Patient information: we received a 12 years old male adolescent, brought to our service for dysphagia for both liquids and solids, which started a year ago. At its beginning, the patient could improve the swallowing by drinking more water during meals and by having small bites. Dysphagia progressively worsened as in following months, appeared regurgitation following meals after some hours, made of undigested food of the latest meals. In the last 6 months, parents describe a progressive wasting for which they have consulted a medical centre where no clear solution has been found. Then the patient started experiencing a retrosternal discomfort, described as heartburn as well as sialorrhea. As symptoms were worsening, parents decided to consult at our hospital. The patient has normal infancy and childhood periods, without any specific swallowing complaint. There was no history of caustic ingestion and the patient has never travelled in South America or any other region endemic to Trypanosoma cruzi. He had no herpetic infection earlier, neither a severe bacterial infection. Such disorder has never been noticed in his siblings, neither in his father nor mother s family.
Timeline: first symptoms were experienced by the child in October 2019 as dysphagia, which progressively worsened from then to April 2020. From May 2020, parents report wasting for which they attended a medical centre in June 2020, with no solution provided. Additional symptoms (heartburn and sialorrhea) appeared in September 2020, which lead parents to attend our hospital on 07 October 2020, where diagnosis has been retained on 8 October 2020 and surgery performed a day later.
Clinical finding: on physical examination, the patient was conscious. He was wasted and with moderate dehydration. Body temperature was normal, he had polypnea and tachycardia, his blood pressure was normal. He weighed 26 Kg and had a height of 124 Cm, his body mass index (BMI) was 11.9 Kg/m2, (Figure 1). The rest of the examination was not contributive. There were neither alacrimia, nor features of adrenal insufficiency, nor vitiligo or deafness.
Diagnostic assessment: investigations included blood tests: glucose, natrium and potassium, which shown moderate hypoglycaemia. Imaging included an esophagogram which showed a dilated esophagus, with stasis of the contrast meal and a progressive narrowing picturing the classic bird s beak of achalasia cardia (Figure 2). Due to lack of equipment and financial constraint, no further investigation could be done, manometry and endoscopy, respectively.
Therapeutic intervention: at our service of emergency, rehydration has been started as well as parenteral nutrition. After two days of reanimation, the patient underwent a median sus umbilical laparotomy. A Heller esophago-cardiomyotomy has been performed with a five centimetre incision, running from the lower esophagus to the cardia, on the anterior face of the stomach. A Toupet fundoplication has been added as an antireflux procedure, with stitches placed at esophago-cardiomyotomy s edges (Figure 3).
Follow-up and outcomes: paracetamol has been used as postoperative analgesic (15 mg/Kg, qid), what about antibiotic? and oral feeding started 24 hours after surgery. The postoperative period has been smooth and the patient has been released on postoperative day five. The patient has been seen on postoperative day 10 with no major complaint and then, on the third and sixth months after surgery. All symptoms have remarkably regressed since postoperative day five. At the latest follow-up (6 months after surgery), the patient doubled his weight, going from 24 to 48 Kg. The patient will be yearly followed and during his second decade, endoscopic screening for esophageal carcinoma will be started.