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小儿颅咽管瘤的经蝶窦显微手术:关于适应症和方法的特殊考量

Transsphenoidal microsurgery for pediatric craniopharyngioma: special considerations regarding indications and method.

作者信息

Im So-Hyang, Wang Kyu-Chang, Kim Seung-Ki, Chung You-Nam, Kim Hee-Soo, Lee Chul-Hee, Cho Byung-Kyu

机构信息

Department of Neurosurgery, Seoul National University College of Medicine, Seoul, Korea.

出版信息

Pediatr Neurosurg. 2003 Jul;39(2):97-103. doi: 10.1159/000071321.

DOI:10.1159/000071321
PMID:12845200
Abstract

Although the transsphenoidal approach offers a narrow operative field and poor visual control through a small sellar opening, this approach has the advantage of being associated with minimal surgical trauma, especially in children. We share our experience of good surgical outcome achieved by the transsphenoidal approach in selected craniopharyngiomas in childhood, and report on our pediatric series of transsphenoidal complete removals of six relatively large craniopharyngiomas and one Rathke's cleft cyst. The most common presenting symptom was visual dysfunction (6/7). All tumors were 'prechiasmatic' cystic masses with moderate to marked suprasellar extensions and elevated diaphragm sellae. The tumors measured from 25 to 37 mm in maximum diameter in the midsagittal plane (median 33 mm). In 5 of the 7 tumors, the suprasellar portion (range 11-27 mm) was larger than the intrasellar portion (range 8-14 mm). Gross total resection was achieved in all patients. Special attention should be paid to multicystic craniopharyngiomas to prevent the possibility of incomplete tumor resection. The 'bone in a fat pocket' method was useful for preventing postoperative cerebrospinal fluid leakage. Vision was improved in all six patients who had preoperative visual disturbances. Hypopituitarism was provoked by radical tumor removal in all patients and managed by hormonal supplementation therapy. Transsphenoidal surgery is an appropriate approach for the radical excision of intrasellar-suprasellar 'prechiasmatic' craniopharyngiomas, even in children, and even if the tumor has a relatively large suprasellar component.

摘要

尽管经蝶窦入路提供的手术视野狭窄,通过较小的鞍区开口进行视觉控制不佳,但该入路具有手术创伤最小的优点,尤其是在儿童中。我们分享了经蝶窦入路在儿童期特定颅咽管瘤中取得良好手术效果的经验,并报告了我们小儿经蝶窦完全切除6例相对较大颅咽管瘤和1例拉克氏囊肿的系列病例。最常见的首发症状是视觉功能障碍(6/7)。所有肿瘤均为“视交叉前”囊性肿块,鞍上有中度至明显延伸,鞍膈抬高。肿瘤在矢状面最大直径为25至37毫米(中位数33毫米)。7例肿瘤中有5例,鞍上部分(范围11 - 27毫米)大于鞍内部分(范围8 - 14毫米)。所有患者均实现了肿瘤全切除。对于多囊性颅咽管瘤应特别注意,以防止肿瘤切除不完全的可能性。“脂肪袋内骨片”方法有助于预防术后脑脊液漏。术前有视觉障碍的6例患者视力均有改善。所有患者因肿瘤根治性切除导致垂体功能减退,并通过激素补充疗法进行处理。经蝶窦手术是切除鞍内 - 鞍上“视交叉前”颅咽管瘤的合适方法,即使在儿童中,即使肿瘤鞍上部分相对较大。

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