RESUMEN
UNLABELLED: There is only sparse information about the development of children after successful treatment for intracranial germ-cell tumours. Between January 1981 and June 1992, 26 children with intracranial germ-cell tumours were treated in the University Hospital Hamburg-Eppendorf. We report on treatment results, long standing residuals and the "quality of life" of these patients. The long-term event-free survival was 88% for the germinomas and 43% for the malignant teratomas. Of the patients 58% had no relevant functional neurological deficits and 37% had mild impairment. Only 1 patient with metastatic disease was severely handicapped. Six patients showed neuro-endocrine dysfunction. All of them had suprasellar/hypothalamic lesions and all received successful substitution therapy. As to neuropsychological functions, 53% of the patients had no or only mild disturbances. The most affected function was speed of information processing. Of the children 69% were able to proceed with their education at the same level as before therapy. The overall self-assessment revealed good results in 75% of the patients. CONCLUSION: After surgical removal and radiation therapy long-term survival of intracranial germinomas amounts to 88%. Despite craniospinal axis radiation severe residuals are rare and a good quality of life is common. In malignant teratomas treatment regimens including chemotherapy are much less successful.
Asunto(s)
Daño Encefálico Crónico/diagnóstico , Neoplasias Encefálicas/cirugía , Neoplasias de Células Germinales y Embrionarias/cirugía , Complicaciones Posoperatorias/diagnóstico , Adolescente , Daño Encefálico Crónico/mortalidad , Neoplasias Encefálicas/diagnóstico , Neoplasias Encefálicas/mortalidad , Neoplasias Encefálicas/patología , Niño , Preescolar , Personas con Discapacidad/psicología , Supervivencia sin Enfermedad , Femenino , Estudios de Seguimiento , Humanos , Lactante , Masculino , Neoplasias de Células Germinales y Embrionarias/diagnóstico , Neoplasias de Células Germinales y Embrionarias/mortalidad , Neoplasias de Células Germinales y Embrionarias/patología , Examen Neurológico , Pruebas Neuropsicológicas , Complicaciones Posoperatorias/mortalidadRESUMEN
The authors studied the intelligence, memory, visuomotor skills and la nguage of 298 six-year-old children with very low birthweight (VLBW) (less that 1500g). Of 591 VLBW childern born July 1983 to June 1986 within 50km of the centre of Hamburg, Germany, 330 were traceable at age six years and 298 of these were seen by a neuropaediatrician and a psychologist; the other 19 were too severely disabled for psychological assessment with the standardized tests used. The mean memory performance of VLBW children at age six years was below the standard mean in all diagnostic and socio-economic subgroups. As expected, visuomotor development was clearly influenced by neurological but not socio-economic status. Intelligence and language skills were much more closely related to socio-economic background that to neurological morbidity. However, VLBW children with hyperactivity, clumsiness or cerebral palsy differed significantly in intelligence and visuomotor performance from those without neurological symptoms.
Asunto(s)
Trastornos del Conocimiento/diagnóstico , Discapacidades del Desarrollo/diagnóstico , Personas con Discapacidad , Recién Nacido de muy Bajo Peso , Parálisis Cerebral/complicaciones , Niño , Trastornos del Conocimiento/etiología , Discapacidades del Desarrollo/etiología , Femenino , Estudios de Seguimiento , Humanos , Recién Nacido , Inteligencia , Masculino , Memoria , Desempeño Psicomotor , Factores SocioeconómicosRESUMEN
Tumors of the cerebral hemispheres comprise a big variety of histologic tumor types. Therefore, in the literature usually only specific subgroups such as benign gliomas and temporal lobe tumors are reported. In this study we report on 44 tumors of the cerebral hemispheres, including 9 angiomas. Apart from the treatment results concerning event free survival, the neurological and neuropsychological outcome of the patients were assessed. A peculiarity in the hemispheric tumors is their association with focal epilepsies. In 94% of our patient series, epileptic seizures had been the first tumor associated symptom and approximately 62% developed focal epilepsy. Seizure types, their association with tumor location and histology, the success of tumor therapy in concern of the epilepsy and the significance of the electroencephalogram in the follow up care of these patients were assessed separately.
Asunto(s)
Neoplasias Encefálicas/terapia , Corteza Cerebral , Epilepsias Parciales/terapia , Glioma/terapia , Adolescente , Adulto , Daño Encefálico Crónico/diagnóstico , Daño Encefálico Crónico/mortalidad , Daño Encefálico Crónico/patología , Neoplasias Encefálicas/mortalidad , Neoplasias Encefálicas/patología , Corteza Cerebral/patología , Corteza Cerebral/cirugía , Quimioterapia Adyuvante , Niño , Preescolar , Terapia Combinada , Craneotomía , Epilepsias Parciales/mortalidad , Epilepsias Parciales/patología , Femenino , Estudios de Seguimiento , Glioma/mortalidad , Glioma/patología , Humanos , Lactante , Masculino , Complicaciones Posoperatorias/diagnóstico , Complicaciones Posoperatorias/mortalidad , Complicaciones Posoperatorias/patología , Radioterapia Adyuvante , Tasa de SupervivenciaRESUMEN
A 2-month-old boy underwent surgery for removal of a right temporal melanotic neuroectodermal tumor of infancy (MNTI). Histologically the tumor tissue showed signs of malignancy. The child was reexamined several times up to the age of 5 years. Neuroradiological evaluation showed no evidence of tumor recurrence or metastases. No resulting handicap was observed during neurological and psychological follow-up examination at the age of 5 years. Our findings confirm that surgical removal as the therapy of choice provides an excellent prognosis for this kind of tumor in spite of its histologically malignant appearance.
Asunto(s)
Neoplasias Encefálicas/patología , Encéfalo/patología , Tumor Neuroectodérmico Melanótico/patología , Encéfalo/cirugía , Encéfalo/ultraestructura , Neoplasias Encefálicas/cirugía , Neoplasias Encefálicas/ultraestructura , Estudios de Seguimiento , Humanos , Lactante , Masculino , Necrosis/patología , Tumor Neuroectodérmico Melanótico/cirugía , Tumor Neuroectodérmico Melanótico/ultraestructura , Pronóstico , Tomografía Computarizada por Rayos X , Resultado del TratamientoRESUMEN
Since living related liver transplantation was first performed in 1989, more than 150 cases have been performed worldwide, mostly in the United States and Japan. This paper reports the first series of living related liver transplantation in Europe. Twenty living related liver transplantation surgeries were performed over a 13-mo period, with an overall patient survival of 85%. For patients who underwent elective transplantation (n = 13), the survival rate was 100%. Technical complications included one arterial thrombosis necessitating retransplantation and five bile leaks requiring surgical revision. The technical improvements that permit avoidance of these complications are discussed. A detailed description of the living related liver procurement is given. All procurements yielded grafts of excellent quality. No intraoperative complications occurred, and no reoperations were necessary. No heterologous blood transfusion was needed. In two patients, incisional hernias developed after wound infection. Living related liver transplantation does not absolve the transplant community of efforts to promote cadaveric organ procurement. Nevertheless, living related liver transplantation does have the advantage of a readily available graft of excellent quality, permitting transplantation with optimal timing under elective conditions. Several centers are now preparing living related segmental liver transplants, following the model of our protocol, for three reasons: (a) to obtain superior results compared with cadaveric liver transplantation; (b) to overcome cadaveric organ shortage and further reduce pretransplantation mortality and (c) to provide viable organs in countries where cadaveric organ procurement is not established. When performed by a team experienced in pediatric liver transplantation and in adult liver resection, living related liver transplantation is an excellent modality for the treatment of end-stage liver disease in children.
Asunto(s)
Trasplante de Hígado/métodos , Padres , Donantes de Tejidos , Adulto , Niño , Preescolar , Femenino , Rechazo de Injerto , Supervivencia de Injerto , Humanos , Lactante , Trasplante de Hígado/estadística & datos numéricos , Masculino , Persona de Mediana Edad , Complicaciones Posoperatorias , Tasa de SupervivenciaRESUMEN
Germ-cell tumors can be subdivided into germinoma, embryonal carcinoma, choriocarcinoma, endodermal sinus tumor (yolk-sac tumor), and teratoma. They are also distinguished by their production of secreted markers such as alpha-fetoprotein produced in endodermal sinus tumors and embryonal carcinoma or beta-human chorionic gonadotropin, produced by choriocarcinoma and embryonal carcinoma. Germinoma and teratoma produce none of the markers. Because it has been proposed that teratomas may differentiate from multipotent stem cells contained in embryonal carcinoma and are thus lineage related, the presence of markers indicates the presence of a nongerminomatous germ-cell tumor. Nongerminomatous germ-cell tumors are an invariably fatal subgroup within the pediatric pineal region germ-cell tumors. There is no effective, established therapeutic regimen. We report the treatment regimen for three children diagnosed with this highly aggressive tumor entity. The children were first given a course of chemotherapy with bleomycin, etoposide, and cisplatin. This resulted in the normalization of markers and the shrinkage of tumors. These were then removed by the infratentorial supracerebellar approach. Removal was followed by a second course of chemotherapy with vinblastine, ifosfamide, and cisplatin; after which the children underwent radiotherapy. All three children are well and without evidence of residual or recurrent disease 20, 30, and 32 months after surgery, respectively. We propose this therapy regimen for children in whom the markers are positive.
Asunto(s)
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Neoplasias Encefálicas/tratamiento farmacológico , Glándula Pineal , Pinealoma/tratamiento farmacológico , Protocolos de Quimioterapia Combinada Antineoplásica/efectos adversos , Bleomicina/administración & dosificación , Neoplasias Encefálicas/patología , Neoplasias Encefálicas/radioterapia , Neoplasias Encefálicas/cirugía , Niño , Preescolar , Cisplatino/administración & dosificación , Terapia Combinada , Relación Dosis-Respuesta a Droga , Esquema de Medicación , Etopósido/administración & dosificación , Estudios de Seguimiento , Humanos , Imagen por Resonancia Magnética , Masculino , Glándula Pineal/patología , Glándula Pineal/efectos de la radiación , Glándula Pineal/cirugía , Pinealoma/patología , Pinealoma/radioterapia , Pinealoma/cirugíaRESUMEN
As part of a longitudinal follow-up program a single case analysis of the history of 53 children with a birthweight of less than 1501 g, who had developed cerebral palsy, was performed. In 17 children (32%) a definite explanation for the neurological impairment and in another 17 (32%) at least a possible explanation could be found. In 19 of the 53 children (36%) the history was uneventful and did not offer a causal event. The common documentation in obstetric and pediatric case files left the cause of cerebral palsy undetected in one third of the cases.
Asunto(s)
Parálisis Cerebral/etiología , Recién Nacido de Bajo Peso/crecimiento & desarrollo , Parálisis Cerebral/epidemiología , Femenino , Estudios de Seguimiento , Alemania/epidemiología , Humanos , Recién Nacido , Recien Nacido Prematuro/crecimiento & desarrollo , Estudios Longitudinales , Masculino , Factores de RiesgoRESUMEN
At the departments of neurosurgery and pediatrics of the University Hospital of Hamburg 21 patients after neurosurgery and radiation of cerebellar tumors have been examined as to neuropsychological, neurological and ophtalmological disabilities. A standardized questionairy for the parents concerning different aspects of their children's quality of life (physical, mental, social health and functional limitations) was performed. 16 children showed minor neurological signs with no or mild disability, in 5 children severe disability was found. In 4 patients morphological changes in the eyeground, in 2 patients amblyopia was detected. 19 patients underwent a neuropsychological examination. The group of patients with medulloblastoma or ependymoma (n = 10) scored in nearly all tests below the group with astrocytoma (n = 9). 3 out of 7 patients in the medulloblastoma or ependymoma group had to abandon school, one patient needs special education. All patients with astrocytoma performed fairly well at school. Parents of patients with medulloblastoma or ependymoma classified their children's quality of life not as good as did parents of children with astrocytoma. The amount of objective disability had no influence on the parents estimation of their children's quality of life. Besides the neurological examination the neuropsychological and quality of life assessment proved to be an essential part of follow up. For a more comprehensive understanding of quality of life, qualitative assessment strategies should be included in further research.
Asunto(s)
Daño Encefálico Crónico/etiología , Neoplasias Cerebelosas/cirugía , Complicaciones Posoperatorias/etiología , Calidad de Vida , Adolescente , Astrocitoma/cirugía , Niño , Preescolar , Terapia Combinada , Ependimoma/cirugía , Femenino , Estudios de Seguimiento , Humanos , Lactante , Masculino , Meduloblastoma/cirugía , Pruebas NeuropsicológicasRESUMEN
In a national study the prevalence of open meningocele and myelomeningocele as well as the geographical distribution in the Federal Republic of Germany was determined among the life-born infants during 1982 and 1983. The prevalence rates of the single districts and states and the federal territory were established based upon standardized reports of nearly all Children's Hospitals, Departments of Child Surgery and the major Centers of Neurosurgery. In Hamburg the study was extended by establishing the number of abortions because of medical indications after the prenatal diagnosis of a myelomeningocele. In 1982 and 1983 there were 354 resp. 342 infants born with an open myelomeningocele, thus the prevalence rate was 0.569 and 0.575/1000 live births. The average prevalence rate was 0.57/1000 live births. Compared with international studies our data are corresponding with reports from countries with a relative low frequency. The prevalence rates of the states in the federal territory differ between 0.42/1000 live births in Hamburg and 0.78/1000 in Bremen; these differences are not statistically significant. In 9 from 247 districts, the reported rates were significantly higher than the average rate. Because of the small number of children born with this malformation in the single districts, any conclusions from these results are not warranted. Quite different from reports of other countries (Great Britain, United States of America) this study did not show essential geographic differences in the federal territory. Prenatal diagnosis and subsequent termination of pregnancy lowered the prevalence rate of open meningomyelocele at birth in Hamburg from 0.61/1000 to 0.42/1000.
Asunto(s)
Meningomielocele/epidemiología , Aborto Eugénico/estadística & datos numéricos , Estudios Transversales , Alemania Occidental , Humanos , Recién Nacido , Diagnóstico PrenatalRESUMEN
A considerable number of high risk brain tumours require an interdisciplinary approach with surgery, radiation and chemotherapy rather than one single therapeutic regimen. Children with a medulloblastoma must be subdivided into four different risk groups depending on the size of the tumour, on the age of the child and on cerebral spinal fluid cytology findings. The treatment protocol for ependymomas depends on the location of the tumour and on histological grading. Pineal region tumours can be successfully removed; further therapy depends on the type of the tumour. Children with hemispheric high grade astrocytomas gain substantially from local radiation therapy. The GPO (Gesellschaft für Pädiatrische Onkologie) has most recently taken the initiative to found a pediatric neuro-oncology working group in order to establish and to evaluate different treatment modalities.
Asunto(s)
Neoplasias Encefálicas/terapia , Antineoplásicos/uso terapéutico , Neoplasias Encefálicas/tratamiento farmacológico , Neoplasias Encefálicas/radioterapia , Neoplasias Encefálicas/cirugía , Neoplasias Cerebelosas/tratamiento farmacológico , Neoplasias Cerebelosas/radioterapia , Neoplasias Cerebelosas/terapia , Terapia Combinada , Ependimoma/radioterapia , Ependimoma/cirugía , Ependimoma/terapia , Glioma/tratamiento farmacológico , Glioma/radioterapia , Humanos , Meduloblastoma/tratamiento farmacológico , Meduloblastoma/radioterapia , Meduloblastoma/terapia , Grupo de Atención al Paciente , Pinealoma/tratamiento farmacológicoRESUMEN
At the department of neurosurgery and pediatrics of the University Hospital of Hamburg 18 children with pineal region tumors were treated by surgical removal and craniospinal axis radiation. Total or nearly total removal was achieved in 13 cases. In case of astrocytoma and pineocytoma no further treatment was performed. Children with germinoma, pinealoblastoma and teratoma underwent craniospinal axis radiation postoperatively. All children thus treated, except one with a highly malignant pinealoblastoma, are so far relapse-free. Two patients with germinoma and two patients with malignant mixed teratoma, which showed extensive growth and/or primary metastases already at admission died. Subject of this report is an assessment of quality of live and a comprehensive follow-up of neurological, endocrinological and psychointellectual conditions of the surviving children. Parent assessment of quality of life indicate for all but two children life quality as excellent or reasonable. One child was disabled by severe motor dysfunction, visual defects and convulsions. In two children minor neurological handicap was found. Minor oculomotor dysfunction was the most frequent neurological finding. Four children showed endocrinopathies. In three cases tumor associated panhypopituitarism persisted after treatment. Only one child developed growth hormone and ADH deficit succeeding surgery and craniospinal axis radiation. The children were examined with a neuropsychological test-battery. In four children severe neuropsychological deterioration was found. Three children showed no remarkable deterioration, five only minor or moderate psychological deficits. Generally verbal intelligence seemed to be on average, non-verbal intelligence and ability for concentration showed decreased scores.
Asunto(s)
Neoplasias Encefálicas/cirugía , Glándula Pineal/cirugía , Pinealoma/cirugía , Complicaciones Posoperatorias/etiología , Calidad de Vida , Niño , Desarrollo Infantil , Preescolar , Disgerminoma/cirugía , Hormona Folículo Estimulante/sangre , Estudios de Seguimiento , Hormona del Crecimiento/sangre , Humanos , Hidrocortisona/sangre , Hormona Luteinizante/sangre , Teratoma/cirugía , Tirotropina/sangreRESUMEN
In Germany, the relative frequency of pineal region tumours seems to be much higher than hitherto assumed. At the University Hospital Hamburg, from 1980-1985 17 children with pineal region tumours were encountered amongst 102 children with CNS tumours. Two-cell-type germinoma is the most frequent pineal region tumour. Cerebrospinal fluid cytology is highly successful in identifying this germ cell tumour. Surgical removal has become a reasonably safe procedure in the treatment of pineal region tumours and was successful in all 10 cases so treated. In addition, our patients with two-cell-type germinomas received craniospinal axis radiation. All children, treated by both surgical removal and craniospinal axis radiation are so far relapse-free and are functioning on a pretreatment level.
Asunto(s)
Neoplasias Encefálicas/patología , Glándula Pineal/patología , Pinealoma/patología , Teratoma/patología , Neoplasias Encefálicas/epidemiología , Neoplasias Encefálicas/terapia , Niño , Terapia Combinada , Alemania Occidental , Humanos , Pinealoma/epidemiología , Pinealoma/terapia , Teratoma/epidemiología , Teratoma/terapiaRESUMEN
This is a report of a 1 1/2-year-old male with megaloblastic anaemia and a progressive neurological disorder clinically resembling leucodystrophy. The child was exclusively breast fed and his mother had been a strict vegetarian for more than 4 years before the child was born. After Vitamin B12 therapy the anaemia disappeared and the neurological condition dramatically improved.
Asunto(s)
Anemia Macrocítica/etiología , Anemia Megaloblástica/etiología , Encéfalo/patología , Lactancia Materna , Dieta Vegetariana/efectos adversos , Deficiencia de Vitamina B 12/complicaciones , Atrofia/etiología , Femenino , Humanos , Lactante , Masculino , EmbarazoAsunto(s)
Adenoma/cirugía , Hormonas Hipofisarias/metabolismo , Neoplasias Hipofisarias/cirugía , Acromegalia/etiología , Adenoma/complicaciones , Adenoma/metabolismo , Adolescente , Niño , Preescolar , Síndrome de Cushing/etiología , Humanos , Métodos , Microcirugia , Síndrome de Nelson/etiología , Neoplasias Hipofisarias/complicaciones , Neoplasias Hipofisarias/metabolismo , Hueso Esfenoides , Instrumentos QuirúrgicosAsunto(s)
Muerte Súbita del Lactante/etiología , Peso al Nacer , Tronco Encefálico/fisiopatología , Edad Gestacional , Corazón/inervación , Humanos , Lactante , Pulmón/inervación , Monitoreo Fisiológico , Neuronas/fisiología , Riesgo , Muerte Súbita del Lactante/fisiopatología , Muerte Súbita del Lactante/prevención & control , Sinapsis/fisiologíaRESUMEN
Since 1981, 96 infants considered at increased risk of SIDS underwent home monitoring for prolonged sleep apnea: 23 infants after a near miss for SIDS event, 28 siblings of a SIDS victim and 45 infants with a variety of perinatal risk factors. For a total of 65 infants the course of home monitor surveillance was completed by September 1984 with a duration ranging from 6 to 15 month: 26% (4/15) of the near miss for SIDS group, 23% (3/13) of the SIDS siblings and 13% (5/37) of the perinatal risk cases developed more than one prolonged apneic episode with additional symptoms requiring vigorous intervention by parents. Two infants of the perinatal risk group became SIDS victims: despite an apnea alarm after 15 seconds the parents were unable to resuscitate their infant in one case, the other died from SIDS about 4 month after monitoring was discontinued because of an uneventful course and normal polygraphic sleep recordings The large number of prolonged apneas requiring intervention and the two SIDS cases (3% of the total study group) indicate a considerably increased risk of prolonged life-threatening sleep apnea and SIDS in the population monitored.