Case proves far more than just lip service.
Case Presentation:
A 60-year-old male with a past medical history of diabetes and hypertension presented to the ED with the chief complaint of lower lip swelling over two days. He denied any trauma to his lip, exposure to new foods, or history of food or drug allergies. There were no systemic symptoms including fevers, nausea, vomiting, odynophagia, dysphagia or respiratory symptoms.
On initial exam, vitals were: BP 130/91, HR 113, RR 18, SpO2 96% on room air, temp 36.9C. The patient was maintaining his airway, appeared comfortable and was in no respiratory distress. His lower lip was significantly swollen, erythematous with some inner lip ecchymosis, and slightly excoriated; however, there were no localized fluctuance (figures 1-2).
There was no involvement of his tongue, palate or posterior oropharynx. Initial labs revealed a slight leukocytosis and hyperglycemia with blood glucose in the 400s. He was given a trial of Benadryl, Solumedrol and Pepcid for a possible allergic reaction; however, after about one hour, the lip swelling appeared to have increased and spread to his lateral mouth. ENT consultation was suspicious of an infectious process.
A point of care soft tissue ultrasound was performed at the bedside, which showed a subcutaneous complex fluid with tracking and subcutaneous air (figure 3).
Bedside drainage was attempted via needle aspiration, without any gross purulent return. The patient was started on Unasyn for a suspected infectious process. His pain and swelling continued to worsen at which point a face CT scan with IV contrast was performed that demonstrated marked edema with associated soft tissue gas and non-loculated fluid highly concerning for necrotizing fasciitis for the face (figures 4-5). The patient was taken to the OR for emergent debridement.
Intraoperatively, an incision over the mucosa of the lower lip revealed murky fluid and non-viable muscle consistent with a necrotizing soft tissue infection.
Debridement of non-viable tissue included the following: the entire lower lip, bilateral oral commissures, skin of the bilateral lower face and chin, and mucosa including the anterior mandibular gingiva and gingivobuccal sulcus extending to the retromolar trigone bilaterally.
The infection tracked to the right parapharyngeal space necessitating resection of a portion of the pterygoid musculature (figure 6).
A tracheostomy was performed given the possibility of long-term intubation and for wound care considerations (figure 7). The patient was started empirically on vancomycin, piperacillin-tazobactam and clindamycin pending cultures.
The patient was subsequently taken back to the OR for further debridement revealing minimal extension of necrosis from the initial resection and PEG tube placement before proceeding with reconstruction using an anterolateral thigh free flap and a split-thickness skin graft nine days later.
He was able to be weaned from the ventilator and titrated off. Cultures within this time frame were positive for pan-sensitive Klebsiella pneumonia and the patient was narrowed to Unasyn. The patient underwent PICC line placement for an anticipated total of three weeks of intravenous antibiotic treatment.
The patient’s hospital course was complicated by a neck hematoma requiring operative control and also by wound breakdown requiring nu-gauze packing prior to discharge home with home health care. At follow-up, the patient was found to be healing appropriately with close follow-up by endocrinology for management of his diabetes mellitus figure 8).
Discussion:
Necrotizing fasciitis (NF) is a rapidly progressive soft tissue infection associated with high morbidity and mortality.[1,2] The infection can be polymicrobial (type I) and monomicrobial (type II). Type I infections are caused by aerobic and anaerobic bacteria. Type II infections are most commonly caused by group A streptococcus and staphylococcus aureus.
While NF can occur in healthy patients with no underlying medical history, there are several associated risk factors. These include immunosuppression, particularly diabetes, trauma, surgery, and obesity amongst others.
Diagnosis of NF can be particularly challenging as early signs can be difficult to distinguish from less harmful soft tissue infections such as cellulitis. It most often presents over the time course of hours and can lead to rapid tissue destruction, systemic illness, and death if not promptly recognized and intervened upon.
Clinical findings include erythema, edema, severe pain out of proportion to exam, and crepitus.[1] Skin necrosis and bullae can be seen but may not be apparent until later stages of the disease. Signs of systemic illness can also be seen such as fever, tachycardia, and hypotension.
Labs are non-specific in making the diagnosis of NF. CBC may reveal leukocytosis with left shift. Inflammatory markers such as ESR and CRP may be elevated. Lactic acidosis may be observed indicating decreased perfusion and cell death. A definitive diagnosis of NF is made surgically. Intraoperative findings include a friable fascia with a dusky-gray appearance and exudate without obvious pus.[1]
Imaging, particularly, CT with IV contrast can be helpful, however, should not delay surgical management if there is strong clinical suspicion based on exam and history. The presence of gas in soft tissues, fluid collections, and inflammatory changes are all characteristic image findings. Management of necrotizing fasciitis is prompt surgical debridement along with antibiotics and hemodynamic support. Delay in surgery is associated with increased mortality.[1]
NF most commonly affects the extremities, trunk, genitals, and perineum. These locations account for approximately 60% of all cases[2]. Head and neck involvement is seen in only 5% of cases. Within this subset, facial NF is even less common. A review by Shindo et al found only 35 cases.[3] The origin of infection can be dental, sinus, peritonsillar, or salivary gland. There has also been a case report of labio-facial NF secondary to acne.[4] Post-surgical cases have also been reported.
With regards to this patient, it was unclear what the inciting event for his infection was. There was no trauma, recent procedure, or dental infection. He did have diabetes which places him in a higher risk category.
Interestingly, the only organism isolated from intraoperative cultures was Klebsiella pneumonia. Most commonly, GAS and staph aureus are inciting organisms for facial NF although a whole host of responsible organisms have been reported including Klebsiella pneumonia.[5]
A recent review highlighted Klebsiella as an emerging cause of NF.[5] Although primarily seen in East Asia, there has been an increasing number of cases in Western countries. In a review by Rahim et al, it was also found that monomicrobial Klebsiella pneumonia NF was associated with a 40% mortality rate even with surgical intervention.[6]
It also had a propensity for rapid spread and multifocal involvement which highlights the importance of prompt diagnosis and intervention. A pitfall with facial and particularly labial necrotizing fasciitis is that it may initially be hard to differentiate from angioedema. As with this patient, there have been prior case reports of lip infections initially being misdiagnosed as angioedema.[4]
A thorough clinical history should be obtained for inciting events along with a meticulous physical exam. Evidence of trauma, poor dental hygiene, recent infection, and immunosuppression should all be taken into account. Careful monitoring should be performed and diagnostic workup should shift quickly if the patient is worsening or not responding to standard treatment for angioedema.
Imaging can be useful particularly when the patient is stable but should not delay surgery if there is already high suspicion. Ultrasound allows for quick bedside assessment for necrotizing soft tissue infection with the use of the STAFF exam. This evaluates for subcutaneous thickening, air, and fascial fluid.[7,8,9] While the diagnosis should not be hinged on this exam, it can help differentiate necrotizing fasciitis from angioedema. A CT with IV contrast can also be performed to evaluate for an infectious process.
Conclusion
This report highlights a case of labial necrotizing fasciitis, initially misdiagnosed as angioedema. This is a rare entity and requires a high index of suspicion given the severity of the disease. Early diagnosis and surgical intervention are paramount as delays are associated with worse outcomes and increased mortality. A provider, when encountered with lip swelling, must keep NF as a differential diagnosis particularly in patients with risk factors and poor response to conventional therapy.
References:
- Stevens, Dennis L., and Amy E. Bryant. “Necrotizing Soft-Tissue Infections.” New England Journal of Medicine, vol. 377, no. 23, 2017, pp. 2253–2265., doi:10.1056/nejmra1600673.
- Stevens, Dennis, and Larry Baddour. “Necrotizing Soft Tissue Infections.” UpToDate, uptodate.com/contents/necrotizing-soft-tissue-infections.
- Shindo, Maisie L., et al. “Necrotizing Fasciitis of the Face.” The Laryngoscope, vol. 107, no. 8, 1997, pp. 1071–1079., doi:10.1097/00005537-199708000-00013.
- Eltayeb, Amel Salah, et al. “A Case of Labio-Facial Necrotizing Fasciitis Complicating Acne.” BMC Research Notes, vol. 9, no. 1, 2016, doi:10.1186/s13104-016-2041-3.
- Cheng, Nai-Chen, et al. “Recent Trend of Necrotizing Fasciitis in Taiwan: Focus on Monomicrobial Klebsiella Pneumoniae Necrotizing Fasciitis.” Clinical Infectious Diseases, vol. 55, no. 7, 2012, pp. 930–939., doi:10.1093/cid/cis565.
- Rahim, G.r., et al. “Monomicrobial Klebsiella Pneumoniae Necrotizing Fasciitis: an Emerging Life-Threatening Entity.” Clinical Microbiology and Infection, vol. 25, no. 3, 2019, pp. 316–323., doi:10.1016/j.cmi.2018.05.008.
- Castleberg, Erik, et al. “Diagnosis of Necrotizing Faciitis with Bedside Ultrasound: the STAFF Exam.” Western Journal of Emergency Medicine, vol. 15, no. 1, 17 Oct. 2013, pp. 111–113., doi:10.5811/westjem.2013.8.18303.
- Fozard, Jessica, et al. “Sonographic Exploration for Fascial Exploration (SEFE) in Necrotizing Fasciitis: a Case Report.” The Ultrasound Journal, vol. 12, no. 1, 2020, doi:10.1186/s13089-020-00168-5.
- Shyy, William, et al. “Sonographic Findings in Necrotizing Fasciitis.” Journal of Ultrasound in Medicine, vol. 35, no. 10, 2016, pp. 2273–2277., doi:10.7863/ultra.15.12068.