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Eur J Pediatr Surg 2016; 26(02): 150-159
DOI: 10.1055/s-0034-1396415
Original Article
Georg Thieme Verlag KG Stuttgart · New York

Treatment and Follow-Up of Patients with Long-Gap Esophageal Atresia: 15 Years' of Experience from the Western Region of Sweden

Linus Jönsson
1   Department of Pediatric Surgery, The Queen Silvia Children's Hospital, Gothenburg, Sweden
,
Lars Göran Friberg
1   Department of Pediatric Surgery, The Queen Silvia Children's Hospital, Gothenburg, Sweden
,
Vladimir Gatzinsky
1   Department of Pediatric Surgery, The Queen Silvia Children's Hospital, Gothenburg, Sweden
,
Karsten Kötz
2   Department of Pediatrics, The Queen Silvia Children's Hospital, Gothenburg, Sweden
,
Ulla Sillén
1   Department of Pediatric Surgery, The Queen Silvia Children's Hospital, Gothenburg, Sweden
,
Kate Abrahamsson
1   Department of Pediatric Surgery, The Queen Silvia Children's Hospital, Gothenburg, Sweden
› Author Affiliations
Further Information

Publication History

10 July 2014

05 October 2014

Publication Date:
05 January 2015 (online)

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Abstract

Objective 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.

Methods This retrospective observational study comprises all patients with LGEA admitted to our department between 1995 and 2010.

Results 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.

Conclusion 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.

Keywords

long-gap esophageal atresia - follow-up study - pulmonary physiology
 

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