Should a patient with a large paraesophageal/hiatal hernia, hypermobile Ehlers‑Danlos syndrome, postural orthostatic tachycardia syndrome, mast‑cell activation syndrome, and a severe psychiatric reaction to systemic steroids inform the surgical and anesthesia teams about her costochondritis?

Yes, Absolutely Inform the Surgical and Anesthesia Teams About Costochondritis

Yes, this patient must disclose her costochondritis to both surgical and anesthesia teams, as it is directly relevant to her complex medical profile involving mast cell activation syndrome (MCAS), hypermobile Ehlers-Danlos syndrome (hEDS), and POTS—all of which significantly impact perioperative risk assessment and management.

Why This Matters for Perioperative Care

The Critical Connection to MCAS

Costochondritis in this patient is not an isolated musculoskeletal complaint—it exists within a constellation of conditions that fundamentally alter perioperative risk. Patients with clonal mast cell disorders are at higher risk of severe reactions with either specific or non-specific triggers during the perioperative period 1. The presence of MCAS alongside hEDS and POTS creates a 31% prevalence of MCAS in this population, with an odds ratio of 32.46 compared to patients without these conditions 2.

The costochondritis may represent ongoing mast cell-mediated inflammation, which is particularly relevant given that:

• Mast cell activation can manifest with diverse symptoms including pain syndromes 3
• 42% of POTS patients with additional non-orthostatic symptoms (including pain) show laboratory evidence of mast cell activation 3
• Recent evidence suggests mast cell stabilization may be therapeutic for complex pain conditions in hEDS patients 4

Perioperative Anaphylaxis Risk

The anesthesia team needs complete information because perioperative immediate allergic reactions occur most commonly around induction of anaesthesia 1. With this patient's MCAS, the surgical team must be prepared for:

• Non-specific mast cell mediator release triggered by multiple perioperative drugs 1
• Potential severe reactions given her underlying mast cell disorder 1
• The fact that she cannot receive systemic steroids (standard anaphylaxis treatment adjunct) due to severe psychiatric reactions

EDS-Specific Surgical Considerations

Surgery in patients with EDS carries high complication risk, including tissue fragility, bleeding, and spontaneous perforations 5. The costochondritis may indicate:

• Chest wall tissue fragility relevant to positioning and surgical access
• Potential for pain amplification postoperatively
• Need for modified pain management strategies (avoiding medications that trigger mast cell degranulation)

What the Teams Need to Know

For the Anesthesia Team:

• Airway management considerations: Chest wall pain may affect respiratory mechanics and tolerance of positioning
• Drug selection: Avoid known mast cell degranulators (certain opioids, muscle relaxants) 1
• Monitoring: Baseline tryptase levels should be obtained for comparison if perioperative reactions occur 1
• Emergency preparedness: Epinephrine must be immediately available, with recognition that steroid administration is contraindicated in this patient 1

For the Surgical Team:

• Tissue handling: EDS requires meticulous surgical technique given connective tissue fragility 5
• Positioning: Costochondritis may limit tolerable surgical positions
• Postoperative pain management: Standard NSAID/opioid approaches may need modification given MCAS

Common Pitfalls to Avoid

Do not dismiss costochondritis as "just musculoskeletal pain" in a patient with this triad of conditions. The 2025 systematic review confirms that hEDS patients present with numerous co-morbidities including POTS, MCAS, anxiety, depression, and chronic pain syndromes 6. Each symptom may represent part of the interconnected pathophysiology.

Do not assume standard perioperative protocols apply. The mortality rate for perioperative anaphylaxis ranges from 0-1.4%, with adequate epinephrine administration being critical 1. This patient's inability to receive steroids removes a key safety net.

Documentation Requirements

The patient should provide written documentation including:

• Complete medication list (especially mast cell stabilizers like cromolyn sodium or montelukast if prescribed) 4
• Previous anesthetic experiences and any reactions
• Specific steroid reaction history with details
• Current symptom severity and functional limitations

The surgical consent process must explicitly acknowledge the increased perioperative risks associated with hEDS, including tissue fragility, bleeding complications, and the potential need for nonsurgical management if intraoperative findings suggest excessive risk 5.