1 - Área de Pediatria Médica, Hospital Dona Estefânia, Centro Hospitalar Universitário de Lisboa Central, Lisboa
2 - Serviço de Cirurgia Pediátrica, Hospital Dona Estefânia, Centro Hospitalar Universitário de Lisboa Central, Lisboa
3 - Serviço 5.1, Hospital Dona Estefânia, Centro Hospitalar Universitário de Lisboa Central, Lisboa
4 - Unidade de Cuidados Intensivos Neonatais, Hospital Dona Estefânia, Centro Hospitalar Universitário de Lisboa Central, Lisboa
5 - Unidade de Gastrenterologia Pediátrica Hospital Dona Estefânia, Centro Hospitalar Universitário de Lisboa Central, Lisboa
- Reunião internacional (ESPGHAN 54th Meeting)
Objectives and study: Long gap esophageal atresia is a rare and complex disease, which challenges encompass non-consensual definitions, treatment and complications management. We aimed to characterize all children undergoing operative repair for long gap esophageal atresia over an 8-year period, to review surgical methods and long-term complications.
Methods: Descriptive retrospective analysis of epidemiological and clinical data of patients admitted to a level III hospital with diagnosis of long gap esophageal atresia between July 1st 2013 and July 1st 2021.
Results: Eleven cases of long gap esophageal atresia were included, with a male predominance (82%). Mean follow up duration was of 5,1 years. Pure esophageal atresia without fistulae (gross type A) was the most frequently found type (9/11), while atresia with a distal fistula (gross type C) accounted for a minority of cases (2/11). Nine newborns had a prenatal diagnosis (8/9 with type A atresia) and preterm birth occurred in 5/11. Six patients had other malformations, mostly VACTERL association (3/11) and those related to Down Syndrome (2/11). The first endoscopic measurement was performed at a mean age of 1 month (min.: 1 day, max.: 2 months), while the average distance between pouches was of 33 mm (min.: 20 mm, max.: 50 mm). In 55%, esophageal lengthening was attempted, either through Foker Procedure (5/11) or magnetic anastomosis (1/11). In most cases (9/11), end-to-end anastomosis was eventually attained, while gastric transposition was employed in two patients. The median age for surgical correction was of 7 months (min.: 1 month, max.: 19 months). Mean hospital admission length was 258 days. All patients suffered complications resulting from either atresia or its correction. Sepsis was the most common peri-operatively complication (4/11). In the long term, all patients developed gastroesophageal reflux disease. 9/11 (82%) had anastomosis strictures requiring endoscopic dilation. Six patients are still undergoing dilations one year after surgery. Seven patients (64%) developed respiratory disease, which was more prevalent in cases where esophageal lengthening was employed (p=0,006). Gastric transposition was associated with a higher prevalence of dumping syndrome (p=0,01).
Conclusions: Long gap esophageal atresia is an ill-defined disease, associated to long hospital stays and life-long complications. In our study, elongation techniques were employed in most cases. End-to-end anastomosis was attempted in all and successful in the vast majority of patients, although technical constrains led to a relatively late definitive correction and long hospital stays.
Palavras Chave: atrésia esofágica, método de foker