A baby diagnosed with epidermolysis bullosa associated with antral web

Abstract

Encountering a neonate with epidermolysis bullosa and pyloric atresia is uncommon in clinical practice. To our knowlege, a complete prepyloric antral web in association with epidermolysis bullosa has not been reported before. The main issues in this case were the prognosis of the overall condition and weighing the risks and benefits of surgery. In other cases, surgical management has had good outcomes, although the risks could include leaks, wound complications, and recurrent obstruction. Uneventful postoperative courses have been reported in many cases. We used abdominal exploration and performedHeineke-Mikulicz antro-pyloroplasty, with an intraoperative finding of a complete antral web. The patient had a smooth recovery with no immediate complications. No major small-term complications occurred, and no further operations were required. One month after the surgery, the patient began full feedings. However, his skin condition worsened with multiple attacks of sepsis; then he developed uncontrollable upper and lower gastrointestinal bleeding. Unfortunately, the patient died at 5 weeks due to sepsis. We report this case to add to the literature, and discuss the management options when facing such a challenging case.

Keywords

• Epidermolysis bullosa
• Antral web
• Pyloric atresia
• Case report