title: Early neurodevelopmental outcome of infants with high-risk fetal lung lesions.
abstract: OBJECTIVE
  To evaluate the neurodevelopmental outcome of infants with high-risk fetal lung lesions defined as (1) requiring fetal intervention and/or ex utero intrapartum therapy (EXIT), or (2) acute respiratory decompensation postnatally necessitating emergent resection within 48 h of life.
  
  
  METHODS
  We reviewed the medical records of 13 consecutive patients with high-risk fetal lung lesions who were enrolled in our prospective interdisciplinary follow-up program. Neurodevelopmental status was evaluated using the Bayley Scales of Infant Development-III (children ≤3 years, n = 12), or the Wechsler Preschool and Primary Scale of Intelligence-III (children ≥4 years, n = 1).
  
  
  RESULTS
  Eight children (62%) underwent prenatal intervention (EXIT, n = 6; fetal resection, n = 1; intrauterine shunt placement, n = 1), and 5 (38%) required emergent resection postnatally. Median age at evaluation was 25 months (range: 5-80). Average scores for cognitive development were found in all children assessed under 3 years of age. The one child who was tested for cognitive ability at 6 years of age scored in the borderline range of intellectual functioning. For language outcome, 15% scored above average, 54% scored within the average range, and 31% had mild deficits. Overall, 77% scored within the average range for neuromotor outcome, while 23% scored within the mildly delayed range. None of the children had severe delays. Cognitive, language, and psychomotor scores were similar between both groups. Hypotonicity was found in 23%. Autism was suspected in one child who underwent an EXIT procedure and was postnatally diagnosed with mosaic trisomy 18.
  
  
  CONCLUSIONS
  The majority of children with high-risk fetal lung lesions have age-appropriate neurodevelopmental scores.
authors.affiliation: The Center for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia and The University of Pennsylvania School of Medicine, Philadelphia, Pa. 19104, USA.
authors.forename: Enrico
authors.initials: E
authors.lastname: Danzer
authors.forename: Jennifer
authors.initials: J
authors.lastname: Siegle
authors.forename: Jo Ann
authors.initials: JA
authors.lastname: D'Agostino
authors.forename: Marsha
authors.initials: M
authors.lastname: Gerdes
authors.forename: Casey
authors.initials: C
authors.lastname: Hoffman
authors.forename: Judy
authors.initials: J
authors.lastname: Bernbaum
authors.forename: Natalie E
authors.initials: NE
authors.lastname: Rintoul
authors.forename: Alan W
authors.initials: AW
authors.lastname: Flake
authors.forename: N Scott
authors.initials: NS
authors.lastname: Adzick
authors.forename: Holly L
authors.initials: HL
authors.lastname: Hedrick
mesh_terms: D002675:Child, Preschool; D003071:Cognition; D015199:Extracorporeal Membrane Oxygenation; D005260:Female; D005865:Gestational Age; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D015931:Intensive Care, Neonatal; D007804:Language Development; D008171:Lung Diseases / Q000151:congenital* / Q000196:embryology* / Q000628:therapy; D009048:Motor Skills; D053580:Muscle Strength; D009129:Muscle Tonus; D009420:Nervous System / Q000254:growth & development*; D011247:Pregnancy; D011296:Prenatal Diagnosis; D012121:Respiration, Artificial; D016216:Ultrasonography, Prenatal