title: Airway implications of congenital sternal agenesis.
abstract: Congenital agenesis of the sternum is an extremely unusual malformation rarely encountered by the practicing Otolaryngologist. It typically arises in conjunction with other midline ventral congenital anomalies, including abdominal, diaphragmatic, and cardiac malformations. We report a case series of two patients managed with tracheotomy placement due to prolonged intubation. The first patient was a 63-day-old infant born at 34 weeks gestation with dysmorphic features, cleft lip and palate, and skeletal dysplasia, including absence of the sternum. The second patient was a 31-day-old infant born with ectopic cordis and diaphragmatic hernia. The inadequate closure of the anterior chest wall secondary to manubrium malformation or a variant contributes significantly to a child's inability to generate adequate ventilatory pressures. As a result, airway management must be considered not only to ensure airway patency but also an appropriate physiological environment to allow for adequate air exchange in the lungs.
authors.affiliation: Division of Pediatric Otolaryngology, Department of Otolaryngology-Head & Neck Surgery, Miller School of Medicine, University of Miami, Miami, FL 33136, USA. rvivero@med.miami.edu
authors.forename: Richard J
authors.initials: RJ
authors.lastname: Vivero
authors.forename: Alexander
authors.initials: A
authors.lastname: Fort
authors.forename: Jose W
authors.initials: JW
authors.lastname: Ruiz
authors.forename: Soham
authors.initials: S
authors.lastname: Roy
mesh_terms: D000015:Abnormalities, Multiple; D017809:Fatal Outcome; D006801:Humans; D007223:Infant; D008297:Male; D011138:Polytetrafluoroethylene; D012121:Respiration, Artificial; D012131:Respiratory Insufficiency / Q000209:etiology; D013249:Sternum / Q000002:abnormalities*; D013524:Surgical Flaps; D019616:Thoracic Surgical Procedures; D014140:Tracheotomy
chemical_list: D011138:Polytetrafluoroethylene