Management of cystic craniopharyngiomas in childhood by a transsphenoidal approach

J Pediatr Endocrinol Metab. 2006 Apr:19 Suppl 1:381-8.

Abstract

The transsphenoidal approach has specific indications in the management of craniopharyngiomas. Usually, it is best reserved for patients with preferably cystic extra-arachnoid-infradiaphragmatic tumors with small suprasellar extension. Moreover, it is definitely less traumatic than transcranial approaches and it has been proven to be feasible also in paediatric patients. When possible, radical removal of these tumours must be the goal of surgery, but this attitude, which reduces but not eliminates the risk of relapse, has to be counterbalanced by heavy morbidity and even mortality, especially in children. In this view, many neurosurgeons favour a more 'conservative' approach with subtotal removal followed by radiotherapy whose dramatic efficacy on craniopharyngiomas is well known. With these premises, a transsphenoidal approach is realistically applicable to a greater number of large cystic craniopharyngiomas if the aim is not radical removal, but is to drain them into the sphenoid sinus to relieve mass effect symptoms (cystosphenoidostomy), and delay radiotherapy and its detrimental effects on visual and pituitary function, especially in younger patients, to a more suitable time after surgery.

MeSH terms

  • Activities of Daily Living
  • Adolescent
  • Child
  • Craniopharyngioma / surgery*
  • Female
  • Humans
  • Male
  • Neurosurgical Procedures* / adverse effects
  • Neurosurgical Procedures* / mortality
  • Pituitary Neoplasms / surgery*
  • Sphenoid Bone*
  • Sphenoid Sinus / physiology
  • Treatment Outcome