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

Hirschsprung's disease has excellent survival to adulthood in non-syndromic cases.

Multi-system functional impairments with associated quality of life impact disproportionately affect female patients with Hirschsprung's disease.

The FINI study was a four-center international cross-sectional study recruiting women with Hirschsprung disease treated as children, aged 20-45.

About 50% of women in the study had ever been or tried to become pregnant.

About 50% of women with Hirschsprung's disease demonstrated an impaired bowel function score and about 16% had a poor score (less than 12 or need for permanent stoma).

The female sexual function index score of less than 26 suggests sexual dysfunction.

There was no statistical difference in overall sexual dysfunction rates between patients and controls, though an increased proportion did not reach statistical significance.

Pain related to intercourse (dyspareunia) was significantly more prevalent in patients compared to controls.

Patients unable to complete the sexual function index due to lack of sexual activity in the last 4 weeks had lower desire subscores than those with confirmed sexual dysfunction, along with lower bowel function scores and more frequent urinary incontinence.

Sexual abstinence may be a symptom prevalent in women with impairment after Hirschsprung's disease surgery.

There was increased incidence of ovarian and adnexal cysts and endometriosis in patients, which may be related to increased detection rate in a population undergoing investigations for other reasons.

There was a 35 times increased prevalence of pelvic inflammatory disease in patients, generally in the absence of any sexually transmitted infection.

Subfertility at 1 and 2 years was increased in patients compared to controls by about 2 to 3 times.

Access to IVF or assisted fertility services was increased 2 to 3 times in patients compared to controls, but not the success with these techniques.

There was no increase in rates of miscarriage or incidence of multiple or recurrent miscarriage in patients compared to controls.

Overall infertility rate was increased approximately 2.5 times in patients compared to controls when intending to conceive a child.

There were no associated factors with subfertility or infertility that could be discerned from the functional or surgical history of patients.

Sterile pelvic inflammatory disease was prevalent in 50% of patients who had subfertility at 2 years.

There was no statistical difference in age of first pregnancy between patients and controls.

The number of live births from the first pregnancy was the same between patients and controls.

There was increased use of cesarean section in patients compared to controls, with the majority being elective or planned.

There was a decreased number of live births per pregnancy in patients compared to controls.

Female patients approaching adulthood should be advised of a potential fertility impact that their condition and its resultant surgery might have.

Teenage girls leaving pediatric care should be advised that if they want to have a family, they shouldn't be waiting and if they're struggling, they shouldn't be struggling for too long.

The infant pelvis is a lot shallower than the adult pelvis and structures are a lot closer together.