Joseph Davidson, MA(Oxon), MBBS MRCS(Eng) - Best of the Best in Pediatric Surgery 2024

So next up in this heat, we've got Doctor Joseph Davidson, who's a heavy hitter in this competition because not only was he selected to represent UUSA, but also selected to represent Baps, and he's presenting on sexual function and fertility outcomes in women with Hirschsprung's disease. So let's see what this one says. Hello and thank you for the opportunity to speak with you all today. Uh, my name is Joe Davidson and I am a, uh, research fellow at Great Ormond Street Hospital for Children and I'm delighted to be able to speak on behalf of my colleagues, uh, across our collaborating centers. And we're going to be presenting some of the work from our, uh, collaborative multicenter study, which was presented at both the BAPS and UPA congresses in the summer of last year. So as we all know, Hirschpring's disease is a condition with excellent survival to adulthood in non-syndromic cases, but we do know from our own work and from that of others that there are multi-system functional impairments with an associated quality of life impact which disproportionately affects female patients. Recent reports have suggested that female patients with Hirschpring disease may have issues surrounding their fertility, with population level data suggesting that they may be older at their first child and they may have fewer children than healthy controls in the general population. Female fertility and sexual function is a very difficult subject to study in Hirshspring specifically because this is a rare condition that affects men more than women. Not only that, but female sexual function is something with a complex interplay of somatic and psychosocial components which may or may not be related to an underlying condition. Our study was a four-center international cross-sectional study, which we called FINI. We recruited women with Hirschpring disease who'd been treated as children at one of our four centers between the ages of 20 and 45. We collected data on their bowel function, sexual function, and relationships, any fertility and obstetric history, and we compared our patients to women from the general population. Our control population and patient population were well matched in terms of the demographics shown here, and we saw that about 50% of women had ever been or tried to become pregnant. The bowel function score that we saw in these women was fairly similar to what we would expect when examining a cohort of women who are adults with Hirschprung disease, with about 50% of patients demonstrating an impaired score and about 16% of patients with a poor score, which is defined here as a bowel function score of less than 12 or the need for a permanent stoma. The female sexual function index. is a validated multilingual questionnaire which assesses female sexual function across a number of subdomains, and an overall score of less than 26 would suggest sexual dysfunction. We saw no statistical difference between the distribution of score across the two cohorts, and although there was an increased proportion of women with sexual dysfunction, this did not reach statistical significance. Interestingly, when we looked at the subdomains associated with this index, we saw no statistical difference in any of them except for pain related to intercourse, which was more prevalent in patients compared to controls. We looked at patients who were unable to complete the entire index for lack of having had sexual activity in the last 4 weeks, and what was interesting was examining these patients. They appeared to have a lower desire sub score, which is the one part of the questionnaire they can complete, than those patients with confirmed sexual dysfunction. They also had lower bowel function score and more frequent urinary incontinence, suggesting that sexual abstinence may well be a symptom that is prevalent in women with impairment after their Hirschpring's disease surgery. In terms of gynecological comorbidity, we saw an increased incidence in ovarian and adnexal cysts and endometriosis, which of course may be related to an increased detection rate in a population of women who are undergoing investigations for other reasons. What was particularly interesting, however, was a 35 times increased prevalence of pelvic inflammatory disease, generally in the absence of any sexually transmitted infection. In terms of fertility outcomes, we saw that subfertility at 1 and 2 years was increased in patients compared to controls by about 2 to 3 times, as was the access to IVF or assisted fertility services, but not the success with these techniques. We saw no increase in the rates of miscarriage incidence of multiple or recurrent miscarriage in patients related to controls, but overall, when intending to conceive a child. We saw an increased infertility rate of approximately 2.5 times in patients compared to controls. What was important to note was that there were no associated factors with subfertility or infertility that could be discerned from the functional or surgical history of these patients. We did, however, notice that sterile pelvic inflammatory disease was prevalent in 50% of the patients who had subfertility at 2 years. In terms of the pregnancy outcomes in this population, we saw no difference between patients and controls statistically in terms of the age of their first pregnancy. And the number of live births from the first pregnancy was the same. We did note that there was an increased use of cesarean section compared to controls, and this seemed to be made up by the majority of them being elective or planned. In terms of pregnancy outcomes overall, there was a decreased number of live births per pregnancy in patients compared to controls, and the difference in the use of elective cesarean sections persisted. In summary, we saw that overall rates of sexual dysfunction in patients were similar to the general population, but there seems to be a higher incidence of dysparyunia in these patients, and that patients with sexual dysfunction seem to have poor functional outcomes. We noted a 3 times increased subfertility rate in patients compared to controls, with a clear burden of pelvic adhesions which one must assume to be related to the surgery that they have had as infants. We note that pregnancy outcomes are not the same compared to controls with fewer live births overall and an increased use of elective cesarean section. We believe that female patients approaching adulthood should be advised of a potential fertility impact that their condition and its resultant surgery might have. Really appreciate the opportunity to speak to you all, and I would welcome any discussion or questions. Thank you very much. Thank you for such an amazing uh presentation. I feel like we've had quite the theme of looking kind of long term at these pediatric, uh, but we're really just small children conditions that now we really need to think about how they impact kids as they're growing up and turning. Into young adults. Um, do you feel like there's any benefit in having this subset of patients have early contact with fertility specialists or a pediatric gynecologist to kinda help, um, prevent, uh, some of this or get involved in preventative therapies? Uh, I, I think that's a, uh, a really good point. Um, I think that one of the things that you really want to try and ensure is that you're not creating a wave of mass hysteria among patients who may or may not have a problem at too early a stage, and I think we've talked about this within our group, our sort of multi-institutional group that we have, um, as to what a sort of reasonable course of action would be, um, and I think. One of the things that would be very sensible to do is that any teenage girl who is sort of leaving your practice uh and, and gonna be moving on to an adult care practitioner should be advised that if, if she wants to have a family, that she shouldn't be waiting and if she's struggling, she shouldn't be struggling for too long because these things are, are, are, are clearly quite prevalent and I think that um uh being aware of the fact that the surgery that they've had could, could have this sort of an impact is, is, is, is definitely relevant. That's great. So what do you think? Hey, Joe, uh, great paper. And again, I agree, Brittany, that I love seeing this new, uh, or growing approach of looking at long-term follow-up. Um, the dysperunia, um, any thoughts on why there's dysperunia? Yeah, I think that the um, the, the sort of working assumption with that, which is sort of carried over from the, the pouch surgery literature, is that when, when you form adhesions in the pelvis in the sort of retrovaginal space, you, you change the angle of the cervix, and, and that can lead to just, it, it's one of the reasons why they, why they suppose that, that, the pouch patients might be having problems conceiving. We haven't been able to do that sort of level of detailed anatomical study here. Um, but certainly if you're changing the anatomy of, of, of the vaginal canal, that's, that's likely to make receptive intercourse more painful. And, uh, uh that's what I, and that's interesting is, is, if we think that's the case, I wonder if, as we move forward, we pay more attention to mitigating that. Cynthia Reyes asks, if you think MIS will affect any of this, that, you know, the, the sterile pelvic inflammatory disease rate may go down, maybe even uh the surgical technique about leading, you know, mitigating dyspunia as well. What are your thoughts on the role of MIS in these numbers? I mean, I hope it does because I think that the, the rate that we see with patients who've had surgery 30 years ago is higher than any of us would like it to be. Um, it may be that it's unavoidable, we, we won't know until people repeat these studies in 10 years' time. Um, I think that, um, the data from pouch surgery is encouraging, so they adopted minimally invasive pouch surgery, you know, a while ago, and, and it seems as though the fertility impact is still there, but it's, it's less so. Um, obviously the, the, the infant pelvis is a, is a rather different beast, it's a lot shallower and these structures are a lot closer together. Um, but, but I do hope that it, it, it might make a difference moving ahead. It's fascinating, and we would have to, I would love to look at that over time and see if we can start getting these numbers to improve. Joe, thank you so much, man. Good job. Thanks. All right.