Staged repair of giant omphalocele in the neonatal period

Maurizio Pacilli, Lewis Spitz, Edward M. Kiely, Joe Curry, Agostino Pierro

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Background/Purpose: The aim of this study was to analyze the outcome of giant omphalocele repaired in the neonatal period. Methods: Twelve consecutive (1997-2004) neonates with giant omphalocele (defect >6 cm with liver herniation) were reviewed. A silo of Prolene mesh (Ethicon) was attached to the fascia and the defect was closed without opening the amniotic sac after sequential reduction. In 2 neonates with ruptured omphalocele a plastic sheet was inserted below the mesh. Data are reported as median and range. Results: Gestational age was 38 weeks (range, 32-40 weeks) and birth weight was 2.9 kg (range, 1.0-3.1 kg). The final closure was achieved at 26 days (range, 16-62 days). Three neonates (25%) died before final closure (causes: ruptured omphalocele, lung hypoplasia, cardiac anomalies, and intestinal failure). In the 9 surviving neonates, mechanical ventilation was required for 8 days (range, 2-20 days), hospital stay was 42 days (range, 23-73 days), and full enteral feeding was achieved on day 12 (range, 4-53 days). Complications included wound infection in 5 neonates and midgut volvulus in 1. Prophylactic Ladd's procedure was performed laparoscopically at a later stage in 4 children. At laparoscopy, intraperitoneal adhesions were minimal and the central liver did not preclude the operation. The 9 survivors are all well after 46 months (range, 12-67 months). Conclusions: Giant omphalocele can be safely repaired in the neonatal period without opening the amniotic sac. Intestinal malrotation should be excluded and Ladd's procedure can be performed laparoscopically at a later stage.

Original languageEnglish
Pages (from-to)785-788
Number of pages4
JournalJournal of Pediatric Surgery
Issue number5
Publication statusPublished - May 2005
Externally publishedYes


  • Exomphalos
  • Intestinal malrotation
  • Ladd's procedure
  • Laparoscopy
  • Neonate
  • Omphalocele
  • Outcome
  • Sequential reduction

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