A 23-year-old male patient was admitted to our clinic due to progressive difficulties in swallowing and a constant weight loss of 23 kg, which had developed over the past three years. During meals, dysphagia was overcome by drinking water. Additional symptoms were retrosternal pain, regurgitations (Eckardt score of 10), and respiratory ailments. While the laboratory results did not show any abnormalities, a computed tomography (CT) scan of the chest revealed a massive extension of the esophagus starting proximally of the cardia up to cervical vertebral body 7. The consequence of this megaesophagus was a transversal widening of the mediastinum up to 6.7 x 5.2 cm in diameter accompanied by a consecutive slit-shaped cranial narrowing of the trachea (Fig. 1). Due to the twisted passage of the esophagus, a standard manometry was not feasible. Upper endoscopy did not show any pathological alterations in the mucous membrane of the esophagus, stomach or duodenum.
Due to the age of the patient and his physical complaints we performed a laparoscopic Heller myotomy with anterior semi-fundoplication 180  according to Dor. Because of the profound tracheal stenosis, intubation was done with fiber optic equipment. Intraoperative findings revealed a distinctive thickening of the muscularis at the high-pressure zone of the LES. Surgical myotomy was performed by incision of muscle fibers with a length of 7.0 cm at the distal esophageal body and additional 2 cm at the proximal stomach. Due to the complicated intubation conditions, for safety reasons, the patient was transferred intubated and ventilated to the intensive care unit. Peri- and post-operative courses were uneventful. The patient was extubated one hour after surgery without complications. After the transfer to the patient ward, he was gradually accustomed to normal diet. He initially complained about occasional heartburn, which was successfully treated with proton-pump inhibitors.
Five months after surgery, the technical postoperative findings appeared normal. The physical examination did not reveal any pathology. Body weight remained stable and retrosternal pain and regurgitations disappeared, resulting in an Eckardt score of 1. The patient tolerated normal diet very well. Conventional postoperative esophageal manometry showed a resting pressure of 5.6 mmHg. The barium swallow test detected a residually altered morphology represented by diminished motility and disturbed contractions. In addition, the barium remained for an extended time within the esophageal body and complete emptying of the stomach was only possible by drinking copious amounts of water (Fig. 2). The performed tracheal X-ray imaging did not present a tracheal compression any longer. Overall, the patient was gratefully satisfied with the early postoperative result. Three and a half years after surgery, the structured interview demonstrated good results. Besides minor difficulties and pain in swallowing during meals, the patient presented in a very good clinical condition with an Eckardt score of 2. The initially reported symptoms of dysphagia, regurgitation, and respiratory ailments were absent.