Biomedical subjects
Publications and source records attributed to A Fortuna.
Intramedullary glioblastomas are uncommon tumors. They occur chiefly in the cervicothoracic segments, have a slight tendency to occur in the early decades of life, and have a short clinical history before diagnosis. We report seven cases and discuss the salient features of these tumors, particularly the pathological features and treatment, in light of the relevant literature.
We report the long-term outcome of surgery for cerebral hydatid cyst in 12 children. In only one child, who had been operated on for cardiac hydatidosis a year earlier, was the brain cyst known to be secondary. Of the 12 children, 10 had a single cyst and 2 had multiple cysts. Five single cysts were removed intact by "hydatid birth," two cysts--one single and one multiple--were removed after puncture and aspiration of the cyst fluid, and the other five--four single and one multiple--were accidentally ruptured in the course of the operation. One patient died of infection of the residual space, 2 died of intracranial recurrence of the parasite (7 and 22 months later, respectively), and one, the child with secondary cerebral echinococcosis, died 6 years later of systemic echinococcosis. Eight patients who had a mean follow-up of 28 years are still living and enjoy very good health, both general and neurological. This long-term follow-up with such good results confirms the usually primary character of cerebral hydatid disease, at least in children. The combination of patent ductus arteriosus and ingestion of unboiled animals' milk might well explain how primary cerebral hydatid disease develops in children.
Two cases of aphasia in polyglot patients who experienced different symptoms in each of the languages they knew are reported. The authors discuss the problem and analyze the available literature in an attempt to formulate a pathogenetic hypothesis of the different involvement of the known idioms sometimes observed in aphasic polyglots. In particular, when time has elapsed between the learning of the mother tongue and other languages, and all the known languages are, consequently, functionally independent, it is possible that the two or more known idioms have distinct anatomical representations, probably localized separately in the two hemispheres. This could explain why, in some polyglots, aphasia affects one of the known languages preferentially. In subjects in whom the different known idioms were learned during early childhood, the anatomical representation of the languages is similar, which explains why, in this kind of polyglot, all the known languages can be equally affected by cerebral damage that causes aphasia.
The long term surgical results in a series of 17 epidermoids of the cerebello-pontine angle are reported. In 11 cases the capsule was removed subtotally and in 6 totally. The absence of mortality among the patients who underwent subtotal removal and the onset of recurrences, if any, only long after the first operation, justify subtotal removal when tight capsular adhesions are present.
We report the long-term results of surgery on spinal lipomas in a series of 18 patients. Four patients had congenital lipomas with spina bifida and/or myelomeningocele and the other fourteen had tumoural (true) lipomas. Simple decompression or subtotal removal afforded a very good prognosis in all patients with intradural lipomas whose preoperative neurological deficits were slight. In the light of the long follow-up, the lack of difference in biological behaviour between congenital and tumoural (true) lipomas leads us to regard the two types as a single maldevelopment entity of dysembryogenetic origin.
Dermoids of the posterior cranial fossa in children are rare. We report the clinical and pathological data on nine children with these lesions. A mean follow-up of 17.3 years after total removal confirms the excellent prognosis irrespective of whether the presenting symptom is meningitis or intracranial hypertension. A brief review of 39 published cases follows.
A case of "spontaneous" spinal epidural hematoma diagnosed by MRI confirms the accuracy of the procedure in establishing the nature of the lesion, in delineating its topography and its effects on the spinal cord. MRI, when available, should be regarded as a primary method of investigation.
Epidermoid tumors occur very rarely in the 4th ventricle. We discuss the history, clinical course, and the surgical results for seven patients. One patient had a remittal of symptoms, probably due to the emergence of the tumor from the 4th ventricle into the cisterna magna. Total removal of the capsule was possible only in the youngest patient. At present, four patients (mean follow-up, 6 years) enjoy good general and neurological health.
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Three patients aged 5 1/2 to 9 years old with mutism after posterior fossa surgery are presented. The entity is discussed with a review of 15 additional previously reported cases in children aged 2 to 11 years. In all 18 patients, a large midline tumor of the posterior fossa (medulloblastoma in nine cases, astrocytoma in five, and ependymoma in four), often attached to one or both lateral recesses of the fourth ventricle, was removed. Mutism developed 18 to 72 hours after the operation (mean 41.5 hours) in patients with no disturbance of consciousness and no deficits of the lower cranial nerves or of the organs of phonation. All of these children had spoken in the first hours after surgery. The disorder lasted from 3 to 16 weeks (mean 7.9 weeks). Speech was regained after a period of dysarthria in six of the 10 cases for whom this information was available. The various hypotheses advanced to explain the pathogenesis of this speech disorder are analyzed.
Adult-onset tethered cord syndrome is uncommon. To date some 90 cases have been reported. We add three personal cases, review the principal features of the syndrome in adults and underline the chief differences between it and the more frequent childhood syndrome.
The epidemiological situation was evaluated in the Nowy Sacz Province in school children and adolescents aged 7, 12 and 14 years in urban and village areas in the school-years 1975-76 to 1985-86. The DMF index, the DMF pattern and the percent proportions of children receiving fluoride prevention of caries were calculated. The number of dentists employed during the period of 10 years was compared. The data are presented in tables. It was found that the proportion of children receiving health care for caries prevention has increased, the mean DMF value in children aged 12 years fell from 4.9 to 4, and favourable changes occurred in the DMF pattern.
Cerebral cavernous angioma is a rare vascular malformation at any age and is very rare in childhood. In the literature available to us, we have been able to trace only 50 cases, to which we have added the 6 cases from our own series. The incidence in pediatric group is higher at 0-2 years (26.8%) and at 13-16 years (35.7%). The clinical onset shows epilepsy in 45.4% of cases, hemorrhagic syndrome in 27.3%, intracranial hypertension in 16.4%, and focal neurological deficits in 10.9%. Furthermore, we discuss the neuroradiological features (CT, angiography, and MRI) and the therapy of pediatric cavernous angioma.
Cerebral meningiomas are rare tumors in children that represent 1.4% of CNS tumors and 1.5% of intracranial meningiomas. We have analyzed 197 cases of cerebral meningiomas under 16: 178 cases were taken from the available literature and 19 from our series. When comparing pediatric meningiomas with those of adults, we noted some differences. Before the age of 16 there is a slight preference for males; the intraventricular variety is more frequent; cystic meningiomas and the absence of dural attachment are more frequent findings; the neuroradiological diagnosis is more difficult.
The clinical findings and the results of surgical treatment in 16 patients with spinal dermoid or epidermoid tumors are reported. In 9 patients the tumor capsule adhered so tightly to the nervous tissue that part of it was left in situ. In a follow-up study ranging from 5 to 30 years with a mean of 14.2 years only 1 patient had a recurrence of the tumor and 10 patients resumed a normal working life.
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Intracranial meningiomas are rare before the age of 16 years. Of 1397 intracranial meningiomas operated on by our service up to 1987, 19 (1.3%) were diagnosed before this age. In the literature accessible to us we have found another 274 cases. An analysis of the literature shows that intracranial meningiomas in paediatric patients differ from those in adults in the following respects: a slight predilection for males, a high frequency within the ventricular system, more frequent presence of intra- and peri-lesional cysts and more frequent absence of dural attachment. In addition, the neuroradiologic diagnosis of nature is more difficult in paediatric patients.
Twenty-two cases of unusual intracranial meningioma associated with a cyst are reported and 144 published cases reviewed. The complex mechanisms leading to the formation of the cyst within the meningioma are analyzed on the basis of the surgical and histopathological evidence. The neuroradiological features (Computed Tomography and Angiography) which supply a correct preoperative diagnosis are discussed. It is clear from the literature how difficult preoperative diagnosis is, being possible only in 38% of cases with CT scanning and in 13% with angiography. It is necessary to remove not only the solid portion of the meningioma but also the cyst wall in order to prevent a recurrence. This is technically difficult for the type II intratumoural variety of Nauta's classification.