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Background: Primary central nervous system (CNS) neuroblastoma is a rare intracranial tumor affecting children mainly in the first years of life. It is usually a supratentorial tumor with a wide spectrum of clinical presentation, seizures, and focal neurological deficits being the most common presenting signs.Case description: A 2-year-old child was admitted to our ward after a generalized seizure. Neurological examination was normal. Radiological studies showed a small DWI hyperintense lesion of the right rectus gyrus. Follow-up brain MRI 8 months later showed a huge growth of the tumor (90 × 80 × 65 mm) with polycyclic and apparently defined margins, cystic components, and diffuse contrast enhancement. Complete tumor removal was performed in two planned surgical steps. Histological diagnosis was CNS neuroblastoma. At a follow-up of 8 months, the child is in good clinical and neurological condition and is completing chemotherapy treatment according to the SIOP PNET 4 protocol.Discussion and conclusion: A thorough review of the literature confirms that primary CNS neuroblastoma has to be considered a distinct entity. The disease related mortality is 12.5%, lower than the one usually reported for other previously described as PNETs tumors. The most relevant factors influencing prognosis are the possibility of obtaining a complete tumor removal and age more than 3 years, which allows to include radiotherapy among treatment options. [ABSTRACT FROM AUTHOR]

Purpose: This study aimed to explain the functional role of lambdoid arch sutures in the development of cerebellar tonsillar herniation. Posterior cranial fossa (PCF) changes were investigated in infants with premature synostosis of the major and minor sutures of the lambdoid arch without premature synostosis of the PCF synchondroses. Methods: Morphometric and volumetric PCF measurements were performed on preoperative high-resolution CT studies in 12 infants with multisutural craniosynostosis involving the lambdoid arch and compared with those of 12 age-matched healthy subjects. Results: All 12 patients had hypoplasia of PCF bone structures and normal volumes of the PCF and neural structures. PCF hypoplasia was related to exocciput length in infants with isolated involvement of major sutures, while it was related to posterior skull base hemifossae in infants with isolated involvement of minor lambdoid arch sutures. Foramen magnum AP diameter was reduced in babies with major suture involvement and tonsillar herniation, while foramen magnum AP and LL diameters were reduced in babies with minor suture involvement without tonsillar herniation. Right and left jugular foramen (JF) areas differed in all infants; however, the area of the smaller JF was significantly reduced only in infants with involvement of minor lambdoid arch sutures. Conclusion: Hypoplasia of PCF bone structures due to sutural synostosis of the lambdoid arch is a required predisposing but not sufficient factor for the development of cerebellar tonsillar herniation through the foramen magnum. Normal PCF volume and foramen magnum anatomy may partly explain the development of cerebellar tonsil herniation in infants with lambdoid arch synostosis. [ABSTRACT FROM AUTHOR]

Paediatric optic pathway gliomas are low-grade brain tumours characterized by slow progression and invalidating visual loss. Presently there is no strategy to prevent visual loss in this kind of tumour. This study evaluated the effects of nerve growth factor administration in protecting visual function in patients with optic pathway glioma-related visual impairment. A prospective randomized double-blind phase II clinical trial was conducted in 18 optic pathway glioma patients, aged from 2 to 23 years, with stable disease and severe visual loss. Ten patients were randomly assigned to receive a single 10-day course of 0.5 mg murine nerve growth factor as eye drops, while eight patients received placebo. All patients were evaluated before and after treatment, testing visual acuity, visual field, visual-evoked potentials, optic coherence tomography, electroretinographic photopic negative response, and magnetic resonance imaging. Post-treatment evaluations were repeated at 15, 30, 90, and 180 days Brain magnetic resonance imaging was performed at baseline and at 180 days. Treatment with nerve growth factor led to statistically significant improvements in objective electrophysiological parameters (electroretinographic photopic negative response amplitude at 180 days and visual-evoked potentials at 30 days), which were not observed in placebo-treated patients. Furthermore, in patients in whom visual fields could still be measured, visual field worsening was only observed in placebo-treated cases, while three of four nerve growth factor-treated subjects showed significant visual field enlargement. This corresponded to improved visually guided behaviour, as reported by the patients and/or the caregivers. There was no evidence of side effects related to nerve growth factor treatment. Nerve growth factor eye drop administration appears a safe, easy and effective strategy for the treatment of visual loss associated with optic pathway gliomas. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Shunt malfunction represents one of the most common problem to deal with in the daily neurosurgical practice. Among the variety of possible presentations, the cyst-like dilatation of the brain around the proximal catheter is one of the most rare and misleading type. The case of a boy with a ‘tumour-like’ proximal shunt malfunction is described and the pertinent literature is analysed. [ABSTRACT FROM PUBLISHER]

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%. [ABSTRACT FROM AUTHOR]

Surgical excision combined with radio-chemotherapy represents the gold standard of therapy of medulloblastoma. The effectiveness of such a combined treatment has encouraged the use of radiotherapy even in young paediatric patients, in spite of the many adverse effects reported in literature, and, in particular, the increased risk of a second radioinduced malignancy. Irradiation is the well-known risk factor for development of benign and malignant thyroid tumours. Children are more exposed to this risk because of their thyroid gland is more sensitive to carcinogenic effect of ionising radiation. Two children underwent radiotherapy for the treatment of a medulloblastoma when they were 3 and 4 years old, respectively. At the age of 20 and 23, both of them underwent the surgical excision of a papillary thyroid carcinoma, 20 and 17 years after the radiotherapeutic treatment, respectively. Radioinduced thyroid tumours are a well-recognised nosographic entities due to the particular sensitivity of this gland to ionising radiations. However, only a few papers on radioinduced thyroid neoplasms after CNS irradiation have been published in the literature. We report on two additional cases of thyroid neoplasms following childhood CNS irradiation for the treatment of a posterior fossa medulloblastoma. [ABSTRACT FROM AUTHOR]

Different treatment options have been proposed for aneurysmal bone cysts (ABCs) with sclerotherapy favored as primary treatment and surgery remaining the mainstay of treatment in case of compression of neural structures. Recurrent spinal ABCs are burdened by increased risk of spinal deformity and instability, further complicating the management of these cases. A 15-year-old boy presented with acute symptoms and signs of spinal cord compression due to a large thoracic ABC. Subtotal resection of the lesion achieved optimal decompression of neural structures with good neurologic recovery, but the remnant of the lesion rapidly grew with recurrent spinal cord compression after 40 days. The patient underwent total surgical resection with full neurologic recovery. Unfortunately, recurrence of the lesion was documented at 3-months' follow-up. This was successfully treated with percutaneous injection of hydroxyapatite cement. Two years' follow-up ruled out any further recurrence of the lesion. Furthermore, spinal deformity and instability were also excluded. Percutaneous sclerotherapy with hydroxyapatite cement proved to be highly effective and safe in the treatment of spinal ABC, though surgery remains mandatory in case of spinal cord compression. The main advantage of sclerotherapy with hydroxyapatite cement seems to be the capacity to regenerate bone with normal radiologic features. [ABSTRACT FROM AUTHOR]

Abstract: 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. [Copyright &y& Elsevier]