Upper limb motor function in young adults with spina bifida and hydrocephalus.

OBJECTIVE: The objective of the study was to measure upper limb motor function in young adults with spina bifida meningomyelocele (SBM) and typically developing age peers. METHOD: Participants were 26 young adults with SBM, with a Verbal or Performance IQ score of at least 70 on the Wechsler scales, and 27 age- and gender-matched controls. Four upper limb motor function tasks were performed under four different visual and cognitive challenge conditions. Motor independence was assessed by questionnaire. RESULTS: Fewer SBM than control participants obtained perfect posture and rebound scores. The SBM group performed less accurately and was more disrupted by cognitive challenge than controls on limb dysmetria tasks. The SBM group was slower than controls on the diadochokinesis task. Adaptive motor independence was related to one upper limb motor task, arm posture, and upper rather than lower spinal lesions were associated with less motor independence. CONCLUSIONS: Young adults with SBM have significant limitations in upper limb function and are more disrupted by some challenges while performing upper limb motor tasks. Within the group of young adults with SBM, upper spinal lesions compromise motor independence more than lower spinal lesions.

The intelligence of hydrocephalic children.

In a group of 78 children with hydrocephalus in the first months of life, the level and pattern of intelligence were considered in relation to various parameters and symptoms of their condition. These included demography (age, sex, handedness); early developmental status; symptoms (visual, motor, and seizure); formative pathology; type of hydrocephalus; site of CSF obstruction; extent and configuration of cortical thinning; and shunt treatment. The common outcome of early hydrocephalus is an uneven growth of intelligence during childhood, with nonverbal intelligence developing less well than verbal intelligence. The origin of this selective cognitive deficit is in neither the hydrocephalic condition itself nor its treatment, but rather in the developmental brain anomalies and symptoms to which the hydrocephalic child is prone: In children with aqueduct blocks and intraventricular hydrocephalus, a selectively thin vertex and occipital lobe; in any hydrocephalic child, ocular abnormalities, motor deficits, and seizures.

Brain tumors in children and adolescents--I. Effects on working, associative and serial-order memory of IQ, age at tumor onset and age of tumor.

Memory impairment was demonstrated in a sample of 46 children and adolescents with brain tumors. The overall distribution of memory scores was skewed positively; over half of the total number of test measures were in the lowest quarter of the score distribution; and more than three-quarters of the individual subjects had at least one memory score in the impaired range. Verbal intelligence accounted for less than one-quarter of the variance in memory scores. The type of memory impairment was analyzed in relation to various demographic and medical variables: age at onset of tumor symptoms, age or duration of tumor, sex, pre-tumor developmental disturbances, pre-tumor closed head injury, post-tumor anticonvulsant treatment and post-tumor epileptic seizures. The working memory task, in which each in a succession of heard words is stored in temporary memory long enough to be compared to or contrasted with incoming words, was unaffected by these variables, as was memory for semantically-based word-picture associations. Memory for the serial order of pictures that corresponded to heard words varied inversely with the age at tumor onset such that the later the onset, the lower the memory test performance.

Brain tumors in children and adolescents--II. The neuroanatomy of deficits in working, associative and serial-order memory.

The neuroanatomy of memory deficits was studied in 46 children and adolescents with brain tumors. CT-scan reconstructions of 88 brain regions were coded with respect to tumor and related damage, and multiple regression procedures established patterns of brain damage predictive of memory deficits. Two forms of memory revealed non-overlapping focal neuroanatomical substrates: memory for the serial order of pictures that corresponded to heard words involved structures in the limbic system and hypothalamic-pituitary axis; whereas working memory, in which each of a succession of heard words is stored in temporary memory long enough to be compared to or contrasted with incoming words, involved the pineal-habenular region and the anterior and medial thalamic nuclei. Memory for semantically-based word-picture associations, in contrast, was unaffected by tumors in several subcortical brain regions. These data bear on current analyses of the neural substrates of associative and representational memory.

Brain tumors in children and adolescents--III. Effects of radiation and hormone status on intelligence and on working, associative and serial-order memory.

The effects on intelligence and memory of two post-surgical conditions (radiation treatment, hormone deficiency and supplementation) were explored in 46 children and adolescents with tumors in a variety of brain sites. Verbal intelligence, but not non-verbal intelligence, varied positively with age at radiation treatment. Memory for word meanings was unrelated to either radiation history or to hormone status. Severe deficits in serial position memory occurred with impaired hormone function and an older age at tumor onset. Severe deficits in working memory were associated with a history of radiation and a principal tumor site that involved thalamic/epithalamic brain regions. Radiation treatment and hormone status affect later cognitive function in children and adolescents with brain tumors. Although the greater vulnerability of the verbal intelligence of the younger radiated child and the serial order memory of the child with later tumor onset and hormone disturbances remain to be explained, and although the form of the relationship between radiation and tumor site is not fully understood, the data highlight the need to consider the cognitive consequences of pediatric brain tumors according to a set of markers that include maturational rate, hormone status, radiation history, and principal site of the tumor.

Microsurgical recovery of a migrated balloon from the internal carotid artery of a child.

A child developed a traumatic intrapetrous internal carotid aneurysm after a routine myringotomy. The aneurysm was treated by occlusion of the internal carotid artery by the detachable balloon technique. Unfortunately, both balloons migrated and ischemic sequelae resulted when one of the balloons became lodged in the bifurcation of the internal carotid artery. The balloon was successfully retrieved during an emergency microsurgical embolectomy, and the child's neurological deficit was reversed. The management of both the intrapetrous aneurysm and the balloon migration are discussed.

Lumbar disc disease in children.

The clinical experience with lumbar disc disease in 38 patients at a large children's hospital is reviewed. Thirty children underwent surgical treatment; eight patients were not operated upon. The surgical results were excellent, with 93% of the children cured or markedly improved on follow-up. The main differential aspects of lumbar disc disease between children and adults are emphasized.

Hydrosyringomyelia and its management in childhood.

Syringomyelia, once regarded as a degenerative disease of adults, is now recognized to be a disorder usually associated with the Chiari malformation and occurring in patients of all ages. We have reviewed 47 patients with syringomyelia treated on the Neurosurgical Service at the Hospital for Sick Children during the years 1977 to 1985. Twelve of these patients had a Chiari I malformation, 30 had a Chiari II malformation, and 5 had an acquired Chiari malformation. Thirty-one of these patients were treated by decompression of the Chiari malformation and plugging of the obex, 5 were treated by a simple posterior fossa decompression, 9 were treated by shunting of the syringomyelic cavity, and 2 were treated by a combined decompression of the posterior fossa and shunting of the syrinx. The Gardner procedure (decompression of the Chiari malformation and plugging of the obex) was the procedure most commonly used in managing our group of patients and resulted in improvement in over 70% of patients.

Transependymal benign dorsally exophytic brain stem gliomas in childhood: diagnosis and treatment recommendations.

Sixteen children with dorsally exophytic transependymal benign brain stem gliomas were treated at the Hospital for Sick Children, Toronto, between 1949 and 1985. The diagnosis of these benign brain stem gliomas was based on neuroradiographic and operative observations. Five children were diagnosed in the pre-computed tomography (CT) era using ventriculography and pneumoencephalography, and 11 children were diagnosed using CT with and without contrast enhancement. Histological diagnosis was obtained in all 16 cases. In 1980, we first described this distinct group of benign brain stem gliomas, which accounted for 8% (total, 121 cases) of all brain stem gliomas diagnosed at our institution. However, with the use of high resolution CT in recent years, earlier and better definition of this particular type of brain stem tumor can be made. During the 10 years (1976 to 1985) in which CT has been used for preoperative evaluation in our institution, we have treated 49 patients with primary brain stem tumors. Eleven of these cases (22%) fell into the category of dorsally exophytic transependymal benign brain stem tumors. These tumors characteristically protrude into and largely fill the 4th ventricle, breaking through the overlying ependyma and frequently extending into the cisterna magna. On CT, they are isodense and enhance brightly with contrast agent. Clinically, these patients have a longer history of symptoms in comparison with patients with the more malignant, intrinsic types of brain stem tumor. All of our patients were treated by subtotal resection. Radiotherapy was utilized in 7 of the 16 patients.(ABSTRACT TRUNCATED AT 250 WORDS)

Experience with surgical decompression of the Arnold-Chiari malformation in young infants with myelomeningocele.

Forty-five infants with myelomeningocele in whom hydrocephalus was absent or adequately controlled developed signs and symptoms of the Arnold-Chiari malformation before the age of 3 months. All of them underwent laminectomy and opening of the dura mater for hindbrain decompression. The clinical presentation included swallowing difficulty, apneic episodes, stridor, bronchial aspiration, arm weakness, and opisthotonos. Within 2 weeks of the initial clinical presentation, the neurological status of 14 patients (31%) deteriorated dramatically and culminated in irreversible neurological deficits. In all patients, compression of the brain stem occurred in the spinal canal. A transverse dural band constricting the dural sac at the C-1 level was noted in 41% of the patients, and a mild degree of arachnoidal adhesion was noted in 23%. The lowermost level of the cerebellar tongue or medullary kink was located at C-1 to C-4 in 28 cases and at C-5 to T-1 in 17 cases. Twenty-eight (62%) of the patients were alive and 17 (38%) had died at the last follow-up assessment. All survivors showed improvement of their overall neurological function. Twenty-four made a complete recovery. The majority of deaths were attributed to respiratory failure. Early recognition of symptoms and prompt decompressive laminectomy are essential for successful management of the Arnold-Chiari malformation in infants.

Acoustic schwannomas in children.

The clinical presentation and treatment of three cases of acoustic schwannoma occurring in children are described. All the tumors were detected late, when they had attained a large size and were extremely vascular. The use of preoperative tumor embolization as an adjunct to surgical excision is discussed.

Surgical management of unilateral and bilateral coronal craniosynostosis: 21 years of experience.

From 1955 to 1975, 116 patients with unilateral and bilateral coronal synostosis, including 39 with craniofacial dysmorphism, were treated surgically. Five techniques were used: multiple linear craniectomies, linear craniectomies with supraorbital grafting, morcellation craniotomies, lateral canthal advancement of the supraorbital margin, and radical cranio-orbitofacial reconstruction. Analysis of clinical and radiological indices of the synostotic process, coupled with evaluation of the surgical results, indicates that the anterior skull base is the site of origin of the bony dysplasia. Therefore, early creation of artificial sutures in the skull base is recommended to provide adequate expansion of the frontal bones and consequently of the entire facial skeleton.

Spontaneous intraparenchymal hemorrhage in full-term neonates.

OBJECTIVE: Spontaneous intraparenchymal hemorrhage is extremely rare in full-term newborns. Reports to date have been limited to descriptions of individual cases, small groups within larger studies of intracranial hemorrhage, and one series of four patients. Structural lesions are rarely identified, and the majority of patients described have been managed without surgical intervention. METHODS: Analysis of a computerized database of pediatric neurosurgical patients from January 1960 to February 2000 identified full-term newborns younger than 3 months of age with nontraumatic intraparenchymal hemorrhages. Prenatal histories, labor and delivery histories, clinical presentations, imaging studies, management, and outcomes were reviewed. RESULTS: Eleven full-term newborns with spontaneous intraparenchymal hemorrhages were identified. The majority had normal prenatal courses. Most presented within the first 2 days of life (6 of 11 patients), and the most common presenting sign was seizure (7 of 11 patients). No cause was identified in 6 of 11 patients; the remainder were attributed to coagulopathy (n = 3), ruptured intracranial aneurysm (n = 1), or hemorrhagic infarction (n = 1). Eight patients underwent surgical hematoma evacuation on the basis of radiographic evidence of significant mass effect, evidence of signs of elevated intracranial pressure, or both. Three patients did not receive surgical intervention. There were no subsequent hemorrhages or deaths during a mean follow-up period of 4.5 years (range, 1-16 yr). Four patients had normal neurological outcomes, four had motor deficits (one of whom additionally demonstrated cognitive delay), and three had delayed speech. CONCLUSION: No cause is identified in most newborns with spontaneous intraparenchymal hemorrhage. Radiographic evidence of mass effect or signs of elevated intracranial pressure may necessitate surgical hematoma evacuation. Outcome varies widely and may be normal, even in patients with sizeable intraparenchymal hemorrhages.

Dermoid cysts of the anterior fontanelle.

A review of the medical records at The Hospital for Sick Children in Toronto, Ontario, from 1967 through 1980 showed that, of the 94 cases of dermoid cyst of the skull treated at this institution by the neurosurgical staff, 25 were located over the anterior fontanelle. This is the largest single group of cases reported to date. The children were 2 months to 13 years of age, but two-thirds were treated before their 1st birthday. Females outnumbered males 2:1, and all of the patients presented with a nontender swelling over the anterior fontanelle, which had been present from birth. The lesions were treated by a variety of surgical approaches, all of which were curative. None of the lesions has recurred. The most interesting finding was that all but 2 of the children were white, in contrast to all previously published series. This confirms our belief that the racial incidence of dermoid cysts of the anterior fontanelle is a reflection of the general population. A review of previously published cases and the pathology and embryology of the lesion is included.

Spontaneous cerebrospinal fluid otorrhea in association with a congenital defect of the cochlear aqueduct and Mondini dysplasia.

Unrecognized spontaneous cerebrospinal fluid (CSF) otorrhea led to recurrent bacterial meningitis in three children. The underlying cause of the spontaneous CSF otorrhea was proved to be a congenital cystic dilatation of the cochlear aqueduct and Mondini dysplasia of the temporal bone. The CSF leak recurred in all patients after an initial surgical attempts to close the defects through a tympanotomy. A suboccipital approach was used successfully to obliterate the CSF fistulas in two children; a translabyrinthine approach was used in the other. A search of the literature revealed that Mondini dysplasia is a congenital anomaly that can commonly cause spontaneous CSF otorrhea in children. The authors discuss the management of this uncommon entity on the basis of a literature review and their own experience.

Primary intracranial choriocarcinoma: a report of two cases and a review of the literature.

Among 848 cases of primary intracranial malignancy seen during a 63-year period at a pediatric hospital, there were 2 cases of primary pineal choriocarcinoma. The clinical and laboratory findings of these 2 cases were similar to those of 33 cases of intracranial choriocarcinoma reported in the literature. In 1 patient with precocious puberty, the diagnosis was confirmed by labeling human chorionic gonadotropin (HCG) within the tumor, which had been stored for 25 years. In the other patient, who is alive and well 18 months after diagnosis, the response to cranial irradiation and intensive chemotherapy was monitored with serial measurements of serum, cerebrospinal fluid, and urinary HCG and with computed tomography. We conclude that intensive chemotherapy, radiation, and tumor resection, if feasible, offer the best chance of curing this otherwise fatal disease.

Myxopapillary ependymoma of the filum terminale and cauda equina in childhood: report of seven cases and review of the literature.

Seven of fourteen children with spinal cord ependymoma had myxopapillary tumors of the filum terminale. These tumors made up 15.9% of all primary spinal neuroectodermal tumors in children (44 cases) seen during a 62-year period (1919 to 1981). Their clinical presentation, radiological features, pathological findings, treatment, and outcome are reported. Six of the seven patients were known to be alive at the time of writing. The seventh patient was lost to follow-up after 3 years without tumor recurrence. Of 5 patients whose primary mode of treatment was operation alone. 3 had intraspinal or intracranial recurrences. Despite tumor recurrences, 2 patients were long term survivors after further operation and irradiation, whereas the third patient recently received craniospinal irradiation for intracranial tumor recurrence. The 2 patients who did not have tumor recurrence after operation alone had been followed for 3 and 7 years, respectively. Two children with subtotal tumor resection and spinal irradiation had no recurrences at 1 and 17 years, respectively. Our data suggest that this unusual subtype of spinal ependymoma is not uncommon during childhood and has a good prognosis. All patients with this tumor require prolonged follow-up for tumor recurrence after operation and irradiation.

Focal midbrain tumors in children.

The clinical and neuroradiological features of focal midbrain tumors in 12 children are described, and the results of their surgical management are presented. Patients with a focal midbrain tumor usually exhibit either symptoms and signs of raised intracranial pressure caused by an obstructive hydrocephalus (50%) or symptoms and signs caused by pressure on the tegmentum and cerebral peduncles. The lesions are confined to the tectal plate or tegmentum with possible extension upward to the thalamus and downward to the pons, displacing but not invading these structures. The edges of the tumor are well defined, and the large majority have a solid consistency with intense regular enhancement after intravenous contrast. Radical resection is hardly ever feasible in brain stem tumors, but in this series, significant reduction of the tumor mass was obtained in 75% of the patients, with no surgical mortality and minimal surgical morbidity and with the majority of patients showing clinical improvement postoperatively. All tumors were nonpilocytic, low-grade astrocytomas. Six patients received adjunctive radiotherapy. The mean follow-up period is 2.5 years, and all patients are alive and doing well. We conclude that focal midbrain tumors in children appear to be a distinct subgroup of brain stem tumors and are very amenable to surgical resection with an excellent long-term prognosis.

Management and outcome of low-grade astrocytomas of the midline in children: a retrospective review.

Low-grade astrocytomas of the midline of the brain can be difficult to manage because of their location. To evaluate treatment and outcome, we performed a retrospective study of children with midline low-grade astrocytomas admitted to The Hospital for Sick Children between 1976 and 1991. Eighty-eight children with biopsy-proven low-grade astrocytomas were identified. Forty-three tumors occurred in the optic pathways or hypothalamus, 13 in the thalamus, 7 in the pineal region, 14 in the midbrain, and 11 in the medulla. Patient follow-up ranged from 6 months to 15 years, with a mean of 4 years, 9 months. Overall outcome was related to the extent of resection, histological type, and location. Partial resections were often associated with involution of the tumor. Response to radiation was variable, and serious sequelae were observed. Thirty-three patients experienced recurrence, often with a good response to subsequent surgery; however, 12 of these patients died. The probability of survival was calculated to be 96% at 1 year, 91% at 5, and 80% at 10 years. Our study suggests that resection should be considered in all patients, both at presentation and recurrence.

Development of anaplastic changes in low-grade astrocytomas of childhood.

The authors present their experience with six children who developed anaplastic astrocytomas after receiving treatment for low-grade astrocytomas. Five children were from a series of 55 children with optic chiasmatic-hypothalamic gliomas who have been studied since 1976. The sixth child initially had a low-grade astrocytoma of the thalamus. The mean age of the children at initial presentation was 5.3 years. Five children were treated with surgery and radiation therapy; one child with a chiasmatic-hypothalamic glioma received radiation therapy alone. The amount of external radiation therapy used in all children was 50-52.5 Gy delivered in standard fractionations over approximately 6 weeks to include the volume of the original tumor plus a margin of 2 cm. The time to anaplastic transformation varied between 2 and 10 years (mean, 6.4 years). At tumor recurrence, the children had seizures or symptoms and signs of raised intracranial pressure. The location of the second tumor in all patients was either at the primary site or within the field of radiation therapy. Five of the six children underwent a second craniotomy and subtotal resection of their malignant gliomas. One child had positive cerebrospinal fluid cytology and multiple intraspinal metastatic tumor nodules detected by magnetic resonance imaging. On histopathological examination, four children had anaplastic astrocytoma, and two had glioblastoma multiforme. Four of the six children have died of their anaplastic astrocytomas (mean time from diagnosis of anaplastic astrocytoma to death, 10 months). Two children underwent chemotherapy and spinal irradiation for their anaplastic astrocytomas, and are currently alive and undergoing treatment. The possible mechanisms by which anaplastic tumors have developed in children treated previously for low-grade astrocytomas is discussed. The data suggest that radiation therapy may have played an integral role in the genesis of anaplastic astrocytomas in these children.