A Complex Urogenital Malformation: Urogenital Sinus with Normal Anus

We present a case of a high urogenital sinus with normal anus. Here is a contrast study showing the most common type of urogenital sinus, one with a long urethra and a short common channel. For such a case, a transperineal total urogenital mobilization is appropriate to gain better exposure to the urogenital complex one. May need to perform an astra approach which involves incising the anterior wall of the rectum to gain more exposure. A trans anorectal approach can be performed with extension to include the posterior sagittal incision. Here you see a case with a longer common channel and a short urethra for a urogenital sinus with normal anus. There are two options, a total urogenital mobilization for a short common channel and a long urethra, and a urogenital separation for a long common channel and a short urethra. Here are some diagrams showing the plan for our case in today's video, the urogenital separation. In prone position, the posterior sagittal incision is open. Opened using a trans anorectal approach, the common channel is thereby exposed. The anterior wall of the vagina is lifted up and separated from the urethra below. The vaginal urethral fistula is repaired, creating the neourethra, and here you see the entroidoplasty completed and the repair of the split rectum. We begin with cystoscopy. And the cystoscopy revealed a long common channel and a short urethra. This unique circumstance conveys to us that the plan needs to be to separate the vagina from the common channel and allow the common channel plus the native urethra to become the neourethra. This is the trans anorectal approach. A total body prep is performed and the patient is placed prone. The Lone Star retractor applied. And here you see the anal canal is being marked with silk sutures to facilitate its reconstruction at the conclusion of the operation after it has been split. The perineal body and the anal canal is opened perfectly in the midline. This is the trans anorectal approach done to gain exposure to the eurogenital sinus. It is an extension of the astra concept, which is incision of the anterior anuss only. Incision in the perineal body is now extended, as is the space behind the anus, the posterior sagittal incision. You see, the Foley catheter is in the common channel, and now we are looking for the posterior vagina. I am placing here a wheatlander retractor and further opening the posterior sagula incision. We have found the vagina and now we open it in the midline and the fistula to the common channel is identified. Here you can see the probe showing this vaginal common channel fistula. We use silk sutures to help mobilize the anterior wall of the vagina off of the urinary tract anterior to it. Once the anterior wall of the vagina is mobilized, the fistula is obvious, and it can now be repaired. The midline silk suture marks the location of this fistula. After primarily repairing the posterior urethra in two layers. We are covering that repair with an ischiorectal fat pad. The vagina now is mobilized so that it comfortably reaches the introitis. It is sutured first to the posterior urethral meatus, and then the roidoplasty can be completed. The perineal body is repaired. And the anal canal reconstructed. The anterior wall of the rectum is repaired with sutures placed on the luminal side up to the edge of the anterior aspect of the anal canal. The posterior sagittal incision is now closed. And the operation is completed.