Edwin G. Fischer

20-year experience in childhood craniopharyngioma.

PURPOSE The management of craniopharyngioma is controversial, and surgery alone is frequently advocated. The purpose of this study was to assess the long-term impact of various treatments in childhood craniopharyngioma. METHODS AND MATERIALS Sixty-one children < or = 21 years of age at diagnosis were treated for craniopharyngioma at Childrens Hospital and the Joint Center for Radiation Therapy in Boston from 1970 to 1990. The median age was 7.5 years (range 10 months-21 years). There were 33 females and 28 males. The median follow-up was 10 years (range 2-20.5 years). Neuroimaging was available for detailed review in 53. Nine children were treated with radiotherapy alone, 15 were treated with surgery alone, and 37 were treated with both surgery and radiotherapy. All patients in the radiotherapy and surgery plus radiotherapy groups were treated with megavoltage radiation with a median dose of 5464 cGy. RESULTS All nine of the children treated with radiation therapy alone are alive; none have recurred. Nine of the 15 children treated with surgery alone have recurred (p = 0.007 Fisher exact test). Two are alive with disease, and seven are alive without disease after treatment at relapse with radiation therapy, surgery, or both. Seven of the 37 patients treated with surgery plus radiotherapy have recurred. Three of the seven patients are dead of disease, three patients are alive with disease, and one patient is alive without disease after further treatment. The 10-year actuarial overall survival was 91% for all patients. The 10-year actuarial freedom from progression for the surgery group was 31% compared with 100% for patients treated with radiation therapy only (log rank p = 0.01), and 86% for patients treated with surgery plus radiotherapy at diagnosis (p = 0.001). There were two treatment related deaths, both in the surgery plus radiotherapy group. A higher incidence of visual loss and diabetes insipidus was associated with the use of aggressive surgery. The size of the tumor at presentation correlated with an increased risk of recurrence; 5 of 6 patients with tumors > or = 5 cm experienced recurrences while only 6 of 30 recurred when the tumor was < 5 cm. CONCLUSIONS Overall survival in childhood craniopharyngioma is excellent. However, patients treated with surgery alone have a significantly worse freedom from progression when compared to patients treated with surgery and radiation therapy or radiation therapy alone.

Reassessment of cerebral capillary changes in acute global ischemia and their relationship to the "no-reflow phenomenon".

Electron and light microscopic studies were performed on rabbit brain to re-examine the structural changes of endothelial cells and perirascular glia following ischemia. Although swelling of perivascular glia occurred, earlier findings of extreme perivascular glial swelling and bleb formation leading to luminal collapse and plugging could not be confirmed. Ischemic brains, however, had a higher proportion of small-diameter capillaries than controls. It is felt that structural changes in ischemic capillary walls in themselves are not sufficient to explain failed cerebral reperfusion, or the no-reflow phenomenon.

Neurourological Implications of the Changing Approach in Management of Occult Spinal Lesions

Occult lesions of the spine in children are a rare but recognizable cause of neurogenic dysfunction involving the lower extremities, and lower urinary and gastrointestinal tracts. We report the preoperative and postoperative urodynamic findings in 40 children with varying spinal abnormalities. Of these patients 28 were neonates or infants (average age 8.7 months) and 12 were older children (average age 11.7 years). Preoperative urodynamic testing revealed normal function in 18 of 28 children (64 per cent) in the younger age group in contrast to 1 of 12 (8 per cent) in the older age group. Of the 10 infants with abnormal studies postoperative urodynamic findings returned to normal in 6, while 2 others remained abnormal but were improved. In contrast, of 11 older children with abnormal preoperative evaluations 3 (27 per cent) reverted to normal postoperatively. The neurourological changes seen in these occult lesions are variable, may occur at any age, are progressive and are potentially reversible by surgical correction but this reversibility diminishes with age.

Posterior fossa decompression for Chiari I deformity, including resection of the cerebellar tonsils

This is an analysis of 19 consecutive cases of symptomatic patients with Chiari I deformities, undertaken to evaluate the long-term effect of posterior fossa decompression and duraplasty, assessed by postoperative imaging. Sixteen of the patients had syringomyelia and three had foramen magnum syndromes without a syrinx. Eighteen patients underwent posterior fossa craniectomy, subpial resection of the cerebellar tonsils, and duraplasty. Four patients were 16 years of age or younger. One of the children with syringomyelia had a posterior fossa decompression without resection of the tonsils. In the 15 patients with syringomyelia whose surgery included resection of the tonsils, the syrinx was reduced or resolved in 14. The patient whose syrinx did not change was a child with a lumbosacral lipoma. Three patients had syndromes of the foramen magnum without a syrinx, and of these only a patient with prior chemical and bacterial meningitis caused by a lumboureteral shunt failed to improve dramatically. When our patients are combined with 40 in the literature treated by decompression and duraplasty, 51 of 55 patients had reduction or resolution of the syrinx. Although it does not clearly affect the result, resection of the tonsils can be done safely.

Spinal Epidural Abscess in Children

This is a report of three children with spinal epidural abscess. The literature is reviewed and the features of this condition in children are noted. Because of the nonspecificity of presenting symptoms in children, the diagnosis may be delayed, resulting in a worse outcome, especially in children under 1 year of age. The extensive laminectomy advised for the treatment of spinal epidural abscesses in adults is undesirable in children because of the risk of spinal deformity and in most cases is probably not necessary.

Risk Factors for Brain Abscess in Patients with Congenital Heart Disease

Brain abscess remains a serious complication of congenital heart disease. To determine risk factors for brain abscess in patients with this condition, data in 26 cases of cyanotic congenital heart disease and brain abscess studied between 1960 and 1973 were reviewed and compared with data in a control group with cyanotic congenital heart disease without brain abscess. Brain abscess occurred in 2 percent of the population with cyanotic congenital heart disease. Tetralogy of Fallot and dextrotransposition of the great arteries accounted for 81 percent (21 of 26) of the cases. The mean arterial oxygen saturation in the patients with brain abscess was 75 percent compared with 86 percent in the control patients (P < 0.01). Among the patients with brain abscess, the survivors (16 of 26) had a mean oxygen saturation of 81 percent whereas those who died had a mean oxygen saturation of 64 percent (P = 0.01). The overall mortality rate in the patients with brain abscess was 38 percent (10 of 26). Among survivors, 54 percent (8 of 15) had complete recovery and 46 percent (7 of 15) had residual neurologic impairment. We conclude that the morbidity and mortality of brain abscess are inversely related to oxygen saturation levels. Since brain abscess is exceedingly rare in patients under age 2 years, corrective surgery before this age would probably reduce the incidence of brain abscess in patients with congenital heart disease.

Cerebral blood flow immediately following brief circulatory stasis.

Cerebral blood flow was studied in rabbits immediately following complete circulatory stasis of varying duration. Systemic arterial pressure was measured continuously. The postischemic circulation was examined both by an infusion of carbon black and, in separate experiments, by injection of 14C-antipyrine into the blood. We examined the relationship between the duration of stasis, the postischemic arterial pressure, and the amount of cerebral reperfusion. As stasis increased from 5 to 30 min the pressure required to achieve reperfusion of the entire brain rose from 20 to 100 torr. Following even temporary exposure to arterial pressures above 110 torr all areas of the brain were generally reperfused. Blood flow in reperfused brain varied directly with arterial pressure, indicating failure of autoregulation. At normal (preischemic) arterial pressure, postischemic cortical flow was twice the normal rate. The data indicate that the pressure required to initiate flow in ischemic brain increases as the duration of stasis is lengthened and that once flow occurs there will be a significant hyperperfusion unless systemic arterial pressure is lowered to the low normal or hypotensive range.

PRE-RADIATION CHEMOTHERAPY FOR INFANTS AND POOR PROGNOSIS CHILDREN WITH MEDULLOBLASTOMA

Beginning in 1984, we started a prospective study to evaluate the role of postoperative, pre-radiation chemotherapy in the treatment of infants and poor prognosis children with medulloblastoma. The study was designed to evaluate the role of pre-radiation chemotherapy in two specific patient populations: (a) children under the age of 2 years in which there was an attempt to delay definitive radiation and thus reduce the risk of toxicity to the developing nervous system; and (b) children over age 2 years with Stage T3 and T4 disease who were known to have a relatively poor prognosis with surgery and radiation. The five patients under age 2 years received cisplatinum (100 mg/m2) every 3 weeks and weekly vincristine (1.5 mg/m2) for a total of 9 weeks. Nitrogen mustard (6 mg/m2), procarbazine (100 mg/m2), and vincristine (1.5 mg/m2) (MOP) were given in 28 day cycles as long as there was no disease progression or until the childs second birthday, at which time the children were referred for radiation therapy. The 13 patients over 2 years of age received the 9 week course of cisplatinum and vincristine and then began radiation. Responses measured by computed tomography were obtained in 10 of 12 children with measurable disease at the start of chemotherapy. With a median follow-up of 22 months, 15 of 18 children were alive and free of disease. Except for mild ototoxicity in one child, the acute side effects have been well tolerated. In conclusion, it appears that some infants can have their radiation delayed until the age of 2 years. Although the follow-up time was short, all but three patients were free of disease, time exceeding the median time to failure with radiation alone. Pre-radiation chemotherapy might improve local control and survival in children with advanced stage medulloblastoma.

Cavernous hemangioma of the thoracic spinal cord

SummaryA 25-year-old woman presented with a four-year history of progressive right-lower-extremity weakness and atrophy and a left hemisensory deficit was found. Metrizamide-enhanced spinal CT scan showed an intramedullary lesion at the level of T1-T2; this had expanded the cord in fusiform fashion but showed no evidence of a cystic component. Surgical resection was performed and the pathological diagnosis was cavernous hemangioma. Two and one-half years later, her left hemisensory deficit was worsening and a spinal MRI showed high signal intensity mass in the region of the previous surgery consistent with chronic hematoma which was re-evacuated with some improvement in the patients neurological condition.

Septation and focal dilatation of ventricles associated with cryptococcal meningoencephalitis

A 35-year-old woman developed temporal lobe seizures. Isolated dilatation of the right temporal horn was demonstrated by computed tomography. She was asymptomatic for the next 10 months while on anticonvulsants before severe headaches, vomiting, and mental confusion prompted hospitalization. Both temporal horns were now dilated, there was marked periventricular edema, and cryptococci were cultured from the ventricular fluid. She succumbed after prolonged systemic and intrathecal antifungal therapy, having developed isolation and dilatation of both frontal horns and third and fourth ventricles. Cryptococcal or other fungal meningoencephalitis should be considered in the differential diagnosis of isolated dilatations of the ventricular chambers as noted in the present case.