Brainstem tegmental necrosis and olivary hypoplasia: raising awareness of a rare neuropathologic correlate of congenital apnea.

This case study describes an instance of death in an early term female newborn with congenital apnea in the clinical setting of multiple congenital anomalies (retrognathia, posteriorly rotated ears, camptodactyly, and arthrogryposis) and prenatal history of polyhydramnios. Postmortem neuropathologic findings were significant for tegmental necrosis in the caudal pons and medulla characterized by a coalescence of micro-calcifications accompanied by neuronal loss, axonal spheroids, gliosis, and a concomitant hypoplasia of the inferior olives. This report raises awareness of the rare lethal entity of brainstem tegmental necrosis and olivary hypoplasia and its nosological relationship to the Mobius syndrome in the context of differential diagnosis of congenital apnea owing to central respiratory dysfunction. (C) 2014 Elsevier Inc. All rights reserved.