Peripheral Gangrene and Extremity Amputations - Painful Preventable Sequelae of Meningococcal Septicaemia: Case Series Reports.

Extremity gangrene is a fairly common pathology, which complicates systemic vascular and endocrine diseases. Most often, it is encountered in diseases like uncontrolled Diabetes Mellitus, presenting as diabetic foot gangrene, severe peripheral arteriosclerosis with gangrene of the extremity complicating severe uncontrolled systemic hypertension and meningococcal septicaemia with peripheral gangrene. It also occurs in some cases of snake bite as well as frost bite (in regions with extreme cold weather conditions). Some of them present as monolateral extremity gangrene. However, others present as bilateral symmetrical peripheral gangrene (SPG) characterized by bilateral extremity ischaemia resulting in gangrene in which there is no major vascular occlusive disease. There is disseminated intravascular coagulation with the gangrene being considered as a cutaneous marker and some of the patients that survive ultimately require amputation of the affected limb(s) in the severe cases. The mild cases end up losing some of the digits or just exfoliation of the dead cutaneous layer. The effects are generally more severe in the lower limbs than in upper limbs. Notable among these are some of those complicating meningococcal sepsis resulting from peripheral intravascular coagulation. We present here, five patients who presented with varying degrees of peripheral gangrene during an epidemic of meningitis and the treatments that were carried out depending on the severity of their cases.

Plastic and Orthopaedic Interventions and Long-Term Sequelae in Children with Meningococcal Septicemia-40 Years of Experience at the University Children's Hospital Zurich.

INTRODUCTION: Meningococcal septicemia is not merely an acute disease with a high lethality, but patients surviving the fulminant phase can suffer from serious long-term sequelae. MATERIALS AND METHODS: The records of 165 patients admitted to the hospital from 1968 to 2008 with the diagnosis of meningococcal septicemia were retrospectively reviewed for early signs at presentation, intensive care management, acute symptoms, the necessity of plastic and orthopaedic surgical management, and long-term orthopaedic sequelae. Possible predictors of these conditions were determined. RESULTS: Overall lethality was 17.5%, and mean time of hospitalization time was 28 days. Integument involvement occurred in 45%, often resulting in the necessity of plastic surgical procedures. Young age is a significant indicator or skin involvement. Amputations were necessary in 9% of all cases, affecting the lower extremities more often than the upper extremities. Six percent of all children suffered from long-term orthopaedic sequelae, such as growth retardation and angular deformities, appearing up to 11 years (mean 4.9 years) after onset of the acute disease. The incidence of amputations and long-term orthopaedic sequelae correlated significantly with severity of the disease. CONCLUSION: Meningococcal septicemia can entail devastating long-term consequences in children surviving the acute phase of the disease. Sequelae may become apparent only years later and cause further damage. To prevent this, a systematic follow-up till adulthood is necessary.

Primary meningococcal septic arthritis with multiple native joint involvement.

A previously healthy 18-year-old woman presented with an acutely painful, swollen right ankle joint, with similar symptoms subsequently developing in the right hip and right wrist. Microbiological samples from the ankle aspirate grew Neisseria meningitidis She had had no preceding clinical symptoms of meningitis or previous contact with a patient with meningitis. She was treated with intravenous antibiotics and surgical drainage.On follow-up, her symptoms had resolved, inflammatory markers had returned to normal and she has no long-term sequelae of septic arthritis. Primary meningococcal septic arthritis in the adult population is extremely uncommon, and makes up approximately 1% of all cases of primary septic arthritis.

[Primary septic arthritis is a rare, atypical manifestation of invasive meningococcal disease].

We present a case report of a 50-year-old man diagnosed with a primary septic arthritis with invasive Neisseria meningitidis serogroup W (MenW) clonal complex 11 identified with culture in blood and synovial fluid. The patient recovered from rapidly instituted relevant antibiotics and synovectomy, but there may be a risk of fatal delayed diagnosis and treatment by an atypical manifestation of invasive meningococcal disease. Invasive MenW disease has been increasing in recent years and has been described with atypical presentations.

Management of septic skin necroses.

Necrotizing fasciitis (NF) and purpura fulminans (PF) are conditions with extensive septic skin necroses that are associated with significant morbidity and mortality. NF is caused by fulminant bacterial spread on the superficial muscle fascia, Group A streptococcus being the main microorganism responsible for it. The major challenge NF poses is timely recognition. Although crucial for patient survival, early diagnosis is difficult because paucity of specific early findings does not allow setting NF apart from other, less severe, differential diagnoses. Surgical therapy consists of early and aggressive debridement of all affected tissue, even if large disfiguring wounds are left back. The responsible microorganism for PF in children is predominantly Neisseira meningitidis. Endotoxin triggered misbalance of anticoagulant and procoagulant activities of endothelial cells leads to disseminated intravascular coagulation (DIC) followed by microvascular thrombosis and bleeding, resulting in hemorrhagic skin infarction and limb ischemia. Although survival in PF is not dependent on surgery, and surgery plays not a key role in the early phase of the disease, early surgical consult to assess if limb perfusion can be improved to achieve limb salvage is still absolutely necessary. Debridement should be postponed until clear demarcation has established. Large defects after NF and PF can be successfully reconstructed with vacuum-assisted fixation of Integra (Integra LifeSciences Corporation, Plainsboro, New Jersey, United States) artificial skin before split-thickness skin grafting. This provides good functional and cosmetic results as well as good stump coverage in case of amputation in PF.

Skin demarcation and amputation level for foot gangrene following meningococcal septicemia.

UNLABELLED: A case report of a 55-year-old woman who was being considered for bilateral below knee or Syme's amputations after gangrene at the level of both forefeet due to meningococcal septicaemia is described. An expectant approach for 6 months revealed that the tissues deep to the necrotic skin were viable and she was subsequently able to mobilize independently with normal footwear after bilateral toe amputations at the metatarsophalangeal joint levels. This case and a literature review suggest that the demarcation of healthy skin from necrotic skin (eschar) should not dictate the level of amputation in dry gangrene of the foot following meningococcal septicemia. Magnetic resonance imaging can overestimate the degree of muscle necrosis. Treatment should be individualized for each patient, but advice should include the option of prolonged conservative treatment to preserve limb length and function. LEVEL OF EVIDENCE: Therapeutic, Level IV: Case study.

Meningococcal sepsis and purpura fulminans: the surgical perspective.

Meningococcal sepsis and purpura fulminans is a rare but highly lethal disease process that requires a multidisciplinary team of experts to optimise morbidity and mortality outcomes due to the breadth of complications of the disease. The surgical perspective involves the critical care management which utilises all currently available measured outcomes of critical care management as well as experimental therapies. Limb loss is common, and is reflective of the high incidence of compartment syndrome compounded by the significant soft tissue loss secondary to purpura and limb ischaemia, presumptively due to digital microemboli. A multidisciplinary approach involving current standards in critical care and early surgical evaluation are important in improving patient outcomes and limb salvage.

Primary meningococcal arthritis requiring surgical drainage.

Primary meningococcal arthritis (PMA) is a relatively rare diagnosis where the role of early surgical intervention for its treatment is not well defined. We report a case of PMA in a young otherwise healthy patient who developed polyarticular joint pain secondary to Niessieria meningitidis without systemic symptoms of meningitis or meningococcemia. He underwent a prolonged course of intravenous antibiotics and serial aspirations of his shoulder. However, symptoms in his shoulder did not improve and he later underwent surgical irrigation and debridement.Intraoperatively, the patient had no signs of articular damage to his right shoulder despite prolonged clinically symptomatic disease. Six weeks after surgery, he has regained normal strength and full range of motion without any deficits.Nonoperative management of PMA is frequently, but not invariably, successful. We report a patient with this diagnosis who ultimately needed surgical evacuation of his shoulder joint to achieve resolution of his symptoms.

Severe skin necrosis in adult: an unusual complication of fulminant meningococcal sepsis.

We report on a 20-year-old female high-school student with fulminant meningococcal sepsis presenting as severe skin necrosis in lower extremities. The wound surface affected 2% of her total body surface area and was covered with black eschar. The adipose tissue beneath the eschar was degenerated and turned to necrosis as to deep fascia and periosteum of lower tibia. Excision of necrotic eschar, then covered with flaps on both legs resulted in a successful outcome.

Mycotic aortic aneurysm: A case of an unlikely location and an unlikely pathogen.

This is an unusual case of a mycotic aortic aneurysm located in the ascending aorta caused by Neisseria meningitidis. The diagnosis was made using two-dimensional (2D) and three-dimensional (3D) transoesophageal echocardiogram (TOE) and cardiovascular magnetic resonance (CMR). Management was with prolonged antibiotics followed by surgical resection. The ascending aorta was replaced with Dacron tube graft. The contribution of multimodality imaging in clearly defining the pathology, its location and directing the timing of surgery, was a striking aspect of this case.

Minimizing limb amputations in meningococcal sepsis by early microsurgical arteriolysis.

PURPOSE: Surgical treatment of children with meningococcal sepsis has mainly involved debridement of necrotic skin and amputation of limbs. This resulted in major functional impairment. On the contrary, when early microsurgical arteriolysis was performed, freeing up the blood vessels, the impaired blood flow could be restored, thereby significantly reducing the amputation levels. METHODS: We prospectively evaluated 14 patients affected by meningococcal sepsis. In 7 patients, microsurgical arteriolysis was performed; standard sepsis treatment was performed on the remaining 7. Ischemia levels on admission were compared with permanent amputation levels after 1 year. RESULTS: Statistically significant decreases (P = .005) in ischemia values were achieved by the arteriolysis, in comparison with final amputation percentages. The functional impairment of the affected limbs was highly reduced compared with the probable loss of function observed on admission. CONCLUSIONS: Our findings show that early microsurgical arteriolysis is a reliable method to reduce the devastating amputations normally found in patients with meningococcal sepsis. This significantly improves the functional outcome in severely ischemic limbs in meningococcal induced septic children.

Application of the vacuum-assisted closure in pediatric patients with orthopedic sequelae of meningococcemia: report of a case successfully treated.

Patients who survive the initial acute phase of fulminant meningococcemia are at an increased risk for serious complications as a result of poor tissue perfusion. It is rare that early surgical intervention is required, as it is relatively difficult to determine the degree of tissue loss early on. Once the patient is stable, debridement of all necrotic tissue is essential and may necessitate extensive removal of skin, subcutaneous tissue, and muscle. Widespread use of the vacuum-assisted closure for complex soft tissue injuries has generally showed accelerated wound healing compared with traditional methods. We report a new possible application of the vacuum-assisted closure system in very young patients with loss of tissue as a result of purpura fulminans secondary to meningococcemia.

[Purulent pericarditis in a patient with knee pain]. / Een purulente pericarditis bij een pijnlijke knie.

A 40-year-old man with pain in his left, swollen knee that persisted for 6 weeks presented with chest pain, dyspnoea and subfebrile temperature. The pain worsened during inspiration and was relieved by sitting up straight. The electrocardiogram showed pericarditis. The patient was treated with high-dose carbasalate calcium. Initially, echocardiography revealed a 2-cm pericardial effusion with no signs of influx inhibition. Blood cultures were positive for Neisseria meningitidis, and treatment was expanded to include antibiotics. Based on a deterioration in patient condition and the tamponade image, pericardiocentesis was performed. Repeated transoesophageal echocardiography showed insufficient drainage of the purulent pericardial effusion. Pericardiectomy was then performed. The patient was doing very well, 3 years after this. If left untreated, the mortality rate for purulent pericarditis approaches 100%. It is therefore important to diagnose at an early stage so that treatment with antibiotics and surgery, which can reduce mortality considerably, can be performed.

Improving outcome in meningococcal disease: don't forget compartment syndrome!

OBJECTIVE: To advocate a surgical intervention that can prevent the loss of limbs in patients with meningococcal disease. DESIGN: Case report. SETTING: Pediatric intensive care unit. PATIENT: A 4-month-old male infant presenting with acute circulatory failure due to meningococcal disease. INTERVENTIONS: Measurements of compartment pressures of all extremities and echo-Doppler of peripheral arteries were performed at regular intervals, starting at admittance to the pediatric intensive care unit. After compartment syndrome in the lower extremities was diagnosed, emergency surgical intervention (fasciotomy and arteriolysis) was performed in the intensive care unit. MEASUREMENTS AND MAIN RESULTS: During surgery, the compartments initially revealed pale, poorly perfused tissue. After decompression, immediate bulging of the muscles and restoration of microcirculation were seen. All digits were spared, and muscle compartments remained vital with exception of the tibialis anterior and extensor hallucis longus muscles in the left leg. Several ecchymoses and purpura of the lower extremities caused skin necrosis, necessitating skin transplants. No other surgical intervention was required. CONCLUSIONS: In meningococcal disease, compartment syndrome can occur within hours after initial presentation due to massive capillary leakage and circulatory failure. Immediate surgical intervention is the gold standard in treatment, making early recognition vital. In all patients presenting with meningococcal disease, compartment syndrome should be considered and early monitoring included in the initial evaluation.

The role of echocardiography in diagnosis and management of isolated meningococcal pericarditis.

Although pericarditis may complicate the course of meningococcemia, it is distinctly unusual as a presenting sign. Herein we report a case of a previously healthy 16-year-old male with isolated meningococcal pericarditis, in which transthoracic echocardiography was of great importance for the initial diagnosis and for guiding the therapeutic approach during the hospitalization period. The patient presented with symptoms of chest pain and fever that deteriorated into cardiac tamponade. Pericardiocentesis was successful and Neisseria meningitidis was identified as the causative agent in the pericardial fluid. Because of failure of clinical resolution, echocardiogram was repeated and showed evidence of maintenance of large echo dense content in pericardial space. The presence of purulent content was confirmed during open-chest surgery. The role of echocardiography for the correct management of this rare form of pericarditis is discussed.

Free perforator flaps in children.

BACKGROUND: Raising perforator flaps is said to be a tedious procedure. The benefits, however, are great. In adults, perforator flaps have proved their usefulness and reliability in various clinical situations. In children, donor sites for free flaps are particularly scarce because of the need for a long and reliable vascular pedicle of sufficient size. There is also the need to minimize donor-site morbidity from aesthetic, functional, and psychological perspectives. METHODS: The authors present a series of 23 consecutive free perforator flaps performed by the first author in 20 children; ages at the time of operation ranged from premature (born at 28 weeks) to 16 years (mean age, 7 years 5 months). Three children presented with upper limb defects; the remaining 17 children sustained major soft-tissue defects of the lower limb. All the lesions necessitated extensive coverage with a free flap. Flaps used in this series included nine deep inferior epigastric artery perforator flaps, seven thoracodorsal artery perforator flaps, and seven compound ("chimera") thoracodorsal artery perforator flaps. RESULTS: All flaps but one were successful. With a follow-up of up to 7 years, the results in this series compare favorably with those of perforator flaps in adults or pediatric free flaps in the literature. CONCLUSIONS: In children, as in adults, perforator flaps are a valuable alternative to the traditional muscle or myocutaneous free flap. Because of the added advantage of reducing donor-site morbidity, perforator flaps have become the authors' preferred option in reconstructive cases in children.

Soft-tissue reconstruction after meningococcal septicemia using a posterior tibial artery perforator flap in a 6-year-old boy.

Fulminant meningococcemia can have life-threatening as well as limb-threatening complications. However, unlike other types of peripheral gangrene, areas of necrosis do not follow known anatomic vascular patterns. Instead, irregular and patchy areas of necrosis are found to exist adjacent to viable tissues. With improved critical care, more patients survive the initial insult and are referred for reconstructive procedures. In this case, a 6-year boy was diagnosed with meningococcemia-induced purpura fulminans. After stabilization, he was transferred to our facility for management of open wounds of both lower extremities and the left elbow. Soft-tissue coverage was accomplished after multiple debridements using various flaps. In particular, a defect of the right midtibia was reconstructed using a posterior tibial artery perforator-based flap with excellent results. This is the first time such a flap has been reported in association with meningococcemia in a patient of this age.