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Jeffrey P. Blount, W. Jerry Oakes, R. Shane Tubbs and Robin P. Humphreys

✓ Intracranial aneurysms are rare in children, and their origins and treatment methods tend to be different from those in these same entities in adults. These lesions tend to be congenital or to have an infectious or traumatic origin. In the current paper the authors trace the historical evolution of the diagnosis and treatment of intracranial aneurysms in children. Based on the literature, these lesions appear to occur in children in less than 3% of all series. The literature also supports the suggestion that symptoms from these aneurysms are often from mass effect and that giant aneurysms and lesions in the posterior cranial fossa are relatively more common in children than in adults. The termination of the carotid artery and the anterior cerebral artery seem to be disproportionately common sites of aneurysm formation in this cohort. Interestingly, surgical outcomes in children appear to be moderately better than in adults. Based on the literature, the claim can be made that a multidisciplinary approach to the management of such aneurysms can yield good outcomes in a very high percentage of children treated.

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Jeffrey P. Blount, W. Jerry Oakes, R. Shane Tubbs and Robin P. Humphreys

✓ Vein of Galen aneurysmal malformations are a mixed group of lesions characterized by an abnormal fistula between abnormal distal branches of the choroidal and/or posterior cerebral arteries and the great vein of Galen. In this paper the authors trace the historical evolution of the current approach to diagnosis and treatment, and the literature is reviewed comprehensively. During the historical era, vein of Galen malformations were described in individual case reports and an early classification system was developed. In the early era of treatment, open surgery was the preferred approach, although morbidity and mortality rates were high. The development of neurointerventional techniques allowed the introduction of occlusive materials into the fistula, with pronounced improvements in clinical outcome.

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Jeffrey P. Blount, R. Shane Tubbs, W. Jerry Oakes and Robin P. Humphreys

✓ Arteriovenous malformations (AVMs) are the most common cause of intracerebral hemorrhage in children. In this paper the authors trace the historical evolution of the recognition, diagnosis, and treatment of pediatric intracerebral AVMs, and they summarize the contemporary approach and current controversies surrounding treatment of these lesions. Important distinctions between adult and pediatric AVMs are emphasized.

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Abhaya V. Kulkarni, James M. Drake, Doron Rabin, Peter B. Dirks, Robin P. Humphreys and James T. Rutka

Object. In the preceding article, the authors described the Hydrocephalus Outcome Questionnaire (HOQ), a simple, reliable, and valid measure of health status in children with hydrocephalus. In the present study, they present their initial experience in using the HOQ to quantify the health status in a typical cohort of children with hydrocephalus.

Methods. The mothers of children with hydrocephalus completed the HOQ and, with the child's attending surgeon, provided a global rating of their children's health. An exploratory analysis was performed using a multivariate analysis of variance (ANOVA) to determine which variables might be associated with worse health status.

The mothers of 80 children, ranging in age from 5 to 17 years, participated in the study. The mean HOQ Overall Health score was 0.68, a value estimated to be equivalent to a mean health utility score of 0.77. The global health ratings provided by the mothers and the surgeons were moderately correlated with the HOQ scores (Pearson correlations 0.58 and 0.57, respectively). Results of the multivariate ANOVA indicated that the presence of epilepsy was strongly associated with a worse health status (p < 0.0001, F-test).

Conclusions. The health status of a typical sample of children with hydrocephalus was measured using the HOQ. The only consistently significant association with health status found was the presence of epilepsy.

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Ian F. Pollack, Harold J. Hoffman, Robin P. Humphreys and Larry Becker

✓ Dorsally exophytic brain-stem gliomas represent a distinctive subgroup of pediatric brain-stem neoplasms that are amenable to radical excision because of their benign histology and growth characteristics. However, their attachment to the floor of the fourth ventricle invariably precludes complete tumor excision. The long-term behavior of the residual tumor remains a subject of concern. To address this issue, the authors reviewed their experience with 18 dorsally exophytic brain-stem gliomas treated between 1974 and 1990. At operation, the tumors filled the fourth ventricle, fungating out of a broad-based area of the dorsal brain stem. The exophytic tumor was resected, but no attempt was made to remove tumor from the brain stem. Histological examination showed that 16 of the tumors were grade I or II astrocytomas, one was a ganglioglioma, and one was an otherwise benign-appearing glioma with several foci of anaplasia that was classified as a grade III astrocytoma. The latter patient was one of only two in the series to receive postoperative radiation therapy; both cases so treated have no evidence of disease on follow-up imaging studies 61 and 135 months postoperatively. One other child who had stable disease postoperatively died of shunt malfunction 18 months after tumor excision. Serial radiographic studies in the other 15 patients have shown no evidence of disease in three, stable residual disease in eight, and tumor enlargement 12, 28, 40, and 84 months postoperatively in four (median follow-up period 113 months). Each of the four patients with tumor regrowth underwent repeat tumor excision. Two of these children received perioperative radiation therapy at the time of disease progression and both showed reduction in tumor volume 28 and 65 months after their second operation. In contrast, both patients who did not receive radiotherapy at the time of disease progression had further tumor enlargement 48 and 84 months after their second operation and underwent a third tumor resection; one received postoperative radiation therapy and has no evidence of disease 58 months after his third operation and the other child has stable disease 27 months postoperatively. Histological examination of tumor specimens obtained at second and third operations showed no change from the appearance of the tumor on the initial resection. The authors conclude that the majority of dorsally exophytic brain-stem gliomas can be managed successfully with subtotal excision and, if necessary, cerebrospinal fluid diversion. The small percentage of tumors in this series that showed recurrent growth remained benign histologically. In these patients, repeat tumor resection and radiotherapy appeared to be effective in maintaining long-term disease control.

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Paul D. Chumas, Derek C. Armstrong, James M. Drake, Abhaya V. Kulkarni, Harold J. Hoffman, Robin P. Humphreys, James T. Rutka and E. Bruce Hendrick

✓ Although the development of tonsillar herniation (acquired Chiari malformation) in association with lumboperitoneal (LP) shunting is well recognized, it has previously been considered rare. In order to ascertain the incidence of this complication after LP shunting, the authors undertook a retrospective study of all patients in whom this form of shunt had been inserted between 1974 and 1991 at The Hospital for Sick Children, Toronto. In the 143 patients, the mean age at insertion was 3.3 years and the indications for shunt placement were hydrocephalus (81%), pseudotumor cerebri (7%), cerebrospinal fluid fistula (6%), and posterior fossa pseudomeningocele (6%). The mean follow-up period was 5.7 years, during which time there was one shunt-related death due to unsuspected tonsillar herniation. Five other patients developed symptomatic tonsillar herniation treated by suboccipital decompression.

Review of all computerized tomography (CT) scans not degraded by artifact showed evidence of excess soft tissue at the level of the foramen magnum in 38 (70%) of 54 patients so studied. In order to confirm that this CT finding represented hindbrain herniation, sagittal and axial magnetic resonance (MR) images were obtained for 17 asymptomatic patients and revealed tonsillar herniation (range 2 to 21 mm) in 12 (70.6%). In addition, some of these asymptomatic patients had evidence of uncal herniation and mesencephalic distortion. Similarities and distinctions are drawn between the morphological changes occurring after LP shunting and those seen in association with the Chiari I and II malformations. Although less than 5% of this study population required treatment for tonsillar herniation, the incidence of this complication was high in asymptomatic patients; MR imaging surveillance for patients with LP shunts is therefore recommended.

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William P. Vandertop, Akio Asai, Harold J. Hoffman, James M. Drake, Robin P. Humphreys, James T. Rutka and Laurence E. Becker

✓ Between January, 1981, and July, 1991, 17 infants under 1 month of age were admitted to The Hospital for Sick Children with the signs and symptoms of a Chiari II malformation. These patients' presentation included swallowing difficulty (71%), stridor (59%), apneic spells (29%), aspiration (12%), weakness of cry (18%), and arm weakness (53%). Decompression of the Chiari II malformation was performed in all patients, with a time interval between onset of symptoms and surgery ranging from 1 to 121 days. Fifteen patients (88%) remain alive, all of whom have shown a complete recovery. The mean follow-up period in this group of patients was 65 months. Two patients died, one due to respiratory arrest 8 months after decompression and the other because of shunt infection and peritonitis 7 years after decompression. These results support the concept that compressive forces, rather than a primary intrinsic disorder of the brain-stem nuclei, play a crucial etiological role in the development of a symptomatic Chiari II malformation. Early recognition of the symptoms of Chiari II malformation should be followed by immediate decompressive laminectomy in order to promote a prompt and full neurological recovery.

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Harold J. Hoffman, Marcia De Silva, Robin P. Humphreys, James M. Drake, Mary Lou Smith and Susan I. Blaser

✓ The cases of 50 patients with craniopharyngioma operated on at The Hospital for Sick Children in Toronto between January, 1975, and December, 1989, are reviewed. All patients were under 18 years of age (mean 9.39 years). Headaches, endocrine deficiences, and visual deficits were the most common symptoms on admission. Forty-five patients underwent what was considered by the surgeon to be total excision of their tumor, and five had subtotal excision. Tumors recurred in 17 patients (mean time of recurrence 32.6 months after surgery). One patient died in the postoperative period and three have been lost to follow-up study. Of the remaining 46 patients, 28 are leading a normal or nearly normal life, although all are receiving endocrine replacement and some have required help to overcome mild deficits in memory or visual acuity. Twelve patients are able to function reasonably well and attend school despite being hampered by intellectual or visual deficits or problems with weight control; four have a significant handicap, and two have died.

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Paul L. May, Susan I. Blaser, Harold J. Hoffman, Robin P. Humphreys and Derek C. Harwood-Nash

✓ A specific group of intrinsic dorsal midbrain tumors was identified in six children by computerized tomography (CT) and magnetic resonance (MR) imaging. Each patient presented with raised intracranial pressure as a result of hydrocephalus due to obstruction of the sylvian aqueduct. No patient had brain-stem signs referable to the tectal tumor initially or subsequently. All six children underwent cerebrospinal fluid (CSF) diversionary procedures. The radiological features were consistent and specific, with all patients showing tectal calcification or primary increased attenuation of the tectal plate on CT scans. In addition, lack of contrast enhancement was noted initially in four patients and eventually in all six patients. In all patients MR imaging showed a focal tectal tumor distorting the colicular plate with no cystic component and increased signal intensity on T2-weighted images. There has been no evidence of progression in these six patients in the follow-up period ranging from 8 months to 17 years (8 months and 2½, 4½, 5½, 8, and 17 years). Diversion of CSF has been the only surgical treatment and no patient underwent deep x-ray therapy. Five patients have had normal intellectual development. In contrast to the majority of previously described periaqueductal and tectal tumors, this group of lesions appeared to be truly benign. The authors suggest that patients presenting with these clinical and radiological features may be managed by CSF diversion, serial examination, and MR imaging.