Abstract | November 8, 2021
Macroglossia With A Peritonsillar Abscess: A Pediatric Airway Challenge in the Emergency Department From Beckwith-Wiedemann Syndrome
Learning Objectives
- Recognize the clinical presentation of Beckwith-Wiedermann (BWS).
- Summarize assessment and evaluation of peritonsillar abscess in BWS.
- Identify pediatric airway management in overgrowth disorders presenting with macroglossia.
Background/Knowledge Gap: Pediatric airway management is a critical skill for emergency physicians. Beckwith-Wiedemann Syndrome (BWS) is an overgrowth disorder caused by genetic mutations with a prevalence of 1 per 15,000 births. Excessive growth can affect almost any body part, including bones, muscles, blood vessels, organs, skin and adipose. Emergency physicians should anticipate a difficult airway when managing peritonsillar abscess in the presence of disorders involving macroglossia, such as Beckwith-Weidemann Syndrome.
Methods/Design: A 6 year-old female presented with a sore throat for one week, a negative strep swab, and inability to tolerate her own secretions. Her history revealed Beckwith-Wiedemann Syndome with a previous Wilms tumor, visceromegaly, and surgical repair of an omphalcele at birth. Physical exam findings demonstrated significant macroglossia and right extremity hemihypertrophy.
Results/Findings: Mallampati score of 3 was appreciated. Computed tomography (CT) scan of soft tissue neck showed evidence of right palatine tonsil lesion measuring 1.7 x 1.0cm with concern for early peritonsillar phlegm/abscess formation. With anatomical landmarks obscured by macroglossia, neither direct nor video laryngoscopy were recommended for definitive airway management. Appropriate options include either awake intubation or fiberoptic bronchoscopy. Difficult airways such as this are most successfully managed in a facility with a full panoply of equipment and trained experts to provide the best outcome. Thus, the patient was transferred to a pediatric hospital where fiberoptic bronchoscopy was successfully utilized for intubation and peritonsillar abscess drainage in the operating room.
Conclusions/Implications: Macroglossia in pediatric patients is a clinical indicator of increased likelihood of airway problems and of greater difficulty in managing the airway. Clinicians should consider BWS in pediatric patients presenting with macroglossia, extremity hemihypertrophy, visceromegaly and a history of abdominal defects. Findings may include a higher Mallampati score and, with a peritonsillar abscess, and increased difficulty tolerating secretions.
