Optimal treatment of pneumothorax in adolescents with Marfan syndrome
Tomohiro Sunouchi1, Yasuo Watanabe1, Kotaro Tomonaga1
1Department of Pediatric Surgery, The University of Tokyo Hospital, 7-3-1 Hongo, Bunkyo-ku, Tokyo 113-8655, Japan.
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).
More Related Videos
06:59Catheter-based Endovascular Angioplasty for Fibrosing Mediastinitis-associated Pulmonary Vein Stenosis
Published on: August 26, 2025
04:19Minimally Invasive Treatment for Thoracolumbar Burst Fracture Using Sagittal Alignment Screws and A Trauma Reduction Device
Published on: November 8, 2024
Related Concept Videos
Flail Chest-II
Assessment:
1. Clinical Evaluation:
History:
Pneumothorax-II
Clinical Manifestations:
Pneumothorax-I
Pneumothorax can be even further classified as spontaneous, traumatic, and tension pneumothorax.
Cardiopulmonary Resuscitation II: ACLS Airway Management
Mitral Stenosis III: Medical Management
Cystic Fibrosis: Management
Sinus disease and chronic...
