Surgery chapters
Oesophageal atresia, tracheo-oesophageal fistula and duodenal atresia
Two classic causes of upper gut obstruction in the newborn: oesophageal atresia with or without tracheo-oesophageal fistula, and duodenal atresia with its double bubble and link to Down syndrome.
- The commonest oesophageal atresia (about 85%) is a blind upper pouch with a distal tracheo-oesophageal fistula: a newborn with frothy saliva, choking and cyanosis on feeding, an NG tube that coils in the upper pouch, and gas in the stomach.
- Look for VACTERL anomalies (especially cardiac); keep the pouch empty with a Replogle tube, nurse head up, then repair via right thoracotomy (divide fistula, join the ends).
- Duodenal atresia gives bilious vomiting from day 1, a double bubble with no distal gas, polyhydramnios, and is linked to Down syndrome; treat with duodenoduodenostomy.
On this page
Overview
Oesophageal atresia (OA) and duodenal atresia are congenital blockages of the upper gut that present within the first day of life. Both cause polyhydramnios before birth, because the fetus cannot swallow and absorb amniotic fluid normally.
Both need early diagnosis, protection of the airway and fluids, then planned surgery. The exam focus is on types, the first clinical clue, the X-ray signs and associations.
Oesophageal atresia and tracheo-oesophageal fistula
Embryology
The trachea and oesophagus develop from the foregut. A ridge (the tracheo-oesophageal septum) normally separates the ventral respiratory diverticulum (lung bud) from the dorsal oesophagus at about weeks 4–5. Incomplete separation leaves a blind oesophagus, an abnormal fistula to the trachea, or both.
Incidence is about 1 in 3500 births.
Types (Gross classification, with approximate frequency)
| Type | Anatomy | Approx. frequency |
|---|---|---|
| C (commonest) | Blind upper pouch + distal TOF (lower oesophagus joins the trachea) | About 85% |
| A | Pure OA, no fistula (long gap) | About 8% |
| E | H-type fistula, no atresia | About 4% |
| B | OA with proximal fistula (upper pouch to trachea) | About 2% |
| D | OA with both proximal and distal fistulas | Under 1% |
(Percentages from Basis of Paediatrics; other books give similar figures.)
"C is Common": blind upper pouch, lower fistula.
Clinical features
- Polyhydramnios antenatally; small or absent stomach bubble on fetal scan.
- Soon after birth: excess frothy saliva / drooling ("bubbly baby"), because saliva cannot be swallowed.
- Choking, coughing and cyanosis with the first feed; milk comes back out of the mouth.
- Aspiration pneumonia: from saliva spilling from the upper pouch, and gastric acid refluxing up the distal fistula into the lungs.
- Abdominal distension (air enters the stomach through the distal fistula with each breath, especially when crying or ventilated).
- H-type fistula: no atresia, so the baby can swallow. Presents later with coughing or cyanosis during feeds, recurrent chest infections and abdominal distension.
A newborn with frothy saliva must have a firm NG tube passed before the first feed. Feeding a baby with OA causes aspiration pneumonia.
Diagnosis
- Pass a firm (10 Fr) nasogastric/orogastric tube: it meets resistance a short way down and coils in the upper pouch.
- Chest and abdominal X-ray with the tube in place:
- tube coiled in the upper pouch (upper mediastinum);
- gas in the stomach and bowel = there is a distal fistula;
- gasless abdomen = pure OA (or OA with only a proximal fistula).
- A contrast study is rarely needed for OA. If used, only a tiny amount of water-soluble contrast under expert care, because aspiration of contrast is dangerous.
- H-type fistula: prone tube oesophagogram and/or bronchoscopy.
A gasless abdomen in OA means no distal fistula (pure atresia). This usually means a long gap between the two ends, which cannot be joined at once.
Associated anomalies: VACTERL
Associated anomalies are common, especially cardiac, renal and skeletal. The VACTERL association:
| Letter | Anomaly |
|---|---|
| V | Vertebral (hemivertebrae) |
| A | Anorectal malformation |
| C | Cardiac (VSD, ASD, tetralogy, right aortic arch) |
| TE | Tracheo-oesophageal fistula with oesophageal atresia |
| R | Renal anomalies |
| L | Limb (radial ray defects, absent radius or thumb) |
Also check for chromosomal disorders (trisomy 18, 21) and duodenal atresia. Echocardiography before surgery is essential: it shows heart defects and the side of the aortic arch (which decides the side of the thoracotomy).
The commonest type is a blind upper pouch with a distal fistula (about 85%), so most babies with OA have gas in the stomach on X-ray.
Preoperative management
- Nil by mouth.
- Replogle tube (double-lumen) in the upper pouch on continuous low suction to stop saliva being aspirated.
- Nurse head up (to reduce reflux of gastric juice through the fistula).
- IV fluids with glucose; antibiotics if aspiration.
- Avoid bag-and-mask ventilation and high-pressure ventilation if possible, because air goes down the fistula and distends the stomach, which may perforate and splints the diaphragm.
Surgery
- Usually within a day or two of birth, once the baby is stable.
- Approach: right posterolateral extrapleural thoracotomy (opposite side if right-sided aortic arch); thoracoscopic repair is possible in expert centres.
- Divide and close the fistula on the tracheal side, then make an end-to-end oesophageal anastomosis.
- Long-gap OA (pure OA): gastrostomy for feeding and a delayed primary repair after the pouches grow (often months), or oesophageal replacement (stomach, colon or jejunum). A cervical oesophagostomy is sometimes used.
Complications after repair
| Early | Late |
|---|---|
| Anastomotic leak | Anastomotic stricture (commonest; treat by dilatation) |
| Recurrent fistula | Gastro-oesophageal reflux (very common; can worsen stricture; may need fundoplication) |
| Pneumonia | Tracheomalacia (barking "TOF cough", episodes of collapse) |
| Dysmotility and food bolus impaction; long-term risk of Barrett change |
Prognosis is good for most babies; it is worse with very low birth weight and major heart disease.
Duodenal atresia
Cause and anatomy
- Failure of the duodenum to recanalise after the normal solid stage of early gut development.
- Forms: a membrane / web (commonest; may be a windsock web that balloons distally), a fibrous cord joining two blind ends, or a complete gap.
- Most obstructions lie distal to the ampulla of Vater, so the vomit is bile-stained.
- Duodenal obstruction can also be extrinsic: annular pancreas, Ladd bands with malrotation, preduodenal portal vein.
About a quarter to a third of babies with duodenal atresia have Down syndrome, and a baby with Down syndrome and bilious vomiting on day 1 has duodenal atresia until proven otherwise.
Associations
| Association | Note |
|---|---|
| Down syndrome (trisomy 21) | About a quarter to a third of babies with duodenal atresia (Basis of Paediatrics: 20–30%) |
| Prematurity, polyhydramnios | Polyhydramnios in about half |
| Malrotation, annular pancreas | Check at surgery |
| Cardiac, oesophageal atresia, anorectal and renal anomalies | Screen before surgery |
Clinical features
- Antenatal ultrasound: double bubble with polyhydramnios.
- Bilious vomiting from the first day of life (non-bilious if the block is above the ampulla).
- No abdominal distension (high obstruction), sometimes upper abdominal fullness and visible gastric waves.
- Meconium may be passed initially.
- Jaundice may occur.
Investigations
- Plain abdominal X-ray: double bubble (gas in the stomach and the dilated first part of the duodenum) and no gas beyond.
- If there is distal gas, think of a partial obstruction (web with a hole, stenosis) or malrotation with volvulus. Here an urgent upper GI contrast is needed, because volvulus is an emergency.
- Screening: echocardiogram, renal ultrasound, spine and limb X-rays, karyotype.
A double bubble with distal gas is not safe to treat as duodenal atresia: exclude malrotation with volvulus urgently.
Management
- NG/OG tube decompression, IV fluids and electrolytes; correct dehydration and alkalosis or other imbalances.
- Assess and stabilise associated anomalies (especially cardiac).
- Surgery is not an emergency once malrotation has been excluded.
- Duodenoduodenostomy (side-to-side or "diamond" anastomosis) bypassing the block. Annular pancreas is bypassed, never divided (dividing it causes a pancreatic fistula).
- A web may be excised, taking care not to injure the ampulla.
- Prognosis mainly depends on the associated anomalies.
"Double bubble, Down's trouble."
Jejunal and ileal atresia (for comparison)
- Caused by an antenatal vascular accident (sterile infarction of a segment), not failed recanalisation.
- Single or multiple; types range from a membrane to an "apple-peel" (Christmas-tree) atresia with a large mesenteric gap.
- Present with bilious vomiting and abdominal distension (more distension the lower the block); multiple fluid levels on X-ray.
- Treated by resection of the dilated end (or tapering) and anastomosis.
| Feature | Duodenal atresia | Jejunoileal atresia |
|---|---|---|
| Cause | Failed recanalisation | Vascular accident in utero |
| Down syndrome | Strong link | No particular link |
| Distension | Little | Marked (lower = more) |
| X-ray | Double bubble | Several dilated loops, fluid levels |
Expect: the commonest type of OA/TOF and its frequency, first clinical clue (frothy saliva, choking with first feed), the bedside NG test and coiled tube on X-ray, gas versus gasless abdomen, H-type fistula presentation, VACTERL components, preoperative care (Replogle suction, head up, avoid bag-mask ventilation), the thoracotomy approach and late complications (stricture, reflux, tracheomalacia); duodenal atresia with double bubble, polyhydramnios, Down syndrome, bilious vomiting, annular pancreas and duodenoduodenostomy, and the need to exclude malrotation.
Quick revision
- OA/TOF: commonest = blind upper pouch + distal fistula (about 85%).
- Frothy saliva, choking on first feed, polyhydramnios.
- NG tube coils in upper pouch; stomach gas = distal fistula; gasless = pure OA.
- VACTERL; echo before surgery.
- Replogle tube, head up, nil by mouth; right thoracotomy; divide fistula, join ends.
- Late: stricture, reflux, tracheomalacia.
- Duodenal atresia: failed recanalisation; double bubble; bilious vomit day 1; Down syndrome.
- Duodenoduodenostomy; bypass (never divide) annular pancreas.
Written by The House Job from standard references (Bailey & Love; Basis of Paediatrics).
FAQ
What is the first sign of oesophageal atresia in a newborn?
Excess frothy saliva and drooling, followed by choking, coughing and cyanosis when the first feed is given.
How is oesophageal atresia confirmed?
A firm nasogastric tube cannot be passed into the stomach; an X-ray shows it coiled in the upper pouch, and gas in the bowel shows a distal fistula.
Why is the vomit bile-stained in most cases of duodenal atresia?
Most atresias lie below the ampulla of Vater, so bile enters the stomach side of the block and is vomited.
What does distal bowel gas with a double bubble suggest?
An incomplete obstruction, or malrotation with volvulus, which must be excluded urgently by an upper GI contrast study.