Thoracic epidural anaesthesia for thoracotomy, oesophageal myotomy and cystotomy-capitonnage in an elderly patient wıth achalasia due to scleroderma☆

1. Introductıon 

Scleroderma (systemic sclerosis) is a chronic, systemic disease involving the skin, lungs, heart, gastrointestinal tract, kidney and musculoskeletal system. The disorder is characterized by three features: tissue fibrosis, small blood vessel vasculopathy and a specific autoimmune response associated with autoantibodies. Scleroderma is classified into two major subsets which are distinguished by whether the extent of skin thickening is limited or diffuse cutaneous scleroderma. The CREST syndrome is an acronym for subcutaneous calcinosis, Raynaud's phenomenon, oesophageal dysfunction, sclerodactyly and telangiectasia [1]. It may cause delay in wound healing. In this article, we report a case with achalasia due to scleroderma and multiple left lung hydatid cysts, in which we performed thoracic epidural blockade in combination with general anaesthesia with air–oxygen and sevoflurane in order to avoid the use of neuromuscular blockade and opioids.

2. Case report 

An 83-year-old, 59kg, 165cm, male patient was hospitalised for achalasia unresponsive to pneumatic dilations. His anaesthetic history included local anaesthesia for cataract surgery and intrathecal local anaesthesia for prostatectomy. His past medical history included pulmonary tuberculosis. On physical examination, he was thin with obvious sclerodactily of both hands and of anaesthetic importance, he had nasal telangiectasia and his mouth opening was restricted to less than half normal.

Investigations revealed the following abnormalities:

Hb: 9.9g/dL, Hct: 31%, sedimentation: 73mm/h. ENA screening: Scl-70 (positive) and ANA: (positive).

He had a forced vital capacity (FEV1) of 2L. Arterial blood gases were: pH: 7.5 PO2:

95.0mmHg PCO2: 38.5mmHg.

CT imaging revealed the dilated oesophagus in addition to multiple left lung hydatid cysts.

Echocardiograhy: minimal pericardial effusion.

The patient was transported to the operating room in a semi-upright position with supplemental oxygen. Premedication was limited to midazolam 1mg i.v. in the operating room before preoxygenation and anaesthesia induction with ketamine 50mg and propofol 50mg i.v. After manual ventilation, a further dose of ketamine 50mg and propofol 50mg i.v. proved sufficient to facilitate laryngoscopy, without using a muscle relaxant. Tracheal intubation was performed with a 39 French gauge left-sided double-lumen tube and the patient ventilated with sevoflurane 1.5% in an equal mixture of air and oxygen, without the need for neuromuscular blockade or opioids.

With continuing patient monitoring, an epidural catheter was inserted at the T11–12 epidural space using the loss of resistance to air technique and bupivacaine 10mL 0.5% with fentanyl 50mcg was administered epidurally. Intraoperatively, blood pressure ranged between 105/65 and 110mmHg/60mmHg, and heart rate ranged between 75 and 87bpm. Arterial blood gases were within normal limits. The duration of surgery was 4.5h. The patient underwent left thoracotomy through the seventh intercostal space to facilitate a modified Heller anterolateral oesophageal myotomy for achalasia. The cystotomy-capitonnage for the cysts was performed in the same session.

On completion of surgery the sevoflurane were discontinued. Twenty minutes after discontinuation of anaesthesia, the patient started to breathe spontaneously, to respond to painful stimuli and to react to the tracheal tube. The patient was extubated awake and transferred fully awake and co-operative, to the intensive care unit (ICU). He did not report pain or awareness during surgery. Spontaneous ventilation and cough reflex were adequate. Arterial blood gases were within normal limits. Postoperative analgesia was maintained by the thoracic epidural using bupivacaine 0.0625% and fentanyl 2mcg/mL at the rate of 4mL/h, with continuing careful monitoring. The patient did not complain of muscle weakness and was able to perform respiratory physiotherapy exercises. Pain relief was assessed every 3h on a visual analogue scale ranging from 0cm (no pain) to 10cm (unbearable pain). The collected scores during the first 24h were between 0 and 2. In the ICU, his blood pressure and heart rate were within normal limits during the first 24h. The patient maintained spontaneous breathing easily with good arterial blood gases. The patient was discharged from the intensive care unit on day 3 and from the hospital on day 9. The postoperative period was uneventful and he has had no recurrence of symptoms in the first 6 months after surgery.

3. Discussion 

Scleroderma is a connective tissue disease which involves skin, lung, gastrointestinal system and kidneys. It is an autoimmune disorder and causes vascular damage especially in capillaries and small arteries [1].

Achalasia and scleroderma account for the majority of surgical procedures performed for motility disorders. Achalasia usually occurs as an isolated finding while scleroderma is part of a generalised collagen-vascular disorders.

The anaesthesiologist should be aware of these complex pathophysiological processes so as to select anaesthetic and analgesic techniques which minimise potential risks during and after surgery, particularly aspiration and other pulmonary complications. In addition, the potential for reduced renal function and intraoperative hypothermia-induced vasospasm must be considered [2].

Regardless of the procedures, the major anaesthetic consideration for patients with oesophageal disease is the risk of pulmonary aspiration [3]. Dyspnea on exertion may also be prominent when chronic aspiration results in pulmonary fibrosis. Patients may additionally present with anaemia and weight loss. Patients with scleroderma should be evaluated for involvement of other organs, especially the kidneys, heart and lungs; Raynaud's phenomenon is also common.

Aspiration of retained food in the oesophagus at the time of induction of anaesthesia and perforation of the oesophageal mucosa are the most common operative complications [12]. Consideration should be given to administering metoclopramide, an H2 blocker, or proton-pump inhibitor preoperatively; awake nasogastric suctioning may also be helpful in decreasing the risk of aspiration. The patient should be transported to the operating room in a semi-upright position with supplemental oxygen.

The goal should be a smooth induction maintaining spontaneous ventilation and haemodynamic stability.

We used intravenous ketamine with the intention of maintaining haemodynamic stability in this patient with reduced cardiac output. With the patient in a semi-upright position a rapid-sequence induction with cricoid pressure was conducted. Minimising or avoiding opioid use is recommended to allow early postoperative extubation in view of the risks associated with aspiration and respiratory mechanics and with respect to variable pharmacokinetics with hepato-renal dysfunction. Instead, epidural blockade was chosen for intraoperative and postoperative analgesia. Although the risk of aspiration may be lower following surgery, the patient should be extubated only when fully awake.

The anaesthetist should be aware of the difficulty in opening the mouth wide enough for laryngoscopy and intubation, the possibility that cardiopulmonary changes may be present and that lesions in the oesophagus, bowel, kidneys, skin and joints are probable [4].

This is the first report on epidural anaesthesia in combination with sevoflurane based general anaesthesia being used to avoid neuromuscular blockade and intravenous opioids for thoracotomy and myotomy for achalasia due to scleroderma [5], [6], [7], [8], [9], [10], [11]. This currently described anaesthetic technique, which avoided muscle relaxants, provided excellent intubating and operating conditions, with effective analgesia into the postoperative period, preserves the function of the diaphragm and allows earlier extubation.

Non-relaxant techniques are a recognised method of anaesthesia in achalasia due to scleroderma.

Thoracic epidural analgesia is ideal in this situation providing effective analgesia while reducing the risk of respiratory failure.

Having demonstrated on the first occasion the safety of the technique, we were able to avoid the delays associated with the need for critical care facilities. We believe that thoracic epidural blockade afforded excellent control of heart rate and pressor responses during surgery and by allowing the early return of adequate spontaneous ventilation, facilitated extubation 20min after the end of surgery.

Scleroderma predisposes to an increased risk of respiratory complications especially after thoracic surgery. We believe that preservation of neuromuscular function and the use of epidural patient-controlled analgesia in the first 2 days after surgery to avoid opioids contributed to early extubation and improved lung function postoperatively.

4. Conclusion 

In conclusion, the combination of thoracic epidural anaesthesia and sevoflurane based general anaesthesia may be a suitable technique for thoracic surgery in achalasia due to scleroderma. It can provide a smooth anaesthesia course and a rapid recovery, with haemodynamic stability, as well as postoperative analgesia.

In addition, avoidance of neuromuscular blockade and opioids may have shortened the recovery time.