Previously healthy premature neonate presents with increasing discomfort
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Editor’s Note: This month’s guest columnists are Pisespong Patamasucon, MD, professor and chief, section on pediatric infectious disease; and Robert K. Gordon, DO, third-year pediatric resident; both of the University of Nevada School of Medicine, Las Vegas.
A previously healthy 19-day-old, 34.5-week premature male was evaluated in the ED with 6 hours of increasing fussiness. Within 2 hours of arrival, he developed erythema and swelling along the left jaw line. During this time, vital signs revealed intermittent bradycardia and hypoxia, but he remained afebrile. He was transferred to the local children’s hospital for further management.
En route to the hospital, he had several more episodes of bradycardia down to the 40s and desaturations into the 60s. In the pediatric ED, he was noted to be lethargic with continued episodes of bradycardia and desaturation and was electively intubated to protect the airway and admitted to the PICU.
Source: Brien JH
His medical history revealed that his 27-year-old, gravida-4, para-1 mother was group B strep (GBS)-negative, but had premature rupture of membranes for 22 hours before delivery. Because of concerns for chorioamnionitis, the mother received four doses of penicillin before delivery, and the infant was treated for presumed sepsis in the NICU. Blood cultures were negative at 72 hours, during which time he received six doses of ampicillin and three doses of gentamicin, which were discontinued. The baby was in the NICU an additional 6 days for respiratory problems, but did well after discharge. Otherwise, his brief medical history was unremarkable.
Examination on admission revealed vital signs showing a rectal temperature of 97.9°F; a blood pressure of 55 mm Hg/23 mm Hg with prolonged capillary refill of 5 seconds; a heart rate of 192; and a respiratory rate of 28/minute with an oxygen saturation of 100% on 40% oxygen. He appeared toxic with cool extremities. Positive findings included adenopathy and induration with erythema of the skin over the left submandibular region (Figures 1 and 2). The swelling and erythema included the left pina and periauricular tissues (Figure 3). The rest of his examination was unremarkable.
Admission laboratory tests included a hemoglobin of 8.2 g/dL; a total white cell count of 700/cmm with 24% neutrophils, 9% bands; ANC of 231; platelet count of 229,000/cmm; and CRP of 17.14 mg/dL. Additional chemistries were within normal limits. Blood, urine, cerebrospinal fluid (CSF) and tracheal aspirate cultures were obtained, and treatment with ampicillin, gentamicin and vancomycin were started. There was only enough CSF for Grams stain (which was negative) and culture, which is pending.
What’s Your Diagnosis?
A. Cellulitis/adenitis syndrome of group B streptococcus
B. Overwhelming sepsis
C. Group A streptococcus erysipelas
D. Early skin infection from methicillin-resistant Staphylococcus aureus
Case Discussion
This neonate had a typical presentation of cellulitis-adenitis syndrome, which is an uncommon presentation of late-onset GBS infection. It is estimated to occur in 2% of late-onset GBS infections. Typically, the affected areas are the face, the pre- and post-auricular and submandibular areas, with the mean age of presentation at 5 weeks. Seventy-five percent of cases occur in males. The clinical findings include poor feeding, irritability, fever or hypothermia (as in this case) in association with localizing cellulitis and adenitis. Progression is very rapid after the onset of fever, with bacteremia and meningitis being frequently seen, with more than 80% being due to serotype III. Additionally,
Initial Grams stains of blood and sputum samples in this patient revealed Gram-positive cocci in chains. Less than 24 hours after obtaining the samples, the blood and sputum cultures grew Streptococcus agalactiae (otherwise known as GBS), which is sensitive to penicillin and gentamicin. The urine culture was sterile, but the CSF also grew GBS, although a few days later.
With the results of the cultures known, vancomycin was discontinued and the patient was continued on ampicillin and gentamicin until clinical improvement and sterilization of the blood and CSF was confirmed. Mechanical ventilation continued until the fifth day of hospitalization. The leukopenia and neutropenia resolved, and repeat CSF and blood cultures were negative. The patient was discharged after completing a 14-day course of antibiotics. Upon discharge, he was active, vigorous, had returned to baseline per parents and had no neurological deficits.
Although the infant’s clinical state may not appear to be that of overwhelming sepsis, meningeal involvement can be found in up to 24% of patients. Lumbar puncture should be performed to exclude meningitis in all cases in neonates and young infants. Early recognition and treatment of this unique clinical syndrome is imperative to avoid a poor outcome. The recommended empiric therapy for neonatal sepsis is a combination of ampicillin plus gentamicin or ampicillin plus cefotaxime, pending culture and sensitivities. If one has reason to believe that S. aureus is possible, or the patient is toxic-appearing, vancomycin should be added to the initial regimen.
GBS infections should be treated with ampicillin and gentamicin, pending clinical improvement, and sterilization confirmed. Outcome can be affected by a delay in initiating antimicrobial therapy; therefore, treatment should be initiated as soon as infection is suspected.
While GBS infections in neonates can present in a variety of ways, including sepsis, erysipelas, cellulitis adenitis syndrome; erysipelas due to group A streptococcus (GAS) can have a similar presentation with fever and facial swelling (Figures 4 and 5). In this case, one can see a generalized rash associated with the GAS infection (Figure 6).
MRSA cellulitis is a bit slower to develop and progress, and more likely to result in an abscess. However, as noted above, with situations such as this, one should cover for this possibility with vancomycin until culture results are known.
This patient was septic, with hypothermia, leukopenia, altered mental status and a positive blood culture, but overwhelming is a relative term, and of the possible answers provided, GBS cellulitis/adenitis was the most correct. Does that remind you of the Board Exams?
Columnist Comments
As I’ve gotten older, I frequently get up very early in the morning. Perhaps I’m trying to cram in as much time awake as I can — while I can. One Saturday last month, I got up about 4:30 a.m. (0430 for those of us who still think in military time) and went to the kitchen. Being too early for the newspaper, I turned on the television. It had been left on an HBO channel from the day before, and a show was just starting. It was called Section 60: Arlington National Cemetery.
I normally go right to the guide button to see the big picture of things that might be of interest. However, within seconds, I found myself staring at this simple, yet powerful documentary, centered on the section of Arlington National Cemetery where those killed in Iraq and Afghanistan are buried. I’ve never seen anything like it. It included simple and graceful observations of surviving relatives and friends who spoke while visiting the grave sites of those who now and forever silently reside there. There was no script; only the sounds of the wind and sometimes rain, mixed with weeping, and occasional comments made by these unrehearsed visitors. I felt like I had been punched in the gut, and soon found myself reaching for the Kleenex.
When I was still on active duty, we frequently took visitors to National Cemetery as part of the standard “Brien Family Site-seeing Tour.” Viewing rows upon rows of uniformed, bright white grave stones always inspired silent reverence, but seeing this documentary and the raw grief caused by these lives sacrificed will take your breath away. As of this writing, the most accurate count of US military personnel killed in Iraq is 4,486 and 2,000 in Afghanistan.
If you would like more information about this documentary, go to: www.arlingtoncemetery.net/section60-hbo-film-001.htm.
I would like to thank Drs. Pisespong Patamasucon and Robert K. Gordon for contributing this very interesting case. As noted in the selected references, Patamasucon has published on this condition before, along with
References:
Albanyan EA. Pediatrics. 1998;102:985-986.
James H. Brien, DO, is a member of the Infectious Diseases in Children Editorial Board, as well as Vice Chair for Education at The Children’s Hospital at Scott and White, and is the Associate Professor of Pediatrics at Texas A&M University, College of Medicine, Temple, Texas, can be reached at jhbrien@aol.com.
Disclosure: Brien reports no relevant financial disclosures.