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Stagno V.,University of Naples Federico II | Navarrete E.A.,Hospital Universitario Reina Sofia | Mirone G.,Santobono Pausilipon Childrens Hospital | Esposito F.,University of Naples Federico II
World Neurosurgery | Year: 2013

Objective: The objective of this study is to review the management of hydrocephalus and, in particular, how different economic and social conditions affect its treatment around the world. Methods: The main techniques for the surgical treatment and the management of hydrocephalus have been retraced through a brief historical overview up to the present day. Results: During the last century, continuous technological progress has favored the diffusion of expensive and sophisticated shunt systems. On the other hand, the advent of minimally invasive endoscopic techniques, such as endoscopic third ventriculostomy, seems to represent a valid route for attempting a natural cerebrospinal fluid diversion, thus avoiding complications related to shunt failure. Conclusions: The management of hydrocephalus around the world is still widely debated. However, definition of proper indications and adequate training in neuroendoscopic techniques seem to represent the preeminent trend for new generations of neurosurgeons. © 2013 Elsevier Inc. All rights reserved.


PubMed | University of Padua, Santobono Pausilipon Childrens Hospital, University of Pavia, University of Bari and 9 more.
Type: Journal Article | Journal: The Lancet. Haematology | Year: 2016

Early T-cell precursor acute lymphoblastic leukaemia was recently recognised as a distinct leukaemia and reported as associated with poor outcomes. We aimed to assess the outcome of early T-cell precursor acute lymphoblastic leukaemia in patients from the Italian Association of Pediatric Hematology Oncology (AIEOP) centres treated with AIEOP-Berlin-Frankfurt-Mnster (AIEOP-BFM) protocols.In this retrospective analysis, we included all children aged from 1 to less than 18 years with early T-cell precursor acute lymphoblastic leukaemia immunophenotype diagnosed between Jan 1, 2008, and Oct 31, 2014, from AIEOP centres. Early T-cell precursors were defined as being CD1a and CD8 negative, CD5 weak positive or negative, and positive for at least one of the following antigens: CD34, CD117, HLADR, CD13, CD33, CD11b, or CD65. Treatment was based on AIEOP-BFM acute lymphoblastic leukaemia 2000 (NCT00613457) or AIEOP-BFM acute lymphoblastic leukaemia 2009 protocols (European Clinical Trials Database 2007-004270-43). The main differences in treatment and stratification of T-cell acute lymphoblastic leukaemia between the two protocols were that in the 2009 protocol only, pegylated L-asparaginase was substituted for Escherichia coli L-asparaginase, patients with prednisone poor response received an additional dose of cyclophosphamide at day 10 of phase IA, and high minimal residual disease at day 15 assessed by flow cytometry was used as a high-risk criterion. Outcomes were assessed in terms of event-free survival, disease-free survival, and overall survival.Early T-cell precursor acute lymphoblastic leukaemia was diagnosed in 49 patients. Compared with overall T-cell acute lymphoblastic leukaemia, it was associated with absence of molecular markers for PCR detection of minimal residual disease in 25 (56%) of 45 patients; prednisone poor response in 27 (55%) of 49 patients; high minimal residual disease at day 15 after starting therapy in 25 (64%) of 39 patients (bone marrow blasts 10%, by flow cytometry); no complete remission after phase IA in 7 (15%) of 46 patients (bone marrow blasts 5%, morphologically); and high PCR minimal residual disease ( 5 10(-4)) at day 33 after starting therapy in 17 (85%) of 20 patients with markers available. Overall, 38 (78%) of 49 patients are in continuous complete remission, including 13 of 18 after haemopoietic stem cell transplantation, with three deaths in induction, five deaths after haemopoietic stem cell transplantation, and three relapses. Severe adverse events in the 2009 study were reported in 10 (30%) of 33 patients with early T-cell precursor acute lymphoblastic leukaemia versus 24 (15%) of 164 patients without early T-cell precursor acute lymphoblastic leukaemia and life-threatening events in induction phase IA occurred in 4 (12%) of 33 patients with early T-cell precursor acute lymphoblastic leukaemia versus 7 (4%) of 164 patients without early T-cell precursor acute lymphoblastic leukaemia. No difference was seen in the subsequent consolidation phase IB of protocol I.Early T-cell precursor acute lymphoblastic leukaemia is characterised by poor early response to conventional induction treatment. Consolidation phase IB, based on cyclophosphamide, 6-mercaptopurine, and ara-C at conventional (non-high) doses is effective in reducing minimal residual disease. Although the number of patients and observational time are limited, patients with early T-cell precursor acute lymphoblastic leukaemia treated with current BFM stratification and treatment strategy have a favourable outcome compared with earlier reports. The role of innovative therapies and haemopoietic stem cell therapy in early T-cell precursor acute lymphoblastic leukaemia needs to be assessed.None.


PubMed | University of Padua, Santobono Pausilipon Childrens Hospital, University of Naples Federico II, Italian National Cancer Institute and University of Cologne
Type: Journal Article | Journal: Journal of translational medicine | Year: 2016

The prognosis of children with metastatic stage 4 neuroblastoma (NB) has remained poor in the past decade.Using microarray analyses of 342 primary tumors, we here developed and validated an easy to use gene expression-based risk score including 18 genes, which can robustly predict the outcome of stage 4 patients.This classifier was a significant predictor of overall survival in two independent validation cohorts [cohort 1 (n=214): P=6.310(-5); cohort 2 (n=27): P=3.110(-2)]. The prognostic value of the risk score was validated by multivariate analysis including the established markers age and MYCN status (P=0.027). In the pooled validation cohorts (n=241), integration of the risk score with the age and/or MYCN status identified subgroups with significantly differing overall survival (ranging from 35 to 100%).Together, the 18-gene risk score classifier can identify patients with stage 4 NB with favorable outcome and may therefore improve risk assessment and treatment stratification of NB patients with disseminated disease.


Terlizzi V.,University of Naples Federico II | Improta F.,University of Naples Federico II | Di Fraia T.,S Maria Delle Grazie Pozzuoli Hospital | Sanguigno E.,S Maria Delle Grazie Pozzuoli Hospital | And 4 more authors.
Journal of Clinical Neuroscience | Year: 2014

We describe, to our knowledge, the first case of arterial ischemic stroke after primary herpes simplex virus type 1 (HSV1) infection in a previously healthy child, without signs of encephalitis. A 10-year-old previously healthy girl was admitted to our hospital with acute left-sided hemiparesis which involved the lower half of her face. Submandibular lymphadenitis and oral vesicular lesions were present. MRI confirmed the suspicion of an acute ischemic stroke. Immunoglobulin M antibodies to HSV1 were detected. Cerebrospinal fluid polymerase chain reaction for herpes virus was negative. She was treated with aspirin (3 mg/kg) and intravenous acyclovir (10 mg/kg every 8 hours) for 21 days. Immunoglobulin G antibodies to HSV1 appeared 16 days after admission. Twelve months after her hospitalization the patient's examination was normal. Stroke should be considered a possible complication of HSV1 primary infection. Guidelines for the management of acute stroke in children are needed. © 2014 Elsevier Ltd. All rights reserved.


Havenith S.,University Utrecht | Lammers M.J.W.,University Utrecht | Tange R.A.,University Utrecht | Trabalzini F.,University of Siena | And 3 more authors.
Otology and Neurotology | Year: 2013

Objectives/Hypothesis: An increasing number of patients with low-frequency residual hearing are fitted with a cochlear implant. The challenge is to optimize cochlear implant device properties and develop atraumatic surgical techniques to preserve residual hearing. In view of the ongoing debate about the optimal procedure for opening the cochlea during cochlear implantation, we reviewed the evidence on the round window and the cochleostomy insertion techniques and compared their effects on postoperative residual hearing. Design: Systematic review. Methods: Electronic databases were systematically searched for relevant studies published up to January 2012. All studies reporting on residual hearing and hearing preservation surgery were included. Results: Sixteen studies, with a total of 170 patients, were included. There were no studies directly comparing both surgical insertion techniques. The methodologic quality of the studies was poor and might be subjected to a high risk of bias. Because there were no studies directly comparing the 2 techniques and controlling for possible influencing factors, differences between studies might also be influenced by intersurgeon variance in many facets regarding cochlear implantation surgery. The available data show a postoperative low-frequency hearing loss ranging from 10 to 30 dB at 125, 250, and 500 Hz, regardless of surgical technique. The number of patients with a postoperative complete hearing preservation ranged from 0% to 40% for the cochleostomy group and from 13% to 59% in the round window group. Conclusion: The available data do not show that there is a benefit of one surgical approach over the other regarding the preservation of residual hearing. To provide solid evidence, a double-blind randomized trial is needed, which compares the clinical outcomes, notably the degree of hearing preservation, of both surgical approaches. © 2013, Otology & Neurotology, Inc.


D'Ascanio L.,Citta Of Castello Civil Hospital | D'Ascanio L.,University of Rome La Sapienza | Lancione C.,Citta Of Castello Civil Hospital | Pompa G.,University of Rome La Sapienza | And 3 more authors.
International Journal of Pediatric Otorhinolaryngology | Year: 2010

Objective: Nasal-breathing impairment has been described as a possible determinant of maxillofacial development in children with adenoids/tonsils hypertrophy. However little is known about the possible influence of nasal septum deviation on craniofacial growth in childhood. We conducted a multicenter cephalometric study to compare skeletal and dental features in children with chronic nasal-breathing obstruction secondary to nasal septum deviation and nose-breathing controls. Methods: Ninety-eight children (59M, 39F; mean age 8.8 years; age range 7-12 years) with obligate mouth-breathing secondary to nasal septum deviation (group 1) and 98 age- and sex-matched nasal-breathing controls (group 2) were evaluated. Nasal-breathing function was assessed in all patients with clinical history, ENT instrumental examination and anterior active rhinomanometry. Cephalometric parameters were recorded in all subjects. Results: Patients of group 1 showed a statistically significant increase of upper anterior facial height (N-palatal plane) and total anterior facial height (N-Me) with regards to group 2. The angular relationships of the sella-nasion, palatal, and occlusal planes to the mandibular plane were greater in group 1 in comparison to controls. The gonial angle (Ar-Go-Me), palatal height and overjet were significantly higher in the mouth-breathing group. A significantly retrognatic position of the maxilla and mandible was recorded in group 1 in comparison to group 2. Most mouth-breathing children showed class II malocclusion, while the majority of control subjects presented normal occlusion. The prevalence of cross-bite was higher in group 1 with respect to controls (p= 0.02). Conclusion: Children with obligate mouth-breathing due to nasal septum deviations show facial and dental anomalies in comparison to nose-breathing controls. Possible physiologic explanations of our findings are reported. © 2010 Elsevier Ireland Ltd.


PubMed | San Carlo Hospital, Santobono Pausilipon Childrens Hospital and University of Naples Federico II
Type: Journal Article | Journal: Journal of ultrasound | Year: 2016

Over the past two decades, continuous ambulatory peritoneal dialysis has emerged as the first-choice dialysis modality in children awaiting for transplantation. Despite the improvements observed in catheter survival over the past several years, the obstruction is one of the reasons for immediate catheter non-function. This study assessed usefulness of ultrasound (US) in visualizing the obstruction of chronic peritoneal dialysis (CPD) catheter and identifying the etiology.Between January 2000 and November 2012, 38 patients (20 M, 18 F) were treated with CPD and examined with US. The type of catheter in all subjects was the straight two-cuff Tenckhoff catheter. We evaluated the typical sonographic signs of the most common CPD catheter complications.We had 12/38 cases with catheter malfunction due to its obstruction. In eight patients, US showed the presence of echogenic material fragmented into the lumen and around the distal tip of the catheter. In the other four patients, US demonstrated the displacement of catheter in bowel loops and an amorphous material as homogeneous echoic area around the distal tip.Our results confirm the effectiveness of US in the assessment of the complications related to intraperitoneal catheters. US is able to identify the catheter obstruction and recognize the etiology (endoluminal strands of fibrin, other materials or the omental wrapping), facilitating a correct therapeutic approach.


PubMed | Johannes Gutenberg University Mainz, Santobono Pausilipon Childrens Hospital and Saarland University
Type: | Journal: World neurosurgery | Year: 2016

Endoscopic third ventriculostomy (ETV) is a safe and less invasive treatment strategy for patients with obstructive hydrocephalus and provides excellent outcome. Nevertheless, repeat ETV in cases of ETV failure is a controversial issue.Between 1993 and 1999, 113 patients underwent a total of 126 ETVs at the Department of Neurosurgery, Mainz University Hospital. Obstructive hydrocephalus was the causative pathology in all cases. A very long term follow-up of up to 16 years could be achieved. All medical reports of patients who received ETV were reviewed and analyzed with focus on ETV failure with following repeat ETV and its initial as well as very long term success.Thirty-one events of ETV failure occurred during the follow-up period. Thirteen patients underwent repeat ETV: three patients during the first 3 months (early repeat ETV), the other 10 patients after 7 to 78 months (late repeat ETV, mean 33 months). All repeat ETV were performed without complications. Follow-up evaluation after successful repeat ETV ranged from < 1 month up to 14 years (mean 7 years). Of the three early revisions, two failed and one other patient died during follow-up while only two of the late repeat ETV failed. Very long term success rate of late repeat ETV up to 14 years yielded 80%.Repeat ETV in cases of late ETV failures represents an excellent option for CSF circulation restoration up to 14 years of follow-up. Repeat ETV in early ETV failure in contrast is not favored by the performing surgeons; and factors of ETV failure should be analyzed very carefully before a decision for repeat ETV is made.


PubMed | Santobono Pausilipon Childrens Hospital and Instituto Giannina Gaslini
Type: Journal Article | Journal: Italian journal of pediatrics | Year: 2016

Five to 10% of children with neuroblastoma present with symptoms of epidural compression (EC). More than half these patients are diagnosed in the first year of life. The case of a neuroblastoma presenting symptoms of EC at birth is exceptional and deserves to be reported.We describe a case of female born at the 36(th) week of pregnancy by caesarian section decided following ultrasonographic discovery of oligohydramnios. At birth, she was noted to have motor deficit involving both legs and continuous urinary dripping. These symptoms were found to be secondary to a paraspinal neuroblastoma infiltrating the spinal canal. Tumor responded well to chemotherapy, but neurologic deficit only slightly improved and bladder dysfunction remained unchanged. At 2years of age, patient is able to walk with help of leg orthoses, suffers chronic constipation requiring daily medications, and has neurologic bladder necessitating multiple daily catheterizations.The finding of a newborn presenting with symptoms of EC secondary to a neuroblastoma invading the spinal canal is quite uncommon. The case described herewith confirms that these rare patients have an excellent survival probability, but almost always develop severe functional sequelae.


PubMed | Santobono Pausilipon Childrens Hospital
Type: | Journal: Journal of neurosurgery. Pediatrics | Year: 2017

OBJECTIVE Neuroendoscopic removal of intraventricular tumors is difficult and time consuming because of the lack of an effective decompression system that can be used through the working channel of the endoscope. The authors report on the utilization of an endoscopic ultrasonic aspirator in the resection of intraventricular tumors. METHODS Twelve pediatric patients (10 male, 2 female), ages 1-15 years old, underwent surgery via a purely endoscopic approach using a Gaab rigid endoscope and endoscopic ultrasonic aspirator. Two patients presented with intraventricular metastases from high-grade tumors (medulloblastoma, atypical teratoid rhabdoid tumor), 2 with subependymal giant cell astrocytomas (associated with tuberous sclerosis), 2 with low-grade intraparaventricular tumors, 4 with suprasellar tumors (2 craniopharyngiomas and 2 optic pathway gliomas), and 2 with pineal tumors (1 immature teratoma, 1 pineal anlage tumor). Hydrocephalus was present in 5 cases. In all patients, the endoscopic trajectory and ventricular access were guided by electromagnetic neuronavigation. Nine patients underwent surgery via a precoronal bur hole while supine. In 2 cases, surgery was performed through a frontal bur hole at the level of the hairline. One patient underwent surgery via a posterior parietal approach to the trigone while in a lateral position. The endoscopic technique consisted of visualization of the tumor, ventricular washing to dilate the ventricles and to control bleeding, obtaining a tumor specimen with biopsy forceps, and ultrasonic aspiration of the tumor. Bleeding was controlled with irrigation, monopolar coagulation, and a thulium laser. RESULTS In 7 cases, the resection was total or near total (more than 90% of lesion removed). In 5 cases, the resection was partial. Histological evaluation of the collected material (withdrawn using biopsy forceps and aspirated with an ultrasonic aspirator) was diagnostic in all cases. The duration of surgery ranged from 30 to 120 minutes. One case was complicated by subdural hygroma requiring a subduro-peritoneal shunt implant. CONCLUSIONS In this preliminary series, endoscopic ultrasonic aspiration proved to be a safe and reliable method for achieving extensive decompression or complete removal in the management of intra- and/or paraventricular lesions in pediatric patients.

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