Otocephaly Complex: Case Report, Literature Review, and Ethical Considerations.
Otocephaly complex is a rare and usually lethal syndrome characterized by a set of malformations consisting of microstomia, mandibular hypoplasia/agnathia, and ventromedial malposition of the ears. Those cases that have been diagnosed prenatally have used an ex utero intrapartum treatment procedure to establish a definitive airway. However, prenatal diagnosis continues to be challenging, primarily because of poor diagnostic sensitivity associated with ultrasonography. We present a case of a newborn with an unanticipated otocephaly complex requiring emergent airway management. In this report, we discuss the medical and ethical issues related to the care of a newborn with this frequently fatal condition.
From the *Department of Anesthesiology & Perioperative Medicine, Rutgers New Jersey Medical School, Newark, New Jersey; †Rutgers New Jersey Medical School, Newark, New Jersey; ‡Department of Internal Medicine-Pediatrics, Rutgers New Jersey Medical School, Newark, New Jersey; and §Department of Chemistry, Biology, and Biomedical Engineering, Stevens Institute of Technology, Hoboken, New Jersey.
Jack
J
Diep
David
D
Kam
Farrah
F
Munir
Steven M
SM
Shulman
Glen
G
Atlas
D019465:Craniofacial Abnormalities / Q000175:diagnosis* / Q000628:therapy; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D028761:Withholding Treatment / Q000941:ethics*; D055815:Young Adult