Cervical Necrotizing Fasciitis: A Silent and Unforeseen Killer in an Immunocompetent and Young Patient

Abstract

Background and Clinical Significance: Necrotizing fasciitis (NF) is a fulminant, life-threatening polymicrobial infection of the soft tissues, characterized by the progressive and widespread necrosis of the subcutaneous tissues and fascial planes. Although the cervical region is an infrequent site of involvement, cervical necrotizing fasciitis (CNF) is distinguished by its rapid anatomical spread, severe systemic complications, and significant mortality rates. Case Presentation: This report details the fatal clinical course of a 37-year-old immunocompetent male with no relevant active comorbidities. The patient initially presented with insidious, non-specific symptoms clinically interpreted as an uncomplicated upper respiratory tract infection/acute pharyngitis, including pharyngodynia, hoarseness, fever, lateral cervical lymphadenopathy, and catarrhal secretion. Within a brief period, his clinical status underwent rapid deterioration, with progressive cervical swelling, dysphonia, swallowing impairment, dyspnoea, shock, and subsequent multi-organ dysfunction syndrome. A total-body computed tomography scan revealed lateral cervical subcutaneous emphysema, a small pneumomediastinum, bilateral pleural effusion and bilateral pulmonary parenchymal consolidations. Conclusions: A comprehensive forensic examination revealed swelling and brownish-green discoloration of the right cervical region, palpable crepitus, and extensive necrosis of the cervical soft tissues, with involvement of the deep fascial layers and regional musculature. Histological examination confirmed massive cervical soft tissue necrosis associated with inflammatory infiltration and air bubbles. Multifocal acute pneumonia was also documented. This case underscores the importance of a meticulous examination of the cervical region during autopsy in suspected infection-related fatalities. Clinically, it highlights the diagnostic challenges posed by the deceptive and paucisymptomatic onset of CNF.

1. Introduction

Necrotizing fasciitis (NF) is a rapidly progressing and potentially fatal polymicrobial bacterial infection of the soft tissues, characterized by necrosis of fascial connective layers and subcutaneous tissue [1,2,3]. This infective-necrotic process can involve the overlying skin and underlying muscles, and swiftly lead to systemic complications [4,5]. The anatomical regions typically affected by this infection include the abdomen, groin, perineum, and extremities [6]. However, the same process can also localize to the soft tissues of the head and neck, in which case it is termed cervical or cranio-cervical necrotizing fasciitis [7].

A 2023 review indicates that the incidence of necrotizing fasciitis varies geographically from 0.2 to 6.9 cases per 100,000 in the overall population annually, with a peak incidence of 15.5 per 100,000 per year reported in Thailand [8]. According to a 2018 review, necrotizing fasciitis in the United States demonstrated a mortality rate of 4.8 per 1,000,000 in the overall population per year over the decade from 2007 to 2017 [4]. Overall mortality rates range from 25–35% [9,10] in some studies, but can span from 20% to 80% in others [5].

Specifically, a 2022 review by V. Gupta et al. [11] reports that the mortality rate for cervical necrotizing fasciitis fluctuates between 4% and 50%, influenced by microbial virulence and pre-existing comorbidities (for example previous cardiovascular pathology or respiratory diseases such as chronic bronchitis, emphysema). Furthermore, a 2022 study by B. Sizer et al. [1] notes an escalation of this mortality rate to as high as 70% following the development of descending mediastinitis.

Cervical necrotizing fasciitis accounts for 1% to 10% of all necrotizing fasciitis cases [4,9,11,12]. Its comparative rarity is likely attributable to the rich vascular supply of the cranio-cervical region [4,11]. Profuse cranio-cervical vascularization optimizes leukocyte recruitment and tissue oxygenation, thereby suppressing anaerobic pathogenicity and mitigating the propensity for necrotizing fasciitis. The infection typically originates from odontogenic or pharyngeal sources and is commonly sustained by coexisting aerobic and anaerobic bacteria [3,6,11]. A notable characteristic of this condition is its propensity for rapid development of severe systemic complications, including airway compromise, descending mediastinitis, or sepsis with multi-organ failure, thereby demonstrating its high lethality [6,11]. Even with adequate treatment, mortality averages between 25% and 35% [10], but can exceed 70% in cases complicated by descending mediastinitis or multi-organ sepsis [1,5].

We present the fatal case of a 37-year-old immunocompetent male with no relevant active comorbidities, in whom cervical necrotizing fasciitis developed after an initially non-specific upper airway clinical presentation. The case is discussed with particular attention to autopsy findings and medico-legal issues related to diagnostic delay and causal assessment.

2. Case Description

The A 37-year-old male, in good general health and with no known active comorbidities or immunosuppressive conditions, developed acute symptoms consistent with an upper respiratory tract infection, including pharyngodynia, hoarseness, lateral cervical lymphadenopathy, fever up to 38 °C and catarrhal secretion. The initial working diagnosis was uncomplicated acute pharyngitis/upper respiratory tract infection, and no clinical suspicion of deep neck infection or cervical necrotizing fasciitis was raised during the first out-of-hospital medical assessment.

Initial management was provided at home by his general practitioner and consisted of oral paracetamol, local anti-inflammatory drugs, antibiotic therapy with amoxicillin/clavulanic acid, systemic corticosteroid therapy, and mucolytic aerosol therapy.

Within the following 24 h, a rapid clinical deterioration was observed, characterized by increasing cervical swelling, dysphonia, and swallowing impairment. Due to difficulty swallowing the oral antibiotic therapy, the patient subsequently contacted the emergency on-call medical service. Oral amoxicillin/clavulanic acid was discontinued and replaced with intramuscular ceftriaxone.

The following day, a modest improvement in general symptoms was noted; however, in the afternoon, there was a new deterioration with the appearance of chest and epigastric pain, profuse sweating, paraesthesia of the limbs and dyspnoea. He then contacted the emergency medical service a second time, reporting his symptoms and clinical progression. He received a visit from the on-call physician, who confirmed the ongoing therapy.

A few hours later, his wife observed an acute alteration in his mental status, with motor incoordination, psychomotor agitation, and confusion, necessitating the intervention of emergency medical services. Upon pre-hospital evaluation, the patient presented with hypotension (Blood Pressure 90/60 mmHg), sinus tachycardia (up to 170 bpm), tachypnea, and cold sweats, while maintaining alertness and verbal interaction.

During transport to the hospital, the patient experienced cardiorespiratory arrest with asystole. Advanced Life Support (ALS) manoeuvres were immediately initiated, leading to the restoration of spontaneous circulation after approximately 10 min. Upon arrival at the Emergency Department, the patient was in severe shock and intubated, exhibiting severe metabolic acidosis (pH 6.9), hypercapnia (pCO₂ 124 mmHg), and hypoxemia (pO₂ 69.4 mmHg), blood lactate level was 18 mmol/L, HCO₃ was 12.8 mEq/L, and the admission temperature was 38.3 °C.

A total body CT scan revealed lateral cervical subcutaneous emphysema (more prominent on the right), a small pneumomediastinum, bilateral pleural effusion, and bilateral pulmonary parenchymal consolidations.

The clinical picture rapidly progressed to multi-organ failure with a comatose state (Glasgow Coma Scale 3).

Blood cultures, as well as molecular nasopharyngeal swabs for respiratory viruses and Staphylococcus aureus, were performed during the first day of admission and yielded negative results. Therefore, no causative microorganism was identified by the available microbiological investigations.

The patient was treated in the Intensive Care Unit with advanced life-support measures, (including mechanical ventilation, hemodynamic support, and broad-spectrum antimicrobial therapy, i.e., amoxicillin and clavulanic acid). Despite these interventions, he developed irreversible cardiac arrest and died approximately 24 h after hospital admission.

An autopsy was performed to ascertain the cause of death and to investigate any potential medical liability.

The external examination revealed significant swelling of the right lateral cervical region, measuring approximately 8 × 4 cm. This area presented with increased consistency, palpable crepitus, brownish-green discoloration (Figure 1A), and a distorted cervical profile. The contralateral region exhibited less pronounced swelling.

Upon dissection of the neck, the subcutaneous tissue in the right lateral cervical area, at the level of the larynx, showed brownish-green discoloration and a foul odour. The deep cervical fascia and adjacent muscles, particularly the sternocleidomastoid, were necrotic (Figure 1B). Modest swelling was also noted on the contralateral side. The laryngeal mucosa showed hyperemic streaks. The mediastinal soft tissues showed macroscopic changes consistent with inflammatory involvement, including the presence of a whitish purulent fluid, supporting the diagnosis of descending mediastinitis. The pulmonary parenchyma exhibited consolidations with positive hydrostatic docimasia, diffuse congestion, and fibrinous deposits at the lung bases.

A portion of the neck was collected and fixed in formalin, and subsequent analysis revealed a brownish-black area within the tissues adjacent to the right neurovascular bundle (Figure 1C).

Microscopic examination revealed multiple foci of acute pneumonia, characterized by a predominantly neutrophilic inflammatory infiltrate, associated with alveolar edema and vascular congestion (Figure 2). In the right cervical region, massive soft tissue necrosis was observed, extending to the fascia, musculature, and subcutaneous tissue. An inflammatory cell infiltrate, primarily composed of lymphocytes and neutrophils (Figure 3), along with areas of haemorrhage and air bubbles, was also present. Overall, the cervical and mediastinal findings were consistent with cervical necrotizing fasciitis complicated by descending mediastinal involvement.

Table 1. Summary of clinical and autopsy data.

General data	36-year-old male, in good general health and with no known comorbidities
Initial symptoms	pharyngitis, hoarseness, lateral cervical lymphadenopathy, fever and catarrhal secretion
Local lesion	cervical swelling and swallowing impairment
Cause of death	Multi-organ dysfunction syndrome (MODS) complicating cervical necrotizing fasciitis
Macroscopic findings	An 8 × 4 cm firm swelling was noted in the right cervical area, characterized by palpable crepitus, brownish-green skin changes, and visible deformity of the neck profile.
Microscopic findings	multifocal acute pneumonia and massive soft tissue necrosis of the right cervical region, with involvement of the fascia, musculature, and subcutaneous tissue.

summarizes the key information.

3. Discussion

Necrotizing fasciitis is a rapidly progressive and highly lethal infection, characterized by the necrotic involvement of deep soft tissues, particularly the superficial and deep fascia, with potential extension to the subcutaneous tissue, muscle, and, in severe cases, the overlying epithelium1. From an anatomopathological perspective, this condition presents as a polymicrobial necrotizing cellulitis (typically sustained by a consortium of aerobic and anaerobic bacteria), where microbial proliferation and toxin release lead to vascular thrombosis, tissue ischemia, and massive necrosis [3,6,12].

From a diagnostic standpoint, necrotizing fasciitis often presents with non-specific signs in its initial phases: swelling, erythema, pain disproportionate to the visible lesion, fever, and systemic symptoms, making immediate clinical diagnosis challenging [8,9]. Early use of imaging diagnostics (CT or MRI) is crucial to document signs of subcutaneous emphysema, fascial necrosis, or deep fluid collections [8]. Treatment is a surgical emergency and is based on three pillars: early aggressive debridement, broad-spectrum antibiotic therapy, and intensive supportive care [4,8,12].

The case we present involves a 37-year-old immunocompetent man with no relevant active comorbidities, who developed symptoms initially interpreted as an uncomplicated upper respiratory tract infection and was managed at home. Within a few days, the clinical picture evolved into cervical necrotizing fasciitis complicated by descending mediastinitis, multifocal acute pneumonia, septic shock, and multi-organ dysfunction syndrome. Autopsy findings confirmed massive necrosis of the cervical soft tissues, with deep fascial and muscular involvement, while histological examination documented multifocal acute pneumonia. The available microbiological investigations did not identify the causative microorganism.

In the present case, the precise primary source of infection could not be established with certainty. The clinical onset, characterized by pharyngodynia, hoarseness, fever, cervical lymphadenopathy, and catarrhal secretion, together with the autopsy finding of laryngeal mucosal hyperemia, makes an upper aerodigestive tract origin plausible. However, no definite odontogenic focus was documented, and the pharyngeal origin should therefore be regarded as presumptive rather than proven.

The infection likely spread from the cervical region along the deep cervical fascial planes. This anatomical pathway may allow caudal extension toward the mediastinum through the retropharyngeal and retroesophageal spaces. Subcutaneous emphysema of the neck and pneumomediastinum were documented by CT, and autopsy findings supported descending mediastinal involvement. Therefore, the overall picture was consistent with cervical necrotizing fasciitis complicated by descending mediastinitis.

The pulmonary findings should be interpreted in this context. The histological evidence of multifocal acute pneumonia, together with the clinical course characterized by septic shock and multi-organ dysfunction, supports the role of pulmonary involvement in the fatal outcome. Nevertheless, aspiration during clinical deterioration or cardiopulmonary resuscitation may have contributed to the pulmonary findings and cannot be completely excluded.

The autopsy was requested by the Judicial Authority to evaluate the possible existence of medical liability, particularly in light of the initial medical management. From a medico-legal perspective, the intervention by the on-call physicians (at two different times) was not entirely correct, considering the patient’s presenting symptoms and their evolution. Specifically, we identified a delay in transfer to a hospital setting that resulted in a diagnostic and therapeutic delay. This management shortfall is particularly relevant given the rapid progression of the pathology, whose course, as is well-known, necessitates urgent assessment and intensive hospital treatment. However, there was insufficient evidence to conclude that optimal medical care would have unequivocally averted the fatality. In other words, the presence of a causal relationship between the identified management deficiencies and the patient’s death was not demonstrated, in accordance with the stringent Italian criminal law standard of a high probability verging on certainty. We concluded that, even if the patient had been transferred to the hospital immediately, survival could not have been guaranteed with any reasonable degree of certainty.

Although cervical necrotizing fasciitis is a well-documented diagnostic pitfall, the significance of this case lies in the extreme rarity of this specific anatomical localization, coupled with its fulminant progression toward a fatal outcome. Rather, its value resides in the forensic documentation of a rapidly fatal case in a young immunocompetent patient and in the medico-legal assessment of diagnostic delay, causal uncertainty, and counterfactual reasoning in a fulminant infectious disease.

In comparison with available scientific literature, our case falls within the group with unfavourable outcomes. A similar outcome is reported by Bono et al. [13], who describe a fatal case of cervical necrotizing fasciitis in a 60-year-old male with a neglected peritonsillar abscess and no comorbidities. Another lethal case is documented by Antunes et al. [14], detailing an extensive cervical necrotizing fasciitis of odontogenic origin in a 45-year-old female with uncontrolled diabetes mellitus. Outside the cervical region, Johnson et al. [15] illustrate a deadly case of necrotizing fasciitis in a 40-year-old male presenting with pain and redness of the left lower limb in the absence of prior injuries.

Henrich et al. [16] document the first known death of a patient with a fully functioning immune system. The case involved a 66-year-old Black male who had no prior history of immune deficiency and no obvious point of entry for the infection. Even with immediate, extensive surgery to remove infected tissue and the administration of antibiotics, the patient died 30 h of hospital arrival from multiple organ failure secondary to uncontrollable systemic infection.

A study by Fais et al. [17] evaluates the literature regarding post-mortem diagnoses of necrotizing fasciitis and introduces a series of seven cases with a fatal outcome. The cohort consisted of three males and four females, aged between 31 and 50 years. In five instances, the clinical history indicated a prior trauma; however, establishing a diagnosis proved highly challenging across all cases. The clinical features clearly attributable to NF emerged almost simultaneously with the symptoms of septic shock, following a fulminant course toward a fatal outcome within 24 h. Furthermore, Type I necrotizing fasciitis was identified in only two of the seven subjects. According to the authors, forensic experts must reconstruct the clinical course and assess the causal link between any medical error and the fatal outcome through counterfactual reasoning. This evaluation of professional conduct must adopt an ex-ante perspective. Given the diagnostic complexity of NF, scientific caution is paramount, as pathogenetic uncertainties or lack of evidence may prevent definitive answers to legal inquiries.

In our case, although the on-call medical service’s intervention was primarily oriented towards pharmacological support, the lack of immediate hospitalization likely contributed to a diagnostic and therapeutic delay which, in a rapidly progressing infection, can be fatal. In this regard, our observation aligns with the findings reported by B. Sizer et al. [1] and V. Gupta et al. [11], who identify surgical timeliness as the primary positive prognostic factor in patients with cervical necrotizing fasciitis. Similarly, Cariati et al. [18] highlight the essential role of early diagnosis and prompt surgical drainage in improving patient prognosis. In parallel, Hua and Friedlander [10] emphasize the need to maintain a high degree of clinical suspicion to ensure early diagnosis and treatment.

A limitation of this study is that antemortem cultures failed to isolate the responsible pathogen and postmortem microbiological sampling was not performed. However, any postmortem sampling would have introduced significant interpretative bias.

From a medico-legal perspective, this case underscores the critical importance of a meticulous post-mortem examination of the cervical region when investigating such a cause of death. Heightened attention should be paid to any greenish discoloration of the overlying skin, and extreme caution should be exercised during neck dissection [19]. Careful external inspection and cervical dissection revealed key macroscopic findings consistent with the pathology. Further macroscopic assessment after formalin fixation and detailed histological analysis were indispensable for fully evaluating the extent and progression of the pathological changes.

In the clinical setting, the frequent non-specific early presentation of cervical necrotizing fasciitis requires a high index of suspicion, especially when upper airway symptoms are associated with progressive cervical swelling, dysphonia, dysphagia, dyspnoea, or systemic deterioration. In such circumstances, prompt hospital referral, urgent imaging, broad-spectrum antimicrobial therapy, airway evaluation, and early surgical assessment are essential. From a forensic perspective, careful external examination and meticulous cervical and mediastinal dissection remain crucial to correctly identify the cause of death and reconstruct the pathological progression.