Rombouts J J, Debauche C, Verellen G, Lyon G
Service de Chirurgie orthopédique et de Traumatologie de l'appareil locomoteur, Cliniques Universitaires Saint-Luc, Bruxelles, Belgique.
Ann Chir Main Memb Super. 1993;12(1):39-44. doi: 10.1016/s0753-9053(05)80259-5.
Four cases of congenital dysfunction of major peripheral nerves are reported and discussed. The first patient was a girl born by spontaneous delivery with an area of skin and muscle necrosis on the dorsal aspect of the forearm and a palsy of the three major nerves of the upper limb. The second patient was a boy born by caesarean section with the same necrotic lesions and a palsy of the ulnar and median nerve. The third patient was a boy born uneventfully with a truncal lesion of the sciatic nerve at the thigh. The fourth who was delivered by caesarean section for alteration of the monitoring presented a severe strangulation of the arm by the umbilical cord with palsy of the radial and ulnar nerve. The palsies recovered spontaneously and the sequelae are mostly due to the muscle necrosis. Congenital dysfunction of major peripheral nerves have been described as a complication of congenital constriction band syndrome, in association with subcutaneous fat necrosis, neonatal gangrene or aplasia cutis congenita. Intrauterine compression of a limb may be favoured by decreased foetal activity, by spontaneous rupture of the amniotic sac, particularly if there is a delay in delivery and by abnormal uterine activity during labour. Our three first cases confirm that the compression may arise before the labour. The fourth demonstrates that umbilical cord strangulation may lead to anoxia of the foetus together with compression of the limb.
报告并讨论了4例主要周围神经先天性功能障碍的病例。首例患者为一名自然分娩的女孩,其前臂背侧有皮肤和肌肉坏死区域,上肢三大神经麻痹。第二例患者为一名剖宫产出生的男孩,有相同的坏死病变,尺神经和正中神经麻痹。第三例患者为一名顺产男婴,其坐骨神经在大腿处有躯干病变。第四例因监护情况改变而行剖宫产,出现脐带严重勒住手臂,桡神经和尺神经麻痹。麻痹症状自发恢复,后遗症主要归因于肌肉坏死。主要周围神经先天性功能障碍已被描述为先天性束带综合征的一种并发症,与皮下脂肪坏死、新生儿坏疽或先天性皮肤发育不全有关。宫内肢体受压可能因胎儿活动减少、羊膜囊自发破裂(尤其是分娩延迟时)以及分娩期间子宫活动异常而加重。我们的前三例病例证实,压迫可能在分娩前就已出现。第四例表明,脐带勒住可导致胎儿缺氧并伴有肢体受压。