Posterior Surgical Ligation and Cyst Decompression -via Needle Puncture- of a Large Anterior Sacral Pelvic Meningocele Through Posterior Sacral Laminectomy.

The first documented description of an anterior sacral meningocele was Bryant's in 1823. Anterior sacral meningocele patients have constipation as a universal symptom; urinary incontinence is also common. All the symptoms are directly related to the pressure from a pelvic mass on adjacent structures. When the patient stands, a headache often develops because the spinal fluid pressure decreases as the meningocele sac fills. Finally, a scimitar-shaped sacrum on a neuroradiological anteroposterior plain assessment is pathognomonic. The coccyx may be absent, and the lower sacral laminae may be absent or incomplete. The surgical options for this rare clinical condition are still matter of debate.Anterior sacral meningocele is a pathology that lacks a current classification and neurosurgical therapeutic standards, even though a similar dynamic has been shown by the related traumatic pseudomeningocele. Anterior approaches (retro- and transperitoneal meningocele neck occlusion with internal cerebrospinal fluid (CSF) cyst drainage) and posterior approaches (posterior sacral laminectomy, dural sac ligation, and CSF cyst drainage) are the available surgical strategies.We now report the case of an adult patient for whom a posterior approach was suggested and performed and report her postoperative surgical follow-up. The surgical rationale is also discussed.

Myelomeningocele Repair: Surgical Management Based on a 30-Year Experience.

BACKGROUND: Myelomeningocele (MMC) is a rare but severe spinal defect resulting from a failed neurulation process. Surgical repair is a delicate procedure that needs accurate neuroanatomical knowledge and adequate surgical planning. MATERIALS AND METHODS: The authors report on the main problems of the surgical management of this type of dysraphism. The technique used for reconstruction, and the management strategies, are analyzed according to the authors' experience with more than 600 cases, in comparison with the relevant literature. RESULTS: Tip and tricks of more than 30 years of MMC surgical management are reported, with special mention of the timing of surgery, the management of early hydrocephalus, and the principles of the repair, from the dissection to the closure of five separate layers (arachnoid, dura, muscular fascia, subcutaneous layer, skin). CONCLUSIONS: Accuracy and attention to the reconstruction are crucial for achieving good results and avoiding perioperative complications.

Posterior resection for childhood lesional epilepsy: neuropsychological evolution.

The aim of this study was to provide information on the neuropsychological evolution of children with symptomatic epilepsy who have undergone surgical resection of posterior (occipitoparietal) lesions. Twelve children with epilepsy with parietal and/or occipital lesions were enrolled in the study and followed after surgical resection: full clinical and epileptic examinations were performed before and after surgery, as was a neuropsychological study of both general and specific cognitive abilities. Epilepsy evolution was generally good (Engel classification IA in nine cases) with persistent selective neurological impairments (eye field defects, sensory unilateral spatial neglect) in some cases, consistent with the lesion site. Neuropsychological defects before surgery in the absence of refractory epilepsy were minimal with a normal global cognitive competence; yet, the relatively low performance scores with some impairment of specific cognitive skills were strictly correlated with defects in visual perceptive skills in both right- and left-sided lesions. Surgery seems to have improved performance abilities, whereas other abnormal specific skills did not change with the exception of working memory that in some cases was defective before surgery and normalized after lesion removal. Our study in this particular cohort of children with epileptogenic occipitoparietal lesions thus confirmed a trend toward a benign epileptic and neurodevelopmental outcome after surgical resection of the lesion.

Correlations between the abnormal development of the skull base and facial skeleton growth in anterior synostotic plagiocephaly: the predictive value of a classification based on CT scan examination.

BACKGROUND: Anterior cranial plagiocephaly, depending on the early hemicoronal suture fusion, is the most relevant form of plagiocephaly in terms of clinical implications. Its estimated incidence ranges between 0.4 and 1 per 1,000 live births. In the present report, we aim at validating the classification of Di Rocco and Velardi, proposing a scheme based on basicranium analysis using CT scans and its predictive value by evaluating the developmental characteristics of a population of adult subjects affected by anterior plagiocephaly who had underwent the surgical correction in the first months of life. MATERIALS AND METHODS: The group of patients here considered was retrieved from among all patients operated upon for craniostenosis in the pediatric neurosurgery unit of Policlinico Gemelli in Rome between January 1, 1980 and December 31, 1989. The study group consisted of 13 patients, seven females and six males, affected by anterior synostotic plagiocephaly ranging in age between 20 and 32 years (mean 25.54 years). We also formed a group of unaffected patients in order to control for normal variability in the population. The subjects of the study group were evaluated using CT scan exams and cephalometric analyses were performed using three-dimensional reconstruction. DISCUSSION AND CONCLUSION: In this study, we were able to associate a facial phenotype to confirm the predictive value of the classification proposed. It is highly probable that the different outcomes depend on the different degrees of involvement in the synostotic process by the various skull base sutures which were essentially unaffected by the surgical procedures.

Surgery of children with frontal lobe lesional epilepsy: neuropsychological study.

AIM OF THE STUDY: was to provide new data about the evolution of neuropsychological findings in patients with lesional frontal lobe epilepsy (FLE) operated on with lesion excision. PATIENTS AND METHODS: Twelve patients with lesional FLE underwent full clinical examination including neurological, neuropsychological and developmental assessments, high-resolution magnetic resonance imaging (MRI), ictal and interictal prolonged EEG monitoring and evaluation of seizure semeiology before and after surgery. The mean follow-up duration was 2 years and 10 months (range=14 months-7 years). Another group of lesional temporal lobe epilepsy, matched for the age at surgery and side of surgery, was likewise studied in order to compare neuropsychological patterns and to try to find out specific features in frontal lobe epilepsy evolution. RESULTS: All patients resulted seizure free at outcome except one belonging to Engel's class II. Before surgery general intelligence was similar in FLE as well as in TLE group. Executive functions and motor coordination were frequently affected in FLE whereas patients with TLE often presented with deficits in naming, visual memory and visuo-spatial attention. After surgery there was a frequent decline of IQ in FLE group together with a slight deterioration, especially of executive functions in some patients. An improvement of behaviour was often observed in both groups. CONCLUSIONS: As already reported in literature, neuropsychological pre-surgical data confirms the involvement of attention and executive functions in lesional FLE. No significant neuropsychological improvement was produced by surgery that determined in some cases a slight decline of general intelligence and specific frontal abilities. Yet, generally behaviour improved and seizures were controlled.

Infant ependymoma in a 10-year AIEOP (Associazione Italiana Ematologia Oncologia Pediatrica) experience with omitted or deferred radiotherapy.

PURPOSE: The protocols of the 1990s omitted or delayed irradiation, using upfront chemotherapy to spare the youngest children with ependymoma the sequelae of radiotherapy (RT). We treated 41 children under the age of 3 years with intracranial ependymoma between 1994 and 2003. PATIENTS AND METHODS: After surgery, chemotherapy was given as follows: regimen I with four blocks of vincristine, high-dose methotrexate 5 g/m(2), and cyclophosphamide 1.5 g/m(2) alternating with cisplatin 90 mg/m(2) plus VP16 450 mg/m(2) for 14 months; subsequently, regimen II was used: VEC (VCR, VP16 300 mg/m(2), and cyclophosphamide 3 g/m(2)) for 6 months. Radiotherapy was planned for residual tumor after the completion of chemotherapy or for progression. RESULTS: We treated 23 boys and 18 girls who were a median 22 months old; 14 were given regimen I, 27 were given regimen II; 22 underwent complete resection, 19 had residual tumor. Ependymoma was Grade 2 in 25 patients and Grade 3 in 16; tumors were infratentorial in 37 patients and supratentorial in 4. One child had intracranial metastases; 29 had progressed locally after a median 9 months. Event-free survival was 26% at 3 and 5 years and 23% at 8 years. One child died of sepsis, and another developed a glioblastoma 72 months after RT. Progression-free survival was 27% at 3, 5, and 8 years, and overall survival was 48%, 37%, and 28% at 3, 5, and 8 years, respectively. Of the 13 survivors, 6 never received RT; their intellectual outcome did not differ significantly in those children than in those without RT. CONCLUSIONS: Our results confirm poor rates of event-free survival and overall survival for up-front chemotherapy in infant ependymoma. No better neurocognitive outcome was demonstrated in the few survivors who never received RT.

Cranial reshaping using methyl methacrylate: technical note.

Reconstruction of the facial skeleton and cranial vault has always posed difficult problems. To obtain a good result, various autologous, homologous, and heterologous grafts and biomaterials can be used. In this work, the authors present their experience in the use of methyl methacrylate (MMA) presenting a simple technique for application of the material that reduces the risk of complications. The authors also performed a review of the international bibliography relative to the use of MMA as an autologous bone substitute, analyzing the risks, complications, and results obtained.Between October 2003 and September 2007, the authors used MMA in procedures of cranial reshaping on 9 patients who underwent surgery to repair the damage caused by cranial-facial traumas and malformations.The technique of layered application of MMA made it possible to obtain excellent aesthetic and functional results in 8 patients, whereas in 1 case, the material had to be removed when the patient developed complications due to infection.Methyl methacrylate still remains the material of choice for cranioplasty on adult patients. The application of the material and sequenced layers makes it possible to reduce perioperative complications to the utmost, providing an excellent aesthetic and functional result.

Role of temozolomide in pediatric brain tumors.

FEATURES OF TEMOZOLOMIDE: Temozolomide (TMZ) belongs to the imidazotetrazine class and it is a DNA-methylating agent that has a good antitumor activity. Despite of dacarbazine, TMZ is spontaneously converted into its active metabolite 5-(3-methyltriazen-l-yl)imidazole-4-carboxamide at physiologic pH, so it is not required in enzymatic demethylation in the liver. TMZ is able to cross the blood brain barrier and is stable at gastric acid pH so it has almost 100% oral bioavailability and is rapidly absorbed after it is taken orally. TEMOZOLOMIDE IN CANCER PATIENTS: On the basis of the relatively safe toxicity and the findings achieved in adult malignant gliomas, phase I and II clinical trials were set up to evaluate the opportunity of using this novel drug in pediatric cancer, too. In this review, we evaluate the antitumor activity of TMZ against high-grade gliomas, low-grade-gliomas, and medulloblastoma/primitive neuroectodermal tumors analyzing several phases I and II clinical trials in children. CONCLUSIONS: In spite of the poor activity of TMZ against pediatric brain tumors, the use of the drug in combination with other compounds should be evaluated in phases I and II clinical trials. Moreover, the evaluation of the methylation status of the O6-methylguanine DNA methyltransferase promoter in glioblastoma biopsy specimens could be assayed as a predictive factor of TMZ efficacy.

Neurotrophic factor expression in childhood low-grade astrocytomas and ependymomas.

BACKGROUND: Neurotrophic factors (nerve growth factor [NGF], brain-derived neurotrophic factor [BDNF] and glial-derived neurotrophic factor [GDNF]) are growth factors implicated in the growth and differentiation of brain nerve cells. An involvement of these factors in the biology and progression of some specific tumours has been suggested. In accordance with the role of neurotrophic factors in tumour behaviour the aim of the present study was to investigate their expression in two childhood brain neoplasms, namely low-grade astrocytomas and ependymomas. MATERIALS AND METHODS: We investigated the NGF, BDNF, GDNF and NGF receptors (TrkA and p75) expression in the tumour tissues, cerebrospinal fluid (CSF) and plasma of ten children affected by low-grade astrocytomas and ependymomas. Control tissue samples (together with CSF and plasma samples) were obtained from patients who underwent surgery for cerebral vascular or epileptogenic lesions. RESULTS: The expression of NGF decreases both in tumour samples and in the CSF of affected children compared with controls. BDNF instead increases in CSF, while the expression of GDNF remains unchanged both in tissues and in CSF. No differences were found in neurotrophic factor plasma levels in patients or in controls. Gene expression of NGF and its high-affinity receptor (TrkA) are reduced in tumour tissues, whereas the number of cells immunopositive to the low-affinity NGF receptor (p75) is increased. CONCLUSION: Reduced expression of NGF and TrkA has been shown in low-grade astrocytomas and ependymomas. These findings may be related to the role of this neurotrophin in cell differentiation and apoptosis. The different expression of NGF, BDNF, and GDNF in low-grade astrocytomas and ependymomas suggests that a different degree of redundancy exists among members of the neurotrophic factor family and that their expression may be correlated with the biology and the behaviour of these tumours.

Dissecting giant vertebro-basilar aneurysms.

OBJECT: Vertebro-basilar dissecting aneurysms (VDAs) are very rare in children. Strokes or subarachnoid hemorrhage are characteristic clinical manifestations, but often only non-specific symptoms are found. Radiological diagnosis may be difficult to obtain and therapy hard to perform. We report on a child who presented with an intracranial mass effect due to a giant VDA. METHODS: The patient was a 8-year-old girl with right hemiparesis, ataxia, dysphagia and dysphonia and worsening of her neurological deficits. Neuroimaging discovered a giant dissecting aneurysm arising from the left vertebral artery and involving the vertebro-basilar junction. Endovascular treatment by coil embolization was performed. The post-operative course initially showed a remarkable improvement in her clinical condition. Afterwards, she developed cerebral hemorrhage and died. CONCLUSION: VDAs may have an unusual presentation and their therapy still remains a challenge. Endovascular embolization is a valid option but, as it is surgery, it may cause the death of the patient.