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.

Recognition memory of item, associative and serial-order information after temporal lobectomy for seizure disorder.

Children, adolescents, and young adults with temporal lobectomy judged the context of their encounters with a word (by deciding about its prior occurrence or familiarity) and also identified word attributes involving associative content and serial order (by indicating word meanings and reconstructing word sequences). Laterality of temporal lobectomy was important for identifying familiarity: individuals with right-sided temporal lobectomies made better judgements than those with left resections about the prior occurrence of target words, with the more successful performance depending on enhanced sensitivity to the familiarity or non-familiarity of what was heard. Identifying the attributes of an item, as contrasted with its prior occurrence, depended on aspects of temporal lobe disorder in conjunction with laterality: deficits of brain structure involving histopathology and significant tissue loss disrupted content memory; compromised brain function resulting from certain post-infantile seizures impaired memory for serial order. In the young brain after temporal lobectomy, there exists a functional dissociation between judging the familiarity of an event and identifying its attributes, a dissociation that depends, not only on laterality of surgery, but also on the structural and functional intactness of the residual brain.

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.

Visual loss in pseudotumor cerebri of childhood. A follow-up study.

The occurrence of visual loss in substantial numbers of adult patients with pseudotumor cerebri is well recognized. In children, the disease has been said to spare the visual system. We evaluated the ophthalmologic features of 36 children with pseudotumor cerebri followed up for one to 12 years. Four patients had rapid, severe loss of visual acuity and visual field changes while receiving medical therapy. Six others had less-severe but prominent abnormalities of visual acuity and/or visual fields at some point during the disease process. Only one patient had a permanent, severe visual impairment, but five had moderate permanent visual abnormalities. A severe degree of papilledema was seen only in the group with visual loss, but some patients in this group had only moderate papilledema. More patients with dural sinus thrombosis had serious visual loss than did those with other associated diseases or idiopathic pseudotumor cerebri. Quantitative perimetry of some sort can be performed in most children and is the preferred sequential test for planning treatment.

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.

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.

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)

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.

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.

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.

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.

Subdural empyema and epidural abscess in children.

From January 1, 1954, through December 31, 1981, 31 children were treated at The Hospital for Sick Children in Toronto, Ontario, for subdural empyema (22 cases), epidural abscess (6 cases), and infected nidi at both subdural and epidural sites (3 cases). All but four of the children with subdural empyema were from 6 months to 2 years of age or from 12 years to 16 years of age; all children with epidural abscess fitted into the older age group. The subdural empyemas developed from varied sources, but the paranasal sinuses, the ears, and the mastoids processes were predominantly affected. Five subdural empyemas developed following craniotomy or shunt procedures. The epidural abscesses developed mostly from infections in the paranasal sinuses and the middle ears. Two of the combined infections developed from the paranasal sinuses; the third followed craniotomy. The infecting bacteria varied, but were usually Streptococci and were common to both the subdural and epidural sites. The subdural empyemas presented as focal seizures in the older group, and that group of patients was more seriously ill and had greater focal deficit; the mortality rate was 13%. Epidural abscesses only rarely presented with focal deficit, and diagnosis was delayed in most instances; no deaths occurred in this group. Two cases are reported in detail to illustrate the principles and pitfalls of surgical management of these intracranial infections in the pediatric age group.

Early neurosurgical repair in craniofacial dysmorphism.

The authors describe the operative repair and results in 16 cases of craniofacial dysmorphism. The craniofacial dysmorphic states are presumably caused by premature closure of basal skull sutures in combination with the coronal sutures. Hydrocephalus is commonly associated with these conditions and contributes to the bizarre appearance as well as the abnormal mentation that some of these patients display. Early neurosurgical intervention using the procedure of lateral supraorbital ridge advancement yields excellent results in the majority of these infants.

Experimental effects of steroids and steroid withdrawal on cerebrospinal fluid absorption.

The authors studied the effects on cerebrospinal fluid (CSF) absorption of chronic administration and acute withdrawal of steroids in dogs. CSF absorption was measured by determining the amount of isotope (indium 111DTPA) recovered over a 4-hour period after injection into the cisterna magna. Resistance to CSF absorption was estimated by determining rates of low of Ringer's lactate infusion into the cisterna magna over a range of pressure gradients between CSF and sagittal sinus. Steroid withdrawal was associated with a marked reduction in CSF absorption and an increase in resistance to CSF flow. Dogs on steroids also showed reduced CSF absorption although the reduction was not statistically significant when compared with controls. The results are discussed in terms of possible mechanisms of action of steroids on CSF absorption, the etiology of the benign intracranial hypertension syndrome and the use of steroids and in the control of intracranial hypertension.

Management of lipomyelomeningoceles. Experience at the Hospital for Sick Children, Toronto.

Ninety-seven children with lipomyelomeningoceles were operated on at the Hospital for Sick Children between January, 1960, and December, 1982. The most common factor that caused these patients to seek help was the cosmetic effect of the mass on their back. However, 22 patients had urinary incontinence and 15 patients had a deformed or weak leg. Sixty of the 97 patients were female and 37 were male. The patients presented for treatment between 6 days and 18 1/2 years of age with a median age of 7 1/2 months. Fifty-six patients presented before the age of 6 months and 35 of these were perfectly normal at the time of presentation. On the other hand, of the 41 patients who were brought for treatment after the age of 6 months, only 12 were normal prior to surgery. When patients were appropriately treated at an early age, with their spinal cords untethered and their dura securely closed with a dural graft, then they remained unchanged neurologically or even improved. However, when treatment was delayed or not done appropriately then they were left with significant neurological sequelae. Lipomyelomeningoceles are serious lesions which without appropriate therapy can result in gross impairment of neurological function.

New lumboperitoneal shunt for communicating hydrocephalus; technical note.

The authors describe a T-tube Silastic shunting device which has been used for lumboperitoneal shunting in 62 patients with communicating hydrocephalus during the past 3 years. In 38 patients it was inserted as a primary shunting procedure; 24 patients had the shunt inserted following problems secondary to arachnoiditis created by a polyethylene-type lumboperitoneal shunt. The shunt described here has not led to arachnoiditis when inserted as a primary shunting procedure. In 61% of patients no operative revisions of their shunt has been required to date, and 81% continue to do well on their T-tube type shunt.

Metastasis via ventriculoperitoneal shunt in patients with medulloblastoma.

The authors have routinely inserted a ventriculoperitoneal shunt in all patients with a posterior fossa tumor and hydrocephalus some 7-10 days prior to craniotomy. Forty-one patients with medulloblastoma were treated in this fashion and of these, four metastasized through the shunt and died of systemic metastases without evidence of recurrent tumor in their central nervous system. A millipore filter which can be incorporated in the shunt has been used by the authors during the past 18 months in an effort to prevent metastatic spread through the shunt.