Systematic review of long term follow-up and transitional care in adolescents and adults with esophageal atresia - why is transitional care mandatory?

Abstract

Purpose: to review recent literature concerning long-term health issues and transitional care in esophageal atresia (EA) patients. PubMed, Scopus, Embase and Web of Science databases were screened for studies regarding EA patients aged more than or equal to 11 years, published between August 2014 and June 2022. Sixteen studies involving 830 patients were analyzed. Mean age was 27.4 years (range 11–63). EA subtype distribution was: type C (48.8%), A (9.5%), D (1.9%), E (0.5%) and B (0.2%). 55% underwent primary repair, 34.3% delayed repair, 10.5% esophageal substitution. Mean follow-up was 27.2 years (range 11–63). Long-term sequelae were: gastro-esophageal reflux (41.4%), dysphagia (27.6%), esophagitis (12.4%), Barrett esophagus (8.1%), anastomotic stricture (4.8%); persistent cough (8.7%), recurrent infections (4.3%) and chronic respiratory diseases (5.5%). Musculo-skeletal deformities were present in 36 out of 74 reported cases. Reduced weight and height were detected in 13.3% and 6% cases, respectively. Impaired quality of life was reported in 9% of patients; 9.6% had diagnosis or raised risk of mental disorders. 10.3% of adult patients had no care provider. Meta-analysis was conducted on 816 patients. Estimated prevalences are: GERD 42.4%, dysphagia 57.8%, Barrett esophagus 12.4%, respiratory diseases 33.3%, neurological sequelae 11.7%, underweight 19.6%. Heterogeneity was substantial (> 50%). Conclusion: EA patients must continue follow-up beyond childhood, with a defined transitional-care path by a highly specialized multidisciplinary team due to the multiple long-term sequelae. What is Known:• Survival rates of esophageal atresia patients is now more than 90% thanks to the improvements in surgical techniques and intensive care, therefore patients’ needs throughout adolescence and adulthood must be taken into account. What is New:• This review, by summarizing recent literature concerning long term sequelae of esophageal atresia, may contribute to raise awareness on the importance of defining standardized protocols of transitional and adulthood care for esophageal atresia patients.

Major surgical conditions of childhood and their lifelong implications: comprehensive review

“…Gastric metaplasia is also notably more common. To date, there have been only a few reports of oesophageal cancer developing in patients with OA/TOF; however, the number of survivors into late adulthood is ever-increasing and this association will become clearer over the next few decades 14 , 15 . International paediatric gastroenterology guidelines recommend surveillance endoscopy with biopsies taken in four quadrants at the gastro-oesophageal junction (GOJ) and anastomosis every 5 to 10 years in adults with OA/TOF, having a low threshold for endoscopic assessment in the presence of new symptoms, and following adult consensus guidelines on metaplasia where present 16–20 .…”

“…Musculoskeletal problems after surgery for OA/TOF are common, such as scoliosis, chest wall deformity, winging of the scapula and shoulder dysfunction, rib fusion and breast disfigurement. Thoracotomy alone is responsible for many of these, and may be one major advantage of adopting minimally invasive approaches 15 , 29 .…”

Time to consider oesophageal atresia as a life-long disease

“…Others more frequent complications have also been described as GERD, which prevalence in adulthood has been estimated at 42.4% (95% CI: 33.2–52.1) in a recent systematic review from Brooks et al . 5 based on 16 studies including 830 patients. The prevalence of ‘respiratory sequelae’ have also been estimated at 33.3% (95% CI: 10.1–69), based on the same review of six studies including 115 patients 5 .…”

“… 5 based on 16 studies including 830 patients. The prevalence of ‘respiratory sequelae’ have also been estimated at 33.3% (95% CI: 10.1–69), based on the same review of six studies including 115 patients 5 . Undernutrition has also been a high prevalence in adult [estimated prevalence of 19.6% (95% CI: 11.6–31.1)] 5 .…”

“…The prevalence of ‘respiratory sequelae’ have also been estimated at 33.3% (95% CI: 10.1–69), based on the same review of six studies including 115 patients 5 . Undernutrition has also been a high prevalence in adult [estimated prevalence of 19.6% (95% CI: 11.6–31.1)] 5 . In 2008, our team implemented the first population-based registry of live births of infants with EA in the world.…”