title: Ex utero intrapartum treatment (EXIT) in the management of cervical lymphatic malformation.
abstract: OBJECTIVE
  The purpose of this study was to review the outcomes and technical details of EXIT procedures performed in fetuses with large cervical lymphatic malformations.
  
  
  METHODS
  A retrospective chart review of fetuses with a prenatal diagnosis of cervical lymphatic malformation evaluated at our center between 1995 and 2013 was performed.
  
  
  RESULTS
  We evaluated a total of 112 fetuses with a prenatal diagnosis of cervical lymphatic malformation. Thirteen of the 112 fetuses (11%) were delivered by an EXIT procedure. Criteria to deliver by EXIT were: 1) deviation/compression/obstruction of the airway, and 2) involvement of the floor of the mouth. Two fetuses developed hydrops. Five fetuses developed polyhydramnios. Eleven EXITs were performed electively at term (n=7; 37-38 weeks) or late pre-term (n=4; 34-36/6 weeks), whereas two patients underwent emergency EXIT at 33 and 38 weeks, respectively. The airway was accessed successfully in 12 of 13 cases. Laryngoscopy only was sufficient in 7, rigid bronchoscopy was required in 4, and 1 required a tracheostomy. In one case with a massive lymphatic malformation of the face, neck, and airway, a tracheostomy was not attempted, and the fetus expired. Four patients had invasion of the larynx by the lymphatic malformation. Five patients required a tracheostomy later. Median time from fetal exposure to intubation was 8 (2-29) min. Median total EXIT time was 105.5 (67-142) min. Median maternal blood loss was 800 (300-1000) ml. Median maternal hospital stay was 4 (3-6) days.
  
  
  CONCLUSIONS
  The EXIT procedure allows controlled airway access in fetuses with cervical lymphatic malformations and evidence of airway impairment on prenatal images.
authors: Center for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia Philadelphia, PA, USA.
authors: Pablo
authors: P
authors: Laje
authors: Center for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia Philadelphia, PA, USA.
authors: William H
authors: WH
authors: Peranteau
authors: Center for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia Philadelphia, PA, USA.
authors: Holly L
authors: HL
authors: Hedrick
authors: Center for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia Philadelphia, PA, USA.
authors: Alan W
authors: AW
authors: Flake
authors: Center for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia Philadelphia, PA, USA.
authors: Mark P
authors: MP
authors: Johnson
authors: Center for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia Philadelphia, PA, USA.
authors: Julie S
authors: JS
authors: Moldenhauer
authors: Center for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia Philadelphia, PA, USA. Electronic address: adzick@email.chop.edu.
authors: N Scott
authors: NS
authors: Adzick
mesh_terms: D000328:Adult; D019468:Disease Management; D005260:Female; D005315:Fetal Diseases / Q000175:diagnosis*; D005865:Gestational Age; D006801:Humans; D007231:Infant, Newborn; D044148:Lymphatic Abnormalities / Q000175:diagnosis / Q000628:therapy*; D042601:Lymphatic Vessels / Q000002:abnormalities*; D008279:Magnetic Resonance Imaging; D008297:Male; D009333:Neck; D011247:Pregnancy; D011296:Prenatal Diagnosis; D012189:Retrospective Studies
keywords: Cervical lymphatic malformation; EXIT; Ex utero intrapartum treatment; Prenatal diagnosis; Tracheostomy