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Clinical follow-up of prenatally diagnosed isolated ventriculomegaly, microcephaly and encephalocele.

This retrospective review identified all cases of isolated ventriculomegaly (without spina bifida), encephalocele and microcephaly in approximately 22,000 consecutive patients through the Ultrasound Department at the University of British Columbia, Grace Hospital. 17 cases of isolated ventriculomegaly, 16 cases of microcephaly and 6 cases of encephalocele were identified. Isolated ventriculomegaly and encephalocele were accurately diagnosed prenatally while microcephaly was not consistently identified prenatally. Patients with isolated ventriculomegaly had a positive family history for cranial abnormalities in 24%. The prognosis is difficult to predict for fetuses with ventriculomegaly, but severe ventriculomegaly generally produced a poor outcome while mild or moderate ventriculomegaly resulted in normal development or marginal developmental delay. Chromosome analysis is recommended in all patients with central nervous system lesions and viral studies are recommended with ventriculomegaly and microcephaly.

Brain↗

Intrasphenoidal encephalocele associated with cerebrospinal fluid fistula and subdural hematomas: technical case report.

OBJECTIVE AND IMPORTANCE: Intrasphenoidal encephalocele is a rare clinical entity that is often complicated by rhinorrhea, recurrent meningitis, and headache, but in no case has the association of rhinorrhea with subdural hematomas been described. A surgical procedure to stop persistent cerebrospinal fluid leakage is reported. CLINICAL PRESENTATION: A 59-year-old man sought care for intractable rhinoliquorrhea of 6 months' duration. Cranial computed tomographic and magnetic resonance imaging scans revealed a basal posterior frontal bony defect and an evocative image suggesting intrasphenoidal encephalocele. INTERVENTION: A transnasal transsphenoidal surgical procedure was performed; the encephalocele was removed, and the sphenoid sinus was filled with an inflatable pouch made of synthetic dura mater containing abdominal fat. Postoperative reduction of the rhinoliquorrhea, but not its total disappearance, was observed. Total disappearance was achieved only after endonasal, transmucosal inflation of the pouch with human fibrin glue. One of the subdural hematomas disappeared spontaneously, and the other was treated by a surgical procedure. CONCLUSION: The possible role of the presented technique in the treatment of cerebrospinal fluid leakage is discussed.

Cerebrospinal Fluid Rhinorrhea↗

Responses to vibroacoustic stimulation in a fetus with an encephalocele compared to responses of normal fetuses.

BACKGROUND: Observation of fetal movement and fetal heart rate (FHR) responses to repeated vibroacoustic stimulation (VAS) might be useful as a measure to assess fetal well-being and to assess the integrity of the fetal central nervous system (CNS). We observed the movement and FHR responses to repeated VAS of a term fetus with a serious brain anomaly as compared to responses of normal term fetuses. SUBJECTS, METHODS, RESULTS: In 37 normal term fetuses and in a term fetus with an encephalocele we studied movement and FHR response to repeated VAS. All normal fetuses responded within 1 s after stimulation with general body movement and FHR acceleration. At 36 gestational weeks, no movement or FHR responses were seen in the fetus with an encephalocele. Repetition of the test in this fetus after one week still showed no response to repeated VAS. CONCLUSION: Normal fetuses showed movement and FHR responses to external stimulation. The fetus with an encephalocele did not respond to repeated VAS with a movement or FHR acceleration. Case studies in fetuses with structural anomalies of the CNS are needed to gain insight into the spectrum of possible responses to VAS.

Acoustic Stimulation↗

Transalar sphenoidal encephalocele and respiratory distress in a neonate: a case report.

We present a full-term newborn infant who suffered from immediate postpartum severe respiratory distress. The infant had an inspiratory stridor as a result of a swelling of the soft palate, extending from the roof of the nasopharynx. Transoral endotracheal intubation resulted in normal saturation levels. Histologic examination after an open biopsy showed mature neuroglial tissue. Radiology demonstrated the presence of a right parapharyngeal process obstructing the nasopharynx and oropharynx and extending to the right middle and posterior fossa, via the foramen ovale. After transoral debulking, the infant was extubated successfully. After an uneventful period of 5 months, the patient was readmitted at our hospital for treatment of meningitis. Subsequently, the inspiratory stridor recurred, and staged surgery was performed. First, a transcranial approach was used to remove a large intradural part of the process and close the defect at Meckel's cave. Two weeks later the retro- and parapharyngeal part of the process were removed transorally. Given the site of the defect of the skull base and the intradural location of the process, the diagnosis is a transalar sphenoidal encephalocele. This is a rare type of basal encephalocele, and has never been reported in an infant nor known to present with respiratory distress. The pathogenesis, clinical presentation, pathology, and therapeutic implications of basal encephaloceles are discussed.

Encephalocele↗

Parietal intradiploic encephalocele. Case report.

An unusual case is presented of an intradiploic encephalocele surrounding a cerebrospinal fluid cyst. The relationship of this developmental anomaly to the more commonly encountered midline encephalocele and isolated reports of temporal bone encephaloceles are discussed.

Encephalocele↗

Primary intranasal encephalocele. Report of four cases.

Four cases of primary intranasal encephalocele are presented. Three of the patients had been treated for nasal polyps. One of these three patients presented with persistent cerebrospinal fluid (CSF) rhinorrhea after fourth a polypectomy, another with recurrent CSF rhinorrhea and bacterial meningitis following a second polypectomy, and the third case with recurrence of meningitis, also following polypectomy. Recurrent bacterial meningitis was the mode of presentation in the fourth case. Encephalocele was the isolated abnormality in three, but the fourth had a degree of associated hypertelorism. The diagnosis of encephalocele should be considered in any patient with a nasal polyp, especially in children and in patients with recurrent bacterial meningitis, with or without rhinorrhea, in the absence of cranial trauma or surgery, or in the absence of external craniospinal anatomical defects.

Adolescent↗

Nasal encephaloceles: definitive one-stage reconstruction.

Nasal encephaloceles can cause complex deformities of the naso-orbital skeleton. As the encephalocele pushes through a defect in the facial skeleton it causes lateral displacement of the medial orbital walls. Correction of this skeletal deformity is necessary to achieve a normal facial contour. Two examples of nasal encephaloceles are presented and the classification, diagnosis, and treatment of this entity are discussed. The correction of these deformities at an early age is recommended. The suggested method of reconstruction is a combined intracranial and extracranial approach with mobilization of the nasal skeleton and medial orbital walls to their normal position. The remaining defects are treated with bone grafts.

Encephalocele↗

Bilateral morning glory syndrome associated with sphenoid encephalocele. Case report.

Morning glory syndrome is a congenital anomaly of the optic disc in which the disc is enlarged and excavated, with white glial tissue in the center. A case is presented of morning glory syndrome associated with sphenoid encephalocele, median cleft lip, and agenesis of the corpus callosum. A 22-day-old boy was referred to the Wakayama Medical College Hospital for management of dyspnea due to a soft-tissue mass in the oral cavity. Magnetic resonance imaging revealed a mass extending through a bone defect in the sphenoid region and into the oral cavity. Surgical repair was attempted through a bifrontal craniotomy. A bone defect was identified in the sphenoid plate, through which the arachnoid membrane was connected to the oral cavity. Both optic nerves were elongated and adhered to the encephalocele. The wall of the meningocele was compressed digitally through the oral cavity and sutured to the dura mater of the bone defect. The operative findings suggest that a basal encephalocele protruding from a bone defect in the sphenoid plate may disturb the normal development of the optic nerve.

Abnormalities, Multiple↗

Spontaneous temporal encephalocele. Case report.

The authors report a 36-year-old woman with a 23-year history of simple and complex partial seizures who was treated surgically for an anteroinferior temporal encephalocele, with resolution of the seizure disorder. This patient's presentation, findings, and response to treatment are typical of those associated with anteroinferior temporal encephalocele, and different from the clinical patterns of four other types of spontaneous temporal encephalocele.

Adolescent↗

Bilateral temporal bone encephaloceles after cranial irradiation. Case report.

Irradiation of the central nervous system may cause significant morbidity, including endocrine dysfunction and intellectual impairment. The authors report a case of bilateral temporal bone encephaloceles in a 21-year-old man who had received prophylactic central nervous system irradiation for acute lymphocytic leukemia in early childhood. Endaural encephaloceles are uncommon, and most occur as a complication of mastoid surgery. The etiology, clinical features, radiological diagnosis, and surgical treatment of temporal bone encephaloceles are discussed.

Adult↗

Encephalocele and associated skull defects.

Encephalocele is a common congenital problem in the practice of Neurosurgery worldwide, with varying sizes of the underlying skull defects. This study was carried out to determine the size of the problem; to assess whether the skull defects are being under-managed or not; and also to determine those patients that will benefit from cranioplasty. The case notes of the patients with encephalocele managed over a 5 year period were reviewed and the relevant data obtained. Seventy-six percent of the patients had occipital encephalocele. The average diameter of the skull defect was 1.8 cm. Only 2 (9.5%) of the patients had cranioplasty. Cosmesis was acceptable to all the patients. No recurrence was noted in the series studied. We therefore concluded that the skull defects are not being under-managed, however large anteriorly based lesions with wide skull defects (i.e >2.5 cm) will require cranioplasty.

Child, Preschool↗

[Nasal encephalocele: differential diagnosis with nasal glioma].

Congenital nose neoplasms are infrequent, between them neurogenic tumors of the middle line include nasal gliomata (glial ectopies) and nasal encephaloceles, according to an existing or lacking communicating link with the intracranial cavity. We report one congenital naso-encephalocele case in a 16-year-old girl suffering from repeated meningitis events after several nasal polypectomies performed in other departments. She underwent complete removal through fronto-neurosurgical and paralateronasal approach, being the young woman asymptomatic for 3 years. We discuss about both intranasal gliomata and encephaloceles, certainly of not easy pathologic identification, stressing the decisive value of imaging techniques, as magnetic resonance, in order to clearcut both the diagnosis and the therapeutical planning.

Adolescent↗

[A case report of sphenoidal encephalocele presenting with cerebral spinal fluid rhinorrhea].

Sphenoidal encephalocele is a rare congenital anomaly. We report a case of sphenoidal encephalocele presenting with cerebral spinal fluid rhinorrhea. A 53-years-old man suffered recurrent fever and rhinorrhea. Computed tomography demonstrated a low density area extending from the left middle cranial fossa into the left sphenoidal sinus. Bone defect was identified in the medial surface of the left middle cranial fossa. Magnetic resonance imaging with contrast medium demonstrated peripheral enhancement of the lesion. We diagnosed the lesion as sphenoidal encephalocele. Surgical repair and amputation of the encaphalocele was performed. Sphenoidal encaphalocele should be considered as a possible diagnosis for recurrent fever and rhinorrhea.

Cerebrospinal Fluid Rhinorrhea↗

[Spontaneous trans-sphenoidal encephalocele presenting with nontraumatic cerebrospinal fluid rhinorrhea (case report)].

Encephaloceles are uncommon and can arise from congenital, traumatic, or spontaneous origins. Approximately 80% of all cerebrospinal fluid rhinorrheas are caused by head injuries. Spontaneous or nontraumatic encephaloceles or cerebrospinal fluid leaks have been the least common in most series, accounting for only 3% to 5% of all cerebrospinal fluid leaks. There is a high incidence of meningitis and brain abscess. Thus, early diagnosis is very important. We present an adult patient with uncomplicated nontraumatic cerebrospinal fluid rhinorrhea that was caused by spontaneous trans-sphenoidal encephalocele.

Cerebrospinal Fluid Rhinorrhea↗

[Nasal encephalocele as a cause of recurrent bacterial meningitis].

Nasal encephaloceles are rare inborn malformations of the central nervous system. We report on a 30 months old boy with recurrent bacterial meningitis where a nasal encephalocele was diagnosed. We describe diagnostic procedures and the development and treatment of this interesting disease. The nasal encephalocele was successfully removed surgically.

Brain↗

Anterior basal encephalocele in the median cleft face syndrome. Comments on nosology and treatment.

Two cases of median cleft syndrome with associated basal encephalocele are presented. The median cleft face syndrome consists of several craniofacial defects and occurs, as far as we know, sporadically. Extracranial defects should be looked for carefully since they may indicate the presence of a specific syndrome with its own prognosis and mode of inheritance. Basal encephaloceles are sometimes part of the syndrome and may cause serious, or even life-threatening, symptoms. With regard to the treatment of basal encephaloceles there still exists a lot of controversion, but most authors agree that it is better to postpone operation until after the age of three because mortality is lower then.

Abnormalities, Multiple↗

Occipital encephalocele and early gestational hyperthermia.

Hyperthermia as a human teratogen has been implicated as one cause for neurulation defects. To determine whether there is an association between early maternal hyperthermia (20 to 28 days' gestation) and isolated occipital encephalocele, record reviews were conducted for the period 1969 through 1979 in three major medical centers in the Pacific Northwest. Control patients consisted of children with Down's syndrome matched for year of birth, sex, and race. Of the 17 patients ascertained with an isolated posterior encephalocele, four (24%) of the mothers gave a history of hyperthermia, due to prolonged fever of at least 1.5 C above normal thermal levels early in gestation. In the control patients and siblings of affected children, no history of maternal hyperthermia was elicited. These data are compatible with the concept that early maternal hyperthermia is one cause in the genesis of isolated occipital encephalocele.

Baths↗

Craniofacial correction of occipital encephalocele.

Occipital encephaloceles are commonly seen birth defects. The incidence is 1 in every 10,000 live births. These defects represent about 15% of the total birth defects reported and treated in the United States. The birth defects encountered today comprise over 50% of all pediatric admissions to general hospitals. Six patients with occipital encephaloceles are treated by a combined craniofacial approach. The corrective measure allows reduction of the herniated encephalocele and correction of the craniofacial deformity in the same operation procedure.

Cerebellum↗