What is Prune Belly Syndrome?

Prune belly syndrome is a serious condition of the abdominal musculature and other parts of the anatomy. It arises in newly-born infants, after it has developed in the womb. It involves a lack of abdominal musculature, kidney and urinary tract problems and undescended testes.[1] The physical appearance of sufferers includes a wrinkled and shrivelled abdominal surface, leading to the name of the condition.

The condition is often regarded as life threatening, affecting life expectancy for sufferers.[2] Surgical interventions addressing PBS problems with the abdominal walls, testes and urinary tract have been found to be helpful for some sufferers. [1] The condition is quite rare: population rates of as little as 1 in 40,000 have been quoted [2].

Prune Belly Syndrome was initially named in 1901 by Williams Osler.[1] It is also known as Eagle-Barrett syndrome, Osler-Parker syndrome and triad syndrome amongst other terms.[1]. Support organisations for sufferers of Prune Belly Syndrome include the Prune Belly Syndrome Network (PBNS).

Causes of prune belly syndrome are as yet still to be established, but there are two main theories. The first is that an intrauterine injury leads to the development of the syndrome. The second is that a blockage of the urinary tract while in utero leads to malformation of the abdominal musculature.[3] According to Ghritlaharey et al, however, these theories do not adequately explain the full range of symptoms associated with Prune belly syndrome.[1]

A small percentage of PBS sufferers are female. (This is quoted in the Ghritlaharey et al study as 3-5% for females.) The term ‘pseudo prune belly syndrome’ has been used for collections of symptoms not quite constituting the requirements to fulfil a clinical diagnosis of prune belly syndrome i.e. for female sufferers and some males.[1]

1 Ghritlaharey R ; Gupta Gaurav ; Kushwaha A ; Chanchlani R. 'Prune belly syndrome associated with incomplete VACTERL ' Journal of Indian Association of Pediatric Surgeons 12:1 (2007):39-41

2 Prune Belly Syndrome Network. 'Prune Belly Syndrome Network: Support and Hope!' www.prunebelly.org (20/10/2009) <http://www.prunebelly.org/Images/pbs%20brochure.pdf>

3 Volmar, Keith E., Nguyen, Tom C., Holcroft, Cynthia J., Blakemore, Karin J., Hutchins, Grover M. 'Phimosis as a cause of the prune belly syndrome: comparison to a more common pattern of proximal penile urethra obstruction. Virchows Archiv. 442:2 (2003):169-172