Berthet Stéphanie, Tenisch Estelle, Miron Marie Claude, Alami Nassiba, Timmons Jennifer, Aspirot Ann, Faure Christophe
Division of Pediatric Gastroenterology Hepatology and Nutrition, Sainte-Justine University Health Centre, Montreal, Québec, Canada.
Division of Pediatric Radiology, Sainte-Justine University Health Centre, Montreal, Québec, Canada.
J Pediatr. 2015 May;166(5):1140-1144.e2. doi: 10.1016/j.jpeds.2015.01.038. Epub 2015 Feb 24.
To report the incidence of congenital vascular anomalies in a cohort of patients with esophageal atresia (EA) and tracheoesophageal fistula (TEF) while describing the clinical presentation, diagnosis, and consequences, and to evaluate the diagnostic value of esophagram in diagnosing an aberrant right subclavian artery (ARSA).
All patients born with EA/TEF between 2005 and 2013 were studied. Preoperative echocardiography reports, surgical descriptions of primary esophageal repair, and esophagrams were reviewed retrospectively.
Of the 76 children born with EA/TEF included in this study, 14 (18%) had a vascular malformation. The incidence of a right aortic arch (RAA) was 6% (5 of 76), and that of an aberrant right subclavian artery (ARSA) was 12% (9 of 76). RAA was diagnosed in the neonatal period by echocardiography (4 of 5) or surgery (1 of 5), and ARSA was diagnosed by echocardiography (7 of 9) or later on the esophagram (2 of 9). Respiratory and/or digestive symptoms occurred in 9 of the 14 patients with vascular malformation. Both long-gap EA and severe cardiac malformations necessitating surgery were significantly associated with vascular anomalies (P<.05). The sensitivity of the esophagram for diagnosing ARSA was 66%, the specificity was 98%, the negative predictive value was 95%, and the positive predictive value was 85%.
ARSA and RAA have an incidence of 12% and 6% respectively, in patients with EA/TEF. A computed tomography angioscan is recommended to rule out such malformations when stenting of the esophagus is indicated, before esophageal replacement surgery, and when prolonged (>2 weeks) use of a nasogastric tube is considered.
报告一组食管闭锁（EA）和气管食管瘘（TEF）患者先天性血管异常的发生率，同时描述其临床表现、诊断及后果，并评估食管造影在诊断迷走右锁骨下动脉（ARSA）中的诊断价值。
对2005年至2013年间出生的所有EA/TEF患者进行研究。回顾性分析术前超声心动图报告、原发性食管修复手术描述及食管造影。
本研究纳入的76例EA/TEF患儿中，14例（18%）存在血管畸形。右位主动脉弓（RAA）的发生率为6%（76例中的5例），迷走右锁骨下动脉（ARSA）的发生率为12%（76例中的9例）。RAA在新生儿期通过超声心动图（5例中的4例）或手术（5例中的1例）诊断，ARSA通过超声心动图（9例中的7例）或稍后通过食管造影（9例中的2例）诊断。14例血管畸形患者中有9例出现呼吸和/或消化症状。长段间隙性EA和需要手术的严重心脏畸形均与血管异常显著相关（P<0.05）。食管造影诊断ARSA的敏感性为66%，特异性为98%，阴性预测值为95%，阳性预测值为85%。
在EA/TEF患者中，ARSA和RAA的发生率分别为12%和6%。在需要进行食管支架置入、食管替代手术之前以及考虑长期（>2周）使用鼻胃管时，建议进行计算机断层血管造影以排除此类畸形。
