Prenatally diagnosed giant omphaloceles: short- and long-term outcomes

• Published in: Prenatal diagnosis 2004-06, Vol.24 (6), p.434-439
• Main Authors: Biard, Jean-Marc, Wilson, R. Douglas, Johnson, Mark P., Hedrick, Holly L., Schwarz, Uwe, Flake, Alan W., Crombleholme, Timothy M., Adzick, N. Scott
• Format: Article
• Language: English
• Subjects: Adolescent; Adult; Biological and medical sciences; Birth Weight; Cesarean Section; Delivery. Postpartum. Lactation; Female; Fundamental and applied biological sciences. Psychology; Genetics of eukaryotes. Biological and molecular evolution; Gestational Age; giant omphalocele; Gynecology. Andrology. Obstetrics; Hernia, Umbilical - complications; Hernia, Umbilical - diagnosis; Hernia, Umbilical - mortality; Humans; Infant, Newborn; Magnetic Resonance Imaging; Male; Medical sciences; Molecular and cellular biology; outcomes; Pregnancy; Prenatal Diagnosis; prenatal features; Prognosis; Quality of Life; Respiratory Tract Diseases - complications
• ISSN: 0197-3851; 1097-0223
• DOI: 10.1002/pd.894

Objectives To review prenatal findings, short‐ and long‐term outcomes of giant omphaloceles (GO) managed at a single institution (1996–2001). Methods Prenatal findings and early postnatal outcomes were retrospectively reviewed. Clinical short‐ and long‐term outcomes were analyzed in eight and five children respectively. Parents and physicians of the children were surveyed by written questionnaires about the children's subsequent health issues. (IRB 2002‐2‐2683). Results Seventeen pregnancies with GO were identified: Eight fetuses were live born, four ended in (terminations), two died in utero and three were lost to follow‐up. Live‐born fetuses had prenatal ascites, extreme levocardia and were delivered by cesarean section at a mean of 37 weeks' gestation with a mean birth weight of 2903 g. All neonates required intubation. Two infants (2/8) died within one year. Four of the six survivors had respiratory insufficiency with a mean ventilation time of 76 days. Respiratory and feeding problems complicated the early neonatal course. Long‐term follow‐up was available for five patients (mean age of 33.2 months). Asthma, recurrent pulmonary infections, feeding problems, gastroesophageal reflux and failure to thrive were the major problems. Conclusions Respiratory and feeding problems were the most common neonatal and long‐term medical management issues. Parents need to be counseled prenatally about the probability of multiple surgeries and long hospitalization following birth. Copyright © 2004 John Wiley & Sons, Ltd.