UAE: Newborn with rare, life-threatening condition saved in complex surgery

Doctors at a hospital in Abu Dhabi performed a complex surgery to save a baby born with a rare and life-threatening congenital condition: tracheoesophageal fistula (TEF) with oesophageal atresia (EA).

The joy of becoming parents for the first time was short-lived for German couple Tim and Sarah, employees at a UAE-based airline. While there were no concerning signs in regular scans of pregnancy, Kai, the newborn struggled to swallow his saliva. It dribbled from his mouth, and his breathing was consistently faster than normal.

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The neonatal team at Danat Al Emarat Hospital for Women and Children quickly intervened, inserting a small tube into Kai’s mouth to clear the oral cavity. In a healthy newborn, this tube would pass unimpeded through the oesophagus into the stomach, but in Kai’s case, it curled up high in his chest, indicating a blockage.

TEF with EA occurs in about one in 4,000 live births. In this condition, the upper part of the oesophagus does not connect to the lower part, and an abnormal connection (fistula) exists between the lower oesophagus and the trachea (windpipe).

This meant that every time Kai breathed, air could enter his stomach, and gastric acid could potentially reflux into his lungs, posing significant health risks. Without treatment, this condition is life-threatening.

Dr Brian MacCormack, a consultant paediatric and neonatal surgeon, explained the condition to the shocked parents, and the urgent need for a thoracotomy (where the chest is opened) in the first few days of Kai’s life. Although the condition was not immediately life-threatening, surgery was necessary within 24 to 48 hours to prevent severe complications later on.

Dr MacCormack reassured Kai’s parents that he would be stable for 12 to 24 hours with a tube in place, which prevented feeding but kept his condition manageable. However, delaying the surgery would significantly increase the risks involved.

Muscle-sparing technique

The next day, the surgical team prepared for the complex procedure. The goal was to connect the two ends of Kai’s oesophagus. The gap was moderate, about the length of three vertebrae. The surgery involved a right-sided thoracotomy, opening the chest between ribs four and five.

Dr MacCormack used a muscle-sparing technique to avoid cutting through major muscles, which would aid in Kai’s long-term recovery.

“This is one of the most technically demanding procedures that we perform as neonatal surgeons. The surgery began with the crucial step of disconnecting the esophagus from the airway, a life-saving manoeuvre. Once the airway was secured, I successfully connected the two ends of the eosophagus using a tube that passed through the nose, down the joint, and into the stomach. This tube facilitated feeding and maintained the connection during the healing process.”