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

Prenat Diagn. 2004 Jun;24(6):434-9. doi: 10.1002/pd.894.

Abstract

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.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Adolescent
  • Adult
  • Birth Weight
  • Cesarean Section
  • Female
  • Gestational Age
  • Hernia, Umbilical / complications
  • Hernia, Umbilical / diagnosis*
  • Hernia, Umbilical / mortality
  • Humans
  • Infant, Newborn
  • Magnetic Resonance Imaging
  • Male
  • Pregnancy
  • Prenatal Diagnosis*
  • Prognosis
  • Quality of Life
  • Respiratory Tract Diseases / complications