婴儿和儿童胸骨后疝

Congenital Morgagni＇s hernia in infants and children

目的探讨罕见的婴儿和儿童胸骨后疝的诊断和治疗。方法收集了36年间（1978年3月至2014年6月）上海交通大学医学院附属新华医院小儿外科收治的8例胸骨后疝患儿的资料，复习了患儿明确诊断时年龄、性别、临床症状、伴发畸形、手术方式及转归。其中男3例，女5例，年龄2d至8岁，7例胸骨后疝位于右侧，左侧1例。伴发其他畸形4例，2例为先天性心脏病、脐膨出和肠旋转不良，异位肾和右腹股沟疝各I例。结果8例均经手术证实，疝内容物为横结肠、小肠、肝左叶、胃、大网膜。早先4例采用传统经腹开放入路，后4例选择腹腔镜下修补，其中1例同时作右腹股沟疝修补。8例中4例有疝囊。行疝囊切除1例，除1例2d新生儿伴脐膨出和肠旋转不良术后夭折外，余7例随访复查胸片未见复发。结论胸骨后疝为罕见的先天性膈疝的一种类型，临床表现各异，伴发其他畸形的比率较高，以先天性心脏病最为常见。推荐经腹入路手术，腹腔镜下修补为首选。相比开放手术其手术创伤小，恢复快，早期能进食，缩短了住院时间且外观更美观。

Objective To explore the diagnosis and treatment of congenital Morgagni＇s hernia （CMH） in infants and children. Methods Retrospective analyses were performed for 8 cases of CMH at our department during 36 years from March 1978 to June 2014. Age at diagnosis, gender, clinical symptoms, associated anomalies, surgical procedures and prognosis were examined. During a 36-year period, 8 eases of CMH were treated at our hospital. There were 3 males and 5 females. Their age at time o{ diagnosis ranged from 2 days to 8 years. Among them, 7 had right-sided Morgagni＇s hernia and I left-sided hernia. Associated anomalies were found in 4 patients. There were congenital heart disease （n = 2）, omphalocele with intestinal malrotation （n = 1） and ectopic kidney ＆ right inguinal hernia （n = 1 ）. Results All diagnoses of CMH were surgically confirmed. The hernial contents included transverse colon, small intestine, stomach, left hepatic lobe and omentum. All cases were operated via a transabdominal approach, Four early cases underwent open surgery while another 4 underwent laparoscopic-assisted operation. And 1 patient underwent simultaneous repair of right inguinal hernia. Among 4 patients with hernial sac, 1 underwent excision. One patient with omphalocele and intestinal malrotation died postoperatively while the remainder recovered well postoperatively without recurrence during follow-ups. Conclusions As a rare type of congenital diaphragmatic hernia, CMH has diverse clinical manifestations with a high incidence of associated common anomalies, including congenital heart disease. A transabdominal approach is recommended for repairing. And laparoscopic-assisted operation should be a first choice. Comparing with an open approach, this less invasive procedure promotes faster postoperative recovery, allows earlier commencement of feeds, contributes to a shorter hospital stay and yields better cosmetic outcomes.