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Susan R. Durham, Peter P. Sun, and Leslie N. Sutton

Object. This outcome study was undertaken to investigate the long-term results obtained in surgically treated pediatric patients with lumbar disc disease by using standardized medical outcome scales and clinical follow-up examination.

Methods. Twenty nine patients 17 years of age or younger underwent surgery between 1968 and 1998 for lumbar disc disease. The follow-up period ranged from 4 months to 30.5 years (mean 8.5 years). Outcome scores (health profiles) were generated using a standardized medical outcome scale, the Short Form health survey questionnaire (SF-36), and a condition-specific back pain outcome scale. Clinical follow-up data were obtained by telephone interview.

The health profile of the study population closely paralleled that of the normal population and was distinctly different from the health profile of adults with low-back pain. Only physical functioning, as measured by a scale of the SF-36, was found to be impaired in a subset of the study population. The rate of reoperation was 24% over the course of the follow-up period. In contrast to similar studies in adults, there were no identifiable predictive factors for either reoperation or poor outcome.

Conclusions. Lumbar disc disease in the pediatric population does not appear to lead to chronic complaints of back pain, and it does not appear to have a negative impact on overall health. This finding suggests that pediatric lumbar disc disease may be a separate entity distinct from adult lumbar disc disease, and therefore, the same conclusions regarding long-term outcome cannot be applied to the pediatric population.

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Leslie N. Sutton, Frank Welsh, and Derek A. Bruce

✓ The bioenergetic mechanisms of vasogenic edema were studied by measuring concentrations of adenosine triphosphate (ATP), phosphocreatine (CrP), and lactate in rapidly frozen edematous white matter in cats. When edema was produced using a cold lesion, it was found that both ATP and CrP were reduced to one-half of control values, and that lactate was elevated. When a correction was applied for dilution, however, it was found that high-energy phosphates were equal to control values, and that lactate was even more significantly elevated. This pattern contrasted with that seen in white-matter ischemia, in which CrP is depressed out of proportion to ATP. Finally, it was found that the white-matter lactate concentration in the plasma infusion model of edema was increased. It is concluded that vasogenic edema induces an increase in lactate, but does not deplete high-energy phosphate compounds in affected white matter.

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Leslie N. Sutton, Derek A. Bruce, and Frank Welsh

✓ The electrophysiological effects of cold-lesion edema and white-matter ischemia were studied in cats by reference to the short-latency somatosensory evoked response. The primary cortical waves were found to be considerably delayed following a period of white-matter ischemia; however, cold-lesion edema appeared to have no significant effect on the evoked response. The authors conclude that vasogenic edema does not interfere with axonal functioning by an ischemic mechanism.

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John A. Boockvar, William Loudon, and Leslie N. Sutton

✓ The history of the treatment for hydrocephalus dates back to the Fertile Crescent thousands of years ago. Despite three millennia of management, significant advances in the surgical treatment of the disease have been infrequent. During the 1950s, a milestone occurred at the Children's Hospital of Philadelphia, with the successful development of the first working shunt valve for the treatment of hydrocephalus. In this historical vignette, based on recent interviews with John Holter, D.Sc. (Hon) and Eugene Spitz, M.D., and on a review of the available literature, the authors narrate the exciting story of the development of the Spitz-Holter valve, which took place in Philadelphia during the early 1950s.

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Todd M. Lasner, Leslie N. Sutton, and Lucy B. Rorke

✓ This 9-year-old boy presented with a multifocal, apparently noninfectious granulomatous process involving the central nervous system. Despite gross-total excision of the large left temporal lesion, it recurred after surgery. Serological studies and cultures failed to demonstrate an infectious agent, and histopathological investigation showed a nonspecific granulomatous process without vasculitis. The patient underwent a second craniotomy and was given corticosteroid therapy. There has been no recurrence during 3 years of follow-up review, the last year of which included no course of steroid medications. The case is presented as a noninfectious idiopathic granulomatous encephalitis that is responsive to surgery and corticosteroid therapy. To the authors' knowledge, it is the first reported case of its kind.

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Ellen G. Shaver, Susan L. Rebsamen, Anthony T. Yachnis, and Leslie N. Sutton

✓ Sinus histiocytosis with massive lymphadenopathy was first described in 1969 by Rosai and Dorfman. The typical clinical characteristics of this disease include painless cervical lymphadenopathy, fever, and weight loss. The condition can present with an extranodal mass in about 25% of patients, and isolated masses without lymph node involvement occur rarely. The authors describe a 5-year-old boy with cavernous sinus syndrome due to an isolated extranodal form of sinus histiocytosis with massive lymphadenopathy in the temporal fossa. Several cases of this disease involving the central nervous system are reviewed. The histopathological and magnetic resonance imaging characteristics are discussed.

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Matthew F. Philips, Steven B. Schwartz, Alexander D. Soutter, and Leslie N. Sutton

✓ Children with shunted hydrocephalus often have a myriad of other medical conditions. When these concomitant problems involve the pleura, peritoneum, and/or the venous system, placement of the distal catheter may prove to be problematic. This report presents preliminary results in three hydrocephalic children following ventriculofemoroatrial shunt placement. The peritoneal and pleural cavities in each of these children were compromised and there was no vascular access into the superior vena cava due to intercurrent disease. An alternative technique for ventriculoperitoneal shunt placement was performed via the femoral vein. Fluoroscopic guidance was used to confirm the intraatrial position of the distal end of the shunt catheter. Follow-up review to date shows no complications. This newly described technique provides a feasible alternative to distal shunt catheter placement in patients in whom more traditional sites are unavailable.

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Derek A. Bruce, Luis Schut, Leonard A. Bruno, James H. Wood, and Leslie N. Sutton

✓ The outcome in 53 children following severe head injury is presented. All children were graded using the Glasgow Coma Scale; 90% made a good recovery or were moderately disabled, and 8% died or were left vegetative. All patients were treated with controlled ventilation and steroids; mannitol, and, if necessary, Nembutal (pentobarbital) were used to maintain the intracranial pressure below 20 torr. With this regimen, only one death occurred due to uncontrollable intracranial hypertension. All patients with a coma scale of 5 or greater recovered well. The worst prognostic sign was the presence of flaccidity: 33% of these patients died or were vegetative. Five of seven patients who were decerebrate or flaccid with bilateral fixed pupils and absent caloric responses made a good recovery or were moderately disabled. The relatively low incidence of mass lesions (23%) and high incidence of diffuse cerebral swelling (34%) suggest a different pathophysiological response of the child's brain to injury, which may play a role in the improved survival of children following severe head injury when compared to adults.

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Leslie N. Sutton, Alan C. McLaughlin, Stephen Dante, Mark Kotapka, Teresa Sinwell, and Elizabeth Mills

✓ In order to test the hypothesis that the cerebral arteriovenous oxygen difference (AVDO2) and venous oxygen content (VO2) could be used to monitor brain energy metabolism in the setting of increased intracranial pressure (ICP), 12 cats were studied with 31P-magnetic resonance spectroscopy. Six cats were subjected to intracranial hypertension by cisternal infusion of saline. Energy failure occurred at an average AVDO2 of 8.4 ± 3.2 vol% (± standard deviation) (range 4.7 to 14.7 vol%). The VO2 at the point of metabolic failure averaged 1.45 ± 0.6 vol% and extended over a narrower range (1.0 to 2.9 vol%). In an additional six cats, ICP was raised to the threshold of metabolic failure and hyperventilation was then instituted (pCO2 10 to 18 torr). Five of the six cats experienced a drop in VO2 with hyperventilation. In two of these animals, hyperventilation resulted in a VO2 of 1.1 vol% or less and in metabolic failure as evidenced by a fall in phosphocreatine. It is concluded that a VO2 of less than 2 vol% is correlated with brain ischemia and that the safety of hyperventilation in the setting of increased ICP can be monitored by the use of VO2.

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William O. Bell, Edward B. Charney, Derek A. Bruce, Leslie N. Sutton, and Luis Schut

✓ Twenty-two patients with closed myelomeningoceles, shunted hydrocephalus, and symptomatic Arnold-Chiari malformations were studied retrospectively. Seventeen of the 22 patients were aged 6 months or younger; five patients were aged 3 to 23 years. Patients in the younger group presented with stridor, apnea, and/or feeding difficulty; those in the older group presented with hemiparesis, quadriparesis, oscillopsia, nystagmus, or opisthotonos. Fourteen of the 17 younger patients underwent surgical decompression of the Arnold-Chiari malformation: 10 within 18 days following the onset of symptoms and four on Day 19 or later. Of the 10 infants with early treatment, five eventually died secondary to continued symptoms and five survived. Of those surviving, three were asymptomatic and two had continuing symptoms but were improved over their preoperative state. Among the four patients undergoing surgery later, two died, one had lessening of stridor, and one had complete relief of symptoms. Of the three infants not undergoing decompression, two died and one eventually became asymptomatic. All five of the older patients underwent decompression and all had complete resolution of their preoperative symptoms and signs. The authors conclude that while decompression of a symptomatic Arnold-Chiari malformation may be effective in children and adults, this treatment does not always improve the clinical condition of infants. This may be the result of ischemic/hypoxic effects on the infant's brain stem, which may not be organized normally at birth.