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双侧先天性膈膨升:手术治疗成功

Bilateral congenital eventration of the diaphragms: successful surgical management.

作者信息

Rodgers B M, Hawks P

出版信息

J Pediatr Surg. 1986 Oct;21(10):858-64. doi: 10.1016/s0022-3468(86)80008-2.

Abstract

Bilateral congenital eventration of the diaphragms is a relatively rare occurrence, associated with an almost uniform mortality. We recently have had experience with three patients who underwent successful treatment of this anomaly. Each patient presented as a newborn with severe respiratory insufficiency. One had had a sibling who died shortly after birth with a congenital diaphragmatic hernia. One infant had associated respiratory distress syndrome. PA and lateral chest roentgenograms demonstrated the anomaly in each patient. In two, the use of ultrasound clearly demonstrated the presence of attenuated diaphragms and paradoxical motion. Each infant underwent transabdominal bilateral diaphragmatic plication, reinforced with transversus abdominis muscle, within the first 2 weeks of life. Two infants showed prompt improvement in arterial blood gases and were extubated following the operation. The infant with respiratory distress syndrome required a tracheostomy for continued mechanical ventilation. This infant developed a recurrent eventration of the left diaphragm 6 months after the initial operation and has undergone a secondary plication with clinical improvement. Unlike unilateral eventration, bilateral eventration of the diaphragms is associated with persistent and significant respiratory symptoms. Surgical plication should be performed urgently in all of these patients, before chronic pulmonary changes occur.

摘要

双侧先天性膈膨升较为罕见,几乎均伴有死亡。我们最近有3例该异常患者成功接受治疗的经验。每位患者均以新生儿重度呼吸功能不全就诊。其中1例患者有1名同胞在出生后不久死于先天性膈疝。1例婴儿合并呼吸窘迫综合征。胸部正位和侧位X线片显示了每位患者的异常情况。其中2例通过超声检查明确显示膈肌变薄和矛盾运动。每位婴儿均在出生后2周内接受经腹双侧膈肌折叠术,并使用腹横肌加强。2例婴儿术后动脉血气迅速改善并拔除气管插管。合并呼吸窘迫综合征的婴儿因需持续机械通气而进行了气管造口术。该婴儿在初次手术后6个月出现左侧膈肌复发性膨升,并再次接受折叠术,临床症状改善。与单侧膈膨升不同,双侧膈膨升伴有持续且明显的呼吸道症状。对于所有这些患者,应在慢性肺部改变出现之前紧急进行手术折叠术。

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