E. Bruce Hendrick

Occult Spinal Dysraphism: Clinical and Urodynamic Outcome after Division of the Filum Terminale

A highly select group of 31 patients presenting with urinary incontinence failed to respond to conservative management and were found to have unstable bladders and spina bifida occulta. After thorough evaluation they were suspected of having neurogenic bladder dysfunction possibly due to a tethered cord. Following division of the filum terminale daytime incontinence resolved in 72%, urodynamic detrusor hyperreflexia disappeared in 59% and bladder compliance improved in 66% of the patients. The operation was well tolerated and did not result in any neurological complications. The clinical, radiological and urodynamic characteristics of these patients before and after treatment are reported.

Language of Hydrocephalic Children and Adolescents

How adequate a substrate for the development of language is the young hydrocephalic brain? To answer this question, the development of five language domains (word finding, fluency and automaticity, immediate sentence memory, understanding of grammar, and metalinguistic awareness) was studied in children and adolescents, 75 with hydrocephalus in the first year of life, and 50 normal controls. The results revealed a limited resilience of language to the effects of early hydrocephalus. Resilience was indicated by the finding that, for several language skills, hydrocephalics scored at comparable levels to normals and improved their test performances as they matured. The limits to resilience were also apparent. Language development was not uniform after early hydrocephalus, and, within particular language domains, hydrocephalics were either less skilled or less able to maintain age-appropriate skill mastery than normal peers. Early hydrocephalus had diverse effects on language development: Of the variables measuring its forms, manifestations, and treatments, some were indicative of preserved language functioning while others were predictive of language deficit. The young hydrocephalic brain mediates language development in a manner that is adequate, but far from ideal.

A randomized controlled trial of perioperative rifampin/trimethoprim in cerebrospinal fluid shunt surgery

A randomized, double-blind, placebo-controlled trial of perioperative rifampin-trimethoprim was undertaken at the Hospital for Sick Children from March 1984 to October 1987, in which 243 patients undergoing 300 cerebrospinal fluid (CSF) shunting procedures were randomized into groups including treatment with rifampin/trimethoprim and placebo, and then followed for a minimum of 2 years. Patients were stratified prior to randomization into those with and those without meningomyeloceles having first insertions of their shunts, and those having revisions. Patients could be entered into the study more than once, but always received the same treatment regimen once allocation had taken place. Among the patients receiving antibiotics there was an infection rate of 12%, versus 19% among patients receiving placebo. Among the surgical procedures, the rates were 9% and 15%, respectively. Because these rates of infection were a substantial increase over the rate of 7.5% overall for the few years prior to implementation of the study, and well over any acceptable rate of infection, the study, was stopped before statistical significance was reached. However, had the study continued, and the proportions of patients becoming infected remained constant, we would have been able to achieve a statistically significant difference in rates of infection, and therefore demonstrate a benefit of rifampin/trimethoprim as prophylaxis against shunt infection. Methodological problems encountered in this and other studies of prevention of CSF shunt infection will be discussed.

Primary intracranial neoplasms in the first year of life

Forty-one infants with histologically verified primary intracranial neoplasms were studied who had been diagnosed and treated during the first year of life at the Hospital for Sick Children during the years 1975–1986. Forty-one percent of tumors were astrocytomas, 22% were primitive neuroectodermal tumors, and 20% were choroid plexus papillomas. Seventy-one percent were located in the supratentorial compartment and 29% in the infratentorial compartment. Thirty percent were in the cerebral hemisphere, 12% in the optic pathway-hypothalamic region, 5% in the thalamus, 17% in the cerebellum, 5% in the brain stem, 12% in the lateral ventricles, 12% in the III ventricle, and 7% in the IV ventricle. The most common presenting features in this series of patients were vomiting and increasing head size. The symptoms and signs before diagnosis were present for 2 months or less in 87% of cases. Forty-four percent of the tumors were totally resected while only a partial resection or biopsy was carried out in 56%. Sixty-six percent of the patients required a CSF diversionary shunt. Twenty-nine percent of patients received radiotherapy. Slightly more than half of these received radiotherapy immediately after surgery, whereas delayed radiation therapy was performed in the remainder. The surgical mortality was 7.3%. Of the entire group, 39% have died, with most dying within 6 months of surgery. Treatment, survival relative to histologic type, psychomotor development, and neurologic function of the survivors are discussed.

Detection and management of gangliogliomas in children.

Forty-two children treated for gangliogliomas were reviewed to identify the best methods of detection and management. Thirty-two of the tumors were supratentorial, four infratentorial, and six were in the spinal cord. Twenty-five patients presented with seizures; the mean duration of symptoms was 5 years in contrast to 1 year in non-seizure patients. Of 31 children studied by computed tomography (CT), calcification in the tumor lesion was found in 19. Magnetic resonance imaging (MRI) showed abnormal high-signal intensity on T2 imaging in six of eight patients. All patients underwent surgical resection and were diagnosed pathologically. Twenty-four patients had total resection, and 14 underwent temporal lobectomies including hippocampectomy. The management of this tumor remains surgical resection without the need for any adjuvant therapy.

Experience with pineal region tumours in childhood

Sixty-one children with pineal region tumours were managed at the Hospital for Sick Children during the period 1950-1982. Histologic verification of these tumours was possible in forty-six children. Although germ cell tumours were the commonest form, germinomas only made up one third of the pineal region tumours. Since 1967, an aggressive approach has been adopted in the management of pineal region tumours at the Hospital for Sick Children with thirty-four patients having direct surgery on their pineal region mass out of a total of forty-one patients with such tumours. The operative mortality for pineal tumour surgery has steadily fallen so that the operative mortality between 1975 and 1982 was 4.3% and there have been no operative deaths since 1977 among the thirteen patients with pineal region tumours who have been operatively treated. Pineal tumours are operable lesions which should be removed if they are benign. Histologic confirmation should be obtained if they are malignant in order to provide for rational management of these lesions.

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.

The value of CSF flow studies in infants with communicating hydrocephalus

The authors describe their technique of dynamic CSF scanning utilizing the radionucleide material Tc-99m diethylentriamine-pentacetic acid (DTPA). Thirty-four infants with macrocephaly and enlarged intracranial CSF spaces were investigated with this technique. The resulting studies were graded according to severity. The grading scheme was of value in determining which patients would benefit from a CSF diversionary shunt.

Trauma of the Central Nervous System in Children

In summary, the care of the patient with head trauma is not dissimilar to any other acutely ill individuals. Careful, systematic observation and recording of changing factors and physical signs is necessary. Proper application and appropriate treatment in response to dynamic changes will provide a satisfactory result as in any other form of illness or trauma. The patient with multiple injuries may call for compromise in treatment, however with the exception of gross hemorrhage, the head injury should take precedence in the early period following injury for there are other factors that may prove fatal. Do not accept unnecessary delay in the treatment of other injuries on the grounds that the patient has had a head injury. The orthopedic surgeon can treat the fractured femur, the plastic surgeon-the face, and the general surgeon-the abdomen, without jeopardizing brain recovery. The treatment of the patient with head trauma, as treatment of any other individual in present day medical practice, has become more of a team effort, not only involving the neurosurgeon, but the pediatrician, the specialist in pulmonary physiology, and other expert groups.

The Definition of a Reduced CSF Absorption Syndrome: Clinical and Experimental Studies

The syndrome of benign intracranial hypertension has been recognized under various titles since Quincke’s description some 75 years ago (1). The cause of the increase in intracranial pressure, and even which intracranial compartment is involved has, however, remained obscure. In a recent review of the available evidence it was argued that the rise in intracranial pressure is secondary to an increase in CSF volume as a result of impaired CSF absorption (2). The present study reports some clinical observations on CSF circulation in patients with benign intracranial hypertension and experimental attempts to determine the cause of the condition.