{
  "abstract": "We present a rare case of achalasia associated with oesophageal atresia (OA) and distal tracheoesophageal fistula (TOF). OA/TOF is a congenital abnormality due to incomplete embryonic development of the foregut. Achalasia is defined as insufficient lower oesophageal sphincter relaxation and absent peristalsis of the oesophagus, and has clear high resolution oesophageal manometry (HROM) diagnostic criteria. It has been postulated that there is an associated abnormality of the Auerbach plexus of the distal oesophagus and fundus of the stomach in patients with OA/TOF.A retrospective case analysis and literature review of OA/TOF and achalasia was undertaken.A 14-year-old girl was assessed at a tertiary paediatric surgical centre for vomiting. The patient had a complete OA with distal TOF repaired at 3 months of age. This was complicated by anastomotic leak, requiring further definitive surgery at 4 months. She also had complete heterotopic tracheal rings of the oesophagus which were excised at 14 months in addition to a partial fundoplication. She later presented with progressive dysphagia and was diagnosed with GORD.She underwent time barium oesophagogram (TBE) at age 6 which demonstrated a long distal oesophageal narrowing, which was not evident at endoscopy. She had a total of 7 upper GI endoscopies which demonstrated distal oesophageal dilatation with gastric pooling and food debris but no stricture. She was then referred for HROM, which demonstrated pan-oesophageal pressurisation with non-relaxing lower oesophageal sphincter: Type II Achalasia.Achalasia in patients with OA/TOF is rare. We recommend inclusion of HROM in addition to TBE in children with OA/TOF having refractory reflux symptoms. Further research is required to evaluate the aetiology of oesophageal dysmotility in patients with OA/TOF.Reference Simoed P, et al. World Journal of Gastroenterology 2012;18(28):3662–3672.",
  "authors": [
    {
      "affiliations": [
        "Sheffield Children’s Hospital"
      ],
      "name": "Sarah Bruce"
    },
    {
      "affiliations": [
        "Great North Children’s Hospital"
      ],
      "name": "Adewale Oyinloye"
    },
    {
      "affiliations": [
        "Great North Children’s Hospital"
      ],
      "name": "Bruce Jaffray"
    },
    {
      "affiliations": [
        "Sheffield Children’s Hospital"
      ],
      "name": "Mike Thomson"
    },
    {
      "affiliations": [
        "Sheffield Children’s Hospital"
      ],
      "name": "Shishu Sharma"
    }
  ],
  "title": "OC69 A rare case of achalasia in a 14 year old with oesophageal atresia and tracheoesophageal fistula",
  "uid": "9d96b434-68f5-54ca-a12d-eb5e3a54efe8"
}