Bethell George S, Fouad Dina, Ogundipe Enitan, Choudhry Mohammed
Department of Paediatric Surgery, Chelsea and Westminster Hospital NHS Foundation Trust, London, UK
University Surgery Unit, University of Southampton, Southampton, UK.
BMJ Case Rep. 2023 Jan 5;16(1):e253394. doi: 10.1136/bcr-2022-253394.
Congenital diaphragmatic hernia (CDH) is encountered in just under 1 in 6000 live births, while congenital duodenal obstruction is seen once every 8000 live births. These congenital anomalies have only been reported together as part of the VACTERL syndrome and therefore in isolation represent an incredibly rare occurrence. This is a case report of a girl born at 34 weeks gestation who had an antenatal diagnosis of left CDH. Five days following operative repair of this, there was extensive pneumoperitoneum and pneumothorax. Upper gastrointestinal contrast study showed a perforation of the duodenum and at laparotomy, a duodenal web was found in the fourth part of the duodenum with perforation immediately proximal. Following duodenoduodenostomy, the postoperative recovery was good. She achieved full enteral feeds and was discharged home. This case highlights the importance of considering rare associations if postoperative recovery is not as expected to prevent delay in undergoing definitive treatment.
先天性膈疝(CDH)在每6000例活产中不到1例,而先天性十二指肠梗阻在每8000例活产中出现1次。这些先天性异常仅作为VACTERL综合征的一部分被报道过,因此单独出现极为罕见。本文报告了1例孕34周出生的女孩,产前诊断为左侧CDH。手术修复后5天,出现广泛的气腹和气胸。上消化道造影显示十二指肠穿孔,剖腹探查时,在十二指肠第四部发现十二指肠蹼,穿孔位于蹼近端紧邻处。十二指肠十二指肠吻合术后,术后恢复良好。她实现了完全经口喂养并出院回家。该病例强调,如果术后恢复未达预期,考虑罕见关联以防止延迟进行确定性治疗的重要性。