Introduction

Central Core Disease (CCD), also known as central core myopathy, is a rare congenital muscle disorder linked to RYR1 gene mutations. It presents with lifelong muscle weakness, delayed motor milestones, and occasionally,  skeletal abnormalities like scoliosis or hip dislocation. Some patients also experience difficulty swallowing and impaired respiratory reserve. Beyond these chronic challenges, surgery introduces unique risks:

• Malignant hyperthermia (MH) occurs when exposed to certain anaesthetics
• Residual muscle weakness after relaxants affects breathing and swallowing
• Rhabdomyolysis, the breakdown of muscle tissue, with risks of kidney injury
• Exacerbation of spinal deformities during positioning

Because of these vulnerabilities, anaesthesia for CCD requires meticulous planning.

Preoperative evaluation: the cornerstone of safety

A comprehensive preoperative assessment significantly reduces risks. It should include:

• Detailed history and medications review, including any previous anaesthetic complications
• Multispecialty input — cardiology, pulmonology, and neurology reviews are valuable. Pulmonology is especially important as many CCD patients have restrictive lung function
• Neurological evaluation — recognising whether the disorder is prejunctional, junctional or postjunctional, since this influences drug response
• Physical examination — attention to spinal deformities, swallowing ability, and mobility

Laboratory tests:

• Baseline creatine kinase (CK) and myoglobin blood tests — help track perioperative rhabdomyolysis (muscle breakdown)
• Arterial blood gases and metabolic panel to detect underlying respiratory or metabolic impairment
• Pulmonary function tests or chest X-ray to quantify respiratory reserve
• Electrocardiogram and, if indicated, echocardiography

This structured evaluation helps tailor anaesthesia, anticipate airway or breathing difficulties, and establish baselines to interpret postoperative complications.

Anaesthetic technique: trigger-free and carefully monitored

Trigger-free anaesthesia is the rule:

• Total Intravenous Anaesthesia (TIVA) using propofol and remifentanil
• Avoidance of volatile agents and succinylcholine
• Use of non-depolarising muscle relaxants (e.g., rocuronium), but always with quantitative monitoring
• Machine preparation with vapouriser removal, fresh soda lime, and prolonged high-flow flushing to eliminate residual volatile anaesthetic

A 63-year-old case report of lung resection in a woman with CCD demonstrates these principles in action. The anaesthetic team prepared the workstation, used TIVA, and performed dual-site neuromuscular monitoring at both the adductor pollicis and the masseter. Recovery was slower in the masseter, highlighting that upper airway muscles may lag behind limb muscles, so extubation should not rely on hand monitoring alone.

Intra-operative vigilance

Key monitoring elements include:

• End-tidal CO₂ — an early sign of MH
• Core temperature — rising fever suggests MH
• Neuromuscular monitoring — at least at the adductor pollicis (hand muscle), and ideally also the masseter (face/jaw muscle) to confirm airway muscle recovery
• Standard parameters — ECG, invasive blood pressure if major surgery, oxygen saturation, and depth of anaesthesia

With these precautions, MH is very unlikely, but the team must be ready with immediate dantrolene if signs appear.

Postoperative priorities

Respiratory support: extra vigilance for chest weakness, scoliosis-related restriction, or residual block.

Monitoring for rhabdomyolysis: blood tests for repeat CK and myoglobin, and renal function if symptoms (muscle pain, dark urine) arise.

Pain relief strategy: multimodal, using epidural or regional blocks where possible to reduce opioids.

Early mobilisation and physiotherapy to aid lung clearance.

The patient in the case study recovered smoothly, was extubated without difficulty, and was discharged home within a week — proof that with planning, CCD surgery can be safe.

Special circumstances

Pregnancy: regional techniques such as spinal and epidural are favoured for labour and caesarean section. If general anaesthesia is essential, it must be trigger-free.

Emergency surgery: even under time pressure, trigger-free anaesthesia and MH preparedness are non-negotiable.

What patients and families should know

Carry documentation of CCD diagnosis and genetic results.

Wear a medical alert bracelet: “Malignant Hyperthermia Susceptible — Avoid Volatile Anaesthetics & Succinylcholine.”

Share the diagnosis with all healthcare providers — including dentists and emergency staff.

Ask hospitals if they stock dantrolene and follow MH protocols.

Conclusion

Anaesthesia in CCD is challenging but safe with appropriate preparation. The risks — MH, rhabdomyolysis, residual weakness, respiratory compromise — can be minimised by:

• Comprehensive preoperative evaluation (history, labs, CK/myoglobin baseline, multispecialty input)
• Trigger-free anaesthesia and machine decontamination
• Dual-site neuromuscular monitoring to protect airway safety
• Close postoperative surveillance for breathing, rhabdomyolysis, and pain control

The case report of lung surgery, combined with modern guidelines, shows that with vigilance, patients with CCD can undergo complex operations safely.