Oesophageal atresia and tracheo-oesophageal fistula

Although maternal polyhydramnios and a small/absent stomach bubble may indicate oesophageal atresia, the condition is rarely definitively diagnosed before delivery. Postnatal presentation includes respiratory symptoms, frothing/ dribbling of saliva, coughing/choking during feeds, and inability to pass a nasogastric tube (NGT) into the stomach.

Provide respiratory support as required - Initiate ABC of resuscitation. Clear oral secretions and nurse infant tilted head up at all times.  Avoid mask ventilation if at all possible – potential gastric or oesophageal pouch distension may lead to respiratory compromise and/ or aspiration through a fistula, or gastric rupture. If mask ventilation is required, apply minimal pressures for a short period, once the infant has stabilised administer supplementary oxygen to maintain acceptable oxygen saturations until more control over the tracheo-bronchial airway can be achieved.  If intubation becomes necessary, inform the surgical team and observe for excessive abdominal distension which may require urgent surgical intervention. 

Insert a Replogle tube/ large bore NGT prior to X-ray.  An initial X-ray should include the whole of the trunk, to determine the length of the oesophageal pouch, to determine if there is gas in the stomach (fistula), and to look for vertebral abnormalities.  Other intestinal atresias (N.B. duodenal atresia and imperforate anus) may lead to distension which will be highlighted on x-ray, and may mandate more urgent surgical intervention.

If the X-ray confirms the diagnosis of oesophageal atresia, follow the guidelines for ongoing care. The infant also needs be examined for other associated abnormalities (including congenital cardiac disease and anorectal malformation).  It is important that a departmental  ECHO is performed preoperatively to exclude major congenital cardiac anomalies and determine the position of the aortic arch (determines incision site).

Renal and Spinal Ultrasound Scans are preformed to exclude associated anomalies. The timing of these is less critical unless there are specific concerns e.g. antenatal anomalies or abnormal renal function.  

Definition:

A Replogle tube - a double-lumen radio-opaque tube - is used for continuous suction and irrigation of secretions collecting in the blind-ending oesophageal pouch of an infant with oesophageal atresia. Specific care requirements of the infant include respiratory vigilance, continual clearance of secretions to minimise the risk of aspiration, and safeguarding of the airway to maintain optimal respiratory function.

Pre-requisites for practitioners:

Registered nurse / midwife with paediatric and/or neonatal experience who is proficient in neonatal airway assessments and suctioning, who can manage the infant at high risk of aspiration (ascertained through staff competency assessments - N.B. unless signed off as competent, the nurse should be supervised).

Indications:

A Replogle tube is passed as soon as the diagnosis of trachea-oesophageal atresia is suspected / recognised. It is maintained within the pre-operative period to clear secretions continuously that would otherwise put the baby at risk of aspiration. Surgical intervention is normally undertaken when the infant is stable (usually within 48hours). 

The finding of a long-gap atresia may call for a ‘delayed primary anastomosis’ and Replogle nursing for 6-8 weeks or longer, to allow the oesophagus time to lengthen.  A formal gastrostomy will be fashioned in the first few days of life to enable enteral feeding. Variations to management plans are individually tailored to meet the specific needs of the infant, focussing on family involvement and maintaining the suck reflex for eventual oral feeding following repair.

PROCEDURE

1. Preparation
2. Insertion of Replogle tube.
   • The Replogle tube is passed orally in the majority of newborn infants
   • The preferred route for older and larger infants with long-gap OA is nasally
3. On-going care
4. Equipment
   • Replogle tube (Argyle size 10FR)
   • Suction tubing and catheters
   • Suction apparatus able to deliver low suction (5-8 Kpa/37-60 mmHg)
   • 1ml syringes
   • 9% sodium chloride ( 5ml ampoule for injection is suitable)
   • Appropriate fixators for replolge tube
   • Marker or tape for replolge tube
   • Monitoring and resuscitation equipment including 2^nd suction apparatus

Figure 1: Replogle tube (from GOSH guideline)

Preparation

Insertion of a replogle tube

On-going care

Ongoing care of long gap atresia

Other information:

Some units temporarily increase suction pressures to an upper limit of 7-10kPa/ 50-70mmHg when thick viscous secretions are present (Southern West Midlands Newborn Network & GOSH).  Note: this is only a temporary measure to remove thick secretions. Beware that high pressures can cause the Replogle tube to adhere to the wall of the oesophagus, becoming altogether ineffective in removing secretions.

The baby with a long gap atresia requires extended care and active family involvement.  Preoperative feeding/ swallowing/ oesophageal motility assessments are made and objectives set to reduce post-operative complications related to poor oral stimulation. Following educational assessments, preparation and support, the family will be guided through replogle tube care, and gastrostomy feeding. Gastrostomy guidelines are separately covered.

Specific pre-operative care:

It is important that an ECHO is performed preoperatively to exclude major congenital cardiac anomalies and determine the position of the aortic arch. A right sided aortic arch may indicate a change to the normal right sided thoracotomy approach. The infant also requires examination for other associated congenital abnormalities (including heart murmur, anorectal malformation). Elective intubation on the ward is positively contra-indicated. The infant is intubated only in theatre, immediately prior to surgery, to avoid possible gastric distension which if untreated may lead to cardio-respiratory compromise or gastric perforation.

Ensure continuous suction is maintained during transport, using portable low pressure suction. Know the lowest suction pressure setting on whatever suction unit is being used and aim to achieve pressure of 5 to 8KPa.  Intermittent suction is acceptable in situations where continuous suction is not available, suggest every 5 to 10 minutes. During neonatal transfers where the infant is secured within the incubator it may not be possible to elevate the infant’s head. In this situation if a replogle tube is in situ, position the infant prone or side-lying. On the rare occasion when no replogle tube is available, the baby should be placed prone. (ScotSTAR Guideline).

Specific post-operative care:

Post operatively, the baby may have a Trans-Anastomotic nasogastric Tube (TAT) in place. This acts as a stent for the oesophageal anastomosis (and can be used for delivering gastric feeds when appropriate). Ensure that this tube is securely fastened and identified with tape labelled as follows:  - DO NOT REMOVE”. The nurse/carer needs to be aware that if the tube is accidentally removed in the first week post-operatively, it must not be reinserted at the beside (as blind passage of the tube can disrupt the anastomosis). The surgeon should be notified immediately in cases of accidental TAT displacement. 

The baby will continue to be nursed head up to reduce the risk of gastric contents refluxing up to the anastomosis. Paralysis may be continued, especially with a tight repair, and ventilation continued for 4 to 7 days - with pain relief and sedation. The surgeon may also request that the infant be nursed supine with head flexed, to reduce tension on the suture line.  NCPAP should be avoided, hence it is best not to hastily extubate the infant.

Careful measurement of the ET tube and suction catheters is required (noted on nursing care chart) to avoid damage of the repaired fistula site (most likely below the ET tube). DO NOT SUCTION BEYOND THE END OF THE ET TUBE (use catheters with measured increments). Oropharyngeal suctioning must avoid the anastomotic suture line - the surgeon will clarify the length of the anastomosis from the mouth.

If the baby has an extra pleural chest drain, do not put on continuous suction (unless specifically instructed to do so) as even low pressures may cause undue stress/damage to the newly repaired oesophagus. Monitor and record drainage. Saliva, air or bubbles in the drain may indicate leak from the anastomosis or fistula site. The surgical team should be notified urgently if this occurs.

Place nasogastric tube on free drainage. Monitor gastric aspirates 2-4 hourly. The initiation of gastric feeds will be determined by the surgical team, with or without a prior contrast swallow that assesses the integrity of the anastomosed suture line.

Post oesophageal repair, babies often display signs of oesophageal dysmotility. Gastrooesophageal reflux is highly likely. Elevating the cot and utilising positioning techniques may help control reflux symptoms. SALT assessment/ input may be required if disordered oesophageal peristalsis or poor oral skills make oral feeding challenging.