✓ A rare case of a primary Ewing's sarcoma arising from the intracranial portion of the right orbital roof is presented. Clinical presentation was that of a rapidly growing tender exostosis, associated with headaches. History indicated that the tumor may have been present for more than 1 year. Skull x-rays showed marked diastasis of the coronal suture and a “mottled” appearance on the right frontal bone suggestive of a sarcoma. Bone scan further confirmed the presence of a malignant bone tumor. Computerized tomography scan showed the intracranial extension of the tumor and a follow-up scan allowed us to see the response of the tumor to therapy. Only two other cases with details as to intracranial localization were found in the literature.
Antonio Alvarez-Berdecia, Luis Schut and Derek A. Bruce
Leslie N. Sutton, Frank Welsh and Derek A. Bruce
✓ The bioenergetic mechanisms of vasogenic edema were studied by measuring concentrations of adenosine triphosphate (ATP), phosphocreatine (CrP), and lactate in rapidly frozen edematous white matter in cats. When edema was produced using a cold lesion, it was found that both ATP and CrP were reduced to one-half of control values, and that lactate was elevated. When a correction was applied for dilution, however, it was found that high-energy phosphates were equal to control values, and that lactate was even more significantly elevated. This pattern contrasted with that seen in white-matter ischemia, in which CrP is depressed out of proportion to ATP. Finally, it was found that the white-matter lactate concentration in the plasma infusion model of edema was increased. It is concluded that vasogenic edema induces an increase in lactate, but does not deplete high-energy phosphate compounds in affected white matter.
Leslie N. Sutton, Derek A. Bruce and Frank Welsh
✓ The electrophysiological effects of cold-lesion edema and white-matter ischemia were studied in cats by reference to the short-latency somatosensory evoked response. The primary cortical waves were found to be considerably delayed following a period of white-matter ischemia; however, cold-lesion edema appeared to have no significant effect on the evoked response. The authors conclude that vasogenic edema does not interfere with axonal functioning by an ischemic mechanism.
Leslie N. Sutton, Debra Gusnard, Derek A. Bruce, Arno Fried, Roger J. Packer and Robert A. Zimmerman
✓ Between 1982 and 1990, a series of 31 children with craniopharyngiomas underwent initial surgery at the Children's Hospital of Philadelphia with an attempt at total tumor removal. Nine (29%) of them were found to have fusiform dilatation of the supraclinoid carotid artery either at the time of surgery for recurrence (one patient) or on routine surveillance with enhanced computerized tomography 6 to 18 months postoperatively (eight patients). The finding of carotid enlargement was confirmed in seven cases with magnetic resonance angiography and in one case with a formal arteriogram. Eight of the nine patients remain alive at a mean of 3.7 years after diagnosis. None have experienced hemorrhage or other symptoms referable to fusiform dilatation of the carotid artery, which is believed to result from surgical manipulation of the carotid artery.
Derek A. Bruce, Luis Schut, Leonard A. Bruno, James H. Wood and Leslie N. Sutton
✓ The outcome in 53 children following severe head injury is presented. All children were graded using the Glasgow Coma Scale; 90% made a good recovery or were moderately disabled, and 8% died or were left vegetative. All patients were treated with controlled ventilation and steroids; mannitol, and, if necessary, Nembutal (pentobarbital) were used to maintain the intracranial pressure below 20 torr. With this regimen, only one death occurred due to uncontrollable intracranial hypertension. All patients with a coma scale of 5 or greater recovered well. The worst prognostic sign was the presence of flaccidity: 33% of these patients died or were vegetative. Five of seven patients who were decerebrate or flaccid with bilateral fixed pupils and absent caloric responses made a good recovery or were moderately disabled. The relatively low incidence of mass lesions (23%) and high incidence of diffuse cerebral swelling (34%) suggest a different pathophysiological response of the child's brain to injury, which may play a role in the improved survival of children following severe head injury when compared to adults.
William O. Bell, Edward B. Charney, Derek A. Bruce, Leslie N. Sutton and Luis Schut
✓ Twenty-two patients with closed myelomeningoceles, shunted hydrocephalus, and symptomatic Arnold-Chiari malformations were studied retrospectively. Seventeen of the 22 patients were aged 6 months or younger; five patients were aged 3 to 23 years. Patients in the younger group presented with stridor, apnea, and/or feeding difficulty; those in the older group presented with hemiparesis, quadriparesis, oscillopsia, nystagmus, or opisthotonos. Fourteen of the 17 younger patients underwent surgical decompression of the Arnold-Chiari malformation: 10 within 18 days following the onset of symptoms and four on Day 19 or later. Of the 10 infants with early treatment, five eventually died secondary to continued symptoms and five survived. Of those surviving, three were asymptomatic and two had continuing symptoms but were improved over their preoperative state. Among the four patients undergoing surgery later, two died, one had lessening of stridor, and one had complete relief of symptoms. Of the three infants not undergoing decompression, two died and one eventually became asymptomatic. All five of the older patients underwent decompression and all had complete resolution of their preoperative symptoms and signs. The authors conclude that while decompression of a symptomatic Arnold-Chiari malformation may be effective in children and adults, this treatment does not always improve the clinical condition of infants. This may be the result of ischemic/hypoxic effects on the infant's brain stem, which may not be organized normally at birth.
A preliminary report
Jeffrey G. Rosenstock, Roger J. Packer, Larissa Bilaniuk, Derek A. Bruce, Jerri-Lynne Radcliffe and Peter Savino
✓ Chiasmatic optic glioma is a rare tumor with an erratic natural history, usually seen in young children. A prior study from this institution demonstrated that these lesions were frequently lethal, despite initial clinical stabilization following radiation therapy, and that visual, intellectual, and late endocrinological disabilities were prevalent. A novel approach was developed in 1977, when an initial clinical response to vincristine was recorded in a child with a recurrent optic glioma. Since then, all children with recurrent optic glioma and all children aged 6 years old and under with newly diagnosed optic glioma have been offered a program of initial therapy with vincristine and actinomycin D for six cycles over 18 months. The four children with recurrent tumor who were treated with that regimen remain clinically stable 13 to 115 months after chemotherapy. Twelve children (eight under 24 months old) with newly diagnosed optic glioma have been treated with this program, and three are still on therapy. Four developed progression while on therapy, and five remain stable from 1 to 60 months posttherapy. The four children who developed progressive disease have been treated with radiation therapy and remain stable. Six of the 12 children showed shrinkage of their tumor on computerized tomography while receiving chemotherapy. This program may serve as an alternative to initial radiation therapy in young children.
Case report and review of the literature
Henry E. Aryan, Hal S. Meltzer, Michael L. Levy, Bartek J. Szkandera and Derek A. Bruce
✓ The authors report the case of a hypothalamic ganglioglioma with left-sided temporal lobe extension in an 8-year-old girl who presented with seizures. Other cases of ganglioglioma involving the hypothalamus have been reported in the literature; however, this site of origin is exceedingly rare and worthy of report.
Treatment involved medial temporal lobectomy with the hypothalamic component of the tumor remaining untouched. The patient recovered postoperatively with no neurological deficits and was seizure free at 12 months. Neither radio- nor chemotherapy was recommended because of the tumor histology, location, and the patient's age. The authors recommend follow up and surgical treatment for possible tumor recurrence. The prognosis for hypothalamic ganglioglioma is unknown.
Leslie N. Sutton, Tim Frewen, Roger Marsh, Jurg Jaggi and Derek A. Bruce
✓ The authors report their investigation of the effects of high-dose barbiturates on the multimodality evoked response in 9 cats. After baseline evoked responses were obtained, boluses of pentobarbital were infused intravenously at regular intervals, amounting to cumulative total doses of 9, 18, 27, 45, 63, 123, and 183 mg/kg at respective infusions. This resulted in gradually increasing serum pentobarbital levels, reaching therapeutic coma levels (4 to 5 mg/dl) after the fifth infusion. At this point, the electroencephalogram was flat, and pressor agents were required to maintain cardiovascular stability. Evoked responses were obtained 15 minutes after each infusion. Brain-stem auditory evoked response (BAER) showed little change in wave latencies at therapeutic coma levels of pentobarbital. Further barbiturates resulted in delay of the late components of this response. In the somatosensory evoked responses (SER), early brain-stem components were relatively unaffected by therapeutic coma levels. Late brain-stem components and the initial cortical response showed progressive latency increase. Late cortical (association cortex) waves were abolished at relatively low doses. The central conduction time was relatively unaffected. The late waves of the visual evoked responses (VER) were abolished with low-dose barbiturates (9 mg/kg). A single positive-negative complex persisted despite massive infusions. It is concluded that evoked responses may prove useful in monitoring patients in deep barbiturate coma, but barbiturate effects must be kept in mind.
Derek A. Bruce, Abass Alavi, Larissa Bilaniuk, Carol Dolinskas, Walter Obrist and Barbara Uzzell
✓ The commonest initial computerized tomography (CT) finding in head-injured children is bilateral diffuse cerebral swelling. Cerebral blood flow and CT density studies suggest that this swelling is due to cerebral hyperemia and increased blood volume, not to edema. The clinical history, course, and outcome of 63 children with this CT pattern are reviewed. Fourteen children had a Glasgow Coma Scale score of greater than 8; all made a complete recovery and follow-up CT scans were normal. Forty-nine children had Glasgow Coma Scale scores of 8 or less. Fifteen had a history of a lucid period following the initial unconsciousness. One of these children died of delayed brain swelling, the others recovered well with minimal neurological deficit. Thirty-four children were rendered immediately and continuously unconscious. There was a high incidence of second lesions on the CT scan, 50% of this group developed intracranial hypertension and five died. All of the others were in coma for periods ranging from weeks to months. Follow-up CT scans showed an extracerebral collection with a density of cerebrospinal fluid in 27% of the patients, and ventriculomegaly with large sulci in 35%, whereas this pattern was seen only once in those with a lucid period. The difference between those with and without a lucid period is related to the degree of primary diffuse impact injury to the white matter.