Mizzi Colin, Farrugia David, Choudhry Muhammad S
Department of Paediatric Surgery, Mater Dei Hospital, Msida, Malta.
Department of Urology, Mater Dei Hospital, Msida, Malta.
European J Pediatr Surg Rep. 2020 Jan;8(1):e74-e76. doi: 10.1055/s-0039-3402741. Epub 2020 Oct 21.
Congenital diaphragmatic herniae (CDH) with associated intrathoracic ectopic kidneys are rare congenital anomalies, with a reported incidence of only 0.25%. The authors report a case of a 24-day-old baby girl who was diagnosed with a left-sided CDH on a chest X-ray taken for pneumonia. Computed tomography scan showed CDH hernia, containing small and large bowel and whole left kidney with adrenal gland. Thoracoscopic reduction in the bowel, kidney, and adrenal gland into the abdomen and primary closure of the defect was achieved with no complications. During investigation of the child, it was discovered that her maternal aunt had also had a left-sided congenital diaphragmatic hernia containing the kidney, which was treated via open surgery after birth; she subsequently developed renal cell carcinoma and required radical nephrectomy of that kidney during her third decade.
先天性膈疝(CDH)合并胸腔内异位肾是罕见的先天性异常,报告发病率仅为0.25%。作者报告了一例24天大的女婴病例,该女婴因肺炎进行胸部X线检查时被诊断为左侧CDH。计算机断层扫描显示CDH疝,疝内容物包括小肠、大肠以及整个左肾和肾上腺。通过胸腔镜将肠管、肾脏和肾上腺回纳至腹腔并对缺损进行一期缝合,未出现并发症。在对该患儿的调查过程中发现,其母亲的姐姐也曾患有左侧先天性膈疝并伴有肾脏,出生后通过开放手术治疗;她随后患上了肾细胞癌,并在30岁时接受了该肾的根治性肾切除术。