Biomedical subjects
Publications and source records attributed to J H Piatt.
The authors present the case of an 11-year-old boy with symptomatic tethering of the hindbrain related to a Chiari Type III malformation repaired at birth. Surgical release of the cord resulted in functional improvement.
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Six hundred and seventy-one operations to insert or revise simple, linear cerebrospinal fluid shunts were performed in a single institution from 1976 through 1989. The results were analyzed using actuarial techniques. Failures within the first 8 months were defined as 'early', and subsequent failures were 'late'. Patient age did not quite attain significance as a determinant of the risk of late shunt failure (p = 0.054). The following factors had no effect on the risk of late failure: interval since last operation, etiology of hydrocephalus, identity of surgeon, burr hole site, type of valve, and whether the shunt was new or revised. Modes of shunt failure in the two phases were compared: there were proportionately more infections among early failures and proportionately more fractures/separations among late failures (chi-squared = 42.9, d.f. = 6, p < 0.000005). Shunts failing during the early phase were more often revised by removal and replacement of the entire system, whereas shunts failing later were more often revised by replacement of valve and/or abdominal catheter (chi-squared = 33.7, d.f. = 3, p < 0.000005). The late phase of shunt survival conformed very closely to an exponential decay model with a constant monthly risk of failure of 0.5% (r2 = 0.98). Contrary to intuition, cerebrospinal fluid shunts did not encounter increasing monthly risks of failure as they grew older. The contempt of familiarity has discouraged study of the mechanisms of shunt failure. Experience suggests that most early shunt failures are attributable to infection and other technical misadventures, but the mechanisms of late failure are distinct and incompletely understood.
We reviewed 27 patients with congenital anomalies of the sacral spine. There were 16 males and 11 females with a mean follow-up of 81.1 months (range 8-211 months). Fifteen patients had sacral agenesis and 12 had sacral dysgenesis. Fifteen patients had neuroimaging of the spine. Seven patients had conus termination below the L2 vertebral body. Four patients had associated thoracic syringomyelia and 6 patients were identified with caudal or dorsal lipoma. There were only two episodes of neurological deterioration, both in a single patient who had a lipomyelomeningocele, in 182 patient-years of follow-up. Four patients with low lying conus had pre-emptive spinal cord exploration for release of tethering in order to prevent neurological deterioration. Patients with agenesis or dysgenesis of the sacrum should undergo magnetic resonance (MR) imaging of the spine in order to detect spinal cord lesions associated with progressive neurological deterioration. Findings on MR imaging are more likely to correlate with clinical course than findings on skeletal radiography.
A minor modification of the technique of peritoneal cerebrospinal fluid shunt insertion allows more anatomical closure of the abdominal wound and may protect the shunt from contamination by superficial abdominal would infection.
Subgaleal hematoma, also known as subaponeurotic hemorrhage, is a serious complication of birth that is associated with vacuum-assisted delivery. Despite a high rate of mortality associated with subgaleal hematoma, it has received relatively little attention in the medical literature. Lack of awareness may lead to delayed diagnosis and serious consequences for infants. This paper is a report of six cases and a literature review. Prevention and early recognition and treatment of the condition can occur only with increased practitioner awareness of this entity.
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Recent advances in biochemical and imaging studies have improved the diagnostic accuracy of Cushing's syndrome. To better define roles for these studies in children, the authors reviewed their experience with this rare group of patients. Fifteen children, aged 11 weeks to 17 years, were treated for noniatrogenic Cushing's syndrome over a 33-year period. All children presented with signs of hypercortisolism. Nineteen different diagnostic tests were used, reflecting changes in how these patients are evaluated. Pathological diagnoses were adrenal cortical carcinoma (3), primary adrenocortical nodular dysplasia (PAND) (2), and pituitary adenoma (10). Children with adrenal cortical carcinoma presented with an adrenal mass and at a younger age (mean, 22.3 months). Key diagnostic features of patients with PAND were a low plasma adrenocorticotrophin hormone (ACTH) and no suppression with high-dose dexamethasone. Children with a pituitary cause of Cushing's syndrome presented at an older age (mean, 15.7 years) and were diagnosed using a combination of high-dose dexamethasone testing, simultaneous inferior petrosal sinus sampling, and/or ovine corticotrophin-releasing hormone stimulation test. A strategy for the diagnosis of Cushing's syndrome in children is presented.
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The antemortem diagnosis of syringomyelia in the setting of sacral agenesis has not been reported before. We describe the clinical and neuroimaging features in 3 patients. None has required surgical intervention to date.
Thirty-eight insertions or revisions of ventriculopleural shunts were performed in 22 patients. The most common indication for pleural cerebrospinal fluid diversion was recent abdominal infection. Very minor pneumothoraces were seen frequently, but no patient became symptomatic or required treatment. Only three shunts in 2 patients were discontinued because of pleural effusions. The median survival of the entire series of ventriculopleural shunts was only 10 months. A historical control group of revised ventriculoperitoneal shunts matched for factors that have been shown to influence shunt survival was constructed from an institutional data base. The survival of simple ventriculopleural shunts in this series was not significantly different from that of simple ventriculoperitoneal shunts in patients of comparable age with a comparable recent shunt revision history (p = 0.68). Pleural cerebrospinal fluid diversion is a simple, safe, and reasonably effective option when clinical circumstances present relative or absolute contraindications to peritoneal diversion.
Neisseria sicca is considered to be a nonpathogenic oral saprophyte. Presented here is an unusual case of cerebrospinal fluid (CSF) shunt infection by N. sicca. Although medical management of the common community-acquired meningitides, including infection by Neisseria meningitidis, is often successful in patients with CSF shunts, removal and replacement of the infected shunt was necessary in this case.
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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.
The authors present what is believed to be the first description of an intracranial arterial aneurysm attributable to birth trauma. A male neonate, the product of a precipitious, instrumented, footling breech delivery, exhibited seizures at the age of 18 hours. A computerized tomography scan of the head showed hemorrhage along the tentorium with a globular component at the incisura. Transfontanel Doppler ultrasound examination detected pulsatile arterial flow within the globular mass. Cerebral angiography demonstrated a 1.5-cm saccular aneurysm arising from a small distal branch of the superior cerebellar artery. The pathogenesis of aneurysms in children is obscure and controversial. Birth trauma may be responsible for some pediatric aneurysms that are currently classified as idiopathic or congenital, particularly aneurysms in the region of the tentorial incisura.
To assess the value of the information derived from pumping the shunt valve in hydrocephalic patients with cerebrospinal fluid shunts, the findings on examination of 200 consecutive patients were recorded prospectively. The status of each shunt was determined on the basis of all available clinical data, and the results of the shunt pumping test were compared to the final status of each shunt for various subsets of the study group. In the most clinically pertinent subsets, the sensitivity of the shunt pumping test in the detection of shunt obstruction was 18% to 20%, and the predictive value of a negative shunt pumping test, indicating shunt patency, was only 65% to 81%. For the hydrocephalic patient with symptoms of shunt malfunction, definitive imaging studies must not be deferred because of reassuring findings on examination of the shunt valve.
While most newborns with birth injury of the brachial plexus make a full spontaneous recovery, the minority who do not can expect lifelong disability from weakness, disturbed patterns of muscle activity, contracture, and deformity. Those children who are destined to a poor recovery can be identified in early infancy. Early reconstruction of the brachial plexus carries low morbidity and has been shown by many to support useful shoulder and elbow function. Patients who are referred later in childhood may still benefit from plexus exploration, but how to best use clinical and electrophysiologic data to plan a surgical intervention that will improve on the natural history remains to be elucidated for this group.
The Scientific Board of the California Medical Association presents the following inventory of items of progress in neurosurgery. Each item, in the judgment of a panel of knowledgeable physicians, has recently become reasonably firmly established, both as to scientific fact and important clinical significance. The items are presented in simple epitome and an authoritative reference, both to the item itself and to the subject as a whole, is generally given for those who may be unfamiliar with a particular item. The purpose is to assist busy practitioners, students, research workers, or scholars to stay abreast of these items of progress in neurosurgery that have recently achieved a substantial degree of authoritative acceptance, whether in their own field of special interest or another. The items of progress listed below were selected by the Advisory Panel to the Section on Neurosurgery of the California Medical Association, and the summaries were prepared under its direction.