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Cases reported "Encephalocele"

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161/632. Reversible transinsular herniation of the lateral ventricle.

    We present a 7-year-old boy, with a history of neonatal intraventricular hemorrhage, leading to hydrocephalus with multiple shunt revisions. The current presentation of shunt failure and resultant hydrocephalus was complicated by herniation of the trigone of the lateral ventricle into the posterior fossa. Despite the dramatic radiological appearance, this herniation of the lateral ventricle was not accompanied by any additional clinical signs or symptoms other than those usually attributed to hydrocephalus. Following successful shunt revision, the patient returned to his baseline clinically with the trigone reverting back to its normal position. We also present a second companion case. (+info)

162/632. Large anterior skull base lesions in patients presenting with obstructive sleep apnea. Report of two cases and review of the literature.

    patients with sleep apnea often are treated by sleep disorder specialists and are studied in a sleep laboratory. The authors present two such patients who ultimately were found to harbor large benign anterior skull base lesions that caused their obstructive sleep apnea (OSA). The first patient had a massive pituitary tumor and had undergone a tracheostomy before the lesion was diagnosed. The second patient had a large frontoethmoidal encephalocele that was diagnosed at the same time as a recommendation for continuous positive airway pressure therapy was being considered. Such therapy in the presence of an encephalocele can be dangerous and even fatal. Although there are case reports of tumors causing OSA, nearly all of these lesions have been large pharyngeal lipomas (some of which were palpable in the neck during physical examination) or growth hormone-secreting pituitary adenomas. The patients reported here were completely unaware of the presence of these large lesions until imaging studies and/or nasal endoscopy were performed. These cases illustrate the need to perform nasopharyngeal endoscopy and also to obtain magnetic resonance images of the head before prescribing therapy for OSA. Neurosurgeons must be aware that large skull base lesions sometimes present only with OSA. (+info)

163/632. Meckel Gruber syndrome: occurrence in non-consanguineous marriages.

    Meckel Gruber syndrome is an uncommon, lethal, autosomal recessive disorder, associated consistently with polycystic kidneys, posterior encephalocoele and polydactly. We report three cases in non-consanguineous marriages, suggesting that the single gene defect occurs more commonly in non-consanguineous marriages than mutant genes associated with other autosomal recessive disorders that are usually related with consanguineous marriages. The usefulness of prenatal diagnosis is discussed. (+info)

164/632. Anterior encephalocele with subcutaneous right facial nodule.

    Encephaloceles consist of heterotopic brain tissue that remains connected to the central nervous system. As such, these lesions can occur anywhere along the midline of the head, neck, and back. The clinical findings associated with an encephalocele are often cutaneous, prompting consultation with a dermatologist. Although abnormalities of the skin overlying the spinal cord are readily recognized by our specialty as markers for dysraphism, head and neck lesions may present a diagnostic challenge. We describe a case of an anterior encephalocele to increase awareness of this disorder and to emphasize the clinical findings that will assist with diagnosis. Our case is of particular interest because of the parasagittal location of the facial nodules and minimal actual midline involvement. (+info)

165/632. Neurocutaneous melanosis associated with Dandy-Walker malformation and a meningohydroencephalocele. Case report.

    Neurocutaneous melanosis and Dandy-Walker malformation are both forms of rare congenital neurodysplasia. Interestingly, 8 to 10% of patients with neurocutaneous melanosis also harbor an associated Dandy-Walker malformation, indicating that these developmental abnormalities share a common origin. The authors describe a case of neurocutaneous melanosis associated with Dandy-Walker malformation and an occipital meningohydroencephalocele with a giant melanotic nevus. Multiple congenital liver masses were also observed in the infant. The occipital nevus was totally excised, and ventriculoperitoneal and cyst-peritoneal shunts were created to prevent subsequent hydrocephalus. Findings in this case support the possibility that excessive melanocytes hinder normal mesenchymal development, causing Dandy-Walker malformation and an occipital meningocele. (+info)

166/632. First-trimester diagnosis of hydrolethalus syndrome in a Chinese family.

    We report a case resembling hydrolethalus syndrome in a Chinese family. Fetal polydactyly, syndactyly, encephalocele and cardiac malformation were detected on ultrasound examination at 12 weeks' gestation. Termination of pregnancy was performed, and postmortem examination confirmed the findings. This is the first report of a first-trimester prenatal diagnosis of hydrolethalus syndrome in the Chinese population. (+info)

167/632. 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. (+info)

168/632. Serial S100B levels before, during and after cerebral herniation.

    Protein S100B has been shown to increase in serum and cerebrospinal fluid (CSF) in various neurological diseases. However, the levels of S100B in conjunction with cerebral herniation have not been studied and the significance of extracerebral S100B has become an important issue. We report on a multi-trauma patient in whom cerebral herniation occurred 2 days after admission. Following this, organ-harvesting procedures were performed for transplantation. We measured serial serum S100B during both the ongoing herniation and the following extracerebral surgery. We found that S100B levels seemed to peak immediately prior to cerebral herniation and then decreased shortly thereafter and concluded that the source of the measured serum S100B in this patient was of predominately cerebral origin. In conjunction with the organ harvesting procedure S100B levels increased, indicating that extracerebral sources of the protein also exist. (+info)

169/632. 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. (+info)

170/632. 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. (+info)
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Last update: April 2009
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