Omphalocele: from diagnosis to growth and development at 2 years of age

Annelieke Hijkoop, Nina C J Peters, Rosan L Lechner, Yolande van Bever, Annabel P J M van Gils-Frijters, Dick Tibboel, René M H Wijnen, Titia E Cohen-Overbeek, Hanneke IJsselstijn
2018 Archives of Disease in Childhood: Fetal and Neonatal Edition  
Objective To compare the prenatal frame of reference of omphalocele (i.e. survival of fetuses) with that after birth (i.e. survival of liveborn neonates), and to assess physical growth and neurodevelopment in children with minor or giant omphalocele up to two years of age. Design We included fetuses and neonates diagnosed 2000-2012. Physical growth (SD scores, SDS) and mental and motor development at 12 and 24 months were analysed using general linear models, and outcomes were compared with
more » ... e compared with reference norms. Giant omphalocele was defined as defect ≥5cm, with liver protruding. Results We included 145 fetuses and neonates. Of 126 (87%) who were diagnosed prenatally, 50 (40%) were liveborn, and 35 (28%) survived at least two years. Nineteen (13%) neonates were diagnosed after birth. Of the 69 liveborn neonates, 52 (75%) survived, and 42 children (81% of survivors) were followed longitudinally. At 24 months, mean [95% CI] height and weight SDS were significantly below 0 in both minor (height: -0.57 [-1.05, -0.09]; weight: -0.86 [-1.35, -0.37]) and giant omphalocele (height: -1.32 [-2.10, -0.54]; weight: -1.58 [-2.37, -0.79]). Mental development was comparable to reference norms in both groups. Motor function delay was found significantly more often in children with giant omphalocele (82%) than in those with minor omphalocele (21%, p=0.002). Conclusions The prenatal and postnatal frame of reference of omphalocele differ considerably; a multidisciplinary approach in parental counselling is recommended. As many children with giant omphalocele had delayed motor development, we recommend close monitoring of these children and early referral to physical therapy.
doi:10.1136/archdischild-2017-314700 pmid:29563149 fatcat:5xqeihlaonbo7gtd4s6y5bqrvq