Bruce Hendrick

The Tethered Spinal Cord: Its Protean Manifestations, Diagnosis and Surgical Correction

31 children with spina bifida occulta who have presented with back pain, scoliosis, a progressive neurological deficit involving lower limbs or a neurogenic bladder have been found to have a tethered spinal cord. Release of the tethered spinal cord has always relieved pain, frequently occrected a progressive scoliosis, and arrested or improved neurogenic foot deformities as well as neurogenic bladder.

Posterior fossa medulloblastoma in childhood: Treatment results and a proposal for a new staging system

Seventy-two children with posterior fossa medulloblastoma were diagnosed at the Hospital for Sick Children, Toronto, from 1977 to 1987 and treated by standard methods. The 5- and 10-year survival and disease-free survival rates were 71% and 63%, and 64% and 63%, respectively. Total tumor resection, as determined by the surgeon was the most significant favorable prognostic factor. Post-operative meningitis, a residual enhancing mass lesion on the post-operative, pre irradiation CT scan and dissemination to the brain or cord at diagnosis were unfavorable factors. These four easily definable factors were used to define a staging system with prognostic significance. Five-year disease-free survival rates were for Stage I (total resection, no adverse factor) 100%, Stage II (total resection with one or more adverse factor or less than total resection with no other adverse factor) 78%, and Stage III (less than total resection with one or more adverse factor) 18%. Evaluation of treatment results in medulloblastoma requires that these prognostic factors be known.

Hemispherectomy for Sturge-Weber syndrome.

Patients with Sturge-Weber disease who have the onset of seizures in infancy invariably face a progressively downhill course which leaves them severely hemiplegic, demented and usually institutionalized because of uncontrolled seizures. During the past 12 years, we have carried out 6 hemispherectomies in infants under 1 year of age who presented with seizures and Sturge-Weber disease. The results have been gratifying. Seizures have stopped, hemipareses have been minimal and intellectual development has been good.

Optic glioma in children : surveillance, resection, or irradiation ?

Eighty-seven consecutive children with newly diagnosed optic glioma were managed at University of Toronto hospitals 1958-1990. Overall the 10-year survival, relapse-free survival and freedom from second relapse rates were 84%, 68% and 85%. Twenty-seven patients relapsed or progressed, of whom 40% were free of a second relapse 10 years after the first relapse. Fourteen patients had a second relapse. Thirteen are dead. None survived 5 years after second relapse. Patients with anteriorly located tumors (N = 35), which involved the optic nerve, or chiasm and optic nerves, fared better than those with posteriorly located tumors (N = 52) with spread beyond the chiasm, 10-year survival 95% versus 76%, (p = .02), 10-year relapse-free survival 80% versus 59% (p = .02), respectively. For posterior tumors primary irradiation was more effective than primary subtotal resection for prevention of subsequent relapse, 10-year relapse-free survival 75% versus 41% (p = .02), but salvage therapy was, in part, successful and multivariate analysis of prognostic factors influencing survival for posterior tumors indicated that neither primary resection nor primary irradiation were significant factors. For first relapse, primary irradiation and the presence of neurofibromatosis were the significant favorable factors. Since 1977 and for posterior optic glioma subtotal resection or surveillance were used in 21/29 (72%) patients compared with 4/23 (17%) previously. Ten-year survival rates before and after 1977 were 78% and 67% and 10-year relapse-free survival 64% and 56%, respectively.

Pineal region germinomas in childhood treatment considerations

From 1967-1986, 21 children were treated for pineal germinoma, including 16 biopsy-proven, 2 biopsy non-diagnostic, and 3 metastatic unbiopsied (marker negative) patients. Ten of 18 (56%) biopsied patients underwent partial or sub-total tumor resection. Twenty patients were irradiated, 19 of whom are alive. No irradiated patient died of progressive germinoma, but two patients relapsed in the spinal cord and required treatment intensification for salvage. Long-term survivors have significant morbidity. Determination of the minimum effective treatment remains the chief therapeutic challenge.

Intracranial Meningiomas in Children

Thirteen cases of intracranial meningioma presenting in childhood were encountered at the Hospital for Sick Children in Toronto over a 51-year period. The clinical features, radiology, operative findings and outcome are reviewed.

Subtemporal Decompression for the Slit-Ventricle Syndrome after Shunting in Hydrocephalic Children

During the past 2 years, we have performed subtemporal decompression to deal with recurrent shunt obstruction in 22 hydrocephalic patients with the slit-ventricle syndrome. 13 patients have been followed-up for more than 1 year and 2 others for more than 2 years. The frequency of hospitalization for shunt-revision has been greatly reduced. These results confirm that subtemporal craniectomy is useful in the treatment of patients in whom this syndrome develops.

Brain tumors in children: Long-term survival after radiation treatment

PURPOSE To determine the cause of death in children who survive more than 5 years after radiation treatment of a brain tumor. METHODS AND MATERIAL Nine hundred and twelve consecutive children with a primary brain tumor irradiated at the Princess Margaret Hospital or Toronto-Bayview Regional Cancer Center from 1958 to 1991, were evaluated for long-term outcome. RESULTS Overall 10- and 20-year survival rates were 44% and 37%. Subsequent survival of 377 5-year survivors was, at an additional 10 and 20 years, 78% and 67%. Most (83%) deaths that occurred more than 5 years from diagnosis were a result of relapse of the original tumor. The 10-year survival rate subsequent to relapse was 9% when the first relapse occurred less than one year from diagnosis, 17% for 1-2 years, and 31% when the time to relapse was 3 years or greater. The cumulative actuarial incidence of, and death from, second malignant tumors at 30 years from diagnosis was 18% and 13%, respectively. CONCLUSIONS Death later than 5 years from diagnosis of a brain tumor in children is common and is usually due to progressive disease in slowly evolving low grade tumors. Death from a second malignant tumor becomes more frequent than death from the original tumor after 15 years from diagnosis.

Evaluation, surgical approach and outcome of seizure patients with gangliogliomas.

A retrospective study of 15 children with intracranial gangliogliomas and intractable seizures revealed that tumors associated with seizure were located in the temporal and frontal lobes. These patients underwent lobectomy under electrocorticography. Mesial temporal sclerosis was identified in the hippocampus of the excised temporal lobe in 7 of 13 patients with temporal lobectomies. Eleven of the 15 children were seizure-free over a mean follow-up period of 4 years. The surgical approach to gangliogliomas requires careful pre-operative evaluation, including neuroimaging and electrophysiological study to locate seizure activity. Removal of epileptogenic brain as well as the ganglioglioma is recommended to optimize seizure control.

Gangliogliomas in Children

14 children with central nervous system gangliogliomas are reviewed. The tumors were most frequently located in the cerebral hemisphere and often were cystic and calcified. Total removal was performed