A model fellowship in pediatric neurosurgery.
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Biomedical subjects
Publications and source records attributed to A J Raimondi.
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A series of 45 pediatric patients underwent surgery for intramedullary astrocytoma or ependymoma at the Cook County Hospital or the Children's Memorial Hospital of Chicago (Northwestern University) and the Neurosurgical Department of Rome "La Sapienza" University. Results showed that intramedullary astrocytomas and ependymomas in children differ from one another in terms of prognosis, and particularly as regards surgical strategy as an element of therapeutic management. Gross total removal of 70% of the ependymomas was achieved, in comparison to 33% of the astrocytomas. In astrocytomas the extent of resection did not significantly influence prognosis. Neurological condition on admission, regardless of oncotype, was found to be extremely influential: patients operated in good condition tended to remain neurologically stable or improve in the long term, while those operated on while in poor condition did not show any improvement at all. No definitive conclusions were possible regarding the value of radiotherapy, because it was only performed in 6 of the 45 cases. The authors limited its use to highly malignant lesions in view of the well-known sensitivity of the spinal cord to radiation, especially in children.
Central nervous system malformations are now frequently detected prenatally. Unfortunately, the progress in diagnosis having far outstripped that in therapeutics, the only question to answer is usually whether the pregnancy should be continued or terminated. Pediatric neurosurgeons have to deal with such problems more and more often. They are asked to give an opinion on the prognosis. In many cases the law requires their involvement in the decision about the possibility of a "therapeutic", or more correctly a medical, abortion. The final decision is based on the religious attitutes of the family and the neurosurgeon and on the law of the country in which the medical abortion would be performed. This article reviews the points of view of the main religions and also the laws in different countries. This knowledge may be of some help to neurosurgeons, especially when the religious attitudes of those involved are different or when their final decision is not in accordance with the law of the country.
Pediatric neurosurgery as a subspecialty dealing primarily with such congenital and perinatal pathology as the dysraphic state, and various clinical entities having in common ventriculomegaly, is fast disappearing from the scene of neurosurgery in the industrialized world. Pari passu with this, one observes ever closer collaborative work between pediatric neurosurgeons and specialists in other pediatric disciplines such as oncology, radiology, orthopedics, and maxillofacial surgery: truly multidisciplinary activities. In addition, paramedical and specialized nursing personnel are participating actively, even in the decision-making and treatment-delivery aspects of pediatric neurosurgical care. The pediatric neurosurgeon is no longer the sole decision-maker, nor the automatic captain of the ship. Very probably, as in the whole history of human activities, new instrumentation will change somewhat what we do and with whom we collaborate. However, for the immediate future the major changes most probably will be expressions of socioeconomic readjustments, of ethical redefinitions, and of the to-and-fro movement into and out of pediatric neurosurgery by both neurosurgeons and ancillary medical personnel.
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Twenty-seven of the 1560 patients treated by radiosurgery during the period 1984-1993 had acoustic neurinomas. Four cases were excluded from this study because they had a follow-up of less than 2 years. There were 24 neurinomas treated in 23 patients as one patient had a bilateral tumour. Seven patients underwent radiosurgery for a recurrent tumour (already operated on once or twice), while it was the first treatment for 16 patients. The tumour volume ranged from 1.99 cm3 to 18.30 cm3, and the patient follow-up was from 2 to 8 years. To determine the target on CT/NMR for linear accelerator stereotactic irradiation, the Greitz-Bergström non-invasive head fixation device was used. It was again adopted for subsequent serial imaging, and for repeat radiosurgery when necessary. The total peripheral tumour dose ranged from 12 to 45 Gy. In 9 patients there was a reduction in tumour volume varying from 39 to 100%, while 14 of the neurinomas appeared stable after an average follow-up of 3 years. In one patient there was an increase in size of the tumour. Variable morphological changes were present in 66% of the neurinomas treated. Radiosurgery is indicated as an alternative to microsurgery for inoperable patients and for those who refuse surgery, for recurrent tumours, and as a post-operative complementary treatment for partially removed tumours. A gradual approach to radiosurgery, depending on tumour response, allows a greater efficacy with minimal risk. In the present series no complications were observed. Hearing was preserved at almost the same level as that prior to radiosurgery in all patients.
If the cerebrospinal fluid (CSF) is considered to be all the fluid (liquid), other than blood or the derivatives of its breakdown, that is normally contained within the brain, its cavities, and its spaces, this could be regarded as "brain fluid" in its most elemental form. "Pathological increases in intracranial CSF volume, independent of hydrostatic or barometric pressure", then, could be considered a definition of hydrocephalus. The observation of significant episodic variation in intracranial pressure (ICP) suggests the necessity of substituting the concept of "time-related pressure variations" for the older one of "level of pressure" in patients with defective ICP control mechanisms. It has been assumed that the subarachnoid channels are the first CSF compartment to dilate in response to the hydrocephalic process, reducing the CSF pressure and thereby establishing an equilibrium. When the equilibrium is disturbed, with progressive dilation of the subarachnoid channels, the increase in CSF pressure is transmitted to the ventricular system, resulting in its dilation (extraprenchymal hydrocephalus). Progressive ventricular dilation causes cerebral edema (intraparenchymal hydrocephalus) and obliterates the subarachnoid spaces as the hemispheres are compressed against the dura, resulting in apparent "internal hydrocephalus" in the absence of "external hydrocephalus". Thus, subarachnoid space or ventricular dilation occur as a result of intermittent increases in extraparenchymal CSF volume: the primary pressure force emanating from the subarachnoid and subdural spaces and from the intraventricular compartment. Hydrocephalus, therefore, may be present in a child who does not yet have dilated ventricles but in whom both CSF volume and pressure are increased. Thus, it becomes obvious that the term internal hydrocephalus is of little significance, since increases in intraparenchymal fluid--cerebral edema--cause the same volumetric changes as increases in intraventricular fluid volume. I suggest that hydrocephalus is a pathologic increase in intracranial CSF ("brain fluid") volume, whether intra- or extraparenchymal, independent of hydrostatic or barometric pressure. It may be classified as (1) intraparenchymal (cerebral edema) and (2) extraparenchymal, with the extraparenchymal types subclassified into subarachnoid, cisternal, and intraventricular forms.
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We present a series of 23 patients with dysraphic malformations and adult onset of symptoms (4 meningoceles, 19 spinal hamartomas). Mean age at presentation was 39 +/- 21 years (range 23-67 years). Patients were followed up for a mean period of 19 months (range 0.5-68 months). Only patients with progressive neurological disease were operated on (3 meningoceles and 16 spinal hamartomas). The remaining patients were treated conservatively and continue to be observed clinically. Two of three patients operated for meningoceles improved without recurrence of symptoms. Patients with spinal hamartomas could be divided into two groups according to their main symptom: paraparesis (group A, n = 8) or pain (group B, n = 11). Malformations in group B were typically associated with a tethered cord and tended to be more complex than in group A. The majority of patients in group A showed better long-term results than patients in group B, due to their considerably lower rate of recurrence.
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Of 812 patients with intracranial tumours treated by radiosurgery during the period 1984-1990, 129 had meningiomas. Of these latter, 72 had middle fossa meningiomas. Patients with meningiomas treated by us since March 1990 are not included in this report since we established the investigative principle of a minimum of 30 months follow-up. Seventeen of the 72 patients were treated after incomplete surgical resection, and 21 for tumour regrowth. In 34 patients, radiosurgery was the primary treatment. The tumour volume was calculated by the ellipsoid method. It ranged from 0.588-76.346 ml. Radiosurgery was performed using the non-invasive stereotactic fixation head device (Greitz-Bergström) adapted to the Fixster frame, and dynamic irradiation performed with the linear accelerator, using especially designed collimators. The total tumour dose for each patient ranged from 15-45 Gy. The minimum follow-up was 2 1/2 years and the maximum 8 years. In 50 patients there was tumour shrinkage ranging from 24-91% of the initial tumour volume. Shrinkage was associated with central tumour necrosis in 11 of these 50 patients. In 18 patients the tumour volume remained stable. In 2 patients there was tumour progression and in 2 there was regrowth after initial reduction of tumour volume. There were no significant treatment complications. Radiosurgery is preferable to re-operation in recurrent meningiomas and indicated after incomplete surgical removal. In high risk patients, as well as in "unresectable" meningiomas, it is an obvious alternative to microsurgery.
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Pediatric neurosurgery very likely became an entity because of the admission of children with CNS diseases to children's hospitals and the development of subspecialization. This latter, in pediatric neurosurgery, has been more a result of events, such as the individual's working propinquity to other pediatric specialists and his being comfortable with children and their parents, than of methodical adherence to a philosophical precept. A fellowship in pediatric neurosurgery, in my opinion, has negative value, in that it would create a working/learning situation in which the fellow would take essential educational and responsibility experiences from the resident and would be delegated patient-care and teaching accountabilities he is not ready to assume. Two, more appropriate, alternatives could be either an additional year as a resident, or a proper staff appointment, depending upon the candidate and the institution. The pediatric neurosurgeon, like other subspecialists in our discipline, may be identified on the basis of what he does, how well he does it, and which societies recognize him as member. Since subspecialization as we see it today is inevitable and represents progress, a certificate (in the subspecialty of pediatric neurosurgery) awarded by the American Board of Neurosurgery would have positive implications, as would, for example, a Continuing Education Award, given by the AANS, the CNS, or the American Society for Pediatric Neurosurgery. Such certificates or awards could serve differing purposes or provide differing recognition as the life of our subspecialty unfolds.
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Ten infants and children with choroid plexus papilloma of the III ventricle are presented. Hydrocephalus of various degrees was present in all patients, and seven patients had bilateral ventriculoperitoneal shunts prior to craniotomy. All patients were investigated with computed tomography and angiography. Tumor was resected through the transfrontal-transventricular approach in nine and through a transcallosal approach in one. One patient died intraoperatively due to an uncontrollable hemorrhage from a subependymal vein at its point of entry into the homolateral internal cerebral vein, and another died shortly after surgery due to hypothalamic trauma. The remaining eight patients are alive without recurrence over a minimum follow-up period of 3 years; three have mental retardation and seizure disorder. Despite this tumor's deep location and vascularity and occurrence in infancy, choroid plexus papillomas of the III ventricle can be successfully resected. Appropriate care for hydrocephalus and intra- and postoperative management are important.
The nature and sequence of the radiation-induced changes in the brain were studied postmortem in 34 children with glioma, 22 of whom underwent central nervous system radiation therapy. Twenty received whole-brain or whole-neuroaxis radiation at a total mean dosage of 4063 cGy. Brain tissue alterations were analyzed histologically by means of various staining methods, including immunohistochemical techniques. The histological features of irradiated brains were compared with those of non-irradiated brains. Microscopic findings included demyelination (seven cases), focal necrosis (six cases), cortical atrophy (four cases), endothelial proliferation (four cases), and telangiectatic vascular proliferation with vascular thickening and oozing of a thick fluid (one case). Such findings were rare in non-irradiated patients. Demyelination was observed earliest in a patient who died 5 months after radiation therapy and was more common after 9 months. Focal necrosis was first observed 9 months post-irradiation but was more advanced and extensive after 1 year. Calcified foci were found only after 60 months. Various vascular changes such as vascular thickening and thrombosis suggested ischemic insult to the brain as a late effect of radiation injury. The results of this study suggest that the immature brain may be more sensitive to radiation than is the adult brain, and that the manifestations of radiation-induced injury depend on the time elapsed after irradiation.