PICU Management of Malignant Hyperthermia

PICU Doc On Call

Welcome to PICU Doc On Call, A Podcast Dedicated to Current and Aspiring Intensivists.

• Hosts:
• Dr. Pradip Kamat: Children’s Healthcare of Atlanta/Emory University School of Medicine
• Dr. Rahul Damania: Cleveland Clinic Children’s Hospital

Introduction:

• Pediatric Intensive Care Unit (PICU) physicians passionate about medical education in the acute care pediatric setting
• Episode focus: A case of a 23-month-old ex-28 week premie presenting with sudden high fever and rapidly rising ETCO2 during surgery

Case Presentation:

• Presented by Dr. Rahul Damania
• 23-month-old ex-28 week premie intubated during hernia repair surgery
• Noticed rapidly rising ETCO2, unprovoked tachycardia, and elevated temperature
• Transferred to PICU, exhibiting rigidity, clenched jaw, metabolic acidosis, and elevated lactate.
• Consideration of Malignant Hyperthermia (MH) crisis

Key Points:

• Elevated temperature, hypercapnia, metabolic acidosis, and unprovoked tachycardia raise concern for MH
• Organized discussion on pathophysiology, clinical signs, symptoms, and management

Multiple Choice Question:

• Diagnosis of MH crisis during scoliosis repair
• Correct Answer: D) Sarcoplasmic reticulum
• Dantrolene acts on the sarcoplasmic reticulum to inhibit calcium release, crucial in MH management

Clinical Presentation of MH Crisis:

• Tachycardia, acidosis, muscle stiffness, and hyperthermia are hallmark features
• Potential life-threatening complications underscore the urgency of recognition and treatment

Triggers and Pathophysiology of MH Crisis:

• Triggered by inhalational agents and depolarizing neuromuscular blocking agents
• Pathophysiology involves defective Ryanodine receptor leading to uncontrolled calcium release

Differential Diagnosis:

• Includes sepsis, thyroid storm, pheochromocytoma, and neuroleptic malignant syndrome
• Differentiation from similar conditions crucial for accurate management

Diagnostic Approach:

• High clinical suspicion
• Genetic testing (ryanodine receptor gene sequencing) and Caffeine Halothane Contracture Test (CHCT) for diagnosis
• Immediate workup during crisis includes blood gas, lactate, CPK, CMP, and urine analysis

General Management Framework:

• MH crisis is a medical emergency requiring rapid intervention
• Dantrolene Na administration, supportive measures, and continuous monitoring in PICU
• Utilization of Malignant Hyperthermia carts and involvement of specialized hotlines

Clinical Pearls and Pitfalls:

• Early recognition is crucial.
• Proper administration of Dantrolene Na without delay
• Extended monitoring period in PICU to ensure stability

Conclusion:

• Importance of recognizing and managing MH crisis
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• Website picudoconcall.org for additional resources

References:

• Fuhrman & Zimmerman - Textbook of Pediatric Critical Care Chapter
• Malignant Hyperthermia Association of the United States
• What is MH?
• [Managing a crisis](https://www.mhaus.org/ healthcare-professionals/managing-a-crisis/)
• Rosenbaum HK, Rosenberg H. UpToDate: Malignant hyperthermia: diagnosis and management of acute crisis.