Blackburn, SC

Cross, K

Curry, J

Davidson, JR

De Coppi, P

Eaton, S

Kyrklund, K

Pakarinen, MP

Thompson, DS

English

UCL Discovery

Research Letter Sexual Function, Quality of Life and Fertility appear to be affected in women operated for Hirschsprung’s Disease in childhood  Hirschsprung’s Disease (HSCR) is a congenital disorder characterised by an aganglionic segment of distal intestine. Patients typically undergo pull-through surgery to remove affected bowel in infancy. Long-term functional and quality of life has been reported for these patients1, however little objective study of sexual function has been attempted2, especially in women (HSCR has a 3:1 male preponderance.)  Adult patients completed multi-domain sexual function, fertility and sexual quality of life questionnaires as part of a comprehensive long-term institutional outcomes study (Supplementary  document 1). Data are displayed as median [IQR] or mean and standard deviation (s.d.). One hundred and thirty-seven patients (41 female, median age 29y[25-34]) returned questionnaires on sexual function and fertility. Approximately three quarters of men (69/96, 72%) and half of women (23/41, 56%) were in a stable relationship (p=0.08). Ninety-two percent of men (88/96) and 93% (38/41) of women reported having been sexually active, with coital debut at median 17y [16-19; p=NS between sexes]. Sexual Function Assessed by the Erectile Harness Score, 91/94 (97%) men reported an erection sufficient for penetration. Eight (9%) reported occasional issues with sexual climax, and two men had retrograde ejaculations. One male patient reported anorgasmia with absent erections, also reporting very poor bowel function and a history of psychiatric illness. Neither post-surgical complications nor redo surgery appeared to correlate with erectile or ejaculatory dysfunction. Onset of menarche in female respondents was at a median 13y[12-14], with one case of delayed menarche at 19 years. Frequent dyspareunia was reported by 19/38 (50%) sexually active women, and was reported by all women (n=7) with poor bowel outcome (vs. 12/31, p=0·008).  Fertility Among 36 males attempting to father biological children, spontaneous pregnancies were achieved for 30(83%) and 3(8·3%) were successful with in vitro fertilisation (IVF). Contrastingly, in the seventeen women who had attempted to conceive only 8(47%) conceived spontaneously and a further 3(18%) after IVF. Female sex was the only factor associated with subfertility on multivariate analysis (OR 10.3 [2·2-48.8], p=0·003), while surgical complications (redo surgery, anastomotic leak, need for emergency abdominal surgery) and poor bowel outcome were not predictive. Sexual Quality of Life Eighty-five sexually active males completed the SQOL-M with a median score of 100[86·4-100]. Sixteen(19%) had a score lower than -1s.d. and 12(14%) scored lower than-2s.d. of a normal population (Figure 1)3. Thirty-six sexually active females completed the SQOL-F; median score was 77[62·5-85] with 17(47%) and 8(22%) scoring below 1 and 2 standard deviations below the normal population mean (below-1s.d. vs. male patients, p=0.002; no difference for -2s.d., p=0.2; Figure1)4.  Compared to normative data3,4, there was no evidence for lower SQOL in male patients (88·2(19·7) vs. 87·1(13·7), p=0·66) whereas female patients reported significantly lower SQOL (71·9(20·7) vs. 90·7[14·97]; p=0·0003, Hedge’s g =1·0 (Large effect).)  On multivariate analysis, an impaired SQOL score (-2s.d.) was associated with a poor bowel outcome (OR 5.8[1.2-27.6], p=0.027) but not with sex, presence of a stable relationship, or the need for redo or emergency surgery.  Figure. Female patients SQOL-F scores (Left, n=36) and male patients SQOL-M scores (Right, n=85). Data presented as a % of the maximum score with reference to normative mean/SD from published literature3,4, comparison with Student’s t-test. Shaded lines demonstrate -1 and -2 standard deviations below normal population mean. Proportion of patients ≥ -1s.d. and -2 s.d. identified n(%).      Conclusion The data presented objectively describes sexual functioning and fertility in patients with HSCR and reveals hitherto unrecognised issues affecting female patients. Clearly there is a concern that pull-through surgery may lead to pelvic adhesive disease, as has been demonstrated in ileoanal pouch surgery in women with ulcerative colitis5; this may explain the unexpectedly high frequency of both subfertility and dyspareunia in this group. Ultimately, the underlying Female Male050100% of Maximum Score8(22) 12(14)p=0.0003; g=1.017(41) 16(19)reasons for the observed phenomenon cannot be conclusively identified from survey-based data and qualitative investigation alongside full work up in the fertility clinic is necessary. Given the low frequency of HSCR in females this will require multi-regional if not international collaboration.   Supplementary Methods Ethical Approval Approval was obtained from the National Health Service Research and Ethics Committee (17/LO/1692) and from the Health Research Authority; written informed consent was obtained from all participants.  Study Population As part of a comprehensive study of long–term outcomes of HSCR, UK-resident adults >18 years of age without cognitive impairment, with histologically confirmed HSCR commencing treatment at Great Ormond Street Hospital (GOSH) between 1978 and 2013 were invited to complete cross-sectional outcomes questionnaires on sexual function, fertility, sexual quality of life and bowel function. Functional outcomes Bowel function was assessed using the Rintala Bowel Function Score (BFS, 7-items, max. score 20)7. Limited assessment of individual items with this instrument is possible for patients with an end stoma or antegrade continence enema (ACE) conduit. A normal outcome was defined as BFS>17, and a poor outcome a BFS<12 or requiring stoma/ACE8.  Sexual function was explored both in terms of physical (erectile hardness score for men, dyspareunia for women) and quality of life metrics (SQOL-M3 and SQOL-F4). Patients scoring -1 and -2 standard deviations below the reference population were noted graphically and compared between sexes as well as a comparison of overall scores to the reference cohort. Subfertility was defined here as an inability to conceive without assistance. 

Sexual function, quality of life, and fertility in women who had surgery for neonatal Hirschsprung's disease

https://discovery.ucl.ac.uk/id/eprint/10119984/1/Research%20Letter_Accepted.pdf

Sexual function, quality of life, and fertility in women who had surgery for neonatal Hirschsprung's disease

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