title: Brainstem tegmental necrosis and olivary hypoplasia: raising awareness of a rare neuropathologic correlate of congenital apnea.
abstract: 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 microcalcifications 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 Möbius syndrome in the context of differential diagnosis of congenital apnea owing to central respiratory dysfunction.
authors.affiliation: Department of Pediatrics, Drexel University College of Medicine, St. Christopher׳s Hospital for Children, Philadelphia, PA; Department of Pathology and Laboratory Medicine, Drexel University College of Medicine, St. Christopher׳s Hospital for Children, Philadelphia, PA; Department of Neurology, Drexel University College of Medicine, Philadelphia, PA. Electronic address: Christos.Katsetos@drexelmed.edu.
authors.forename: Christos D
authors.initials: CD
authors.lastname: Katsetos
authors.affiliation: Department of Pediatrics, Drexel University College of Medicine, St. Christopher׳s Hospital for Children, Philadelphia, PA.
authors.forename: Carol E
authors.initials: CE
authors.lastname: Anderson
authors.affiliation: Department of Pathology and Laboratory Medicine, Drexel University College of Medicine, St. Christopher׳s Hospital for Children, Philadelphia, PA.
authors.forename: Miguel A
authors.initials: MA
authors.lastname: Guzman
authors.affiliation: Department of Pediatrics, Drexel University College of Medicine, St. Christopher׳s Hospital for Children, Philadelphia, PA; Department of Pathology and Laboratory Medicine, Drexel University College of Medicine, St. Christopher׳s Hospital for Children, Philadelphia, PA.
authors.forename: Judy Mae
authors.initials: JM
authors.lastname: Pascasio
authors.affiliation: Department of Pediatrics, Drexel University College of Medicine, St. Christopher׳s Hospital for Children, Philadelphia, PA; Department of Pathology and Laboratory Medicine, Drexel University College of Medicine, St. Christopher׳s Hospital for Children, Philadelphia, PA.
authors.forename: Jean-Pierre
authors.initials: JP
authors.lastname: de Chadarévian
authors.affiliation: Department of Pediatrics, Drexel University College of Medicine, St. Christopher׳s Hospital for Children, Philadelphia, PA; Department of Neurology, Drexel University College of Medicine, Philadelphia, PA.
authors.forename: Agustin
authors.initials: A
authors.lastname: Legido
mesh_terms: D000015:Abnormalities, Multiple / Q000473:pathology; D001049:Apnea / Q000151:congenital* / Q000473:pathology*; D001927:Brain Diseases / Q000151:congenital* / Q000473:pathology*; D001933:Brain Stem / Q000473:pathology*; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D009336:Necrosis