What is the appropriate workup for a 22-year-old female with a history of Ehlers-Danlos Syndrome (EDS) presenting to the Emergency Room (ER) with a complaint of a suspected rib dislocation on the right side?

Emergency Room Workup for Suspected Rib Dislocation in a Patient with Ehlers-Danlos Syndrome

The initial emergency room workup for a 22-year-old female with Ehlers-Danlos syndrome presenting with a suspected rib dislocation should include a standard posteroanterior chest radiograph as the first diagnostic test, followed by a targeted physical examination to assess for joint hypermobility, skin findings, and cardiovascular stability. 1

Initial Assessment

Physical Examination

• Assess vital signs with particular attention to:
  • Hemodynamic stability (blood pressure, heart rate)
  • Respiratory status (respiratory rate, oxygen saturation)
  • Signs of pneumothorax or hemothorax (decreased breath sounds, tracheal deviation)
• Perform a focused examination of the chest wall:
  • Locate the area of pain and tenderness
  • Check for visible or palpable deformity
  • Assess for crepitus or instability
  • Evaluate for paradoxical chest wall movement (flail segment)
• Document Beighton score to assess joint hypermobility (score ≥5 indicates hypermobility) 2
• Examine skin for hyperextensibility, fragility, and bruising characteristic of EDS 2

Imaging Studies

1. Standard Posteroanterior (PA) Chest Radiograph

   • First-line imaging study for suspected rib dislocation 1
   • Can detect complications more important than the rib dislocation itself, such as pneumothorax, hemothorax, or flail chest 1

2. Dedicated Rib Views

   • Not routinely recommended as they rarely change management (in only 0.23% of cases) 1
   • May prolong report turnaround time without significant clinical benefit 1

3. CT Chest

   • Consider if:
     • Chest radiograph is abnormal or inconclusive
     • Patient has severe pain disproportionate to radiographic findings
     • There is concern for associated injuries (vascular or visceral)
     • Patient shows signs of respiratory compromise
   • CT has higher sensitivity for detecting rib fractures and dislocations but does not necessarily alter management in uncomplicated cases 1

Special Considerations for EDS Patients

Cardiovascular Assessment

• Obtain baseline ECG to evaluate for arrhythmias
• Consider echocardiogram if:
  • Patient has known aortic abnormalities
  • Patient has vascular type EDS (type IV)
  • There are signs of hemodynamic instability 2

Vascular Evaluation

• For patients with vascular type EDS (type IV), maintain high suspicion for arterial complications 1, 2
• Consider CT angiography if there are signs of vascular compromise or if the patient has vascular type EDS 2

Pain Management

• Provide appropriate analgesia:
  • NSAIDs (if no contraindications)
  • Acetaminophen
  • Consider muscle relaxants for associated muscle spasm
  • Opioids may be necessary for severe pain 3
• Apply ice to reduce inflammation and pain

Management Algorithm

1. If normal chest radiograph and stable patient:

   • Provide pain management
   • Educate on breathing exercises
   • Arrange follow-up with primary care or EDS specialist
   • Discharge with precautions

2. If abnormal chest radiograph or unstable patient:

   • Proceed with CT chest
   • Consult appropriate specialist (thoracic surgery, vascular surgery)
   • Consider admission for observation or intervention

3. If signs of pneumothorax, hemothorax, or vascular compromise:

   • Immediate intervention (chest tube placement, vascular surgery consultation)
   • Admission to appropriate level of care

Pitfalls and Caveats

• Tissue Fragility: Patients with EDS have increased tissue fragility, which may complicate procedures and interventions. Handle tissues gently during examination and procedures 1, 2

• Misdiagnosis: Rib dislocations may be misdiagnosed as simple musculoskeletal pain in EDS patients. Maintain high clinical suspicion even with normal initial imaging 4

• Vascular Complications: Patients with vascular type EDS are at risk for arterial rupture. Even minor trauma can lead to significant vascular complications 1, 2

• Pain Management Challenges: EDS patients often have chronic pain and may require individualized pain management approaches 3

• Cardiac Involvement: While earlier studies suggested high rates of cardiac abnormalities in EDS, more recent evidence indicates cardiac involvement may be less common than previously thought 5

By following this structured approach, emergency physicians can effectively evaluate and manage suspected rib dislocations in patients with Ehlers-Danlos syndrome while remaining vigilant for potential complications specific to this connective tissue disorder.