1/10. Atretic cephalocele--report of two cases with special reference to embryology.We report two cases of atretic cephalocele, a diverse form of cranium bifidum. The patients were 15-year-old and 3-month-old girls, who each had a hard, nonpulsatile, nonreducible lump covered by alopecic scalp in the parieto-occipital area. They were surgically treated. In case 2, microscopical examination of the operative specimen revealed a meninges under the mass, which was devoid of nervous tissue. Such lesions have rarely been reported, and their essential nature is still the subject of controversy. Pathological and embryological aspects of atretic cephalocele are discussed on the basis of the findings; the neural crest remnant was assumed to be the developmental origin of the lesion in each of these cases.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
2/10. Is there an upper limit of intracranial pressure in patients with severe head injury if cerebral perfusion pressure is maintained?Authors of recent studies have championed the importance of maintaining cerebral perfusion pressure (CPP) to prevent secondary brain injury following traumatic head injury. Data from these studies have provided little information regarding outcome following severe head injury in patients with an intracranial pressure (ICP) greater than 40 mm Hg, however, in July 1997 the authors instituted a protocol for the management of severe head injury in patients with a glasgow coma scale score lower than 9. The protocol was focused on resuscitation from acidosis, maintenance of a CPP greater than 60 mm Hg through whatever means necessary as well as elevation of the head of the bed, mannitol infusion, and ventriculostomy with cerebrospinal fluid drainage for control of ICP. Since the institution of this protocol, nine patients had a sustained ICP greater than 40 mm Hg for 2 or more hours, and five of these had an ICP greater than 75 mm Hg on insertion of the ICP monitor and later experienced herniation and expired within 24 hours. Because of the severe nature of the injuries demonstrated on computerized tomography scans and their physical examinations, these patients were not aggressively treated under this protocol. The authors vigorously attempted to maintain a CPP greater than 60 mm Hg with intensive fluid resuscitation and the administration of pressor agents in the four remaining patients who had developed an ICP higher than 40 mm Hg after placement of the ICP monitor. Two patients had an episodic ICP greater than 40 mm Hg for more than 36 hours, the third patient had an episodic ICP greater than of 50 mm Hg for more than 36 hours, and the fourth patient had an episodic ICP greater than 50 mm Hg for more than 48 hours. On discharge, all four patients were able to perform normal activities of daily living with minimal assistance and experience ongoing improvement. Data from this preliminary study indicate that intense, aggressive management of CPP can lead to good neurological outcomes despite extremely high ICP. Aggressive CPP therapy should be performed and maintained even though apparently lethal ICP levels may be present. Further study is needed to support these encouraging results.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
3/10. Encephalocoele involving the petrous bone.The rare occurrence of an encephalocoele through a defect in the tegmen of the petrous bone is reported. The case illustrates the association of this abnormality with recurrent meningitis and the importance of imaging in defining the nature and site of the lesion prior to surgery.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
4/10. Craniofacial approach for the neonatal management of frontonasal encephalocoeles.Three nasofrontal encephalocoeles managed at the University of South alabama Medical Center are presented. Each consisted of abnormal brain originating from one frontal lobe, although the amount of cerebral tissue and the accompanying skin and meningeal layers varied. The encephalocoeles protruded through a defect in the anterior skull base near the cribiform plate. Repair in the neonatal period was required in two of the infants because of the size of the lesion and obstruction of the nasal airway. The operative approach utilized a bifrontal craniotomy with resection of the encephalocoele intradurally, repair of the anterior cranial fossa dura and osteoplastic repair of the foramen cecum defect. The closure of the facial defect depended upon the nature of the skin covering the herniation; either absence or excess of skin occurred. The preoperative evaluation disclosed associated congenital deformities in 2 of 3 patients in this series. Computerized tomographic scanning was of importance in preoperative planning. The operative technique can be modified to allow for each child's unique anatomy. Repair of nasofrontal encephalocoeles in the neonatal period may simplify the required operative procedures.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
5/10. Sphenoethmoidal encephalomeningocele and midline anomalies of face and brain.An autopsy case of sphenoethmoidal encephalomeningocele associated with anophthalmia, agenesis of the corpus callosum, cleft palate and nasal septum defect is presented. A small colloid cyst and a cyst of unknown nature were found in the third ventricle. Based on a review of the literature, a peculiar association of sphenoethmoidal encephalomeningocele with callosal defect and midline facial anomalies seems not to be fortuitous and we proposed to call it "Sakoda complex" as a distinctive disease entity. Pathogenetic mechanism and significance of separation of this syndrome are discussed from a embryological standpoint. Anophthalmia and the cyst of unknown nature are interpreted as midline anomalies that may be attributed to the same pathogenetic cause.- - - - - - - - - - ranking = 2keywords = nature (Clic here for more details about this article) |
6/10. The pathology of occipital encephalocoele and a discussion of the pathogenesis.The midline structures of 5 infant brains with occipital encephalocoeles were studied. Though all cases showed a uniform finding of a midline defect of the occipital bone with a herniated mass, the extent of the bony defect, the amount and nature of the herniated tissues and the degree of distortion of the intracranial structures varied markedly. Two of the cases showed multiple associated systemic malformations and another case had an inverse cerebellum. In spite of such a marked variability in presentation, the common findings of herniation and displacement of the mesencephalon and roof of the diencephalon allowed us to postulate that the primary defect of occipital encephalocoeles is an abnormality of the tissues overlying the mesencephalon of the developing brain. Initial herniation of the mesencephalon and subsequent movement of the rest of the brain as a result of growth determines the tissues present in the hernia sac at birth. Other theories of genesis of occipital encephalocoeles are discussed.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
7/10. Nasal masses in infants and children.Nasal or perinasal masses in children may be classified as congenital or developmental masses, inflammatory lesions, or benign or malignant neoplasms. Because of a possible connection with the central nervous system, nasal masses may be difficult to manage. A premature biopsy, or even a noninvasive palpation, may precipitate intracranial infection, meningitis, or severe bleeding. The indolent nature and benign appearance of these lesions shold not lead to deferral of complete evaluation and appropriate treatment.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
8/10. Non-traumatic cerebrospinal fluid rhinorrhea.Non-traumatic cerebrospinal fluid rhinorrhea is uncommon. review of the literature revealed that 45% of cases are high pressure leaks and 55% of cases are normal pressure leaks. Meningoencephaloceles are very rare occurrences in the latter category and we present the third reported case occurring in the frontal sinus. Radiographically this lesion appeared as a mucocele because of its expansile nature, however non-traumatic mucoceles have not been associated with cerebrospinal fluid rhinorrhea. The only non-osseous expansile lesion of the paranasal sinuses found to be associated with this complication is the meningioencephalocele.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
9/10. Fatal intracranial hemorrhage following pediatric oral surgical procedure.Deaths during dental and oral surgical procedures may lead to litigation alleging malpractice. For this reason, and because of their sudden and unexpected nature, they often come to the attention of forensic pathologists. We review the clinical and anatomic findings of a 3-year-old boy who expired following an oral surgical procedure in the temporomandibular region. During the operation, perforation of the base of the skull occurred causing laceration of a branch of the middle meningeal artery and fatal subdural and epidural bleeding.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
10/10. Atretic encephalocele/myelocele--case reports with emphasis on pathogenesis.Atretic encephaloceles or myelomeningoceles are frequently solid due to hamartomatous proliferation of fibrous tissue and blood vessels. Because of the fibrous nature of the tumor with no cystic cavity and unusual location with no connection to CNS, they are frequently regarded as insignificant hamartomas. Apart from this terminology, they are also described as cutaneous meningiomas or hamartomas with ectopic meningothelial elements by the presence of meningothelial cells. We report a case of atretic encephalocele in the parietal scalp of an 8 year-old boy and a case of myelomeningocele in the posterior mediastinum of a 31 year-old woman. The terms atretic encephalocele and myelomeningocele are more appropriate for these cases because they include their pathogenesis and the non-neoplastic nature of the lesion.- - - - - - - - - - ranking = 2keywords = nature (Clic here for more details about this article) |
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