Abdominal Defect {Exomphalos}

Exomphalos. It encompasses a spectrum of abnormalities ranging from a small defect with the gut prolapsing into the cord {a hernia into the cord} through umbilical defects less than 5cm wide {Exomphalos minor} to larger umbilical defects {Exomphalos major or giant omphalocele}. {1} An Omphalocele is a herniation or protrusion of the abdominal contents into the base of the umbilical cord. The herniation of intestines into the cord occurs in about 1 in 5000 births and herniation of liver and intestines in 1 in 10000 births. {2} Although many are isolated defects, some are part of constellation of malformation {such as Beckwith- Weidman syndrome or Trisomy 18}, and a few are associated with maternal intake of valproic acid. {3} Prenatal ultrasound detects these defects after 14 weeks gestation as during 1st trimester midgut normally is herniated. {3,4} Surgical closure of the defect may be accomplished with a primary closure or a staged repair once the neonate is hemodynamically stable. {4} Urgent surgery is not indicated for the exomphalos with an intact amniotic sac. Ruptured exomphalos requires urgent surgery. {1}

E-published: August 2012 Vol 9 Issu 8 Art # 52

References :

Stringer DA, Sugarman I, Smyth AG. Congenital Defects And Surgical Problems. In: Rennie JM (ed). Roberton's Text Book of Neonatology. 4th Edn. Elsevier, Churchill Livingstone, 2005: 733-734. Stoll BJ. The Umblicus. In: Kliegman RM, Jenson HB, Behrman RE, Stanton BF (eds). Nelson Textbook of Pediatrics. 18th Edn. Saunders, Elsevier, Philadelphia, 2007:776. Berseth CL, Poenaru D. Abdominal wall problems. In: Taeusch HW, Ballard RA, Gleason CA (eds). Avery's Diseases of the Newborn. 8th Edn. Elsevier, Saunders, Philadelphia, 2005: 1113-1117. Azizkhan RG, Frykman PK. Abdominal Wall Defects. In: Rudolph CD, Rudolph AM, Hostetter MK, Lister G, Siegel NJ (eds). Rudolph's Pediatrics. 21st Edn. 2003:1399-1400