Jönsson Linus, Friberg Lars Göran, Gatzinsky Vladimir, Kötz Karsten, Sillén Ulla, Abrahamsson Kate
Department of Pediatric Surgery, The Queen Silvia Children's Hospital, Gothenburg, Sweden.
Department of Pediatrics, The Queen Silvia Children's Hospital, Gothenburg, Sweden.
Eur J Pediatr Surg. 2016 Apr;26(2):150-9. doi: 10.1055/s-0034-1396415. Epub 2015 Jan 5.
This retrospective study aims to report treatment results in patients with long-gap esophageal atresia (LGEA), gross A + B type, and discuss the value of different clinical findings and physiological tests in the follow-up.
This retrospective observational study comprises all patients with LGEA admitted to our department between 1995 and 2010.
A total of 16 patients were included. Their mean gestational age was 35(+2) weeks and their mean birth weight was 1,945 g (-2.5 standard deviation scores). No catch-up growth in height could be seen and they remained smaller than the average population during the study period. Gastrostomy was performed as the first surgical procedure. Overall, 11 of the 16 patients had a delayed primary anastomosis. Elongation of the distal esophageal segment was required in 3 of the 16 patients and a colonic interposition in 2 of the 16 patients. The median age at definitive surgery was 150 days. All the patients had gastroesophageal reflux after their definitive surgery. Three of the 16 patients required surgery due to aspiration and all 3 had a pathological lung clearance index (LCI) at multiple-breath washout (MBW). At the age of 1 or 7 years, the LCI was pathological in 4 of the 14 patients, and spirometry showed an obstruction in 9 of the 14 patients.
LGEA is a severe congenital malformation, with severe morbidity. No mortality was seen. MBW could be a useful tool for the early detection of progressive pulmonary damage.
本回顾性研究旨在报告长间隙食管闭锁（LGEA）、大体A + B型患者的治疗结果，并探讨不同临床发现和生理检查在随访中的价值。
本回顾性观察性研究纳入了1995年至2010年间在我科住院的所有LGEA患者。
共纳入16例患者。他们的平均孕周为35（+2）周，平均出生体重为1945 g（-2.5标准差评分）。在研究期间，未观察到身高的追赶生长，他们仍低于平均人群。首次手术为胃造瘘术。总体而言，16例患者中有11例进行了延迟一期吻合。16例患者中有3例需要延长远端食管段，16例患者中有2例需要结肠间置术。确定性手术的中位年龄为150天。所有患者在确定性手术后均有胃食管反流。16例患者中有3例因误吸需要手术，且这3例患者在多次呼吸冲洗（MBW）时均有病理肺清除指数（LCI）。在1岁或7岁时，14例患者中有4例LCI呈病理状态，14例患者中有9例肺功能测定显示有阻塞。
LGEA是一种严重的先天性畸形，发病率高。未观察到死亡病例。MBW可能是早期发现进行性肺损伤的有用工具。
