- Exomphalos and gastroschisis are two different congenital anomalies
- Differ markedly in their clinical appearance
- Overall incidence is approximately 1: 3000 live births
- Usually diagnosed prenatally on ultrasound
- Exomphalos and gastroschisis can usually be differentiated prenatally
- Do not inevitably require delivery by caesarian section
Exomphalos (omphalocele)
- An omphalocele always has a sac
- The sac may be intact or ruptured
- It has three layers - peritoneum, Wharton's jelly and amnion
- Umbilical cord arises from apex of sac
- Sac contains intestinal loops, liver, spleen and bladder
- Often associated with other major congenital anomalies
- Prognosis depends on theses associated anomalies
- Mortality is approximately 40%
Picture provided by Osama Bawazir, Alberta Children's Hospital, Calgary, Canada
Treatment
- No consensus exists on the optimal management of large unruptured omphaloceles
- Treatment depends on the size of the lesion
- Aims of treatment are to reduce contents into small abdominal cavity
- If bowel is covered there is no urgency to do this
- Treatment options are both surgical or conservative and included
- Biological dressings
- Polymer films
- Direct surgical closure
- Skin flap closure
- Small defects can usually be closed surgically
- Surgical closure of large defects may require staged procedures
- Overzealous reduction can result in caval compression
- After conservative treatment a ventral hernia repair may be required at about one year of age
Gastroschisis
- A gastroschisis never has a sac
- Umbilical cord arises from normal place in abdominal wall
- Usually to the left of the abdominal wall defect
- Evisceration usually only contains intestinal loops
- Rarely associated with major congenital anomalies
- But may be associated with intestinal atresia
- Infants have better prognosis than those with an omphalocele
- Mortality is approximately 10%
Treatment
- Can often be treated by direct full-layer closure of abdominal wall
- May be associated with postoperative gut dysfunction
- Usually require postoperative nutritional and ventilatory support
Bibliography
Langer J C. Abdomnal wall defects. World J Surg 2003; 27: 117-124.
Segel S Y, Marder S J, Parry S, Maconers G A. Fetal abdominal wall defects and mode
of delivery: a systematic review. Obstet Gynecol 2001; 98: 867-873. |