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儿童非神经母细胞瘤性病变的肾上腺切除术

Adrenalectomy for non-neuroblastic pathology in children.

作者信息

Traynor Michael D, Sada Alaa, Thompson Geoffrey B, Moir Christopher R, Bancos Irina, Farley David R, Dy Benzon M, Lyden Melanie L, Habermann Elizabeth B, McKenzie Travis J

机构信息

Department of Surgery, Mayo Clinic, 200 First Street SW, Rochester, MN, 55905, USA.

Division of Pediatric Surgery, Mayo Clinic, Rochester, MN, USA.

出版信息

Pediatr Surg Int. 2020 Feb;36(2):129-135. doi: 10.1007/s00383-019-04589-9. Epub 2019 Nov 5.

Abstract

BACKGROUND

Adrenalectomy for non-neuroblastic pathologies in children is rare with limited data on outcomes. We reviewed our experience of adrenalectomy in this unique population.

METHODS

Retrospective study of children (age ≤ 18) who underwent adrenalectomy with non-neuroblastic pathology from 1988 to 2018. Clinical and operative details of patients were abstracted. Outcomes included length of stay and 30-day postoperative morbidity.

RESULTS

Forty children underwent 50 adrenalectomies (12 right-sided, 18 left-sided, 10 bilateral). Six patients (15%) presented with an incidental adrenal mass while 4 (10%) had masses found on screening for genetic mutations or prior malignancy. The remaining 30 (75%) presented with symptoms of hormonal excess. Nineteen patients (48%) underwent genetic evaluation and 15 (38%) had genetic predispositions. Diagnoses included 9 patients (23%) with pheochromocytoma, 8 (20%) with adrenocortical adenoma, 8 (20%) with adrenocortical carcinoma, 7 (18%) with adrenal hyperplasia, 2 (5%) with metastasis, and 6 (14%) with additional benign pathologies. Of 50 adrenalectomies, twenty-five (50%) were laparoscopic. Median hospital length of stay was 3 days (range 0-11). Post-operative morbidity rate was 17% with the most severe complication being Clavien-Dindo grade II.

CONCLUSION

Adrenalectomy for non-neuroblastic pathology can be done with low morbidity. Its frequent association with genetic mutations and syndromes requires surgeons to have knowledge of appropriate pre-operative testing and post-operative surveillance.

摘要

背景

儿童非神经母细胞瘤性病变的肾上腺切除术很少见,关于其预后的数据有限。我们回顾了我们在这一特殊人群中进行肾上腺切除术的经验。

方法

对1988年至2018年接受非神经母细胞瘤性病变肾上腺切除术的儿童(年龄≤18岁)进行回顾性研究。提取患者的临床和手术细节。预后指标包括住院时间和术后30天发病率。

结果

40名儿童接受了50次肾上腺切除术(右侧12例,左侧18例,双侧10例)。6例患者(15%)为偶然发现肾上腺肿块,4例(10%)在筛查基因突变或既往恶性肿瘤时发现肿块。其余30例(75%)表现为激素过多症状。19例患者(48%)接受了基因评估,15例(38%)有遗传易感性。诊断包括9例(23%)嗜铬细胞瘤、8例(20%)肾上腺皮质腺瘤、8例(20%)肾上腺皮质癌、7例(18%)肾上腺增生、2例(5%)转移瘤和6例(14%)其他良性病变。50次肾上腺切除术中,25次(50%)为腹腔镜手术。中位住院时间为3天(范围0 - 11天)。术后发病率为17%,最严重的并发症为Clavien-Dindo II级。

结论

非神经母细胞瘤性病变的肾上腺切除术可在低发病率下完成。其与基因突变和综合征的频繁关联要求外科医生了解适当的术前检查和术后监测。

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