National Neurosurgical Centre, Beaumont Hospital, Dublin, Ireland.
Trinity College Dublin, Dublin, Ireland.
Childs Nerv Syst. 2021 May;37(5):1547-1561. doi: 10.1007/s00381-021-05108-9. Epub 2021 Mar 5.
Endoscopic extended transsphenoidal surgery (EETSS) has gained popularity for treatment of craniopharyngiomas. The aim of this study is to assess the outcome of endoscopic extended transsphenoidal surgery (EETSS) for newly diagnosed paediatric craniopharyngiomas.
Patient details were obtained from a prospective database of all endoscopic transnasal operations performed by a single surgeon. Outcomes including visual function, pituitary function, body mass index (BMI), postoperative neurological deficit, extent of resection and recurrence on follow-up were obtained. Obesity was defined as BMI percentile of equal to or greater than 95%.
Between January 2011 and January 2020, 15 of 16 children (5-18 years old) with newly diagnosed craniopharyngiomas underwent EETSS. Four patients had a conchal-type sphenoid sinus. Gross total resection (GTR) was achieved in 4 patients and near total resection (NTR) in 5 patients. The remaining 6 had subtotal resection (STR). Postoperative radiotherapy was used in 6 patients (4 with STR, 2 with NTR). There were no postoperative deaths, strokes or CSF leaks. Normalisation of visual fields (VF) occurred in 9/13 patients with preoperative VF defects. One patient developed a new visual field defect. During a median follow-up period of 74 (8-104) months, 2 patients have required further surgery for tumour progression following initial STR, where a tumour remnant was left in situ to preserve the pituitary stalk. 6/11 patients developed new anterior pituitary dysfunction as a result of surgery and 9/12 developed new diabetes insipidus (DI). At the time of last follow-up, 14/15 children had anterior panhypopituitarism, 13/15 had DI and 1 patient developed new onset obesity. Two patients, who were obese preoperatively, were no longer obese at last follow-up.
EETSS can be performed as the first option in the majority of children with newly diagnosed craniopharyngioma, despite factors such as small nose, non-pneumatised sphenoid sinus, small sella or purely suprasellar tumour location. Preservation of the pituitary stalk at the expense of leaving residual tumour may not be in the best interests of the patient.
内镜扩大经蝶窦手术（EETSS）已广泛应用于颅咽管瘤的治疗。本研究旨在评估内镜扩大经蝶窦手术（EETSS）治疗新诊断的小儿颅咽管瘤的疗效。
从一位外科医生进行的所有内镜经鼻手术的前瞻性数据库中获取患者详细信息。获取的结果包括视觉功能、垂体功能、体重指数（BMI）、术后神经功能缺损、切除范围和随访时的复发情况。肥胖定义为 BMI 百分位数等于或大于 95%。
2011 年 1 月至 2020 年 1 月，16 例新诊断为颅咽管瘤的儿童（5-18 岁）接受了 EETSS。4 例患者为蝶窦型，4 例患者行全切除（GTR），5 例患者行近全切除（NTR），6 例患者行次全切除（STR）。6 例患者术后行放疗（4 例 STR，2 例 NTR）。无术后死亡、中风或脑脊液漏。13 例术前存在视野缺损的患者中，9 例术后视野正常化。1 例患者出现新的视野缺损。中位随访 74（8-104）个月后，2 例患者因初次 STR 后肿瘤进展需要再次手术，术中将肿瘤残留在原位以保留垂体柄。11 例患者中，6 例因手术导致新的垂体前叶功能障碍，9 例患者出现新的尿崩症（DI）。末次随访时，15 例患儿中 14 例出现全垂体前叶功能减退，15 例出现 DI，1 例出现新发肥胖症。2 例术前肥胖的患者，末次随访时已不再肥胖。
尽管存在鼻小、蝶窦未气化、鞍底小或单纯鞍上肿瘤位置等因素，内镜扩大经蝶窦手术仍可作为新诊断颅咽管瘤患儿的首选治疗方法。为保留垂体柄而残留肿瘤可能不符合患者的最佳利益。
