Anaesthesia at the Edge: Bariatric Surgery in a Patient with Rare Muscular Dystrophy

A 19-year-old male with Limb-Girdle Muscular Dystrophy (LGMD) Type 1, thoracolumbar scoliosis, and morbid obesity (BMI 41 kg/m²) was scheduled for laparoscopic sleeve gastrectomy. Severely limited in mobility and unable to stand independently, this patient presented one of the most complex anaesthetic challenges our team has encountered.

LGMD patients carry a heightened risk of potentially life-threatening Malignant Hyperthermia-like Reactions (MHT) when exposed to volatile anaesthetic agents or succinylcholine. With this in mind, our anaesthesiology team planned a meticulous TIVA (Total Intravenous Anaesthesia) approach — avoiding all known trigger agents — and prepared the theatre to strict Malignant Hyperthermia-safe protocols, including the availability of dantrolene.

Comprehensive preoperative cardiac and respiratory evaluation showed no contraindications to surgery. Anaesthesia was carefully conducted using manually titrated propofol and remifentanil infusions, guided by Bispectral Index (BIS) monitoring. Non-depolarising neuromuscular blockade was administered at reduced dosing with continuous quantitative monitoring. Throughout the procedure, haemodynamic stability was maintained without incident.

The patient emerged smoothly, was extubated awake with full respiratory effort, and recovered without complications. No rhabdomyolysis, MHT signs, or positioning-related injuries occurred. This case demonstrates that with meticulous planning, advanced monitoring, and a skilled multidisciplinary team, even the highest-risk patients can be taken safely through major surgery.

A note of institutional progress: BSH now has BIS monitoring available for all high-risk anaesthetic cases, and Target-Controlled Infusion (TCI) technology is on its way — further enhancing our anaesthetic precision and patient safety.

 Key Learning Points:

• LGMD patients should be treated as Malignant Hyperthermia-susceptible; avoid volatile agents and succinylcholine.
• Manual TIVA guided by BIS monitoring is a safe and effective alternative when TCI is unavailable.
• Meticulous positioning and pressure-point protection are critical in patients with neuromuscular disease.
• Multidisciplinary preoperative planning is the foundation of safe anaesthesia in complex, rare conditions.

Credit: Case managed and reported by Dr. Fadi Alshalak  &  Dr. Barkha Begum, Anaesthesiology Department, Bahrain Specialist Hospital