Shiohama Tadashi, Fujii Katsunori, Hayashi Masaharu, Hishiki Tomoro, Suyama Maiko, Mizuochi Hiromi, Uchikawa Hideki, Yoshida Shigetoshi, Yoshida Hideo, Kohno Yoichi
Department of Pediatrics, Chiba University Graduate School of Medicine, Chiba, Japan.
Brain Dev. 2013 Apr;35(4):363-6. doi: 10.1016/j.braindev.2012.06.002. Epub 2012 Jun 26.
Phrenic nerve palsy is a peripheral nerve disorder caused by excessive cervical extension due to birth trauma or cardiac surgery. We describe two new patients with phrenic nerve palsy associated with birth trauma. Both patients exhibited profound dyspnea and general hypotonia immediately after birth. A chest roentgenogram and fluoroscopy revealed elevation of the diaphragm, leading to a diagnosis of phrenic nerve palsy associated with birth trauma. Since they had intermittently exhibited dyspnea and recurrent infection, we performed video-assisted thoracoscopic surgery (VATS) plication in both cases, at an early and a late stage, respectively. Both patients subsequently exhibited a dramatic improvement in dyspnea and recurrent respiratory infection. Interestingly, the late stage operated infant exhibited spontaneous recovery at 7 months with cessation of mechanical ventilation once. However, this recovery was transient and subsequently led to an increased ventilation volume demand, finally resulting in surgical treatment at 15 months. Histological examination of the diaphragm at this time showed grouped muscle atrophy caused by phrenic nerve degeneration. To our knowledge, this is the first pathologically proven report of grouped muscle atrophy of the diaphragm due to phrenic nerve degeneration, suggesting that partial impairment of phrenic nerves resulted in respiratory dysfunction with incomplete recovery. We conclude that recently developed VATS plication is a safe and effective treatment for infants with phrenic nerve palsy, and should be considered as a surgical treatment at an early period.
膈神经麻痹是一种由出生创伤或心脏手术导致颈椎过度伸展引起的周围神经疾病。我们描述了两名与出生创伤相关的膈神经麻痹新病例。两名患者出生后立即出现严重呼吸困难和全身肌张力减退。胸部X线片和荧光透视显示膈肌抬高,从而诊断为与出生创伤相关的膈神经麻痹。由于他们间歇性出现呼吸困难和反复感染,我们分别在早期和晚期对这两名患者进行了电视辅助胸腔镜手术(VATS)折叠术。两名患者随后呼吸困难和反复呼吸道感染均有显著改善。有趣的是,晚期接受手术的婴儿在7个月时出现自发恢复,曾一度停止机械通气。然而,这种恢复是短暂的,随后导致通气量需求增加,最终在15个月时进行了手术治疗。此时膈肌的组织学检查显示由膈神经变性引起的成组肌肉萎缩。据我们所知,这是第一份经病理证实的关于膈神经变性导致膈肌成组肌肉萎缩的报告,表明膈神经部分受损导致呼吸功能障碍且恢复不完全。我们得出结论,最近开发的VATS折叠术是治疗膈神经麻痹婴儿的一种安全有效的方法,应在早期考虑作为手术治疗方法。