Sesia Sergio B, Haecker Frank-Martin
Pediatric Surgery Department, University Children's Hospital Basel, Basel, Switzerland.
BMJ Case Rep. 2012 Apr 23;2012:bcr0820114638. doi: 10.1136/bcr.08.2011.4638.
Intrathoracic renal ectopia associated with a congenital diaphragmatic hernia represents a very rare congenital malformation. The incidence of intrathoracic renal ectopia with congenital diaphragmatic hernia is reported to be less than 0.25%. The authors report on a 5-month-old female infant with late-presenting congenital diaphragmatic hernia associated with a left-sided intrathoracic ectopic kidney and adrenal gland. While prenatal ultrasound showed no pathology, the postnatal ultrasound assumed a left-sided renal agenesia. After recurrent episodes of tachypnoea, a chest x-ray confirmed the diagnosis of congenital diaphragmatic hernia which was repaired without complications. Intraoperatively, intrathoracic ectopic kidney was diagnosed. The authors discuss embryogenesis, the diagnostic algorithm and therapy as well as follow-up of this rare malformation.
胸腔内肾异位合并先天性膈疝是一种非常罕见的先天性畸形。据报道，胸腔内肾异位合并先天性膈疝的发病率低于0.25%。作者报告了一名5个月大的女婴，患有迟发性先天性膈疝，伴有左侧胸腔内异位肾和肾上腺。产前超声检查未发现病变，但产后超声检查推测为左侧肾缺如。在反复出现呼吸急促后，胸部X线检查确诊为先天性膈疝，并顺利进行了修复手术，无并发症。术中诊断出胸腔内异位肾。作者讨论了这种罕见畸形的胚胎发生、诊断方法、治疗以及随访情况。
