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A three-week-old girl presented to the emergency department with a swollen purple labia (Figure 1) that had developed over a 24 h period. She was feeding and voiding well, and there was no recent change in formula or brand of diapers. There was no history of trauma. The baby was somewhat irritable, and her diaper area was very tender to touch. She was afebrile, and had no sick contacts. She had no cyanosis or respiratory distress. Her medical history showed that she was born to a 19-year-old gravida 2, para 2 mother who had limited antenatal care. The baby was born by planned caesarian section due to active herpes lesions. There was no rupture of membranes at the time of delivery. Neither mom nor baby received antiviral medications. The baby had a heart murmur detected at birth, and was diagnosed with double-outlet right ventricle. Surgery was scheduled for a later date. She was not receiving any medications and had no known allergies. The Children’s Aid Society had previously been involved with the family, and the family physician described the social circumstances as extremely high risk. The child was referred to the on-call paediatrician at the regional sexual assault and child abuse centre because of concerns of nonaccidental injury.
On initial physical examination, her heart rate was 140 beats/min, respiratory rate was 32 breaths/min, temperature was 37.7°C, oxygen saturation was 99% and her weight was 3.042 kg. The patient’s respiratory examination was normal. On cardiac examination, she had a pansystolic murmur, but normal cap refill and good pulses. She had no peripheral edema. Her abdomen was soft and nontender, with no hepatosplenomegaly. Her neurological examination was normal. Her skin examination was normal, apart from an impressive purple discolouration in the diaper area, with tender swollen labia (Figure 1). A blood sample revealed a white blood cell count of 12.8×109/L, with a 28% left shift; a hemoglobin level of 123 g/L and a platelet count of 391×109/L. The infant was admitted for further observation, and her clinical progression overnight led to the diagnosis.
Differential diagnosis at the time of admission included infection, cardiogenic edema and nonaccidental injury. Given that the child looked well, her rash did not look typical for infection and her bloodwork was remarkable only for the left shift, she was admitted for observation and no active treatment was started. However, 10 h after admission, the on-call paediatrician was paged urgently due to significant deterioration in the infant’s respiratory status. On examination, the infant was tachypneic, cool, mottled and reacting poorly. The purple discolouration had turned to red, and migrated laterally and superiorly to the level of the umbilicus. A chest x-ray demonstrated cardiomegaly, but no abnormalities in her lung fields. She was intubated to stabilize her respiratory state. She had profound metabolic acidosis with a pH of 6.9 and a bicarbonate concentration of 3 mmol/L. Her mean blood pressure fell to 29 mmHg, and normal saline and vasopressors were administered. She was catheterized for urine, but had insufficient output to send a culture. She was not stable enough for lumbar puncture. Based on her clinical presentation, she was diagnosed with septic shock and treated with meningitic doses of ampicillin, cefotaxime and acyclovir. She required significant fluid resuscitation as well as inotropic support, but with aggressive treatment, she recovered fully from her illness. No organism was cultured.
The child presented with an unusual rash (Figure 1), but otherwise unremarkable physical examination, and quickly deteriorated into septic shock. With the unusual skin rash, and a high band count, the patient did not meet low-risk criteria for serious bacterial infection, according to the Rochester criteria (Table 1). However, a MEDLINE search of the literature (1950 to 2008) failed to reveal any case reports of purple labial discolouration with sepsis in infants. To avoid overdiagnosis of sexual abuse, several sources suggest a full and complete evaluation of perianal rashes. The vast majority of children referred for evaluation due to disclosure of sexual abuse have normal or nonspecific findings. Perianal streptococcal infection is common in infants and children. However, this typically presents as a bright red confluent rash, with or without impetiginous lesions. Candidal diaper dermatitis may also be considered, yet appears as ‘beefy red’ and macerated. The possibility of dependent edema was raised, given the patient’s cardiovascular condition; however, this is rarely localized to such a small area and was summarily ruled out due to the clear demarcation of the discolouration.
Sepsis and septic shock are commonly seen in the paediatric population. One retrospective analysis (2) reported that 26.7% of total admissions to a paediatric intensive care unit were identified as septic shock. The attack rate of sepsis in full-term infants is 0.1% to 1%, and the associated mortality rate is roughly 20%.
Once the diagnosis of sepsis is established, aggressive treatment is necessary and life-saving. The causative organism should be sought, so that an appropriate treatment regimen can be started. Initial treatment should include boluses of isotonic crystalloid to regain hemodynamic stability. Laboratory studies should include base deficit, lactate concentration and venous oxygen saturation to identify the severity of shock and to monitor the response to fluid therapy. The patient should be monitored for metabolic derangements (eg, glucose and calcium), so that adjustments can be made. Finally, the need for vasopressors and stress-dose hydrocortisone should be anticipated to correct for adrenal insufficiency. In the event that severe shock persists, additional steps need to be taken (3).