Omphalocele | |
---|---|
Infant before and after surgical treatment for exomphalos. | |
Specialty | Medical genetics |
Symptoms | Visible organs |
Usual onset | Congenital |
Treatment | Surgical repair |
Prognosis | varies- see below |
An omphalocele or omphalocoele, also known as an exomphalos, is a rare abdominal wall defect.[1] Beginning at the 6th week of development, rapid elongation of the gut and increased liver size reduces intra abdominal space, which pushes intestinal loops out of the abdominal cavity. Around 10th week, the intestine returns to the abdominal cavity and the process is completed by the 12th week.[2][3] Persistence of intestine or the presence of other abdominal viscera (e.g. stomach, liver) in the umbilical cord results in an omphalocele.
Omphalocele occurs in 1 in 4,000 births and is associated with a high rate of mortality (25%) and severe malformations, such as cardiac anomalies (50%), neural tube defect (40%), exstrophy of the bladder and Beckwith–Wiedemann syndrome. Approximately 15% of live-born infants with omphalocele have chromosomal abnormalities. About 30% of infants with an omphalocele have other congenital abnormalities.