Abstract
Background: Periocular necrotizing fasciitis (PNF) is a rare but life-threatening emergency that requires immediate recognition, as delayed diagnosis can worsen patient outcomes. To address this critical issue, we conducted the largest and most comprehensive systematic review to date, providing valuable insights into the diagnosis and treatment of PNF to improve clinical practice and patient prognosis. Methods: A search on Pubmed, Scopus, Embase, and WOS from January 2013 to August 2024 was performed. Only the cases of NF affecting the periocular region were included with no age limitations. Article selection and data extraction were performed independently by two investigators to avoid bias. Bias on individual studies is low as they represent case reports or case series, and publication bias is partially addressed including all the large case series even if no individual data could be retrieved. Results: The cohort included a total of 183 patients with PNF, with detailed patient-specific data for 107 individuals and only aggregated data for another 76. The average age at diagnosis was 54.2 years, and females constituted 44% of the population sample. Notably, 49.6% of the patients were immunocompromised. Streptococcus pyogenes was the predominant causative organism, identified in 79.8% of the cases. Most infections were unilateral (72.1%) without extension beyond the periocular area (54.7%). Most patients (89.6%) underwent surgical debridement alongside intravenous antibiotics. Septic shock occurred in 26.8% of the patients, and the overall mortality rate was 4.9%. Visual acuity was unaffected in 67.5% of the patients, though 18.2% progressed to blindness on the affected side. Reconstructive efforts predominantly involved skin grafting, both free and local pedunculate flaps as well as secondary healing in some instances. Conclusions: This systematic review summarizes the understanding of periocular necrotizing fasciitis' (PNF) demographic trends, clinical manifestations, causative pathogens, and patient outcomes. Vigilance for PNF should be heightened when the clinical assessment of the patient's eyelids reveals rapidly spreading edema and induration, subcutaneous emphysema, or necrotic bullae and/or eschar. Prompt identification and expedited intervention, including debridement and targeted antibiotic therapy, critically influence prognosis. Despite optimal management, patients may still suffer from significant aesthetic impairment, severe complications such as vision loss, or death due to septic shock.