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Prenatally diagnosed giant omphaloceles: short- and long-term outcomes
Jean-Marc Biard1, R Douglas Wilson, Mark P Johnson
1The Center for Fetal Diagnosis and Treatment at The Children's Hospital of Philadelphia, Philadelphia 19104-4399, USA.
Insights
Giant omphalocele (GO) management presents significant neonatal and long-term challenges, primarily respiratory and feeding issues. Prenatal counseling should address potential surgeries and extended hospitalizations for affected infants.
Area of Science:
- Perinatal Medicine
- Neonatology
- Pediatric Surgery
Background:
- Giant omphalocele (GO) is a complex congenital anomaly requiring specialized management.
- Understanding prenatal findings and long-term outcomes is crucial for optimizing care.
Purpose of the Study:
- To review prenatal findings and short- and long-term outcomes of giant omphaloceles (GO).
- To analyze clinical outcomes and parental/physician-reported health issues in infants with GO.
Main Methods:
- Retrospective review of prenatal findings and early postnatal outcomes.
- Analysis of short- and long-term clinical outcomes in eight and five children, respectively.
- Survey of parents and physicians regarding subsequent health issues.
Main Results:
- Seventeen pregnancies with GO were identified; eight fetuses were live-born.
- Live-born infants experienced prenatal ascites, levocardia, and required intubation.
- Survivors faced significant respiratory insufficiency, feeding problems, asthma, and recurrent infections.
Conclusions:
- Respiratory and feeding difficulties are the most common neonatal and long-term issues in GO management.
- Prenatal counseling must inform parents about the likelihood of multiple surgeries and prolonged hospitalizations.
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.