11/38. Thoracic meningocele, meningomyelocele or myelocystocele? Diagnostic difficulties, consequent implications and treatment.spina bifida cystica is a closing disorder of the neural tube which infrequently occurs in the thoracic region. A rare lesion called myelocystocele is a variant of spina bifida cystica and is associated with syringomyelia, Chiari type 2 malformation and hydrocephalus. Usually the patient has no neurological deficit, but future deterioration can occur due to posterior tethering of the spinal cord by adhesions. The prenatal diagnosis by ultrasound study can be misleading and in order to attain the correct diagnosis, especially if abortion is considered, a prenatal MRI scan should be done before the parents are counselled, and should be repeated prior to operative treatment. Surgical correction of myelocystocele is not only for cosmetic reasons, but also to untether the spinal cord prophylactically to prevent future neurological deterioration. In this case report, we present a child born with a thoracic myelocystocele, the diagnostic difficulties, consequent implications and surgical treatment.- - - - - - - - - - ranking = 1keywords = meningocele (Clic here for more details about this article) |
12/38. Anterior cervicothoracic meningocele treated by cystopleural shunt. Case report and review of the literature.Anterior meningocele is a rare disease which is commonly observed in the sacral region. Anterio cervicothoracic meningocele is generally diagnosed in adult age and there are few reports in infancy. Different treatment procedures are performed such as ligation, resection and wrapping. The case of a 9-month-old male infant with acute respiratory distress and cyanosis is described. He had also recurrent lung infections for 3 months. The magnetic resonance imaging revealed an anterior cervicothoracic meningocele which was compressing the lung, aorta, esophagus and trachea associated with skeletal abnormalities and C2 - C7 syringohydromyelia. There was no evidence of neurofibromatosis. He was submitted to thoracotomy and treated with cystopleural shunt without valve system. Postoperatively his respiratory distress improved dramatically. Radiologically, the size of meningocele decreased and the syringomyelic cavity disappeared. There was no evidence of recurrence during his followup. Cystopleural shunt procedure is a reliable, simple and effective treatment modality in anterior cervicothoracic meningocele cases. This treatment regimen prevents possible neural damage and also treatments to the associated anomalies such as syringohydromyelia.- - - - - - - - - - ranking = 2.25keywords = meningocele (Clic here for more details about this article) |
13/38. Neurolisteriosis causing hydrocephalus, trapped fourth ventricle, hindbrain herniation and syringomyelia.central nervous system infection by listeria monocytogenes is relatively uncommon, but is known to be highly morbid and fatal. We describe a case of listeria meningoencephalitis, wherein the acute episode was followed by a hitherto unreported conglomeration of severe and progressive neurological sequelae, in the form of supratentorial hydrocephalus, aqueduct block, trapped fourth ventricle, hindbrain herniation and syringomyelia. Pertinent literature is reviewed and the pathogenesis of the observed sequelae is explored.- - - - - - - - - - ranking = 2.3952093362903keywords = herniation (Clic here for more details about this article) |
14/38. Atlanto-occipital dislocation with traumatic pseudomeningocele formation and post-traumatic syringomyelia.STUDY DESIGN: A case report of traumatic atlanto-occipital dislocation complicated by the development of anterior and posterolateral pseudomeningoceles and the late development of syringohydromyelia is presented. OBJECTIVE: To describe a unique post-traumatic and postsurgical course following atlanto-occipital dislocation. SUMMARY OF BACKGROUND DATA: syringomyelia is a significant potential long-term complication in patients recovering from traumatic atlanto-occipital dislocation. Cord enlargement and increased T2 signal can be a marker of abnormal cerebrospinal fluid flow dynamics. This "presyrinx state" can be seen before clinical evidence of neurologic compromise. Pseudomeningocele formation after atlanto-occipital dislocation is rare, with only 3 reported cases. To our knowledge, all reported cases describe retropharyngeal pseudomeningoceles, and posterolateral pseudomeningocele as seen in this case has not previously been described. methods: A single case is reported with an emphasis on the imaging findings related to the patient's subsequent neurologic deterioration. RESULTS: Following a pedestrian-motor vehicle collision, the patient received initial evaluation and treatment at a local foreign medical facility, where his cervical spine was cleared. Several days following stabilizing treatment and surgery, the patient was transferred to a foreign-based united states military medical facility and ultimately to our institution, where magnetic resonance imaging demonstrated occipitocervical dissociation. The patient was taken to the operating room for surgical stabilization. Four months after his index operation, the patient underwent halo removal. Follow-up magnetic resonance imaging revealed thickening of the cervical spinal cord in conjunction with diffuse high cord T2 signal and a small low cervical segment of syringomyelia. The patient was observed with follow-up magnetic resonance imaging obtained after 1 month. At this time, the low cervical syrinx had enlarged slightly, a small thoracic syrinx was observed, and cine imaging of cerebrospinal fluid flow demonstrated obstruction at the level of the foramen magnum. The patient was taken to the operating room fordecompression of the foramen magnum and posterior fossa and duraplasty. One month later, the patient's clinical condition began to deteriorate, and repeat imaging showed continued enlargement of the patient's syrinx and hydrocephalus. He was admitted for an urgent shunt procedure but unfortunately sustained cardiorespiratory arrest while on the ward awaiting surgery. CONCLUSIONS: Atlanto-occipital dislocation is rarely survivable, and delayed diagnosis can negatively affect long-term clinical outcome. This case illustrates how, despite early signs of improvement, post-traumatic syringomyelia may occur months or even years after spinal trauma and should always be considered in patients who experience late neurologic deterioration after atlanto-occipital dislocation.- - - - - - - - - - ranking = 2keywords = meningocele (Clic here for more details about this article) |
15/38. Significant scoliosis regression following syringomyelia decompression: case report.We present the case of a 5-year-old boy presenting with a 54-degree scoliosis secondary to a Chiari I malformation with a holocord syringomyelia extending from C1 to T10. Neurosurgical treatment involved posterior fossa craniectomy with decompression, and partial C1 laminectomy. At follow-up 7 years later, at age 12, radiographs revealed only a 4-degree scoliosis, and follow-up MRI revealed a deflated syrinx. We report this case to reveal the most significant scoliosis regression seen in our experience that may occur in younger patients after neurosurgical syringomyelia decompression for Chiari I hindbrain herniation.- - - - - - - - - - ranking = 0.47904186725806keywords = herniation (Clic here for more details about this article) |
16/38. Hindbrain-hernia-related syringomyelia without syringobulbia, complicated by permanent nocturnal central hypoventilation requiring non-invasive ventilation.INTRODUCTION: In the absence of syringobulbia, hindbrain hernia does not commonly cause permanent respiratory complications. We present two patients who developed permanent central nocturnal hypoventilation following acute deterioration of hindbrain-hernia-related syringomyelia despite successful surgery. patients: Two children (one boy and one girl, aged 7 and 13 years, respectively) presented with acute neurological deterioration. The boy presented with a 6-week history of progressive tiredness and weakness and a short history of respiratory depression. The girl presented with a 2-year history of left hemiparesis and a short history of left hemiplegia and respiratory depression. On magnetic resonance scan, both had hindbrain hernia, hydrocephalus and cervical syringomyelia. Following cranio-vertebral decompression and, later, a ventriculoperitoneal shunt, the syringomyelia remained well controlled in both patients. In contrast, their respiratory depression improved minimally. Repeated multichannel respiratory monitoring revealed a persistently slow sleeping respiratory rate, with nocturnal hypercapnia and hypoxemia, managed successfully by night-time non-invasive ventilation. Neither the boy nor the girl, after 7 and 4 years, respectively, has developed any complications of nocturnal hypoventilation. When awake, both breathe normally. RESULT: Both children appear to have suffered a permanent impairment of respiratory control, resulting in nocturnal hypoventilation. This is usually seen with brain stem lesions and is unexpected when syringomyelia does not extend above C2, in the absence of syringobulbia. DISCUSSION: Central nocturnal hypoventilation is postulated to be due to permanent damage of central brain stem pathways controlling the respiratory muscles, due to local pressure at the cranio-cervical junction associated with acute deterioration of the hindbrain herniation. CONCLUSION: Sudden deterioration of hindbrain hernia and cervical syringomyelia can rarely impair the central mechanisms of respiratory control, with long-term implications on quality of life.- - - - - - - - - - ranking = 0.47904186725806keywords = herniation (Clic here for more details about this article) |
17/38. Syringobulbia in a pediatric population.OBJECTIVE: To better understand the presentation, management, and outcome of syringobulbia in the pediatric age group. methods: The University of iowa pediatric neurosurgery database was searched for patients under the age of 18 with a diagnosis of syringobulbia. The patients' records were retrospectively reviewed for demographic data, chief complaint and presenting symptoms, neurological and radiographic findings, treatment, outcome, and complications. Children with open neural tube defects and Chiari II malformations were excluded. RESULTS: Six pediatric patients were identified as meeting inclusion criteria. The average age at time of surgery was 14.8 years. The chief complaints were vision impairment in three children and numbness, gait instability, and headache worsened with Valsalva in one patient each. Other prominent symptoms included sleep apnea and weakness. All patients showed at least one cranial nerve dysfunction. Radiographs revealed hindbrain herniation and associated syringomyelia in all cases. Two patients had scoliosis. Treatment was posterior fossa decompression with cerebellar tonsillar shrinkage, opening of foramen of Magendie, and duraplasty. Two patients also required concomitant ventral decompression. The cavity of syringobulbia communicated with syringomyelia and the fourth ventricle in most children but was distinct from the fourth ventricle. Two patients received fourth ventricle to subarachnoid shunts. Follow-up averaged 3.2 years, and all patients clinically improved after surgery. magnetic resonance imaging documented resolution of syringobulbia in all cases, with syringomyelia improving in all cases. There was no permanent morbidity or mortality in the series. CONCLUSION: Syringobulbia is strongly associated with Chiari malformation and syringomyelia, and patients often present because of cranial nerve palsies. Posterior fossa decompression is a safe and effective treatment.- - - - - - - - - - ranking = 0.47904186725806keywords = herniation (Clic here for more details about this article) |
18/38. Arachnoid cyst resulting in tonsillar herniation and syringomyelia in a patient with achondroplasia. Case report.achondroplasia has been associated with varying degrees of cervicomedullary and spinal compression, although usually in the pediatric population. Large arachnoid cysts have also been found to result in tonsillar herniation and syringomyelia. The authors present the case of a patient with achondroplasia who presented with symptoms of foramen magnum compression and syringomyelia, and who was subsequently found to have a large posterior fossa arachnoid cyst. This 38-year-old woman with achondroplasia presented with an 8-month history of headache and numbness of the hands and fingers. Admission magnetic resonance (MR) imaging of the head and spine revealed a large arachnoid cyst in the posterior cranial fossa, a 6-mm tonsillar herniation consistent with an acquired Chiari malformation, and a large cervicothoracic syrinx. The patient was treated using suboccipital craniectomy, C-1 laminectomy, fenestration of the arachnoid cyst, and decompression of the acquired Chiari malformation with duraplasty. Surgical decompression resulted in improvement of the presenting symptoms, adequate decompression of crowding at the foramen magnum, and resolution of the syrinx. Although there was only partial reduction in the retrocerebellar cisternal space on follow-up MR imaging, no residual symptoms were related to this.- - - - - - - - - - ranking = 2.8742512035484keywords = herniation (Clic here for more details about this article) |
19/38. Fibrous dysplasia of the cranial bones: a case report and review of the literature.Fibrous dysplasia (FD) is a relatively uncommon disorder that affects primarily the cranial region; its occurrence in the cranial base in combination with hindbrain herniation and aneurysmal bone cyst (ABC) constitutes an extremely rare condition. We report a case of polyostotic fibrous dysplasia with progressive occipital, temporal, and clival involvement. Clinical findings and differential diagnosis with special emphasis on the imaging features were discussed. A small posterior fossa volume has been thought to lead to hind brain herniation. The resultant obstruction to the CSF pathways at the level of the foramen magnum has been implicated in the development and subsequent progression of syringobulbia.- - - - - - - - - - ranking = 0.95808373451613keywords = herniation (Clic here for more details about this article) |
20/38. Symptomatic syringomyelia secondary to clinically obscure infratentorial tumour.The formation of a cervical spinal cord syrinx as a result of an infratentorial mass, even though uncommon, has been reported in international literature. In such cases, syringomyelia is usually asymptomatic, while the tumour-related symptoms and signs predominate. We report a patient with a posterior fossa tumour and secondary syringomyelia. In this patient, syringomyelia symptoms and signs were present, and a cervical spine magnetic resonance imaging (MRI) showed a large cervical syrinx. A more careful clinical examination though, revealed a sub-clinical posterior fossa syndrome and brain MRI revealed a large infratentorial meningioma. A posterior fossa craniotomy was performed, followed by complete tumour resection and almost complete remission of the syrinx and its related symptoms. The authors discuss the role of posterior fossa tumour induced tonsillar herniation in the development of secondary syringomyelia, the mechanisms leading to syrinx formation and the conditions that must be fulfilled for that to happen.- - - - - - - - - - ranking = 0.47904186725806keywords = herniation (Clic here for more details about this article) |
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