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Biomedical subjects

A L Albright

Publications and source records attributed to A L Albright.

At least 19 recordsLinked to original sources
PubMed2001

Slit-ventricle syndrome secondary to shunt-induced suture ossification.

OBJECTIVE: To report five children with slit-ventricle syndrome who were found to have increased intracranial pressure despite functioning cerebrospinal fluid shunts. METHODS: Computed tomographic scans demonstrated erosion of the inner table of the cranium and sclerosis of the cranial sutures, particularly the coronal suture. Magnetic resonance imaging scans demonstrated no cerebrospinal fluid over the convexities. The patients were treated with cranial expansion operations that included removal of the sclerotic sutures, which were examined histologically. RESULTS: Postoperatively, symptoms resolved for all children. Sutures were abnormal and contained foci of cartilage and bone within abnormally arranged fibrous tissue. CONCLUSION: We postulate that chronic overdrainage of cerebrospinal fluid via shunts dampens the normal cerebral pressure waves; growth of the calvarium is thus understimulated, and this leads to ossification of the sutures, which become unable to expand to allow normal brain growth. Shunt-induced craniostenosis should be considered for children with symptoms of slit-ventricle syndrome for whom shunts are functional but intracranial pressure is increased. Cranial expansion operations may be more appropriate treatments than subtemporal decompressions for such children, given the diffuseness of the suture pathological features.

Cerebral Ventricles↗
PubMed2000

Outcome analysis of endoscopic III ventriculostomy.

A retrospective chart review was performed on 54 patients who had undergone endoscopic III ventriculostomy (E3V) in the past 6 years. Patient charts were reviewed to establish age at the time of operation, sex, preoperative diagnosis, preoperative shunt procedures, success or failure of the E3V, duration of success, and complications. Success of an E3V was determined by the resolution of preoperative symptoms and avoidance of a CSF shunt. The most recent clinic visit with adequate documentation of signs and symptoms of hydrocephalus was used as the last date of follow-up. The overall success rate was 74%. Children over the age of 3 years with an acquired CSF obstruction had a significantly greater probability of successful treatment (P=0.05). Younger children, especially those with hydrocephalus attributable to obstruction of the arachnoid villi, as in intraventricular hemorrhage (IVH), were less likely to benefit from E3V.

Adolescent↗
PubMed2000

Correlation of neurosurgical subspecialization with outcomes in children with malignant brain tumors.

OBJECTIVE: This study was performed to evaluate the association between the type of neurosurgeon (general or pediatric) and either the extent of tumor removal or the frequency of complications in children undergoing malignant brain tumor resections. METHODS: Data were analyzed from three recent Children's Cancer Group studies: two on medulloblastomas/primitive neuroectodermal tumors and one on malignant gliomas. Neurosurgeons were classified as general neurosurgeons, as designated pediatric neurosurgeons in their institutions, or as members of the American Society of Pediatric Neurosurgeons (ASPN), which requires pediatric neurosurgical experience and practice standards. RESULTS: Data forms from 732 children were analyzed; 485 were from children with medulloblastomas/primitive neuroectodermal tumors, and 247 were from children with malignant gliomas. Operations were performed by 269 neurosurgeons, including 213 general neurosurgeons, 29 designated pediatric neurosurgeons, and 27 ASPN members. The mean number of operations per surgeon was 1.8, 4.9, and 7.6 for general neurosurgeons, designated pediatric neurosurgeons, and ASPN members, respectively. There was a significant relationship between the extent of tumor resection or the amount of residual tumor and the type of neurosurgeon. Designated pediatric neurosurgeons and ASPN members were more likely to remove more than 90% of the tumor and to leave less than 1.5 cc of residual tumor than were general neurosurgeons (P<0.05). In these studies, the probability of extensive tumor removal correlated with the number of operations the neurosurgeon performed (P<0.01). Neurological complications occurred in the following proportion of cases: general neurosurgeons, 23%; designated pediatric neurosurgeons, 32%; and ASPN members, 18%. CONCLUSION: Pediatric neurosurgeons are more likely than general neurosurgeons to extensively remove malignant pediatric brain tumors. In these tumors, extent of removal has been demonstrated to influence survival.

Brain Neoplasms↗
PubMed2000

Thalamic stimulation for choreiform movement disorders in children. Report of two cases.

Surgery for movement disorders is most commonly performed in patients with dyskinesia and tremor associated with Parkinson's disease or in those with essential tremor. The role of ablative surgery or deep brain stimulation in patients with choreiform movements is poorly defined. The authors placed thalamic stimulation systems in two children with disabling choreiform disorders due to intracerebral hemorrhage or cerebral palsy. Each patient displayed choreiform movements in the upper extremities both at rest and with intention, which interfered with daily activities and socialization. Both children obtained significant improvement in their choreiform movements, and their upper extremity function improved with no incidence of morbidity. Thalamic stimulation appears to be a promising and nonablative approach for children with choreiform movement disorders.

Activities of Daily Living↗
PubMed1999

Reliability and responsiveness of the Barry-Albright Dystonia Scale.

The reliability and responsiveness of the Barry-Albright Dystonia (BAD) Scale, a 5-point ordinal severity scale for secondary dystonia, was assessed. For interrater reliability, 13 raters scored 10 videotaped patients; for intrarater reliability, two raters rated the videotape again. For test-retest reliability, patients were rated on two occasions. Four inexperienced raters scored patients, received training, then scored additional patients. To assess responsiveness, we compared patient and physician global ratings of change (better, same, and worse) with BAD Scale score changes for 18 patients on intrathecal baclofen (ITB) trials. We assessed reliability with the intraclass correlation coefficient (ICC). The mean ICC for total BAD Scale scores were as follows: interrater reliability 0.866, intrarater reliability 0.967 and 0.978, test-retest reliability 0.978 (before training) and 0.967 (after training). We found the BAD Scale responsive to change, with most improved scores in patients rated by the patient, family, and neurosurgeon as 'better'. The total scores were reliable for experienced raters. We recommend training for clinicians interested in using the scale.

Adolescent↗
PubMed1999

Outcome data and analysis in pediatric neurosurgery.

OBJECTIVE: The purpose of this study was to analyze the outcomes of five commonly performed pediatric neurosurgical operations: 1) initial shunt insertion; 2) first shunt revision; 3) craniotomy for brain tumor; 4) correction of sagittal synostosis; and 5) release of tethered cords. A second purpose was to analyze the neurological outcome data after tethered cord releases. METHODS: Morbidity and mortality records, patient charts, and operative records were reviewed to determine length of hospitalization and, for each disorder, the pertinent outcomes such as duration of shunt function and incidence of infection or neurological morbidity. RESULTS: Many outcome data were expected, such as a high long-term shunt function rate after primary shunt insertion (65% at 5 yr), a low mortality rate (1%) and permanent morbidity rate (10%) after craniotomy for brain tumor, and a low frequency of transfusion (20%) for sagittal synostosis operations. The outcomes among the three neurosurgeons varied more than expected, e.g., the duration of hospitalization after sagittal reconstructions ranged from 3.1 to 5.8 days; the frequency of infections of primary shunt revisions ranged from 0 to 15%; and the neurological morbidity after tethered cord releases ranged from 0 to 12%, with all neurological morbidity occurring in patients undergoing their second or third tether release. CONCLUSION: The data may serve as a basis for outcome comparisons for these procedures. Outcome data allow us to analyze factors to improve patient care, but outcome analysis is complex.

Adolescent↗
PubMed1999

A randomized, controlled study of a programmable shunt valve versus a conventional valve for patients with hydrocephalus. Hakim-Medos Investigator Group.

OBJECTIVE: A multicenter prospective randomized controlled study was performed to assess the safety and efficacy of a Codman Hakim programmable shunt valve (Codman/Johnson & Johnson, Raynham, MA) versus a conventional valve system of the surgeon's choice for the treatment of patients with hydrocephalus. METHODS: Enrollment was stratified on the basis of whether the patient was undergoing initial shunt insertion or revision of an existing valve system at study entry. Study end points were: 1) valve explantation, and 2) shunt failure (surgical intervention for any component of the shunt). A total of 377 patients were enrolled onto the study, with 235 undergoing first shunt insertion (119 experimental, 116 control) and 142 undergoing revisions (75 experimental, 67 control). RESULTS: During a follow-up interval of 104 weeks after the first implantation on-study, explantation of the valve was required in 62 (32%) of 194 experimental valves, compared with 71 (39%) of 183 control valves. Two-year survival rates of the original shunt without revision of any component were 52% (62 of 119) and 50% (58 of 116) in experimental and control patients, respectively, who underwent initial shunt insertion, and 43% (32 of 75) and 43% (29 of 67) in experimental and control patients, respectively, who underwent replacement of an existing valve. No statistically significant difference was observed between experimental and control patients in the survival of either the valve or the overall shunt system. Control of hydrocephalus as assessed symptomatically and by imaging was comparable in the two treatment groups. Although problems related to inability to achieve the desired pressure setting were reported in 22 experimental valves, in all but four instances no additional programming was attempted because the patients were functioning well clinically. The most common reasons cited for valve explantation and shunt revision were infection (9.8% frequency in the overall cohort) and proximal shunt malfunction, which occurred with comparable frequency in the experimental and control groups. CONCLUSION: Safety and efficacy of the Codman Hakim programmable shunt valve is comparable to conventional valves in the overall population of patients with hydrocephalus. However, the current study was not designed to assess the efficacy of programmable versus conventional valves in the management of individual hydrocephalus problems, and it had insufficient statistical power to support such comparisons. This study provides a rationale for examining whether the theoretical advantages of a programmable valve for managing challenging hydrocephalus problems can translate into meaningful improvements in shunt and valve survival.

Adolescent↗
PubMed1999

Plasma baclofen levels in children receiving continuous intrathecal baclofen infusion.

To determine the plasma baclofen concentrations of children undergoing continuous intrathecal baclofen infusion for treatment of cerebral spasticity, we assayed plasma samples from six children, 8 to 18 years of age, who were receiving intrathecal baclofen at constant rates of 77 to 400 micrograms/day. Plasma levels were at or below the limit of quantification (10 ng/mL) in all patients.

Administration, Oral↗
PubMed1999

Metastasis stage, adjuvant treatment, and residual tumor are prognostic factors for medulloblastoma in children: conclusions from the Children's Cancer Group 921 randomized phase III study.

PURPOSE: From 1986 to 1992, "eight-drugs-in-one-day" (8-in-1) chemotherapy both before and after radiation therapy (XRT) (54 Gy tumor/36 Gy neuraxis) was compared with vincristine, lomustine (CCNU), and prednisone (VCP) after XRT in children with untreated, high-stage medulloblastoma (MB). PATIENTS AND METHODS: Two hundred three eligible patients with an institutional diagnosis of MB were stratified by local invasion and metastatic stage (Chang T/M) and randomized to therapy. Median time at risk from study entry was 7.0 years. RESULTS: Survival and progression-free survival (PFS) +/- SE at 7 years were 55%+/-5% and 54%+/-5%, respectively. VCP was superior to 8-in-1 chemotherapy, with 5-year PFS rates of 63%+/-5% versus 45%+/-5%, respectively (P = .006). Upon central neuropathology review, 188 patients were confirmed as having MB and were the subjects for analyses of prognostic factors. Children aged 1.5 to younger than 3 years had inferior 5-year estimates of PFS, compared with children 3 years old or older (P = .0014; 32%+/-10% v 58%+/-4%, respectively). For MB patients 3 years of age or older, the prognostic effect of tumor spread (MO v M1 v M2+) on PFS was powerful (P = .0006); 5-year PFS rates were 70%+/-5%, 57%+/-10%, and 40%+/-8%, respectively. PFS distributions at 5 years for patients with M0 tumors with less than 1.5 cm2 of residual tumor, versus > or = 1.5 cm2 of residual tumor by scan, were significantly different (P = .023; 78%+/-6% v 54%+/-11%, respectively). CONCLUSION: VCP plus XRT is a superior adjuvant combination compared with 8-in-1 chemotherapy plus XRT. For patients with M0 tumors, residual tumor bulk (not extent of resection) is a predictor for PFS. Patients with M0 tumors, > or = 3 years with < or = 1.5 cm2 residual tumor, had a 78%+/-6% 5-year PFS rate. Children younger than 3 years old who received a reduced XRT dosage had the lowest survival rate.

Adolescent↗
PubMed1998

Current management of pediatric atlantoaxial rotatory subluxation.

STUDY DESIGN: A retrospective clinical review of 20 children seen during a 7-year period who had atlantoaxial rotatory subluxation. OBJECTIVE: To define the effectiveness of imaging and treatment measures and to identify risk factors for recurrence, the series was reviewed to analyze cause, management, and outcome. SUMMARY OF BACKGROUND DATA: Rotatory subluxation of the atlantoaxial complex remains a poorly understood entity. Despite many reports in the literature, there is no consensus about which imaging studies should be used for diagnosis and which patients benefit from collar immobilization, traction, or surgical fusion. METHODS: Between August 1990 and April 1997, 20 children with atlantoaxial rotatory subluxation were treated. Fourteen patients (70%) were girls and six (30%) were boys (mean age, 6.4 years). All patients had torticollis and neck pain with decreased cervical motion for a mean of 11.2 days before diagnosis. Seven patients (35%) had a history of pharyngitis or otitis media, four (20%) had recently undergone head or neck surgery, and four (20%) had sustained a traumatic injury; in five patients (25%), no clear cause was determined. All patients were neurologically intact and underwent plain cervical radiographs and dynamic cervical computed tomography to document atlantoaxial rotatory subluxation. Patients were then treated with a rigid cervical collar and anti-inflammatory agents (n = 5) or with cervical traction followed by immobilization (n = 15). RESULTS: In four of the five patients in collars, reduction occurred spontaneously, whereas the fifth required cervical traction and eventual fusion for recurrence. In the 16 patients treated with traction (median, 1.8 kg), the normal atlantoaxial alignment was restored in 15 patients (94%) within a mean of 4 days. Of the 20 patients treated overall, conservative management failed in 6 (30%), and they required posterior fusion because of recurrence of the atlantoaxial rotatory subluxation or unsuccessful reduction. The major factor predicting the failure of conservative management was the duration of subluxation before initial reduction. Patients with long-standing subluxation were more likely to experience recurrence and require surgery. There were no complications noted. At follow-up, all patients who were treated conservatively remained neurologically intact with a normal atlantoaxial relation. All patients who underwent surgery remained neurologically intact and had radiographic documentation of fusion. CONCLUSION: Optimal management of atlantoaxial rotatory subluxation entails early diagnosis with plain cervical radiographs and dynamic computed tomography. Closed reduction with cervical traction followed by rigid immobilization accomplished reduction in 15 of 16 patients (94%) and was curative in 10 of 16 patients (63%). Although reduction was achieved more rapidly and effectively with traction than with a collar, there may be a role for simple immobilization without reduction in patients with a short duration of symptoms. There does not appear to be a correlation between cause of atlantoaxial rotatory subluxation, age, or sex and the likelihood of recurrence.

Atlanto-Axial Joint↗
PubMed1998

Propionibacterium [correction of Proprionibacterium] acnes infections of cerebrospinal fluid shunts.

Ventricular cerebrospinal fluid shunt infections with Propionibacterium acnes are generally low-grade, indolent infections. Typical presentations include gradual shunt malfunction, nausea, headache, malaise, and infrequently, fever. In all, 489 shunt procedures performed between January 1992 and December 1995, and in 15 of these cases P. acnes was subsequently cultured from reservoir taps or an intraoperative culture which was obtained when the existing shunt components were revised. Six of these, representing 14.6% of shunt infections, were considered to be true P. acnes shunt infections, as they were associated with either CSF leukocytosis or the identification of gram-positive rods by gram stain. The others were considered to be probable contaminants. Detailed analysis of all 15 of these cases revealed that no patient had positive CSF cultures after removal of the infected shunt and the initiation of antibiotics. Given the benign characteristics of P. acnes shunt infections, the broad sensitivity to antibiotics, and the rapid sterilization of the cerebrospinal fluid, it may be possible to treat such cases with short-term perioperative antibiotics and replacement of the shunt components, in place of prolonged external ventricular drainage and antibiotics. This would have eliminated 8 operative procedures and reduced the estimated length of stay by 77 patient-days in these 15 children.

Adolescent↗
PubMed1998

Intraoperative hypothermia and ventricular shunt infections.

Several recent studies have demonstrated a relationship between intraoperative hypothermia and postoperative infection. A study was therefore conducted to evaluate the relationship between intraoperative hypothermia and ventricular shunt infections. Sixty-eight children who underwent ventricular shunt placement, including revisions, over a six year period subsequently developed a shunt infection (overall shunt infection rate of 5%). Mean age was 8 years (range, neonate to 20 years). The last 74 children who underwent ventricular shunt placement without subsequent infection served as a comparison group. The anesthetic records of all cases were reviewed to determine the lowest core temperature recorded during the surgical procedure. The lowest core temperature varied from 33.9 degrees C to 37.7 degrees C (mean 36.0 degrees C). Hypothermia was defined as a temperature less than 35.1 degrees C. No relationship was found between hypothermia and the subsequent occurrence of a shunt infection (P = 0.45). When those children less than 2 years old were excluded from analysis, there was a trend towards statistical significance (P = 0.07). In summary, this study failed to show any significant relationship between the occurrence of intraoperative hypothermia and subsequent ventriculoperitoneal shunt infection in a group of pediatric patients.

Adolescent↗
PubMed1998

Medulloblastoma and glioblastoma multiforme in a patient with Turcot syndrome: a case report.

BACKGROUND: Turcot syndrome (TS) or the glioma-polyposis syndrome, is a rare, heritable disorder thought by some authors to be a variant of familial adenomatous polyposis (FAP). It is characterized by central nervous system (CNS) neoplasms and gastrointestinal polyposis. METHODS: We present a case report of a patient who developed a medulloblastoma at age 5 years. Ten years later, she developed adenocarcinoma of the colon. Seven months after resection of this Dukes' C2 adenocarcinoma, she presented with a second primary CNS tumor, a glioblastoma multiforme. The patient's colonic adenocarcinoma and glioblastoma were evaluated histologically and cytogenetically. RESULTS: Cytogenetic analysis revealed the presence of chromosomal instability in both tumors. This unusual case of two primary CNS neoplasms in a patient with TS is presented with a review of the literature. CONCLUSIONS: The implications of the cytogenetic analysis are discussed in conjunction with the present knowledge of the molecular biology of TS.

Adenocarcinoma↗
PubMed1998

Recurrent tethering: a common long-term problem after lipomyelomeningocele repair.

The authors reviewed the records of 94 patients who underwent initial repair of a lipomyelomeningocele between 1982 and July 1996 at the Children's Hospital of Pittsburgh to determine the incidence and time course of symptomatic retethering. In each of these patients, the initial operative goals were to microsurgically debulk as much of the lipoma as possible to allow the conus to move freely within the spinal canal, to divide any tethering arachnoidal adhesions, to close the pia if possible and to reconstitute a capacious thecal sac, using a dural graft if necessary. With a median follow-up of 58 months, 19 of these patients (20.2%) required 28 subsequent operations for symptomatic retethering. Median time between the initial procedure and reoperation for retethering was 52 months. The primary complaint of 12 patients was intractable low-back or leg pain. Other common symptoms were progressive bowel and/or bladder dysfunction, deterioration of motor function and foot deformities. The decision to reoperate was based predominantly on the clinical situation of the patient; magnetic resonance imaging was used to confirm the location and extent of tethering. Patients with transitional lipomas had a significantly higher frequency of symptomatic retethering than those with caudal or dorsal lesions (p < 0.05). No other clinical or technical feature correlated with an increased frequency of retethering. In particular, none of a variety of types of dural graft materials appeared to entirely prevent symptomatic retethering. Following reoperation, pain complaints resolved and many of the other symptoms improved partially or resolved completely. Although the long-term results were also favorable in the majority of patients, a small subgroup (n = 6) exhibited repetitive symptomatic tethering that proved increasingly difficult to treat. We concluded that symptomatic retethering is a common problem in children with lipomyelomeningoceles, even after an adequate initial operation. To date, no type of graft material has been shown to entirely prevent this problem. Close long-term surveillance of such patients is required to allow detection and treatment of symptomatic retethering.

Adolescent↗
PubMed1998

Infusion of intrathecal baclofen for generalized dystonia in cerebral palsy.

UNLABELLED: Generalized dystonia occurs in 15 to 25% of persons with cerebral palsy (CP) and responds poorly to medical and surgical treatments. OBJECT: After the authors observed a woman whose dystonic CP was dramatically improved by continuous infusion of intrathecal baclofen, they designed this pilot study to evaluate the effect of this treatment on a group of patients with dystonic CP. METHODS: The authors assessed the short-term response to intrathecal baclofen infusion in 12 patients with dystonic CP. An intrathecal catheter was inserted percutaneously and connected to an external microinfusion pump. The infusion began at a rate of 100 microg/day and was increased by 50 microg every 12 hours until the dystonia abated, adverse effects occurred, or the dose reached 900 microg/day with no improvement. Two observers, one blinded and one not blinded to the patient's treatment status, viewed videotapes made before and after the infusions and graded the dystonia in eight body regions, using a 5-point scale. Overall and regional scores were compared by using Wilcoxon signed-rank tests. CONCLUSIONS: Dystonia diminished in 10 of 12 patients whose average daily dose of intrathecal baclofen was 575 microg. Overall dystonia scores and scores for the extremities, trunk, and cervical regions were significantly better after infusion (p = 0.003). The two observers' scores were not significantly different. Programmable infusion pumps were subsequently implanted in eight patients for long-term therapy and improvement was sustained in six (p < 0.05). Intrathecal baclofen infusion is a promising treatment option for generalized dystonia associated with CP. The effects of intrathecal baclofen infusion on dystonia can be evaluated by using short-term continuous infusions.

Adolescent↗
PubMed1998

Survival and prognostic factors following radiation therapy and chemotherapy for ependymomas in children: a report of the Children's Cancer Group.

OBJECT: Ependymomas in children continue to generate controversy regarding their histological diagnosis and grading. optimal management, and possible prognostic factors. To increase our knowledge of these tumors the authors addressed these issues in a cohort of children with prospectively staged ependymomas treated with radiotherapy and chemotherapy. METHODS: Children between the ages of 2 and 17.3 years harboring an intracranial ependymoma confirmed by a central review of the tumor's pathological characteristics were treated according to Children's Cancer Group Protocol 921 from 1986 to 1992. Treatment following surgery and postoperative tumor staging (including brain computerized tomography or magnetic resonance [MR] imaging, spinal MR imaging or myelography, and cerebrospinal fluid cytological investigation) included craniospinal irradiation with a local boost to the primary tumor and patient randomization to receive adjuvant chemotherapy with either 1) CCNU, vincristine, and prednisone, or 2) the eight-drugs-in-1-day regimen. Centralized review of the tumor pathological characteristics revealed 20 ependymomas and 12 anaplastic ependymomas in the 32 children included in the study. Diagnoses made at the individual institutions included anaplastic (malignant) ependymoma (15 patients), ependymoma (four patients), ependymoblastoma (nine patients), ependymoastrocytoma (one patient), and primitive neuroectodermal tumor (three patients), which were discordant with the centralized review diagnosis in 22 of 32 cases. Only three of the 32 patients had metastatic disease (two with M and one with M3 stages). At surgery, 47% of tumors were estimated to be totally resected. Among the 14 of 17 patients who suffered a relapse and were evaluated for site of relapse, 10 (71%) had an isolated local relapse, three (21%) had concurrent local and metastatic relapse, and only one (7%) had an isolated metastatic relapse. Kaplan-Meier estimates of 5-year progression-free survival (PFS) and overall survival rates were 50 +/- 10% and 64 +/- 9%, respectively. CONCLUSIONS: Predictors of PFS duration included an estimate of the extent of resection made at surgery (total compared with less than total, p = 0.0001) and the amount of residual tumor on postoperative imaging as verified by centralized radiological review (< or = 1.5 cm2 compared with > 1.5 cm2, p < 0.0001). No other factors, including centrally reviewed tumor histopathological type, location, metastasis and tumor (M and T) stages, patient age, race, gender, or chemotherapy treatment regimen significantly correlated with PFS duration. The pattern of predominantly local relapse and the important influence of residual tumor or the extent of resection on PFS duration confirms a prevailing impression that local disease control is the major factor in the prediction of outcome of ependymoma. Survival rates were comparable with those reported by other investigators who have treated patients with similar doses of radiation and no chemotherapy.

Adolescent↗