Otocephaly Complex: Case Report, Literature Review, and Ethical Considerations. 2016

Jack Diep, and David Kam, and Farrah Munir, and Steven M Shulman, and Glen Atlas
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.

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.

UI MeSH Term Description Entries
D007231 Infant, Newborn An infant during the first 28 days after birth. Neonate,Newborns,Infants, Newborn,Neonates,Newborn,Newborn Infant,Newborn Infants
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D055815 Young Adult A person between 19 and 24 years of age. Adult, Young,Adults, Young,Young Adults
D017809 Fatal Outcome Death resulting from the presence of a disease in an individual, as shown by a single case report or a limited number of patients. This should be differentiated from DEATH, the physiological cessation of life and from MORTALITY, an epidemiological or statistical concept. Fatal Outcomes,Outcome, Fatal,Outcomes, Fatal
D019465 Craniofacial Abnormalities Congenital structural deformities, malformations, or other abnormalities of the cranium and facial bones. Abnormalities, Craniofacial,Abnormality, Craniofacial,Craniofacial Abnormality
D028761 Withholding Treatment Withholding or withdrawal of a particular treatment or treatments, often (but not necessarily) life-prolonging treatment, from a patient or from a research subject as part of a research protocol. The concept is differentiated from REFUSAL TO TREAT, where the emphasis is on the health professional's or health facility's refusal to treat a patient or group of patients when the patient or the patient's representative requests treatment. Withholding of life-prolonging treatment is usually indexed only with EUTHANASIA, PASSIVE, unless the distinction between withholding and withdrawing treatment, or the issue of withholding palliative rather than curative treatment, is discussed. Withdrawing Care,Cessation of Treatment,Withdrawing Treatment,Care, Withdrawing,Treatment Cessation,Treatment Cessations,Treatment, Withdrawing,Treatment, Withholding,Treatments, Withdrawing,Treatments, Withholding,Withdrawing Treatments,Withholding Treatments

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