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经蝶窦入路治疗儿童囊性颅咽管瘤

Management of cystic craniopharyngiomas in childhood by a transsphenoidal approach.

作者信息

Zona Gianluigi, Spaziante Renato

机构信息

Department of Neurosurgery, San Martino University Hospital, University of Genoa, Genoa, Italy.

出版信息

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.

摘要

经蝶窦入路在颅咽管瘤的治疗中有特定的适应证。通常,它最适合用于那些患有囊状蛛网膜外膈下肿瘤且鞍上扩展较小的患者。此外,它肯定比经颅入路创伤小,并且已被证明在儿科患者中也是可行的。在可能的情况下,手术的目标必须是彻底切除这些肿瘤,但这种做法虽然降低了复发风险但并未消除复发风险,而且会带来严重的发病率甚至死亡率,尤其是在儿童中。从这个角度来看,许多神经外科医生倾向于采用更“保守”的方法,即次全切除后进行放疗,放疗对颅咽管瘤的显著疗效是众所周知的。基于这些前提,如果目标不是彻底切除,而是将大的囊性颅咽管瘤引流至蝶窦以缓解占位效应症状(囊蝶窦造口术),并将放疗及其对视觉和垂体功能的有害影响推迟到手术后更合适的时间,那么经蝶窦入路实际上适用于更多的大囊性颅咽管瘤,特别是在年轻患者中。

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