A 66 year-old woman with a history of atrial fibrillation, hypertension, gastritis, and hypothyroidism presented to our clinic in July 2014 with progressive dysphagia for solids and liquids over 18 months. Her symptoms occurred daily and resulted in an 11-kg weight loss over this period of time. She denied heartburn, regurgitation, choking or coughing during eating, chest pain, vomiting, abdominal pain, or change in bowel habits. Her medications included levothyroxine, metoprolol, pantoprazole, and warfarin. In June 2013, she underwent a barium swallow, upper endoscopy, and conventional manometry. The barium swallow showed a mildly dilated esophagus with tertiary contractions, delayed emptying of the esophagus, and a narrowed gastroesophageal junction (Fig. 1). The upper endoscopy demonstrated mild-moderate antral gastritis, normal appearing esophageal mucosa, and it was noted that the patient might have a tight gastroesophageal junction. The patient then underwent conventional manometry which was consistent with NSEMD. The patient was initiated on pantoprazole 40 mg daily. Unfortunately, after completing these tests she missed 3 follow-up appointments and was seen in our clinic only one year later.
Given her progressive dysphagia symptoms and weight loss, a high resolution esophageal manometry (HREM) was performed (Fig. 2). The study showed a normal median integrated residual pressure of 8 mmHg, normal mean resting pressure of 21 mmHg, normal mean residual pressure of 7 mmHg, and mean DCI of 10 770 mmHg  sec   cm. Of the 10 swallows, 30% were hypercontractile (> 8000 mmHg  sec   cm), 50% were normal and 20% simultaneous. Overall, the findings were consistent with jackhammer esophagus based on the Chicago Classification of Motility Disorders. Pantoprazole was increased to 40 mg twice daily with plan to add a pain modulator if her symptoms recurred.
Unfortunately, she was lost to follow-up again until she was seen in our clinic a year later in June 2015. She continued to have worsening dysphagia, lost an additional 7 kg, and reported new onset of occasional post-prandial chest tightness. At this point, a timed barium esophagram was performed (Fig. 3). This study demonstrated diffuse esophageal dilatation with retained secretions and barium with only 0-20% change in esophageal volume after 5 minutes. In addition, tertiary peristaltic waves within the distal esophagus, limited emptying of the contrast into the stomach and an air-fluid level were also noted. A repeat HREM was performed (Fig. 4) revealing a median integrated residual pressure of 71.5 mmHg, mean resting pressure of 97 mmHg, and mean residual pressure of 90 mmHg. There was panesophageal pressurization with every swallow and 80% of the swallows demonstrated prolonged pan-pressurization, all consistent with type II achalasia. Therapeutic options were discussed with the patient, and she elected to undergo Heller myotomy. The patient was referred to General Surgery but presented urgently to GI clinic 2 weeks later for worsening dysphagia, dehydration, weakness, inability to tolerate oral intake, and an additional 7-kg weight loss. The patient was admitted to the hospital and was noted to develop acute kidney injury. The patient underwent aggressive volume resuscitation. In addition, a nasogastric tube was placed and she was started on tube feeds to optimize her nutritional status. A week after discharge the patient underwent Heller myotomy with Dor fundoplication. On a follow-up visit with her general surgeon, she reported that she was tolerating liquids without any difficulties. The plan at that time was to initiate a soft diet.