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
- Feedback, subscription, and reviews encouraged
- 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.
