Facial Swelling

Author: The Infamous Peter Gruber, MD
(Cases initially presented by Nestor Agbayani, MD)

Case 1

45 y/o M PMHx ESRD on HD via RIJ permacath p/w facial and oral swelling x 10 days now, with difficulty swallowing 3d. VSS

  • First time having this; no change in meds
  • Visibly worse when lying flat, especially in the morning after waking up
  • Getting worse now with difficulty swallowing liquids and solids

Initial Thoughts?

  • Allergic reaction
    • but this wouldn’t be visibly so positional
  • Bradykinin reaction to ACE or ARB
    • but this also not positional and generally doesn’t give dysphagia.
    • He is also not taking ACE or ARB.
  • Infection with collection of pus (ie retro/parapharyngeal/ludwig/lemiere neck infection with pulm involvement)
    • Also doesn’t change visibly with position
  • Some mass or blockage which exerts more pressure supine than sitting up

Physical Exam:

  • No respiratory distress- speaking full sentences with normal voice
  • Facial swelling is worse on lower face
  • Neck mildly swollen
  • Opens mouth with pain
  • No swelling of space under tongue; no swelling of lips or tongue
  • Swelling of face and neck visibly decreased when sitting up

Differential:

  • Angiodema: Hereditary vs ACE vs Allergic
  • Infection: Ludwig vs Retro/Parapharyngeal vs Lemiere infection
  • Superior vena cava syndrome/central vein stenosis
  • Malignancy head neck/Pancoast tumor
  • Lymphedema
  • Myxedema 
    • but no perioral/generalized or leg swelling or other findings of hypothyroid (ie bradycardia/bradypnea/hypothermia)

Interventions:

  • Steroids, Benadryl, Pepcid, Epi IM
    • but no improvement in swelling

Studies Ordered:

  • CXR: RIJ cath in place
  • Labs unremarkable
  • ENT scope: normal airway
  • CT Face and Neck:
    • subclavian vein and svc partially visualized due to permacath and thrombus;
    • Right IJ with possible web-post thrombotic sequelae forming fibrous laminar structure;  
    • mild subQ edema within face maybe 2/2 compromised venous outflow from neck
  •  Duplex: Acute dvt in right brachial and axial veins

Diagnosis: SVC syndrome provoked by central HD catheter

Gruber’s Musings on SVC syndrome:

  • We commonly think of tumor i.e. breast or lung or lymphoma causing compression of SVC (“outside job”)
    • However, 20-40% SVC caused by indwelling central vein catheters including peripherally inserted central vein catheters and indwelling intracardiac wires which can cause central venous stenosis (inside job)
    • Indwelling catheter SVC:
      • 60% from thrombus;
      • 40% mechanical obstruction from endoluminal catheter
  • Clinical Signs/Symptoms:
    • face/neck swelling,
    • distended neck veins,
    • cough/sob, orthopnea, 
    • upper extremity swelling;
    • distended chest vein collaterals  
    • Dysphagia, headache, head plethora, and change in MS less often
    • Signs and symptoms are often positional
  • Imaging
    • Patients with high clinical suspicion for SVC syndrome should undergo imaging of the upper body and vasculature.
    • Ultrasound of the jugular, subclavian, and innominate veins can help to identify a thrombus within the vessel lumen.
    • CT or MRI of the chest and neck to localize and describe the svc obstruction
  • Management of SVC
    • Raise head of bed which decreases swelling and improves breathing
    • Steroids decrease edema and steroid responsive malignancies
    • Address underlying pathology:
      • Anticoagulate,
      • Remove indwelling device;
      • Angioplasty,
      • Biopsy/Resection chemo
  • Take home points:
    • SVC is a serious and not uncommon complication of HD patients with central vein catheters
    • Important that we consider this dx in the HD pt or pt with intracardiac device
    • Have seen a case of SVC syndrome in a pt who had ICD placed for Brugada noted on EKG but was aymptomatic.
      • In review, pt did not meet criteria for placement of ICD (i.e. no h/o syncope, no aborted sudden cardiac death or FH SCD and no EP inducible VT or VF)

Case 2:

71 y/o M PMHx HTN, CKD p/w tongue and facial swelling x 5 hours.  

  • Bit his tongue 5 days ago.  
  • No new meds. 
  • Meds: dozazosin, gabapentin, meclizine, lotrel

Physical exam:

  • Anxious  
  • BP: 179/86, HR: 99, RR: 17, SpO2: 97% on RAr 
  • Asymmetric moderate severe left tongue swelling
  • U/S of tongue: no fluid collection
  • Left cheek mildly swollen
  • Speech mildly garbled
  • No drooling; no trismus
  • Area under tongue not swollen

Initial Thoughts? and What do you want to do?

  • Asymmetric focal swelling with slow onset and no urticaria/itching or other organ involvement more typical of ACE bradykinin mediated angioedema than histamine allergic angioedema (see Table 1 below)
  • Airway cart at bedside
  • Steroids, H1 and H2 blockers with epi available if needed
Table 1

Now the tongue becomes more swollen…

  • Tongue protruding from mouth and patient unable to close mouth
  • Drooling
  • Worsening change in voice
  • ENT and anesthesia consults placed but you need to have double set-up with neck prepared in case unsuccessful attempt at oral or nasotracheal intubation
  • You need to have a clear airway plan i.e. will make one attempt at oral or nasal intubation and then go to surgical airway—
    • Otherwise too many intubation attempts (causing airway edema) and surgical airway is only done after patient arrests
  • EM resident nasotracheally intubates pt using ambuscope and ketamine
  • You look up lotrel and see it is a combo HTN med which contains ACE

What could you have given this ACE angioedema patient that may have avoided intubation?

  • TXA

Gruber’s Musings:

  • TXA 1 gm IV over 10 minutes Q4h PRN avoided intubation in a 31/31 ACE pts (doi:10.1016/j.revmed.2018.04.014)
  • TXA is front-line for ACE induced angioedema
  • TXA also has role in the crashing hereditary angioedema patient
  • TXA stops conversion of plasminogen to plasmin which is a critical step in the formation of bradykinin (see Figure 1 below)
  • There is little role for icatibant, C1 esterase inhibitors or FFP as no evidence showing they decrease the rate of airway obstruction, need for intubation and they need to be transported from outside the ED and/or take time to thaw long after the need to secure the airway
  • TXA finally finds a clear use (other than OTC in UK for menstrual bleeding)
  • Recent large (450 pts) United Kingdom RCT of topical TXA in epistaxis to reduce the need for nasal packing showed no benefit for TXA – Ann Emerg Med 2021
Figure 1

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