The Changing Face of Craniopharyngioma Treatment in Young Children and its Challenges at a Single Centre in a Developing World Context

dc.contributor.advisorFigaji, Anthony
dc.contributor.authorMankahla, Ncedile
dc.date.accessioned2020-02-13T12:17:01Z
dc.date.available2020-02-13T12:17:01Z
dc.date.issued2019
dc.date.updated2020-02-12T10:17:04Z
dc.description.abstractObjective: To retrospectively review our institutional experience with the treatment of paediatric craniopharygiomas and assess the evolution in management and influence on patient outcomes. Patients and Methods: A retrospective review from January 1995 to December 2015 of children age <14 treated at a single institution. Data collected included admission clinical features, endocrine function, surgery performed, surgical outcome, intracystic therapy and radiotherapy. Long-term functional outcome was calculated considering hormonal dependence, level of independence and schooling. Results: There were 41 patients with a mean age of 84.2 months: 57% were female. Primary surgical resection was performed in 36 patients: 80.5% had subtotal resection, 11% had gross total resection and the rest had biopsy only. Of surgical approaches, 60,7% had pterional craniotomy and 39,2% supraorbital keyhole craniotomy. No surgical mortalities occurred but 2 patients had new post-operative neurological deficits. Stereotactic placement of intracystic catheters transitioned to endoscopic. Intracystic treatments transitioned from Yttrium (1) to Bleomycin (6) to Interferon Alpha (6). Radiotherapy was given in 30 patients, median dose 54Gy. Final Wen functional outcome was 21,8% Class I, 32% Class II and 46% Class III. There were no early deaths in the series but 5 patients died more than 6 years after diagnosis, mostly due to endocrine crises from poor chronic care. Conclusion: The findings reflect a multidisciplinary team approach consisting of maximal safe resection with radiotherapy, intracystic agents and endocrine support. For a cohort limited to young children, our results are similar in number and outcomes to other published series. Mortality remains low but lifelong dependence on endocrine replacement is a significant contributor to long-term morbidity and mortality. This has important implications for patients referred from large distances and where primary and secondary follow up care is poor.
dc.identifier.apacitationMankahla, N. (2019). <i>The Changing Face of Craniopharyngioma Treatment in Young Children and its Challenges at a Single Centre in a Developing World Context</i>. (). ,Faculty of Health Sciences ,Division of Neurosurgery. Retrieved from http://hdl.handle.net/11427/31106en_ZA
dc.identifier.chicagocitationMankahla, Ncedile. <i>"The Changing Face of Craniopharyngioma Treatment in Young Children and its Challenges at a Single Centre in a Developing World Context."</i> ., ,Faculty of Health Sciences ,Division of Neurosurgery, 2019. http://hdl.handle.net/11427/31106en_ZA
dc.identifier.citationMankahla, N. 2019. The Changing Face of Craniopharyngioma Treatment in Young Children and its Challenges at a Single Centre in a Developing World Context.en_ZA
dc.identifier.ris TY - Thesis / Dissertation AU - Mankahla, Ncedile AB - Objective: To retrospectively review our institutional experience with the treatment of paediatric craniopharygiomas and assess the evolution in management and influence on patient outcomes. Patients and Methods: A retrospective review from January 1995 to December 2015 of children age <14 treated at a single institution. Data collected included admission clinical features, endocrine function, surgery performed, surgical outcome, intracystic therapy and radiotherapy. Long-term functional outcome was calculated considering hormonal dependence, level of independence and schooling. Results: There were 41 patients with a mean age of 84.2 months: 57% were female. Primary surgical resection was performed in 36 patients: 80.5% had subtotal resection, 11% had gross total resection and the rest had biopsy only. Of surgical approaches, 60,7% had pterional craniotomy and 39,2% supraorbital keyhole craniotomy. No surgical mortalities occurred but 2 patients had new post-operative neurological deficits. Stereotactic placement of intracystic catheters transitioned to endoscopic. Intracystic treatments transitioned from Yttrium (1) to Bleomycin (6) to Interferon Alpha (6). Radiotherapy was given in 30 patients, median dose 54Gy. Final Wen functional outcome was 21,8% Class I, 32% Class II and 46% Class III. There were no early deaths in the series but 5 patients died more than 6 years after diagnosis, mostly due to endocrine crises from poor chronic care. Conclusion: The findings reflect a multidisciplinary team approach consisting of maximal safe resection with radiotherapy, intracystic agents and endocrine support. For a cohort limited to young children, our results are similar in number and outcomes to other published series. Mortality remains low but lifelong dependence on endocrine replacement is a significant contributor to long-term morbidity and mortality. This has important implications for patients referred from large distances and where primary and secondary follow up care is poor. DA - 2019 DB - OpenUCT DP - University of Cape Town KW - Neurosurgery LK - https://open.uct.ac.za PY - 2019 T1 - The Changing Face of Craniopharyngioma Treatment in Young Children and its Challenges at a Single Centre in a Developing World Context TI - The Changing Face of Craniopharyngioma Treatment in Young Children and its Challenges at a Single Centre in a Developing World Context UR - http://hdl.handle.net/11427/31106 ER - en_ZA
dc.identifier.urihttp://hdl.handle.net/11427/31106
dc.identifier.vancouvercitationMankahla N. The Changing Face of Craniopharyngioma Treatment in Young Children and its Challenges at a Single Centre in a Developing World Context. []. ,Faculty of Health Sciences ,Division of Neurosurgery, 2019 [cited yyyy month dd]. Available from: http://hdl.handle.net/11427/31106en_ZA
dc.language.rfc3066eng
dc.publisher.departmentDivision of Neurosurgery
dc.publisher.facultyFaculty of Health Sciences
dc.subjectNeurosurgery
dc.titleThe Changing Face of Craniopharyngioma Treatment in Young Children and its Challenges at a Single Centre in a Developing World Context
dc.typeMaster Thesis
dc.type.qualificationlevelMasters
dc.type.qualificationnameMMed
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