The Belgian Society for Neurosurgery.
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Biomedical subjects
Publications and source records attributed to F Van Calenbergh.
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Patients with relapsed malignant glioma have a poor prognosis. We developed a strategy of vaccination using autologous mature dendritic cells loaded with autologous tumour homogenate. In total, 12 patients with a median age of 36 years (range: 11-78) were treated. All had relapsing malignant glioma. After surgery, vaccines were given at weeks 1 and 3, and later every 4 weeks. A median of 5 (range: 2-7) vaccines was given. There were no serious adverse events except in one patient with gross residual tumour prior to vaccination, who repetitively developed vaccine-related peritumoral oedema. Minor toxicities were recorded in four out of 12 patients. In six patients with postoperative residual tumour, vaccination induced one stable disease during 8 weeks, and one partial response. Two of six patients with complete resection are in CCR for 3 years. Tumour vaccination for patients with relapsed malignant glioma is feasible and likely beneficial for patients with minimal residual tumour burden.
PURPOSE: Schwannomas of the lower cranial nerves are rare tumours. We have surgical experience in six patients. MATERIAL AND METHODS: We have retrospectively reviewed the files of the six patients with jugular foramen schwannomas that were treated in multidisciplinary collaboration in the departments of neurosurgery and ENT. RESULTS: From a clinical point of view, the most important finding was that these tumours usually mimic a vestibular schwannoma. The imaging findings however always allowed the differentiation. The surgical technique included in all cases a suboccipital approach for the intracranial part. When large, the extracranial extension had to be removed through a lateral cervical or transfacial approach. There was no mortality and minor morbidity, with good functional outcome. In subtotally removed tumours, recurrence occurred. CONCLUSION: A combined neurosurgical and ENT approach allows for safe removal of these often large skull base tumours.
BACKGROUND: Non-traumatic acute subdural haematomas enable study of the morbidity and mortality due to the haematoma without the effect of trauma. Whereas it is known that coagulation disorders worsen the outcome of spontaneous intracerebral haematomas, this has not been studied in non-traumatic acute subdural haematomas. METHODS: In a series of 13 non-traumatic acute subdural haematomas admitted to our department between January 1995 and March 2002, we had 9 coagulopathy associated haematomas and 3 haematomas corresponding to the syndrome of 'spontaneous acute subdural haematoma of arterial origin'. Both groups were compared. FINDINGS: Age and gender distribution were comparable. The bleeding source was a cortical artery in 2 of the 2 non-coagulopathy related haematomas operated on, but also in 2 of the 4 coagulopathy associated haematomas that underwent surgery. The average haematoma thickness was higher in the coagulopathy related haematomas. The mean Glasgow Coma Score on admission was 7.7 and the mortality rate was 55.6% in the coagulopathy related group. In the non-coagulopathy related haematomas the mean Glasgow Coma Score was 12.0 and the mortality rate 33.3%. The latter mortality rate corresponds well to that of a historical group of 'spontaneous acute subdural haematomas of arterial origin' collected from the literature. INTERPRETATION: The outcome was worse in the non-traumatic acute subdural haematomas that were associated with a coagulation deficiency. While in all non-traumatic acute subdural haematomas the interval to surgery should be minimized, early recognition and urgent correction of coagulation deficiencies is certainly indicated.
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Desmoplastic infantile ganglioglioma is a rare intracranial tumor of early childhood with a usually excellent prognosis despite malignant features both radiologically and histologically. We present the case of a desmoplastic infantile ganglioglioma with histologically highly anaplastic features and both intracerebral and pial metastases. After partial resection the tumor was rapidly progressive and new metastases appeared. A combination of vincristine and carboplatinum was used according to the Low Grade Glioma Protocol of the International Society of Pediatric Oncology, with a temporary good response. When histologically characterized by highly anaplastic features, it seems the biologic behavior of this tumor remains uncertain. The aggressive behavior and the responsiveness to chemotherapy in this case may challenge the belief in the benign nature of these rare tumors.
The purpose of this article is to review less common presentations of degenerative disc disease on MR imaging. The images of eight patients were retrospectively analyzed. Six of them had transligamentous (or noncontained) disc herniations, the fragments of which were located in the posterior epidural space in three of them. One patient had a transdural disc fragment and one patient had a disc cyst. The cyst was located in the ventrolateral epidural space. On T2-weighted images, the migrated disc fragment returned a higher signal than the disc of origin in 6 of 7 patients. The disc cyst returned a signal similar to that of cerebrospinal fluid. The MR appearances of disc fragments can be puzzling, particularly if they are located in the posterior epidural space. It is important to recognize the abnormalities in order to differentiate them from less common lesions such as hematoma, abscess and neurinoma.
BACKGROUND: Lhermitte-Duclos disease (LDD) is a hamartomatous overgrowth of cerebellar ganglion cells, which replace granular cells and Purkinje cells. In recent years several cases involving the association between LDD and Cowden's syndrome (CS), an autosomal dominant condition characterized by multiple hamartomas and neoplastic lesions in skin and internal organs, have been reported. METHODS: We reviewed the medical records and imaging studies of six patients with LDD who were treated at our institution, and we looked at other possible symptoms of CS. RESULTS: Other clinical findings suggestive of CS were apparent in five patients: These included mucocutaneous lesions, acral keratosis, thyroid adenoma, fibrocystic disease, ovarian cyst, intestinal polyposis, and arteriovenous malformation. Only in the youngest patient, a 5-year-old boy, were no cutaneous or other signs found, despite extensive clinical and ultrasound examination. CONCLUSION: Our observations strengthen the hypothesis that LDD is a neurological manifestation of CS. Patients with LDD should receive a thorough dermatological and systemic screening, because some of the lesions (breast, etc...) can develop into malignant tumors.
Brain tumours in pregnancy are rare. In this case vomiting and headache were the only signs. An assessment of the patient with vomiting in pregnancy to help reach a diagnosis when faced with vomiting in pregnancy is given. Following the diagnosis of a brain tumour during pregnancy, management should be tailored to the individual patient.
The treatment of subdural collections in infants remains controversial. In order to evaluate the treatment guidelines that we developed on the basis of our earlier experience, we have reviewed the results obtained in 31 consecutive infants with symptomatic chronic and subacute subdural collections treated with external drainage. Using our guidelines for removal of the drains, there was only a very low rate of permanent shunting (4/31), with a low complication rate and good clinical results. However, the long period of external drainage, and therefore of hospitalization, might make if general application of these guidelines problematic.
BACKGROUND: The technique of posterior fossa decompression, nowadays preferred by most neurosurgeons as the first surgical option in the treatment of hindbrain associated syringomyelia, was reintroduced in our center in 1989. We present a retrospective analysis of 22 patients with this pathology who underwent the procedure since then. METHODS: In all patients diagnosis was made by MRI of the craniospinal junction. The operation consisted of a suboccipital craniectomy, removal of the posterior arc of C1, laminectomy of C2 or C3 when necessary and a wide duraplasty at the end. The intradural manipulations (opening of the arachnoid membrane, coagulation or resection of the tonsils) were not uniform. Postoperatively short- and long-term clinical outcome and MRI findings were assessed. RESULTS: Sixteen out of 21 patients (76%) experienced an improvement in the early follow-up period. In the late follow-up period 13 out of 19 patients (68%) were improved, whereas five patients (26%) experienced a marked deterioration. There was no unequivocal effect on all symptoms and signs. Postoperative MR images showed a favorable result in 16 out of 20 patients (80%), consisting of syrinx collapse or reduction of the syrinx diameter. CONCLUSIONS: We conclude that decompression of the posterior fossa is a safe procedure with a considerable chance of clinical improvement. Although total syrinx collapse is not as frequently seen as in syrinx shunting procedures, the clinical outcome may be better. Moreover, there seemed to be no unequivocal correlation between clinical outcome and postoperative syrinx size in the present study.
We describe the first observation of a child with a posterior fossa subdural effusion with secondary hydrocephalus and tonsillar herniation. We diagnosed this entity in a 14-month-old girl with no history of trauma or coagulation disorder. The patient presented in our emergency department with opisthotonus and raised intracranial pressure resulting from supratentorial hydrocephalus. An emergency ventriculo-peritoneal shunt was placed, which resolved the symptoms only temporarily. Eventually external drainage of the subdural fluid was performed. The collection gradually disappeared, and both the external subdural shunt and the ventriculo-peritoneal shunt were removed. The patient made a complete neurological recovery. We review the physiopathology and treatment of subdural effusions in general, and propose some guidelines for the management of symptomatic effusions occurring in the posterior fossa in particular.
We retrospectively reviewed 32 patients operated on for lipoma of the conus and lipomyeloschisis, the two main anatomical subtypes of congenital lumbosacral lipomas associated with tethered cord syndrome. Surgery was proposed to patients when they were symptomatic, and in most cases for progressive symptoms. The evolution of the different symptoms was studied separately. In most patients, symptoms improved or stabilized after surgery; in some, however, postoperative worsening, at least of some of the symptoms, was seen. This postoperative worsening became apparent either early or late after the operation, and was not associated with surgical trauma or postoperative complications. We suggest it was caused mainly by the natural course of the disease (especially in the case of the orthopedic deformities), and in some cases by retethering. Our series is not large enough to detect statistical significance for the different symptoms or for the anatomical subgroups. Importantly, according to our analysis by the different symptoms, the operation did not seem to protect the patients from later development of new deficits. This can be interpreted as an argument against prophylactic surgery in asymptomatic patients.
The development of secondary tumours is a rare but well known late effect of radiation therapy of lesions in the central nervous system. Most radiation-induced tumours are of mesenchymal origin, but on rare occasions gliomas can occur. We describe a patient in whom a supratentorial anaplastic astrocytoma developed 15 years after surgery and radiotherapy for a childhood posterior fossa medulloblastoma. A concise review of the pertinent literature is given.
Only nine cases of primary multifocal leptomeningeal gliomatosis (PMLG) have been reported. We describe the first child with malignant features of PMLG who was diagnosed during life. The differential diagnosis based on imaging findings includes infectious and lymphoproliferative disorders.
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Three patients with a spontaneous spinal epidural hematoma are presented, one of which is exceptional because it could be attributed to thrombolysis with alteplase. The other two were correlated with oral anticoagulant therapy. Our second case suggests that Morbus Kahler may be an underlying pathology in spinal extradural hematoma. In the third patient neither an obvious 'locus minoris resistentiae' nor a pathological coagulation test could be held responsible for the spontaneous bleeding. All three patients underwent laminectomy and evacuation of the hematoma. Although two of our cases are examples of complete recuperation after late operative intervention, early surgery is preferable. Permanent clinical attention together with a low threshold to perform magnetic resonance imaging (MRI) leads to a quicker diagnosis of spinal epidural hematoma. We show that administration of gadolinium may facilitate correct preoperative localization of the hematoma because there may be contrast enhancement of the hematoma. Finally, the postoperative policy concerning thromboembolic prophylaxis is discussed.
This article reviews the clinical presentation, pathophysiology, diagnostic strategies, and therapeutic management of closed spinal dysraphism in infancy. Four groups of symptoms are distinguished: (1) cutaneous abnormalities, (2) lower motor neuron dysfunction due to congenital spinal and nerve root abnormalities, (3) upper motor neuron dysfunction due to tethering of the spinal cord, and (4) symptoms caused by associated malformations. Guidelines are proposed concerning timing and type of diagnostic investigations in infancy. This essentially encompasses a neurological assessment, including spinal ultrasonography and nuclear magnetic resonance imaging of the spine and the brain, and a urological assessment, including ultrasonography of kidneys and bladder, cystourethrography and a urodynamic study. As to the tethered cord syndrome it is concluded that first, already in infancy, a progressive neurological dysfunction can be detected; second, surgical untethering should be performed only upon appearance of upper motor neuron signs or upon progression of lower motor neuron signs. Despite its frequently asymptomatic course, the diagnosis of a congenital lumbosacral lipoma, and in the more general sense, of a closed spinal dysraphism, implies a periodic, multidisciplinary follow-up for life.