Ciurea A V, Saceleanu V, Mohan A, Moreanu M S, Toader C
"Carol Davila" University School of Medicine - Department of Neurosurgery.
"Lucian Blaga" University of Sibiu, Faculty of Medicine - Department of Neurosurgery.
Acta Endocrinol (Buchar). 2020 Jan-Mar;16(1):103-109. doi: 10.4183/aeb.2020.103.
Craniopharyngiomas (CPH) are benign tumors, rarely encountered in children, representing 5-6% of all intracranial tumors.
This study aimed to analyze the surgical management and quality of life in a series of CPH pediatric cases.
This was a multicenter study performed over a 25-year period (1994 - 2019) in Bucharest.
152 children (0-17 years old) were treated for CPH. Preoperative manifestations were intracranial hypertension, endocrine dysfunction, visual impairment, ataxia, intellectual performance decrease.
Considering all surgical approaches used, we advocate for pterional approach to best fit in CPH. We achieved gross-total removal (GTR) in 83 cases (54.4%), near-total resection (NTR) in 13 cases (9%), partial resection (PTR) in 51 cases (33.3%). 5 cases were biopsies (3.2%). Gamma Knife Surgery was performed in 10 cases (6.5%), all recurrences. At 6 months GOS revealed: Good Recovery 70 cases (46.2%), Moderate Disability 62 cases (40.7%), Severe Disability 13 (8.5%), Vegetative State 2 cases (1.3%), Deceased 5 cases (3.2%). Complications were: diabetes insipidus (89.3%); hypopituitarism (66.4%); hypothalamic damage (17.7%); visual deterioration (18.4%).
Surgery remains the main option, but GTR complications prove the necessity for a multidisciplinary approach. Outcome predicting factors are: age, tumor size, hydrocephalus degree, hypothalamic dysfunction.
颅咽管瘤（CPH）是一种良性肿瘤，在儿童中很少见，占所有颅内肿瘤的5%-6%。
本研究旨在分析一系列儿童颅咽管瘤病例的手术治疗及生活质量。
这是一项在布加勒斯特进行的为期25年（1994 - 2019年）的多中心研究。
152名0至17岁的儿童接受了颅咽管瘤治疗。术前表现为颅内高压、内分泌功能障碍、视力损害、共济失调、智力下降。
考虑到所有使用的手术入路，我们主张翼点入路最适合颅咽管瘤。我们实现了83例（54.4%）的全切除（GTR），13例（9%）的近全切除（NTR），51例（33.3%）的部分切除（PTR）。5例为活检（3.2%）。10例（6.5%）进行了伽玛刀手术，均复发。术后6个月格拉斯哥预后评分（GOS）显示：良好恢复70例（46.2%），中度残疾62例（40.7%），重度残疾13例（8.5%），植物状态2例（1.3%），死亡5例（3.2%）。并发症包括：尿崩症（89.3%）；垂体功能减退（66.4%）；下丘脑损伤（17.7%）；视力恶化（18.4%）。
手术仍然是主要选择，但全切除的并发症证明了多学科方法的必要性。预后预测因素包括：年龄、肿瘤大小、脑积水程度、下丘脑功能障碍。
