Endoscopic balloon dilation of esophageal stricture in dystrophic epidermolysis bullosa patient: challenges faced and safety of procedure.

Abstract

Background: Epidermolysis Bullosa (EB) stands as the prototype category of disorders featuring subepidermal fragility, characterized by skin blistering induced by minimal trauma. The gastrointestinal tract is a common site of extracutaneous injury. Esophageal stricture (ES) is one of the severe complications, with nearly 70% of patients experiencing ES within the initial 25 years of life.

Case report: We present a 11-year-old female child of dystrophic EB (DEB) who presented with dysphagia. Barium swallow showed a short segment proximal ES. We faced many challenges before endoscopy owing to difficult intravenous access, restricted mouth opening, multiple dental caries and low haemoglobin. Dental extraction under general anaesthesia and fibreoptic intubation with a smaller sized endotracheal tube guided over epidural catheter was done at another tertiary care institute. Child had severe bleeding due to airway manipulation.

Management: At our centre endoscopy guided serial balloon dilation (BD) of ES was performed without intubation under total intravenous anaesthesia (TIVA) without any complications. The stricture was serially dilated under direct visualization till 12 mm in three sessions at three-weekly intervals using CRE (controlled radial expansion) fixed and wire-guided balloon dilators. During the first session 20 mg of triamcinolone acetate injection was also topically applied without mucosal invasion. No such further topical or submucosal applications were attempted due to risk of perforation.

Conclusion: Endoscopy guided BD of ES is safe and effective in EB patients when done by experienced team.