Background: Patients who survive the initial acute phase of fulminant meningococcemia are at increased risk for serious orthopaedic complications. This report describes our experience with purpura fulminans related to meningococcemia, with emphasis on musculoskeletal sequelae and their treatment.
Methods: We retrospectively reviewed the cases of forty-eight patients (twenty-two boys and twenty-six girls) who survived the acute phase of meningococcal septicemia and developed musculoskeletal sequelae. Early sequelae required surgical treatment within six months after the onset of sepsis and were primarily amputations. Late sequelae required surgical management six months or more following the initial infection and included growth disturbances, stump overgrowth, scar contractures, and soft-tissue and bone infections.
Results: The mean age at the onset of sepsis (and standard deviation) was 2.6 ± 3.0 years, and the mean duration of follow-up was 11.7 ± 5.1 years. The mean number of surgical procedures required was 4.4 ± 2.9 per patient. Early sequelae included amputations at a variety of levels. Upper-extremity amputations were less frequent than lower-extremity amputations. Growth disturbances in the upper extremities were less frequent than growth disturbances in the lower extremities. Stump overgrowth occurred in only eleven patients (23%) with a lower-extremity amputation. The prevalence of scar contractures in the upper extremities was similar to that in the lower extremities. Eight patients (17%) acquired soft-tissue and bone infections at the distal portion of the amputation stump, at a mean of 7.2 ± 5.4 years after the initial sepsis. Thirteen patients (27%) were found to have developmental delay at the time of follow-up, and it was severe in seven of them.
Conclusions: All patients who survive meningococcal septicemia should be followed by an orthopaedic surgeon who is experienced in pediatric limb deformities and amputations. Children requiring surgery for purpura fulminans are often limited by physical disability due to amputation, scarring, and abnormal bone growth. Despite advances in orthopaedic management, children are still at risk of developing physical and developmental limitations.
Level of Evidence: Prognostic Level IV. See Instructions to Authors for a complete description of levels of evidence.
1Department of Pediatric Orthopedics, Shriners Hospital for Children, 3101 S.W. Sam Jackson Park Road, Portland, OR 97239. E-mail address for F. Canavese: email@example.com