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Abdominal Defect {Exomphalos}
Question :
Niraj kumar, Shallini Gupta, Deepak kumar, Sanjeev Kumar Digra, Pankaj Kumar Gupta
Department of Pediatrics, Department of Pharmacology, Department of Anesthesiology, Government Medical College, Jammu, India.

Address for Correspondence: Dr Niraj Kumar, Department of Pediatrics, Government Medical College, Jammu, India. Email: nirajkdr@gmail.com

Two full-term vaginally delivered male newborn babies had this abdominal wall defect. In both cases there was no associated cardiac or any other anomaly, no dysmorphic features and no significant antenatal history. What is the diagnosis_?
 
Answer :
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}

References:
1. 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.
2. 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.
3. 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.
4. 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

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

DOI No. : 10.7199/ped.oncall.2012.52