congenital umblical hernia

CONGENITALUMBILICAL HERNIA

Omnia M. Korani2011

Definition

a weakness of the baby's abdominal wall where the umbilical cord joins it.

This weakness allows the abdominal contents, mainly the bowel and the liver to protrude outside the abdominal cavity.

Omphalocele. (Membranous Sac)

During fetal life the intestine develops inside the umbilical cord and then usually moves inside the abdomen at

10th week.

Occasionally, the intestine stays inside the umbilical cord and so develops outside the abdominal wall.

Because the contents are lying outside

the abdominal cavity often does not develop and remains small in size.

Congenital umbilical hernia

The presence of an omphalocele is often associated with other malformations.

suggesting that this anomaly is not a simple failure of umbilical ring closure.

Multiple Malformatins_ Frontal Encephalocele_ Dysplastic Ear

Omphalocele - Small Penis

- Spina bifida

Types

Exomphalos major Exomphalos minor

E. Minor E. Major

Defect < 5 cm > 5 cm

Content Intestine Viscera + liver

Covering Layer of wharton’s jelly + amniotic memb.

Amniotic memb. only .

TTT 1ry repair Staged repair

Exomphalos major involving liver, bowel covered by sac

Treatment It’s an emergency operation

as fluids and a lot of body heat are lost through the exposed intestines.

Type of operation depend on the type of the abdominal wall defect (major or minor ).

The goal of the radical cure is to introduce the viscera into an abdominal cavity

they have never occupied before and maintain them there without creating intrabdominalhypertension!!!?

Small exomphalos

‘primary repair’ under general anesthesia.

As a small amount of intestine outside the abdomen it Takes about 30 min.

where the surgeons move the intestines back inside the abdomen and then close up the muscles and skin.

1ry repair

Large exomphalos

a ‘staged repair’ as the exophalos is so large A mesh sack is placed over child’s intestine, which allows it to be contained and protected.

This sack is suspended above the child so that gravity moves the intestine back inside the abdomen.

The sack will be tightened regularly until all the intestine is inside the abdomen. This could take a number of days

The abdominal wall defect in omphalocele is covered with a synthetic membrane

There are Associated factors lead to complications

prematurity,

associated malformations

and finally the risks incurred from the effects of an abdominal hypertension following a hasty and forced reduction of the herniated viscera.

The outcome

Screening

An omphalocele is often detected through a detailed fetal

ultrasound.

Antenatal ultrasound demonstrates a large protroberance from

the anterior abdominal wall, consistent with an omphalocoele.

References

http://congenitalmalformations.blogspot.com/

http://www.gosh.nhs.uk/gosh_families/information_sheets/abdominal_wall_defects/abdominal_wall_defects_families.html

http://en.wikipedia.org/wiki/Omphalocele

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