Tuberous Sclerosis: Diagnostic and Surgical Considerations

McLaurin, Robert L.; Towbin, Richard B.

doi:10.1159/000120217

Cited by 19 publications

(9 citation statements)

References 3 publications

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“…The imaging characteristics of tuberous sclerosis have been well described [3,16,24] and are similar to our find ings. CT scans tend to demonstrate an isodense to slightly hypodense intraventricular tumor that has variable calcifi cation.…”

Section: Discussionsupporting

confidence: 88%

“…This may reflect the more successful opening of the fora men of Monroe because of better visualization, or the cal losal dissection itself may allow for ventricular decompres sion into the subarachnoid space. Lastly, as we gained experience with this approach for these and other tumors, we found the perioperative morbidity to be less than with the transcortical corridor [16,27], There have been many different terms used for this tumor in the past, including astrocytoma, embryonal glio ma, spongioblastoma, neurinoma, ependymoma, and spongioneuroblastoma. Despite uncertainty regarding the cell of origin, the histology is quite characteristic [28].…”

Section: Discussionmentioning

confidence: 96%

“…We currently obtain an MRI preoperatively, immediately postoperatively, and then yearly for surveillance. The best operative approach is arguable, and there are proponents of both the transcor tical and the transcallosal approaches [13,15,16,18,19,23,27], We currently favor the transcallosal approach in most instances. Since these tumors are frequently large enough to cross the midline, the ease with which one can gain access to both lateral ventricles via this approach is helpful.…”

Section: Discussionmentioning

confidence: 99%

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Subependymal Giant Cell Astrocytomas in Children

et al. 1994

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Between 1977 and 1991, at the Children’s Hospital of Philadelphia, 10 patients, 5–16 years of age, were diagnosed as having subependymal giant cell astrocytomas. These patients accounted for 1.4% of all pediatric brain tumors seen during that time interval. One patient received a course of radiation therapy, which was ineffective in preventing tumor growth. All underwent surgical resections with the goal being maximal tumor debulking, if not complete resection. In 6 patients this was accomplished by the frontal transventricular route and, in the more recent patients, surgery was performed using a transcallosal approach. There were 2 perioperative deaths, and 2 other patients died later of causes unrelated to tumor progression. The remaining 6 patients remain alive and stable at a mean of 6.7 years of follow-up (range 1.8–12.4). None of these patients has received additional radiation therapy. Two patients have no other evidence of tuberous sclerosis. The use of modern radiographic and surgical techniques has made the treatment of this disease safer than in the past.

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Section: Discussionsupporting

confidence: 88%

Section: Discussionmentioning

confidence: 96%

Section: Discussionmentioning

confidence: 99%

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Subependymal Giant Cell Astrocytomas in Children

et al. 1994

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“…Cortical tubers, the most frequently encountered brain lesions, are formed by a variable amount of heterotopic neurons, disordered glial cells, and calcifications [7]; the periventricular lesions are characterized by subependymal neuronal and glial cells [8]. In about 15% of the cases, a subependymal lesion, usually located at the level of the foramen of Monro near the head of the nucleus caudatus, has the tendency to increase in volume [9], These lesions, so-called 'subepen dymal giant cell astrocytomas' for their histological fea tures, can often cause hydrocephalus by obstructing the CSF pathways [10,11]. When an obstmetive hydrocepha lus is present, the neurosurgeon's task is to correct the impaired spinal fluid circulation; authors' opinions di verge concerning the best surgical approach.…”

Section: Introductionmentioning

confidence: 99%

On the Treatment of Subependymal Giant Cell Astrocytomas and Associated Hydrocephalus in Tuberous Sclerosis

Rocco

Iannelli²,

Marchese³

1995

Pediatr Neurosurg

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Between 1980 and 1992, 10 children affected by tuberous sclerosis and intraventricular subependymal giant cell astrocytomas were surgically treated at the Institute of Neurosurgery, Section of Pediatric Neurosurgery, Catholic University of Rome. Nine patients presented with signs and/or symptoms of intracranial hypertension; in all of them the neuroradiological investigations demonstrated the presence of a space-occupying lesion in the region of the foramen of Monro with secondary ventricular dilation. In the remaining patient, a 5-month-old male infant, an intraventricular mass was discovered by means of an ultrasound examination performed after the first epileptic fit. Three patients underwent a ventriculoperitoneal CSF shunt as first surgical procedure; in 2 of them it was subsequently necessary to remove the intraventricular tumor due to the frequent occlusion of the CSF shunt device. Seven subjects underwent the direct surgical excision of the lesion. In all of them the procedure resulted in the control of the associated hydrocephalus. On the basis of such an experience, the authors conclude that the surgical removal of the intraventricular tumors in patients with tuberous sclerosis and hydrocephalus is the most appropriate treatment. In fact, in the series considered here, the removal of the tumor was not accompanied by significant morbidity, and was followed by improvement in clinical conditions. In particular, in cases in whom the occurrence of hydrocephalus was associated with a worsening in the seizure disorder, the tumor removal and the correction of intracranial hypertension was followed by a significant reduction in frequency or even by the disappearance of the seizures. However, in no case presenting with mental impairment was a significant improvement observed in mental performances as a consequence of the surgical treatment.

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“…21,[74][75][76] Multidisciplinary discussion agreed that neither CT nor MRI are ideal for patients with TS and, as long as behaviour and fits are monitored for changes which would then lead to further investigation, no regular neuro-radiological follow up is necessary. 74,77 However, each patient should have a CT/MRI carried out for baseline reference. 21 Carers should be made aware of warning signs, eg worsening fits, increased headaches, changes in behaviour, or nausea.…”

Section: Patients With Ts and Dyspnoea Or Pneumothoraces Should Be Rementioning

confidence: 99%

Guidelines and care pathways for genetic diseases: the Scottish collaborative project on tuberous sclerosis

et al. 1998

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In Scotland a national audit project has been undertaken to devise evidencebased guidelines for the clinical management of patients with tuberous sclerosis (TS), a dominantly inherited multisystem disorder. In order to facilitate the audit and use of these guidelines a 'Care Pathway' was devised to form the patient records. We describe the process of guideline development for TS and our TS Care Pathway.

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Tuberous Sclerosis: Diagnostic and Surgical Considerations

Cited by 19 publications

References 3 publications

Subependymal Giant Cell Astrocytomas in Children

Subependymal Giant Cell Astrocytomas in Children

On the Treatment of Subependymal Giant Cell Astrocytomas and Associated Hydrocephalus in Tuberous Sclerosis

Guidelines and care pathways for genetic diseases: the Scottish collaborative project on tuberous sclerosis

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