马凡综合征青少年气胸的最佳治疗方法。

Optimal treatment of pneumothorax in adolescents with Marfan syndrome.

机构信息

Department of Pediatric Surgery, The University of Tokyo Hospital, 7-3-1 Hongo, Bunkyo-ku, Tokyo 113-8655, Japan.

出版信息

J Pediatr Surg. 2021 Jul;56(7):1103-1106. doi: 10.1016/j.jpedsurg.2021.03.021. Epub 2021 Mar 27.

DOI:10.1016/j.jpedsurg.2021.03.021

PMID:33896616

Abstract

PURPOSE

Pneumothorax often develops in patients with Marfan syndrome (MFS). Here, we examined the effects of conservative and surgical pneumothorax treatments in children with MFS.

METHODS

In this study, 23 patients, less than 20 years old, diagnosed with both MFS and pneumothorax between 1999 and 2019 were included. All data were collected retrospectively from patients' medical records.

RESULTS

In total, 18 of 23 patients (78%) had relapsed pneumothorax either on the ipsilateral or contralateral side. Among these 18 patients, 6 (26%) patients had multiple relapses. Conservative and surgical treatments of pneumothorax were attempted in 33 and 29 lungs, respectively. The conservative treatment was attempted as a definitive therapy in 21 lungs. Twelve conservative treatments (57%) failed, which required surgical intervention. In 9 lungs (43%) with successful conservative treatment, 6 (67%) had ipsilateral relapses. In contrast to the above findings, only 4 (13%) ipsilateral relapses were observed in 29 surgical treatments.

CONCLUSIONS

Our study revealed a low response and high relapse rate when MFS adolescents who diagnosed pneumothorax were subjected to the conservative treatment modality. Thus, we recommend surgical intervention as the first line of therapy to treat pneumothorax in adolescents diagnosed with MFS.

LEVEL OF EVIDENCE

Ⅲ (Treatment Study).

摘要

目的

马凡综合征（MFS）患者常并发气胸。本研究旨在探讨 MFS 合并气胸患儿的保守和手术治疗效果。

方法

本研究纳入了 1999 年至 2019 年间诊断为 MFS 合并气胸且年龄小于 20 岁的 23 例患者。所有数据均来自患者病历的回顾性收集。

结果

23 例患者中，共有 18 例（78%）同侧或对侧气胸复发，其中 6 例（26%）患者多次复发。分别对 33 侧和 29 侧肺部尝试了保守和手术治疗。21 侧肺部尝试了保守治疗作为确定性治疗。12 例保守治疗（57%）失败，需要手术干预。在 9 例（67%）成功的保守治疗中，有 6 例（67%）同侧复发。相比之下，在 29 例手术治疗中，仅观察到 4 例（13%）同侧复发。