Paola Peretta

Ospedale Infantile Regina Margherita Sant Anna, Torino, Piedmont, Italy

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Publications (19)38.56 Total impact

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    ABSTRACT: Background The management of children with cancer during the end-of-life (EOL) period is often difficult and requires skilled medical professionals. Patients with tumors of the central nervous system (CNS) with relapse or disease progression might have additional needs because of the presence of unique issues, such as neurological impairment and altered consciousness. Very few reports specifically concerning the EOL period in pediatric neuro-oncology are available.ProcedureAmong all patients followed at our center during the EOL, we retrospectively analyzed data from 39 children and adolescents with brain tumors, in order to point out on their peculiar needs.ResultsPatients were followed-up for a median time of 20.1 months. Eighty-two percent were receiving only palliative therapy before death. Almost half the patients (44%) died at home, while 56% died in a hospital. Palliative sedation with midazolam was performed in 58% of cases; morphine was administered in 51.6% of cases. No patient had uncontrolled pain.Conclusions The EOL in children with advanced CNS cancer is a period of active medical care. Patients may develop complex neurological symptoms and often require long hospitalization. We organized a network-based collaboration among the reference pediatric oncology center, other pediatric hospitals and domiciliary care personnel, with the aim to ameliorate the quality of care during the EOL period. In our cohort, palliative sedation was widely used while no patients died with uncontrolled pain. A precise process of data collection and a better sharing of knowledge are necessary in order to improve the management of such patients. Pediatr Blood Cancer © 2014 Wiley Periodicals, Inc.
    Pediatric Blood & Cancer 08/2014; · 2.35 Impact Factor
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    ABSTRACT: The craniopharyngioma is a benign intracranial nonglial tumor derived from a malformation of the embryonic tissue. Represents approximately 6-9% of brain tumors in children. It grows close to the optic nerve, hypothalamus and pituitary. The most frequent histological variety in children is adamantinomatous. The initial symptoms of intracranial hypertension is headache and nausea, followed by visual disturbances, impaired hormonal changes such as the secretion of GH, gonadotropins, TSH and ACTH and central diabetes insipidus. We present the clinical case of MD, 5yrs at age, which shows signs of intracranial hypertension syndrome: neuroradiological findings raise the diagnosis of adamantinomatous craniopharyngioma for which the child underwent to sub-total surgical removal of the lesion and radiosurgery treatment. During the disease develops visual impairment, and secondary diabetes insipidus, hypothyroidism hipocotisolism that takes therapy with desmopressin (Minirin), Cortone acetate and L-tiroxine. For the failure of previous therapies, the child has performed chemotherapy with cisplatin (30 mg/sqm/day) and Etoposide (150 mg/mq/day). A year after the end of the last cycle of chemotherapy was detected new progression of the lesion with the appearance of worsening headache and vomiting in the upright position. TC notes the expansion of the third ventricle and the patient undergoes surgery craniotomy. This clinical case underlines the difficulties in treatment of recurrent craniopharyngioma in situations where the anatomical location do not permit aggressive radical surgery. Anyway, new studies are needed to evaluate the effectiveness of systemic chemotherapy as a method of experimental treatment that could reduce the progression of disease.
    Minerva pediatrica 12/2013; 65(6):673-6. · 0.72 Impact Factor
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    ABSTRACT: This prospective surveillance study was designed to estimate the incidence of healthcare-associated infections (HAIs) and to analyze the risk factors for their development in a pediatric neurosurgical unit. The study was performed in an Italian teaching hospital from October 2008 through March 2010. All children (0-18 years) undergoing neurosurgery were included and monitored daily for the development of HAIs. The study included 260 patients, with a mean age of 4.3 ± 4.7 years. Thirty-six HAIs were detected in 25 patients; catheter-related infections were the most frequent. Etiological identification was available in 22 cases; Gram-negative bacteria were the most commonly isolated pathogens. The incidence density was 11.0/1,000 patient days, and the incidence rate was 13.8/100 patients. The crude mortality was 0%. The risk of developing HAIs was related to the length of hospital stay, while the higher the age of the patients, the lower the risk of developing HAIs. To our knowledge, this survey is the first study to evaluate the overall incidence of HAIs and to explore the risk factors implicated in their development in neurosurgical pediatric patients. The most effective strategies to prevent these infections are reduction of the length of the hospital stay and improvement in device management.
    Pediatric Neurosurgery 02/2012; 47(4):261-5. · 0.50 Impact Factor
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    ABSTRACT: 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. 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. 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. 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.
    International journal of radiation oncology, biology, physics 07/2011; 80(3):807-14. · 4.59 Impact Factor
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    ABSTRACT: To evaluate retrospectively the operative findings and long-term results of a repeat endoscopic third ventriculostomy (ETV) in pediatric hydrocephalic patients readmitted after the first procedure with symptoms and/or signs of intracranial hypertension and/or radiological evidence of increased ventricular dilation and/or occluded stoma on follow-up radiological examinations. We analyzed a series of 482 ETVs in pediatric patients from 2 Italian departments of pediatric neurosurgery. The clinical charts of 40 patients undergoing a second ETV were selected and reviewed retrospectively. The pre- and postoperative radiological findings and operative films were analyzed retrospectively. Forty patients underwent a total of 82 ETVs. Thirty-eight patients were operated on twice and 2 were operated on 3 times. During the second procedure, the stoma was found to be closed in 28 patients without underlying adhesions, to be open but with significant arachnoid adhesions in the prepontine cistern in 8 patients, to be open without adhesions in 2 patients, to have a pinhole orifice in 1 patient, and to be closed with underlying adhesions in 1 patient. The second procedure allowed reopening of the stoma or lysis of the arachnoid adhesions in 35 patients and was abandoned in 3 patients because of extensive arachnoid adhesions or because the stoma was found to be wide open (2 patients). In 30 patients (75%), the second ETV was effective, and the 2 patients who underwent a third ETV remained shunt free. In 10 patients (25%), a ventriculoperitoneal shunt was eventually placed. Age younger than 2 years at the time of the first procedure and arachnoid adhesions in the subarachnoid cisterns observed during the second procedure are the main negative prognostic factors for the success of a second ETV. A second ETV can be performed with a reasonable chance of restoring patency of the stoma and avoiding placement of an extrathecal shunt. Every effort should be made to detect subarachnoid adhesions in the cistern on preoperative imaging study to select potential candidates and avoid unnecessary procedures.
    Neurosurgery 10/2009; 65(3):539-47; discussion 547. · 3.03 Impact Factor
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    ABSTRACT: We present the case of a 6-year-old male affected by an infratentorial tumor. Histological diagnosis was melanotic medulloblastoma. Immunohistochemistry showed in the melanin rich areas positive cells for HMB45. We performed a proteomic study to compare protein profiles in melanotic versus non-melanotic areas. Protein profiles of different areas of the tumor displayed similarity, with the exception of seven proteins. In accordance with the hypothesis that melanotic medulloblastomas produce oculo-cutaneous melanin, proteomic analysis showed melanocytic-associated antigens and epidermal autoantigen 450K in the pigmented nodule; both these proteins have a significant role as markers of melanotic elements.
    Pediatric Blood & Cancer 07/2009; 52(7):875-7. · 2.35 Impact Factor
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    ABSTRACT: Aneurysms in children are rare.We describe a large spontaneous dissecting aneurysm of the posterior cerebral artery. The clinical presentation was characterized by headache as the sole symptom due to a mass effect leading to hydrocephalus. Acute treatment with a temporary ventricular shunt was followed by occlusion of the aneurysm via an endovascular approach leading to a complete recovery of the patient.
    Interventional Neuroradiology 09/2008; 14(3):325-30. · 0.73 Impact Factor
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    ABSTRACT: We report on an Italian boy, born to normal and nonconsanguineous parents with a prenatal diagnosis of ventriculomegaly and subependymal glial heterotopias. At birth bilateral macrostomia was diagnosed without other evident facial anomalies. Magnetic resonance imaging (MRI) showed triventricular hydrocephalus and aqueductal stenosis and confirmed the nodules of glial heterotopia. The bilateral macrostomia was surgically corrected with the vermilion square flap method and W-plasty technique and follow up MRI at 6 months showed mild increase of ventricular dilatation without signs of active hydrocephalus. The association between macrostomia and hydrocephalus has been reported only in rare cases of complex malformative syndromes but never with isolated macrostomia.
    The Cleft Palate-Craniofacial Journal 10/2007; 44(5):558-61. · 1.24 Impact Factor
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    ABSTRACT: The aim of this study is to retrospectively evaluate a series of consecutive patients affected by post-hemorrhagic hydrocephalus in prematurity, treated with an implant of an Ommaya reservoir followed by ventriculo-peritoneal (VP) shunt and/or endoscopic third ventriculostomy (ETV) to evaluate the safety and efficacy of these treatment options in the management of the condition. Between 2002 and 2005, 18 consecutive premature patients affected by intra-ventricular haemorrhage (IVH) grades II to IV, presenting with progressive ventricular dilatation, were operated for implant of an intra-ventricular catheter connected to a sub-cutaneous Ommaya reservoir. Cerebrospinal fluid was intermittently aspirated percutaneously by the reservoir according with the clinical requirements and the echographic follow-up. The patients who presented a progression of the ventricular dilatation were finally operated for VP shunt implant or ETV according with the MRI findings. One patient had grade II, 5 had grade III, and 12 had grade IV IVH. The mean age at IVH diagnosis was 5.2 days; the mean age at reservoir implant was 17.3 days. The Ommaya reservoir was punctured on an average basis of 11.4 times per patient (range 2-25), and the mean interval between aspirations was 2.7 days. The mean CSF volume per tap was 20 ml. One patient died for pulmonary complications during the study period. Out of the 17 survivors, 3 did not develop progressive ventricular dilatation, and their reservoir was removed; 14 developed progressive hydrocephalus, 5 of whom were implanted with a VP shunt and 9 received an ETV. Amongst the five shunted patients, two were re-admitted for shunt malfunction and had their shunt removed after ETV after 6.1 and 20.5 months, respectively. Amongst the nine patients who received an ETV, five had to be re-operated for VP shunt implant at an average interval of 2.17 months (range 9-172 days) because of increasing ventricular dilatation. Two of them had a redo third ventriculostomy with shunt removal at 11 and 25.1 months, respectively, after insertion. The first was reimplanted with a VP shunt 4 days later; the second remains shunt free. Therefore, at the end of the follow-up period, 10 out of 17 children affected by post-hemorrhagic hydrocephalus in prematurity were shunt free (59%). The combination of Ommaya reservoir, VP shunt, and the aggressive use of ETV as a primary treatment or as an alternative to shunt revision allowed for a significant reduction of shunt dependency in a traditionally shunt-dependent population. Further studies are warranted to optimise the algorithm of treatment in these patients.
    Child s Nervous System 08/2007; 23(7):765-71. · 1.16 Impact Factor
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    ABSTRACT: The authors describe the clinical and radiological findings in a case of brainstem arteriovenous malformation (AVM) associated with a hepatic patent ductus venosus (PDV) in a 12-year-old child. The AVM was discovered on magnetic resonance (MR) imaging performed because of slight mental retardation and headache. The malformation was otherwise asymptomatic and no treatment was proposed. An abdominal ultrasonography study performed 1 year later because of hyperammonemia revealed a PDV, which was surgically ligated. One year later, MR images and angiograms showed complete resolution of the brainstem AVM. This report is the first documentation of an association between these two entities in the same patient, and the possible pathophysiological interactions between them are discussed.
    Journal of Neurosurgery 03/2007; 106(2 Suppl):147-52. · 3.23 Impact Factor
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    ABSTRACT: The aim of this study is to report on the role of neuroendoscopy during the management of hydrocephalus that led to the diagnosis of intracranial tumoral dissemination and the subsequent finding of a spinal cord glioma. We present two children each with an intramedullary astrocytoma that presented initially with hydrocephalus without spinal cord symptoms. In both cases leptomeningeal gliomatous dissemination was asserted during routine endoscopy for the management of hydrocephalus. The diagnosis of a cervical and a lower thoracic intramedullary tumor was made soon after on magnetic resonance imaging. Spinal cord MRI with contrast should be considered initially in selected cases of hydrocephalus without evident diagnosis. The intraoperative diagnosis of gliomatous dissemination and secondary hydrocephalus due to unrecognized spinal cord gliomas was possible, in our experience, with the routine use of the neuroendoscope.
    min - Minimally Invasive Neurosurgery 01/2007; 49(6):347-52. · 1.14 Impact Factor
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    ABSTRACT: Neuroendoscopic surgery is being used as an alternative to traditional shunt surgery and craniotomy in the management of hydrocephalus and intracranial fluid-filled cavities. In this study, the authors evaluated the incidence and type of complications occurring after neuroendoscopic procedures that were performed in a consecutive series of pediatric patients at a single institution to determine the effectiveness of neuroendoscopy in such patients. Four hundred ninety-five neuroendoscopic procedures were consecutively performed in 450 pediatric patients at one institution over a 10-year period. Charts were retrospectively reviewed. A complication was defined as follows: 1) any postoperative neurological deficit that was not observed before surgery; 2) any event occurring during surgery that resulted in the procedure being aborted; or 3) any adverse event occurring within 7 days postsurgery that resulted in a modification of the normal postoperative care. However, headache, vomiting, and fever without cerebrospinal fluid (CSF) pleocytosis were not considered complications. Complications were observed in 40 (8.1%) of 495 procedures. Two patients had two complications. One patient died of diffuse brain edema following endoscopic biopsy sampling of a basal ganglia tumor (mortality rate 0.2%). Other complications observed were abandonment of the procedure in eight cases, CSF leakage in 11 (with associated wound infection in one), intraventricular hemorrhage in six (with external drainage needed in four), intraparenchymal hemorrhage in three, subdural collection in eight (with subdural-peritoneal shunt placement needed in seven), transient oculomotor palsy in two, and transient hemiparesis in one. Many complications can be avoided by determining the correct diagnosis and using suitable techniques and instruments. Most complications can be managed conservatively and do not produce long-term morbidity. Complex procedures in most patients and simple procedures in patients with preoperative risk factors carry the highest hazard. Every attempt should be made to optimize the surgical technique. The most serious and potentially the most lethal complication remains arterial bleeding from injury to the basilar artery complex.
    Journal of Neurosurgery 10/2006; 105(3 Suppl):187-93. · 3.23 Impact Factor
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    ABSTRACT: Brainstem tumors (BSTs) are usually gliomas and are divided into diffuse BSTs (DBSTs) and focal BSTs (FBSTs). The aim of this study is to investigate the different outcomes of these two entities. Thirty-one patients with BSTs were admitted to our institution from 1995 to 2003. Patients with DBSTs were treated with locoregional radiotherapy (1.8 Gy/day for 54 Gy) and weekly vincristine for radiosensitization (1.5 mg/sm for six total doses). Patients with FBSTs underwent surgical resection. Chemotherapy and/or radiotherapy were considered in progression. Fourteen patients were diagnosed as having DBSTs. The responses to treatment were ten cases of partial response, three of stable disease, and one of progressive disease. General and/or neurological symptoms improved in more than 80% of patients. The median time from diagnosis to progression and to death were, nonetheless, 8 (range of 3-13) and 13 (range of 4-25) months, respectively, with a 2-year overall survival rate of 12.3% [standard error (SE) 11.2]. Seventeen patients were diagnosed as having FBSTs. Gross total removal was achieved in 4/17 cases, subtotal removal in 7/17, and partial removal in 6/17. There was one surgery-related death. Eight out of 17 patients had adjuvant chemo- and/or radiotherapy after progression: 6/8 are without neurological symptoms and 2/8 have died due to tumor progression. The 4-year overall and disease-free survival rates are 87.4 (SE 8.4) and 58.8% (SE 11.9), respectively, the extent of resection being the most important prognostic factor (p=0.012). DBSTs continue to carry a dismal prognosis, thus demanding new treatment modalities; FBSTs can be treated surgically and patients benefit from a better prognosis.
    Child s Nervous System 10/2006; 22(9):1127-35. · 1.16 Impact Factor
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    ABSTRACT: Interhemispheric arachnoid cysts are very rare, and they are often associated with complex brain malformations such as corpus callosum agenesis and hydrocephalus. Debate remains concerning the proper management of these lesions. Placement of shunts and microsurgical marsupialization of the cyst are the traditional options. Using endoscopic methods to create areas of communication between the cyst, the ventricular system, and/or the subarachnoid space is an attractive alternative to the use of shunts and microsurgery. Between 2000 and 2005, seven consecutive pediatric patients with interhemispheric arachnoid cysts underwent neuroendoscopic treatment involving cystoventriculostomy in two patients, cystocisternostomy in two, and cystoventriculocisternostomy in three. There were three cases of associated hydrocephalus, six cases of corpus callosum agenesis, and one case of corpus callosum hypogenesis. The follow-up period ranged from 12 to 49 months (mean 31.6 months). Endoscopic procedures were completely successful in all but two patients. In one of the remaining two patients, a repeated endoscopic cystocisternostomy was performed with success because of closure of the previous stoma. In the other, a subcutaneous collection of cerebrospinal fluid (CSF) was managed by insertion of an lumboperitoneal shunt. A subdural collection of CSF developed in three patients; it was treated with insertion of a subduroperitoneal shunt in one patient and managed conservatively in the other two patients, resolving spontaneously without further treatment. Neurodevelopmental evaluation performed in six patients showed normal intelligence (total intelligence quotient [IQ] > 80) in three patients, mild developmental delay (total IQ 50-80) in two, and severe developmental delay (total IQ < 50) in one. Endoscopic treatment of interhemispheric cysts can be considered a useful alternative to traditional treatments, even if some complications are to be expected, such as subdural or subcutaneous CSF collections and CSF leaks due to thinness of cerebral mantle and to the often-associated multifactorial hydrocephalus.
    Journal of Neurosurgery 09/2006; 105(3 Suppl):194-202. · 3.23 Impact Factor
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    ABSTRACT: To discuss the results obtained by giving adjuvant treatment for childhood ependymoma (EPD) at relapse after complete surgery only. Between 1993 and 2002, 63 children older than 3 years old entered the first Italian Association for Pediatric Hematology and Oncology protocol for EPD (group A), and another 14 patients were referred after relapsing after more tumor excisions only (group B). Prognostic factors were homogeneously matched in the two groups. We report on the outcome of group B. Mean time to first local progression in group B had been 14 months. Tumors originated in the posterior fossa (PF) in 10 children and were supratentorial (ST) in 4; 11 had first been completely excised (NED) and 3 had residual disease (ED). Diagnoses were classic EPD in 9 patients, anaplastic in 5. Eight children were referred NED and 6 ED after two or more operations, 5 had cranial nerve palsy, 1 had recurrent meningitis, and 2 had persistent hydrocephalus. All received radiotherapy (RT) to tumor bed and 5 also had pre-RT chemotherapy. Six of 14 patients (6/10 with PF tumors) had a further relapse a mean 6 months after the last surgery; 4 of 6 died: progression-free survival and overall survival at 4 years after referral were 54.4% and 77%, respectively. Considering only PF tumors and setting time 0 as at the last surgery for group B, progression-free survival and overall survival were 32% and 50% for group B and 52% (p < 0.20)/70% (p < 0.29) for the 46 patients in group A with PF tumors. Local control was 32% in group B and 70.5% in group A (p = 0.02). Relapsers after surgery only, especially if with PF-EPD, do worse than those treated after first diagnosis; subsequent surgery for tumor relapse has severe neurologic sequelae.
    International Journal of Radiation OncologyBiologyPhysics 09/2006; 65(5):1440-5. · 4.18 Impact Factor
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    ABSTRACT: With two study protocols, one retrospective and the other prospective, we evaluated hypothalamo-hypophysial dysfunction (HHD) in paediatric patients treated for traumatic brain injury (TBI) in the neurosurgical or intensive care department at our hospital. The retrospective group comprised 22 patients who had experienced TBI 0.7-7.25 years before the study. The prospective group included 30 patients assessed at TBI (T0), 26 of 30 after 6 months (T6), and 20 of 26 after 12 months (T12). Auxological and hormonal basal parameters of hypothalamo-hypophysial function were evaluated at recall in the retrospective group, and at T0, T6 and T12 in the prospective group. Basal data and standard dynamic tests in selected patients revealed one with precocious puberty, one with total anterior hypopituitarism, one with central hypogonadism, and one with growth hormone (GH) deficiency in the retrospective group; three patients with cerebral salt-wasting syndrome, one with diabetes insipidus and seven with low T3 syndrome at T0 (all transient), one with hypocorticism at T6 confirmed at T12, and one with GH deficiency at T12 in the prospective group. The results of our study show that post-TBI HHD in our paediatric cohort is not uncommon. Of the 48 patients who underwent a complete evaluation (22 retrospective study patients and 26 prospective study patients evaluated at T6) five (10.4%) developed HHD 6 months or more after TBI. HHD was newly diagnosed in one previously normal patient from the prospective group at 12 months after TBI. GH deficiency was the most frequent disorder in our paediatric cohort.
    Journal of pediatric endocrinology & metabolism: JPEM 06/2006; 19(5):691-703. · 0.71 Impact Factor
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    ABSTRACT: Invasive aspergillosis is an uncommon but often lethal complication in immunocompromised patients. Despite the progress obtained with new antifungal drugs, intracranial aspergillosis often requires a combined medical and surgical approach. Most cases previously reported in immunocompromised children were fatal. We describe 4 immunosuppressed children with intracranial aspergillosis successfully treated with surgery and antifungal long-term therapy.
    The Pediatric Infectious Disease Journal 05/2006; 25(4):379-81. · 3.14 Impact Factor
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    ABSTRACT: The authors studied the role of the sole posterior fossa bony decompression in the management of symptomatic children affected by Chiari type I anomalies. The series in the pediatric literature of this subject were reviewed and compared with that presented in this article. From May 1994 to December 1998, 53 patients (3 months to 26 years) were observed. They were divided into: asymptomatic patients (27), who received no surgical treatment and were only subject to clinical observation; symptomatic patients (brain stem compression 16, syringomyelia 10, including 7 with holocord). All the symptomatic patients were treated with the same surgical approach: bony decompression of posterior fossa with removal of the posterior arch of C-1 and the outer layer of the dura without dural opening. In all 16 (100%) of the 16 patients with brain stem compression the symptoms resolved or improved; in patients with syringomyelia the symptoms were resolved or improved in 94.4% of cases. Two children required further surgery after 13 and 24 months, respectively. This series seems to demonstrate that even a simple extradural surgical approach, with a lower rate of postoperative complications and short stay in hospital, is sufficient to arrest the disease and to improve the symptomatology in a high percentage of cases (97.2%), which is comparable to that achieved with other, more aggressive, procedures.
    Child s Nervous System 12/2000; 16(10-11):707-18. · 1.16 Impact Factor
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    ABSTRACT: A boy is described with early puberty and Chiari 1 malformation. It is not known whether there was an etiological relationship between these two conditions, or whether they were coincidental. This report emphasizes the utility of MRI when early puberty is diagnosed, particularly in males.
    Journal of pediatric endocrinology & metabolism: JPEM 08/2000; 13 Suppl 1:849-51. · 0.71 Impact Factor