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一名4岁儿童右肺发育不全:通过膈肌移位进行纵隔手术固定,患儿从呼吸窘迫综合征中完全康复。

Aplasia of the right lung in a 4-year-old child: surgical stabilization of the mediastinum by diaphragm translocation leading to complete recovery from respiratory distress syndrome.

作者信息

Krivchenya D U, Dubrovin A G, Krivchenya T D, Khursin V N, Lysak C V

机构信息

Department of Pediatric Surgery, The National Medical University, Kiev, Ukraine.

出版信息

J Pediatr Surg. 2000 Oct;35(10):1499-502. doi: 10.1053/jpsu.2000.16424.

Abstract

Lung aplasia is defined as unilateral absence of the lung with preservation of main bronchus remnant at the tracheal bifurcation. Patients usually die soon after birth and there is no specific therapy for this condition, as evidenced by the literature. The authors present a case of an infant that was asymptomatic with this malformation until 3 months of age, when the child had respiratory distress syndrome. Subsequently, lung aplasia was diagnosed. The authors performed an extrapleural dissection and cephalad translocation of diaphragm to reduce the mediastinal shift and heart rotation, to relieve a kink and compression of the trachea by the aortal arch and truncus arteriosus, as well as to relieve hyperinflation of lung parenchyma and provide recovery from respiratory distress syndrome. This new approach resulted in complete recovery from respiratory distress syndrome and full tolerance of physical exercise. The child underwent follow-up for 4 years. Diaphragmatic translocation may be useful in treatment of respiratory disorders associated with lung aplasia.

摘要

肺不发育被定义为一侧肺脏缺如,气管分叉处保留主支气管残端。文献表明,患者通常在出生后不久死亡,且针对这种情况没有特效疗法。作者报告了一例婴儿病例,该婴儿在3个月大之前一直无症状,之后出现呼吸窘迫综合征,随后被诊断为肺不发育。作者进行了胸膜外剥离和膈肌头侧移位,以减少纵隔移位和心脏旋转,缓解主动脉弓和动脉干对气管的扭结和压迫,以及缓解肺实质过度膨胀,并使呼吸窘迫综合征得以恢复。这种新方法使呼吸窘迫综合征完全恢复,患儿对体育锻炼完全耐受。该患儿接受了4年的随访。膈肌移位可能对治疗与肺不发育相关的呼吸障碍有用。

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