The Case
The helicopter emergency medical crew was dispatched to a hospital 40 minutes away from the main base to transport a previously healthy female in her late teens who presented to a referring Emergency Department (ED) with three days of fever, nausea and vomiting. Patient was seen earlier on this same day at a walk-in clinic where she was noted to be febrile to 40.5 Celsius. She was originally taken by her boyfriend to the clinic because of increasing confusion and concerns about a red rash noted on her chest, abdomen and thighs. The patient thought that she had the flu. The nurse practitioner (NP) instructed the boyfriend to take the patient immediately to the local hospital emergency room for care. On admission to the ED, the patient was noted to be hypotensive (Systolic blood Pressure 70 mmHg), tachycardic in the 130’s and was noted to have a diffuse erythrodermic rash as previously described. Labs revealed a lactate of 7.5 mmol/L and a Creatinine of 2.2mg/dl. Pelvic examination revealed retained tampons (x2) in the vaginal vault as well as necrotic tissue with inflammation and exudate. A central line was placed and the patient was given 4000 ml of crystalloid and started on a Norepinephrine infusion. The patient was pan-cultured and antibiotics were initiated. Vancomycin, Zosyn (and after consultation with the receiving pediatric ED) she was started on Clindamycin. The patient was then transferred by helicopter to the regional pediatric academic medical center.
Pathophysiology of Toxic Shock Syndrome
There exists strains of streptococcal and staphylococcal bacteria that are capable of producing super-antigen toxins which cause wide spread activation of T-Lymphocytes. This wide spread activation results in a massive release of cytokines creating a syndrome that mimics septic shock. Although most people have acquired antibodies to these toxins, some individuals develop Toxic Shock Syndrome (TSS) when those bacteria colonize a person without these protective antibodies. It is thought that this disease occurs often in younger individuals because they have not yet encountered these toxins and lack any immunity to them.
Although the majority of cases of Staphylococcal TSS seen are associated with menstrual infection (retained tampons) there are non-menstrual cases of toxin secretion from sources such as soft tissue infection, sinusitis, sinus packing and pneumonia.
What You See and Hear
Because many of these patients are young and without multiple co-morbidities, they can present to a healthcare provider appearing “ill” but not critically so. Their appearance is deceptive and can cause delays in providing emergent treatment that is crucial to their recovery.
These patients often seek care after experiencing a few of days of non-specific combinations of flu-like symptoms (fever, headache, redness of the eyes, muscle aches, sore throat, nausea/vomiting and confusion). With gradual (sometimes rapid) onset of profound hypotension and tachycardia consistent with distributive shock i.e. sepsis.
A clue to the diagnosis of TSS is the presence of a diffuse erythematous rash resembling a sunburn. Although not always present (may be subtle or transient) this rash is a visual clue to the early diagnosis of TSS verses a garden variety septic shock.
Laboratory studies are generally non-specific in TSS (e.g., renal failure, DIC, thrombocytopenia, leukocytosis). Although blood cultures are important, they are neither sensitive or important enough to delay immediate care in the treatment of TSS.
Management
EARLY RECOGNITION: is key to the treatment of the patient with TSS. Despite not meeting all of the case definitions of TSS it is imperative to begin treating the patient based on clinical suspicion. Fever, nausea, vomiting and that sunburn like RASH should raise your suspicions for the diagnosis of TSS. Additionally, severe focal soft tissue pain out of proportion to examination plus systemic toxicity (e.g., high fever or elevated shock index) should lower your threshold for this diagnosis.
Those at risk for TSS: patients with history of using super-absorbent tampons, those with surgical wounds, a local or deep tissue infection, diaphragm or contraceptive sponge use and those having a history of recent childbirth, miscarriage or abortion.
RESUSCITATION: The rapid progression from onset to multi-organ system failure in TSS requires aggressive fluid resuscitation and frequently vasopressor, inotropic and respiratory support.
REMOVE THE SOURCE OF INFECTION: Debridement and/or tampon removal.
ANTIBIOTICS: Broad-spectrum antibiotics should be administered as soon as possible if you suspect TSS (preferably after blood cultures have been drawn). In this case the patient was prescribed Vancomycin, Zosyn and on recommendation from Michigan Medicine, Clindamycin (thought to Inhibit the synthesis of the toxins).
Take Home Points:
- Rapid diagnosis is key. Like all other forms of severe sepsis and septic shock, early broad-spectrum antibiotic implementation is of utmost importance.
- Suspicion of this syndrome cannot be overemphasized. Diagnosis is often based upon clinical picture. Systemic illness plus a diffuse blanchable erythematous rash can be the key indicator for more aggressive management.
- Initial aggressive management strategies should be consistent with the current standards for severe sepsis and septic shock.
Although infrequently requested to transport (incidence of TSS is estimated to be around 0.8 to 3.4 per 100,000 in the United States. CDC Feb 2019), Toxic Shock Syndrome (TSS) can be lethal causing death and disability.
References:
Farkas, J. (2017, June 04). Early suspicion of toxic shock syndrome. Retrieved April 3, 2019, from https://emcrit.org/pulmcrit/early-suspicion-of-toxic-shock-syndrome/
Nickson, C. (2012, December 16). Staphylococcal Toxic Shock Syndrome. Retrieved April 9, 2019, from https://lifeinthefastlane.com/ccc/staphylococcal-toxic-shock-syndrome/
Strom, M. A., Hsu, D. Y., & Silverberg, J. I. (2017). Prevalence, comorbidities and mortality of toxic shock syndrome in children and adults in the USA. Microbiology and immunology, 61(11), 463-473.
Wilkins, A. L., Steer, A. C., Smeesters, P. R., & Curtis, N. (2017). Toxic shock syndrome–the seven Rs of management and treatment. Journal of Infection, 74, S147-S152.