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Open Access Case report

A case of ultrasound-guided prenatal diagnosis of prune belly syndrome in Papua New Guinea – implications for management

Maria Ome1, Regina Wangnapi1, Nancy Hamura2, Alexandra J Umbers1, Peter Siba1, Moses Laman1, John Bolnga2, Sheryle Rogerson3 and Holger W Unger1*

Author Affiliations

1 Papua New Guinea Institute of Medical Research (PNG IMR), Vector Borne Disease Unit (VBU), P.O. Box 378, Madang 511, Papua New Guinea

2 Department of Obstetrics and Gynaecology, Modilon General Hospital, P.O. Box 2119, Madang, 511, Papua New Guinea

3 Royal Women’s Hospital, Melbourne, VIC, 3052, Australia

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BMC Pediatrics 2013, 13:70  doi:10.1186/1471-2431-13-70

Published: 7 May 2013

Abstract

Background

Prune belly syndrome is a rare congenital malformation of unknown aetiology and is characterised by abnormalities of the urinary tract, a deficiency of abdominal musculature and bilateral cryptorchidism in males. We report a case of prune belly syndrome from Papua New Guinea, which was suspected on pregnancy ultrasound scan and confirmed upon delivery.

Case presentation

A 26-year-old married woman, Gravida 3 Para 2, presented to antenatal clinic in Madang, Papua New Guinea, at 21+5 weeks’ gestation by dates. She was well with no past medical or family history of note. She gave consent to participate in a clinical trial on prevention of malaria in pregnancy and underwent repeated ultrasound examinations which revealed a live fetus with persistent megacystis and anhydramnios. Both mother and clinicians agreed on conservative management of the congenital abnormality. The mother spontaneously delivered a male fetus weighing 2010 grams at 34 weeks’ gestation with grossly abnormal genitalia including cryptorchidism, penile aplasia and an absent urethral meatus, absent abdominal muscles and hypoplastic lungs. The infant passed away two hours after delivery. This report discusses the implications of prenatal detection of severe congenital abnormalities in PNG.

Conclusion

This first, formally reported, case of prune belly syndrome from a resource-limited setting in the Oceania region highlights the importance of identifying and documenting congenital abnormalities. Women undergoing antenatal ultrasound examinations must be carefully counseled on the purpose and the limitations of the scan. The increasing use of obstetric ultrasound in PNG will inevitably result in a rise in prenatal detection of congenital abnormalities. This will need to be met with adequate training, referral mechanisms and better knowledge of women’s attitudes and beliefs on birth defects and ultrasound. National medicolegal guidance regarding induced abortion and resuscitation of a fetus with severe congenital abnormalities may be required.

Keywords:
Congenital abnormality; Eagle-Barrett syndrome; Management; Papua New Guinea; Prenatal diagnosis; Prune belly syndrome; Ultrasound