Thoracoscopic oesophagectomy for end-stage achalasia

Abstract

Achalasia cardia is an oesophageal motility disorder characterised by aperistalsis and failure of relaxation of the lower oesophageal sphincter. The management is predominantly palliative with focus on addressing the sphincter that involves either pneumatic dilatation or Heller myotomy which relieves dysphagia in the majority of the cases. End-stage achalasia (ESA) is characterised by failed myotomy, massively dilated and tortuous oesophagus with nutritional deterioration due to progressive dysphagia and vomiting. In these subgroups of patients, oesophagectomy may be the last resort. While oesophagectomy has been described for ESA before, thoracoscopic oesophagectomy has not been reported previously. Hereby, we report our experience of performing minimally invasive oesophagectomy (thoracoscopic) with the gastric pull-up.

INTRODUCTION

Achalasia is a primary motility disorder of the oesophagus. It is characterised by impaired relaxation of the lower oesophageal sphincter (LOS) and loss of peristalsis in the oesophagus.[1] Progressive dysphagia to both solids and liquids, regurgitation, chest pain, weight loss and occasional respiratory symptoms are commonly encountered symptoms. The primary surgical modality is laparoscopic Heller's myotomy (LHM) which reduces the pressure gradient across the LOS to facilitate and improve oesophageal emptying.[1]

Inadequate therapy leads to progressive dilatation and tortuosity of the oesophagus that results in end-stage achalasia (ESA). ESA occur either from failed myotomy or persistent absence of oesophagal peristalsis leading to dilatation with axis deviation. Hence, it may necessitate oesophageal resection to restore gastrointestinal function. Both transhiatal and transthoracic oesophagectomy have been reported for ESA with associated pros and cons.[2,3,4] We hereby discuss the case of ESA managed with the thoracoscopic mobilisation of the dilated oesophagus with gastric pull-up and cervical oesophagogastric anastomosis.

CASE REPORT

A 25-year-old female presented to us with a history of painless, progressive dysphagia to both solid and liquid food for the past 4 years. Initially, she was evaluated elsewhere where barium swallow and oesophagal manometry were suggestive of achalasia cardia. She had undergone an LHM with posterior fundoplication. Post-LHM, she did not have significant relief.

She was reassessed at another centre and was found to have insufficient myotomy. Hence, they performed re-do LHM with the extension of myotomy. She had partial relief of symptoms for 3 months, but thereafter had a relapse of dysphagia. Her symptoms of dysphagia and intermittent episodes of vomiting persisted and progressed over 2½ years before she was referred to us for further management.

At the time of evaluation, her body mass index was 21 kg/m². She was investigated with a barium swallow, a computed tomography (CT) thorax and an endoscopy. The barium study revealed a markedly dilated oesophagus with delayed emptying and axis deviation [Figure 1a]. Endoscopy confirmed a fluid-filled mega-oesophagus with patent previous myotomy site. CT scan reinforced findings of mega-oesophagus (5 cm) with a deviation of axis [Figure 1b]. Manometry was suggestive of aperistaltic oesophageal body. In view of persisting dysphagia even after two attempts at myotomy, dilated oesophagus with axis deviation and the absence of oesophagal peristalsis, we decided to proceed with oesophageal resection and gastric pull-up after discussing with the patient.

Figure 1.

(a) Barium swallow and (b) contrast enhanced computed tomography thorax coronal multiplanar reformation image depicting dilated oesophagus with axis deviation and delayed emptying

Thoracoscopic oesophagectomy was performed in prone position [Figure 2a]. Oesophagus was found dilated with the thickened wall with increased vascularity. Thoracoscopy permitted safe dissection to tackle the peri-oesophageal adhesions and increased vascularity. The total operative time was ~330 min with ~100 ml of blood loss.

Figure 2.

(a) Thoracoscopic mobilisation of dilated oesophagus; (b) resected specimen of end stage achalasia showing grossly dilated oesophagus throughout its extent

The abdominal procedure was performed by a limited upper midline incision. The cervical oesophagus was mobilised and divided through left neck incision. Gastric pull-up with end-to-side cervical oesophagogastric anastomosis was done in a single layer, with interrupted polyglactin 3-0 sutures. Resected specimen showed dilated oesophagus throughout its extent [Figure 2b].

She had an uneventful post-operative course. Feeding through jejunostomy placed intra-operatively was started on the 2^nd post-operative day (POD). She was allowed orally on 5^th POD after getting an oral contrast study which showed no evidence of leak. She was discharged on the 8^th POD.

Histopathology of the resected specimen was consistent with achalasia, revealing marked hypertrophy of the muscularis propria, and an absence of ganglion cells in myenteric plexus of Auerbach's. At 3 months of follow-up, her oral intake is good with the improved quality of life.

DISCUSSION

LHM is the most commonly performed procedure for achalasia cardia.[1] However, being a palliative procedure, the management of failure is a therapeutic challenge. Various causes of failure including inadequate myotomy, tight fundoplication, development of reflux stricture, or development of malignancy need to be considered. Early failure usually indicates incomplete myotomy or obstruction by fundoplication and needs to treated by completion myotomy or revision of fundoplication. However, persistent symptoms in patients who underwent a sufficient myotomy or an extended myotomy later with an associated significantly dilated oesophagus, are likely to warrant resection.

ESA requiring oesophagectomy is reported in ~5% of all achalasia patients.[5] However, patient selection is of utmost importance before opting for this irreversible step. Oesophagectomy is usually indicated in those with a tortuous and dilated oesophagus with axis deviation, with failed re-do myotomy, and if symptoms are upsetting quality of life significantly.

Both transthoracic and transhiatal approaches have been described for oesophagectomy in achalasia.[2,3,4] However, oesophagectomy is technically difficult in ESA due to a combination of factors. Primarily, the altered anatomy of mega-oesophagus with axial deviation poses a problem. Second, hypertrophied oesophageal wall with prominent vessels makes haemostasis difficult during mediastinal dissection. Finally, prior surgery leads to scarring and adhesions with undersurface of the left lobe of liver, lung and aorta making transhiatal mobilisation difficult. The intra-operative need for conversion to thoracotomy while performing transhiatal oesophagectomy has been reported.[2,3] Hence, some surgeons primarily recommend a transthoracic approach for oesophagectomy in achalasia.[4,6] However, thoracotomy itself is fraught with increased post-operative pain and morbidity, especially chest related complications prolonging hospital stay.

Hence, we used a minimally invasive approach to mobilise oesophagus. Thoracoscopic mobilisation of oesophagus provides clear visualisation with magnification during oesophageal dissection, easy haemostasis and can avoid injury to adjacent structures. The intra-operative time and blood loss were comparable and perioperative respiratory complaints were minimal. The proposed advantage of this approach makes it useful as compared to other laparoscopic transhiatal or transthoracic approach. Minimally invasive oesophagectomy (MIE) in the prone position for malignancy has been reported to be safe and technically feasible.[7,8] Till date, thoracoscopic oesophagectomy for ESA has not been reported in English literature.

CONCLUSION

Thoracoscopic mobilisation of the oesophagus in the experienced hands is safe and useful for ESA following failed measures for achalasia. It reduces the risk of intra-operative injuries and avoids the need for thoracotomy. Larger series may pave the way for thoracoscopic mobilisation (MIE) being the standard of care in benign conditions like achalasia cardia.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.