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The surgical implications of purpura fulminans
J R Cohen1, R Lackner, A Keller
1Department of Surgery, Long Island Jewish Medical Center, New Hyde Park, New York 11042.
Insights
Purpura fulminans is a rare, severe condition in children following infection. Early heparin treatment can prevent tissue loss and improve outcomes in this critical illness.
Area of Science:
- Pediatrics
- Hematology
- Infectious Diseases
Background:
- Purpura fulminans is a rare, catastrophic syndrome in children, often following infection.
- It presents with fever, purpura, hypotension, disseminated intravascular coagulation, and potential gangrene.
- This syndrome can lead to severe morbidity and mortality in affected children.
Purpose of the Study:
- To describe the clinical presentation and outcomes of children treated for purpura fulminans.
- To evaluate the efficacy of heparin therapy in managing this condition.
- To highlight the importance of early diagnosis and intervention.
Main Methods:
- Retrospective case series of six children treated for purpura fulminans.
- Clinical data including age, sex, symptoms, laboratory findings, and treatment were reviewed.
- Treatment involved systemic support, antibiotics, and intravenous heparinization.
Main Results:
- Five of six patients presented with altered mental status and high fever.
- All patients had purpuric involvement of extremities, with absent pulses.
- Two patients died from sepsis; survivors received heparin, with two experiencing significant tissue loss and two improving without loss.
Conclusions:
- Immediate heparinization is crucial for managing purpura fulminans to prevent thrombosis and limit tissue damage.
- While heparin is the mainstay, supportive care and antibiotics are essential.
- Conservative management of lesions is recommended until demarcation, with reconstructive surgery as needed for survivors.
Abstract:
Purpura fulminans is an uncommon catastrophic syndrome that occurs in children, typically one to four weeks after a seemingly benign infectious process. The child usually presents with a high fever, purpuric ecchymosis, hypotension, disseminated intravascular coagulation, and gangrene of the extremities. We have recently treated six children, whose mean age was 22 months; three were male and three were female. Five of the six had a change of mental status upon initial examination. Their mean temperature was 104 degrees F. All six children had purpuric involvement of their extremities; three had involvement of their hands, two had involvement of their faces, and two had involvement of their trunks. All had absent palpable pulses and sluggish capillary refill in the involved hands and feet. Two patients died shortly after admission as a result of severe end-stage sepsis. The platelet counts in these two patients, and the white blood cell counts were markedly depressed. The mean platelet count of the survivors was 370,000 and the mean white blood cell count was 25,000. Lumbar punctures were positive for bacterial meningitis in five patients and viral meningitis in one patient. All patients were treated with intravenous heparin. Of the four survivors, two lost significant tissue and required multiple plastic reconstructive procedures, and two improved on heparin alone with no tissue loss. In addition to systemic support and intravenous antibiotics, the mainstay of treatment is one of immediate heparinization and a continuous heparin drip. Heparin prevents subsequent small vessel thrombosis and limits tissue loss due to ongoing purpura. Conservative management of the purpuric lesions is the treatment of choice until final demarcation occurs.