Durell Jonathan, Dagash Haitham, Eradi Bala, Rajimwale Ashok, Nour Shawqui, Patwardhan Nitin
Department of Paediatric Surgery, Leicester Royal Infirmary, Leicester, United Kingdom LE1 5WW.
Department of Paediatric Surgery, Leicester Royal Infirmary, Leicester, United Kingdom LE1 5WW.
J Pediatr Surg. 2017 Aug;52(8):1252-1254. doi: 10.1016/j.jpedsurg.2017.04.019. Epub 2017 May 2.
The presence of 13 pairs of ribs on pre-operative chest x-ray has been postulated to be an indicator for long gap esophageal atresia (EA). This study sought to determine the validity of this theory and identify associated pathological conditions in patients with EA and abnormal rib count.
Babies with EA from January 2005 - December 2012 were retrospectively analyzed. Information was gathered from neonatal health records and operation notes. Chest x-rays were reviewed to determine rib count. Long gap EA was defined as failure to achieve primary esophageal anastomosis. Statistical analysis performed with Fisher's exact test.
Seventy-six patients were identified. Eight patients had long gap EA, with none of these patients having 13 pairs of ribs. Paradoxically, 10 patients with esophageal atresia +/- trachea-esophageal atresia (EA +/- TEF) and supernumerary ribs underwent primary repair. Nine patients had 11 pairs of ribs, of which 2 had pure EA and a long gap. Using Fisher's exact test to compare the groups of supernumary ribs and non-supernumary ribs there is a p value of 0.587. VACTERL association was identified in 40% of those with supernumerary ribs. Various associated syndromes and concomitant abnormalities were identified.
We found no association between 13 pairs of ribs and long gap in esophageal atresia. Those with 13 pairs of ribs were more likely to have associated anomalies, although this was not statistically significant. Our cohort of patients was found to have a range of pathology related to genetic syndromes, further atresias, and malformations, which is well known to be associated with children born with EA +/- TEF.
Prognosis study - level IV.
术前胸部X光片显示有13对肋骨被认为是长段食管闭锁（EA）的一个指标。本研究旨在确定该理论的有效性，并识别食管闭锁且肋骨计数异常患者的相关病理状况。
对2005年1月至2012年12月期间患有食管闭锁的婴儿进行回顾性分析。从新生儿健康记录和手术记录中收集信息。复查胸部X光片以确定肋骨数量。长段食管闭锁定义为未能实现一期食管吻合。采用Fisher精确检验进行统计分析。
共确定76例患者。8例为长段食管闭锁患者，这些患者中无一例有13对肋骨。矛盾的是，10例患有食管闭锁±气管食管瘘（EA±TEF）且有多余肋骨的患者接受了一期修复。9例患者有11对肋骨，其中2例为单纯食管闭锁且为长段。使用Fisher精确检验比较有多余肋骨组和无多余肋骨组，p值为0.587。在40%有多余肋骨的患者中发现了VACTERL综合征。还识别出了各种相关综合征和伴随异常。
我们发现13对肋骨与食管闭锁中的长段之间无关联。有13对肋骨的患者更可能有相关异常，尽管这在统计学上不显著。我们的患者队列被发现有一系列与遗传综合征、进一步的闭锁和畸形相关的病理状况，这些状况众所周知与患有EA±TEF的患儿有关。
预后研究 - 四级。
