What is the role of surgical intervention in the management of Purpura Fulminans (PF), a rare bleeding disorder characterized by thrombosis and hemorrhagic necrosis of the skin?

From the Guidelines

Surgical intervention for purpura fulminans should involve aggressive debridement of necrotic tissue, fasciotomies to relieve compartment syndrome, and possibly amputations in severe cases where tissue is non-viable, as recommended by the most recent guidelines 1. The goal of surgical intervention is to remove devitalized tissue, prevent further systemic inflammatory response, and improve antibiotic penetration to infected areas.

• Early surgical consultation is essential once purpura fulminans is diagnosed, as rapid progression can lead to irreversible tissue damage.
• The surgical team should work alongside critical care specialists managing the underlying cause, typically sepsis, disseminated intravascular coagulation (DIC), or protein C deficiency.
• Before surgery, patients should be stabilized with appropriate blood products including fresh frozen plasma (10-15 mL/kg), cryoprecipitate, and platelets as needed to correct coagulopathy.
• Protein C concentrate (50-100 IU/kg initially, followed by 50 IU/kg every 6 hours) may be administered in cases of protein C deficiency, as suggested by the American Society of Hematology guidelines 1.
• Following initial debridement, patients typically require multiple staged procedures with regular dressing changes, and eventual skin grafting or reconstructive procedures once the acute phase has resolved. The use of liver transplantation as a treatment option for pediatric patients with congenital purpura fulminans due to homozygous protein C deficiency is also considered, as it is curative of protein C deficiency, but has its own acute and chronic risks and burden of care 1.

From the Research

Surgical Intervention for Purpura Fulminans

Surgical intervention for purpura fulminans is a critical aspect of management, as it can significantly impact patient outcomes. The following points highlight the key aspects of surgical intervention:

• Aggressive surgical debridement of irreversibly devitalized tissue improves survival, often at the cost of disfigurement 2
• Early diagnosis and surgical intervention, including compartment release and sympathectomy, can minimize amputations in surviving patients 3
• Surgery is the primary treatment for necrotizing fasciitis, with early surgical fasciotomy and debridement being essential 4
• Amputations are commonly required, with below-the-knee amputations being the most common procedure 5
• Reconstruction techniques, such as skin grafts and flaps, are used to preserve as much length as possible, particularly where open growth plates are involved 6

Types of Surgical Procedures

The types of surgical procedures performed for purpura fulminans include:

• Fasciotomies to limit ischemic changes 4, 6
• Debridement to remove dead tissue 2, 4, 6
• Amputations to remove gangrenous limbs 3, 5, 6
• Reconstruction techniques, such as skin grafts and flaps, to preserve length and promote healing 5, 6

Timing and Considerations

The timing and considerations for surgical intervention are crucial:

• Early diagnosis and surgical intervention are critical to minimize amputations and improve outcomes 3
• Delayed debridement may be necessary to allow ischemic tissue to fully demarcate 6
• The decision to perform surgical intervention should be made in conjunction with the patient's surrogate or healthcare agent, taking into account the patient's wishes and values 2