Mid-oesophageal traction diverticulum in a patient with systemic lupus erythematosus

Abstract

In this report, a case of a large mid-oesophageal traction diverticulum in a 66-year-old woman with systemic lupus erythematosus has been presented. She was initially managed conservatively with active surveillance for 6 years. When her symptoms progressed, she had repeat endoscopy and CT scan which showed an increase in size of the diverticulum to 6 cm in diameter. Her dysphagia had progressively deteriorated and she was only managing a liquid diet. She, therefore, proceeded to resection of the diverticulum by right thoracotomy and stapled diverticulectomy. She made an excellent postoperative recovery and at last review, 5 months after the operation, she was back at work, had put on weight, and was tolerating a normal diet.

Background

Oesophageal diverticula are rare outpouchings of oesophageal wall with a prevalence of 2/100 000 population/year.¹ The mean age of onset of oesophageal diverticula is 69 years² with an equal gender distribution.³

They are classified anatomically from proximal to distal as hypopharyngeal (Zenker’s diverticula) which are the most common (70%), mid-oesophageal and epiphrenic (10% and 20%, respectively).⁴ The line separating the latter two is considered to be 5 cm below the carina.⁵ The majority (85%) of cases of oesophageal diverticula are associated with a motility disorder with the highest concordance seen in epiphrenic diverticula.⁶ These are usually false diverticula with only mucosa and submucosa herniating through the muscular layer and the proposed mechanism is due to pulsion and high pressure. Mid-oesophageal diverticula, in contrast, are proposed to be generated by traction. Mediastinal inflammation is a possible cause of this due to adhesion to the muscular wall of the oesophagus and traction during swallowing. Mid-oesophageal traction diverticula are mostly seen on the right due to close association of the oesophagus with subcarinal lymph nodes in this area.⁷ They are conical in shape and often taper cranially. Hence, it is very rare for them to develop to a large size.⁸

Known associations of traction diverticula are causes of mediastinal inflammation including tuberculosis, histoplasmosis, anthracosis, sarcoidosis^{9 10} and rarely systemic lupus erythematosus.^{1 11}

Two-thirds of oesophageal diverticula are asymptomatic¹² and they are a rare cause of dysphagia.⁹ The natural history of asymptomatic oesophageal diverticula is relatively poorly described due to their rarity but most asymptomatic oesophageal diverticula are manged conservatively with surveillance imaging or endoscopy. Symptomatic patients with diverticula >4 cm are usually treated surgically due to the increased risk of aspiration pneumonia and malignancy.¹³ The prevalence of cancer in diverticula, however, is low (0.3%–3%).¹² Common symptoms of oesophageal diverticula include dysphagia, (postural) regurgitation, weight loss, epigastric/retrosternal pain. Patients also report respiratory symptoms such as nocturnal cough.⁷

Case presentation

A 66-year-old woman with a background of SLE (diagnosed 30 years ago) and associated arthritis and non-epileptic seizures presented with intermittent dysphagia and weight loss. She was independent, still worked full time and was a non-smoker. Her regular medications included hydroxychloroquine, carbamazepine, bisphosphonate, calcium supplements and prednisolone. Investigations at initial presentation, 6 years prior, had confirmed a mid-oesophageal diverticulum and she had initially opted for conservative management with dietary changes. Subsequently, she began to lose weight as her dysphagia worsened to the point at which she could only manage liquids. She also suffered with odynophagia. After fully informed consent, she proceeded to resection of this diverticulum via right thoracotomy and stapled diverticulectomy.

Investigations

When the patient first presented with dysphagia, a barium swallow was performed which demonstrated a large (5.5× 3.3 cm) mid oesophageal diverticulum with a wide (3 cm) neck. There was no obstruction to the flow of barium which rapidly reached the proximal small bowel. Three years subsequently, she underwent an upper gastrointestinal endoscopy which confirmed a large diverticulum from 28 to 32 cm containing food residue, and a small sliding-type hiatus hernia. A contrast-enhanced computed tomography scan of chest and abdomen in the weeks prior to surgery confirmed the large right-sided mid-oesophageal diverticulum at the level of the pulmonary veins, 6 cm in maximal dimension and containing food debris. The superior oesophagus was fluid filled (figure 1). There were features of aspiration with wide-spread tree in bud nodularity and bronchiectasis in both lungs. A repeat upper gastrointestinal endoscopy at this point demonstrated compression of the true lumen due to the large diverticulum (figure 2).

Figure 1.

CT scan showing large fluid filled oesophageal diverticulum.

Figure 2.

Large oesophageal diverticulum seen at endoscopy. The true lumen is in the centre of the image and the entrance to the diverticulum is on the right side.

Treatment

The patient was initially managed conservatively with dietary changes and no alterations were made to her regular medications (hydroxychloroquine, carbamazepine, bisphosphonate, calcium supplements and prednisolone). She had two acute admissions over the 6 years prior to surgery with retrosternal pain and dysphagia and she was successfully managed conservatively with antispasmodics.

Dieticians worked very closely with her in order to improve her weight before surgery. She needed fortified supplements and soft/sloppy diet to meet her daily caloric requirements.

She underwent diverticulectomy via an open transthoracic approach. Extensive adhesions were identified between the oesophagus and the right main bronchus and subcarinal nodes. After division of these the oesophagus was mobilised and the diverticulum was identified. The thoracic duct was also ligated at the hiatus to reduce the risk of chyle leak following oesophageal mobilisation. The right vagus nerve was also in close proximity to the diverticulum and was therefore divided. A stapled diverticulectomy was then performed (figures 3 and 4) using 2 firings of the Ethicon Echelon Flex Endopath Stapler (Johnson & Johnson Medical, New Jersey, USA) using 60 mm gold cartridges, re-enforced with bovine pericardium (Peri-Strips Dry Staple Line Reinforcement with Veritas (PSDV), Baxter, Berkshire, UK) after on-table endoscopy to ensure no encroachment of the true lumen. Antireflux surgery was not performed. Myotomy was not performed as the oesophagus was very thin which would have been a risk of mucosal perforation and also because the distal lumen appeared intact at endoscopy.

Figure 3.

Intraoperative image of stapling the diverticulum using Ethicon Echelon Flex Endopath stapler (Johnson & Johnson Medical N.V.).

Figure 4.

Intraoperative image of the oesophagus post diverticulectomy.

Outcome and follow-up

Postoperatively, the patient was nursed on an intermediate dependency unit. She was fed through a surgically placed feeding jejunostomy. She started soft oral feeds when she was stepped down to the ward on day 6. Once she started tolerating oral feeds, she was eventually weaned off her jejunostomy feeds. She did not have any major complications during her inpatient stay. She was discharged on postoperative day 10.

Consequent outpatient reviews noted that she was tolerating most oral foods except for certain kinds of meats. She had good energy levels and had resumed normal work (8 hourly shifts during weekdays). She did not have any problems with acid reflux and had managed to put on weight with the help of protein supplements. She did not experience any flare-ups of systemic lupus erythematosus postoperatively. The operation did not affect the management of her systemic lupus erythematosus. There were no changes made to her regular medications either.

Discussion

Surgical treatment of oesophageal diverticula can be through minimally invasive (laparoscopic/thoracoscopic/robotic assisted) or open (transabdominal/transthoracic) techniques. However, the mortality and morbidity rates can be as high as 11% and 75%, respectively. A meta-analysis of 25 observational studies showed that success rates for both approaches are similar.⁴ The length of hospital stay is longer for open surgery compared with minimally invasive surgery.⁴ Studies have shown a morbidity rate of 23% in thoracoscopic diverticulectomy as compared with 15% in thoracotomy.¹²

Very few cases of oesophageal diverticula in patients with systematic lupus erythematosus have been reported. One previous case has described a 49-year-old woman with systematic lupus erythematosus and a mid-oesophageal diverticulum who underwent a thoracoscopic-assisted oesophagectomy.¹ Another 74-year-old woman with no significant comorbidities had a 10 cm mid-oesophageal diverticulum treated surgically with open thoracotomy and diverticulectomy. In both these cases and others such reported, patients had a good outcome with resolution of their symptoms at follow-up.¹³

Major postoperative complications of surgical treatment of oesophageal diverticula include bleeding, anastomotic or staple line leak, fistulation, recurrent laryngeal nerve injury, mediastinal or pleural collections, chylothorax, pneumonia, pulmonary embolism.¹⁴ Staple line leak rates can be up to 33%.^{3 4} It has been suggested that staple line leak rates increase when more than one cartridge is used due to weak points created when suture lines cross over. Hence, thoracotomy, again, might be a better option as it allows a stapler with longer jaw to be used allowing stapling with just one cartridge, especially useful in diverticula with wide necks.³ However, an articulated stapler may still be effectively used thoracoscopically. It has been proposed that manual oversewing after stapling can decrease the rate of suture line leak.³ Staple line re-enforcement materials can also be used. These can be absorbable (L-lactic acid-co-epsilon-caprolactomne), semi absorbable (bovine pericardium) or non-absorbable (polytetrafluoroethylene). These re-enforcements help with buttressing the staple line and prevent anastomotic complications.¹⁵

Diverticulectomy is considered to be a better option compared with diverticulopexy because of better symptom resolution rate (85% and 65%, respectively). Despite a significant understanding of the pathology of oesophageal motility disorders, they can be difficult to diagnose. Hence, a definitive recommendation regarding routine versus slective myotomy and use of antireflux treatments in patients with oesophageal diverticula cannot be easily made.⁴ Usually, diverticulectomy with myotomy is adopted in patients with proven motility disorders in whom a pulsion diverticulum is the diagnosis. Diverticulectomy alone has been reported in patients with traction diverticula.^{4 13 16} However, a routine myotomy has been reported to reduce the rate of staple line leak from 26% to 12.4%.⁴

As with any operation, it has been shown that patients with significant co-morbidities who underwent oesophageal diverticulectomy had poorer postoperative outcomes.² However, one prior report describes a patient with severe ischaemic cardiomyopathy and a large mid-oesophageal diverticula who underwent trans-hiatal oesophageal diverticulectomy and made a good overall recovery postoperatively without any complications.⁵ Another case report demonstrates an oesophageal diverticulectomy in a patient with tuberculous lymphadenitis. Again, the patient made a good recovery while being treated postoperatively with antituberculous medications.¹⁷

For symptomatic patients, in whom surgery may be too risky, options such as salivary diversion and direct enteral feeding via gastrostomy or jejunostomy may be used. Endoscopic treatments including pneumatic dilatation of distal strictures, and even endoscopic stapled diverticulostomy for Zenker’s diverticula can be additional options in selected cases.¹¹

Learning points.

• Oesophageal diverticula are rare conditions and the majority are asymptomatic. These can be safely managed conservatively but in some cases symptoms will progress.

• Mediastinal inflammation is proposed to be the cause of mid-oesophageal diverticula due to traction. There have been very few cases of oesophageal diverticula reported in patients with systemic lupus erythematosus.

• Several surgical/endoscopic treatment options exist for management of oesophageal diverticula and these must be tailored to every individual case.

• Patients often have a good symptomatic outcome following surgery and this should be considered in progressive cases. Multidisciplinary team involvement with dietetic support is important for optimal recovery.