DeVile, Catherine Josephine; (1999) A follow-up study of the outcome of children post-craniopharyngioma surgery. Doctoral thesis (M.D), UCL (University College London).

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Abstract

The management of craniopharyngiomas in childhood remains both complex and controversial. Although histologically benign, this tumour often follows a more malignant course, not only in terms of local disease progression but also in terms of visual, neurological, neuropsychological and endocrine outcome. Seventy-five children diagnosed as having a craniopharyngioma between the ages of 1.0 and 16.4 years and treated from 1973 to early 1994 were studied to investigate the associated morbidity and mortality of this tumour and its treatment and to demonstrate which pre- and intra-operative factors were indicative of a poor outcome as defined by a quantitive assessment of morbidity. All patients had tumour surgery which entailed attempted total excision in 59 cases and subtotal resection or cyst aspiration in 16 cases. Thirty-seven children received radiotherapy, 21 following tumour recurrence. The study involved a review of clinical details and cranial imaging of all patients and a follow-up study assessment of 66 survivors - which included ophthalmological, neurological, psychological and growth and endocrine evaluation. Sixty-three patients underwent magnetic resonance imaging with a 3-dimensional volume acquisition sequence. Predictors of high morbidity included severe hydrocephalus, intra-operative adverse events and young age at presentation. Predictors of increased hypothalamic morbidity included symptoms of hypothalamic disturbance already established at diagnosis, greater height of the tumour in the mid-line, and attempts to remove adherent tumour from the region of the hypothalamus at operation. Large tumour size, young age, and severe hydrocephalus were predictors of tumour recurrence, whereas complete tumour resection (as determined by post-operative neuroimaging) and radiotherapy given electively after subtotal excision were significantly less likely to be associated with recurrent disease. Risk factors for poor cognitive outcome included complications at the time of operation and multiple surgical procedures. Treatment with radiotherapy did not significantly influence intellectual outcome. At follow-up assessment, 15% of all patients were blind, 24% had severe neurological sequelae, 56% had evidence of hypothalamic dysfunction, excluding the endocrinopathies which were almost universal, and 75% of patients had evidence of behavioural or educational difficulties. Although severe hypothalamic syndromes were uncommon (16%), they contributed significantly to morbidity and mortality and the clinical manifestations - particularly post-operative weight gain - correlated well with the extent of hypothalamic damage seen on magnetic resonance imaging. Based on these findings, it is clear that close liaison with a multidisciplinary team is essential, so that the spectra of possible sequelae can be identified early and appropriate support instituted promptly. An individualised, more flexible treatment approach is proposed whereby surgical strategies may be modified in an attempt to provide long-term tumour control with the lowest morbidity.

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