Toxic Shock Syndrome
Toxic Shock Syndrome: Excerpt from The 5-Minute Pediatric Consult
Mark L. Bagarazzi, MD
Toxic Shock Syndrome - BASICS
Toxic Shock Syndrome - description
- Toxic shock syndrome (TSS) is an acute febrile illness characterized by myalgia, vomiting, diarrhea, pharyngitis, diffuse desquamating macular erythroderma, erythema of the mucous membranes and conjunctiva, multiorgan system involvement by direct inflammatory damage or ischemia, disseminated intravascular coagulopathy (DIC), and hypotension.
- TSS is most commonly caused by group A β-hemolytic streptococci (GABHS or Streptococcus pyogenes) or TSS toxin-1 (TSST-1)–producing strains of Staphylococcus aureus (including methicillin-resistant isolates). Cases have also been reported in association with group B, C, and G1 streptococci and Streptococcus mitis.
Toxic Shock Syndrome - general prevention
- Avoidance of tampon use after 1st episode of TSS
- Scrupulous wound care
- Limitation of intravaginal foreign body use (e.g., tampon, sponge) and strict adherence to manufacturers’ directions
Toxic Shock Syndrome - epidemiology
- Early 1980s: >90% of cases (almost exclusively owing to S. aureus) occurred in menstruating women; associated with superabsorbent tampon use. The frequency of cases occurring in menstruating women dropped in the mid-1980s because of changes to less absorbent or tampons of different composition. In 1996, <50% of cases were associated with menstruation.
- Mean age: 22 years
- Incubation period for postoperative S. aureus–mediated TSS can be <12 hours.
- Preceding varicella infection dramatically increases the risk for acquiring invasive disease owing to GABHS including TSS.
- Currently, ~60% of cases occur in girls and women.
Toxic Shock Syndrome - incidence
- Current incidence of menses-related disease: 1–5 per 100,000 women of menstrual age per year
- Streptococcal TSS incidence is highest among young children.
Toxic Shock Syndrome - risk factors
- Use of superabsorbent tampon, diaphragm, or contraceptive sponge; local or invasive staphylococcal or streptococcal infection: Including but not limited to bacteremia, endocarditis, pyogenic arthritis, sinusitis, osteomyelitis, pneumonia, pharyngitis, pancreatitis, cholecystitis, abscess, cervical adenitis
- Superficial and surgical wounds, childbirth, abortion, varicella infections, immunosuppressive (including corticosteroids and NSAIDs) or immunomodulatory therapy
Toxic Shock Syndrome - DIAGNOSIS
Toxic Shock Syndrome - signs & symptoms
- US Centers for Disease Control and Prevention (CDC) criteria for diagnosis of staphylococcal TSS:
- Fever 38.9°C (102.0°F) or higher
- Diffuse macular erythroderma
- Desquamation 1–2 weeks after onset, particularly affecting palms and soles
- Hypotension below 5th percentile for children, orthostatic changes >15 mm Hg or orthostatic syncope, or dizziness
- Involvement of three or more organ systems: Gastrointestinal, muscular, mucous membrane, renal, hepatic, hematologic, or neurologic
- All 5 of the above criteria with blood culture(s) positive for S. aureus only and negative serology for Rocky Mountain spotted fever (RMSF), leptospirosis, and measles; 4 of 5 criteria termed probable. 4 criteria plus death before desquamation yields complete syndrome:
- CDC criteria for diagnosis of streptococcal TSS:
- Hypotension or shock
- Any 2 of the following: Renal impairment, DIC, thrombocytopenia, hepatic impairment, adult respiratory distress syndrome, erythematous macular rash that may desquamate, or soft-tissue necrosis
- Isolation of GABHS from a normally sterile site constitutes a definite case. Isolation of GABHS from a nonsterile site constitutes a probable case.
- Pitfalls:
- Production of TSST-1 by isolate is only presumptive evidence unless case meets diagnostic criteria.
- Failure to meet CDC diagnostic criteria
- Failure to identify soft-tissue or muscular site of local infection
- Failure to identify or remove foreign body
- Erythroderma may not be appreciated if patient is already hypotensive.
- Diagnosis still must be considered in the absence of an identifiable focus.
Toxic Shock Syndrome - history
- Tampons, contraceptive sponges, or diaphragms are all known risk factors: Use may occur at any time during menses.
- Any surgical procedures including catheters (e.g., IV, peritoneal dialysis): Incubation period for postoperative TSS may be as short as 12 hours.
- Nonsurgical wounds, burns, childbirth, abortion, or puerperal infections
- Other active streptococcal or staphylococcal infections: History frequently elicits probable recent infections.
- Abrupt onset of high fever, rapid-onset hypotension, rapidly accelerated renal failure, and multisystem organ failure are all historical findings associated with either staphylococcal or streptococcal TSS. Chills, malaise, headache, pharyngitis, fatigue, and dizziness or syncope are also seen frequently.
- Profuse watery diarrhea (often with fecal incontinence), vomiting, abdominal pain, generalized erythroderma, conjunctival injection, and severe myalgias are all historical findings seen commonly in staphylococcal TSS, but less frequently in streptococcal TSS. The presence of a foreign body is also more common with staphylococcal TSS than with streptococcal TSS.
- Evidence of increasingly painful local soft tissue infection (e.g., abscess, cellulitis, myositis, or necrotizing fasciitis) are all historical findings seen commonly in streptococcal TSS but less frequently in staphylococcal TSS.
- Tampons with ingredients such as polyacrylate, polyester foam, cross-linked carboxymethylcellulose; or claims of superabsorbency are associated with TSS.
Toxic Shock Syndrome - physical exam
- Any sign of soft-tissue infection (e.g., cellulitis, necrotizing fasciitis, myositis, soft-tissue abscesses, sinusitis): Often seen before onset of disease
- Overall appearance: Patients with TSS are always moderately to severely ill.
- Altered vital signs: Fever, tachycardia, tachypnea, orthostasis, or frank hypotension. Tachycardia is the prelude to hypotension.
- Abnormal skin, mucous membranes, and soft tissues: Erythroderma, peripheral cyanosis and edema, bulbar conjunctival hyperemia, subconjunctival hemorrhages, beefy red mucous membranes, and muscle tenderness
- Mental status changes: Altered mental status, including somnolence, agitation, disorientation, and obtundation within 24–72 hours are seen with TSS
- Intensity of erythroderma: May be most intense surrounding the infected focus (e.g., perineum)
- Desquamation: Begins on trunk and extremities 5–7 days after symptom onset. Full thickness desquamation of fingers, toes, palms, and soles begins 10–12 days after onset.
- Vesicle or bullae formation, or presence of violaceous hue: Ominous findings associated with increased fluid loss and potentially shock
Toxic Shock Syndrome - tests
Toxic Shock Syndrome - lab
- Antibodies to TSST-1 (available for informational purposes/research only from Toxin Technology, Inc., www.toxintechnology.com): Test result will be positive several weeks after acute presentation.
- Antibodies to antistreptolysin O (ASO), antideoxyribonuclease B, or other streptococcal extracellular products: May increase 4–6 weeks after infection in streptococcal-mediated disease
- Complete blood count: Usually reveals thrombocytopenia early in the course of disease, thrombocytosis during the recovery phase, anemia early in disease, normal or slightly elevated leukocyte count with left shift, and absolute lymphopenia. Neutropenia is more ominous than lymphopenia.
- Blood cultures: Positive in 60% of streptococcal TSS, rarely (<5%) positive in staphylococcal TSS
- Local cultures: S. aureus may be isolated from vagina or cervix in menstrual TSS, or from other infectious focus in nonmenstrual cases. Isolation of group A streptococci from a sterile site is a definite case, whereas isolation from a nonsterile site constitutes a probable case.
- Coagulation studies: May reveal prolonged prothrombin and partial thromboplastin times (PT/PTT) with or without evidence of DIC: Low fibrinogen, elevated fibrin degradation products
- Urinalysis: May reveal sterile pyuria
- Lumbar puncture: May reveal cerebrospinal fluid pleocytosis
- Creatine phosphokinase (CPK): May be elevated, reflecting skeletal muscle involvement
S. aureus isolated from nares or vagina may represent a false-positive finding because 10–30% of those affected are healthy carriers.
Toxic Shock Syndrome - differencial diagnosis
- Septic shock owing to Neisseria meningitidis
- Streptococcal and staphylococcal scarlatiniform eruptions
- Leptospirosis
- Rocky Mountain spotted fever without characteristic rash
- Fulminant viral infection (e.g., adenovirus)
- Kawasaki disease, although TSS may present simultaneously with Kawasaki disease. Coronary artery dilatation has been reported in cases presenting as TSS.
- Toxic epidermal necrolysis (TEN)
Toxic Shock Syndrome - TREATMENT
Toxic Shock Syndrome - special therapy
- Remove all foreign bodies.
- Surgical debridement with myositis and necrotizing fasciitis; abscess drainage
- Antibiotics: Parenteral administration with antistreptococcal and staphylococcal therapy eradicates source of the toxin, but does not affect the course of the acute illness.
Toxic Shock Syndrome - medication
- Use both a bacterial cell wall inhibitor, such as semisynthetic antistaphylococcal penicillins (i.e., nafcillin, oxacillin, dicloxacillin, and cefuroxime or ampicillin/sulbactam) as well as a protein synthesis inhibitor (e.g., clindamycin) to end production of toxins, enzymes, and cytokines.
- Continue therapy for 10–15 days or until causative bacteria is eradicated on follow-up cultures.
- Clindamycin or erythromycin if patient is allergic to penicillin
- IV immunoglobulin (IVIG):
- Placebo-controlled multicenter study did not show a significant benefit in 28-day survival in patients with definite streptococcal TSS
- Anecdotal reports of efficacy for streptococcal TSS
- May be considered for infections refractory to aggressive therapy or in patients with infection in an area that cannot be drained
- Optimal regimen is not known, although single doses of 1–2 g/kg, as well as several days of 150–450 mg/kg/d have been studied.
- Corticosteroids: Have not been systematically studied
Toxic Shock Syndrome - FOLLOW UP
- Poor prognosis is often heralded by development of pulmonary edema, falling cardiac index, and rising pulmonary capillary wedge pressure.
- Renal failure and cerebral edema (i.e., complication of fluid resuscitation) are other indications of complicated course.
- Temperature usually returns to normal within 2 days.
- Toxin-mediated cardiomyopathy should resolve if fatal arrhythmia does not occur during decompensated stage.
- Gastrointestinal, hepatic, and musculoskeletal changes resolve rapidly with rare permanent sequelae except for muscle weakness.
- Hair and nail loss may occur 4–16 weeks after illness onset; should resolve within 5–6 months.
- Encephalopathy is common, rarely causes seizures; both usually resolve within 4–5 days.
- Reporting of TSS is mandatory in many states.
Toxic Shock Syndrome - prognosis
- Recurrences are associated with inadequate treatment (parenteral antibiotics).
- Mortality 5–7% for staphylococcal disease. Myocardial and pulmonary failure: Most common causes of death
- Mortality is higher in nonmenstrual TSS (men and women >45 years of age) owing to delayed recognition.
- Death usually occurs within the 1st few days; may occur as late as 2 weeks following onset.
- Permanent renal damage is extremely rare.
Toxic Shock Syndrome - complications
Multisystem organ failure secondary to distributive shock/hypotension including:
- Pulmonary edema
- DIC
- Acute renal failure (oliguric and nonoliguric)
- Hepatic failure
- Myocardial edema and decreased contractility with or without arrhythmias
- “Stunned” myocardium demonstrating severe ventricular contractile dysfunction
- Cerebral edema with toxic or ischemic encephalopathy
- Metabolic disturbances
- Telogen effluvium; temporary hair and nail loss
- Neuropsychologic disturbances including memory loss; abnormal EEG rare
Toxic Shock Syndrome - bibliography
American Academy of Pediatrics. Toxic shock syndrome. In: Pickering LK, ed. 2006 Red Book: Report of the Committee on Infectious Diseases, 27th ed. Elk Grove Village, IL: American Academy of Pediatrics; 2006: 660–665.- Bisno AL, Stevens DC. Streptococcal infection of skin and soft-tissues. N Engl J Med. 1996;334:240–245.
- Darenberg J, Ihendyane N, Sjolin J, et al. Intravenous immunoglobulin G therapy in streptococcal toxic shock syndrome: A European randomized, double-blind, placebo-controlled trial. Clin Infect Dis. 2003;37:333–340.
- Reich HL, Crawford GH, Pelle MT, et al. Group B streptococcal toxic shock-like syndrome. Arch Dermatol. 2004;140:163–166.
Toxic Shock Syndrome - CODES
Toxic Shock Syndrome - icd9
040.82 Toxic shock syndrome
Toxic Shock Syndrome - FAQ
- Q: Can TSS recur?
- A: Yes. Inadequate eradication of the nidus of infection, as in continuing sinusitis or a case that involves a foreign body, can lead to recurrent staphylococcal TSS. Moreover, some people with some immune system defects may develop recurrent TSS.
- Q: Can TSS be diagnosed in patients who have no risk factors?
- A: Yes, there have been reports meeting the case definition where none of the known associated factors was present.
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Book Source Details
- Book Title: The 5-Minute Pediatric Consult
- Author(s): M. William Schwartz MD; et al.
- Year of Publication: 2008
- Copyright Details: The 5-Minute Pediatric Consult, Copyright © 2008 Lippincott Williams & Wilkins.
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Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.
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More About This Book:
Title: The 5-Minute Pediatric Consult
Authors: M. William Schwartz MD; et al.
Publisher: Lippincott Williams & Wilkins
Copyright: 2008
ISBN: 0-7817-7577-9
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