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  • 11
    ISSN: 1432-2307
    Keywords: Key words Spleen ; Light chain deposition disease ; Sea blue histiocytes ; Ceroids ; Spontaneous rupture
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  Splenic involvement by a light chain deposition disease (LCDD) associated with sea-blue histiocytosis occurred in a 55-year-old man presenting with LCDD of the kidney without myeloma. Lambda light chain deposits were demonstrated by immunohistochemistry in vessel walls and along the ring fibres of the red pulp sinuses. Accumulation of sea blue histiocytes in the cords was also present. Stiffness of the walls of the red pulp sinuses resulting from light chain deposits may have induced accumulation and destruction of circulating blood cells. Lipid catabolism with production of ceroids may have resulted in lipidic histiocytosis with a sea blue histiocyte pattern.
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  • 12
    ISSN: 1433-0350
    Keywords: Key words Hydrocephalus ; Infancy ; Intellectual outcome ; Scholastic integration ; Prognostic factors
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The purpose of this report is to analyze the long-term outcome in hydrocephalic children treated by shunt placement and in particular their psycho-intellectual development. In the case of shunt placement postoperative mortality is virtually nil, and the overall mortality rate after 10 years of follow-up has been reduced to less than 5%. Morbidity, in contrast, is far from satisfactory. This study was conducted in 129 consecutive children with nontumorous hydrocephalus who underwent a first shunt insertion before the age of 2 years between 1979 and 1982 and who were followed up for at least 10 years. The final neurological examination revealed a motor deficit in 60%, visual or auditory deficits in 25%, and epilepsy in 30%. The final IQs were above 90 in 32% of the children, between 70 and 90 in 28%, between 50 and 70 in 19%, and lower than 50 in 21%. Integration into the normal school system was possible for 60% of the children, but half of them were 1–2 years behind their age group or having difficulties; 31% were attending special classes or were in institutions; and 9% were considered ineducable. The presence of behavioral disorders was a determinant factor for scholastic and social integration. Such disorders were frequent, and were characterized as severe in 30%. A relationship between final outcome and etiology, initial ventricular size, and epilepsy was observed. These results are used as the basis of a discussion on how morbidity might be improved.
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  • 13
    ISSN: 1433-0350
    Keywords: Neonatology ; Intracranial hematomas ; Hydrocephalus ; Dystocic deliveries ; Perinatal anoxia ; Bleeding disorders
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Acute intracranial hematomas in 17 term neonates are reported: 3 were subdural in the posterior fossa; 14 supratentorial, either subdural (8), intracerebral (1 frontal, 4 occipital) or intraventricular (1). The hematomas were aspirated in 3 cases and surgically evacuated in 10. In the remaining 4, the effusion was left in situ. Three patients died: 2 due to dramatic preoperative deterioration and 1 to hemostatic difficulties during surgery. In the 14 survivors, neurological sequellae are major in 3, nil or minor in 11. Comparison of these cases to the 110 others already published allows the following conclusions: (1) the cause of such hematomas is usually multifactorial, combining obstetrical trauma, anoxia and/or coagulation disorders; (2) early diagnosis of the hematoma requires early echography and/or tomodensitometry; (3) when the hematoma causes a midline shift, the authors advocate its early evacuation by aspiration, or craniotomy if it is clotted; (4) the prognosis is poor when there is associated severe perinatal anoxia; if there is no anoxia, the prognosis seems good, but as the follow-up is usually short, definite conclusions are difficult; (5) in our series hydrocephalus requiring treatment occurred in 2 of the 14 survivors.
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  • 14
    ISSN: 1433-0350
    Keywords: Stereotaxy ; Fibrin gene ; Surgical approach of space-occupying lesions
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract When intracerebral space-occupying lesions are small or located deeply within the brain parenchyma, it is sometimes difficult to localize them at surgery or to design the most direct and least hazardous surgical approach. Therefore, we have developed a method that combines conventional neurosurgical techniques with craniotomy using stereotactic guidance. We have used the Brown-Roberts-Wells (BRW) stereotactic system, which allows for computed tomography (CT) or magnetic resonance imaging (MRI) guidance and does not interfere with the absolute sterility mandatory each time a flap is created. Eleven patients were operated on using this method. The deep tumors were approached through a linear incision of the cerebral cortex. Then a needle, fixed in the right position on the arc system of the BRW, was inserted toward the surface of the lesion; the exposure was finally widened by inflation of a rubber balloon set on the stereotactic needle. This technique allows the two lips of the cortical incision to be glued at the end of the operation. Gluing with a fibrin glue avoids the postoperative subdural collections that often develop when the ventricle has been opened. Except for one case, the post-operative course was uncomplicated in these patients. No permanent postoperative neurological worsening was observed even after the removal of an intrathalamic tumor.
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  • 15
    Electronic Resource
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    Monatsschrift Kinderheilkunde 148 (2000), S. 496-497 
    ISSN: 1433-0474
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
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  • 16
    ISSN: 1433-0350
    Keywords: Supratentorial tumors ; Astrocytomas ; Ependymomas ; Secondary malignancies ; Paediatric patients
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Sixty-four malignant hemispheric tumors in children less than 15 years old were treated in the pediatric neurosurgical department of the H ôpital des Enfants Malades between 1970 and 1989. (1) These tumors evolved rapidly in most cases. However the pre-operative evolution in 20% of the patients had a duration of more than 6 months, which favors the hypothesis that at least one-fifth of these tumors result from malignant transformation of a benign lesion. This observation should prompt neurosurgeons to operate on all benign hemispheric tumors as soon as they are diagnosed. (2) Five of the 64 patients had two successive malignant diseases. In four cases the other malignant disease was an acute lymphoblastic leukemia. (3) Among the malignant hemispheric tumors, the grade III and IV astrocytomas had a dismal prognosis. As it is known from previous studies that grade I and II astrocytomas have a good prognosis in children, it can be concluded that grading these tumors is essential. By contrast, almost one out of two patients with malignant ependymoma was alive 5 years after treatment. This implies that the grading of ependymomas is of modest prognostic values. (4) The harmful effect of radiotherapy was evaluated by comparing the functional outcome of children operated for a benign hemispheric tumor to that of children operated and irradiated for a malignant hemispheric tumor.
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  • 17
    ISSN: 1433-0350
    Keywords: Malignant tumors of the posterior fossa ; Intellectual outcome ; Radiotherapy to posterior fossa ; Radiotherapy to cerebral hemispheres ; Radial surgery ; Brain stem lesions
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The purpose of this study was to determine the respective parts played by cerebral hemisphere irradiation, posterior fossa irradiation, and surgery in the poor late functional results often observed in children treated for medulloblastoma. To do this we compared the intellectual outcome in a series of 59 children operated on for medulloblastoma, who had received whole-brain irradiation, to that observed in a series of 37 children operated on for ependymoma of the posterior fossa, who had received radiotherapy only on the posterior fossa. Only patients who had survived for more than 2 years without recurrence were included. At the assessment 1 year after treatment, intellectual outcome was somewhat better in the ependymoma group, but the difference was not statistically significant. At the long-term follow-ups at 5 and 10 years the results remained stable in the children treated for ependymoma, around 60% having an IQ above 90, whereas the intellectual level of the children treated for medulloblastoma was seen to have deteriorated progressively: 20% had an IQ above 90 5 years after treatment and only 10% at the 10-year followup. This progressive degradation is most likely due to the irradiation of the cerebral hemispheres, as this prophylactic irradiation constituted the only difference between the two groups. Moreover, irradiation to the posterior fossa did not seem to affect intellectual functions, since in the group of children with ependymomas the proportion of IQs above 90 was high and remained stable over the years. Surgery was certainly responsible for some poor results. The percentage of IQs above 90 observed 1–2 years after treatment was between 70 and 80 when no postoperative complications occurred, and only between 20–40% in the presence of postoperative complications. Postoperative aggravation was in most cases related to a brain-stem lesion. These results encourage the reduction, when possible, of irradiation to the cerebral hemispheres and underline the importance of the quality of surgery.
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  • 18
    ISSN: 1433-0350
    Keywords: Key words Desmoplastic ; Brain tumour ; Infantile ; Surgery ; Astrocytoma ; Ganglioglioma
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  The authors report on the clinicopathological aspects of and management strategies for the group of rare, large hemispheric childhood tumours recently classified as desmoplastic infantile ganglioglioma (DIGG), desmoplastic astrocytoma of infancy (DACI) and pleomorphic xanthoastrocytoma (PXA). Between 1985 and 1997, ten children (4 with DACIs, 4 with DIGGs and 2 with PXAs) with a median age of 9.5 months were operated on. All these patients had complete surgical resections, with two having a preoperative biopsy. This led to an erroneous diagnosis in both cases of malignant grade 4 astrocytoma. As a result, one patient had preoperative chemotherapy with no effect. There was one perioperative death. Histology revealed heterogeneous tumours with malignant looking areas in 8 of the specimens. None of the patients has had any postoperative adjuvant treatment. All surviving patients are alive at follow-up (median 4.2 years). Despite their often malignant appearance, these tumours have an excellent prognosis, but they can present formidable surgical challenges when they occur in very young age patients. We believe that surgical excision can offer a cure and that adjuvant treatment is not necessary. Finally, biopsy is of little value and may even lead to an erroneous diagnosis and subsequent mismanagement.
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  • 19
    ISSN: 1433-0350
    Keywords: Children ; Medulloblastoma ; Psychological sequelae ; Radiotherapy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A series of 120 medulloblastomas in children operated on between 1967 and 1987 at the Hôpital des Enfants-Malades has been reviewed in order to check whether the conclusions of our study published 10 years ago have remained valid and, in particular, to verify whether the quality of life of these patients, which had been found to be poor at the time, had improved or worsened over the years. The postoperative mortality for the whole series was 6.5% there have been no deaths in the 35 patients operated on after 1980. The overall survival rate for the 120 children was 60% at 5 years and 53% at 10 years; for the patients who completed radiotherapy, the survival rates was 73% at 5 years and 64% at 10 years. Survival rates were surprisingly better in patients treated when under 6 years of age than in older children. They were also better in girls than in boys, and in desmoplastic compared with other medulloblastomas; however, the differences were not significant. When comparing the groups after total or subtotal resection of tumors, survival rates were not significantly different, but were lower in the small group of partial resections. Cell differentiation did not influence the prognosis. Psychological sequelae were significant and worsened over the years. Five years after treatment 58% of the children showed an IQ above 80; 5 years later this group included only 15% of the patients. These psychological sequelae were related to age at the time of radiotherapy: the younger the child, the lower the final IQ. Five years after treatment, 40% of the children had a normal academic level; 5 years later this group was reduced to 11%. Ten years after treatment, 36% of the patients were unempolyed and 64% worked in a protected environment. No patient had normal employment. These disastrous results concerning the quality of life of these medulloblastoma patients justify new trials with reduced doses of irradiation over the hemispheres, at least in low-risk groups.
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  • 20
    ISSN: 1433-044X
    Keywords: Schlüsselwörter ; Bakterielle Arthritis • Salmonella enteritidis • Hüftgelenk • Pathologische Diagnostik • Therapie ; Key words ; Bacterial arthritis • Salmonella enteritidis • Hip joint • Pathologic diagnosis • Therapy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary Secondary haematogenous joint involvement is seen in less than 1 % of patients with Salmonella infections. These atypical infections are frequently encouraged by pre-existing local or systemic disease. We present a case of a patient with known alcohol abuse who developed a septic infection of her right hip requiring resection of the femoral head. Histologic analysis showed signs of pre-existing osteonecrosis probably induced by alcohol intake. Cartilage and bone were invaded and destructed by an aggressive granulation tissue. Initially, a biopsy evaluated without knowledge of the positive bacteriological result had been interpreted as indicative of a tumour. The onset, clinical course, diagnosis and therapy of joint involvement by Salmonella are discussed with regard to our case and the relevant literature. This case illustrates the necessity for clinicians to share all their information about the patient with the pathologist.
    Notes: Zusammenfassung Sekundäre hämatogene Gelenkinfektionen werden bei weniger als 1 % aller systemischen Salmonellosen beobachtet. Lokale Gewebeschädigungen oder systemische Grunderkrankungen begünstigen das Zustandekommen dieser atypischen Infektionen. Wir stellen den Fall einer alkoholabhängigen Patientin dar, bei welcher eine Salmonellenkoxitis auf dem Boden einer ischämischen Knochennekrose des Hüftkopfes entstand. Die weit fortgeschrittene Zerstörung des Gelenks erforderte eine Resektionsarthroplastik. Histologisch fand sich eine destruierende Invasion von Knorpel und spongiösem Knochen durch zellreiches Entzündungsgewebe. In Unkenntnis des bakteriologischen Befundes war zunächst der Verdacht auf eine neoplastische Veränderung geäußert worden. Entstehung, Verlauf, Diagnostik und Therapie der Salmonellenarthritis werden anhand dieses Falles und der Literatur diskutiert. Die erfolgreiche Befundung einer Knochenbiopsie ist auf eine gute Kommunikation zwischen Kliniker und Pathologe angewiesen.
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