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Pseudo Prune Belly syndrome: a case report with unilateral abdominal defect.

ABSTRACT: Prune Belly syndrome occurs in 1/40,000 live births and predominantly in males. It is characterized by triad: cryptorchidism, abdominal wall, and urinary tract abnormalities. Patients with partial or unilateral abdominal wall deficiency, unilateral undescended testis, and female neonates with abdominal wall laxity are classified as Pseudo Prune Belly syndrome. In the 3%-5% of patients with Prune Belly syndrome are affected by Pseudo Prune Belly syndrome, indeed case reports available are very few. We described a case of a male patient born with a large abdominal hernia. Thoracoabdominal X-ray confirmed the large abdominal hernia and revealed a malformation of the rib cage with curved ribs. Magnetic resonance imaging showed thinning of the abdominal wall and ultrasonography detected rectus and oblique muscles hypoplastic with diastasis rectus muscles and stretching of the Hunter's line. Cryptorchidism or urinary tract abnormalities were not detected. The first surgical operation was performed at 2 years of life when the general conditions were stable.

SUBMITTER: De Bernardo G

PROVIDER: S-EPMC6543186 | biostudies-literature | 2019 Aug

REPOSITORIES: biostudies-literature

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Pseudo Prune Belly syndrome: a case report with unilateral abdominal defect.

De Bernardo Giuseppe G Giordano Maurizio M De Brasi Daniele D De Brasi Daniele D Esposito Francesco F De Santis Rita R Sordino Desiree D

Radiology case reports 20190529 8

Prune Belly syndrome occurs in 1/40,000 live births and predominantly in males. It is characterized by triad: cryptorchidism, abdominal wall, and urinary tract abnormalities. Patients with partial or unilateral abdominal wall deficiency, unilateral undescended testis, and female neonates with abdominal wall laxity are classified as Pseudo Prune Belly syndrome. In the 3%-5% of patients with Prune Belly syndrome are affected by Pseudo Prune Belly syndrome, indeed case reports available are very fe ...[more]

PMID: 31193917

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Project description: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.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.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.

Dataset Information

Pseudo Prune Belly syndrome: a case report with unilateral abdominal defect.

Publications

Pseudo Prune Belly syndrome: a case report with unilateral abdominal defect.

Similar Datasets

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