Biomedical subjects
Publications and source records attributed to J Milstein.
The authors report on a neonate with gastroschisis repaired at birth who later had abdominal distension, emesis, feeding intolerance, and an abnormal stooling pattern. Total colon and partial small bowel aganglionosis (TCAS), or Hirschsprung's disease, was diagnosed subsequently. This is the first report of this combination of gastrointestinal anomalies. J Pediatr Surg 36:638-640.
Workers' compensation health benefits are broader than general health benefits and include payment for medical and rehabilitation costs, associated indemnity (lost time) costs, and vocational rehabilitation (return-to-work) costs. In addition, cost liability is for the life of the claim (injury), rather than for each plan year. We examined device evaluation and coverage policy in workers' compensation over a 10-year period in Washington State. Most requests for device coverage in workers' compensation relate to the diagnosis, prognosis, or treatment of chronic musculoskeletal conditions. A number of specific problems have been recognized in making device coverage decisions within workers' compensation: (1) invasive devices with a high adverse event profile and history of poor outcomes could significantly increase both indemnity and medical costs; (2) many noninvasive devices, while having a low adverse event profile, have not proved effective for managing chronic musculoskeletal conditions relevant to injured workers; (3) some devices are marketed and billed as surrogate diagnostic tests for generally accepted, and more clearly proven, standard tests; (4) quality oversight of technology use among physicians may be inadequate; and (5) insurers' access to efficacy data adequate to make timely and appropriate coverage decisions in workers' compensation is often lacking. Emerging technology may substantially increase the costs of workers' compensation without significant evidence of health benefit for injured workers. To prevent ever-rising costs, we need to increase provider education and patient education and consent, involve the state medical society in coverage policy, and collect relevant outcomes data from healthcare providers.
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PURPOSE: To assess the utility of surveillance neuroimaging in detecting recurrent disease in patients treated for medulloblastoma. PATIENTS AND METHODS: Records and scans of 59 consecutive patients treated for medulloblastoma between 1984 and 1993 in one institution were retrospectively reviewed. RESULTS: Nineteen of 59 patients had recurrence of tumor, of which 17 were available for this study. Eleven of the 17 recurrent patients were asymptomatic at the time of detection. The median time to recurrence was 13 months (range, 3 to 90). CONCLUSION: Surveillance scanning detected a majority of recurrences before onset of symptoms. Although the outcome of those with recurrent disease remains poor, early detection with minimum disease provides the best setting in which to test newer therapies. Patients and their parents also were more likely to elect pursuing further treatment when relapse was detected asymptomatically.
Improved prognosis for pediatric brain tumors has stimulated research into the quality of life of survivors. To assess cognitive function and psychosocial and family adjustment among this population, 18 long-term survivors of childhood medulloblastoma or posterior fossa ependymoma treated with surgical resection and craniospinal irradiation were interviewed and administered achievement tests and psychosocial questionnaires. A majority of parents reported significant difficulty caring for their child with a brain tumor, but no significant adverse effects upon the family. Academic achievement was significantly impaired in 12/18 subjects. Psychosocial adjustment was normal in 10/18 subjects. Although specific treatment variables (radiation dosage, chemotherapy, etc.) were not significantly related to these two outcome measures, impaired academic achievement was correlated with young age at diagnosis (p < 0.05) and impaired psychosocial adjustment was correlated with greater time since diagnosis (p < 0.05). Overall quality of life reported by these individuals appears to be acceptable but neuropsychological and psychosocial examination is clearly indicated as part of the follow-up program.
We describe a case of cerebellar neuroblastoma with histologic documentation of maturation into a ganglioglioma sixteen months later. Only chemotherapy was administered following the initial surgery and the child is well and disease-free three years following her final surgical procedure. The outcome of this patient supports previous hypotheses that the cerebellar neuroblastoma may be a less malignant tumor than its other primitive neuroectodermal posterior fossa counterparts. Furthermore, this case suggests a role for second-look surgery in the management of selected pediatric brain tumors.
The clinical and therapeutic features of 20 patients with medulloblastoma treated at Children's Hospital and Medical Center, Seattle, from 1980 to 1987, were retrospectively analyzed with regard to prognosis. The overall actuarial 5-year survival rate was 63%, with 57% of patients free from recurrence at 5 years. Residual contrast enhancement on CT scans taken immediately postoperatively was associated with a significantly decreased 5-year recurrence-free survival rate; the 5-year recurrence-free survival rate was 100% for those patients without post-operative residual enhancement compared to 41% for those patients with residual enhancement. A high risk group of patients with residual contrast enhancement persisting one year following diagnosis was identified. No patient in this group survived without disease progression. Other factors, including sex, age at diagnosis, evidence of tumor dissemination, or extent of surgical resection as reported by the neurosurgeon, did not significantly influence prognosis.
The authors reviewed the hospital charts of 415 pediatric patients treated for benign or malignant primary brain tumors over the past 20 years at the Children's Hospital Medical Center, Seattle. Patients' ages ranged from the neonatal period to 18 years. A shunt was placed in 152 patients (37%), 45 before and 94 after surgery. Confirmation of extraneural metastases was based on clinical and diagnostic examination. Factors analyzed as possibly influencing the occurrence of extraneural metastases were: 1) the shunt: type, valve, location, filter, and revisions; 2) extent of resection; 3) pathology; and 4) treatment regimen. Eight of the 415 patients developed extraneural metastases during life. All eight patients had a medulloblastoma (cerebellar primitive neuroectodermal tumor). These eight patients were separated into Group A (without a shunt) and Group B (with a shunt). In Group A (five patients), the mean interval from primary diagnosis to metastasis was 15 months. Two children had gross total resection of the tumor. The predominant location of metastases in Group A was: bone (two cases); cervical lymph nodes (one); lung/bone (one); and retroperitoneal pelvic mass (one). Three Group A patients had a simultaneous central nervous system (CNS) recurrence. Of the three Group B patients, two had a ventriculoperitoneal (VP) shunt and one a ventriculoatrial (VA) shunt; all were placed postoperatively. One Group B patient had a simultaneous CNS recurrence. No shunt revisions were performed in these three patients. The mean time from primary diagnosis to metastasis was 25 months. One patient had a total tumor resection. The predominant location of metastases was bone (one case), retroperitoneal pelvic mass (one), and abdominal cavity with ascites (one case). Only one patient in the entire series had a filter placed; this resulted in shunt obstruction and was removed 1 month following placement. It is concluded that cerebrospinal fluid shunts, regardless of type, location, revision rate, or filter insertion, do not predispose pediatric patients with brain tumors to develop extraneural metastases. A diagnosis of shunt-related metastases should be based on the development of intra-abdominal (VP shunt) or pulmonary (VA shunt) dissemination primarily with or without additional sites. The diagnosis of medulloblastoma is an important factor related to metastasis occurrence while the extent of resection and postoperative therapy are not influential.
Although the prognoses of children with medulloblastoma have improved dramatically over the last several decades, the reported survival for very young children with this tumor remains poor. We undertook a retrospective review of patients less than 36 months of age at the time of the diagnosis of a medulloblastoma who were treated at our institution during a 36-year period. Of 28 such patients, 10 patients are presently surviving without disease at a median of 150 months from diagnosis. The presence of a metastatic tumor at the time of diagnosis was identified as a significant indicator of a poor prognosis, and all completely staged patients without metastasis are surviving without a recurrence of disease. This report suggests that the survival of very young children with a medulloblastoma may not be as poor as has been previously reported, particularly if a disseminated tumor is not present at the time of diagnosis.
We present a case of a 17-year-old male with an unbiopsied brainstem glioma who developed drop metastases a few months following completion of hyperfractionated radiation therapy to the primary site. The literature is reviewed with reference to intraspinal metastases from brainstem gliomas.
We nonselectively interviewed 11 patients aged 3 through 16 years who had survived critical illnesses, including cardiac arrests and profound comas. Any memory of a time they were unconscious was considered to be a near-death experience (NDE) and was recorded. Seven of these children had memories that included being out of the physical body (six patients), entering darkness (five patients), being in a tunnel (four patients), and deciding to return to the body (three patients). We also interviewed 29 age-matched survivors of illnesses that required intubation, narcotics, benzodiazepines, and admission to an intensive care unit. None of them had any memories of the time they were unconscious. In our study population, NDEs were clearly associated with surviving a critical illness. The elements of NDEs reported are similar to those previously described in adults. No children described elements of depersonalization as part of their NDEs. A core NDE, triggered by the process of dying or resuscitation efforts, may be a natural developmental experience. We present a neurophysiologic hypothesis as to the cause of NDEs.
Infantile spasms constitute a severe seizure disorder unresponsive to standard anticonvulsants. Both prednisone and adrenocorticotropic hormone (ACTH) have produced remission of seizures in some patients. The mechanisms of action of these hormones are not known. Eight infants with infantile spasms were treated with prednisone for 2 weeks. This controlled the seizures in two patients. In the remaining six patients, prednisone was continued and ACTH was added. This treatment produced cessation of spasms in four patients. Serum prednisone and cortisol were measured at a number of points during treatment. In infants receiving prednisone and then prednisone plus ACTH, serum cortisol was suppressed to about one-quarter of baseline levels with the initiation of prednisone, and remained suppressed during ACTH administration. We conclude that ACTH can exert its effect on infantile spasms in the setting of adrenal suppression, and can act without stimulating endogenous cortisol production. A CNS site of action is suggested and should be sought.
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Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.