Steinert’s disease is a type of muscular dystrophy with myotonia as a characteristic feature. Clavicle fractures are the primary type of scapular lesions that occur more frequently in men, mostly during cycling and equestrian sports.

The patient has a history of hoarseness and recurrent episodes of dyspnea and fatigue. He had previously undergone an anaesthetist-surgical procedure of cholecystectomy and cataract surgery.

The patient had locomotion disorder and right shoulder pain due to fall from height. A fracture with a deviation of proximal third of the right clavicle was shown by X-ray; therefore, surgical osteosynthesis under regional block associated with total intravenous anaesthesia was decided.  

Surgical room was prepared to prevent hypothermia, and conventional monitoring was done with cardioscopy, pulse oximetry and non-invasive blood pressure (NIBP). To monitor cerebral perfusion, non-invasive arterial pressure cuff was placed on the left arm. Skin antisepsis and local anaesthesia were performed without any sedatives and fully awake condition. The right supraclavicular nerve block was performed with the guidance of ultrasound. Two ramifications of supraclavicular nerve were identified, and 1 ml of 0.75% ropivacaine anaesthetic solution was infused in each branch. The upper right trunk block was performed in combination, and after identifying fusion of C5 and C6 roots, 10 ml of 0.75% ropivacaine anaesthetic solution was infiltrated. Following this, intravenous target-controlled infusion of propofol was given (target of 4 mcg/ml-1). A laryngeal mask number 4 was introduced, and SIMV (synchronized intermittent mandatory ventilation) was applied. No opioid or muscle relaxant was administered.  

The peripheral nerve block is the preferred anaesthetic technique for patients with Steinert's disease. General anaesthesia cannot be given to these patients because of the risk of pulmonary and cardiac dysfunction. Therefore, regional anaesthesia is recommended to avoid the drugs which trigger myotonic seizures.

For cervical plexus block and interscalene block, phrenic nerve palsy is a major risk. The Steinert's patients with the respiratory deficit are high in risk to phrenic nerve palsy. Therefore, upper trunk block and supraclavicular block emerged as a valid option to these patients because, at these sites, the distance from the phrenic nerve is more which reduces the possibility of paralysis. To allow ventilation control and a comfortable awakening, the laryngeal mask was used, which allowed greater control of the airway.

In summary, the supraclavicular nerve block proves to be an efficient technique for clavicle fracture osteosynthesis surgery. Additionally, by using ultrasound in regional anaesthesia, more selective blocks are possible with higher patient's safety and lower morbidity. It is imperative to consider preventive measures for the myotonic crisis while treating a Steinert's patient because of the peculiarities of the disease.   

For patients with clavicle fracture and Steinert's disease, superior trunk and cervical plexus block with venous anaesthesia under laryngeal mask proved to be work better. Selective blocks are now possible with the use of ultrasonography in regional anaesthesia, thereby increasing success rates of the anaesthetic-surgical procedure and lowering morbidity rates.

1. FMB Bisinotto, DC Fabri, MS Calçado, et al. Anesthesia for video-laparoscopic cholecystectomy in a patient with Steinert's disease: case report and literature review. Rev Bras Anestesiol, 60 (2010), pp.181-186.

2. J. Maybin, P. Townsley, N.Bedforth et al.Ultrasound guided supraclavicular nerve blockade: first technical description and the relevance for shoulder surgery under regional anaesthesia. Anaesthesia, 66 (2011), pp.1053-1055.

3. D. Burkett-St Laurent, SV Chan, KJ Chink. The superior trunk brachial plexus block. Can J Anaesth, 61(2014), pp.1098-1102.

4. D. Cope, J. Miller. Local and spinal anaesthesia for cesarean section in a patient with myotonic dystrophy. Anesth Analg, 65 (1986), pp.687-690.

5. X. March, J. Ross, G.Vizuete, et al.General anaesthesia combined with epidural anaesthesia in a case of Steinert's disease. Rev Esp Anestesiol Reanim, 39 (1992), p.133.

6. J. Kessler, I. Schafhalter-Zoppoth, AT Gray. An ultrasound study of the phrenic nerve in the posterior cervical triangle: implications for the interscalene brachial plexus block. Reg Anesth Pain Med, 33 (2008), pp. 545-550.