Dysphagia

Up to 90% of adults born with OA/TOF have occasional symptoms of dysphagia, and 25% have daily symptoms. (37) Cartabuke et al. (37) found this was predominantly to solids with 72% reporting this, but a significant minority also report dysphagia to liquids (44%).

Causes

1. Dysmotility. Manometry studies on adults with repaired OA/TOF show universally disordered peristalsis. Deurloo et (18) found 75% had low or moderate contraction and 35% had retrograde contraction. All had ineffective swallowing. In addition, 25% were found to have undiagnosed strictures. This follows on from another study in children where 66.7% had absent peristalsis and 33.3% weak peristalsis. (37) Poor motility is a result of poor nerve supply to the oesophagus below the repair site, and thus peristalsis is usually absent in the lower oesophagus below the repair. If a colon interposition graft was used (long-gap OA) to join the gap in the oesophagus, peristalsis is also impaired or absent in this segment. (36) There is growing anecdotal evidence that the oesophagus post OA repair deteriorates more rapidly than the normal oesophagus, and dysmotility can increase with age.
2. Strictures. A stricture is a narrowing in the oesophagus from scar tissue. The most common place for a stricture to occur is at the anastomosis (join) from the original surgery where the oesophagus was repaired. When tissues heal, they contract – and this can result in narrowing. Many children will have had dilatations of the oesophagus to improve their swallowing; residual narrowing may persist into adulthood. (38) The normal oesophagus in an adult is about 18mm to 20mm wide (similar in diameter to your thumb) and if this is reduced by even 3mm or 4mm it can lead to problems with swallowing. The inevitable dysmotility compounds the effect of a stricture, so even a very mild stricture can cause significant dysphagia. Severe strictures reduce the oesophagus to only 2mm or 3mm in diameter and this will restrict swallowing to liquids only. Strictures can also occur as a result of reflux, with damage to the oesophageal lining by acid. Acid reflux will cause inflammation of the wall of the oesophagus; if this is severe, it can heal by forming scar tissue. This scarring tends to contract and cause narrowing of the oesophagus in the same way as above. Sometimes the join from the original surgery can behave like a stricture even if it is not particularly narrowed because there is disproportion between the diameter of the tube above and below the anastomosis. The typical example of this is when the colon has been used to bridge the gap in long-gap atresia of the oesophagus. The colon is wider than the oesophagus and therefore there is always some disproportion. Over years the colon graft often dilates further and this disproportion becomes more pronounced. The difference in size between the colon and the oesophagus will often cause food to stick above the join even if the join itself is not narrowed. (36)
3. Diverticulum. Rarely, a diverticulum (pouch) can form in the oesophagus, usually at an area of weakness just above an area of high pressure. The most common place for a diverticulum is just above the site of the original anastomosis, but a diverticulum may also form above an area of persistent spasm in the lower oesophagus. This can cause dysphagia in two ways:
   a) As a result of food going into the diverticulum rather than down the oesophagus
   b) As a result of the stricture or area of spasm which led to the diverticulum in the first place.
4. Hiatus hernia. In sliding hiatus hernias, the diaphragm may press on the top of the stomach and this pressure can cause dysphagia. The presence of the hiatus hernia can also worsen any reflux, which may exacerbate the dysphagia (36); 28% of adults born with OA/TOF were found to have hiatus hernia on endoscopy. (5)

Symptoms

1. Sensation of food or fluid getting ‘stuck’
2. Choking – reported in 33% post repair whilst eating (8)
3. Regurgitation
4. Worsening heartburn/reflux symptoms

Investigations

These are suggested when symptoms change, food is repeatedly getting stuck for prolonged periods (or needing hospital visits), weight loss or recurrent aspiration occurs. The aim is to look for new or worsening strictures or malignant changes and assess degree of dysphagia and whether oral feeding remains safe.

• Gastroscopy
• Oesophageal manometry and 24-hour pH studies (see below)
• Barium swallow will not aid in diagnosis of dysphagia and will provide additional unnecessary radiation

Treatment

Whilst there is no way to cure underlying issues with peristalsis, there are some measures that can ameliorate symptoms:

• Feeding adaptations – dietician/speech and language therapist input may be helpful, though many adults born with OA/TOF have spent their lifetime modulating their food intake to help their dysphagia, such as avoiding meats and difficult textures, hyperalimentation (drinking excess water), use of carbonated beverages.
• Treatment of GORD and oesophagitis (see below).
• Prokinetics (metoclopramide/domperidone (both 10mg up to three times a day), macrolide antibiotics such as azithromycin 250mg, dosage varies between clinicians between three times a week to daily).
• Treatment of any structural obstruction, such as strictures, stenosis or diverticula. This needs the involvement of gastroenterology or GI surgeons for consideration of endoscopic oesophageal dilatation (most commonly), stenting (temporary to hold open resistant strictures) or, rarely, surgery is required for a resistant stricture – either an operation to open up the stricture permanently (oesophagoplasty) or to remove the stricture. If the stricture is related to reflux, it is essential to treat the underlying reflux properly, otherwise the stricture will recur rapidly. (36)
• In severe cases, rarely, tube feeding (gastrostomy, nasogastric (NG), percutaneous endoscopic gastrostomy (PEG)) may be needed. (39)