- 2-month-old male presents for evaluation. He was born at 37 weeks gestation.
- He was noted to have urine and stool coming out from a penoscrotal hypospadias.
- Prenatally, he was diagnosed with solitary right kidney.
- He also had VSD, hemivertebrae, hydronephrosis of the right kidney with megaureter and agenesis of the left kidney.
- Colostomy and vesicostomy were placed on day 3 of life.
- Distal colostogram showed a fistula.
- Cystoscopy was performed.
- Insertion of the distal ureter was identified immediately below the bladder neck.
- Electrostimulator was used to evaluate the anus, which had good contraction. The distal rectal length was normal.
- This is a complex anomaly.
- The anus must be preserved in order to give the patient the best prognosis for bowel control.
- Posterior sagittal trans-anorectal would be an ideal approach to repairing this type of malformation. This would create a “T” anastomosis.
- Having solitary kidney, reflux and the ureter below the bladder neck is concerns for future urological function. A reimplant may be indicated. The vesicostomy should remain in place. The kidney function should be followed closely.
- Colostomy should be performed on this type of complex malformation.
- Laparoscopy is a useful technique for bladder neck fistulas.
Patient Case Discussion