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Forniceal glioma in children

Clinical article

Thomas Blauwblomme, Pascale Varlet, John R. Goodden, Marie Laure Cuny, Helene Piana, Thomas Roujeau, Federico DiRocco, Jacques Grill, Virginie Kieffer, Nathalie Boddaert, Christian Sainte-Rose and Stéphanie Puget

Object

Five to ten percent of pediatric brain tumors are located in the ventricles. Among them, forniceal lesions are rare and their management has not often been described. The aim of this study was to review the clinical, radiological, and histopathological features as well as the feasibility of surgical excision and the outcomes in these patients.

Methods

From a retrospective analysis of 250 cases of supratentorial pediatric glioma, the records of 8 children presenting with forniceal lesions were selected and reviewed.

Results

The median age of patients in the cohort was 13.5 years. Presenting features included intracranial hypertension (7 cases), hypothalamic dysfunction (2), and memory dysfunction (3). Complete resection was possible in only 1 case, where the lesion was mainly exophytic; the remaining patients had either a partial resection or biopsy. On histological review, the tumors were confirmed as pilocytic astrocytoma (4 lesions), WHO Grade II astrocytoma (3), and ganglioglioma (1). Postoperatively, working and retrograde memory was normal for all patients, but the authors found a mild alteration in verbal episodic memory in 5 patients. Despite fatigability for 5 patients, academic achievement was normal for all but 2, both of whom had preoperative school difficulties. Additional treatment was required for 5 patients for tumor progression, with a median interval of 19 months from surgery. At a median follow-up duration of 4.9 years, all patients had stable disease.

Conclusions

In this series, forniceal gliomas were found to be low-grade gliomas. They are surgically challenging, and only exophytic lesions may be cured surgically. Due to the high rate of progression of residual disease, adjuvant therapy is recommended for infiltrative tumors, and it yielded excellent results.

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Ian A. Anderson, Anand Goomany, David T. Bonthron, Maggie Bellew, Mark I. Liddington, Ian M. Smith, John L. Russell, Lachlan M. Carter, Velupandian Guruswamy, John R. Goodden and Paul D. Chumas

Object

There are no published papers examining the role of ethnicity on suture involvement in nonsyndromic craniosynostosis. The authors sought to examine whether there is a significant difference in the epidemiological pattern of suture(s) affected between different ethnic groups attending a regional craniofacial clinic with a diagnosis of nonsyndromic craniosynostosis.

Methods

A 5-year retrospective case-notes analysis of all cases involving patients attending a regional craniofacial clinic was undertaken. Cases were coded for the patients' declared ethnicity, suture(s) affected by synostosis, and the decision whether to have surgical correction of synostosis. The chi-square test was used to determine whether there were any differences in site of suture affected between ethnic groups.

Results

A total of 312 cases were identified. Of these 312 cases, ethnicity data were available for 296 cases (95%). The patient population was dominated by 2 ethnic groups: white patients (222 cases) and Asian patients (56 cases). There were both more cases of complex synostosis and fewer cases of sagittal synostosis than expected in the Asian patient cohort (χ2 = 9.217, p = 0.027).

Conclusions

There is a statistically significant difference in the prevalence of the various sutures affected within the nonsyndromic craniosynostosis patient cohort when Asian patients are compared with white patients. The data from this study also suggest that nonsyndromic craniosynostosis is more prevalent in the Asian community than in the white community, although there may be inaccuracies in the estimates of the background population data. A larger-scale, multinational analysis is needed to further evaluate the relationship between ethnicity and nonsyndromic craniosynostosis.

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Ian A. Anderson, Louise F. Saukila, James M. W. Robins, Christopher Y. Akhunbay-Fudge, John R. Goodden, Atul K. Tyagi, Nick Phillips and Paul D. Chumas

OBJECTIVE

The aim of this study was to provide a comprehensive benchmark of 30-day ventriculoperitoneal (VP) shunt failure rates for a single institution over a 5-year study period for both adult and pediatric patients, to compare this with the results in previously published literature, and to establish factors associated with shunt failure.

METHODS

A retrospective database search was undertaken to identify all VP shunt operations performed in a single, regional neurosurgical unit during a 5-year period. Data were collected regarding patient age, sex, origin of hydrocephalus, and whether the shunt was a primary or secondary shunt. Operative notes were used to ascertain the type of valve inserted, which components of the shunt were adjusted/replaced (in revision cases), level of seniority of the most senior surgeon who participated in the operation, and number of surgeons involved in the operation. Where appropriate and where available, postoperative imaging was assessed for grade of shunt placement, using a recognized grading system. Univariate and multivariate models were used to establish factors associated with early (30-day) shunt failure.

RESULTS

Six hundred eighty-three VP shunt operations were performed, of which 321 were pediatric and 362 were adult. The median duration of postoperative follow-up for nonfailed shunts (excluding deaths) was 1263 days (range 525–2226 days). The pediatric 30-day shunt failure rates in the authors’ institution were 8.8% for primary shunts and 23.4% for revisions. In adults, the 30-day shunt failure rates are 17.7% for primary shunts and 25.6% for revisions. In pediatric procedures, the number of surgeons involved in the operating theater was significantly associated with shunt failure rate. In adults, the origin of hydrocephalus was a statistically significant variable. Primary shunts lasted longer than revision shunts, irrespective of patient age.

CONCLUSIONS

A benchmark of 30-day failures is presented and is consistent with current national databases and previously published data by other groups. The number of surgeons involved in shunt operations and the origin of the patient’s hydrocephalus should be described in future studies and should be controlled for in any prospective work. The choice of shunt valve was not a significant predictor of shunt failure. Most previous studies on shunts have concentrated on primary shunts, but the high rate of early shunt failure in revision cases (in both adults and children) is perhaps where future research efforts should be concentrated.