Surgical outcomes in adults with purpura fulminans: a systematic review and patient-level meta-synthesis

Abstract

Background Cutaneous manifestations of purpura fulminans (PF) present many challenges for clinicians and surgeons. In a state of septic shock complicated by limb ischemia, surgical interventions are necessary to control the pathological cascade and improve patient outcomes. The objective of this article was to report etiologies and surgical outcomes associated with cutaneous manifestations in adults. Methods This systematic review and meta-analysis compared 190 adult patients with etiologies, signs and symptoms, and surgical outcomes associated with cutaneous manifestations of PF. The PubMed, EMBASE, Cochrane Library, Web of Science, and Scopus databases were systematically and independently searched. Patient and clinical characteristics, surgical interventions, outcomes, and complications were recorded. Results Seventy-nine studies were eligible for the systematic review, and 77 were eligible for meta-analysis using Preferred Reporting Items for Systematic Reviews and Meta-analysis (PRISMA) and Cochrane guidelines. A total of 71/190 (38%) cases reported surgical debridement. Fasciotomies were reported in 12/190 (6%) cases and 20 procedures. Amputations were reported in 154/190 (81%) cases. Reconstruction was reported in 45 cases. Skin grafts were applied in 31 cases. Flaps were used for reconstruction in 28 cases. Median (IQR) surgical procedures per patient were 4 (4, 5) procedures. Infectious organisms causing PF were 32% Neisseria meningitidis (n = 55) and 32% Streptococcus pneumonia (n = 55). Coagulase-negative Staphylococcus (95% confidence interval (CI)(8.2–177.9), p = 0.032), Haemophilus influenza (95%CI (7.2–133), p = 0.029), Streptococcus pneumonia (95% CI (13.3–75.9), p = 0.006), and West Nile Virus (95%CI (8.2–177.9), p = 0.032) were associated with significantly more extensive amputations compared to other organisms. Conclusion This systematic review and patient-level meta-analysis found the most common presentation of PF was septic shock from an infectious organism. Neisseria meningitidis and Streptococcus pneumonia were equally the most common organisms associated with PF. The majority of cases were not treated in a burn center. The most common surgeries were amputations, with below-the-knee-amputations being the most common procedure. Skin grafting was the most commonly performed reconstructive procedure. The most common complications were secondary infections. Organisms with significantly more extensive amputations were coagulase-negative Staphylococcus, Haemophilus influenza, Streptococcus pneumonia, and West Nile Virus. Interpretation of findings should be cautioned due to limited sample data.

Septic Shock and Purpura Fulminans Due to Streptococcus pneumoniae Bacteremia in an Unvaccinated Immunocompetent Adult: Case Report and Review

“…The rarity of this hematologic emergency is best illustrated by the fact that only 306 patients were diagnosed with PF in a multicenter retrospective cohort study from France, which included admissions from 55 ICU over the period of 17 years [23]. Furthermore, only 190 adult patients who required surgical intervention for PF were described in a systematic review and meta-analysis of 79 and 77 studies, respectively [24]. [25].…”

“…Given the significant morbidity and mortality associated with PF, rapid recognition and timely initiation of treatment is essential for a favorable outcome. Ideally, care should be provided by a medical and surgical multidisciplinary team with experience in treatment of this disease [24,29,43]. The initial management should be focused on the treatment of sepsis, which includes supportive care in an ICU, administration of intravenous fluids, inotropes, antimicrobials, steroids, and mechanical ventilation, if necessary.…”

“…These patients benefit from earlier transfer to burn units, particularly in cases where all 4 extremities are involved or there is more than 20% of body surface area affected. However, a recent review reported that only 7% of these types of patients were managed in a burn center [24].…”

“…They postulated that early surgical decompression of involved extremities may be beneficial as aggressive fluid resuscitation and endotoxin-induced fluid shifts can lead to tissue edema, which in turn might result in compartment syndrome, further worsening blood flow to an already compromised extremity [43]. A more recent systemic review and meta-analysis by Klifto et al [24] reviewed 190 cases in which surgical intervention was performed on patients with PF. Out of the 190 cases reviewed, 71 patients required debridement only (38%), 12 patients had fasciotomies (6%), and 154 patients underwent amputation (81%), with below-the-knee-amputations (BKA) the most common procedure.…”

“…Patients who survived the initial insult of PF often undergo additional reconstructive procedures such as skin grafts and skin flaps. Management of these patients is complex and expensive, with a reported median hospital stay of 57 days (range from 11 to 292 days) [24].…”