ESPES, Vicenza, Italy, 11 - 13 September 2019, pp.56
We report here a case with Triple A syndrome, who had an esophageal perforation.
She is cured with endoscopic stent application.
13 years old female with swallowing difficulty had an openNissen fundoplication with
a diagnosis of GERD elsewhere. She is diagnosed as Triple A syndrome afterwards.
We performed a laparotomy, converted the Nissen to a modified Toupet
fundoplication and a Heller myotomy was added. After discharge she was admitted
with an esophageal perforation repaired primarily, however a recurrent perforation
developed. We decided to insert an endoscopic esophageal stent and periesophageal
drainage catheter. After a profound intensive care and treatment for 8 weeks, she was
discharged in normal nutritional and general condition.
After one week, the stent was removed endoscopically and some endoscopic clips
were applied to the site of the former perforation. She is in an excellent general
condition without any swallowing difficulty and gained weight.
Triple A syndrome is a mitochondrial oxidation defect, and therefore, in any
condition with the need of mitochondria has the risk of serious complication as in our
case with repeated esophageal perforation. Endoscopic stent application and
intensive supportive therapy may be the only successful treatment modality for failed
repair of esophageal perforation.