Pokorny W J, McGill C W, Harberg F J
J Pediatr Surg. 1984 Aug;19(4):394-7. doi: 10.1016/s0022-3468(84)80260-2.
Morgagni hernias during infancy are associated with significant respiratory symptoms as well as other congenital anomalies, particularly congenital heart disease. During the past 6 years we have had the opportunity to treat five infants less than 1 year of age with Morgagni hernias. A review of the literature revealed an additional seventeen detailed case reports of infants with Morgagni hernias. Each of our patients was symptomatic as were all but two of those previously reported. These patients presented with tachypnea, dyspnea, and cyanosis which was very similar to the presentation of Bochdalek hernias seen during infancy. Thirteen of 16 patients in whom detailed descriptions of associated anomalies were given including four of our own patients, had significant congenital anomalies. Nine involved the heart, including three infants with dextracardia, three with ventricular septal defects, and two with anomalous pulmonary venous return. Five infants were retarded, including three with Trisomy 21. Two of the previously reported patients had large omphaloceles and other stigmata of Cantrell's syndrome. All but one of the patients reported, including ours, had a hernia sac. The liver, colon, and small bowel were most commonly found in the hernia sac; however, the presence of the stomach and spleen have been reported. While both the transabdominal and transthoracic approaches to Morgagni hernias have been advocated and, indeed, repair of the defect can be accomplished through both approaches, the transthoracic approach allows better exposure of incarcerated viscera, particularly the frequently found anomalous left lobe of the liver.
婴儿期的莫尔加尼疝与严重的呼吸道症状以及其他先天性异常有关,尤其是先天性心脏病。在过去6年里,我们有机会治疗了5例年龄小于1岁的莫尔加尼疝婴儿。文献回顾显示,另有17篇关于莫尔加尼疝婴儿的详细病例报告。我们的每一位患者都有症状,之前报告的患者中除2例外也都有症状。这些患者表现为呼吸急促、呼吸困难和发绀,这与婴儿期所见的博赫dalek疝的表现非常相似。在给出相关异常详细描述的16例患者中,有13例包括我们自己的4例患者有明显的先天性异常。9例涉及心脏,包括3例右位心婴儿、3例室间隔缺损婴儿和2例肺静脉异位引流婴儿。5例婴儿智力发育迟缓,包括3例21三体综合征患儿。之前报告的2例患者有巨大脐膨出和坎特雷尔综合征的其他体征。包括我们的患者在内,报告的患者中除1例外都有疝囊。疝囊中最常见的是肝脏、结肠和小肠;然而,也有胃和脾脏存在的报告。虽然经腹和经胸两种方法都被提倡用于治疗莫尔加尼疝,而且实际上通过这两种方法都可以完成缺损修复,但经胸方法能更好地暴露嵌顿的内脏,特别是经常发现的异常左肝叶。