Periorbital Facial Necrotizing Fasciitis in Adults: A Rare Severe Disease with Complex Diagnosis and Surgical Treatment—A New Case Report and Systematic Review

(1) Background: Necrotizing fasciitis (NF) is a severe and aggressive pathology with a rapid progression and high mortality risk. Periocular NF is a rare condition associated with a lower mortality risk but significantly higher disabling sequelae. (2) Methods: We present the case of a 67-year-old homeless patient, a victim of assault, with multiple untreated comorbidities (diabetes mellitus, cardiac conditions, and schizophrenia) and a delayed diagnosis of periocular necrotizing fasciitis. The condition showed a cyclical evolution influenced by the existing comorbidities, and the patient underwent both surgical and medical treatment with a multidisciplinary team. Additionally, we report a systematic review of cases from the literature. (3) Results: The patient’s survival outcomes were favorable; however, the sequelae were disabling, not only concerning aesthetic aspects but also due to the loss of the affected eye globe. The systematic review revealed the rarity of such cases and the peculiarities of the presented case compared to those reported in the literature up to this point. (4) Conclusions: Understanding the signs, symptoms, and predisposing factors, as well as the potential rare localizations of NF, including the periocular region, can lead to the early diagnosis and treatment with good functional and aesthetic outcomes, minimizing significant disabilities.


Introduction
Necrotizing fasciitis (NF) is a rare, progressive, and potentially life-threatening infection affecting the soft tissues.It is characterized by extensive necrosis of the fascial planes and surrounding tissues [1][2][3].While the first specific description of NF can be attributed to Hippocrates, it was not until 1871 that Jones provided the initial modern account of the condition.Subsequently, in 1952, Wilson introduced the term "necrotizing fasciitis" [2][3][4].
Similar to other infections, NF involves the interplay of a precipitating event, an infectious agent, and the host.Among the precipitating events, odontogenic infections are the most commonly associated cause, and Group A Streptococcus is the predominant infectious agent [3].The pathogenesis of NF is characterized by the invasion of bacteria into the subcutaneous tissues, rapid horizontal spread of infection along the deep fascial planes, and release of bacterial toxins, leading to tissue ischemia and liquefactive necrosis [5,6,9,10].
Diagnosing NF at an early stage can be challenging due to the absence of specific symptoms, often resulting in missed diagnoses by healthcare practitioners.Left untreated, NF can rapidly progress to septic shock and result in fatalities.Therefore, upon suspicion of NF, immediate management should include resuscitation tailored to the patient's individual needs, administration of broad-spectrum intravenous antibiotics, and early surgical intervention [6,[11][12][13][14].
We present the case of an elderly, polymorbid, homeless patient who, following a traumatic incident (assault), presented with signs and symptoms indicative of oculo-palpebral fasciitis, with the diagnosis confirmed through imaging examination (RMI).The patient underwent surgical intervention in multiple stages.Given the rarity of this condition, which is seldom encountered and documented in the medical literature, we have included this case in a systematic review that comprehensively analyzes the demographic characteristics, epidemiological trends, etiological factors, mechanisms of lesion development, associated systemic disorders, initial clinical presentations, diagnostic modalities, and treatment approaches for cases of facial necrotizing fasciitis reported over the past decade.Our objective is to advance our understanding of this intricate and severe condition while furnishing valuable insights for the benefit of clinical practice.

Case Report
We report the case of a 67-year-old homeless male patient admitted to the Plastic Surgery and Reconstructive Microsurgery Department of "St.Spiridon" Emergency Hospital in 2017.Informed consent was obtained, and an information sheet on the use of the data was delivered to the patient.Clinical, biological, imagistic, and surgical data were extracted from the patient's medical records.For publication, approval was obtained from the Hospital Ethics Committee.
According to the patient's medical history, it was documented that he had encountered a traumatic event due to an aggressive act roughly 14 days prior to admission.This incident resulted in the formation of wounds in the zygomatic region, in left upper brow area, with extension to the scalp and left parietal region.Furthermore, the patient's medical records revealed a history of diabetes, alcohol misuse, and intermittent treatment for psychiatric disorders, specifically schizophrenia.Initially, the patient was admitted to the Ear, Nose, and Throat (ENT) department due to anterior epistaxis.
The patient's overall health was significantly compromised, manifesting as pronounced edema affecting the left hemiface, particularly the upper eyebrow and eyelid, in the infraorbital regions, with extension to the occipital region, accompanied by areas of tissue necrosis.Deep-seated devitalized and infected tissues were observed in the wounds located at the left eye and upper eyelid, along with the presence of viscous, yellowish discharge.Additionally, substantial subcutaneous emphysema was noted.Furthermore, an approximately 10/7 cm area of necrosis was estimated in the vertex region of the patient's scalp (Figure 1).Subsequently, the patient was transferred to the Plastic and Reconstructive Microsurgery Clinic.Laboratory examinations revealed the presence of anemia and thrombocytosis.Emergency imaging, conducted via magnetic resonance imaging (MRI) upon the patient's admission to the clinic, confirmed the presence of subcutaneous emphysema and significant edema in the left hemiface.Remarkable symmetry in terms of fascial thickness and characteristics was observed, alongside significant alterations in the subcutaneous cellular tissue (Figure 2).Given the patient's local clinical presentation and overall medical condition, an urgent surgical intervention was necessary (CK = 2150/U/L, CRP = 43.11/mg/dL,WBC = 20.57× 10 3 /µL).Under general anesthesia with orotracheal intubation, a multidisciplinary surgical approach involving plastic surgeons and ophthalmologists was executed.The surgical procedure comprised several pivotal stages: in the initial phase, extensive surgical debridement was performed, involving the removal of all necrotic and devitalized tissues, including the enucleation of the left eyeball, fasciectomy, and parieto-occipital necrectomy.Subsequently, this intervention exposed the parietal bone (Figure 3).The excised pieces were sent for histopathological examination, confirming the diagnosis.Laboratory examinations revealed the presence of anemia and thrombocytosis.Emergency imaging, conducted via magnetic resonance imaging (MRI) upon the patient's admission to the clinic, confirmed the presence of subcutaneous emphysema and significant edema in the left hemiface.Remarkable symmetry in terms of fascial thickness and characteristics was observed, alongside significant alterations in the subcutaneous cellular tissue (Figure 2).Laboratory examinations revealed the presence of anemia and thrombocytosis.Emergency imaging, conducted via magnetic resonance imaging (MRI) upon the patient's admission to the clinic, confirmed the presence of subcutaneous emphysema and significant edema in the left hemiface.Remarkable symmetry in terms of fascial thickness and characteristics was observed, alongside significant alterations in the subcutaneous cellular tissue (Figure 2).Given the patient's local clinical presentation and overall medical condition, an urgent surgical intervention was necessary (CK = 2150/U/L, CRP = 43.11/mg/dL,WBC = 20.57× 10 3 /µL).Under general anesthesia with orotracheal intubation, a multidisciplinary surgical approach involving plastic surgeons and ophthalmologists was executed.The surgical procedure comprised several pivotal stages: in the initial phase, extensive surgical debridement was performed, involving the removal of all necrotic and devitalized tissues, including the enucleation of the left eyeball, fasciectomy, and parieto-occipital necrectomy.Subsequently, this intervention exposed the parietal bone (Figure 3).The excised pieces were sent for histopathological examination, confirming the diagnosis.Given the patient's local clinical presentation and overall medical condition, an urgent surgical intervention was necessary (CK = 2150/U/L, CRP = 43.11/mg/dL,WBC = 20.57× 10 3 /µL).Under general anesthesia with orotracheal intubation, a multidisciplinary surgical approach involving plastic surgeons and ophthalmologists was executed.The surgical procedure comprised several pivotal stages: in the initial phase, extensive surgical debridement was performed, involving the removal of all necrotic and devitalized tissues, including the enucleation of the left eyeball, fasciectomy, and parieto-occipital necrectomy.Subsequently, this intervention exposed the parietal bone (Figure 3).The excised pieces were sent for histopathological examination, confirming the diagnosis.
The Laboratory Risk Indicator for Necrotizing Fasciitis (LRINEC) scoring system was employed for diagnostic evaluation, yielding a score of 9, indicative of a high-risk condition.Additionally, wound secretions were collected for microbiological examination to identify the causative pathogen and perform an antibiotic sensitivity test in preparation for targeted antibiotic therapy.
Following the fifth day of postoperative care, during which the patient's overall health had significantly improved and achieved a state of balance, a secondary suture was performed.Subsequently, we proceeded with the coverage of the exposed parietal bone using a rotation fascial flap and a free split-thickness graft, using a similar approach as in orbital cases.
The patient remained stabilized throughout this period and received treatment for all diagnosed conditions.However, due to the patient's unstable psychiatric status, despite the treatment administered in the hospital, he exhibited psychomotor agitation and engaged in self-mutilation for a brief but consequential period.This self-harming behavior resulted in injuries to the temporal fascial flap, necessitating subsequent surgical interventions.
The postoperative outcome following the initial surgical intervention demonstrated favorable results, with effective coverage of the defects in the osseous partimosis.A satis-factory progression was observed after eradicating the infectious focus and the coverage of the exposed bone with a fascial flap, subsequently covered with a split-thickness skin graft harvested from the right thigh.However, during the hospitalization period, despite receiving appropriate psychiatric treatment, the patient experienced an episode of psychomotor agitation with self-mutilation tendencies.Consequently, he inflicted injuries to the cranial extremity, resulting in bleeding at the surgical site, subsequent ischemia, and eventual necrosis of the skin and fascial flap.The Laboratory Risk Indicator for Necrotizing Fasciitis (LRINEC) scoring system was employed for diagnostic evaluation, yielding a score of 9, indicative of a high-risk condition.Additionally, wound secretions were collected for microbiological examination to identify the causative pathogen and perform an antibiotic sensitivity test in preparation for targeted antibiotic therapy.
Following the fifth day of postoperative care, during which the patient's overall health had significantly improved and achieved a state of balance, a secondary suture was performed.Subsequently, we proceeded with the coverage of the exposed parietal bone using a rotation fascial flap and a free split-thickness graft, using a similar approach as in orbital cases.
The patient remained stabilized throughout this period and received treatment for all diagnosed conditions.However, due to the patient's unstable psychiatric status, despite the treatment administered in the hospital, he exhibited psychomotor agitation and engaged in self-mutilation for a brief but consequential period.This self-harming behavior resulted in injuries to the temporal fascial flap, necessitating subsequent surgical interventions.
The postoperative outcome following the initial surgical intervention demonstrated favorable results, with effective coverage of the defects in the osseous partimosis.A satisfactory progression was observed after eradicating the infectious focus and the coverage of the exposed bone with a fascial flap, subsequently covered with a split-thickness skin graft harvested from the right thigh.However, during the hospitalization period, despite receiving appropriate psychiatric treatment, the patient experienced an episode of psychomotor agitation with self-mutilation tendencies.Consequently, he inflicted injuries to the cranial extremity, resulting in bleeding at the surgical site, subsequent ischemia, and eventual necrosis of the skin and fascial flap.
Microbiological analysis of the patient's samples revealed a polymicrobial infection, specifically categorized as oculo-palpebral fasciitis type I. Acinetobacter cloacae with susceptibility to Tobramycin and Colistin were identified.The patient received Microbiological analysis of the patient's samples revealed a polymicrobial infection, specifically categorized as oculo-palpebral fasciitis type I. Acinetobacter cloacae with susceptibility to Tobramycin and Colistin were identified.The patient received intravenous Ceftriaxone and Colistin at a dosage of 3 million units every 24 h as part of the ongoing antibiotic therapy, according to the recommendations of the infectious disease specialist.
Simultaneously, the patient developed a Clostridium difficile infection, characterized by the presence of both type A and B strains.This infection exhibited a recurrent course, necessitating the patient's transfer to an Infectious Disease service for specialized management and treatment (Figure 4).
Simultaneously, the patient developed a Clostridium difficile infection, characterized by the presence of both type A and B strains.This infection exhibited a recurrent course, necessitating the patient's transfer to an Infectious Disease service for specialized management and treatment (Figure 4).Following the patient's psychiatric stabilization and the successful treatment of the Clostridium difficile infection, the patient returned to the Plastic Surgery clinic for the continuation and completion of surgical treatment.
Under general anesthesia with orotracheal intubation, the necrotic area (the temporal fascial flap) was excised, and the resulting defect, after bone deperiosting, was addressed through the application of a free split-thickness graft, harvested using an electrodermatome from the upper inner thigh of the right leg (Figure 5).Following the patient's psychiatric stabilization and the successful treatment of the Clostridium difficile infection, the patient returned to the Plastic Surgery clinic for the continuation and completion of surgical treatment.
Under general anesthesia with orotracheal intubation, the necrotic area (the temporal fascial flap) was excised, and the resulting defect, after bone deperiosting, was addressed through the application of a free split-thickness graft, harvested using an electrodermatome from the upper inner thigh of the right leg (Figure 5).Following extensive medical care and close monitoring, the patient's condition significantly improved, ultimately leading to their discharge after a total of 40 days of hospitalization.
A collaborative and multidisciplinary approach was adopted throughout the patient's hospitalization, ensuring continuous communication and co-ordination among various departments, including Diabetes; Ear, Nose, and Throat (ENT); Ophthalmology; Infectious Diseases; and Psychiatry.This concerted effort allowed for comprehensive and integrated care, addressing the diverse medical needs and ensuring the optimal management of the patient's condition.
Despite the ophthalmologist's recommendation for the patient to return to the Ophthalmology Department to initiate the necessary procedures for an ocular prosthesis, the patient has not returned to the hospital (Figure 6).The last follow-up examination the patient attended was conducted two years after the surgical procedure.Following extensive medical care and close monitoring, the patient's condition significantly improved, ultimately leading to their discharge after a total of 40 days of hospitalization.
A collaborative and multidisciplinary approach was adopted throughout the patient's hospitalization, ensuring continuous communication and co-ordination among various departments, including Diabetes; Ear, Nose, and Throat (ENT); Ophthalmology; Infectious Diseases; and Psychiatry.This concerted effort allowed for comprehensive and integrated care, addressing the diverse medical needs and ensuring the optimal management of the patient's condition.
Despite the ophthalmologist's recommendation for the patient to return to the Ophthalmology Department to initiate the necessary procedures for an ocular prosthesis, the patient has not returned to the hospital (Figure 6).The last follow-up examination the patient attended was conducted two years after the surgical procedure.

Systematic Review
We conducted a comprehensive systematic review in adherence to the guidelines provided by "Preferred Reporting Items for Systematic Reviews and Meta-Analyses" (PRISMA) (Figure 7).

Systematic Review
We conducted a comprehensive systematic review in adherence to the guidelines provided by "Preferred Reporting Items for Systematic Reviews and Meta-Analyses" (PRISMA) (Figure 7).
The primary objective of this review was to evaluate the existing literature on necrotizing fasciitis, particularly in the context of facial infections among adult populations.To ensure a thorough investigation, a meticulous search was performed in the PubMed database, utilizing the precise search terms "Necrotizing fasciitis", "facial", and "periorbital".The search was specifically confined to case reports published from January 2018 to May 2023.The study was not registered.

Systematic Review
We conducted a comprehensive systematic review in adherence to the guidelines provided by "Preferred Reporting Items for Systematic Reviews and Meta-Analyses" (PRISMA) (Figure 7).The primary objective of this review was to evaluate the existing literature on necrotizing fasciitis, particularly in the context of facial infections among adult populations.To ensure a thorough investigation, a meticulous search was performed in the PubMed database, utilizing the precise search terms "Necrotizing fasciitis", "facial", and "periorbital".The search was specifically confined to case reports published from January 2018 to May 2023.The study was not registered.
Initially, abstracts of the identified articles were screened, and.subsequently, full-text articles were obtained, if available, for a more detailed assessment of their relevance by the authors.In order to maintain the focus of the review, several exclusion criteria were applied.Articles that primarily focused on pediatric cases, those that addressed different aspects other than necrotizing fasciitis, those that examined necrotizing fasciitis occurring in other anatomical regions, those lacking clear localization information, or those that failed to provide sufficient patient-related details were excluded from the final analysis.
A meticulous iterative process was employed to ensure accurate data extraction, and Microsoft Excel was utilized for basic statistical analysis of the extracted data.This methodical approach allowed for a comprehensive evaluation of the available literature and enabled the synthesis of meaningful findings.

Systematic Review
During the initial search process, 82 articles were identified as potentially relevant to the study.Among these articles, one was published in a language other than English and was, subsequently, excluded from further analysis due to language barriers.This resulted in a final pool of 81 articles that were deemed eligible for assessment.
Upon careful evaluation, it was determined that 9 articles focused on pediatric cases, rendering them unsuitable for the current investigation.Additionally, 28 articles A B C D Initially, abstracts of the identified articles were screened, and.subsequently, full-text articles were obtained, if available, for a more detailed assessment of their relevance by the authors.In order to maintain the focus of the review, several exclusion criteria were applied.Articles that primarily focused on pediatric cases, those that addressed different aspects other than necrotizing fasciitis, those that examined necrotizing fasciitis occurring in other anatomical regions, those lacking clear localization information, or those that failed to provide sufficient patient-related details were excluded from the final analysis.
A meticulous iterative process was employed to ensure accurate data extraction, and Microsoft Excel was utilized for basic statistical analysis of the extracted data.This methodical approach allowed for a comprehensive evaluation of the available literature and enabled the synthesis of meaningful findings.

Systematic Review
During the initial search process, 82 articles were identified as potentially relevant to the study.Among these articles, one was published in a language other than English and was, subsequently, excluded from further analysis due to language barriers.This resulted in a final pool of 81 articles that were deemed eligible for assessment.
Upon careful evaluation, it was determined that 9 articles focused on pediatric cases, rendering them unsuitable for the current investigation.Additionally, 28 articles explored different aspects which deviated from the specific objective of this study.Moreover, 10 articles failed to provide any relevant data, thereby disqualifying them from inclusion in the analysis.
Ultimately, a total of 34 articles met the established criteria and were included in the study.These articles included case reports of adult patients with facial necrotizing fasciitis.It is worth noting that most of the included studies presented a single case report, while a subset of studies featured two or more case reports, with a total of 38 cases, including our case (Tables 1 and 2).Surgical treatment: Surgical debridement was required in all cases.In most cases, the defect was covered by skin graft (13 cases) and flaps (8 cases).In 3 cases, negative-pressure wound therapy (NWPT) was used.

Discussion
Necrotizing fasciitis is a severe condition with a rapid course and high mortality risk (35%) [14][15][16][17].Periocular involvement is rarely reported in the literature, being both uncommon and debilitating, sometimes resulting in the need for exenteration of the affected eye globe [4,11,18,19].Despite the relatively lower mortality rate in these cases (ranging from 6 to 14%), the context of this pathology can be posttraumatic or postoperative.In the reported case, the patient, homeless, fell victim to an assault [20,21].The onset of the disease is favored by the presence of comorbidities such as diabetes mellitus, malignant tumors, and immunosuppression, as reported in the literature [22,23].Similarly, in the presented case, periocular necrotizing fasciitis was observed in a patient with poor living conditions (homeless), and multiple comorbidities, including diagnosed diabetes mellitus, cardiovascular disease, and psychiatric pathology [22,24].
The patient's psychiatric condition further complicated the surgical treatment progression, as there was an episode of psychiatric distress with self-harm, leading to fascial tissue necrosis, consequently necessitating a subsequent surgical intervention to cover the exposed bone.
In addition to a clinical examination with the detection of characteristic signs and symptoms, including the presence of cutaneous emphysema, the diagnosis of periocular necrotizing fasciitis requires imaging examination, such as an MRI, to confirm or support the diagnosis [2,3,36,37].Surgical treatment is always necessary and must be combined with antibiotic treatment tailored to the sensitivity of the germs involved in the etiology of the disease.Surgical intervention must often be aggressive, with extensive devitalized tissue excision, often resulting in large soft tissue defects [4,6].In the reported case, due to the high risks of cerebral involvement, exenteration of the eye globe was necessary, an incapacitating operation for the patient [38].
In all cases from the literature, the covering of the remaining soft tissue defects postnecrectomy required various reconstructive techniques, from split-thickness skin grafts to local or distant flaps (Table 2).These techniques are always selected based on the size and location of the defect, with respect, if possible, to the aesthetic units of the face, aiming for the best possible functional and aesthetic outcome.
Given the severity of the disease, the localization, and the involvement of various anatomical regions of the face, a multidisciplinary surgical approach (involving plastic surgeons, ophthalmologists, and craniofacial surgeons) becomes necessary.In the described case, the complexity of the disease, the complications, and the patient's comorbidities required collaboration with specialists in infectious diseases and psychiatry.
Despite the reduced mortality in cases of periocular necrotizing fasciitis, the remaining sequelae of such a disease can often be significant, both aesthetically and functionally, sometimes leading to disabilities, vision impairment, or even loss of the eyes.

Conclusions
Necrotizing fasciitis, a severe condition with a high mortality risk, necessitates thorough comprehension, particularly concerning the possibility of uncommon localizations, such as facial involvement, notably periocular.Additionally, obtaining a precise and comprehensive patient history enables the identification of predisposing factors for this pathology.
Early diagnosis (both clinical and imaging) can lead to prompt intervention, minimizing invasiveness and complexity, resulting in reduced patient hospitalization time and fewer functional and aesthetic sequelaes.Successful outcomes in these instances rely heavily on interdisciplinary collaboration, ensuring the patient receives a comprehensive and integrated treatment.

Figure 2 .
Figure 2. MRI examination: (A-C) subcutaneous emphysema and edema of the left hemiface.

Figure 2 .
Figure 2. MRI examination: (A-C) subcutaneous emphysema and edema of the left hemiface.

Figure 2 .
Figure 2. MRI examination: (A-C) subcutaneous emphysema and edema of the left hemiface.

Figure 4 .
Figure 4. Postoperative evolution and the causes of flap necrosis.Following the patient's psychiatric stabilization and the successful treatment of the Clostridium difficile infection, the patient returned to the Plastic Surgery clinic for the continuation and completion of surgical treatment.

Figure 4 .
Figure 4. Postoperative evolution and the causes of flap necrosis.

Figure 4 .
Figure 4. Postoperative evolution and the causes of flap necrosis.

Figure 7 .
Figure 7. Search process and records included and excluded.

Figure 7 .
Figure 7. Search process and records included and excluded.

Table 1 .
Parameters of the cases included in the facial necrotizing fasciitis systematic review.