Jane John A, Prevedello Daniel M, Alden Tord D, Laws Edward R
Department of Neurosurgery, University of Virginia Health System, Charlottesville, Virginia 22908, USA.
J Neurosurg Pediatr. 2010 Jan;5(1):49-60. doi: 10.3171/2009.7.PEDS09252.
The majority of pediatric craniopharyngiomas are treated using a transcranial approach. Although there is an increasing acceptance of transsphenoidal resection in adults, there are few reports describing this approach in the pediatric population. The purpose of this study is to report the outcomes after transsphenoidal surgery in a consecutive series of pediatric patients with craniopharyngiomas treated at a single institution with the goal of gross-total resection (GTR).
Twenty-three patients with pathologically proven craniopharyngiomas were identified who were 18 years of age or less at the time of surgery. The medical records and imaging studies of the patients were retrospectively reviewed. One patient who was lost to follow-up after surgery was excluded.
Among the 22 patients included in the study, 11 underwent transsphenoidal surgery as the primary procedure and 11 underwent transsphenoidal surgery as a secondary procedure after a previous procedure. All patients had at least some sellar component to their tumor and all had either anterior or posterior pituitary dysfunction at presentation. In the entire cohort, a GTR was achieved in 15 (68%) of 22 patients, a radical subtotal resection in 4 (18%) of 22 patients, a subtotal resection in 1 patient, and a partial resection in 2 patients. The degree of resection was higher in the primary transsphenoidal group. After a mean follow-up of 82 months, 4 patients (18%) experienced recurrence. Recurrence occurred in 13% after GTR compared with 28.5% after all other degrees of resection. Tumor recurred in 9% of the primary transsphenoidal group and in 30% of patients who had undergone other therapies prior to the transsphenoidal operation. No patient who had panhypopituitarism experienced a gain of function postoperatively, 67% developed new panhypopituitarism, and 56% experienced new diabetes insipidus. Vision improved or normalized in 9 (64%) of 14 patients presenting with visual loss. Complications included 1 death 3 weeks postoperatively, 2 CSF leaks, and new obesity in 37%.
Transsphenoidal resection of pediatric craniopharyngiomas results in a high rate of both visual improvement and GTR with a low associated risk of recurrence. The transsphenoidal approach should be considered in selected pediatric patients with craniopharyngioma, especially those with infradiaphragmatic origin.
大多数儿童颅咽管瘤采用经颅入路治疗。尽管经蝶窦切除术在成人中越来越被接受，但关于该方法在儿童患者中的报道较少。本研究的目的是报告在单一机构连续治疗的一系列儿童颅咽管瘤患者经蝶窦手术后的结果，目标是实现全切除（GTR）。
确定23例经病理证实为颅咽管瘤的患者，这些患者在手术时年龄为18岁或以下。对患者的病历和影像学研究进行回顾性分析。排除1例术后失访的患者。
在纳入研究的22例患者中，11例将经蝶窦手术作为主要手术，11例在先前手术后将经蝶窦手术作为二次手术。所有患者的肿瘤至少有部分鞍区成分，且所有患者在就诊时均有垂体前叶或后叶功能障碍。在整个队列中，22例患者中有15例（68%）实现了GTR，22例患者中有4例（18%）实现了根治性次全切除，1例患者为次全切除，2例患者为部分切除。初次经蝶窦手术组的切除程度更高。平均随访82个月后，4例患者（18%）出现复发。GTR后复发率为13%，而其他切除程度后复发率为28.5%。初次经蝶窦手术组的复发率为9%，在经蝶窦手术前接受过其他治疗的患者中复发率为30%。全垂体功能减退的患者术后均未出现功能恢复，67%出现新的全垂体功能减退，56%出现新的尿崩症。14例视力丧失患者中有9例（64%）视力改善或恢复正常。并发症包括术后3周1例死亡、2例脑脊液漏以及37%的患者出现新发肥胖。
儿童颅咽管瘤经蝶窦切除术后视力改善率和GTR率较高，复发风险较低。对于选定的儿童颅咽管瘤患者，尤其是起源于膈下的患者，应考虑经蝶窦入路。
