Late presentation of ectopia vesica with malignant transformation. A case report and review

INTRODUCTION: Exstrophy of the bladder is a rare congenital anomaly usually treated in neonatal or childhood period. When combined with renal agenesis and presents for the first time in the adulthood with malignant transformation, is an extreme rarity. CASE PRESENTATION: We present a case of 65 years single male who presented with a right irreducible inguinal hernia and an unreconstructed Ectopia Vesicae with fungating tumor. He was anemic with impaired renal function, left renal agenesis and right sided hydronephrosis, hydroureter and distal ureteric stricture. He underwent palliative excision of Ectopia Vesicae and urinary diversion via ureterosegmoidostomy after his condition was optimized. Histopathology showed metaplastic squamous mucosa and a moderately differentiated mucinous adenocarcinoma. Three weeks later he had good continence and normal renal function. He was sent to a distant radio-oncology center for further management. DISCUSSION: In 1851 the first ureterosegmoidostomy for ectopia vesicae was done. Later on it becomes more popular. Some people preferred deferring it until the age of 4 years while others advocates earlier reconstruction. Plastic operation, during neonatal life was also described. In the majority of cases, the fibrotic nature of the bladder and the absence of the sphincter make the reconstruction almost impossible. We performed the only possible option in our setting as our patient had a complex congenital anomalies which present late in life complicated with advanced malignant transformation. CONCLUSION: Despite the plethora of congenital malformation and advanced malignancy, surgical excision and diversion with adjuvant chemo-radiation provided a good palliation for this patient.