Shumyle Alam, M.D., a pediatric urologist at the Medical University of South Carolina, discusses a vaginoplasty and bladder revision case to show the importance of a holistic vision for pediatric patients with complex bladder, kidney, pelvic, and genital conditions, especially as they navigate the transition from adolescence to adulthood. Alam also discusses reproductive potential in patients who have undergone extensive corrections of the genitourinary tract since childhood, a conversation that was not even possible a decade ago.
Mhm. My name is small. Um I'm a pediatric urologist here at M. U. S. C. I. Specialized in complicated generally urinary disorders and reconstruction of the general urinary tract as well as participate in kidney transplant and the care of Children with multiple medical needs. With regards to urology taking care of complicated patients. I intend to see patients from all walks of life and in pediatrics on occasion we do have a little bit of internal myopia where we think a reconstruction is going to go very well. But as the child ages into adulthood, sometimes the needs change. I've been very lucky in my practice that some of these older patients will reach out to me and we can assess them on a case by case basis to see if surgeries need to be revised or if there's other issues that need to be addressed in this particular instance. This is a child who was born with what's called a clerical malformation and she underwent a very good operation when she was a baby. But unfortunately as she became a young adult, some of the things that she needed to move on with her life were not possible. She had some scar tissue at the level of her vagina which concluded even the use of a tampon. And of course this is not really compatible with what a young 27 year old lady would like to do. Young women with this condition don't always know that there are options. She was very upset by the fact that she could not even use a tampon. But she did not also realized that there's a cosmetic approach that can be used to restore some of that um structural anatomy as well as cost basis as Children with this condition age. There are certain aspects of their general urinary system which can show problems with time. And she had two issues. One was clearly a cosmetic and functional issue regarding her vagina and her enjoyable anatomy. The second however was a more serious issue regarding her bladder. She had suffered from multiple urinary tract infections. She had suffered from multiple hospitalizations. She had significant gi issues which unfortunately had not been adequately addressed and she had unfortunately undergone a colostomy to mitigate those problems. All these problems are actually fixable without hospitalizations and colostomy. However she had seen multiple providers and wasn't really in a center that had expertise in dealing with this condition. And so sometimes when you see providers who are very good at the individual components that they treat, they're not always as well versed in taking care of a patient from a holistic standpoint. And when she arrived she had a colostomy. She had scar tissue in her vagina. She had an undiagnosed neurogenesis bladder and she required management of pretty much everything. She was quite unhappy with her colostomy. She was unhappy with her vagina of course and she was very tired of being admitted to the hospital with multiple infections and that's how she presented to us and the surgery that we embarked upon. Was really only after a period of time of observation, testing and study to get to the um the fix if you will. That that she requires. When we took her to the operating room and examined her under anesthesia, we found that she had a clinical variant known as uh what's called a posterior America. And that term was coined by a gentleman by the name of Alberto pena who really led the forefront and the that kind of started the management the modern management of calico. And that means she had a very thick and pubis. Her urethra was oriented a little bit posterior lee and her vaginal opening was very narrow and also impacted by the pubis and her rectum was in the proper position but was d functional eyes because of her colostomy. We started the procedure by looking inside her bladder and documented that she in fact had suffered from multiple infections which had changed her bladder anatomy. And we put a super pubic tube in just to mitigate things because it was very difficult for her to categorize and it would be even more difficult. After surgery, when we evaluated her vagina, we found that she had a circumferential stenosis or psychiatric at the level of the perineum with hair bearing skin. This was not something that would have been amenable to dilation and dilation in the past have been very painful for her. And so she required a revision surgery to repair this And so the first stage of the operation is we actually removed the site metrics by advancing the vagina. So removing the unhealthy hair varying tissue and actually mobilized the vagina such that we could bring the vagina forward to a normal location at the level of the perineum. In addition, we were able to move her labia menorah to create a more cosmetic appearance of her vaginal opening or her android. This and at the same time create actually a hood for her clitoris. Which in the case supposed to europe America is a good distance away from the urethra uh compared to normal anatomy. And sometimes it is uh overstimulated in certain situations. And by doing this procedure we were able to hopefully create a more functional um use for her in the future as well as from cosmetic approach. She now has normal external external genitalia, reproduction is the part that's a little bit um more complicated. She does have internal anatomy that is duplicated. She actually has two uteruses or you terry, she has normal ovaries and normal fallopian tubes. But sometimes these situations complicate pregnancy. There's a risk of ectopic pregnancy. There's a risk of uterine rupture. But with again more of a holistic approach and am FM involvement and high risk involvement in a center that takes care of this condition. We have had successful pregnancies in patients with clasico. And that is something that we've had over the course of the last decade, Not the last 2030 years. And so when this child was born she was actually told by her treating physicians that she could never have Children. And that's actually not true. She possesses the plumbing so to speak to have Children. But it will have to be sort of carefully followed and managed. So I think the important aspects of the procedure is that if you take it down to its component parts and say we performed a labial plastic or cosmetic procedure of the vagina. Many centers will say that yes that is something that their physicians are able to do and that's actually a true statement. But when you combine that with the condition like she has called WAka or if you combine that with someone who's had previous surgery which hasn't been successful. Some of the techniques are a little bit more specialized and probably uh it would be wise to do them in the center that has expertise. And here at M. U. S. C. We have all the pieces in place to be able to provide holistic care for a patient who is seeking these fixes to their problems including patients who've had surgeries in the past that may require some revisions. Only yesterday I actually had the opportunity to see her post operatively and we took her to the operating room to change the super pubic tube, evaluate her bladder and to evaluate the functional and cosmetic results of the repair. I'm happy to report that sick tricks is completely gone. She was able to accommodate the largest Hagar dilator that we have which means that her enjoy. This is open. She will not need to do dilation for the rest of her life. She will need to do it initially and then it will stay open with regards to her urethra because we mobilize the vagina. She's actually now Catherine visible and is currently, as we speak, undergoing catheterization. Teaching in the clinic to see if we can remove the super pivot tube. Should that fail, we will proceed with another means for her to categorize. And um we are now starting the process of addressing her colostomy and potentially reversing that to allow her more Cosmetic functional anatomy that's consistent with the life that she probably wants to live as a 27 year old.