Angio odema 

10 NOV 2012

A Case of Upper Airway Obstruction- Angioneurotic Edema

 

Authors:         Dr Shafi Ejas.T.M, Dr Yoosuf Viju

Moderator       Dr Jiju Joseph

HOD                Dr Venugopalan .P.P

 

A 40/F was brought in to the Emergency Department with complaints of sudden onset fever, sore throat and respiratory distress

General Impression: Sick, anxious with facial swelling and minimal stridor, following which she was shifted to the Resuscitation bay

Primary Survey

Airway    :  Not maintainable, anticipated difficult intubation

                  Swollen tongue, edematous mucosa.

                  Rapid sequence Intubation (Bougie assisted) with

                   ETT size 7

Breathing   : Labored

Circulation  : CRT < 2 sec.

                        Monitor: Tachycardia, rate > 200

Disability        : Response to verbal commands. Pupils PEARL

Exposure        :  Facial Puffiness, Dry skin, scaly maculopapular rash lower extremities

Vitals               :  BP 220/140mm Hg. Temp 100F Spo2 80% on RA

Initial Medications administered in Emergency Department

1)      Inj Hydrocortisone 100mg IV stat

2)      Inj Pantoprazole 40mg IV stat

3)      Inj Adenosine 6mg IV Push

4)      Inj Etomidate 20mg IV stat  (RSI)

5)      Inj Atracurium 25mg IV stat  (RSI)

6)      Inj Midazolam 2mg IV stat

7)      Inj Vecuronium 4mg IV stat ( Post RSI after tube confirmation)

8)      Inj Ceftriaxone 1.5 g stat ATD

Post Intubation Vitals: HR 200. BP 200/100. Spo2 100 %.

SECONDARY SURVEY

Signs and symptoms:  Underwent usual session of Hemodialysis 8 hours back and was discharged, developed high grade fever with throat pain and difficulty in swallowing.

Allergies:        Peanut allergy

Medications: MMF, Oral steroids

Past History: CKD stage V on RRT .Active SLE past 5 years, on treatment for Lupus

                        nephritis since 3 years. Neurogenic Bladder on CSIC, Recurrent UTI and

                        Background of recurrent attacks of angioneurotic edema, seizure disorder

                        (This history was unavailable during ED presentation)

Head to Toe Examination:     

Facial Puffiness

Pallor

Swollen tongue and edematous mucosa

Marked precordial pulsatio

Coarse crepitations B/L lung fields

 Abdomen: distended

 Extremities: B/L edema with maculopapular skin rashes

  Nervous system: Confused. Not fully assessed

Point of Care Ultrasound       :  Multiple B Lines bilateral, Moderate LV dysfunction

                                                   No pneumothorax.

                                                   IVC: Normal

Investigations:  Hb 8.9. TC 14.300.Neutrphils 80%

                            Urine Pus Cells > 1000

                            K+ 4, Na+ 135, Ca -9mg/dl

                            Platelets: 2, 00,000

ED Management   :  Endotracheal Intubation and mechanical ventilation

                                     Hemodynamic stabilization

                                      Initiation of antibiotics and supportive therapy

ED Disposition    :  Medical ICU admission.

                                Cross consultation: Nephrology, Critical Care

Differential Diagnosis        :   Angioneurotic Edema

                                                     Retro Pharyngeal Abscess

                                                     Anaphylaxis

                                                     Urosepsis

Discussion and Course in Hospital   : 

 This 40/F who is a known case of Chronic Kidney Disease on RRT since 2 years, has been having active SLE since 5 years and on treatment for Lupus Nephritis since 3 years. She was weaned of immuno suppression due to fairly advanced kidney disease for which she was continued on Dialysis. She had a background of recurrent soft tissue infections and urosepsis which necessitated minimization of her immuno suppression therapy. She also had recurrent episodes of angioneurotic edema.

She was presently admitted with acute onset facial puffiness and fever along with severe respiratory distress. She had features of Angioneurotic edema and needed intubation and mechanical ventilation. She was started on plasma infusions and IV dexamethasone combined with continuation of dialysis. She needed prolonged mechanical ventilation because of her non resolving angioneurotic edema but she was successfully weaned off ventilator after 5 days. She had features of severe pulmonary edema while in hospital and needed aggressive dialysis.5 days later developed features of facial cellulites with secondary fungal infection which was managed with appropriate therapy. She had low C3 levels and a Rheumatology consultation was taken and it was decided to enhance her immuno suppression. She was transferred out to ward and discharged after one week.

Final Diagnosis       :   Angioneurotic Edema with Urosepsis

Angioneurotic Edema

Dr. William Osler remarked in 1888 that some cases may have a hereditary basis; he coined the term "hereditary angio-neurotic edema"

The link with C1 esterase inhibitor deficiency was proved in 1963.

-    Rapid swelling of  dermis , mucosa and sub mucosal tissues

-   Can be Hereditary or acquired

-   Medical Emergency due to airway obstruction. Epinephrine may be life saving in allergic cases. Hereditary angioneurotic edema does not respond to epinephrine.

-    ACE inhibitors is a common medication which can precipitate this condition

-    Bradykinin, is believed to be the predominant mediator leading to increased vascular permeability and vasodilatation that induces typical angioedema attacks

-    Diagnosis is by history and clinical picture , (renal function, angiotensin  II receptor blocker

-    Hereditary angioedema does not respond to antihistamines, corticosteroids, or epinephrine. Acute treatment consists of C1-INH concentrate from donor blood, which must be administered intravenously. In an emergency, fresh frozen blood plasma, which also contains C1-INH, can also be used as was done for this patient.

Lessons Learnt:

-     Angioneurotic Edema may present as a life threatening emergency which requires aggressive Emergency department management and stabilization

-     Full background history may not be initially available in the emergency scenario. Hence high index of suspicion and systematic approach is needed

-     When the diagnosis is unclear on presentation, always support ABC’s.

References:

 

      Angioedema: role of bradykinin".

5)      Online references (Wikipedia – angioedema)

 

 

 

 

 

                        :