Peroral endoscopic myotomy (POEM) in a 3-year-old girl with severe growth retardation, achalasia, and Down syndrome.

Achalasia is a primary motility disorder with an annual incidence of 0.11 cases/ 100000 children [1]. In the field of natural orifice transluminal endoscopic surgery (NOTES), the peroral endoscopic myotomy (POEM) [2–3] is the only treatment to be performed routinely in humans. We report the case of a 3-year old female patient with Down syndrome who presented with severe growth retardation and weight at less than the 3rd percentile curve. Achalasia was suspected because of vomiting, recurrent cough, aspiration pneumonia, and growth retardation; it was confirmed by barium esophagogram, endoscopy, and esophageal manometry. The first treatment was pneumatic balloon dilation (balloon diameter 20mm). Although the lower esophageal sphincter pressure improved after dilation, the patient continued to vomit daily. POEM was performed 20 days after balloon dilation. With the exception of the gastroscope, which had a diameter of 9.0mm (GFXQ 260; Olympus, Tokyo, Japan), POEM was performed using the same equipment as that used in adult patients and followed the same steps (●" Fig.1): distal attachment (MH-587, Olympus); triangle-tip knife (KD640L; Olympus) to dissect the submucosal layer and perform myotomy; coagulating forceps (Coagrasper, FD411QR; Olympus) for hemostasis; CO2 insufflator (UCR; Olympus), VIO-300D electrogenerator (ERBE, Tübingen, Germany), and hemostatic clips (EZ-CLIP, HX-110QR; Olympus) to close the mucosal entry. The generator set-upwas also the same as that used for adults: endocut Q-mode (effect 2) for mucosal entry, spray coagulation mode (effect 2; 50 watts) to create the submucosal tunnel and for myotomy, and soft coagulation (effect 5; 80 watts) for hemostasis. The operation time was 198 minutes. There were no intraoperative or postoperative complications. Upper digestive endoscopy and barium esophagogram confirmed smooth passage through the gastroesophageal junction, without leakage (●" Fig.2). At 1 year after the procedure, the patient was asymptomatic and a normal weight for her age. To our knowledge, this is the first case of POEM in a 3-year-old child, and it demonstrates that POEM is feasible and successful inpediatric patientswith achalasia.