Locally Increased Incidence of Periorbital Necrotizing Fasciitis

A Case Series

The infection necrotizing fasciitis (“flesh-eating disease,” NF) was observed as early as the 5th century BC by Hippocrates. It received its name by Wilson in 1952 (1). The cause is mostly pathogen ingress after minor trauma/injury. The extremities—but also the groin and abdomen—are primarily affected; the face and periorbital region less so thanks to excellent blood circulation (2). Mortality in periorbital NF (PNF) is lower—10–14.4%—than for other body regions (20–35%). Because of systemic toxicity, however, severe disease courses with septicemia and even multiorgan failure have been observed (2). Timely diagnosis and rapid adequate treatment can prevent serious sequelae (vision loss, death). To date the disease has been considered as very rare (incidence 0.24/1 million/year [3]); but we recently observed an abnormal increase in the number of PNF cases in our hospital.

Methods

We report on five patients who presented with PNF to the university eye hospital at Ludwig Maximilian University of Munich between January and March 2023.

Results

The clinical findings are summarized in the Table. All patients were male. The patients had had symptoms for a maximum of three days. Clinically, all cases presented with periorbital redness as well as gangrenous skin findings originating from the medial canthus. Cutaneous injuries as a possible entry route were not noted in the patients’ medical histories. Only one patient had diabetes. On imaging, air trapping was seen (3/5) as were fluid levels in the paranasal sinuses (5/5), but no abscess formation. In all cases the findings deteriorated rapidly, with septicemia (septic shock 4/5, multiorgan failure 1/5). Microbiology confirmed in each patient group A streptococci without antibiotic resistance. All received systemic antibiotics and immediate surgical debridement with resection of necrotic tissue, with vital, still perfused skin and muscle tissue left in place. Swabs and specimens were taken for microbiologic and histopathologic testing. The affected subcutaneous tissue was flushed intraoperatively with hydrogen peroxide and gentamicin solution and sterile rubber flaps were put in place as drainage. In case of submandibular and occipital spread of infection, surgical care was delivered on an interdisciplinary basis, involving specialists in oral and maxillofacial surgery (2/5). All patients survived.

Table

Case series of periorbital necrotizing fasciitis. Clinical, laboratory, and microbiologic findings, therapy, and disease course of patients 1–5

Discussion

The increased incidence of PNF that we recently observed at our hospital seems to exceed the incidence of 0.24/1 million/year described in the literature (3). The clinical presentation and course of PNF in our patient collective resembled the cases described by Amrigh et al in a review article (2). Periorbital erythema and edema are typical symptoms, which within very few hours take on a livid discoloration, form blisters, and become gangrenous. These alarm signals require immediate investigation. If PNF is suspected, the patient should be directly admitted to a hospital providing interdisciplinary care (oral and maxillofacial surgeons and intensive care specialists). Microbiology confirmed group A streptococcus in all cases; in NF this is the most common pathogen, with Pseudomonas aeruginosa in second place (1). In accordance with recommendations from the literature, the therapeutic approach consisted of high-dose intravenous antibiotics and immediate surgical debridement. Recommended antibiotics are β-lactam antibiotics (such as penicillin or cephalosporin) and in addition clindamycin, in order to inhibit the production of streptococcal toxins. Resection of necrotic tissue is required to prevent a loss of perfusion resulting from thrombosed vessels, thereby enabling easier antibiotic flooding and lowering the bacterial load to reduce the associated secretion of exotoxins. We followed Wilson’s recommendation (1) and retained as much perfused (vital) tissue as possible in order to obviate subsequent tissue defects with resulting eyelid malformations (2). If results do not improve, repeated debridement is required; this was the case in two of our patients. We recommend reconstructing the periorbital region only after completed healing and stabilization of the patient’s general condition, unless significant lagophthalmos represents an acute risk to the eye surface.

Systemic complications as sequelae of septicemia were observed in four of our five patients. These complications explain the mortality due to PNF of 8.5–14.4% observed in the literature (2).

The World Health Organization (WHO) on 15 December 2022 reported increased incidence rates of scarlet fever and invasive group A streptococcal infections in the Netherlands, the UK, France, Sweden, and Ireland (4). In Germany, limited data are available because group A streptococcal infections are not notifiable. According to the Robert Koch-Institute (RKI), however, an increase of invasive and non-invasive group A streptococcal infections were also observed in Germany (5). The suspected cause is increased exposure in the setting of reduced immune defense after years of COVID-related isolation measures. Structural changes of the group A streptococci, tested for by analyzing specific surface antigens (M protein, emm-typing) in the National Reference Center for Streptococci at University Hospital Aachen, have not been confirmed to date. The group A streptococci identified in our patients could be assigned to the known strains.

This study is a single-center case series. Analyses at a national level are required to establish potential nationwide increases in case numbers; further microbiologic analyses are also required to determine the causes and assess possible changes in the virulence of group A streptococci.

The local increases in the incidence of PNF provide reason to raise awareness among doctors. Early diagnosis and a prompt initiation of adequate high-dose antibiotic treatment, combined with surgical debridement, lower the mortality and morbidity risk. Further analyses are, however, required to confirm an increase in the incidence and to determine possible causes.

Anna Schuh, Leonie Keidel, Birte Siegmund, Sven Otto, Siegfried Priglinger, Christoph Hintschich

Conflict of interest statement
The authors declare that no conflict of interest exists.

Manuscript received on 11 May 2022, revised version accepted on 29 June 2023.

Translated from the original German by Birte Twisselmann, PhD.

Cite this as:
Schuh A, Keidel L, Siegmund B, Otto S, Priglinger S, Hintschich C: Locally increased incidence of periorbital necrotizing fasciitis—a case series.

Dtsch Arztebl Int 2023; 120: 595–6. DOI: 10.3238/arztebl.m2023.0164

References

1. Wilson B: Necrotizing fasciitis. Am Surg 1952; 18: 416–31.

2. Amrith S, Hosdurga Pai V, Ling WW: Periorbital necrotizing fasciitis—a review. Acta Ophthalmol 2013; 91: 596–603

3. Flavahan PW, Cauchi P, Gregory ME, Foot B, Drummond SR: Incidence of periorbital necrotising fasciitis in the UK population: a BOSU study. Br J Ophthalmol 2014; 98: 1177–80

4. World Health Organization (15 December 2022). Disease Outbreak News; Increased incidence of scarlet fever and invasive Group A Streptococcus infection – multi-country. Available at: www.who.int/emergencies/disease-outbreak-news/item/2022-DON429 (last accessed on 11 July 2023).

5. Robert Koch Institut (22 December 2022). Infektionskrankheiten A-Z; Inzidenzanstieg von Gruppe-A-Streptokokken-Infektionen (z.B. Scharlach). Available at: www.rki.de/DE/Content/InfAZ/S/Scharlach/invasive_Gruppe-A-Streptokokken-Infektionen.html (last accessed on 11 July 2023).