Exstrophy-Epispadias Complex Variants: A Hybrid Case
A full-term male newborn (birth weight: 3410 g) with a prenatal diagnosis of single umbilical artery presented with an abdominal wall lesion consistent with bladder exstrophy. On physical examination, there was a normally inserted umbilical cord and below it, an abdominal midline defect with a mucosal plate, with no urine leakage. Urine output was normally per-urethral. As no fistulous tract was found after trying cannulation and no methylene blue spillage was noted after retrograde bladder filling, communication between mucosal plate and bladder or ureters was initially excluded. An initial diagnosis of true duplicate bladder exstrophy was therefore made. The patient had normal male external genitalia, normally positioned and conformed anus, and no other macroscopic malformations.

Abdominal ultrasound (US) showed normal kidneys, a left ureter with a 3 mm distal tract dilatation, and a normal bladder. A symphysis pubis diastasis was described on plain X-ray. To confirm urinary tract anatomy, also a voiding cystourethrogram was performed: no communication between the normal bladder and the abdominal lesion was documented; a first-grade left vesicoureteral reflux was described. During the next days, urine leakage was noted but, even if the everted mucosa was wet, still no fistula was found. In order to clarify the diagnosis (true bladder exstrophy duplication versus superior vesical fistula), we decided to perform a computed tomography urography (CTU): no fistulous tract was documented. At the age of 14 days, the baby was taken to the operating room. An initial cystoscopy showed normal urethra and bladder and a left ectopic ureteral meatus located at the bladder neck. A dot-like solution of continuity was observed at the bladder dome, consistent with a possible urinary fistula. The exstrophy bladder plate was isolated and a tubular connection between this and the normal bladder was found, but no communication between the structures were confirmed. Both the bladder plate and its connection were excised and the bladder wall reconstructed. A tension-free abdominoplasty was performed without the necessity of an osteotomy; symphysis pubis was approximated with non-absorbable stitches. The post-operative course was uneventful and patient was discharged on the sixth postoperative day. The histopathologic examination was consistent with bladder mucosa and did not describe any fistulous tract. There were no complications during the outpatient follow-up and urinary tract US, performed after one month from surgical correction, was normal.