Giant omphaloceles with a small abdominal defect: Prenatal diagnosis and neonatal management

G. Pelizzo, G. Maso, C. Dell'Oste, G. D'Ottavio, R. Bussani, F. Uxa, G. Conoscenti, J. Schleef

Research output: Contribution to journalArticlepeer-review

Abstract

A giant omphalocele is a liver-containing protrusion through an abdominal defect wider than 5 cm in diameter. The giant form with a small abdominal wall defect is a rare condition which, to our knowledge, has not been described previously. We describe three cases with the typical features of elongated vascular liver pedicle and angiomatosis of the hepatic portal system. The abnormal liver organogenesis, due to extra-abdominal development, represented a significant risk factor for hepatic thrombosis after visceral reduction and liver rotation. All the neonates underwent surgery on the first day of postnatal life. One died because of a postoperative liver infarction, and the survivors needed prolonged respiratory support. Prenatal sonographic features, timing, delivery, type of surgical repair, and postnatal outcome are reviewed. A prenatal sonographic diagnosis could be useful to evaluate the abdominal ring and serial ultrasound examinations are recommended to detect promptly ominous signs of hepatic and bowel damage. Color Doppler may be useful to assess the anatomy of the abdominal vessels and their relationships with the herniated organs, although it was not used in any of the cases reported here. This congenital malformation might be considered as a pathological entity separate from giant omphalocele with large abdominal defect, with a severe prognosis due possibly to its different embryological development.

Original languageEnglish
Pages (from-to)786-788
Number of pages3
JournalUltrasound in Obstetrics and Gynecology
Volume26
Issue number7
DOIs
Publication statusPublished - Dec 2005

Keywords

  • Abdominal wall defect
  • Omphalocele
  • Prenatal diagnosis
  • Sonography
  • Surgery

ASJC Scopus subject areas

  • Obstetrics and Gynaecology
  • Radiology Nuclear Medicine and imaging
  • Radiological and Ultrasound Technology
  • Reproductive Medicine
  • Acoustics and Ultrasonics

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