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CLINICAL IMAGES Table of Contents  
Ahead of print publication
Prenatal diagnosis of giant omphalocele on ultrasonography


 Department of Radiology, St. John's Hospital, Bengaluru, Karnataka, India

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Date of Submission23-Jul-2021
Date of Decision11-Aug-2021
Date of Acceptance20-Aug-2021
Date of Web Publication11-Oct-2021
 


How to cite this URL:
Ravikanth R. Prenatal diagnosis of giant omphalocele on ultrasonography. Apollo Med [Epub ahead of print] [cited 2021 Nov 30]. Available from: https://www.apollomedicine.org/preprintarticle.asp?id=327973





  Description Top


We report the case of a fetus with a giant omphalocele and whose perinatal outcome was favorable. This is a 34-year-old woman in labor with no personal or family history. Current pregnancy was followed from 37 weeks of gestation, with discovery of omphalocele prenatally whose diameter was equal to 92 mm at 37 weeks of gestation containing liver [Figure 1]a and [Figure 1]b. Prior antenatal scans were not done, and the patient was completely unaware of fetal anomaly she was about to encounter in her child. The herniated assembly was covered by the amniotic membrane, without fetal ascites visualization. Additional malformations encountered were polyhydramnios and two-vessel umbilical cord [Figure 2]. A cesarean was programmed at 39 weeks of gestation. Omphalocele defect with prosthetic closure was attempted on postnatal day 1. Adequate care was taken to prevent hypothermia. Third space fluid losses were compensated with replacement with crystalloids and colloids. An emergency surgery was organized and the defect was closed by using prosthesis. Postoperative period was uneventful. The baby was admitted for 72 h in the neonatology unit without particular metabolic or infectious complications. Currently, the infant is 12 months old with good psychomotor development.
Figure 1: High-resolution transverse ultrasonography image demonstrating an anterior abdominal wall defect containing a portion of the liver (a). Color Doppler image demonstrating cord insertion onto the apex of the abdominal protrusion, and the course of the umbilical vein through the protruding part of the liver (b)

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Figure 2: High-resolution transverse ultrasonography image demonstrating polyhydramnios and a 2-vessel umbilical cord in a neonate with omphalocele at 37 weeks of gestational age

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  Discussion Top


An omphalocele is an abdominal wall defect believed to result from a folding abnormality during development. It has an incidence of 1 in 4000–5000 live births and is characterized by a defect at the umbilicus through which bowel and other abdominal viscera herniate and are covered by a thin membrane, consisting of amnion, Wharton's jelly, and peritoneal covering.[1] With improved neonatal care and surgical technique, live-born infants without chromosomal abnormalities or associated severe anomalies have a good prognosis.[2] Omphaloceles are frequently classified as small or giant. Omphalocele is associated with major or minor malformation in more than 50% of cases. The most common is cardiac anomalies. Others include musculoskeletal, gastrointestinal, and genitourinary anomalies. Syndromic associations include Beckwith–Wiedemann syndrome, pentalogy of Cantrell, and OEIS complex. Chromosomal anomalies occur in 20%–50% cases of omphalocele. Gastroschisis is a smaller paraumbilical defect usually to the right of midline, containing only bowel loops and not covered by a membrane. Associated anomalies are rare with gastroschisis (unlike with an omphalocele) except for related bowel abnormalities such as intestinal atresia or malrotation. A giant omphalocele is defined as one in which a majority of the liver is contained in the defect.[3] Predictions of overall outcome in giant omphalocele require analysis of complications such as major cardiac anomalies, pulmonary hypertension, polyhydramnios, postnatal sac rupture, and repair other than primary defect.[4] Immediate postoperative complications include increased intra-abdominal pressure and respiratory instability due to diaphragmatic elevation. Other complications include necrotizing enterocolitis, prolonged ileus, respiratory distress, feeding intolerance, gastroesophageal reflux, and neurodevelopmental delay. Advances in surgical therapies, anesthetic techniques, and nursing care have improved the outcome for infants with omphalocele. Survival rate is 75%–95% for infants with small omphaloceles. Complications occur mostly with giant omphaloceles. Infants with associated anomalies and giant omphalocele have a poor outcome.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patients have given their consent for their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Lamquami S, Mamouni N, Errarhay S, Bouchikhi C, Banani A. Antenatal diagnosis of isolated omphalocele. Pan Afr Med J 2015;21:233.  Back to cited text no. 1
    
2.
Hijkoop A, Peters NC, Lechner RL, van Bever Y, van Gils-Frijters AP, Tibboel D, et al. Omphalocele: From diagnosis to growth and development at 2 years of age. Arch Dis Child Fetal Neonatal Ed 2019;104:F18-23.  Back to cited text no. 2
    
3.
Akinkuotu AC, Sheikh F, Olutoye OO, Lee TC, Fernandes CJ, Welty SE, et al. Giant omphaloceles: Surgical management and perinatal outcomes. J Surg Res 2015;198:388-92.  Back to cited text no. 3
    
4.
Nolan HR, Wagner ML, Jenkins T, Lim FY. Outcomes in the giant omphalocele population: A single center comprehensive experience. J Pediatr Surg 2020;55:1866-71.  Back to cited text no. 4
    

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Correspondence Address:
Reddy Ravikanth,
Department of Radiology, St. John's Hospital, Bengaluru - 560 034, Karnataka
India
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/am.am_85_21



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