Patient Management
Mary Whitmore, DDS
Resident
Case Western Reserve University — UH Rainbow Babies and Children’s Hospital, Cleveland, OH
Case Western Reserve University
INDEPENDENCE, Ohio, United States
Margaret Elaine Ferretti, DMD, MPH
Program Director
Case Western Reserve University
Rainbow Babies and Children's Hospitals, Case Western Reserve University
Cleveland, Ohio, United States
Margaret Elaine Ferretti, DMD, MPH
Program Director
Case Western Reserve University
Rainbow Babies and Children's Hospitals, Case Western Reserve University
Cleveland, Ohio, United States
Costello syndrome is a very rare genetic condition that occurs with a mutation in the HRAS gene. Symptoms usually include developmental delay, intellectual disability, distinctive facial features, loose folds of extra skin (especially on the hands and feet), unusually flexible joints and heart abnormalities. This report discusses a 26-year-old male with prolonged hospitalization in the PICU at Rainbow Babies and Children’s Hospital with a chief complaint of macroglossia and multifocal pneumonia. The cause of the macroglossia was determined to be multiple intubations due to repeated bouts of pneumonia and self-traumatization. Upon clinical exam, patient was heavily sedated and breathing via tracheostomy. The patient’s tongue was wrapped to decrease swelling and all dentition was buccally displaced and slightly mobile due to size and force of tongue. At the parents and medical staffs request, all dentition was extracted, and tongue was resected by the otolaryngology team under general anesthesia. Patient has recovered well from surgery with reduced pain, but it raises the question if this invasive and extensive treatment can be avoided in the future for patients with macroglossia.