Anwar Khan, MBBS, MD, Muzammil Hafeez, MBBS, DCH, MRCPCH (UK), Wasim Khan, MBBS, DCH, MRCPCH (UK)

Prune belly syndrome (PBS) is also known as Eagle – Barret syndrome.

It is classically characterized by

• Deficient development of abdominal muscles that causes the skin of the abdomen to wrinkle like a prune.
• Bilateral cryptorchidism.
• Abnormalities of the urinary tract such as bilateral gross hydronephrosis, megaureter and megacystis. (1)

Our case had an unusual presentation with a hugely distended abdomen with smooth, shiny skin due to the presence of massive urinary ascites.

Case:

A 30 weeks gestational age male preterm infant was born by Cesar- ean section to a 33 year old Para 3 mother. Antenatal ultrasound scan showed megacystis, and dysplastic kidneys the bladder was filled with hyperechoic shadows with suspected vesicoenteric fistula. Initially, there was anhydramnios but later normal amount of amniotic fluid was noted. Abdomen showed evidence of ascites on the antenatal scan. A large mass compressed the chest with evidence of pulmonary hypoplasia. ( fig 1 and 2)

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References:

1. Samal SK, Rathod S. Prune belly syndrome – A rare case report, J Nat Sci Biol Med. 2015 Jan-Jun;6(1):255-7.

2. Xu W et al. A case of Prune Belly Syndrome.Pediatric and Neonatology Journal (2015) 56, 193-196

3. Wright JR Jr, Barth RF, Neff JC, et al. Gastrointestinal malformations associated with prune belly syndrome: three cases and a review of the literature. Pediatr Pathol 1986; 5:421

4. Loder RT, Guiboux JP, Bloom DA, Hensinger RN. Musculoskeletal aspect of Prune Belly syndrome, by, Am J Dis Child. 1992 Oct

5. Atılgan T et al. Atypical prune belly syndrome presented with isolated fetal ascites – Archives of disease in childhood 2017; 102: A183.

Disclosures:

No conflicts of interest or financial disclosures relevant to this article are reported.