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Craniopharyngioma Clinical Status Scale: a standardized metric of preoperative function and posttreatment outcome

机译：颅咽管瘤临床状况量表：术前功能和治疗后结局的标准化指标

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Object Controversy persists concerning the optimal treatment of craniopharyngiomas in children, and no standard outcome metric exists for comparison across treatment modalities, nor is there one that adequately reflects the multisystem dysfunction that may arise. Methods The authors retrospectively analyzed the records of 86 consecutive children who underwent a uniform treatment paradigm of attempted radical resection performed by a single surgeon. Excluding 3 perioperative deaths and 3 patients with inadequate follow-up, 80 children (34 girls and 46 boys; mean age 9.56 years; mean follow-up 9.6 years) composed the study group (53 primary and 27 previously treated/recurrent tumors). Building on existing classification schemes proposed by De Vile for hypothalamic dysfunction and Wen for overall functional outcome, the authors devised a more nuanced classification system (Craniopharyngioma Clinical Status Scale [CCSS]) that assesses outcome across 5 axes, including neurological examination, visual status, pituitary function, hypothalamic dysfunction, and educational/occupational status at last follow-up (there is a 4-tiered grading scale in each domain, with increasing values reflecting greater dysfunction). Results There was a significant increase in pituitary dysfunction following treatment—consistent with the high rates of diabetes insipidus and hypopituitarism common to the surgical management of craniopharyngiomas—and less dramatic deterioration in hypothalamic function or cognitive domains. Significant improvement in vision was also demonstrated, with no significant overall change in neurological status. Preoperative CCSS scores predicted postoperative outcome better than clinical characteristics like patient age, sex, tumor size, and the location or presence of hydrocephalus. Conclusions Preoperative CCSS scores predicted outcome with higher accuracy than clinical or imaging characteristics. In lieu of randomized trials, the CCSS may provide a useful outcome assessment tool for comparison across treatment paradigms and surgical approaches. Long-term follow-up is critical to the analysis of outcomes of craniopharyngioma treatment, given the often-delayed sequelae of all therapies and the high recurrence rates of these tumors.
机译：关于儿童颅咽管瘤的最佳治疗方法仍存在争议，并且尚无标准结果指标可用于比较各种治疗方式，也没有一种方法能充分反映可能出现的多系统功能障碍。方法作者回顾性分析了86名连续儿童的记录，这些儿童均接受了由单名外科医生进行的尝试性根治性切除术的统一治疗范例。排除3例围手术期死亡和3例随访不足的患者，由80名儿童（34名女孩和46名男孩;平均年龄9.56岁;平均随访9.6岁）组成了研究组（53例原发性和27例先前治疗/复发的肿瘤）。在De Vile提出的下丘脑功能障碍和Wen提出的整体功能结局的现有分类方案的基础上，作者设计了一种更细微的分类系统（颅咽管瘤临床状况量表[CCSS]），该系统通过5个轴评估结局，包括神经系统检查，视觉状态，垂体功能，下丘脑功能障碍和上次随访时的教育/职业状况（每个领域都有4层的分级量表，数值增加反映更大的功能障碍）。结果治疗后垂体功能障碍明显增加，这与颅咽管瘤手术治疗中常见的尿崩症和垂体功能低下率高有关，而下丘脑功能或认知范围的急剧恶化则较少。还显示了视力的显着改善，神经系统状态没有明显的总体变化。术前CCSS评分比患者年龄，性别，肿瘤大小以及脑积水的位置或存在等临床特征更好地预测了术后结果。结论术前CCSS评分预测的结果比临床或影像学特征具有更高的准确性。代替随机试验，CCSS可能提供有用的结果评估工具，用于比较治疗范例和手术方法。鉴于所有疗法的后遗症经常发作且这些肿瘤的高复发率，长期随访对于分析颅咽管瘤的治疗结果至关重要。

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