Radiosurgery in the management of pediatric brain tumors.

A total of 114 patients with benign and malignant intracranial tumors were treated by Valentino at the Flaminia Radiosurgical Center using a Philips 6-MeV linear accelerator between 1987 and 1995. The tumor locations break down as follows: 36 in the cerebral hemispheres, 14 in the region of the hypothalamus/optic chiasm, 21 in the III ventricle/pineal region, 3 in the basal ganglia, 27 in the posterior fossa, 13 in the brain stem. Seventy-nine patients had multivariate/combined treatment consisting of surgery or biopsy followed by chemotherapy, radiotherapy and/or radiosurgery. Thirty-five were not operated on or biopsied but were treated primarily by radiosurgery, which was associated with chemotherapy and conventional radiotherapy. The short- and long-term results were evaluated separately for each pathology in an attempt to derive guidelines for future treatment. For tumors of the pineal region, we are of the opinion that radiosurgery is the treatment of choice in children and that more than one-third of patients can be cured by this means. The remaining patients require surgery and/or chemotherapy in addition. For medulloblastomas radiosurgery may be useful to control local recurrence if coupled with chemotherapy. In the case of ependymomas, partly because of the extreme malignancy of the lesions in our series, radiosurgery did not succeed in controlling local recurrence. We fear that limiting treatment to radiosurgery, rather than prescribing conventional radiotherapy when indicated, could permit CNS seeding. For craniopharyngiomas radiosurgery proved useful for controlling solid remnants. In glial tumors radiosurgery helped either to "sterilize" the tumor bed after removal or to treat remnants of the lesions in critical areas; for diffuse brain stem gliomas it should be considered the treatment of choice.

Congenital torticollis in association with craniosynostosis.

The incidence of congenital torticollis in association with plagiocephaly is 1 in 300 newborns, with the torticollis resulting from pathologically sustained contraction of the sternocleidomastoid. Such conditions as facial asymmetries, craniovertebral anomalies, cervical hemivertebra, and mono- or polydysostoses may also be associated with torticollis diagnosed during the neonatal period. With particular reference to synostotic (coronal and/or lambdoidal) plagiocephaly, a clear distinction is made in this paper between posterior neurocranial flattening secondary to the sustained rotation of the skull resulting from torticollis and that seen in synostotic plagiocephaly. The rarity of torticollis with sustained contraction of the sternocleidomastoid muscle relative to the frequency of occipital-parietal flattening in newborn kept in the supine position has not been discussed in the literature and is therefore of clinical importance. In light of the fact that the prognosis and, consequently, the treatment plan vary directly with the presence or absence of synostoses, clinical evaluation also includes cephalometrics, plain skull X-rays, and CT imaging. If the torticollis is associated with neurocranial deformity but synostosis is absent, cervical traction and physiotherapy resolve the symptoms. When, however, the clinical picture is complicated by synostotic plagiocephaly, corrective surgery is necessary, though cervical traction and physiotherapy are essential to provide early and complete cure of the torticollis.

Intramedullary astrocytomas and ependymomas in the pediatric age group: a retrospective study.

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.

Medical abortion: ethics, laws and religious points of view, A study by the 1994-1995 Ethics and Morals Committee of the ISPN.

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.

The future perspectives of pediatric neurosurgery.

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.

Tumour response and morphological changes of acoustic neurinomas after radiosurgery.

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.

Occult dysraphism in adulthood: clinical course and management.

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.

A unifying theory for the definition and classification of hydrocephalus.

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.

The results of radiosurgical management of 72 middle fossa meningiomas.

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.

The education and identification of a pediatric neurosurgeon.

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.

Choroid plexus papillomas of the III ventricle in childhood. Their diagnosis and surgical management.

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.