Good Riddance Thanks to Vaccine
The following case study was used by James P. Keating, MD, MSc, medical director, St. Louis Children’s Hospital Diagnostic Center, and his co-editor, Andrews J. White, MD, division director of pediatric rheumatology/immunology, as part of the “Patient of the Week” (POW) series. Many of the POW case studies cover uncommon illnesses, or common illnesses with unusual symptoms that can be overlooked. If you would like to be added to the POW e-mail distribution list, send an e-mail to jkeating@wustl.edu or white_a@wustl.edu.
Greenville, IL: Jamie Baum/Tracy Hall
ED: Julie Leonard/Joe Gunn
PICU: Matt Goldsmith/Bill Waldrop/Robin Puente
ENT: Keiko Hirose
Rad: Bill McAlister/Bob McKinstry
Vaccine development: Penelope Shackelford
A 21-year-old female with 18q chromosomal deletion and developmental delay (functioning at about 5-year-old level) presented with CC: one-day of sore throat.
HPI: She awoke with dysphagia and drooling. CT scan showed a large, soft-tissue density extending from the left pharyngeal mucosal space into left carotid space, involving the left aryepiglottic fold and the pyriform sinus. The epiglottis was thickened with a C-shaped appearance. There was narrowing of the upper airway. This was interpreted as supraglottitis/epiglottitis, and she was transferred by helicopter to our emergency unit. Ceftriaxone was given. Bedside fiberoptic laryngoscopy in the ED confirmed the diagnosis, and she was taken to the OR. Anesthesia was induced using IV dexmedetomidine. The fiberoptic bronchoscope was passed through the oral cavity and 5.0 endotracheal tube with a cuff was passed through the glottis into the trachea. The epiglottis was large, pink, swollen and edematous. Once she was safely intubated, laryngoscopy revealed the epiglottis was completely obstructing the supraglottic airway. She was transported to the intensive care unit with stable intubation.
PE: T 102 Wt 92.5 kg. Some muffling of voice and audible fluid in the oropharynx but no stridor, crackles or cyanosis. Allergies: Penicillin, ampicillin, amoxicillin.
Course: In the PICU she remained intubated for 72 hours. She developed a reassuring leak around her ETT as the edema/inflammation subsided, and she was uneventfully extubated and discharged on hospital day No. 6.
Diagnosis: Supraglottitis/epiglottitis, uncertain agent.
Comment: The clinical and epidemiologic patterns of epiglottitis have changed since the widespread immunization (circa 1992) of children against hemophilus influenza (the most common cause in children until that time). It was a dread childhood illness but is now virtually absent in the pediatric age group. By default, it has become an adult condition usually due to pneumococcus, parainfluenza virus, and rarely meningococcus or other bacterial pathogens. A smaller proportion of adults require/receive airway intervention.
Before universal immunization of children against hemophilus influenza, epiglottitis was a common (4/100,000) cause of death or brain damage in the pediatric population. Since the development and implementation of an effective vaccine, it has dropped to 0.1/100,000. A child would go to bed with mild symptoms of a URI only to awaken with air hunger, drooling and intense stridor and dyspnea. If airway support could be mustered rapidly and PICU care was available for three to four days, the child recovered completely. Successful outcome required sophisticated caregivers including the primary care physician, emergency room staff, transport staff, pediatric anesthesiologists and intensivists, otolaryngologists and helicopter pilots. Particularly in small communities, those resources were/are not available. We remain thankful to Penny Shackelford, MD, and her colleagues who developed the effective vaccine in the research labs here at WUSM/SLCH and elsewhere.
