What is Oesophageal Atresia?
Oesophageal Atresia (OA) is a congenital defect where there is a discontinuity of the oesophagus. The oesophagus normally joins the back of the throat to the stomach in the abdomen and in OA, somewhere in the upper chest where the oesophagus runs through, there is a disruption of this tube.
There are different defect types, most commonly, the upper end is blind ending and the lower end of the esophagus is connected to the trachea (main airway) to form a Tracheo-oesophageal Fistula (TOF).
1 in every 3500 newborns in the world suffers from oesophageal atresia
The cause of oesophageal atresia is still unclear but it is known to happen during the separation of a single tube (the primitive foregut) to form the trachea and airway system in front and the oesophagus at the back.
There are different classifications of oesophageal atresia. For example, the “pure” oesophageal atresia (type A) is one where the oesophagus is blind ending on both sides, this type may be detected during fetal development on the antenatal scans at 20 weeks. Findings on the scans are: excessive amniotic fluid because the fetus is unable to swallow amniotic fluid due to the obstruction of the esophagus and the second is an absent stomach “bubble”(the stomach has shrunken with no amniotic fluid to stretch it).
Symptoms of Oesophageal Artresia
Choking when feeding
For most babies with OA, they would not have an antenatal diagnosis (as mostly type C, here amniotic fluid can be swallowed, flow into the trachea and then into the stomach via the TOF). Babies are often born at full term and probably look normal in appearance. On occasions, the baby might be seen to drool saliva or bubbling froth from the mouth. When the baby is fed, they will cough and splutter the milk. This is because there is no passage for the milk to flow into and hence it“overflows” into the airway and causing the baby to choke on the milk.
This usually happens within the first day of life and the doctor looking after the baby would request for a tube to be placed via the nose into the stomach (nasogastric tube). The tube will coil at the blind end upper oesophagus and the diagnosis is made and confirmed with a chest X-ray with the tube in place. Once confirmed, the paediatrician will refer the baby to a paediatric surgeon.
Breathing problems
Rarely, some babies with TOF may have difficulties breathing, as the stomach will also be filled with the inhaled air from each breath via the “fistula”. Gradually a full stomach can push on the diaphragm, making it harder to breathe. Premature infants with OA, who require respiratory support (ventilator) have a significantly higher risks of this complication.
They may require urgent surgery if that was the case, to disconnect the fistula between the airway and the lower oesophagus. If the baby remains stable after the disconnection, during the operation and if the two ends of the oesophagus are reasonably close together, then the oesophagus can be joined together.
Treatment of Oesophageal Artresia
Minimally Invasive Thoracic Surgery (MITS)
The purpose of surgery is to establish an oesophagus which is a continuous tube so the baby can start to feed orally. Any fistulas are divided and the airway end is repaired. The two free ends of the oesophagus are then stitched together circumferentially.
Open surgery on the chest, otherwise called a thoracotomy, usually requires a large incision on the side of the chest. Some muscles will be cut on the way in and the ribs on the side stretched apart for the surgeon to get access to the area of interest. This will affect the chest wall and muscle function, leaving a long scar after the operation.
Thoracoscopic (keyhole or minimally invasive surgery) procedure requires only 3 almost invisible incisions without cutting any muscles. By this method, the baby will experience significantly less pain and suffering, they heal quickly and there will also be no chest wall deformity or horrible large scars in the future.
This procedure is possibly one of the most challenging for a paediatric surgeon and an experienced skilled paediatric laparoscopic surgeon should be doing it by this technique.
Surgery to connect the oesophagus (long gap)
If the gap between the upper and lower esophagus is long, the oesophageal may not be able to be pulled together and joined (anastomosis). An anastomosis which is tight risks the join to leak when fed or it may narrow down making it hard for the child to swallow. This is more common with the Pure OA (type A). In such cases, attempt to join will be deferred to later. Instead, a feeding tube is placed into the stomach directly through the abdomen. Feeding into the stomach this way will help stimulate the lower esophagus to grow. There will also be a tube in the upper oesophagus to stimulate that part to grow too and to suck out any saliva that might pool there. Once the distance between the upper and lower esophagus is reasonable, then the operation can be performed to connect them. Some infants can undergo esophageal connection surgery in 3 to 6 months. In some cases, the baby may have wait nearly 1 year for the surgery.
In these type of reconstructive surgeries, it is vital that the surgery is done right in the first instance, otherwise the child might have to undergo many corrective surgeries in their lifetime.
Patient Story
“She kept vomiting with gagging noise from the day she was born. She was then sent to the Neonatal Intensive Care Unit (NICU). There, they found the devastating reason for this!” described the first-time parents, Mr & Mrs Lim on their 2.4kg child’s condition before the procedure.
She was diagnosed with Oesophageal Atresia which is a condition where a child is born with a discontinuity to the oesophagus. For the first time in PHKL, the pioneering keyhole or thoracoscopic surgery was conducted a month ago on the 2-day old child who is dearly known to us as ‘Baby Mulan Ahbi’.
Normally the procedure to treat this condition is done with a large cut to the side of the baby’s chest, but with keyhole surgery, 3 small cuts measuring 3mm each was made on Baby Mulan. Her recovery therefore was quick and almost pain-free.
Baby Mulan Ahbi received care and treatment from our multidisciplinary team from the day she showed the symptoms right until she was discharged from NICU.”
FAQ regarding Oesophageal Artresia Surgery (MITS)
Q1: What are the common complications of this procedure?
- A: Complications are uncommon and they are: Esophageal leaks, narrow esophagus, abnormal peristalsis and gastro-oesophageal reflux. If there was a leak, another surgery to repair the esophagus is likely. If there is a problem with gastro-oesophageal reflux, laparoscopic fundoplication may be required. The long term risks of narrowing may require the narrowed area, usually at the joint to be stretched via endoscopy.
Q2: How long does it take for the surgery to be completed?
- A: It takes roughly about 3 hours because of the small space available for the surgeons to perform the surgery on the baby.
Q3: How long does the patient need to be hospitalized?
- A: Usually the patient can be discharged at about day 4 or 5 post-surgery by the keyhole method and almost double that, with the open method.
Originally published in Easily Sin Chew on 31-07-2018 in Mandarin.
