Anesthesiological management for merosin-deficient congenital muscular dystrophy patient undergoing emergency surgery

Total intravenous anesthesia (TIVA) with reAdīness of difficult airway management and wound local anesthetic infiltration has been accepted as safest anesthesia method for muscular dystrophy patients. Here, we report a case of a 20-year old male with hereditary muscle dystrophy of merosin deficiency who was admitted to Riga East University hospital due to testicular torsion. The patient’s physical status class (ASA) was III-IV, body weight 45 kg. While assessing anatomy, all parameters predicted difficult airways - impaired temporomandibular joint motion, mouth opening 2 cm, thyromental distance 3.5cm, Mallampati IV, macroglossia, long upper front teeth, restricted neck movements, thorax deformation. Additionally, patient reported daily assistance of CPAP devise due to sleep apnea. Awake fiberoptic intubation approach with endotracheal tube (ID 7) was used after 2.5 mg of Midazolam administration intravenously (i/v). Depth-of-anaesthesia was monitored by Narcotrend index as electroencephalography measures. At awake patient had 95-100, during the surgery 55-65. TIVA was initiated with Propofol 2 mg/kg bolus following by continuous i/v infusion 1-3 mg/kg/h. Sedoanalgesia was continued with Phentanyl 50 mcg boluses. To reduce requirements of opioids, Ketamine 30-50 mg i/v was parallel used without myorelaxation. At the end of the surgery unexpected peripheral vein rupture occurred and Sevoflurane was inhaled (MAC 0.5-0.6) until a stable venous approach was secured. The general condition of patient remained stable. Extubation was performed when patient was fully awake. Anesthesia time was 1 hour, 45 minutes, surgery lasted 20 minutes. On the second postoperative day patient had signs of basal pneumonia which was successfully treated. TIVA with low dose opioids combined for a short period with inhalation agent proved to be an effective and safe anesthesia method for the muscular dystrophy patients management.