SEARCH · Search PubMed
Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.
Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.
A case is reported of a right lateral frontal encephalocele without communication with the midline structures and in concurrence with dysplasia of the orbit, eyeball, and eyelid.
Computed tomographic and plain X-ray observations in a patient with corpus callosum lipoma associated with frontal encephalocele are reported. The rarity of the lesion and the specific diagnostic criteria on CT are emphasised.
The brain from an infant with a cystic occipital mass present at birth is examined in serial section. The occipital mass proved to be a rhombic roof ventriculocele. Within the posterior fossa, it was bound to an occipital lobe encephalocele which issued as a diverticulum of the left lateral ventricle through a microgyric cortical defect in the territory of the left posterior cerebral artery. The posterior medial aspects of both cerebral hemispheres were herniated downward into the widened tentorial gap. Craniolacunae were prominent on the inner aspect of the skull. The aqueduct and central canal of the spinal cord were widely dilated, although the lateral ventricles were collapsed. It is suggested that hydrocephalus secondary to obstruction to flow of CSF through the rhombic roof entrained a sequence of events giving rise to the rhombic roof ventriculocele and causing occlusion of the posterior cerebral artery and subsequent diverticulation of the lateral ventricle through an infarcted region of the posterior-medial hemisphere.
We report here on congenital sphenoorbital encephalocele which could not be disclosed by computed tomography including contrast medium application or by orbital sonography. Surgery and histological examination were necessary to establish correct the diagnosis.
Report on the case of a female infant born with an occipital encephalocele and colobomas of the optic disc, chorioid, and retina. These clinical signs together with respiratory disturbances suggest a Joubert syndrome and a Pfeiffer syndrome. The differential diagnosis of these syndromes is discussed. An analysis of the clinical data indicates a genetic risk of 25% for further siblings.
A 31 year old Chinese man was found to have a large basal encephalocele extending into the nasopharynx associated with a cleft palate and chiasmal damage producing visual field defects. He was found to have marked hypothalamic-pituitary dysfunction including deficiencies of growth hormone, thyrotropin, adrenocorticotropic hormone and gonadotropins, diabetes insipidus, adipsia with essential hypernatremia and poikilothermia. This man illustrates the importance of careful assessment of patients with craniofacial anomalies and outlines a simple investigational protocol.
OBJECTIVE: Teratoma is the most frequently encountered intracranial tumor at birth and constitutes 18-20% of all germ cell tumors. They are usually located in pineal and suprasellar regions. The authors aim to report an extremely unusual presentation, location, and appearance of a teratoma in a newborn. CASE REPORT: A soft tissue swelling in the vertex was detected in a 1-month-old girl. Neurological examination was normal. A big, cystic-solid lesion beginning from pineal region and extending to the scalp was detected in magnetic resonance imaging. It is interesting to note that cerebral venous angiography showed that the superior sagittal sinus (SSS) was divided into three branches at the level of the lesion and they joined together distally. The tumor was excised totally. Histopathological examination revealed the diagnosis of a mature cystic teratoma. CONCLUSION: A mature cystic teratoma mimicking parietal encephalocele is extremely rare. Germ cell tumors should be kept in mind in the differential diagnosis of all midline lesions with unusual radiographic appearance. Cerebral venous angiography or MR angiography must be performed for the diagnosis and the surgical planning in lesions located near SSS.
Osteopetrosis is a condition in which there is a defect in bone resorption by osteoclasts. With thickening of the skull and skull base, the cranial capacity becomes compromised and skull foramina gradually occlude, resulting in a wide range of neurological symptoms and signs. We present a case of autosomal dominant osteopetrosis with temporal lobe epilepsy and nasal obstruction due to acquired bifrontal encephaloceles associated with a decreased intracranial capacity. Neurosurgical reconstruction of the frontal skull base alleviated the symptoms of epilepsy and nasal obstruction.
We conducted a ten-year follow-up of an unusual pedigree with an autosomal recessive vitreoretinal degeneration, severe myopia, and congenital encephalocele. All five affected members (four girls and one boy) also had early, recurrent bilateral detachments. Color vision testing disclosed an acquired tritan dyschromatopsia and electroretinography showed subnormal photopic and scotopic amplitudes, delayed b-wave implicit times and 30-Hz flicker-phase relations, and absent scotopic b-wave oscillations.
Two cases of occipital encephalocele diagnosed late in pregnancy are presented. Early diagnosis of this problem is possible. Parents might elect to terminate pregnancy as the outcome is invariably dismal.
A 13-year-old girl was found to have a "morning glory" optic disc anomaly associated with remnants of the primitive hyaloid vasculature, midline cleft lip and palate, agenesis of the corpus callosum, and a sphenoidal encephalocele. The association of these developmental anomalies indicates that the "morning glory" optic disc anomaly may occur as part of a more extensive syndrome of midline cranioencephalic dysraphism.
We present the case of a patient seen in the Emergency Department (ED) at the height of enteroviral meningitis season with the chief complaint of the worst headache of his life. He was subsequently found to have pneumococcal meningitis as the result of an encephalocele located within the left ethmoid sinus. The key features of the patient's past medical history, the steps to diagnosis, and a discussion of this exceedingly rare entity are detailed.
Congenital encephaloceles are rare lesions which are usually seen in the occipital region, in the West. They may rarely be seen in the frontal region and they have distinct diagnostic features, together with several other pathological conditions occurring in this region. In order to emphasize these points, a retrospective analysis of 35 cases which have been operated on in our clinic, is made in this study. The findings are compared with the data obtained from the literature.
The anterior skull base of a patient with a craniofacial cleft with naso-ethmoidal encephalocele was reconstructed using temporo-parietal and frontal galea-pericranial flaps and free cranial bone grafts, to prevent meningitis caused by trans-nasal infection. The patient had frequent episodes of meningitis before surgery, however, no episodes of meningitis have been observed up to 1 year postoperatively.
A case of congenital encephalocele of the middle ear presenting as a conductive hearing loss is reported. In retrospect it is possible that CT scanning with metrizimide contrast studies may have been helpful in establishing the diagnosis (Cooper and Kassel, 1982). However, it is stressed that even in the absence of radiological abnormality of the temporal bone, surgical exploration of the middle cranial fossa should be undertaken.
An infant of a diabetic mother lived 13 days after birth. She had a small dysplastic 12.6 gm. brain, partly forming a frontal encephalocele. The uncleaved forebrain contained a mass of poorly organized heterotopic cerebellar cortex. The cerebellum itself had normal lamination, but was small and continuous with the dysplastic tissue. The ventricular system was absent except for a few midline ependymal rosettes, and the cerebral cortex was not developed. The cerebellar dysplasia resembled a proliferative and invasive lesion by its rostral extension.
Brain in the middle ear or nasal cavity: heterotopia or encephalocele? The answer to this question is greatly influenced by clinical information. Sometimes, however, this information is insufficient and the pathologist's opinion may influence patient management. It seems that most pathologists tend to get the answer wrong. This article will enable you to arrive at the correct answer.
INTRODUCTION: Transnasal endoscopic repair of cerebrospinal fluid (CSF) fistulas is recommended for patients with CSF leaks who do not respond to conservative treatment. It is a safer and more successful alternative to transcranial surgery. PATIENTS AND METHODS: We present our experience on using transnasal endoscopy for the repair of anterior skull base cerebrospinal fluid fistulas. Between 1999 and 2003 we observed 20 patients with CSF rhinorrhea. The etiology was heterogeneous: post-traumatic in 6 cases, iatrogenic in 6 cases (one interesting case of meningioma of the sphenoethmoid plate), dysembryogenetic - due to encephaloceles - in 4 patients (one with Cruzon syndrome and one with Down syndrome) and idiopathic in the other 4 patients. Use of a rigid transnasal endoscope allowed the localization and repair of all fistulas, with the use of fluorescein in 6 cases. Different grafts were used, in particular fat, bone or chondral septum with mucoperiosteum or perichondral mucosa. Generally the graft was inserted with the underlay or the sandwich technique. Lumbar drainage was used in the postoperative period only in 6 cases. No antibiotic prophylactic therapy was used. RESULTS: Endoscopy was successful in 90 % of patients at the first attempt, and in 95 % of patients at the second approach. We had two late complications such as infections. In one child with a post-traumatic fistula and shunt for hydrocephalus, we observed meningitis 2 years after the first endoscopic surgery and he underwent both transnasal endoscopic surgery and transcranial surgery. The second patient was a woman with a spontaneous fistula, who had rhinoliquorrhea three years after the first surgical treatment. During surgery a strange similar purulent material filling the submucous space of the ethmoid roof was found, suggestive for an intranasal abscess that was removed. We did not see any complications such as hematomas or seizures. The follow-up (range: 6 months to 3 years) made both with MRI and rhinoscopy has not shown any relapse until now in 19 of 20 patients treated only with endoscopy. CONCLUSIONS: The endoscopic approach is highly effective and safe in the treatment of CSF fistulas, with great visualization and minimal invasiveness, for which it is associated to a very low morbidity. The fluorescein technique is extremely helpful for the diagnosis and surgery of CSF leaks.