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Hani J. Marcus, Fahid T. Rasul, Ziad Hussein, Stephanie E. Baldeweg, Helen A. Spoudeas, Richard Hayward, Noor ul Owase Jeelani, Dominic Thompson, Joan P. Grieve, Neil L. Dorward, and Kristian Aquilina

OBJECTIVE

The management of children with craniopharyngioma has evolved over time, with a trend toward less invasive neurosurgical approaches as surgeons have sought to balance oncological control and treatment-related morbidity. To this end, the aim of this study was to evaluate the safety and effectiveness of the current management of children with craniopharyngioma compared to the previous management methods used at the authors’ treatment center.

METHODS

A prospectively maintained database was searched over a 14-year period between January 1, 2005, and December 31, 2018, to identify all children 17 years of age or younger with a new diagnosis of craniopharyngioma. A retrospective case note review was performed for each child to extract data on the presentation, investigation, treatment, and outcome of their illness. Morbidity was assessed in the same fashion as in previous cohorts, according to the following categories: visual loss, pituitary dysfunction, hypothalamic dysfunction, neurological deficits, and cognitive impairment.

RESULTS

In total, 59 children were identified with craniopharyngioma during the study period. A total of 92 operations were performed, including cyst drainage (35/92; 38.0%), craniotomy and resection (30/92; 32.6%), and transsphenoidal resection (16/92; 17.4%). Approximately two-thirds of all operations were performed using image guidance (66/92; 71.7%) and one-third were performed using endoscopy (27/92; 29.3%). The majority of children had adjuvant therapy comprising proton beam therapy (18/59; 30.5%) or conventional radiotherapy (16/59; 27.1%). The median follow-up duration was 44 months (range 1–142 months), and approximately one-half of the children had no evidence of residual disease on MRI studies (28/59; 47.5%). Of the remaining 31 children, there was a reduction in the volume of residual disease in 8 patients (8/59; 13.6%), stable residual disease in 18 (18/59; 30.5%), and tumor growth in 5 patients (5/59; 8.5%). There was significantly reduced morbidity (p < 0.05) in all categories in the current cohort compared with our last cohort (1996–2004).

CONCLUSIONS

The authors’ institutional experience of pediatric craniopharyngioma confirms a trend toward less invasive neurosurgical procedures, most of which are now performed with the benefit of image guidance or endoscopy. Moreover, the authors have identified an expanding role for more targeted radiotherapy for children with residual disease. These advances have allowed for tumor control comparable to that achieved in previous cohorts, but with significantly reduced morbidity and mortality.

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Sebastian M. Toescu, Gargi Samarth, Hugo Layard Horsfall, Richard Issitt, Ben Margetts, Kim P. Phipps, Noor-ul-Owase Jeelani, Dominic N. P. Thompson, and Kristian Aquilina

OBJECTIVES

The goal of this study was to characterize the complications and morbidity related to the surgical management of pediatric fourth ventricle tumors.

METHODS

All patients referred to the authors’ institution with posterior fossa tumors from 2002 to 2018 inclusive were screened to include only true fourth ventricle tumors. Preoperative imaging and clinical notes were reviewed to extract data on presenting symptoms; surgical episodes, techniques, and adjuncts; tumor histology; and postoperative complications.

RESULTS

Three hundred fifty-four children with posterior fossa tumors were treated during the study period; of these, 185 tumors were in the fourth ventricle, and 167 fourth ventricle tumors with full data sets were included in this analysis. One hundred patients were male (mean age ± SD, 5.98 ± 4.12 years). The most common presenting symptom was vomiting (63.5%). The most common tumor types, in order, were medulloblastoma (94 cases) > pilocytic astrocytoma (30 cases) > ependymoma (30 cases) > choroid plexus neoplasms (5 cases) > atypical teratoid/rhabdoid tumor (4 cases), with 4 miscellaneous lesions. Of the 67.1% of patients who presented with hydrocephalus, 45.5% had an external ventricular drain inserted (66.7% of these prior to tumor surgery, 56.9% frontal); these patients were more likely to undergo ventriculoperitoneal shunt (VPS) placement at a later date (p = 0.00673). Twenty-two had an endoscopic third ventriculostomy, of whom 8 later underwent VPS placement. Overall, 19.7% of patients had a VPS sited during treatment.

Across the whole series, the transvermian approach was more frequent than the telovelar approach (64.1% vs 33.0%); however, the telovelar approach was significantly more common in the latter half of the series (p < 0.001). Gross-total resection was achieved in 70.7%. The most common postoperative deficit was cerebellar mutism syndrome (CMS; 28.7%), followed by new weakness (24.0%), cranial neuropathy (18.0%), and new gait abnormality/ataxia (12.6%). Use of intraoperative ultrasonography significantly reduced the incidence of CMS (p = 0.0365). There was no significant difference in the rate of CMS between telovelar or transvermian approaches (p = 0.745), and multivariate logistic regression modeling did not reveal any statistically significant relationships between CMS and surgical approach.

CONCLUSIONS

Surgical management of pediatric fourth ventricle tumors continues to evolve, and resection is increasingly performed through the telovelar route. CMS is enduringly the major postoperative complication in this patient population.

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Ahmed Elawadly, Luke Smith, Alessandro Borghi, Khaled I. Abdelaziz, Adikarige Haritha Dulanka Silva, David J. Dunaway, Noor ul Owase Jeelani, Juling Ong, and Greg James

OBJECTIVE

Endoscopic strip craniectomy with postoperative helmet orthosis therapy (ESCH) has emerged as a less invasive alternative to fronto-orbital remodeling for correction of trigonocephaly. However, there is no standardized objective method for monitoring morphological changes following ESCH. Such a method should be reproducible and avoid the use of ionizing radiation and general anesthesia for diagnostic imaging. The authors analyzed a number of metrics measured using 3D stereophotogrammetry (3DSPG) following ESCH, an imaging alternative that is free of ionizing radiation and can be performed on awake children.

METHODS

3DSPG images obtained at two time points (perisurgical and 1-year follow-up [FU]) of children with metopic synostosis who had undergone ESCH were analyzed and compared to 3DSPG images of age-matched control children without craniofacial anomalies. In total, 9 parameters were measured, the frontal angle and anteroposterior volume in addition to 7 novel parameters: anteroposterior area ratio, anteroposterior width ratios 1 and 2, and right and left anteroposterior diagonal ratios 30 and 60.

RESULTS

Six eligible patients were identified in the operated group, and 15 children were in the control group. All 9 parameters differed significantly between perisurgical and age-matched controls, as well as from perisurgical to FU scans. Comparison of FU scans of metopic synostosis patients who underwent surgery to scans of age-matched controls without metopic synostosis revealed that all parameters were statistically identical, with the exception of the right anteroposterior diagonal ratio 30, which was not fully corrected in the treated patients. The left anterior part of the head showed the most change in surface area maps.

CONCLUSIONS

In this pilot study, ESCH showed satisfactory results at 1 year, with improvements in all measured parameters compared to perisurgical results and normalization of 8 of 9 parameters compared to an age-matched control group. The results indicate that these parameters may be useful for craniofacial units for monitoring changes in head shape after ESCH for trigonocephaly and that 3DSPG, which avoids the use of anesthesia and ionizing radiation, is a satisfactory monitoring method.

Free access

Richard Hayward, Jonathan A. Britto, David Dunaway, Robert Evans, Noor ul Owase Jeelani, and Dominic Thompson