Prune Belly syndrome (PBS) is a rare congenital anomaly of uncertain etiology almost exclusive to males. We report a case of term male baby born to a 39-year-old grand multipara with previous four normal vaginal births. There was no history of genetic or congenital anomaly in her family. Examination of the baby revealed hypotonia, deficient abdominal muscle, cryptorchidism, palpable kidney, and bladder. Ultrasound examination of the abdomen revealed bilateral gross hydronephrosis and megaureter. Provisional diagnosis of PBS was made and the baby was admitted in neonatal intensive care units for further management. Routine antenatal care with ultrasonography will help in detecting renal anomalies, which can be followed postnatally. Early diagnosis of this syndrome and determining its optimal treatment are very important in helping to avoid its fatal course.
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| http://dx.doi.org/10.4103/0976-9668.149218 | DOI Listing |
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Prune belly syndrome (PBS), or Eagle-Barrett syndrome, is a rare congenital disorder marked by abdominal wall muscle deficiency, urinary tract anomalies, and cryptorchidism, causing significant abdominal wall laxity and functional impairment. This case report discusses an innovative approach to abdominal wall reconstruction in a 19-year-old male patient with PBS and associated conditions, including chronic renal failure and spina bifida. Previously, he underwent distal ureterectomy and vesicoureteral reimplantation at the age of two years to correct urinary tract dilation and bilateral orchiopexy.
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School of Medicine, University of Tasmania, Hobart, Tasmania, Australia.
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