Biomedical subjects
E C Ventureyra
Publications and source records attributed to E C Ventureyra.
Intracranial plasma cell granuloma.
Plasma cell granulomas (PCGs) are benign, inflammatory masses of unknown etiology composed of polyclonal mature plasma and lymphoid cells. The lung is their most common location, and occurrence within the cranial cavity is extremely rare. We report the case of an 11-year-old girl who presented with seizures and was diagnosed as having a dural-based right frontal tumor that extended toward the sagittal sinus and the falx. The lesion was totally excised, together with the abnormal dura. A limited cortical excision was also performed using electrocorticographic guidance. Histopathologic diagnosis of intracranial plasma cell granuloma was reached after extensive immunohistochemical tests and electron microscopy. This is the third case of PCG with description of changes in the neighboring cerebral tissue. Although PCGs are well-circumscribed lesions, lymphoplasmocytic inflammation, neuronal loss, and reactive gliosis occur within the adjacent cortex. Disturbed cortical lamination, as we have observed, appears to be a histological finding that has not been described previously.
The preoperative assessment in the planning of surgical management of medically refractory epilepsy in the pediatric patient.
Surgical intervention in refractory epilepsy in children offers the hope of seizure control. For the patient, the preoperative assessment is a major factor in whether he or she is selected as a patient who will potentially benefit. It also plays an important part in the choice of the surgical intervention to be used. This paper reviews the investigations available and suggests an investigational algorithm for use in the pediatric population.
Transcutaneous vagus nerve stimulation for partial onset seizure therapy. A new concept.
A new concept for transcutaneous vagus nerve stimulation for control of partial onset seizures is described. The rationale for the application of this innovative noninvasive method is discussed.
Temporal lobe focal cortical dysplasia: MRI imaging using FLAIR shows lesions consistent with neoplasia.
Focal cortical dysplasia (FCD), a form of neuronal migration disorder, is a malformative lesion of the neocortex that occurs during development of the brain. It can cause partial and generalized epilepsy. Seizures occur at an early age and are often resistant to medication. Surgical resection has been found to be beneficial in these patients. Dual pathology, in the form of mesial temporal sclerosis, has been associated with FCD. At the Children's Hospital of Eastern Ontario, four patients with temporal lobe FCD have recently, been identified. This paper discusses how these children presented and how they were managed, with particular emphasis on their MRI findings and differential diagnoses. In three of the four patients neuroimaging studies showed lesions consistent with a neoplastic process because of the large volume and mass effect. Radiologically, FCD may mimic the MRI appearance of tumors, such as dysembryoplastic neuroepithelial tumors, primitive neuroectodermal tumors, gangliogliomas, oligodendrogliomas, and astrocytomas. These lesions are best visualized on fluid-attenuated inversion recovery (FLAIR) imaging, a technique that has recently become applicable in the clinical setting, as we help demonstrate in this series. With better MRI capability, milder forms of FCD and microdysplasia may be distinguished.
Medulloblastoma in children--the Ottawa experience.
A retrospective review of 36 children diagnosed with medulloblastoma in the Ottawa area between 1974 and 1997 was completed (mean age 7.8+/-4.2 years, range 1.2-15.3 years). Via a suboccipital approach, complete tumor resection was achieved in 75% and subtotal resection (>90%) in 25%, without any operative mortality. The tumor was located in the vermis in 39% and in the cerebellar hemisphere in 11%; it occupied both locations in 50%. In 47% of the children a ventriculoperitoneal shunt was required. Postoperatively, craniospinal radiation at 3600 cGy with a boost to the posterior fossa was administered. Chemotherapy was used in 56%. The 1-year survival rate was 92%, and survival plateaued at 54% at 5 years. Children less than 3 years of age fared worse than those over 3 years old. While the male-to-female ratio was 1.6:1, there was no gender difference in survival. Chang's classification was used to grade the tumors. T stage did not have an impact on survival, but M stage did. No statistically significant difference in survival was found between the patients who had a total resection and those who had a subtotal resection. There was no difference in survival in terms of tumor location, hydrocephalus or ventriculoperitoneal shunt. Chemotherapy showed no survival benefit. The recurrence rate was 26%, and its timing followed Collin's law. Recurrence led to death within 1-9 months. GH deficiency was diagnosed in 5 patients and hypothyroidism in 4 patients. The mean follow-up time was 4.4+/-3.7 years, with a range of 2.5 months to 16.5 years. Fourteen patients died, 5 were lost to follow-up, and 7 were transferred to adult care without persistent disease. Ten children are presently being followed up by the Neuro-oncology Clinic. Four children continue to be followed through psychology services. Our results are comparable to those in larger series, and are similar to those of the Montreal Children's Hospital.
Electrocorticography.
Electrocorticography (ECOG), the intra-operative recording of cortical potentials, has played an important role in the surgical management of patients with medically refractory epilepsy. It has been used 1) to localize the epileptogenic tissue; 2) map out cortical functions; and 3) predict the success of the surgery. Despite its common use, few studies have been done to prove its effectiveness in these areas. The technique used in children for recording ECOG is very similar to that used in adults except for the limitations imposed by the child's age. Anaesthesia must often be used. Based upon a computerized medical literature search, a review of this procedure was done. Pre-resection localization, and post-resection prediction of outcome was done for temporal and extra-temporal resection, both lesional and nonlesional. Most of the available studies were in adult patients. All were retrospective in nature. Evidence for the role of pre-resection ECOG in determining the degree of resection felt necessary to obtain good clinical outcome was limited. Similarly the post-resection ECOG predication of surgical outcome was restricted.
Predictors of seizure outcome following cortical resection in pediatric and adolescent patients with medically refractory epilepsy.
Several preoperative clinical variables have been reported to have value as predictors of seizure outcome following the surgical resection of epileptogenic focus in adults who have had medically refractory epilepsy. The present paper reports the results of a retrospective review of the ability of these variables to predict seizure outcome in a group of pediatric patients who had medically refractory epilepsy and underwent surgical resection of an epileptogenic focus at the Children's Hospital of Eastern Ontario. Variables in this review included age at first seizure, age at time of surgery, duration of seizures, seizure type, sex, family history, etiology, level of intelligence, EEG data, results of imaging studies, findings on examination of the central nervous system, and location and site of surgical resection. We found 64 patients who met the entry criteria for this review. Normal intelligence and tumor as etiology were associated with a good postoperative seizure outcome in patients who had a temporal resection; no variables had positive correlation with outcome in the extratemporal group. Caution must be used in the extrapolation of data and inclusion of studies of predictors of seizure outcome for adults to pediatric age groups.
Epilepsy surgery for 5- to 18-year old patients with medically refractory epilepsy--is it cost efficient?
This study was designed to examine the question of whether cortical resection for children and adolescents with medically refractory epilepsy was less costly and more effective than the medical alternative. The study design used a decision analysis model. Data on outcome probabilities were based on literature review and local experience. The time span of the study was 25 years. Though the initial costs for the surgically treated group were significantly greater than those for the medical group, the time-cost lines intersected at 14 years after surgery. Surgery remained cheaper thereafter.
Hydrocephalus and epileptic seizures.
Is there an association between shunted hydrocephalus and the development of epileptic seizures? To answer this question a retrospective review of the medical records of 197 patients with shunted hydrocephalus was undertaken. In this series 17% of patients with hydrocephalus developed seizures. No correlation was found between the occurrence of epileptic seizures and a shunt malfunction, the number of shunts placed, the age of the patient at the initial shunt procedure or the location of the shunt. Patients with hydrocephalus who had significant cognitive delay or significant motor disability were significantly more likely to develop seizures than patients who did not. The findings of this review support the hypothesis that the occurrence of seizures in children with hydrocephalus is related to an underlying diffuse encephalopathy and not to the hydrocephalus or to procedures related to the treatment of this disorder.
Epidermoid cysts of the pineal region.
Localization of epidermoid cysts to the pineal region is rare. The 7-year-old boy now reported presented with an 18-month history of progressive ataxia. CT and MRI scans demonstrated a 2.5x2.5 cm cyst at the pineal region with obstructive hydrocephalus. At surgery via an occipital transtentorial approach, a characteristic "pearly tumour" was encountered, and complete resection was achieved. We present the management of this child with pineal region epidermoid cyst and review 11 cases reported in the literature since 1968. In all, 8 of the 12 patients were males. The age at the time of diagnosis ranged from 7 years to 69 years. Parinaud's syndrome and hydrocephalus are the most common presenting findings. All but 1 patient underwent direct surgical resection; 1 had stereotactic decompression. Surgical treatment brought about complete resolution of the presenting symptoms and signs in 10 of the 12 cases. One patient had persistent upgaze palsy. One patient died from progression of the pineal region mass. This patient presented with hemiparesis, which is a marker of clinical aggressiveness. The authors advocate direct surgical attack as opposed to stereotactic diagnosis and aspiration to: (1) obtain maximal resection and thereby limit the potential for recurrence and delayed complications of the cyst; (2) possibly avoid shunt placement in patients who present with hydrocephalus; and (3) decrease the likelihood of sampling error.
Latex allergy in neurosurgical practice.
The possibility of latex allergy and the implications of the potential life-threatening allergic reactions among patients (for example those with spina bifida), health care professionals, and latex industry workers are discussed. Latex allergy is becoming increasingly widespread in medical and surgical practice. Although early reports of latex allergy date from 1927, only over the last decade has more attention been paid to this condition. This is due to an increasing number of reported cases of adverse reactions to latex, varying in severity from mild to fatal. Latex allergy has become a major problem in children with spina bifida, who need to undergo many major aggressive diagnostic and therapeutic procedures. Risk groups have been identified and include patients having multiple surgical procedures, such as those with spina bifida. In this review we aim to emphasise some aspects of the current management of surgical patients with latex allergy.
Epilepsy surgery for 5- to 18-year old patients with medically refractory epilepsy - is it cost efficient?
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A different kind of wisdom.
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Long-term socioeconomic outcome following surgical intervention in the treatment of refractory epilepsy in childhood and adolescence.
Surgical treatment of refractory epilepsy in childhood and adolescence has been shown to be effective in reducing the seizure frequency. This paper examines the question: "Does this result in a better socioeconomic outcome in later years?" Patients who underwent a surgical procedure for the treatment of their medically refractory epilepsy at our hospital, had more than 2-years' follow-up, and were less than 18 years old at time of survey were included. From a retrospective chart review, age at onset and at surgery, duration of seizures prior to surgery, years of follow-up, type of surgery, and neurological status were obtained. From a telephone survey, seizure frequency after surgery, marital, financial and driving status, level of education, and employment status were ascertained. Sixty-four patients in our epileptic surgical series meet entry criteria. Significantly higher levels of education, employment status and independence were found in patients with a class I Engel outcome compared to other Engel outcomes.
Unusual association of intractable temporal lobe seizures and intracranial aneurysms in an adolescent: is it a coincidence?
Intracranial aneurysms in the pediatric age group are rare occurrences. They usually present with subarachnoid hemorrhage or mass effect. Their association with epilepsy has rarely been reported; such concurrence may not be a coincidence. We present a 16-year-old girl with a 5-year history of medically intractable complex partial seizures. Preoperative electrophysiological and neuroimaging studies demonstrated an epileptogenic focus and atrophy in the right mesial temporal lobe, and ipsilateral incidental aneurysm at the carotid artery bifurcation. The patient underwent a complete right anterior temporal lobectomy, followed by clipping of the aneurysm. We concluded that the epilepsy management in association with cerebral aneurysms is controversial, but when surgery is indicated, clipping of the aneurysm and resection of the epileptogenic focus may provide the optimal outcome. The relevant literature is reviewed and the possible mechanisms of production of epilepsy by intracranial aneurysms are discussed.
Spontaneous intracranial hemorrhage of structural origin during the first year of life.
Symptomatic intracranial hemorrhage (ICH) in term infants is not common, but when it occurs it is usually secondary to trauma, coagulation disorders and/or hypoxia. The possibility of a structural cause for an infantile ICH is unfortunately not seriously considered until very late. In this paper we report the cases of five full-term infants, each of whom developed ICH secondary to a structural lesion during the 1st year of life. Three presented during the newborn period. A congenital saccular aneurysm of the middle cerebral artery in an 8-month old male infant; a posterior fossa arteriovenous malformation in a 2-week old female neonate; a deep parietal cavernous angioma in a 6.5-month-old male infant; a temporoparietal low-grade astrocytoma in a 12-day old male neonate and a temporoparietal desmoplastic ganglioglioma in a 9-day-old male neonate were the structural lesions that were causative for hemorrhage. In all cases but one, the diagnosis was reached by computerized tomography and/or magnetic resonance imaging. All infants underwent surgery for the removal of the hematoma and of the lesion causative for the bleed. All are alive at 19, 3, 11.5, 10, and 5 years, respectively. We discuss the diagnosis of ICH with special emphasis on contemporary imaging modalities and stress the benefits of aggressive and timely surgical treatment. We then consider a concise analysis of the world literature on the occurrence of structural causes of ICH during infancy.
Outcome and life prospects after surgical management of medically intractable epilepsy in patients under 18 years of age.
A retrospective analysis of seizure outcome and quality of life assessment was done in 64 patients under 18 years of age with medically refractory epilepsy who underwent 64 primary and 16 repeat operative procedures in an attempt to control their epilepsy. At least 2 years' follow-up data were available for each patient. Operative procedures were 44 temporal lobe resections; 16 extratemporal resections; and 4 hemispherectomies. Effective control of previously intractable seizures was obtained in most patients: 55%, 11%, and 17% achieved Engel class I, II, and III status, respectively. Successful seizure control was thus obtained in 83%, while 17% (Engel class IV) failed to improve significantly after operation. Quality-of-life measures parallelled the improvements in seizures control, being highest in Engel I, outcome group and lowest in Engel IV outcome group. In appropriately selected pediatric and adolescent patients with medical refractory epilepsy, surgical management can offer a safe and effective adjunct to medication.