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.
Pulmonary arterial hypertension (PAH) in pediatric patients is associated with significant morbidity and mortality. Few studies exist to evaluate the incidence of PAH in pediatric oncology patients treated with carboplatin and thiotepa followed by hematopoietic stem cell transplant (HSCT). We describe two pediatric patients who developed PAH following high dose chemotherapy with carboplatin and thiotepa followed by autologous HSCT. These cases highlight the need for a surveillance protocol and reinforce the need for cardiologist-oncologists or close collaboration between providers in these sub-specialties who provide care to patients undergoing this type of chemotherapy regimen.
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