Limb Girdle Muscular Dystrophy

A CASE OF LIMB-GIRDLE MUSCULAR DYSTROPHY FOR TOTAL THYROIDECTOMY: ANAESTHETIC MANAGEMENT

INTRODUCTION
Limb-girdle muscular dystrophy (LGMD) refers to a genetically heterogeneous group of muscular dystrophies that present with weakness mainly involving the shoulder and hip girdles.1 LGMD has a predominantly proximal distribution of weakness which early in the course of the disease spares distal, facial, & extra ocular muscles. Most childhood onset cases have a pelvifemoral distribution of weakness. Adult-onset involves both shoulder and pelvic girdles with gradually increasing proximal limb weakness, thereby leading to restriction of mobility and eventually to wheelchair confinement. 2,3 Most of the complications from anaesthesia
Her medical history revealed autosomal dominant limb-girdle muscular dystrophy diagnosed 16 years back. She has 2 children who are diagnosed with same condition. Preoperatively her vitals were: Pulse-110bpm with regular rhythm, BP-140/80 mm of Hg in supine position, temperature of 37.20C, with clear chest on auscultation. She had quadriparesis with hypotonia of all 4 limbs. Power was: shoulder-2/5, elbow-3/5, wrist-4/5, hip-2/5, knee-2/5 and ankle-2/5. Deep tendon reflexes were absent with normal superficial reflexes. Airway assessment included adequate mouth opening with class I mallampati. Local examination of neck revealed a swelling of about 3×3 cm which moves with deglutition. Laboratory investigations: Hb-9.8g/dL, platelet-2.5laks/mm3, normal renal and liver function tests and normal thyroid profile. ECG shows tachycardia in sinus rhythm. Preoperative echocardiogram was normal with ejection fraction of 58%. CT scan of neck shows presence of hypodense lesion of 2.9×2.5 cm with specks of calcification suggestive of neoplastic process. FNAC was in favour of follicular neoplasm. Written consent was obtained. Procedure was explained to her in detail the previous day and was advised to stay nil per oral from 12 at midnight. For aspiration prophylaxis tablet pantoprazole 40 mg at night and at 7.00 am next morning and a night dose of tablet
As the patient was susceptible to malignant hyperthermia and sensitive to nondepolarising agents, we planned to do under regional anesthesia with a backup of total intravenous anesthesia. In operation room standard monitoring was done with Electrocardiogram, non-invasive blood pressure, pulse oximetry, end tidal carbon dioxide and temperature monitoring. Baseline vitals were recorded PR-100bpm, NIBP-140/80 mm of Hg, RR-18min, temperature -37.10 C, SpO2-100% on room air. An 18 gauze intravenous cannula was secured on left upper limb and ringer lactate transfusion started. Under strict aseptic precautions, bilateral superficial cervical plexus block and superior laryngeal nerve block was performed with local anesthetic mixture of 10 mL of 0.5%bupivacaine and 10 mL of 2% lignocaine. ECG monitoring showed occasional ectopics. Sedation was maintained with propofol infusion at 10mL/hr, midazolam 1mg i.v and inj.fentanyl 100µg i.v. Oxygen was supplemented via face mask. Duration of surgery was 120 minutes and vitals were maintained throughout the procedure. Postoperatively she was conscious, alert and obeying commands. She was shifted to SICU for