Short myotomy
Incomplete Myotomy
This may occur because of scar tissue at the level of the GEJ secondary to prior endoscopic treatment [2, 7, 16–18]. Both pneumatic dilatation and intrasphincteric injection of botulinum toxin can cause scarring at the level of the GEJ with fibrosis and loss of the normal anatomic planes. In these cases, the myotomy is more difficult, perforation of the mucosa is more common , and the results are less predictable [16].
Lack of Separation of the Muscle Edges
Tight Closure of the Hiatus
We do not advocate hiatal closure in the average patient with achalasia because sutures that narrow the hiatal opening may impair esophageal emptying. Hiatal closure should be considered only for the rare patient who has an associated large hiatal hernia; and in those patients, we recommend the hiatus be closed only partially to avoid persistence of dysphagia.
Wrong Type of Fundoplication
Wrong Configuration of the Fundoplication
Too many stitches at this level will cause constriction of the GEJ . Patti et al. showed that problems with the construction of a Dor fundoplication can be a cause of both persistent and recurrent dysphagia [2]. A Toupet fundoplication (240° posterior) may also cause angulation of the esophagus and problems with esophageal emptying [11].
Recurrent Dysphagia
These are patients who experience substantial relief for months or years after the initial Heller myotomy and then experience progressive dysphagia. The specific cause of recurrent dysphagia is not always easy to elucidate as progression of disease, scarring in the area of the previous Heller, or cancer may be causing it. Most common causes of recurrent dysphagia are:
Scarring of the Distal Edge of the Myotomy
360° Fundoplication
A partial fundoplication is the procedure of choice in conjunction with a Heller myotomy as it takes into consideration the lack of esophageal peristalsis. Because both a Dor and a Toupet fundoplication are effective in controlling reflux in only 80–90% of patients, some authors proposed the use of a Nissen fundoplication [21]. This approach, however, is associated with poor long-term results [22, 23]. For instance, Rebecchi et al. compared 71 patients who underwent a laparoscopic Heller myotomy and Dor fundoplication with 67 patients who had a Heller myotomy and a Nissen fundoplication [23]. After 10 years, dysphagia was present in 2.8% and 15% of patients, respectively. Similar problems have been reported by others [22].
Gastroesophageal Reflux Disease
Postoperative reflux is present in 50–60% of patients when a myotomy alone is performed and in 10–20% when a partial fundoplication is added. Abnormal reflux is considered a common cause of recurrent dysphagia. Csendes et al. showed that there is a progressive clinical deterioration of the initially good results over time and that this deterioration is mainly due to an increase in pathologic reflux and the development of short or long-segment Barrett’s esophagus [24]. Unfortunately, most patients that develop pathologic reflux are asymptomatic [1]. It is therefore very important, particularly when operating on young patients, to perform an ambulatory pH monitoring after the operation [25]. If abnormal reflux is present, acid-reducing medications should be prescribed and closer endoscopic follow-up performed.
Esophageal Cancer
Achalasia patients are at increased risk of developing squamous cell carcinoma. In addition, if pathologic reflux occurs after the myotomy, Barrett’s esophagus and adenocarcinoma can develop causing recurrent dysphagia [26]. Although precise guidelines about endoscopic follow-up in achalasia patients have not been established, an upper endoscopy should be routinely performed every 3–5 years.
Diagnostic Evaluation
When a patient complains of recurrent dysphagia, it is important to perform a complete workup to try to identify the cause in order to formulate a tailored treatment plan [27].
The first step should always be to review the entire history – in particular that which existed before the first operation – and to review, when possible the diagnostic tests performed before the initial operation. It is at this time that we have found that some of these patients did not have achalasia to begin with. Once this process is complete, we like to review the report of the original operation. Often there are clues that explain the symptoms, such as the description of scar tissue due to prior treatment, failure of identifying the anatomic planes, or a short myotomy .
The symptomatic evaluation is the next step. It determines which symptoms are present and compares them to the symptoms present before the first operation. In addition, it distinguishes between persistent and recurrent dysphagia.
A barium swallow is very useful to determine the cause of the dysphagia. It identifies the area of obstruction, assesses the degree of esophageal dilatation, the emptying of the barium from the esophagus into the stomach, and shows the overall shape of the esophagus. It might help distinguish between a short myotomy, a tight closure of the hiatus, and a constricting or malpositioned fundoplication. Loviscek et al. reported a series of patients with recurrent dysphagia after Heller myotomy who underwent redo surgery and were able to correlate the preoperative radiologic findings on barium swallow to the postoperative improvement in symptoms. All patients with a straight esophagus (normal or dilated caliber) had improved dysphagia after revisional surgery, whereas dysphagia improvement was less consistent if the esophagus was sigmoid in shape [27].
An upper endoscopy should be carried out in every patient as it can show if there is mucosal damage due to reflux, candida esophagitis due to slow emptying, or cancer. Endoscopic evaluation can also reveal angulation of the distal esophagus due to a malpositioned or overly tight fundoplication.
Esophageal manometry is essential to confirm the diagnosis of achalasia and to measure the pressure and relaxation of the lower esophageal sphincter. When compared to the preoperative test, it can show if the myotomy has been extended appropriately onto the gastric wall or if a residual high-pressure zone is still present.
Ambulatory 24-hour pH monitoring should be performed in patients with recurrent dysphagia. It is important to look not only at the reflux score, but to review the pH tracing to distinguish between real reflux and false reflux due to stasis and fermentation. This test should be routinely done even in asymptomatic patients after a Heller myotomy as reflux can be often “silent” [1]. This is particularly important when operating on children as a life-long exposure to reflux can cause Barrett’s esophagus or even esophageal cancer [24, 26, 28].
When pseudoachalasia secondary to the presence of cancer is suspected, endoscopic ultrasound and computed tomography can help establish the diagnosis [29].
Treatment
Pneumatic Balloon Dilatation
A balloon dilatation should always be considered in patients with recurrent dysphagia. Contrary to common belief, the perforation rate is very low due to the fact that the myotomy is covered by the stomach if a Dor was performed or by the left lateral segment of the liver if a Toupet was added to the myotomy. Zaninotto et al. documented recurrent dysphagia in 9 of 113 patients (8%) after laparoscopic Heller myotomy and Dor fundoplication [19]. Seven of the nine patients were effectively treated by balloon dilatation (median two dilatations, range 1–4), while two required a second operation. Similar results were described by Sweet et al. who reported on the effectiveness of dilatation for the treatment of both persistent and recurrent dysphagia [7].
Revisional Surgery
If dysphagia is not relieved by dilatations, a reoperation must be considered. When consenting the patient, it is important to stress that even though most cases can be performed laparoscopically, a laparotomy might be needed. In addition, patients must be aware that in case of severe damage to the mucosa during the course of the operation, an esophagectomy may be necessary.