Oesophageal atresia

Key points

Pathophysiology

Abnormal septation of the caudal foregut weeks 4-5

Types B-E Trachea forms as a diverticulum of the foregut - failure to separate fully from oesophagus

Pure OA (Type A) - oesophagus fails to recanalise during week 8

Gross classification incidence

A: (Pure oesophageal atresia, no fistula) 7%

B : (Proximal fistula) 2%

C: (Distal Fistula) 86%

D: (Proximal and Distal fistula) 1%

E: (No atresia, just fistula: H/N type) 4%

Antenatal features

Polyhydramnios

Small stomach

Upper pouch

Associations

50% of patients have additional abnormality

VACTERL Vertebral, Anorectal, Cardiac, Tracheo-Esophageal, Renal, Limb (radial)

VUR is most common renal, ASD/VSD most common cardiac

CHARGE Coloboma, Heart, choanal Atresia, Genital, ear abnormalities

Trisomy 21, 18

History and Examination

Drooling, choking, respiratory distress, aspiration

Radiology

H- type fistula - N shaped, going obliquely up from oesophagus to trachea. Usually found between C7 + T2

If no distal gas, 20% chance of proximal fistula

BAPS CASS 2017 -

Type A - Usually 5 vertebral body gap

Type B - Usually 2.5 vertebral body gap

Management of post operative complications

Anastomotic Stricture

Due to GORD, tension, leak, not all layer closure

Treat with balloon dilatations, steroid (triamcinolone) injections if not effective after 8-10 times

If failing dilatations, consider CT for cartilaginous element and vascular ring, then MDT to discuss topical mitomycin C, fundoplication

Anastomotic Leak

Small:

• Place chest drain

• IV antibiotics and antifungals

• Repeat contrast at later date

Large:

<48 hours - Thoracotomy and repair

>48 hours - Thoracotomy

• If ends healthy repair - can use pleural/pericardial/intercostal flap overlay to reinforce

• If not healthy - close ends, place chest drain, gastrostomy, replogle tube

May need oesophagostomy if not resolved

Tracheal leak (bubbling from chest drain)

Thoracotomy

Closure of trachea with non-absorbable sutures

Muscle flap interposition

Recurrent fistula

Options:

• Endoscopic sealants

• Place guidewire as per H type

  Thoracotomy 1 rib space above previous, transpleural

  Repair fistula and put pleura/other flap in between

Accidental TAT removal

Do not repass blindly

Options:

• Leave it out - conservative management

• Replacement under fluoroscopy guidance

• Replacement under GA under endoscopic vision

• Gastrostomy as last option

Standard scenario

X-ray suggestive of OA

Look at level NG coiled

Look for double bubble = duodenal atresia

If no distal gas, assume long gap

VACTERL features - hemivertebrae, extra rib, abnormal sacrum, cardiomegaly

Check antenatal scans for, polyhydramnios, small stomach, dilated upper pouch, cardiac defects

Examine child for VACTERL features

Place OG replogle yourself - should stop at 9-13cm from gums, then XR with light pressure on it - tip should be at T2-4

Consent and counsel parents for stricture, leak, failed anastomosis

Echo for right sided aortic arch and other cardiac defects

Procedure if ventilated + distal gas on XR + distended OR duodenal atresia OR premature

If severe distension (can be due to anorectal malformation) - manoeuvres include advancing ET tube or decompressing stomach with cannula (plug cannula after)

Emergency thoracotomy, transpleural - grasp fistula and stabilise

Transfix and divide fistula

If stable can proceed to anastomosis

If not - close end of fistula and close - do anastomosis when stable

If ventilated but NOT distended OR duodenal atresia OR premature - can wait for daytime hours

Standard procedure

Essential Operative Steps

Bronchoscopy:

1. To determine site of the lower fistula

2. To rule out the proximal fistula

3. To look for tracheobronchomalacia and laryngeal abnormalities

Ideally before intubation, 9.5Fr cystoscope and laryngoscope, extend neck, coordination between anaesthetist to suction, go down quickly with scope, take care not to injure vocal cords as will cause sublgottic stenosis, withdraw slowly to visualise

Risks - prolonged procedure, decompensating patient

Usually right sided thoracotomy or thoracoscopic approach

Left-sided considered in:

• Right-sided aortic arch (If right sided aortic arch, can anastomose laterally (on right of))

• Frozen right chest from repeated thoracotomies

  
  

Muscle sparing thoracotomy

Extra- or trans-pleural

Ligation of azygos vein is optional (always clamp before ligating)

Identify the lower pouch/fistula

Always clamp first before ligating

Stay suture in the anterior wall of fistula

Ligate and divide the fistula

Close the tracheal defect

Pass a feeding tube into the lower fistula into the stomach

Identify and free up upper pouch

Assess the Gap

General Rule of Thumb:

1 vertebral body = 1cm: Primary anastomosis

2 vertebral bodies = 2cm: Tight anastomosis

3 vertebral bodies = 3cm: Consider as long gap

Full thickness anastomosis of posterior wall making sure mucosa is included

Pass TAT

Full thickness anastomosis of anterior wall making sure mucosa is included

Use of chest drain and post-operative contrast swallow before feeding varies between centres (BAPS CASS 2013) Choose an approach and justify it

If tight anastomosis:

Post op paralysis for 48 hours

Keep neck in flexed position

Post op follow up of OA and how to schedule dilatations - early follow up

PPI for 18 months - no consensus on duration, and probably not effective in preventing stricture (BAPS CASS 2014)

Next dilatation if tight stricture, early follow up if not

Follow up

Check for known complications:

1.        Impaired motility

2.        Tracheobronchomalacia

3.        Gastro oesophageal reflux disease

4.        Barrett’s oesophagus in 43%

5.        Recurrent fistula

6.        Recurrent respiratory infections

7.        Nerve damage: Winged scapula

Some patients may need to drink water with meals

May need oesophgeal manometry if severe

Tracheobronchomalacia improves with age and growth

If necessary, Aortopexy can be done

Anticipate gastro-oesophageal reflux in all patients

Aggressive antireflux therapy at least till at least 18 months of age

Severe cases may need fundoplication

Unknown risk of oeosphageal cancer

Do endoscopy and biopsies as a teenager, then 5-10 years after

If BE found - 3 years, if high grade dysplasia - do ablative therapies

May need respiratory team follow up if tracheomalacia, recurrent respiratory tract infections

Examine for MSK complications - scoliosis, winged scapula

Special scenario - long gap atresia

Long gap known pre op

ERNICA long gap consensus

A. Bronchoscopy to assess for fistula and other airway abnormalities - check for blocked lower fistula

B. Upper midline laparotomy + Stamm gastrostomy (away from greater curvature to preserve vessels for later gastric pull up, and away from fundus in case of later fundoplication) and gap assessment using neonatal endoscope or Heger

Bolus gastrostomy feeds to expand stomach + Sham oral feeds

C. Options for definitive management:

1. Conservative management with gastric feeds and first gap assessment in 4-6 weeks, repeat monthly until gap <2 vertebral bodies or delayed anastomosis around 3 months

2. Stretching the upper pouch only and delayed anastomosis

3. Stretching the upper and lower pouch using internal or external sutures (Foker’s principle - see below) and delayed anastomosis

Consent and counsel parents for:

Failed anastomosis

Increased risk of stricture

Vocal cord palsy

Tracheomalacia

4. Oesophageal replacement:

• Gastric pull up

• Jejunal interposition

• Colonic interposition

2017 BAPS CASS - Type B typically have gaps of 2.5 vertebral bodies, Type A - longer gap

Both delayed primary anastomosis and oesophageal replacement occurred at a median of 3 months

APSA long gap review - these procedures usually occurred between 3-9 months of life

Foker technique

2x Stays through pleget + clip on upper and lower pouches bring out crossing through skin

Place through and tie inside piece of feeding tube to avoid cutting skin

Place gauze under tube daily to increase traction

Paralyze

Serial X-Rays

Likely 2 weeks to closure

Internal traction sutures can be used without paralysis

Give another 3 months of traction

If fails - gastric pull up

Other methods:

Van der Zee thoracoscopic internal traction sutures - return to theatre every 48 hours for tightening

Circular myotomy (can be either proximal or distal ends) is not recommended by ERNICA, but is suggested to be safe by APSA systematic review

Gastric pullup

ERNICA long gap consensus paper - do not do thoracotomy for pull up (thoracic anastomosis causes severe reflux)

GOSH typically performs at 1 year of age

Pre-op: CT for vascular ring, bronchoscopy for missed fistula

Have cardiac and ENT surgeons available in case of complications

Steps:

Take down gastrostomy

Mobilise stomach

Sacrifice left gastric and distal oesophagus

Mikulicz Pyloroplasty

Isolate cervical oesophagus

Bring fundus up through posterior mediastinum checking orientation of stays

Anastomosis in neck

Close crura, place jejunostomy if not established oral or sham feeds

Jejunal interposition

CHUGs talk BAPS 2024 -

If patient has oesophagostomy, makes procedure difficult because it makes a high difficult anastomosis and the blood supply on the vascular pedicle is more stretched - stomach has better blood supply

Can do at 2-4 months of age

Colonic interposition is now last resort

Long gap intra-op discovery

Options:

1. Fully mobilise upper pouch

2. Anastomosis under tension

3. Mobilise lower pouch - risks pulling stomach into chest - severe reflux

4. Replogle + gastrostomy + internal traction sutures (to pouches or to pre-vertebral fascia) and operate at 3 months - follow above

Special scenario - Pre-operative emergencies

Preoperative Respiratory Distress

Review baby immediately

Rule out abdominal distension

Check the replogle tube yourself: blocked, displaced etc.

Flush/replace and make sure it is working/suctioning

Make sure upper pouch is empty

Reassess

Check Echocardiogram

If persists: Emergency thoracotomy to ligate fistula vs intubation beyond the fistula

Preoperative Cyanosis

Review baby immediately

Make sure upper pouch is clear

Check echocardiogram

Urgent cardiology review

Preoperative Abdominal Distension

Gastric distension:

Emergency Thoracotomy to ligate fistula or pressure controlled needle drain then thoracotomy

Distension with air in peritoneal cavity:

Gastric Perforation

Emergency laparotomy and ligate oesophago-gastric junction and repair hole

Special scenario - OA/TOF extreme prematurity

Urgent thoracotomy - transfix and divide fistula only (If cannot close fistula, do a laparotomy and place silastic band around GOJ)

Then manage with TPN until sufficient weight to perform open gastrostomy

Manage as per long gap OA with serial gap assessments before thoracotomy and anastomosis

Or

Just gastrostomy (if possible) and replogle tube for upper pouch, allow to grow for primary anastomosis

Special scenario - stridor/life threatening event after OA/TOF repair

Differentials:

1. Recurrent fistula

2. Tracheomalacia

3. Vascular ring

4. Foreign body

Resuscitate - involve medical team and PICU if needed

History

Duration

Feed tolerance

Diet

Foreign body ingestion

Examination

Drooling

Cyanosis

Investigations

CXR

Refer to ENT: MLTB + endoscopy

CT for vascular ring

Management based on diagnosis

Page edited by Mrs Charnjit Seehra BSc November 2024

Page edited by Prof. Ashok Daya Ram MBBS, FRCS, FRCPS, FEBPS, FRCS (Paed Surgery), Consultant Paediatric and Neonatal Surgeon, Norfolk and Norwich University Hospital, Norwich, UK. January 2025

References

Elseth, Anna, et al. "Esophageal Atresia and Tracheoesophageal Fistula." Pediatric Surgery NaT, American Pediatric Surgical Association, 2022. Pediatric Surgery Library, www.pedsurglibrary.com/apsa/view/Pediatric-Surgery-NaT/829035/all/Esophageal_Atresia_and_Tracheoesophageal_Fistula.

Long AM, Tyraskis A, Allin B, Burge DM, Knight M. Oesophageal atresia with no distal tracheoesophageal fistula: Management and outcomes from a population-based cohort. J Pediatr Surg. 2017 Feb;52(2):226-230. doi: 10.1016/j.jpedsurg.2016.11.008. Epub 2016 Nov 13. PMID: 27894760.

Allin B, Knight M, Johnson P, Burge D; BAPS-CASS. Outcomes at one-year post anastomosis from a national cohort of infants with oesophageal atresia. PLoS One. 2014 Aug 25;9(8):e106149. doi: 10.1371/journal.pone.0106149. PMID: 25153838; PMCID: PMC4143357.

Burge DM et al. British Association of Paediatric Surgeons Congenital Anomalies Surveillance System (BAPS-CASS). Contemporary management and outcomes for infants born with oesophageal atresia. Br J Surg. 2013 Mar;100(4):515-21. doi: 10.1002/bjs.9019. Epub 2013 Jan 18. Erratum in: Br J Surg. 2013 Jul;100(8):1117. Cusick, E [corrected to McNally, J]; de la Hunt, M [corrected to Hosie, G]. PMID: 23334932.

Baird R et al. Management of long gap esophageal atresia: A systematic review and evidence-based guidelines from the APSA Outcomes and Evidence Based Practice Committee. J Pediatr Surg. 2019 Apr;54(4):675-687. doi: 10.1016/j.jpedsurg.2018.12.019. Epub 2019 Feb 7. PMID: 30853248.

Dingemann C, et al. ERNICA Consensus Conference on the Management of Patients with Long-Gap Esophageal Atresia: Perioperative, Surgical, and Long-Term Management. Eur J Pediatr Surg. 2021 Jun;31(3):214-225. doi: 10.1055/s-0040-1713932. Epub 2020 Jul 15. PMID: 32668485.