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Lifetime Management for Patients with Oesophageal Malformations

OESOPHAGEAL atresia (OA) is a birth defect that causes the oesophagus to end in a pouch rather than connecting to the stomach. This defect can be life-threatening, making it difficult for babies to swallow safely. Another similar defect, tracheoesophageal fistula, also results in an atypical connection between the oesophagus and trachea. To treat these oesophageal malformations, babies typically undergo surgery immediately after birth.

The latest information shared at a session of the virtual meeting of the North American Society for Pediatric Gastroenterology, Hepatology & Nutrition (NASPGHAN) suggests that management of patients with oesophageal conditions does not end in childhood, and that care should continue into adulthood.

Diana Lerner, Associate Professor of Pediatric Gastroenterology, Aerodigestive Center at the Children’s Hospital of Wisconsin, Milwaukee, USA, highlighted that the management of OA is multifactorial, and these patients usually have other health risks such as inflammatory conditions, gastrointestinal issues, and respiratory problems. Therefore, patients should be closely monitored from childhood through to adulthood, and receive multidisciplinary care from a team of medical experts including gastroenterologists and respiratory specialists.

Inflammation of the oesophagus in patients with OA is common, and occurs in 17–28% of patients. Scientists have suggested that the susceptibility to inflammation of the oesophagus could be due to genetics and other factors such as neonatal admission.

Other complications include anastomotic stricture. Lerner explained why there have not been any significant improvements due to this complication: “Symptoms can be vague, and all diagnostic studies have flaws. A child may be asymptomatic while drinking a totally liquid diet even with significant stenosis, and a radiologist may read some findings of anastomotic narrowing as ‘expected.’ I urge you to get good at reading your oesophagram and interpreting your own studies”

To end the session, Lerner discussed diagnostic and management tips that could help physicians get an early diagnosis. She enclosed how a solid swallow oesophagram might provide more information, and how oesophageal dilations have a high success rate of ~70% for treating strictures. Dysmotility of the oesophagus remains an issue even after OA has been surgically treated. However, the European Society for Paediatric Gastroenterology Hepatology and Nutrition (ESPGHAN) and NASPGHAN have an algorithm that can help manage and evaluate reflux.

Comorbidities associated with oesophageal malformations that can arise even after treatment in childhood emphasise the importance of continuous surveillance, multidisciplinary management, and awareness of these complications.