lobe encephalocele has rarely been reported in association with medically intractable complex partial seizures. The four previously reported cases were unsuspected preoperatively. We present the case of an 18-year-old woman with intractable complex partial seizures since age 13. Seizure onset was electrically localized to the right temporal lobe. Preoperative neuroimaging studies revealed a middle fossa defect and inferior herniation of the right temporal lobe. Pathologic examination of the resected encephalocele revealed prominent features of meningoangiomatosis. We believe this to be the first case of temporal lobe encephalocele and epilepsy to be diagnosed preoperatively, and the first case also to be associated with meningoangiomatosis. The relevant literature on meningoangiomatosis and on temporal lobe encephalocele as a cause of epilepsy is reviewed.

Adolescent↗
PubMed1988

Transmastoid encephaloceles. A case report.

Encephaloceles are uncommon abnormalities occurring in the pediatric age group. For the otolaryngologist they are usually encountered in the nasopharynx or nose, but may also be found in other sites adjacent to the brain. Encephaloceles usually develop as a result of prior mastoid surgery, trauma, or infections; but it is extremely rare for large encephaloceles to occur congenitally in the mastoid. It is even more uncommon for there to be a second mastoid defect which would allow the brain to present as a subcutaneous postauricular mass. The case history of a child with congenital subcutaneous transmastoid encephalocele is presented and the management discussed. In this child, the right temporal lobe of the brain herniated through a congenital defect in the tegmen and then through the mastoid cavity to exit through a lateral wall defect in the mastoid cortex. It then presented as a postauricular subcutaneous mass. The initial diagnosis was a subperiosteal abscess. The correct diagnosis was not made until surgery despite a normal preoperative CT evaluation. The world literature is reviewed and similar diagnostic and therapeutic problems are discussed.

Child, Preschool↗
PubMed2006

Anterior encephaloceles: a series of 103 cases over 32 years.

Anterior encephalocele is a rarely reported CNS malformation with a geographical predilection for South-East Asia. The paucity of literature prompted us to analyse our results with hemiorbital advancement and classical Tessier's operation in 103 cases over 32 years (1971-2002). In our series, the frontoethmoidal subtype was the most common (80%), followed by the orbital (8%), transethmoidal (8%), transsellar (3%) and interfrontal types (1%). All patients with classical frontoethmoidal encephalocele had swelling over the nose or inner canthus since birth, with varying degrees of hypertelorism; and those with the nasopharyngeal subtype commonly presented with nasal obstruction and cerebrospinal fluid (CSF) rhinorrhoea. Neurofibromatosis was present in seven patients, all of whom had an orbital encephalocele. The diagnostic workup included a computerised tomography scan of the head in 96 patients and magnetic resonance imaging of the brain in 16 patients. The associated findings on imaging were hydrocephalus (15%), corpus callosum agenesis (7%), arachnoid cyst (3%), porencephalic cyst (3%) and single ventricle (3%). A classical Tessier's operation was performed in the initial 30 cases. However, since 1988, we have been performing a single stage hemiorbital advancement and repair of the encephalocele. There were three deaths, one due to pneumonia and two due to fulminant meningitis. Twenty-two patients (22%) had postoperative CSF leak, of which five required lumboperitoneal shunt placement. We believe that hemiorbital advancement offers satisfactory results with less morbidity than the Tessier's operation.

Adolescent↗
PubMed2006

[Intradiploic encephalocele in an adult. A case report and review of the literature].

We describe a rare case of parietal intradiploic encephalocele in a 51-year-old woman with no history of head trauma. The patient presented with a 1-month history of left hemiparesthesia. A hard indolent scalp lesion was palpable on examination in the right parietal area. Skull x-rays and cranial computed tomography examination demonstrated a lytic lesion that was consistent with a malignant osteolytic skull lesion. Magnetic resonance imaging of the head revealed an intradiploic cyst that included a round tissue mass contiguous with the parietal cortex. Surgery confirmed the diagnosis of encephalocele and the patient underwent surgical resection of the herniated brain, duraplasty, and cranioplasty. The presenting hemiparesthesia persisted at the 6-month follow-up. Encephaloceles of the cranial vault are a rare complication of skull fractures and rarely occur in adults. These lesions can be difficult to distinguish from congenital encephaloceles in patients with no history of head trauma. The pathogenesis, clinical and radiological nuances and the role of surgery are discussed.

Cerebellar Diseases↗
PubMed2004

Endoscopic treatment of encephaloceles of the lateral wall of the sphenoid sinus.

OBJECTIVE: The suitability of the endoscopic approach for the treatment of an encephalocele of the lateral wall of the sphenoid is discussed. This is a retrospective review of 4 cases diagnosed with temporosphenoidal encephalocele and having a history of CSF leak who were surgically treated using an endoscopic endonasal approach between January 2001 and June 2002 at the Department of Otolaryngology of Sant'Orsola-Malpighi University Hospital and the Department of Neurosurgery at Bellaria Hospital in Bologna. METHODS: Three patients were female between 48 and 73 years of age (mean: 61 years). All patients had suffered from a CSF leak for 5 months to 18 years. None of the patients had a past medical history of head trauma. A fourth patient had undergone a previous microscopic approach for a previously misdiagnosed CSF leak wrongly ascribed to an empty sella. Three patients underwent an ethmoid-pterygo-sphenoidal endoscopic approach (EPSEA), while the patient who had undergone previous microscopic surgery, was treated using a transnasal transsphenoidal endoscopic approach. RESULTS: The follow-up of the patients ranged from 10 to 26 months (mean: 18 months) and no case of a recurrent CSF leak was observed postoperatively. CONCLUSIONS: In our report, the endoscopic approach was a useful tool for the treatment of encephaloceles of the lateral wall of the sphenoid sinus. In skilled hands, this technique permits both the resection of the encephalocele and the subsequent reconstruction of the defect also with a low rate of morbidity.

Aged↗
PubMedDate not supplied

Axial skeleton and pituitary gland in human fetuses with spina bifida and cranial encephalocele.

The purpose of this study was to investigate the axial skeleton and the pituitary gland in fetuses with spina bifida or cranial encephalocele in order to elucidate the pathogenesis of the conditions. The findings were related to former investigations performed on normal fetuses and on fetuses with anencephaly and rachischisis. Eight human fetuses from spontaneous or therapeutic abortions, 11-28 weeks of gestational age, were investigated. Radiographs were taken of the axial skeleton and histological investigation, including immunohistochemical marking for thyroid-stimulating hormone was performed on tissue blocks of the cranial base, including the sella turcica and the pituitary gland. Radiography revealed only minor malformations in the axial skeleton and not in all cases. The types of malformations resembled those seen in anencephaly and rachischisis. Histological investigations revealed severe malformations in the sella turcica region in spina bifida and minor ones in cranial encephalocele. Pharyngeally located adenopituitary gland tissue occurred in all fetuses. Anencephaly and cranial encephalocele seemingly are conditions resulting from different expressivity of the same multifactorial process of maldevelopment involving mesoderm (skeleton), neurectoderm (spinal cord and brain), and surface ectoderm (adenopituitary gland tissue). It is suggested that the molecular biological signaling between the notochord, the scleroderm, and the surface ectoderm is disturbed in spina bifida and cranial encephalocele.

Bone and Bones↗
PubMed1983

Frontonasal encephalocele--"long nose hypertelorism".

Two varieties of frontonasal encephalocele are presented, with variations in the surgical technique necessitated by the different anatomical findings. The encephalocele pushes the nasal skeleton posteriorly and caudally and widens the distance between the medial orbital walls. This causes the long nose and the telecanthus. In this variety of frontonasal encephalocele, hypertelorism is uncommon. There may be absence of dura in relation to the encephalocele. The suggested method of correcting the deformity involves a combined intracranial and extracranial approach. The nasal skeleton is mobilized and placed in its correct position and the telecanthus is corrected. All skull defects are bone grafted. Correcting the deformity at an early age is recommended.

Child↗
PubMed2003

Nasal glioma and encephalocele: diagnosis and management.

OBJECTIVE: To review the biology of nasal glioma and encephalocele and to present an algorithm for preoperative evaluation and surgical management. DESIGN: Retrospective review and analysis. SETTING: Tertiary care medical center: 1970 to 2002. PATIENT: Sixteen patients with glioma (n = 10) and encephalocele (n = 6). OUTCOME: Age at the time of presentation, sex, signs and symptoms, imaging findings, surgical approach, pathology, complications, rate of recurrence, and follow-up were recorded. RESULTS: Ten patients presented with nasal glioma with a mean age of 9 months. All patients underwent surgical excision. No complication was encountered with a mean follow-up of 3.5 years. Six patients presented with encephaloceles with a mean age of 15.5 months. All patients underwent surgical excision. Complications included cerebrospinal fluid leak (n = 1) and epiphora (n = 1). Follow-up was 1 to 14 years (mean, 4 years). CONCLUSION: Nasal glioma and encephalocele are rare, benign, congenital lesions with a potential for intracranial extension. Evaluation should include a complete rhinologic and neurologic examination. Preoperative imaging with a thin-cut axial and coronal computed tomography scan and/or multiplanar magnetic resonance imaging is essential. Surgical intervention should be performed soon after diagnosis to alleviate the increased risk of meningitis. A frontal craniotomy approach is recommended if intracranial extension is identified based on preoperative evaluation, followed by an extracranial resection. If there is no evidence of intracranial extension, a conservative extracranial approach is recommended.

Algorithms↗
PubMed1983

Basal encephalocele and morning glory syndrome.

Basal encephaloceles are often associated with other midline anomalies such as hypertelorism, broad nasal root, cleft lip, and cleft palate. Optic disc anomalies such as pallor, dysplasia, optic pit, coLoboma, and megalopapilla have been reported to occur in patients with basal encephalocele We report a case of a child with a sphenoethmoidal encephalocele and morning glory syndrome of the optic nerve. The presence of such optic nerve anomalies with facial midline anomalies should alert the clinician to the possible presence of a basal encephalocele.

Encephalocele↗
PubMed2002

Posterior fontanelle giant encephalocele.

A 4-day-old baby was admitted with a large posterior fontanelle encephalocele. The baby was the third child of a consanguineous marriage. Two older siblings, 5 and 3 years old, were normal. The baby had a small head with a circumference of 30 cm only and an encephalocele with a circumference of 37 cm. The baby was active and there was no other neural tube defect or any other congenital anomalies. Noncontrast CT scan of the head with bone window showed a large posteriorly located encephalocele above the occipital bone, containing a small amount of brain tissue and a large volume of CSF. The baby was operated on in the lateral position and the encephalocele was excised. The dural defect was closed directly. The baby had an uneventful postoperative recovery. Twenty months later, the baby was well with no gross neurological deficits.

Cerebellum↗
PubMed2004

Endoscopic management of anterior skull base encephaloceles.

Encephaloceles are relatively rare phenomena produced by the protrusion of brain and dura through an anterior skull base defect. Although they can occur as congenital defects, encephaloceles can also present after trauma. The diagnosis is usually made with nasal endoscopy and imaging studies. This report reviews our recent experience repairing 5 encephaloceles in 4 patients. The diagnostic approach and the technical aspects of surgical management are discussed. Although encephaloceles are a rarity, this diagnosis should be considered as part of the differential diagnosis in evaluating a patient with a unilateral polypoid nasal mass, particularly in the setting of recurrent meningitis or cerebrospinal fluid rhinorrhea.

Adult↗
PubMed1977

Inverse cerebellum and occipital encephalocele. A dorsal fusion defect uniting the Arnold-Chiari and Dandy-Walker spectrum.

The malformation of inverse cerebellum and occipital encephalocele is situated morphologically between the Arnold-Chiari and Dandy-Walker malformations. In the three reported cases, hydrocephalus was not present, concomitant malformations of the lamina terminalis were present in two, and polymicrogyria was found in all three. The primary defect in the malformation is a complex occipital encephalocele composed of miniature hemispheres connected to the brain stem by an extension of the midbrain tectum. The cerebellar folia extend ventrally and cover the basilar artery. We propose that the encephalocele arises through the processes of overgrowth and dysraphism and thus falls into the organogenetic malformations of Yakovlev. Supporting this theory is the consistent observation of the duplicate hemispheres in the encephalocele and the hydromyelia in the examined spinal cords.

Arnold-Chiari Malformation↗