OMPHALOCELE
The term "omphalocele" comes from the Greek words "omphalos" meaning "umbilicus"
and "cele" meaning "cavity". An omphalocele is a cavity at the base of the umbilical cord
filled with abdominal organs. This congenital malformation occurs in about 1 of every 5000 births and was formerly first
detected at the time of birth, but ultrasonography has now made prenatal diagnosis much more common. Between the sixth and
tenth weeks of fetal development, the intestines grow much faster than does the abdominal cavity. This causes the intestines to
protrude into the umbilical cord. If the intestines fail to return to the abdomen by the eleventh week, an omphalocele
develops.
Unfortunately, about half of all children with an omphalocele have at least one associated congenital anomaly. Areas that
must be evaluated include: the spine for scoliosis or hemivertebrae; the intestinal tract for esophageal atresia, small bowel
atresias, duplications or imperforate anus; the heart and lungs for pulmonary hypoplasia, pulmonary artery stenosis, atrial
septal defect or tetralogy of Fallot; and the limbs for club feet or syndactyly. Syndromes which may have an omphalocele as one
of their features include the Beckwith-Wiedemann syndrome, the Pentalogy of Cantrell, and several Trisomy syndromes.
In the majority of cases, a thin shiny membrane covers the protruding organs and the umbilical cord arises from the surface of
this sac rather than from the surrounding skin. The opening in the abdominal wall is usually relatively large. The liver is
always partly within the sac and has a dark purplish color; some or all of the intestines are generally found within the sac as
well. Occasionally the sac will have ruptured prior to or during delivery, and because of the exposed organs, an incorrect
diagnosis of
gastroschisis, will be made.
Although a child with an omphalocele is optimally treated at a children’s hospital having a Level III nursery and
specialists who are familiar with this and similar problems, delivery may take place at any maternity hospital within reasonable
transport distance from a children’s hospital. Extraordinary measures at the time of delivery such as Caesarian section, are
unnecessary. Immediately after birth, an IV should be placed and antibiotics administered to combat infection. Heat and water
are quickly lost from the moist membranes, so they must be wrapped with warm moistened gauze and covered with clean plastic
kitchen wrap to help retain heat and moisture. Additional fluids are given through the IV.
Immediate surgical repair is generally reserved for the smallest defects which have no serious accompanying anomalies or when
the sac is ruptured. Should surgery be necessary, it can nearly always be postponed until the child has been evaluated for the
most serious associated problems and treatment options considered. Larger defects may be difficult to close completely because
the abdominal cavity may be too small, and attempts to force an immediate closure may expose the child to worse risks than the
defect itself. Larger omphaloceles may require gradual reduction by enlarging the abdominal cavity to accommodate the intestinal
contents. A Silastic (plastic) pouch is sometimes placed over the sac to contain the bowel, and the size of the pouch then
reduced daily. This gradually returns the organs to the abdomen, enlarging it slowly until surgical closure is possible; this
process may take ten days or more. Non-operative treatment involves the application of Silvadene cream to toughen the sac and to
allow skin to cover it over a period of several months. Surgery is postponed in these cases for several months to years to allow
the abdominal cavity to enlarge as the baby grows.
The majority of children with an omphalocele who have no other anomalies will go on to lead normal lives. Complications occur in accordance with the severity of the associated defects.