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CASE REPORT

Devastating Complications Of Staphylococcal Toxic Shock Syndrome In A Ten Months Old Girl

 Sameer K Khan * ,Anil Khanna*,Debasis Chakravarty **

* Senior House Officer
** Staff Grade Orthopaedic Surgeon
Department of Trauma and Orthopaedics, Peterborough District Hospital, Cambridgeshire, UK

Address for Correspondence:  

Mr Anil Khanna
Department of Trauma and Orthopaedics
Peterborough District Hospital
Peterborough & Stamford Hospitals NHS Foundation Trust
Peterborough PE3 6DA,
Cambridgeshire, UK
Email:  dranilkhanna@yahoo.com
Ph: 0044-7894657349

Abstract:

Staphylococcal toxic shock syndrome is a multi systemic illness that occurs infrequently, with an incidence less than 0.05 cases per 100,000 populations. A missed diagnosis of staphylococcal toxic shock syndrome can have potentially long-lasting squeal. This report stresses the need to employ a high index of suspicion when treating unwell children with febrile illness and cutaneous signs.

J.Orthopaedics 2008;5(1)e11

Case Report:

A ten months old Chinese girl with normal developmental milestones, presented in casualty with a two weeks history of illness and cough. She had been spiking temperature for two days and had experienced two episodes of diarrhoea and vomiting. On admission, she was febrile and irritable with a grunting respiration. She was tachycardiac and hypotensive with a capillary refill time of 5 seconds. The conjunctivae and oropharynx looked inflamed.

Urgent blood tests showed raised CRP (215 mg/l), leukopenia (WCC 3x109/l), reduced platelets (94x109/l), and a deranged coagulation profile (D-dimer 16166 ng/ml, INR 1.7). Arterial blood gas analysis revealed metabolic acidosis. A chest x ray revealed right-sided lower lobe consolidation. The Cerebrospinal Fluid analysis was normal.

Figure 1. Chest radiograph showing pneumonic consolidation in the right lung base

She was intubated, ventilated and resuscitated with intravenous fluids and inotropic support. Intravenous Ceftriaxone and Benzylpenicillin were commenced immediately. She received fresh frozen plasma, cryoprecipitate and Vitamin K to correct her coagulation profile. Within hours of admission, three petechial spots developed on her back, followed by a purpuric rash on her legs that became rapidly progessive. She also developed poor perfusion in both lower extremities. She had established global purpura fulminans by the time she was transferred to the paediatric intensive care unit.

The patient had a protracted ICU admission. Initially she developed a picture consistent with ARDS, which improved with high frequency oscillation, nitric oxide and steroids. By this time, a virulent Staphylococcus Aureus had been grown from blood cultures, skin scrapings from her purpuric rash, throat swab, and endotracheal secretions. The microbiologist recommended starting her on a poly-antimicrobial regime, consisting of Flucloxacillin, Clindamycin and Imipinem. She also received electrolyte infusions, blood and platelet transfusions. By the second week, her cardiovascular system had stablilised. Investigations revealed essentially normal immunoglobins and T cell subsets, normal coagulation profile and Proteins C and S.

By now, the perfusion in her lower extremities had deteriorated further. These became gangrenous and developed two clear demarcation lines. This necessitated bilateral below-knee amputations, followed by split-skin grafting to both stumps. The right stump healed well, such that it was fitted with a tuber-bearing prosthesis two months later. However, the left lower limb stump  developed an infection and assumed a fixed flexion deformity at the knee. A sinus developed on the anteromedial aspect of the stump, and a swab taken from it returned positive for staphylococcus aureus. MRSA was also isolated from nose, throat, axillary and groin swabs.

 

Figure 2. A healed low trans-tibial amputation on the right, and an infected stump on the left, with the knee in fixed flexion, and a sinus anteromedially.

Radiographs showed evidence of chronic osteomyelitis, with an involucrum overlying nearly all of the femoral diaphysis. She was continued on a long-term course of oral clindamycin. A radiograph taken a couple of weeks later revealed a pathological fracture at the junction of the middle and lower thirds of the diaphysis. A wait-and-see policy was followed with continued antibiotics, weekly inflammatory markers and monthly x-rays. The fracture eventually healed, with only some residual infection in the region of the distal diaphysis. This was addressed with a limited incision and curettage, from which she recovered well. She is doing well developmentally and is now awaiting a left-sided prosthesis, nearly 14 months after initial presentation.

 

 Figure 3. Radiograph showing large involucrum and sequestrum involving femoral shaft, with a fracture line visible in the lower femoral shaft.  

Discussion:

Staphylococcal toxic shock syndrome is a multisystemic1 illness that occurs infrequently, with an incidence less than 0.05 cases per 100,000 population2,3. It is caused by Exotoxin-producing strains of Staphylococcus aureus. The Centre for disease control has established major, minor, and exclusionary criteria to aid diagnosis4.

Major Criteria:

Fever > 38.9

Rash (diffuse macular erythroderma)

Desquamation

Hypotension / orthostatic syncope

Minor Criteria: Multisystem involvement (3 or more criteria must be met)

Gastrointestinal: vomiting or diarrhea at onset of illness

Muscular: severe myalgia or creatine kinase level twice upper limit of normal for laboratory

Mucous membrane: vaginal, oropharyngeal, or conjunctival hyperemia

Renal: blood urea nitrogen or creatinine level at least twice upper limit of normal for laboratory, or >5 white blood cells per high-power field in absence of urinary tract infection

Hepatic: total bilirubin, aspartate aminotransferase, or alanine aminotransferase at least twice upper limit of normal for laboratory

Hematologic: platelets <100,000/mm3

Central nervous system: disorientation or alterations in consciousness without focal neurologic signs when fever and hypotension are absent.  

Exclusionary Criteria: Normal results on the following tests (if performed):

Blood, throat, or cerebrospinal fluid cultures (blood culture may be positive for Staph aureus)

Absence of other explanation for the clinical presentation.  

This patientís initial presentation satisfied three major (fever, rash, hypotension) and three minor criteria (vomiting/diarrhoea, conjunctival hyperaemia, thrombocytopaenia), and both the exclusionary criteria for case definition. Treatment in such cases is mainly supportive. It is aimed at correcting the multiorgan dysfunction with circulatory and inotropic support, maintaining respiratory and renal function, parenteral antibiotics, and correction of disturbed haematological indices and electrolytes.

We need to employ a high index of suspicion when treating unwell children with febrile illness and cutaneous signs. The differential diagnosis includes Kawasaki syndrome, streptococcal toxic shock syndrome, leptospirosis and measles. A missed diagnosis of staphylococcal toxic shock syndrome can have potentially long-lasting sequelae, as experienced by this patient.

References:

  1. Behrman RE, Kleigman RM, Jenson HB eds (2004). Nelsonís Textbook of Paediatrics. 17th ed. Saunders Ltd, Pennsylvania: 865-866.  
  2. Centers for disease control. Follow-up on Toxic Shock Syndrome - United States. MMWR Morb Mortal Wkly Rep 1980; 29(25); 297-9.  
  3. Hajjeh RA, Reingold A, Weil A et al (1999) Toxic shock syndrome in the United States: Surveillance Update, 1979-1996. Emerg Infect Dis 5:807-10. 
  4. Centers for disease control and prevention. Case Definitions for Public Health Surveillance. MMWR Morb Mortal Wkly Rep October 19, 1990; 39; 38-9.


This is a peer reviewed paper 

Please cite as : Anil Khanna : Devastating Complications Of Staphylococcal Toxic Shock Syndrome In A Ten Months Old Girl

J.Orthopaedics 2008;5(1)e11

URL: http://www.jortho.org/2008/5/1/e11

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