Clinical case: abdominal wall malformation?

[Stefan Johansson]

Dear all,

I have been asked to post the following clinical case born in a Swedish hospital:

Term infant, normal pregnancy and delivery.

The infant has a "ridge" of umbilical-like tissue, stretching from the umbilicus and to sternal tip. The ridge is freely movable from the deeper structure. No secretion. There are no external malformation apart from this ridge. The infant is well, breast-feeding normally, has passed meconium and urine, not vomiting etc.

A plain abdominal x-ray has shown no pathologies.

It seems that the infant has some kind of abdominal wall defekt, but what is the diagnosis? Has anyone seen something similar?

[Stefan Johansson]

Some feedback:

an incomplete and mild form of Pentalogy of Cantrell seems the main differential diagnosis.

This infant has no serious internal malformations, such as cardiac malformation, diaphr hernia etc., and is doing fine.

The pentalogy of Cantrell is a very rare disorder/syndrome characterized by a combination of severe defects of the middle of the chest including the sternum, diaphragm, heart, and abdominal wall. This defect can affect males or females and is apparent at birth or shortly after.

[Mariam]

This is interesting. this is the first time I see such a case

[Guest galaxy_7]

we had a similar case about 2 years ago and turned to be some kind of hemangioma and our vascular surgeon operated on it .. u must search for other hidden visceral hemangiomas

thanx

emad

[Namah_elhouni@yahoo.com]

we had a similar case 10 years ago and we thought of cantrell pentalogy ,our baby had in addition to skin lesion he had bifed sternum with herniation of lung ,and he was operated for this defect,but he developed a picture similar to asphyxiating thorax and start to have difficulty in breathing we send him back to surgeon then we lost contact with him