Midline abdominal wall defect (AWD) with visceral extrusion into umbilical cord base
IMAGING
General Features
Radiographic Findings
Fluoroscopic Findings
Ultrasonographic Findings
MR Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Gross Pathologic & Surgical Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Selected References
Revels JW et al: An algorithmic approach to complex fetal abdominal wall defects. AJR Am J Roentgenol. 214(1):218-31, 2020
Chock VY et al: Prenatally diagnosed omphalocele: characteristics associated with adverse neonatal outcomes. J Perinatol. 39(8):1111-7, 2019
Peters NCJ et al: The validity of the viscero-abdominal disproportion ratio for type of surgical closure in all fetuses with an omphalocele. Prenat Diagn. 39(12):1070-9, 2019
Victoria T et al: Fetal anterior abdominal wall defects: prenatal imaging by magnetic resonance imaging. Pediatr Radiol. 48(4):499-512, 2018
Abdelhafeez AH et al: The risk of volvulus in abdominal wall defects. J Pediatr Surg. 50(4):570-2, 2015
Gamba P et al: Abdominal wall defects: prenatal diagnosis, newborn management, and long-term outcomes. Semin Pediatr Surg. 23(5):283-90, 2014
Lakshminarayanan B et al: Abdominal wall defects. Early Hum Dev. 90(12):917-20, 2014
Kelly KB et al: Pediatric abdominal wall defects. Surg Clin North Am. 93(5):1255-67, 2013
Frolov P et al: Clinical risk factors for gastroschisis and omphalocele in humans: a review of the literature. Pediatr Surg Int. 26(12):1135-48, 2010
Mac Bird T et al: Demographic and environmental risk factors for gastroschisis and omphalocele in the National Birth Defects Prevention Study. J Pediatr Surg. 44(8):1546-51, 2009
Vachharajani AJ et al: Outcomes of exomphalos: an institutional experience. Pediatr Surg Int. 25(2):139-44, 2009
Islam S: Clinical care outcomes in abdominal wall defects. Curr Opin Pediatr. 20(3):305-10, 2008
Kamata S et al: Prenatal detection of pulmonary hypoplasia in giant omphalocele. Pediatr Surg Int. 24(1):107-11, 2008
Kumar HR et al: Impact of omphalocele size on associated conditions. J Pediatr Surg. 43(12):2216-9, 2008
Mann S et al: Prenatal and postnatal management of omphalocele. Prenat Diagn. 28(7):626-32, 2008
Blazer S et al: Fetal omphalocele detected early in pregnancy: associated anomalies and outcomes. Radiology. 232(1):191-5, 2004
Hwang PJ et al: Omphalocele and gastroschisis: an 18-year review study. Genet Med. 6(4):232-6, 2004
Wilson RD et al: Congenital abdominal wall defects: an update. Fetal Diagn Ther. 19(5):385-98, 2004
Halbertsma FJ et al: Cardiac diverticulum and omphalocele: Cantrell's pentalogy or syndrome. Cardiol Young. 12(1):71-4, 2002
Macayran JF et al: PAGOD syndrome: eighth case and comparison to animal models of congenital vitamin A deficiency. Am J Med Genet. 108(3):229-34, 2002
Ericson A et al: Congenital malformations in infants born after IVF: a population-based study. Hum Reprod. 16(3):504-9, 2001
Headley BM et al: Left-lung-collapse bronchial deformation in giant omphalocele. J Pediatr Surg. 36(6):846-50, 2001
Puthenpurayil K et al: Pelvic ectopia of the liver in an adult associated with omphalocele repair as a neonate. AJR Am J Roentgenol. 177(5):1113-5, 2001
Koivusalo A et al: Cryptorchidism in boys with congenital abdominal wall defects. Pediatr Surg Int. 13(2-3):143-5, 1998
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References
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KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
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TERMINOLOGY
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Exomphalos
Definitions
Midline abdominal wall defect (AWD) with visceral extrusion into umbilical cord base
IMAGING
General Features
Radiographic Findings
Fluoroscopic Findings
Ultrasonographic Findings
MR Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Gross Pathologic & Surgical Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Selected References
Revels JW et al: An algorithmic approach to complex fetal abdominal wall defects. AJR Am J Roentgenol. 214(1):218-31, 2020
Chock VY et al: Prenatally diagnosed omphalocele: characteristics associated with adverse neonatal outcomes. J Perinatol. 39(8):1111-7, 2019
Peters NCJ et al: The validity of the viscero-abdominal disproportion ratio for type of surgical closure in all fetuses with an omphalocele. Prenat Diagn. 39(12):1070-9, 2019
Victoria T et al: Fetal anterior abdominal wall defects: prenatal imaging by magnetic resonance imaging. Pediatr Radiol. 48(4):499-512, 2018
Abdelhafeez AH et al: The risk of volvulus in abdominal wall defects. J Pediatr Surg. 50(4):570-2, 2015
Gamba P et al: Abdominal wall defects: prenatal diagnosis, newborn management, and long-term outcomes. Semin Pediatr Surg. 23(5):283-90, 2014
Lakshminarayanan B et al: Abdominal wall defects. Early Hum Dev. 90(12):917-20, 2014
Kelly KB et al: Pediatric abdominal wall defects. Surg Clin North Am. 93(5):1255-67, 2013
Frolov P et al: Clinical risk factors for gastroschisis and omphalocele in humans: a review of the literature. Pediatr Surg Int. 26(12):1135-48, 2010
Mac Bird T et al: Demographic and environmental risk factors for gastroschisis and omphalocele in the National Birth Defects Prevention Study. J Pediatr Surg. 44(8):1546-51, 2009
Vachharajani AJ et al: Outcomes of exomphalos: an institutional experience. Pediatr Surg Int. 25(2):139-44, 2009
Islam S: Clinical care outcomes in abdominal wall defects. Curr Opin Pediatr. 20(3):305-10, 2008
Kamata S et al: Prenatal detection of pulmonary hypoplasia in giant omphalocele. Pediatr Surg Int. 24(1):107-11, 2008
Kumar HR et al: Impact of omphalocele size on associated conditions. J Pediatr Surg. 43(12):2216-9, 2008
Mann S et al: Prenatal and postnatal management of omphalocele. Prenat Diagn. 28(7):626-32, 2008
Blazer S et al: Fetal omphalocele detected early in pregnancy: associated anomalies and outcomes. Radiology. 232(1):191-5, 2004
Hwang PJ et al: Omphalocele and gastroschisis: an 18-year review study. Genet Med. 6(4):232-6, 2004
Wilson RD et al: Congenital abdominal wall defects: an update. Fetal Diagn Ther. 19(5):385-98, 2004
Halbertsma FJ et al: Cardiac diverticulum and omphalocele: Cantrell's pentalogy or syndrome. Cardiol Young. 12(1):71-4, 2002
Macayran JF et al: PAGOD syndrome: eighth case and comparison to animal models of congenital vitamin A deficiency. Am J Med Genet. 108(3):229-34, 2002
Ericson A et al: Congenital malformations in infants born after IVF: a population-based study. Hum Reprod. 16(3):504-9, 2001
Headley BM et al: Left-lung-collapse bronchial deformation in giant omphalocele. J Pediatr Surg. 36(6):846-50, 2001
Puthenpurayil K et al: Pelvic ectopia of the liver in an adult associated with omphalocele repair as a neonate. AJR Am J Roentgenol. 177(5):1113-5, 2001
Koivusalo A et al: Cryptorchidism in boys with congenital abdominal wall defects. Pediatr Surg Int. 13(2-3):143-5, 1998
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