Cold Urticaria and Other Causes of Oculofacial Postoperative Swelling: Important Prevention, Diagnostic, and Management Strategies

John R. Burroughs, MD · Richard L. Anderson, MD · James R. Patrinely, MD

Potential causes of increased postoperative swelling include rough tissue handling, poor wound placement, excessive cauterization, and lymphatic disruption. Another contributing factor is poor patient compliance with cool compresses, head elevation, and rest. Poorly controlled blood pressure and blood thinners (e.g., prescribed medications, vitamins, or herbal supplements) cause excessive bleeding and postoperative bruising and swelling. Progressive postoperative periorbital and orbital inflammation is usually considered infectious or toxoallergic in etiology. Clinical onset, signs, symptoms, and response to empiric therapy often help distinguish the diagnosis.

Surgeons should also be aware of primary acquired cold urticaria (PACU), an under-recognized and potentially serious cause of postoperative swelling. A recent case series of oculofacial plastic surgery patients who developed progressive postoperative swelling attributable to PACU elucidates the importance of diagnosing this condition.1 Cold urticaria is either primary or secondary with both localized and systemic forms, with the systemic being potentially fatal.2,3

Sample Case of Cold Urticaria

A 44-year-old woman developed right-sided frontalis weakness and eyebrow ptosis following Ramsay Hunt syndrome, which required a direct brow lift. Ten years later, she underwent a repeat right direct brow lift and a levator repair with preoperative antibiotics and antivirals given. Twenty-four hours postoperatively, the patient developed right-sided periorbital inflammation, pruritus, chemosis, and pain (Slide 1). The clinician, assuming this was an ointment reaction, stopped the patient's topical ointment and she was started on oral antihistamines. Over the next 24 hours, she remained afebrile, but the involved areas, though only mildly erythematous, became significantly more swollen and eventually spread to her ipsilateral face and neck.

Slide 1

Slide 1

The differential diagnoses considered included toxoallergic reaction, cold urticaria, and infection. She was admitted to the hospital, treated with intravenous antibiotics, antivirals, and, eventually, intravenous corticosteroids. Orbital computed tomography (CT) imaging showed profound preseptal, facial, and cervical swelling but no localized abscess (Slide 2A and Slide 2B). Her white blood cell count and differential were normal. The swelling resolved within 1 week and all cultures remained negative. Three months later, she required a ptosis revision, and by 24 hours postoperatively she again developed unilateral swelling. Her ice packs were stopped and diphenhydramine (Benadryl, Pfizer) and oral corticosteroids were given, resulting in full recovery within 3 days. She was diagnosed with cold urticaria, which was confirmed by a cold stimulation test (CST). Systemic workup was negative for any secondary causes.

Slide 2A

Slide 2A


Slide 2B

Slide 2B

Cold Urticaria

Cold urticaria syndromes, whether familial or acquired, are characterized by the development of hives and/or angioedema following cold exposure. Generally, these syndromes are benign and self-limited. The most common form is PACU, but secondary forms exist and cold urticaria is potentially life-threatening.2,3 PACU is nonfamilial and diagnosed by a history of cold-induced reaction, positive CST, and exclusion of systemic causes.2

PACU can begin at any age, but the mean age of onset is 18 years to 26 years, with a mean duration of approximately 6 years to 9 years.2,4-7 No obvious gender predilection occurs, and the natural history is variable with spontaneous recovery possible.2 Most patients, however, experience a chronic course.4

The systemic workup includes complete blood count with differential, erythrocyte sedimentation rate, serum protein electrophoresis, and cryoglobulin and cryofibrinogen testing. Tests for antinuclear antibody, rheumatoid factor, infectious mononucleosis spot test, and syphilis serology are also indicated if there is a suggestive history.3 The major cause of secondary acquired cold urticaria is cryoglobulinemia, whether primary or secondary to malignancy.2 The next most common category is infectious, which may follow mononucleosis, syphilis, or parasitic infections.

In addition to a thorough history, the CST is necessary to confirm the diagnosis of PACU. The test is performed by placing ice in a plastic bag on the patient's forearm for 1 minute. After the ice is removed, the exposed area is observed for 5 minutes for development of a confluent wheal-and-flare reaction.3 If there is no response, then the test is repeated at a different location of the forearm at increasing intervals (such as 3 minutes or 5 minutes) for as long as 10 minutes.8

Patients with an early response to the CST (< 3 minutes) generally have severe forms of cold urticaria and a high prevalence of systemic reactions. Other predictors of severity include a history of systemic reaction to cold exposure or oropharyngeal angioedema following ingestion of cold beverages. Cold urticaria is divided into three subtypes: localized urticaria, generalized reactions but without development of hypotension, and systemic reactions with hypotension and/or airway compromise.2,8

PACU Management

Patients with PACU are best managed by education, cold avoidance, and symptom relief. This condition is most frequently managed by an allergist. Secondary causes must be excluded before diagnosing PACU. Education efforts focus on cold avoidance and crisis management for severe reactions.8 Patients should be prescribed and instructed to carry injectable epinephrine.3 Additionally, they must be aware that the syndrome severity can be unpredictable and even life-threatening. Fatalities have been reported following total-body cold exposure, as well as drownings secondary to cold-induced shock during aquatic activities.3

Traditional first-line treatment has been oral antihistamines with preference for the non-sedating agents, which have shown comparable efficacy with improved compliance. These include loratadine (Claratin, Schering-Plough Pty), cetirizine HCl (Zyrtec, Pfizer), and desloratadine (Clarinex, Schering Corp.).3,9 Despite a reduction of cold-induced symptoms, antihistamines cannot prevent a severe systemic reaction during aquatic activities or extreme cold exposure.

Three simple questions should identify most individuals with this condition and would be useful to include on new patient screening paperwork or during preoperative counseling:

  1. Do you have any history of hives or swelling after contact with ice or any other cold objects?
  2. Do you have difficulty drinking cold beverages?
  3. Do you have any other history of recurrent hives, swelling, or anaphylaxis?

Patients thus identified could be referred for further evaluation and/or counseled and treated perioperatively.

Patients with known cold urticaria undergoing surgical procedures require several precautions to avoid severe reactions. First is consultation with an allergist for optimal medication prophylaxis, including antihistamines. The operating room should be kept warm and patients covered with blankets.8 IV fluid access, warmed IV fluids, and strict avoidance of cool compresses are imperative.3,8 For patients who have demonstrated an inflammatory component or are undergoing a prolonged surgical procedure, preoperative corticosteroids may be considered to inhibit mediator amplification associated with late-phase inflammatory reactions when exposed to prolonged cold.8,10,11 Otherwise, corticosteroids do not demonstrate a suppressive effect in PACU, and antihistamines remain the primary treatment. Lastly, all patients with cold urticaria should undergo surgery in a setting where appropriate resuscitative personnel and equipment are immediately available.

PACU presents with a varied natural history and unpredictable systemic reaction risk. Patients newly diagnosed with cold urticaria require a systemic workup and avoidance education. Early recognition is essential to ensure patient safety, optimal treatment, and exclusion of systemic causes. Known patients with this disorder may successfully undergo surgical procedures with appropriate consultation and safety precautions. Surgeons should be aware of this cause of progressive postoperative swelling that requires a distinctly complex management.

Other Causes and Management Strategies for Postoperative Swelling

More common causes of postoperative swelling as outlined earlier include surgical issues (e.g., surgical disruption of lymphatics, poor wound handling, and excessive cautery), an adverse reaction to topical or oral agents, and infections. Intraoperatively, a patient's head is placed at a slight elevation (reverse Trendelenburg) and, when switching from one side to the other, the operative site is covered with cool, dampened gauze, which minimizes bleeding and reduces swelling. The authors use a nonstick type of bipolar cautery (SILVERGlide, Stryker Instruments), which provides fast and precise bleeding control and avoids excessive cauterization. Incisions, whenever possible, should be placed along resting skin tension lines to lessen the risk of significant postoperative lymphatic disruption. For patients in whom it is safe to take a COX-2 inhibitor, the nonsteroidal anti-inflammatory drug celecoxib (Celebrex, Pfizer), given preoperatively and, in some cases, postoperatively, may lessen pain and swelling. Control of blood pressure during surgery is essential and patients should be reminded to stay on their antihypertensives on the day of surgery. Clonidine (Catapres, Boehringer Ingelheim Pharmaceuticals) 0.1 mg to 0.3 mg, taken orally, quickly lowers blood pressure intraoperatively for in-office surgeries. The authors also give patients a comprehensive list of medications and supplements to avoid preoperatively that have blood-thinning properties and ask them to check with their primary care physicians to ensure they may be safely discontinued prior to surgery.

Reactions to topical agents can take several days to a week before becoming apparent. Neomycin-associated dermatitis can take as long as a week, but some topical drug-induced reactions can occur more quickly. Early infections commonly present with erythema, swelling, and pain before any purulent wound discharge develops. Therefore, a high index of suspicion for an infection should be present when swelling progressively worsens after the first 48 hours postoperatively. Also, any form of purulent discharge is highly suggestive of an infectious cause, though sterile braided-suture reactions can be confounding, but these usually present no sooner than 7 days to 10 days.

Patients with seasonal allergy-related eye complaints, thyroid eye disease, and those with preoperative edema will generally swell more profusely, and often a methylprednisolone (Medrol, Pharmacia/Pfizer) dose pack immediately following surgery will adequately treat. Even mild festoons in patients will likely worsen for several months following surgery. The authors also routinely recommend vitamin C and arnica montana to patients undergoing multiple periocular procedures to lessen bruising and speed resolution. Lymphatic massage (synergy) treatments help postoperative edema, and some topicals, including Preparation H (Wyeth, Madison, NJ), often help reduce longstanding eyelid edema and festoons. For persistent cases, radiofrequency treatment to edematous areas may help. Injection of hyaluronidase into swollen areas is a temporary option patients may find helpful. Persistent chemosis may respond favorably to judicious bipolar cautery, but the authors do not recommend this unless considerable time has been given for spontaneous resolution (e.g., 6 months to 12 months) and conservative methods have failed.

Conclusion

Most causes of postoperative swelling are not serious and are easily managed. Even PACU is generally mild in presentation, but it can be easily overlooked and misdiagnosed as a localized adverse reaction to injected anesthetic, topical antibiotic ointments, or early preseptal cellulitis following eyelid or facial surgery. Rarely, this condition can be fatal and have serious underlying systemic disease, and it should be recognized by the surgeon to ensure optimal surgical results and general medical management. Three simple screening questions should identify most patients with this disorder: 1) Do you have any history of hives or swelling after contact with ice or any other cold objects?; 2) Do you have difficulty drinking cold beverages?; and, 3) Do you have any other history of recurrent hives, swelling, or anaphylaxis?

References

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  2. Wanderer AA, Grandel KE, Wasserman SI, Farr RS. Clinical characteristics of cold-induced systemic reactions in acquired cold urticaria syndromes: Recommendations for prevention of this complication and a proposal for a diagnostic classification of cold urticaria. J Allergy Clin Immunol. 1986;78:417-423.

  3. Lee CW, Sheffer AL. Primary acquired cold urticaria. Allergy Asthma Proc. 2003;24:9-12.

  4. Neittaanmaki H. Cold urticaria. Clinical findings in 220 patients. J Am Acad Dermatol. 1985;13:636-644.

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  7. Doeglas HM, Rijnten WJ, Schroder FP, et al. Cold urticaria and virus infections: A clinical and serological study in 39 patients. Br J Dermatol. 1986;114:311-318.

  8. Wanderer AA. The spectrum of cold urticaria. Immunol Allergy Clin North Am. 1995:15;701-723.

  9. Juhlin L. Inhibition of cold urticaria by desloratadine. J Dermatolog Treat. 2004;15:51-59.

  10. Johnston WE, Moss J, Philbin DM, et al. Management of cold urticaria during hypothermic cardiopulmonary bypass. N Engl J Med. 1982;306:219-221.

  11. Wanderer AA, Nuss DD, Tormey AD, Giclas PC. Urticarial leukocytoclastic vasculitis with cold urticaria. Report of a case and review of the literature. Arch Dermatol. 1983;119:145-151.