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Background: The CustomBone Service is a bioceramic implant suitable for cranial repair in both adults and children, although there are no clinical data about its use in children under 7 years of age. This surveillance study investigates the outcome in this age group., Method: Twenty-eight children under 7 years old (range, 2.5-6 years) received CustomBone Service from July 2006 to May 2013 in 16 international hospitals. Data of 23 children (12 males and 11 females), harboring 24 prosthesis, were available with a minimum follow-up of 1 year., Findings: Sites of the cranial defect were frontal or parietal (20.8 % each), parieto-temporal (16.7 %), fronto-parietal or occipital (12.5 % each), fronto-parieto-temporal or fronto-temporal (8.3 % each). Initial diseases were trauma (54.2 %), malformation (37.5 %), or tumor of the bone/skin (8.3 %). Rupture of the implant occurred in a single case during the implant (1/26 surgeries, 3.8 %) and the cranial repair was achieved by means of the back-up prosthesis. Five adverse events were registered during the follow-up period consisting of three cases of fracture and two of exposure/infection of the prosthesis. All cases required the removal of the device (20.8 %)., Conclusions: The failure rate of CustomBone Service under 7 years of age was higher than reported in adults and children over 7 years old (20.8 vs. 3.8 %), However, CustomBone Service may be considered a valid option under 7 years old since other materials are burdened by more significant rates of complications in the long-term period. Due to specific properties of this material, indication to CustomBone Service in toddlers should be carefully evaluated by the surgeon on a case-by-case basis.

Background: Although endoscopic septostomy is widely adopted in the treatment of unilateral or specific types of hydrocephalus, there is no consensus on surgical indications, technical aspects and postoperative outcome. In particular, the choice of the ventricular access has been recently debated. We investigated the results of endoscopic septostomy performed through a standard precoronal ventricular access using a rigid endoscope., Methods: Patients who underwent an endoscopic septostomy at our Institution from March 2001 to March 2011 were retrospectively identified. Clinico-radiological data and video recordings of the endoscopic procedures were reviewed., Results: Sixty-three patients (50 children and 13 adults) were collected. In adults, the obstruction of the cerebrospinal fluid (CSF) pathway was exclusively secondary to a tumor (neoplastic or pseudoneoplastic lesion). In the pediatric group hydrocephalus was most commonly due to a neoplasm (33 out of 50 patients), post-hemorrhagic and/or post-infectious hydrocephalus affecting 11 children and malformative hydrocephalus the remaining six children. We were able to perform the septostomy in all but two patients, presenting with a scarred multilayered septum secondary to post-hemorrhagic hydrocephalus. In 37 cases, one or more other endoscopic procedures were performed contemporarily. The mean follow-up was 24 months (min-max: 5-96 months). Overall, all but one patient benefited clinically and radiologically from the endoscopic septostomy. Two patients harboring a pineal/mesencephalic tumor experienced a late obstruction of the stoma secondary to neoplastic infiltration of the septum., Conclusions: Endoscopic septostomy can be safely performed through a standard burr-hole. The effectiveness of this approach is testified by an early success rate of more than 95% and a long term success rate of 92%.

Background: Decompressive craniectomy is an effective treatment option in case of refractory intracranial hypertension after severe head injury. The incidence of complications following cranial repair after decompressive craniectomy for traumatic brain injury is not negligible, particularly in infants and young toddlers. However, only a few dedicated papers can be found in the literature., Method: We describe the complications observed in two boys and one girl under 1 year of age that were treated in the last decade by hemicranial decompressive craniotomy and enlarging hemispheric duraplasty, and subsequent cranial repair by means of autologous bone-flap replacement., Findings: Despite good clinical and neurological outcome, the postoperative clinical course was complicated in all cases by early or late evidence of subdural fluid collections associated to the occurrence of hydrocephalus and causing recurrent dislocation and progressive resorption of the autologous bone flap., Conclusions: Infants less than 1 year old, undergoing decompressive craniectomy after traumatic brain injury, experience a high rate of complications following subsequent cranial repair. Subdural collections and resorption of the autologous bone flap are to be considered as extremely common complications.

The aim of this study is to identify possible risks factors for the occurrence of cerebellar mutism syndrome (CMS) in children with posterior cranial fossa tumours. Children diagnosed with posterior fossa tumours consecutively admitted to our institution between 2006 and 2008 were the subjects of this prospective study. Besides standard neurological and radiological evaluations, all children underwent thorough neuropsychological assessments at admission and following surgery. Children under two or older than 16 years of age and those with a severe pre-operative clinical condition precluding neuropsychological assessment were excluded. Thirty-four children met the inclusion criteria. They were divided into two groups. Group I consisted of 23 children with normal language on admission and group II had 11 children showing pre-operative language impairment (PLI). PLI was observed in 11 children (32.4%: group II). Post-operatively, seven out of 34 children developed CMS (20.6%), all of them belonging to group II. In group II, indeed, the incidence of CMS was 63%. No case of CMS was observed in group I. PLI regressed after the operation in three out of the four subjects belonging to group II who did not develop CMS. PLI remained unchanged in the last child of this group. Posterior fossa tumour resection can have different effects on children with pre-existing language impairment (PLI). PLI can be considered a subclinical state of CMS in some children with posterior fossa tumour. However, in some children with PLI, the tumour resection may improve the linguistic abilities, as well as the other neurocognitive performances. In the present series, children with normal pre-operative language function did not develop post-operative mutism.

Background: The practice of endoscopic endonasal surgery is still limited in children because of the relative rarity of sellar lesions in the paediatric population. However, such an approach is increasingly reported in the literature as an alternative option to standard sublabial microsurgery, and its surgical results are being analysed. No information on the trend of the postoperative course is currently available. The goal of this paper is to assess the impact of these two different transsphenoidal approaches on the postoperative course., Methods: Thirty-one consecutive paediatric patients were treated for sellar region lesions by transsphenoidal surgery. Fourteen children (group A) underwent surgery with a microsurgical sublabial transsphenoidal approach between 2000 and 2005; the remaining 17 children (group B) underwent surgery with an endoscopic endonasal transsphenoidal approach between 2005 and 2009. The two groups were compared as to rate of admission to the PICU, relief from preoperative symptoms, early surgical complications, postoperative pain (Wong-Baker FACES Pain Scale) and length of postoperative hospital stay., Results: Children in group B showed decreased rates of admission to the PICU (35% versus 100%) and peroperative blood transfusions (23% versus 71%), and a shorter mean postoperative hospital stay (4 days versus 5.7 days). The pain perception was significantly lower in group B than in group A, as demonstrated by the scores registered during both the early (2.05 ± 0.74 versus 2.92 ± 0.91) and late postoperative period (0.82 ± 0.95 versus 1.64 ± 0.84). No remarkable differences in terms of operative time, symptom improvement and early complications were detected., Conclusions: Endoscopic endonasal surgery can improve the quality of the postoperative course in children regardless of the type of lesions treated and the surgical complications.

Background: The aim of the study was to evaluate the results of instrumented rod and wire fusion in children with craniovertebral junction (CVJ) instability and os odontoideum., Methods: We evaluated seven children (mean age 9.85 years) with Down and Morquio's syndromes and primary os odontoideum. X-ray, computerized tomography (CT) scan and magnetic resonance (MR) imaging of the CVJ showed reducible instability in all of the cases but one. All the children underwent surgical correction by means of posterior wiring, instrumentation, fusion and external orthosis. A posterior wiring technique was also utilized in the only child with irreducible preoperative atlantoaxial instability, which, however, proved to be reducible under general anesthesia., Findings: At maximum follow-up (observation range 28 to 106 months, mean 59.42 months), the clinical picture was improved in all the patients. The postoperative neuroradiological investigations demonstrated satisfactory bony fusion with neural decompression in all patients., Conclusions: A wiring technique to correct atlantoaxial instabilities has been shown to be more relevant in these children with syndromic atlantoaxial dislocation and os odontoideum due to its simplicity, safety (continuous fluoroscopic assistance is not necessary and there is no risk of neuro-vascular injuries) and lower costs (no complex hardware devices; no neuronavigation systems are required). Preoperative irreducibility of the C1-C2 shift is not an absolute criterion for transoral decompression in children since os odontoideum can be reduced under general anesthesia.

In a selected patient population, we evaluated the glycemic response to different infusional policies in the management of posterior cranial fossa tumor (PFT) removal. We analyzed the perioperative course, prospectically collected, of 137 children undergoing 150 surgical procedures. Patients were divided in two groups according to different intraoperative fluids (group A, 2.5% glucose; group B, crystalloids). In group B glycemia remained below 125 mg dl(-1), while group A showed persistently supranormal glycemic plasma values, reaching statistical significance at the end of surgery (P < 0.018). As no perioperative mortality occurred and no differences were found between groups regarding PICU respiratory or infectious complications, PICU length of stay (LOS) was assumed as the main outcome indicator. LOS was not influenced by group A or B inclusion, while a new indicator, namely the Glycemic Stress Index (GSI), representing both glycemic intraoperative change and procedure length, showed significantly different results in the study groups (P = 0.004). Our clinical experience suggests that both intraoperative glucose-free solutions are safe, and GSI can be a useful tool to identify prolonged PICU stay patients.

Background: Little is known about the nature of spine surgery training received by European neurosurgical trainees during their residency and the level of competence they acquire in dealing with spinal disorders., Methods: A three-part questionnaire entailing 32 questions was devised and distributed to the neurosurgical trainees attending the EANS (European Association of Neurosurgical Societies) training courses of 2004., Results: Of 126 questionnaires, 32% were returned. The majority of trainees responding to the questionnaire were in their final (6(th)) year of training or had completed their training (60.3% of total). Spinal surgery training in European residency programs has clear strengths in the traditional areas of microsurgical decompression for spinal stenosis and disc herniation (77-90% competence in senior trainees). Deficits are revealed in the management of spinal trauma (34-48% competence in senior trainees) and spinal conditions requiring the use of implants and anterior approaches, with the exception of anterior cervical stabilisation., Conclusions: European neurosurgical trainees possess incomplete competence in dealing with spinal disorders. EANS trainees advocate the development of a postresidency spine subspecialty training program.

Background: According to Menezes' algorithm, pre-operative dynamic neuroradiological investigation in C1-C2 dislocations (C1C2D) instability is strongly advocated in order to exclude those patients not eligible for posterior fixation and fusion without previous anterior trans-oral decompression. Anterior irreducible compression due to C1C2D instability, it is said, needs trans-oral anterior decompression. We reviewed our experience in order to refute such a paradigm., Methods: The study involves 23 patients who were operated on for cranio-vertebral junction (CVJ) instability; all of them had C1C2D of varying degree on x-ray, computerised tomography (CT) and magnetic resonance (MR) imaging of the CVJ. Pre-operatively, irreducible C1C2D was demonstrated only in 3 patients, (2 with Down's Syndrome, one of them was harbouring os odontoideum, 1 Rheumatoid Arthritis), i.e. 13.04%; the remaining 19 (86.9%) had reducible C1-C2 dislocation. After an unsuccessful traction test conducted in the pre-operative phase under sedation, it was possible to completely reduce the C1C2D (with a combination of axial traction with light extension of the neck on the chest and a light flexion of the head on the neck by using a Mayfield head holder) and proceed to posterior fixation in all the patients under general anaesthesia using a precise "timing sequences fixation technique". Wiring (C0 and C3 were fixed first being stretched up to approximately 10 lbs, then C2 in order to pull up this vertebra last by forcing approximately 8 lbs) or screw fixation methods were used to achieve fusion along with post-operative external orthosis and neuroradiological assessment of the C1C2D. The instrumentation produced a lever and pulley effect which assisted reduction of the dislocation., Findings: At follow up (range 34-55 months-mean 45.33 months) the clinical picture was improved or stable in all patients., Conclusions: Pre-operative irreducibility of the C1C2D should not be an absolute indication for trans-oral decompression. An attempt to reduce the dislocation under general anaesthesia and during posterior fixation should be attempted in Down's syndrome, os odontoideum and rheumatoid arthritis.

Objective: To evaluate perioperative management, early outcomes and hemocoagulative disorders in infants up to 1 year old, undergoing neurosurgery for brain tumors removal., Design: Retrospective evaluation of prospectively collected data regarding all infants aged <1 year admitted to PICU from 1994 to 2004, following intracranial mass removal., Interventions: none., Setting: University Hospital PICU in a tertiary neurosurgical referral centre., Patients and Participants: All eligible infants were enrolled in the study. Population was constituted by 43 infants and subdivided in two groups, according to their intra-operative blood loss. Babies having blood loss exceeding the preoperative estimated volemia were classified in group A; the remaining babies were included in group B., Results: Intraoperative transfusions, PICU length of stay, need for post-operative mechanical ventilation and cardiovascular support were all significantly higher in group A than in group B. No early postoperative mortality occurred. ROC analysis and multiple logistic regression showed the age as the only variable independently associated with blood loss exceeding preoperative volemia (cut-off 60.3 days; OR = 0.11, CI 0.02-0.55, sensitivity 82.4%; specificity 67%). Postoperatively, platelet count, prothrombin activity and fibrinogen resulted significantly depressed in group A, representing a dilutional coagulopathy. A marked dispersion of aPTT values was recorded in group B, where most infants exhibit aPTT shortening, suggesting a hypercoagulability status. Three episodes of clinical disseminated intravascular coagulation (DIC) were registered in group B., Conclusions: We illustrated the relationships between intraoperative blood loss, transfusions and haemostatic impairment in babies following brain tumor removal. Youngest infants had the higher risk to experience hemocoagulative disorders. These infants showed significantly higher impact on the global PICU burden of care, as represented by the need of mechanical ventilation, cardiovascular support and PICU length of stay.

Background: The effectiveness of antibiotic pre-treated ventricular catheters in reducing the risk of CSF infections (determined on CSF cultures) resulting from the use of per-operative external ventricular drainages (EVD) and the success rate of post-operative endoscopic third ventriculostomy (ETV) in the management of persistent hydrocephalus after posterior cranial fossa tumour removal are assessed., Method: Forty-seven children (group I) were prospectively managed by means of per-operative antibiotic impregnated EVD, post-operative ICP monitoring, and ETV. The results of this group were compared with those of a control group composed by 44 children treated with the same protocol as above except for the use of not-impregnated catheters (group II)., Findings: The rate of positive CSF cultures due to EVD resulted significantly lower in group I (2.1% vs 31.8%); there was no clinical evidence of CSF infections. The success rate of ETV was the same in both groups (75%). Failures of ETV occurred in the patients with subarachnoid tumour seeding and/or tumour extension to the basal cisterns. All the children of group II with failed ETV also showed a bacterial growth in the CSF., Conclusions: Antibiotic pre-treated catheters in our experience considerably limited EVD-related bacterial growth in the CSF. Preoperative hydrocephalus resolved in 60% of the cases after tumour removal, thus confirming recent data from the literature against the routine use of preoperative ETV. In our experience postoperative ETV had a high success rate; poor results were obtained in children with tumour seeding and/or the evidence of positive CSF cultures.

The aim of this study was to evaluate longitudinally functional and neuro-radiologic findings in childhood optic gliomas (OG), by comparing flicker visual evoked potentials (F-VEPs) with brain magnetic resonance imaging (MRI) changes. Fourteen children (age range: 1-13 years) with OGs underwent serial F-VEP, MRI and neuro-ophthalmic examinations over a 38 month (median, range: 6-76) follow-up. F-VEPs were elicited by 8 Hz sine-wave flicker stimuli presented in a mini-Ganzfeld. Contrast-enhanced MRI examinations were performed. Results of both tests were blindly assessed by independent evaluators. F-VEPs were judged to be improved, stable or worsened if changes in the amplitude and/or phase angle of the response exceeded the limits of test-retest variability (+/-90th percentile) established for the same patients. MRI results were judged to show regression, stabilization or progression of OG based on its changes in size (+/-20%) or extension. Two to seven pairs of F-VEP/MRI examinations per patient (median: 4) were collected. Based on a total of 38 pairs of F-VEP/MRI examinations, both tests agreed in showing worsening (progression), stabilization and improvement (regression) in 5, 15 and 10 cases, respectively. In 3 cases, F-VEPs showed a worsening and MRI a stabilization, while in 5 cases F-VEPs showed an improvement and MRI a stabilization. Agreement between F-VEP and MRI changes was 78.9% (95% CI: +/- 37%, K statistics = 0.67, P < 0.001). The results indicate that longitudinal F-VEP changes can predict changes in MRI-assessed OG size and extension, providing a non-invasive functional assay, complementary to neuro-imaging, for OG follow-up.

Purpose: The objective of the study was to evaluate the efficacy and toxicity of Temozolomide (TMZ) administered for 5 consecutive days in three daily dosing in children with recurrent or refractory high-grade glioma., Patients and Methods: Twenty-four patients with a median age of 10.5 years were enrolled onto this open-label, multicenter, phase II study. The patients were previously treated with surgical resection (17 of 24), radiotherapy (19 of 24) and chemotherapy (18 of 24). Therapy was administered orally three times a day for 5 consecutive days at the dose of 200 mg/m(2)/dx5 for chemotherapy naive patients. In patients heavily pretreated with chemotherapy the starting dose was of 150 mg/m(2)/dx5., Results: A total of 95 cycles were administered. The median progression free-survival (PFS) was 3 months for the entire group while disease stabilization was obtained in 7 patients (29.1%), all with supratentorial tumors. No CR or PR was observed. TMZ treatment showed a limited toxicity. Thrombocytopenia was the most common hematological adverse effect. Our data suggest a marginal activity of TMZ in children with recurrent high-grade glioma.

The possibility to remove a previously inserted CSF shunt device in hydrocephalic children is a well known though rare event for paediatric neurosurgeons. A retrospective analysis of our experience with a series of 850 children affected by non tumoral hydrocephalus shows that obvious shunt independence could be demonstrated in 3.2% of the patients (27 cases). The time interval between the CSF shunt insertion and removal ranged between 8 months and 12 years (mean: 8 years). Parameters analysed to search for any predictive elements were age at surgery, aetiology, type of prosthesis utilised, time interval between insertion and removal of the shunt, number of the possible revisions. The results of the study suggest that the highest incidence of shunt independence is reached in subjects operated on in early infancy, as 24 of 27 removed shunts were in patients operated on under 6 months of age, and the remaining in 2 children treated when less than 2 years old. Such a finding could be explained on the grounds of a delay in maturation of the CSF absorption mechanisms followed by a late normalisation in these patients. As regards to aetiology, 41% of the 27 patients considered in this series were affected by a post-haemorrhagic hydrocephalus, which was progressive, as demonstrated by serial neuroradiological examinations and echo-Doppler cerebral studies at the time of the surgical treatment. In six children the hydrocephalus was associated with myelomeningocele. Five patients had aqueduct stenosis and 2 communicating hydrocephalus. The types of CSF shunting system we utilised did not play any role in determining or facilitating shunt independence. No correlation was observed with the need and the number of shunt revisions. The role of the interval time between the insertion and the removal of the shunt was not analysable, because of the possible acquisition of the shunt independence prior to its demonstration at the moment of the surgical revision of the CSF shunt (elective lengthening because of the physiological body growth) or to the radiological demonstration of CSF shunt device disconnection. The same constraint prevents the evaluation of the actual overall incidence of shunt independence in shunted hydrocephalic children, as some of them could have harboured a non-functioning CSF shunt device, though unnoticed.

Background: Extra-axial chronic fluid collections are pathological conditions occurring more frequently in the paediatric age, particularly in children less than two-year-old. Although recognised for many years and repeatedly reported in the literature, some aspects of their treatment are still under debate. This review of the management of chronic subdural collections is based on our experience of 72 cases treated between January 1984 and December 2000., Method: Patients were divided into four groups according to aetiology. Thirty-four cases (47%) occurred following various surgical procedures; 14 cases (20%) occurred as complications of leptomeningeal infections; 13 cases (18%) were post-traumatic, whereas no obvious aetiology could be detected in the last 11 cases (15%). The collections were unilateral in 34 patients (47%) and bilateral in the other 38 (53%)., Findings: Thirty-five patients (48%) were treated by means of a temporary subdural external drainage (SED) (for a total of 38 SED procedures), which was maintained for a mean of 5.8 (+/-3.4) days; it was effective in 26 cases, whereas in the other 9 it was necessary to perform a subdural-peritoneostomy (SPS). Three of these 38 SEDs were complicated by infection. In one more child the external drainage was complicated by a chronic subdural haematoma. A SPS was performed in 44 cases (61%), 9 being failed external drainages. Only in 16 (38%) of these 44 patients the SPS was removed after an average of 27.0 (+/-16.6) months. Three patients (4%) were treated by craniotomy and resection of the neomembranes lining the subdural fluid collection. Obstruction of the SPS occurred in 6 children, infection in 4. Good results were obtained with either external or internal drainage. In fact post-operative neuroradiological investigation disclosed in all the cases complete or almost complete cerebral re-expansion; also the clinical outcome was generally very good, although largely dependent upon the basic clinical condition. Unilateral subdural-peritoneal and/or external drainage was effective also in case of bilateral collections. Only 2 patients required temporary bilateral external drainage.

In spite of their congenital origin, only 18-20% of cerebral AVMs are diagnosed during infancy and childhood. Intracranial haemorrhage is the presenting clinical manifestation in 75-80% of paediatric patients and is associated with a high morbidity and mortality. The natural history of untreated cerebral AVMs in children is worse than in adults, in relation to a longer life expectation, a higher annual risk of AVM bleeding (3.2% vs. 2.2%) and a higher incidence of posterior fossa and basal ganglia AVMs, most of which present with massive haemorrhages. The surgical excision remains the treatment of choice for parenchymal AVMs in children; AVM complete removal is currently achieved in 70-90% of the patients. With the advent of new agents for endovascular management, preoperative AVM embolization has further improved surgical results. Stereotactic radiosurgery appears to be a successful treatment option in small or moderate sized AVMs. Recent studies have demonstrated low complication rates with this technique in paediatric patients. We reviewed our experience with 37 paediatric AVMs treated at the Section of Paediatric Neurosurgery of the Catholic University of Rome between 1980 and 1997. Twenty-three patients underwent surgery as the only treatment modality; endovascular embolization was combined with the surgical treatment in a further four cases. Radiosurgery was utilized as the only treatment in three patients and in combination with other techniques in an other three children (with surgery in one case and with AVM embolization in the remaining two subjects). No treatment was carried out in three patients because of excessively critical condition on admission; endovascular embolization failed in a further patient because of the anatomical complexity of the malformation. Previous studies have demonstrated a quite strict correlation between AVM complexity based on Spetzler and Martin's grading system and patients outcome. A less direct relationship has been observed in the present study. In our experience the factors which were more closely predictive of patients' outcome were the occurrence of an AVM bleeding and the neurological status on admission. In spite of the low number of cases in the single subsets of patients this study seems to support the role of AVM embolization and radiosurgery as effective adjuvant techniques in the management of cerebral AVMs in children.

The overall prognosis in children with medulloblastoma/PNET has not significantly improved over the past decade. Intensive neoadjuvant chemotherapy has not yet adequately explored. We evaluated the short-term clinical results of an intensive chemotherapy regimen in high risk children with newly diagnosed MB/PNET, after surgery and before radiation. Twelve previously untreated patients with high-risk medulloblastoma/PNET, according to Chang's classification, were treated with the following chemotherapy regimen: high dose carboplatin 600 mg/m2/day on days 1 and 2; the same course was administered 4 weeks later. One month later, high dose cyclophosphamide 2 g/m2/day on days 1 and 2, followed by an identical course 4 weeks later. Vincristine 1, 5 mg/m2 i.v. was given on the first day of each course. Systemic evaluation of the disease included imaging of the entire neuraxis, including MRI of the entire spine. Out of 12 enrolled, 7 patients were able to be evaluated for a residual disease after surgery. After two cycles of high dose carboplatin, we noted 1 CR, 4 PR and 2 MR. After the subsequent two cycles of high dose cyclophosphamide we observed an additional response in 4 cases. On the other hand, 4 patients clearly showed evidence of PD immediately after the first course of cyclophosphamide (2 cases) or following the second course. Three of the 4 patients had shown respectively 1 CR and 2 PR after the second course of carboplatin. Whereas it was confirmed that 2 courses of high dose carboplatin is effective in high risk MB/PNET children, we observed an unacceptable number of PD during the subsequent high dose cyclophosphamide therapy. A review from the literature also suggests that, in general, the longer radiotherapy is delayed, the higher the incidence of PD. In the search for the optimal drug combination in "sandwich chemotherapy" for children with high risk MB/PNET, PD must be reduced to an acceptable incidence, since a high number of PD may significantly lower the probability of long-term survival.

Although histologically benign, craniopharyngioma can regrow either from macroscopic remnants of the tumour left behind at operation, or even after an apparently gross total removal. Recurrence rates vary significantly in the literature, depending on the efficacy of surgical treatment and also on the growth potential of the tumour itself. The main factor influencing tumour regrowth is obviously the extent of surgical resection, as total removal carries a much lesser risk of recurrence compared to subtotal or partial resections (although in such cases radiation therapy can lower this risk significantly). Other factors involved are the duration of follow-up and patient's age at operation, as children tend to relapse more frequently than adults. Even in the "microsurgery" era, characterized by high percentages of total resections, recurrences remain high and continue to represent a major problem of craniopharyngioma treatment. Twenty-seven children and adolescents were operated on for craniopharyngioma at the Department of Neurosurgery, Section of Pediatric Neurosurgery, Catholic University Medical School, Rome between June 1985 and June 1997. Total tumour resection was achieved in 18 cases, subtotal in 7 and partial in 2 instances. One patient died post-operatively. Post-operative neuroradiological investigations confirmed the operative findings, although 3 children with an apparently gross total removal showed a residual non-enhancing calcium fleck adherent to the hypothalamus (which remained stable at the following examinations). Three of the 9 patients with less than total removal underwent post-operative radiation therapy. Out of the 26 surviving patients 6 presented a recurrence of their craniopharyngioma, 2 after an apparently gross total removal and 4 after a subtotal or partial resection (one of them had received radiation therapy). The diagnosis was merely neuroradiological in 5 cases, as only one child presented a clinical picture suggestive of tumour regrowth. Surgery was the first therapeutic option in all the cases. Total tumour resection was accomplished in 3 cases, subtotal in 2 and partial in the last one. One child died post-operatively. Four of the 5 survivors received radiation therapy. All the patients are presently alive and stable (mean follow-up: 5.6 yrs). The authors conclude that surgery should be the first therapeutic option in case of recurrent craniopharyngioma and that radiation therapy should also be considered but only as adjuvant therapy.

A series of 22 patients under the age of 15 years with cavernomas were treated at the Section of Paediatric Neurosurgery of the Catholic University of Rome between 1981 and 1995. The most common symptoms at presentation were epilepsy, in 12 children, and intracranial hypertension, in 9 children; seizures were present on admission in 14 patients; no patient had a family history of cerebro-vascular malformations, two had multiple lesions. Radiological signs of significant acute and subacute haemorrhage were found in 17 cases. The supratentorial compartment was the most frequent location of the lesions, with only three subtentorial cavernomas. The post-surgical results were good: only four children had a persistence of pre-surgical neurological signs, even though they were ameliorated by the treatment. No patient showed a progression of pre-operative neurological signs, nor the onset of new deficits. Only one child died, but his clinical condition was critical before surgery, because of deep coma, with bilaterally dilated and fixed pupils, and no response to any stimulation. Our results suggest that the younger children present significant haemorrhage more frequently than the older ones; this is demonstrated by the analysis of the mean age of the patients with macro-haemorrhage (= 7 years) and the average age of those without macro-haemorrhage (= 11 years). The removal of cavernomas was always able to control the seizure disorders of our patients, proving that in these cases lesionectomy alone may be sufficient to resolve epilepsy.

Late deterioration of the neurological condition in patients operated on for myelomeningocele repair has been repeatedly reported in the literature. At the present time magnetic resonance imaging (MRI) allows one to recognize various pathological conditions which can be amenable to surgical correction in these patients, such as Chiari type II malformation, hydro/syringomyelia, decompensated hydrocephalus, and tethered spinal cord. The authors report their experience with 26 myelodysplastic children operated on for myelomeningocele repair in the early neonatal period, who exhibited late deterioration at variable time intervals from the first operation. The children were examined pre-operatively by means of MRI; the results were compared with those provided by MRI in 46 myelodysplastic children who underwent the investigation as a routine follow-up control. The MRI findings were subdivided into 4 main groups of increasing severity from 1 to 4; in some subjects, associated pathological conditions (Chiari II malformation, hydromyelia, etc.) were detected as well. Twenty-two out of the 26 patients with late neurological deterioration were operated on. Eleven of them (grades 2 to 4) underwent detethering of the fixed conus, with an improvement of their clinical picture from mild to good. On the other hand the remaining 11 subjects (grades 1 to 2) improved their condition following the correction of the associated abnormalities (malfunctioning CSF shunt: 5 cases; hydromyelia: 4 cases; symptomatic Chiari II malformation: 2 cases).

The authors report on 51 infants with intracranial tumours treated in an eleven-year period; these infants represent 13% of the total population of children with intracranial tumours who have been operated on in the same institution during the same period of time. Males (28 cases) were slightly more frequent. Astrocytomas (17 cases), medulloblastomas (12 cases), and ependymal tumours (5 cases) were the commonest histologic types. Signs and symptoms of increased intracranial pressure were by far the most frequent clinical manifestations, followed by seizure disorders. Thirty tumours were localized within the supratentorial, and 21 within the subtentorial compartment. The parasellar region (10 cases) and the lateral cerebral ventricles (8 cases) for the supratentorial tumours, the inferior cerebellar vermis and fourth ventricle (13 cases) for the infratentorial tumours appeared to be the preferred topographic locations. Craniotomies were carried out in 44 infants, with a total or radical removal of the tumour in 19 cases, a subtotal removal in 6 cases, and a partial removal in 17 cases. In 3 cases only a biopsy procedure was performed. Twenty-nine of these patients required an ancillary procedure such as CSF shunting. Three subjects underwent a biopsy procedure and 1 infant the insertion of a CSF shunting device only. Surgery was not performed in 5 cases. Overall, there were two surgical deaths. Two infants died before any surgical treatment could be performed. Radiation therapy was administered to 9 patients when they had reached three years of age. Chemotherapy was given to 21 infants, according to various chemotherapeutic protocols. During the postoperative period 20 deaths (39%) were recorded. Two patients were lost to follow-up. From 1 to 10 years after the operation, 29 patients are still alive, 14 of them (28%) with a normal psychomotor development, 10 (20%) with some neurological or mental deficits, and 5 (10%) with severe psychomotor retardation. There was no apparent correlation in this series between late outcomes and the histological type of the tumour.

Twenty two of fifty five patients initially suspected of suffering from normotensive hydrocephalus were surgically treated (CSF ventriculoatrial shunt). The results of surgery were related to the findings of the different diagnostic examinations (pneumoencephalography, isotope cisternography, transfer fron CSF to blood of isotope labelled serum albumin, constant infusion manometric test, long lasting intraventricular pressure recording). 1.Pneumoencephalography, intraventricular pressure recording, and, above all, isotope cisternography provided the most reliable data for diagnosis and surgical prognosis. 2. The combined use of pneumoencephalography and isotope cisternography was sufficient for a correct diagnosis and surgical prognosis in about 50% of the patients examined. 3. When the diagnostic information given by the combined results of the two above examinations was not sufficient, intraventricular pressure recording proved to be the most useful examination to supplement it. 4. The relation of the results of the study of transfer of istope labelled serum albumin from CSF to blood and of the constant infusion manometric test to the surgical outcome was uncertain.