Brachytherapy in paediatric craniopharyngiomas: a systematic review and meta-analysis of recent literature.

PURPOSES: Brachytherapy has been indicated as an alternative option for treating cystic craniopharyngiomas. Despite the difficulties regarding the complex nuclear infrastructure and consequent small number of brachytherapy studies, recent discoveries relating tumour characteristics to sensitivity to brachytherapy have stimulated this extensive systematic review and meta-analysis of the recent results aiming to summarise its efficacy and complications. METHODS: The systematic review was structured using PRISMA statements. The MEDLINE database was systematically reviewed from March 2010 to February 2021 to identify qualified trials dealing with radioisotope brachytherapy in cystic craniopharyngiomas in the paediatric population, emphasising tumour control rates and complications. RESULTS: A total of 228 individuals were analysed, of which 66 were children. The minimum average follow-up was 5 years. Considering the paediatric trials, partial and complete responses were achieved in 89% of patients with exclusively cystic lesions, compared to 58% in non-exclusively cystic lesions. The former observed progression in 3% of patients, while in the latter, 35% was reported. Visual and endocrine improvement reached 64% and 20%, respectively, in the first group, in contrast to 48% and 7% in the second group, respectively. There were similar results considering the non-exclusive paediatric series, but with less expressive numbers. CONCLUSION: These results reinforce the positive impact of radioisotope brachytherapy in the treatment of predominant monocystic or multicystic craniopharyngiomas, especially in the paediatric population, grounded by impressive tumour control rates, lower morbidities and single application in the majority of the cases. In the future, specific morphological tumour characteristics might be considered for a more assertive patient selection.

Extraneural metastases in medulloblastoma / Mestástases extraneurais em moduloblastoma

Medulloblastoma is the most common childhood malignant tumor of central nervous system, but it may also occur in adults. It presents high invasive growth with spreading of tumor cells into the leptomeningeal space along the neuroaxis early in the course of the disease. Extraneural metastases are rare but frequently lethal, occurring only in 1 to 5 percent of patients, and are related, in the most of cases, to the presence of ventriculoperitoneal shunt. Here we characterize the clinical profile of five cases of medulloblastoma with systemic spreading of tumor cells, also comparing them to cases already described in the literature.

O meduloblastoma é o tumor maligno mais frequente do sistema nervoso central na infância, mas também pode ocorrer em adultos. Ele apresenta crescimento altamente invasivo com disseminação de células tumorais ao longo do neuroeixo precocemente no curso da doença. Metástases extraneurais são raras mas frequentemente letais, ocorrendo apenas em 1 a 5 por cento dos pacientes, e estão relacionadas, na maioria dos casos, a presença de derivação ventriculperitoneal. Neste artigo ,apresentamos o perfil de cinco casos de meduloblastoma com disseminção sistêmica das células tumorais, comparando-os com os casos já descritos na literatura.

[Intracranial pressure monitoring in children with fulminant hepatic failure]. / Monitorizacion de la presion intracraneal en ninos con insuficiencia hepatica fulminante.

INTRODUCTION: We describe a series of 10 children with intracranial hypertension complicating fulminant hepatic failure submitted to intracranial pressure (ICP) monitoring for intensive care and transplantation management. PATIENTS AND METHODS: Information from pediatrics patients acute liver failure admitted to our hospital was collected in a standard protocol form. We analyzed data from 10 patients, medium age 5.2 years old. In this period we studied aspects as ICP transducer used, number of days with ICP monitoring and complications of ICP monitoring. RESULTS: Hepatitis A was diagnosed in five patients and hepatitis B in two cases. The initial ICP were 2 to 24 mmHg in transducer. Seven patients died, four due to intracranial hypertension, included the patient operated for subdural hematoma, and three with transplantation failure. Only a case of hematoma was verified. CONCLUSIONS: The application of ICP monitoring allows intensive care for aggressive ICP management. It can be used in children without adaptations.

Monitorización de la presión intracraneal en niños con insuficiencia hepática fulminante / Intracranial pressure monitoring in chindren with fulminant hepatic failure

Introducción. Se describe una serie de 10 niños con hipertensión intracraneal como complicación de una insuficiencia hepática fulminante sometidos a una monitorización de la presión intracraneal (PIC) para facilitar la gestión de los cuidados intensivos y del trasplante. Pacientes y métodos. Se recopiló información sobre pacientes pediátricos con insuficienciahepática aguda que ingresaron en nuestro hospital en un formulario estándar de protocolo. Se analizaron los datos de 10 pacientes cuya edad media era de 5,2 años. En este período, se estudiaron aspectos como los transductores de PIC utilizados, el número de días con monitorización de la PIC y las complicaciones de la monitorización de la PIC. Resultados. Se diagnosticóhepatitis A en cinco pacientes y hepatitis B en dos casos. La PIC inicial osciló entre 2 y 24 mmHg en el transductor. Fallecieron siete pacientes, cuatro debido a la hipertensión intracraneal, incluido el paciente operado por hematoma subdural, y tres por fracaso del trasplante. Sólo se verificó un caso de hematoma. Conclusiones. La monitorización de la PIC permite laaplicación de cuidados intensivos para el tratamiento agresivo de la PIC. Se puede utilizar en niños sin necesidad de adaptarla

Introduction. We describe a series of 10 children with intracranial hypertension complicating fulminant hepaticfailure submitted to intracranial pressure (ICP) monitoring for intensive care and transplantation management. Patients and methods. Information from pediatrics patients acute liver failure admitted to our hospital was collected in a standard protocolform. We analyzed data from 10 patients, medium age 5.2 years old. In this period we studied aspects as ICP transducer used, number of days with ICP monitoring and complications of ICP monitoring. Results. Hepatitis A was diagnosed in five patients and hepatitis B in two cases. The initial ICP were 2 to 24 mmHg in transducer. Seven patients died, four due to intracranialhypertension, included the patient operated for subdural hematoma, and three with transplantation failure. Only a case of hematoma was verified. Conclusions. The application of ICP monitoring allows intensive care for aggressive ICP management. It can be used in children without adaptations

[Infections of cerebrospinal fluid shunts in children. Review of 100 infections in 87 children]. / Infecções de derivações liquóricas em crianças. Revisão de 100 infecções em 87 crianças.

An analysis of 100 infections in 87 children treated with shunts in the period of 1982 to 1995 is reported. The clinical presentation has been more frequently secondary to inflammatory signals. Staphylococcus were the most frequently microorganisms found. Infection by Gram negative agents was more aggressive and directly related with failure of therapy. Treatment included since only systemic antibiotics until withdrawal of shunt with use of systemic and intrathecal antibiotics. The best therapeutic results were obtained with withdrawal of shunt system and replacement by external shunt system associated to systemic antibiotics. In our experience this management must be accepted for treatment of this severe complication.

[Cerebellar astrocytomas in childhood. Experience with 25 cases]. / Astrocitomas do cerebelo na infância. Experiência em 25 casos.

The experience with the surgical treatment of cerebellar astrocytomas in 25 children is reported. The clinical presentation, incidence, CT-scan diagnostic studies, pathology, recurrence and treatment aspects are discussed. The series included children until 10 years old with peak (7 cases) in the 7th year of age. The more frequent opening symptoms were: headache, vomit and gait disturbances. No surgical mortality occurred in the series. The authors conclude that surgical radical resection is the best therapeutics for this type of tumor and that radiotherapy is indicated only for tumors with malignant histology.

Actual asymptomatic epidural hematomas in childhood. Report of three cases.

It is well known that the mortality of epidural hematoma (EDH) cases is directly related to the patient's level of consciousness at the time of surgery. The authors report three actually asymptomatic patients with mild head injury, in whom the diagnosis of EDH was possible because of quite broad indications for computed tomographic (CT) scanning, which allowed diagnosis and treatment in an early phase. These cases illustrate the possibility of an EDH in a totally asymptomatic patient.

[Depressed skull fractures in children under 2 years of age. Retrospective study of 43 cases]. / Fratura com afundamento craniano em crianças com menos de dois anos de idade. Estudo retrospectivo de 43 casos.

Depressed skull fractures (DSF) in infancy and childhood are frequent but only a few articles make an analysis in children with age between 0 and 2 years. This is a retrospective study of 43 patients with DSF and age ranged from 0 to 2 years. Falls and traffic accidents were the most common causes. Most patients were admitted in alert state. 69.8% of the patients were submitted to surgical treatment. The parietal bone was more frequently injured (55.8%). Most of the patients had type 1 DSF (the depressed bone remains connected to the cranial vault). The incidence of associated lesions of nervous system was lower than reported in the literature.

[Lipoma of the corpus callosum with extracranial extension through frontal bone defect]. / Lipoma do corpo caloso com extensão extracraniana através de falha óssea frontal.

Intracranial lipoma are usually localized in the corpus callosum and rarely extends to the subgaleal region. Only eight cases of lipoma of the corpus callosum with extracranial extension were reported in the literature. Seizures and mental retardation were the most common clinical findings in these patients. Data from the literature show that resection of intracranial lipoma has catastrophic results. The resection must be restricted to the extracranial portion. We report a child with lipoma of the corpus callosum with extracranial extension with no neurologic deficit submitted to resection of the extracranial extension.

[Clinicopathological study on reported case for bronchogenic carcinoma].

After a primary operation for bronchogenic carcinoma, eight out of 253 patients (3.2%) underwent reoperation for local recurrence or intrathoracic metastasis. The histology was well or moderately differentiated adenocarcinoma in all cases. The average interval between the first and second operation was 34 months (range: six to 63 months). There were three local recurrences, two lung metastases and one mediastinal lymph node metastasis (#3a) ipsilaterally two lung metastases contralaterally. Completion pneumonectomy was underwent in one, wedge or segmental resection in five and excision of lymph node in one case after an initial ipsilateral lobectomy. Two patients underwent contralateral wedge or segmental resection after initial lobectomy. Four of eight patients died of brain, liver, or bone metastases after reoperation, the time of survival averaging 63 months. Two patients died of pneumonia, the time survival averaging 92 months. Two patients are still alive, one in 52 months and another in 20 months after reoperation.

[Magnetic resonance imaging in spinal dysraphisms]. / Ressonância nuclear magnética nos disrafismos espinais.

We studied by magnetic resonance imaging the incidence of associated malformations in a group of 38 patients with diagnosis of spina bifida followed in our outpatient clinic. Twenty-two children were born with spina aperta and 16 with spina bifida occulta. Tethered cord was found in the majority of patients of both groups. Syringomyelia was found in 13 patients. The craniocervical region was studied in 11 patients with the diagnosis of spina bifida aperta; Chiari malformation was found in 8. The results point to the fact that asymptomatic malformations detected by MRI techniques are frequent in patients with spina bifida.

Large clear cell adenoma of the parathyroid in a patient with MEN-1 syndrome. Ultrastructural study of the tumour exhibiting unusual RER formations.

A 48 year old man presented with hyperparathyroidism and acromegaly. Although pancreatic tumour was not demonstrated, the diagnosis of MEN-1 syndrome was made. The parathyroid adenoma was removed surgically and investigated by histology and transmission electron microscopy and was diagnosed as large clear cell adenoma with extensive deposition of glycogen. Much of the endoplasmic reticulum was present in two special forms: annulate lamellae and the rare rail-like configurations both of which showed continuity with the conventional RER as well as with each other. No obvious correlation was apparent between presence of special RER formations and growth rate or endocrine activity of tumour; they may be regarded as signs of cellular dedifferentiation.

Brain tumors in the first two years of life: a review of forty cases.

From 1962 to 1989, 40 infants with brain tumors and less than 2 years old were treated at the Department of Neurology of the Clinical Hospital of the University of São Paulo Medical School. The clinical and neuropathological findings were reviewed as to histological diagnosis, age, sex, signs and symptoms, therapy and outcome. Medulloblastoma was the most common histological type (n = 11), followed by ependymoma (n = 9), choroid plexus tumor (n = 6), astrocytoma (n = 3) and primitive neuroectodermal tumor (n = 2). The tumor was infratentorial in 21 infants, supratentorial in 18 and disseminated in 1.

Dandy-Walker syndrome: posterior fossa craniectomy and cyst fenestration after several shunt revisions.

Ventriculoperitoneal or cyst-peritoneal shunts are the usual treatment for children with Dandy-Walker syndrome. A combined ventricular and cyst shunt is often necessary and, in some cases, multiple revisions have to be done. Two patients with this syndrome were treated with posterior fossa exploration and cyst fenestration after several shunt revisions. The procedure was well tolerated with good results in both cases. The patients' data and preliminary results are presented here.

Steroid transhydrogenase activity of 3-ketosteroid-delta 1-dehydrogenase from Nocardia corallina.

3-Ketosteroid-delta 1-dehydrogenase from Nocardia corallina catalyzes transhydrogenation of 3-keto-4-ene-steroid to 3-keto-1,4-diene-steroid e.g., progesterone to 1,4-androstadiene-3,17-dione. The reaction proceeded linearly at first and then soon slowed down owing to equilibration. The turnover number of this reaction was of the same magnitude as that of the dehydrogenation of 3-keto-4-ene-steroid. The pH optimum was 8.4, which is lower than that of the dehydrogenase reaction. The enzyme has a wide specificity for hydrogen acceptor steroids. The Km' and Kmax' values for these steroids and the values of the corresponding 3-keto-4-ene-steroids were compared. Kinetic studies of the steroid transhydrogenase reaction demonstrated a typical ping-pong mechanism. The enzyme oxidized 1,2-tritiated progesterone and transferred the tritium atoms to the reaction product, 4-androstene-3,17-dione, and water. Transhydrogenation in D2O resulted in the incorporation of a deuterium atom into the C2-position of 4-androstene-3,17-dione. The results indicate that the enzyme catalyzes C1, C2-trans axial abstraction of hydrogen atoms from progesterone, transfer of the 1 alpha-hydrogen to the C1-position of 1,4-androstadiene-3, 17-dione and release of the 2 beta-hydrogen to water. Reaction schemes based on the experimental results are proposed. The enzyme also catalyzes the reduction of 3-keto-1,4-diene-steroids with reduced benzyl viologen.

Biopsia de lesoes encefalicas por trepanacao. / Biopsy of encephalic lesions by trephining

O diagnostico anatomopatologico das lesoes encefalicas de natureza expansiva e necessario para a instituicao do tratamento adequado. Muitas lesoes localizadas nas estruturas cerebrais profundas ou em centros nervosos de grande importancia funcional nao podem ser abordadas por procedimentos neurocirurgicos habituais, sem riscos para os doentes. Nesses casos, a biopsia percutanea, estereotaxica ou assistida pela tomografia computadorizada deve ser empregada para esclarecimento diagnostico. Os autores apresentam o resultado da bipsia por trepanacao de lesoes encefalicas inabordaveis pelos metodos neurocirurgicos tradicionais, em 63 doentes. O procedimento foi realizado a mao livre em 18 casos, por estereotaxia em 28 e por estereotomografia em 17. O indice de mortalidade e de morbidade foi baixo. Cinco doentes foram, durante o mesmo ato operatorio, submetidos a radioterapia intersticial por tecnica de "after loading". Os criterios de selecao, a avaliacao pre-operatoria e as vantagens da biopsia por trepanacao sao discutidos