Short summary

Long-gap esophageal atresia (LGEA) is a congenital disorder, where the child is born with a gap between the esophagus and the ventricle. It is a rare but dangerous condition with many long-term complications. The primary treatment is to surgically bring the two ends together, but in some cases the distance is too long for this to be possible. In these circumstances there are different surgical approaches, but there is currently no golden standard for management of these children. This study seeks to investigate the preferred surgical approach in the Nordic countries alongside with the post-operative complications to attempt to find a golden standard for management of LGEA patients. 

Rationale

Esophageal atresia is a malformation of the esophagus caused by an incorrectly separation of the foregut into trachea and esophagus in the fifth or sixth week of the embryonic life. The anomaly results in an upper and a lower esophageal pouch with or without a trachea-esophageal fistula. Esophageal atresia occurs in every 1/2500 to 1/4000 birth and is primarily classified by the Gross classification. The most common type is a proximal esophageal atresia combined with a distal TEF, also known as the Gross type C. Gross type C is present in approximately 88,5% of cases.

Esophageal atresia is often associated with other congenital abnormalities, often in an association known as the vertebral, anal, cardia, tracheoesophageal, renal and limb association (VACTERL).

Through improvements in prenatal diagnosis, surgery, paediatric anaesthesia, neonatal intensive care and parental nutrition, the survival rate of esophageal atresia patients has increased to approximately 95%. The biggest surgical challenge is still management of long-gap esophageal atresia (LGEA) present in approximately 15% of esophageal atresia patients. The definition of LGEA is inconsistent throughout the literature. In this study, the definition of LGEA will be where the gap between the two pouches was too long to perform a primary end-to-end anastomosis. There are different ways of surgical management to treat LGEA, but the techniques all attempt to create esophageal-gastric continuity. The primary techniques used are performing a delayed primary anastomosis or making an interposition using the colon, the jejunum or the ventricle.
Research in surgical management of LGEA is currently sparse, and as there has been considerable development in pediatric surgery, it is relevant to investigate whether one approach is better than the other.

The aim of this study is to register Danish, Norwegian, Swedish, Finnish and Icelandic patients born with esophageal atresia Gross type C and D. The data will be used to create an overview on how many of the patients where primary anastomosis was not possible, and further used to investigate which surgical approach for LGEA Gross type C and D pediatric surgeons in the Nordic countries then prefer. The data will also be used to investigate postoperative complications such as strictures, leaks and deaths seen within the first year. We will also include parameters such as total length of hospital stay, stay at intensive care units and duration of parenteral nutrition to evaluate the quality of life for the patients.

The study will provide further insight into management of children born with LGEA Gross type C and D and the data can henceforth be used in comparison between operation type and postoperative complications and at best, create a golden standard for treating these children.

Description of the cohort

The cohort will consist of children born with esophageal atresia Gross type C and D in the period from 1^st of January 2000 to 1^st of May 2017. The study will focus specifically on children born with long-gap esophageal atresia where primary anastomosis was attempted but not succeeded or where a delayed anastomosis was planned from the beginning.

Data and biological material

Data will consist of informations including type of congenital esophageal atresia, operation procedures, birth weight and length, post-operative complications, stay at intensive care unit, data on 1-year follow up etc. found in the patients' medical records. 

Collaborating researchers and departments

Surgical Department A, Odense University Hospital, Denmark

• Professor Niels Qvist, MD DMSc

Department of Paediatric Surgery, Surgical clinic C, Rigshospitalet, Copenhagen, Denmark

• Professor and chair head Jørgen Thorup, MD PhD

Department of Gastric and Pediatric Surgery, Oslo University Hospital, Oslo, Norway

• Audun Mikkelsen
• Professor Ragnhild Emblem 

Department of Pediatric Surgery, St. Olavs Hospital, Trondheim, Norway

• Associate Professor, Thorstein Sæter 

Department of Pediatric Surgery, Karolinska University Hospital and Department of Women's and Children's Health, Karolinska Institute, Stockholm, Sweden

• Anna Maria Tollne
• Markus Almström
• Jan Svensson 

Department of Pediatric Surgery, Uppsala University Hospital and Department of Women's and Children's Health, Uppsala University, Sweden

• Helene Lilja
• Felipe Donoso, MD

Department of Pediatric Surgery, Skane University Hospital Lund, Sweden

• Associate Professor Pernilla Stenström, MD
• Einar Arnbjörnsson

Department of Pediatric Surgery, Children's Hospital, University of Helsinki, Helsinki, Finland

• Antti Koivusalo

Publications associated with the project

The project is a continuation of the project:
“Surgical repair of long-gap esophageal atresia: A retrospective study comparing the management of long-gap esophageal atresia in the Nordic countries” by Stadil, T.