title: Pancreatic aplasia in a fetus with asplenia-cardiovascular defect-heterotaxy (Ivemark syndrome).
abstract: BACKGROUND
  Asplenia or polysplenia and complex cardiovascular defects, in association with disturbed body symmetry and malposition of internal organs, constitute the main corpus of malformations in the heterogeneous group of heterotaxy disorders. In affected pregnancies, prenatal diagnosis is possible by ultrasonography, while prognosis and counseling largely depend upon the severity of the cardiac defect.
  
  
  METHODS
  We present a 25 week gestation fetus with typical findings of asplenia-cardiovascular defect-heterotaxy (Ivemark syndrome) and aplasia of the pancreas.
  
  
  CONCLUSIONS
  Pancreatic aplasia emerges as an additional phenotypic feature in Ivemark syndrome and raises the possibility of total pancreatic insufficiency of the affected neonate as an additional, although rare, clinical consideration.
authors.affiliation: First Department of Pathology, Medical School, National Kapodistrian University, Athens, Greece. ankon@med.uoa.gr
authors.forename: Anastasia
authors.initials: A
authors.lastname: Konstantinidou
authors.forename: Stavros
authors.initials: S
authors.lastname: Sifakis
authors.forename: Ourania
authors.initials: O
authors.lastname: Koukoura
authors.forename: Nikitas
authors.initials: N
authors.lastname: Mantas
authors.forename: George
authors.initials: G
authors.lastname: Agrogiannis
authors.forename: Efstratios
authors.initials: E
authors.lastname: Patsouris
mesh_terms: D000015:Abnormalities, Multiple / Q000175:diagnosis / Q000473:pathology; D000328:Adult; D018376:Cardiovascular Abnormalities / Q000150:complications* / Q000175:diagnosis / Q000473:pathology; D005260:Female; D005333:Fetus / Q000002:abnormalities*; D006801:Humans; D010179:Pancreas / Q000002:abnormalities*; D011247:Pregnancy; D013577:Syndrome; D016216:Ultrasonography, Prenatal