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A 9-month-old male presents with 3-day history of fever, emesis
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A 9-month-old male presented to the ED with a 3-day history of fever up to 104°F, emesis, decreased urine output and a weak cry with fatigue. He was seen by his primary 1 day earlier, and with a normal chest radiograph, he was diagnosed with a viral illness. It is noted that he had a brief hospitalization 2 weeks earlier for bronchiolitis. He seemed to be doing well since discharge until the onset of the current problem.
His past medical history was significant for being a 26-week, premature quintuplet, with a 4-month NICU stay, complicated by a grade II intraventricular hemorrhage and GERD, but not chronic lung disease. His immunizations were up to date, and he had no travel, animal contact or sick contacts.
Source: James H. Brien, DO
Examination in the ED found the baby to be somewhat lethargic with a weak cry, and with a bluish lacy appearance to the skin and prolonged capillary refill, consistent with shock. A partial sepsis workup was done, including blood and urine cultures, CBC, comprehensive metabolic panel, INR and UA, revealing thrombocytopenia and a coagulopathy suggestive of sepsis. A chest radiograph revealing bilateral lung consolidation (Figure 1) and a normal head CT were also obtained. The patient was given fluids and antibiotics (vancomycin plus ceftriaxone), and transferred to the PICU.
He soon began having seizures, apnea and purpura of the extremities, and was intubated for ventilatory support. His lumbar puncture around that time revealed a cell count of 20 WBCs (40% segs) and 100 RBCs, protein 805, glucose of 0, and a Gram stain revealing Gram-positive cocci. Within a few hours of arriving to the PICU, his blood and cerebrospinal fluid (CSF) cultures were being reported positive for Gram-positive cocci in pairs and chains.
What’s your diagnosis (cause)?
A. Neisseria meningitidis
B. Group B streptococcus
C. Streptococcus pneumoniae
D. Listeria monocytogenes
Case Discussion
The patient’s CSF and blood cultures revealed S. pneumoniae (C), which is a Gram-positive coccus that is typically found in pairs. It is catalase negative, air tolerant and encapsulated. The capsule contributes to the pathogenesis in two different ways. One of the ways it is pathogenic is that the capsule binds complement-inhibitory factors. The second way it contributes to the pathogenesis of the bacteria is that is secretes factors such as IgA protease, which helps to destroy IgA found in the mucin and ciliated epithelial cell, especially in the respiratory tract, as well as pPneumolysin, which activates the complement pathway and recruits inflammatory cells to the focus of infection. In about 50% of healthy individuals, S. pneumoniae can be carried asymptomatically in the upper respiratory tract. Normally, the mucus and ciliated epithelial cells of the respiratory tract prevent entry of the bacteria into the lungs, but when these barriers are damaged, during or after a viral respiratory infection, the bacteria can gain access to the lungs and possibly to the blood.
S. pneumoniae is the most common cause of bacterial meningitis between the ages of 3 months and 10 years. Since the introduction of the pneumococcal conjugate vaccine, the incidence of invasive pneumococcal disease has significantly declined, especially with the most recent vaccine, PCV13, which includes 13 of the more common serotypes. Although the PVC13 does protect most children from acquiring serious pneumococcal infections, there are risk factors and certain conditions that may increase the risk for pneumococcal infections. Risk factors in children less than 6 months of age include low birth weight and having an older sibling in daycare. For children aged 6 to 23 months, the first 2 months of daycare pose the most risk for pneumococcal infection. Additionally, there are underlying medical conditions that put children at increased risk for pneumococcal disease. Those children can be divided into the following risk groups: immunocompetent children, children with functional or anatomic asplenia, and immunocompromised children. Children who fall under the first group would include children with chronic heart disease, chronic lung disease, diabetes, CSF leaks or cochlear implants. Children with functional/anatomic asplenia are usually children who have sickle cell disease or other hemoglobinopathies. But there may also be children who have congenital asplenia or splenic dysfunction. Immunocompromised children would include children with HIV infection, chronic renal failure or nephrotic syndrome, a malignancy receiving chemotherapy or primary immunodeficiency.
Culture is the “gold standard” in microbiology, but one can usually get a clue of the cause by staining. In this case, the Gram stain was positive for Gram-positive cocci in the CSF, as well as both cultures. If the stains were properly done, this would strongly mitigate against N. meningitis, as it is a Gram-negative coccus, as well as L. monocytogenes, a Gram-positive rod. Then, statistically, group B strep would simply be a distant cause in an infant this age, whereas, in the neonatal period, it would rise near the top of the list.
During surgery for g-tube placement, the surgeons incidentally noticed that the patient did not have a spleen. A confirmatory ultrasound was done that confirmed asplenia. This no doubt contributed to the patient’s severe infection.
Despite being on appropriate antibiotic therapy, the patient suffered severe, irreversible damage to his brain (Figures 2 and 3), with subdural fluid and multiple hemorrhagic cystic areas. His condition continued to decline, and he succumbed after 39 days in the PICU. Upon discovering the patient’s asplenia, we also screened his siblings, revealing one other to also be asplenic, and she was started on prophylactic penicillin. Interestingly, the patient had 40 CBCs done during his hospitalization, with only one showing Howell-Jolly bodies (remnants of the nucleus in some RBCs of patients without a spleen), which was on the day after discovering the absent spleen by surgery.
- For more information:
- Diane Brown, MD, is a third-year pediatric resident at McLane Children’s Hospital, Baylor Scott & White Health, in Temple, Texas.
Disclosure: Brown reports no relevant financial disclosures.
Columnist comments:
This unfortunate case raises an interesting question: should newborns be screened with an ultrasound, much like state-funded screening for other rare congenital conditions? For example, severe combined immunodeficiency syndrome is estimated to occur in 1 per 40,000 to 100,000 live births, whereas congenital asplenia occurs at a rate of approximately 1 per 10,000 to 40,000. Considering the obviously devastating results of not knowing that a child is asplenic without antibiotic prophylaxis, it would seem to be logical to screen newborns for asplenia, just as we screen for these other relatively rare conditions; something on which to ponder.
I want to thank Dr. Diane Brown for her work as guest columnist on this case. – James H. Brien, DO