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Prune Belly Syndrome (Triad Syndrome)

The condition was first described by Frolich (1839) and then called "prune belly syndrome" as a descriptive, because the intestinal pattern is evident through the thin protruding abdominal wall in the infant (Osler, 1901).

Survival of the prune belly child depends on the number of functioning remaining nephrons at birth and the operability of the obstruction.

In some cases there are vestiges of muscle in the abdominal wall and it is not known whether this represents (a) destruction of muscle, or (b) failure of development of muscle. The causes of this malformation are little known, but maternal therapy with estrogens in the first trimester has been implicated frequently.

The triad is:

1. Agenesis of abdominal wall muscles
2. Bladder outflow obstruction
3. Bilateral undescended testes

ICD-10 Code: Q79.4 Prune belly syndrome

References

1. Frolich, F. Der Mangel der Muskeln, insbesondere der Seitenbauchmuskeln. Dissertation: Wurzburg (pub.) 1839.
2. Osler, W. Congenital absence of the abdominal muscles with distended and hypertrophied urinary bladder. Bull. Johns Hopkins Hosp. 12: 331-333, 1901.