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.