Journal of Pediatric Surgery 58 (2023) 490–495

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Journal of Pediatric Surgery

journal homepage: www.elsevier.com/locate/jpedsurg.org

Sexual function and lower urinary tract symptoms after minimally

invasive endorectal pull-through in adolescent males with
Hirschsprung disease
Anders Telle Hoel a,∗, Remi Andre Karlsen b, Marianne Valeberg Fosby c, Kristin Bjørnland a
a
Department of Pediatric Surgery, Oslo University Hospital and Institute of Clinical Medicine, University of Oslo, Oslo, Norway
b
Institute of Clinical Medicine, University of Oslo, Oslo, Norway
c
Department of Pediatric Surgery, Oslo University Hospital, Oslo, Norway

a r t i c l e i n f o a b s t r a c t

Article history: Background: Effects of colorectal resection on sexual function and the lower urinary tract are inade-
Received 13 May 2022 quately studied in patients with Hirschsprung disease (HD). This study aimed to increase existing knowl-
Revised 15 September 2022
edge on sexual function and lower urinary tract symptoms (LUTS) in HD male adolescents operated with
Accepted 18 September 2022
minimally invasive endorectal pull-through (ERPT).
Methods: Non-syndromic male adolescents (12–18 years) operated with ERPT were invited to participate
Keywords: in this single-center cross-sectional study which included a semi-structured interview on sexual function
Hirschsprung disease and LUTS, a questionnaire recording LUTS, and a urodynamic study. Uroflowmetry curves were eligible for
Endorectal pull-through evaluation if the voided volume was >50% of expected bladder capacity. Ethical approval and informed
Minimally invasive surgery
consent were obtained.
Sexual function
Results: Of 37 eligible male adolescents, 35 (95%) with a median age of 14.9 (12.0 -18.3) years were
Lower urinary tract symptoms
Adolescence included. 94% had rectosigmoid aganglionosis, and 97% underwent a minimally invasive ERPT. 34 (97%)
visited the outpatient clinic. The ability to get erections and to ejaculate was assessed in 25/35 (71%)
adolescents. 25/25 (100%) could get an erection, and of adolescents ≥15 years 14/15 (93%) could ejaculate.
32 (91%) returned the questionnaire and underwent urodynamic studies. 15/32 (47%) reported LUTS, but
most had only sporadic LUTS. 31/32 (97%) were highly satisfied with their bladder function. Sporadic
urinary incontinence was reported by 4/32 (13%), but none reported social problems due to this. Of the
eligible 28/32 (88%) uroflowmetry curves, 15/28 (54%) were normal.
Conclusions: Minimally invasive ERPT seems to preserve sexual function and does not induce lower uri-
nary tract symptoms in adolescent HD males.
Level of evidence: III.
© 2022 The Author(s). Published by Elsevier Inc.
This is an open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/)

1. Introduction lopian tubes, and ovaries in females. Damage to these structures

Hirschsprung disease (HD) is a rare congenital disorder that is
characterized by the absence of ganglion cells in the lower diges-
tive tract predominantly affecting males [1]. Surgical management
of HD involves removal of aganglionic bowel and restoration of
bowel continuity. Rectal dissection is an integral part of any pull-
through operation and has an inherent potential for injury to the
urethra, bladder, ureters, sympathetic and parasympathetic lum-
bar nerves, prostate and spermatic cords in males and uterus, fal-
Abbreviations: CAKUT, congenital anomalies of the kidney and urinary tract;
DAN-PSS, danish prostatic symptom score; ERPT, endorectal pull-through; HD,
Hirschsprung disease; LUTS, lower urinary tract symptoms; MCUG, micturating cys-
tourethrogram.
∗
Corresponding author.
E-mail address: andho@ous-hf.no (A.T. Hoel).
may impaire sexual function and induce lower urinary tract symp-
toms (LUTS) [2,3]. There is very limited knowledge about sexual
function and frequency of LUTS after surgical management of HD,
and specifically after minimally invasive endorectal pull-through
techniques (ERPT). A systematic review from 2016 on sexual func-
tion and/or LUTS in HD patients found that only 24 studies had
been published during the last 40 years [4]. The majority of these
studies reported results after open surgery and multi-staged pro-
cedures. Ten studies, including 624 males, had assessed erectile
and/or ejaculatory function, and sexual impairment was very rarely
reported [5–8]. In two more recent studies, sexual function was
not different in 112 HD males and controls, while dyspareunia and
subfertility were more frequent among 41 HD females compared to
the controls [9,10]. In the above mentioned review, 17 studies in-
cluding 2546 patients of both genders found that less than 4% re-
ported any type of LUTS [11–14]. Four more recent studies present

https://doi.org/10.1016/j.jpedsurg.2022.09.017
0022-3468/© 2022 The Author(s). Published by Elsevier Inc. This is an open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/)
 A.T. Hoel, R.A. Karlsen, M.V. Fosby et al. / Journal of Pediatric Surgery 58 (2023) 490–495
contradictory results regarding LUTS; some found no difference be-
tween HD patients and controls, whereas others reported that HD
patients with impaired bowel function had more frequent LUTS
than other HD patients [9,15–17].
In addition to the paucity of studies examining sexual func-
tion and LUTS in HD patients, few studies have applied validated
questionnaires or had an independent investigator interviewing pa-
tients and doing the chart reviews. Most studies are retrospective
and based on chart reviews only. Furthermore, the age distribution
of the patients often varies greatly, making it difficult to draw con-
clusions [4,18–20]. Therefore, the aim of this study was to increase
existing knowledge on sexual function and frequency of LUTS in
HD male adolescents operated with minimally invasive ERPT by
having an independent investigator interviewing the patients, ap-
plying a validated questionnaire and performing urodynamic stud-
ies.
2. Methods
2.1. Study design
This is a single-center cross-sectional study exploring sex-
ual function and LUTS in male HD adolescents. Guidelines for
Strengthening the Reporting of Observational Studies in Epidemi-
ology (STROBE) were applied [21].
2.2. Surgical techniques, perioperative examinations and follow-up
routines
Total transanal ERPT was performed mostly as described by de
la Torre et al., but with a shorter mucosectomy [22,23]. During la-
paroscopic assisted ERPT, the sigmoid colon and rectum were dis-
sected down to the pelvic floor, and the mucosectomy was then
performed transanally as in the total transanal technique.
There was no systematic screening for urinary tract malforma-
tions. Investigations of the urinary tract had been done either be-
cause of urinary symptoms or because of prenatal findings. In ad-
dition, some had undergone abdominal ultrasound including exam-
ination of the urinary tract before HD was diagnosed as a part of
investigations for gastrointestinal symptoms.
Postoperative follow-up routines included frequent outpatient
visits during the first year. Thereafter, frequency of outpatient vis-
its depended on symptoms and availability for follow-up at the pa-
tients’ local hospital.
2.3. Ethics
Written informed consent was obtained from all adolescents
≥16 years and from parents of adolescents <16 years. Adoles-
cents <16 years got age-adapted information about the study. The
study was approved by the regional committee for medical and
health research ethics (2018/2009) and the Hospital’s Data Pro-
tection Officer (18/19101). The study is registered in Clinical Trials
(NCT04106947).
2.4. Patients, clinical setting and data collection
All non-intellectually impaired male adolescents (12-18 years),
fluent in Norwegian, having undergone ERPT and/or follow-up at
our department were eligible for inclusion. Patients were identified
from the hospital’s electronic database and invited to participate
by mail or during outpatient clinics.
Demographics, length of the aganglionic segment, investigations
of the urinary tract, presence of hypospadias, and surgical de-
tails were recorded retrospectively from electronic medical records.
Congenital anomalies of the kidney and urinary tract (CAKUT)
491
was defined as renal agenesis, kidney hypoplasia, hydronephrosis,
and/or vesicoureteral reflux [24].
To register current clinical status, the adolescents had a semi-
structured interview at the outpatient clinic, a clinical investiga-
tion if indicated, and urodynamic studies. They were also asked to
answer a questionnaire recording LUTS. This questionnaire was a
12-item modified version of the Danish Prostatic Symptom Score
(DAN-PSS) (Appendix 1) [25]. LUTS was defined as one or more of
the following symptoms: voiding frequency >8/day, straining, ur-
gency, urge incontinence, stress incontinence, incontinence with-
out physical activity/straining (passive incontinence), and bedwet-
ting (enuresis). Those who did not want to attend the outpatient
clinic, could participate in the study by only answering the ques-
tionnaire which was sent by post.
The semi-structured interviews were led by a surgeon who had
not performed the ERPT operations or been responsible for the
follow-up. The first part of the interview focused on current clin-
ical status, including voiding habits and LUTS. All the adolescents
were asked if they wanted to talk to the interviewer alone. If the
adolescent and his parent(s) agreed, the interviewer talked with
the adolescent alone from the age of 12. This was carried out as an
informal conversation about sexual function. Sexual function was
assessed by the adolescents’ self-reported ability to get erections
and to ejaculate. Erection and ejaculation were reported dichoto-
mously by the presence or absence of these functions.
2.5. Urodynamic studies
Uroflowmetry measuring flow rates and voiding volume was
performed with the Flowstar Laborie/Medical Measurement Sys-
tems BV (MMS). Residual volume was measured by a suprapubic
transcutaneous bladder scanning with the CUBEscan BioCon-700
by Mcube Technology. The uroflowmetry curves were considered
reliable if the voided volume was >50% of expected bladder ca-
pacity [26]. The flowmetry curves were examined by two indepen-
dent investigators (ATH, RAK), and if they disagreed, a third inves-
tigator (KB) was consulted. The flowmetry curves were classified
as normal (bell-shaped) or abnormal (non-bell-shaped: plateau-,
staccato- or interrupted shaped) [26].
2.6. Statistics
Continuous variables are presented as median (min-max). IBM
SPSS software for Windows version 25 (Armonk, NY: IBM Corp.)
was used.
3. Results
3.1. Adolescents
Of 41 identified adolescents who had undergone ERPT, four had
intellectual impairment. Consequently, 37 adolescents were invited
to participate. Two did not respond, and 35 (95%) adolescents with
a median age of 14.9 (12.0 -18.3) years were included. Thirty-
four (97%) visited the outpatient clinic. Two (6%) had agangliono-
sis proximal to the rectosigmoid colon, and 34 (97%) were oper-
ated with minimally invasive techniques, either total transanal or
laparoscopic assisted ERPT (Table 1). One adolescent had been op-
erated abroad with laparotomy assisted ERPT, but follow up was
done at our center. None of the patients had intraoperative uro-
logic injury, postoperative anastomotic leakage or redo surgery.
3.2. Sexual function
Twenty-seven adolescents agreed to talk to the interviewer
about sexual function, but two adolescents (12 years) did not un-
492 A.T. Hoel, R.A. Karlsen, M.V. Fosby et al. / Journal of Pediatric Surgery 58 (2023) 490–495

Table 1 Table 2
Demographics of male adolescents with Hirschsprung disease (n=35). One adoles- Results of selected items from a questionnaire on lower urinary tract symptoms
cent had more than one associated anomaly. answered by male adolescents operated for Hirschsprung disease (n=32).

Patients Daily micturitions 1-3/day 4-8/day >8/day

11 (34%) 20 (63%) 1 (3%)
Age, median (min-max), years 14.9 (12-18.4)
Length of aganglionosis 33 (94%) Micturitions at night Never 1/night 2/night ≥3/night
Rectosigmoid colon 2 (6%) 29 (91%) 3 (9%) 0 0
Proximal to rectosigmoid colon 25 (71%)
Endorectal pull-through operations 9 (26%) Never Seldom Often Always
Total transanal 1 (3%) Straining 24 (75%) 7 (22%) 1 (3%) 0
Laparoscopy assisted 6 (15%) Urge 23 (72%) 7 (22%) 2 (6%) 0
Laparotomy assisted 2 (5%) Urge incontinence 31 (97%) 1 (3%) 0 0
Associated anomalies 4 (10%) Stress incontinence 29 (91%) 3 (9%) 0 0
Neurologic1 1 (3%) Passive incontinence 31 (97%) 1 (3%) 0 0
Urinary tract2
Neoplasm3
1
Hydrocephalus and a neurological disease without intellectual impairment.
2
Kidney hypoplasia, hydronephrosis, and vesicoureteral reflux. Table 3
3
Benign coecal tumor removed prior to diagnosis of Hirschsprung disease. Results from urodynamic studies in male adolescents operated for Hirschsprung dis-
ease. Results are reported only for adolescents with a voided volume >50% of ex-
pected bladder capacity (n=28) and measured residual volume (n=26).
derstand the questions and were excluded. Thus, 25/35 (71%) ado-
Median (min-max)
lescents, median 15.4 (12.4-18.3) years, could be assessed for sex-
Voiding time (s) 40 (15-72)
ual function. All of the 25 adolescents reported that they could get
Duration of flow (s) 36 (15-70)
erections. Time to max flow (s) 12 (2-32)
Eighteen of 25 (72%) adolescents, median 17.0 (13.3-18.3) years, Max flow rate (ml/s) 26 (15-42)
told the interviewer that they could ejaculate. Of the adolescents Mean flow rate (ml/s) 16 (7-27)
≥ 15 years, 14/15 (93%) could ejaculate. Seven adolescents, median Voided volume (ml) 517 (253-1060)
Residual volume (ml) 21 (0-193)
13.3 (12.4-15.9) years, knew they had not ejaculated. None of the
adolescents reported retrograde ejaculation.

3.3. Urology
3.3.1. Investigations and operations in the urinary tract
Seventeen of 35 (49%) adolescents had undergone one or more
previous investigation of the urinary tract. Fifteen (88%) had un-
dergone ultrasound, four (24%) uroflowmetry and measurement
of residual volume, three (18%) a micturating cystourethrogram
(MCUG), two (12%) scintigraphy, and one (6%) a cystometry.
Ultrasound examinations had been normal in all but three.
Findings in these three included unilateral hydronephrosis and hy-
droureter, unilateral hydroureter and a hypoplasic ipsilateral kid-
ney, and isolated unilateral hydronephrosis. These three boys un-
derwent further investigations. MCUG showed vesicoureteral reflux
grade 3 and 4 in the two boys with hydroureter. Scintigraphy was
performed in the same two boys and showed in both significantly
reduced function in the ipsilateral kidney. The boy with isolated
hydronephrosis got a repeat ultrasound showing no hydronephro-
sis. Two boys with initial normal ultrasonographic findings later
underwent MCUG due to epididymo-orchitis and daytime urinary
incontinence, and they had vesicoureteral reflux grade 0 and 2, re-
spectively. Thus, four boys had six CAKUT: Kidney hypoplasia (1),
hydronephrosis (2), and vesicoureteral reflux (3). None of the ado-
lescent males had previously been diagnosed with hypospadia.
Uroflowmetry and measurement of residual volume had been
done previously in four boys because of neurologic disease with-
out intellectual impairment, macroscopic hematuria and urinary
tract infection, late onset bedwetting, and urinary hesitancy. Only
the boy with a neurologic disease without intellectual impairment
had a pathological uroflowmetry with large voided volumes, but
no residual volumes. Cystometry in the same boy showed signs of
a neurogenic bladder. Two boys underwent a urologic operation.
Both had endoscopic Deflux®-injections due to vesicoureteral re-
flux.
3.3.2. Lower urinary tract symptoms (LUTS)
32/35 (91%) LUTS questionnaires were returned. 15/32 (47%)
adolescents reported to have one or more LUTS, but the majority
had only sporadic LUTS (Table 2). Urge, straining and any urinary
incontinence occurred in 9/32 (28%), 8/32 (25%), and 4/32 (13%),
respectively. None had bedwetting or social problems due to uri-
nary incontinence. 31/32 (97%) adolescents reported high or excel-
lent satisfaction with their bladder function. Low satisfaction with
bladder function was noted in only one adolescent, and he had a
neurologic disease without intellectual impairment.
3.3.3. Findings from urodynamic studies
Urodynamic studies were completed by 32/35 (91%) adoles-
cents, but due to a technical problem only 31 curves could be eval-
uated. 28/31 (90%) had a voiding volume >50% of expected blad-
der capacity, and only the curves of these were examined (Table 3).
The uroflowmetry curves were normal in 15/28 (54%). 7/28 (25%)
had a plateau curve, 5/28 (18%) had a staccato curve, and 1/28 (4%)
had an interrupted curve. 19/28 (68%) adolescents had a voided
volume exceeding their expected bladder capacity. Of those with
a plateau curve, all had a voided volume exceeding their expected
bladder capacity.
3.4. Results from semi-structured interviews
The interviews with the adolescents and parents together lasted
from 60 to 90 min, whereas the interviews with the adolescents
alone lasted from 5 to 30 min. Generally, the length of the inter-
view with the adolescents increased by increasing age of the ado-
lescent (results not shown).
One of the first adolescents we interviewed, suggested that in-
formation regarding sexual function was not be reported in medi-
cal records available for the parents as this would make it easier to
talk more freely. Subsequent adolescents were informed that data
on sexual function would not be recorded in their medical records.
None of the adolescents seemed to find it difficult or embarrass-
ing to talk about sex, and they did not attempt to finish the con-
versation before it came to a natural ending. Several adolescents
expressed concerns about the size and appearance of their penis.
 A.T. Hoel, R.A. Karlsen, M.V. Fosby et al. / Journal of Pediatric Surgery 58 (2023) 490–495
Furthermore, 3 (9%) adolescents attending the outpatient clinic had
problems with phimosis. One was referred for surgical treatment,
and two got conservative treatment.
During the semi-structured interview voiding habits were in-
vestigated further as a supplement to the questionnaire on LUTS.
Frequency of micturition was assessed by going through a normal
day. We found that the rates of daily micturitions reported dur-
ing semi-structured interviews and in the questionnaires were ex-
actly the same. Overall, two thirds of the adolescents reported nor-
mal voiding habits. The twelve adolescents who reported <4 or
>8 daily micturitions got advice how to normalize their voiding
frequency. Those who had large residual volumes were advised to
“double-void” morning and evening.
4. Discussion
The main finding of this study is that sexual function in adoles-
cent HD males generally is unaffected by minimally invasive ERPT.
Furthermore, sporadic LUTS were common, but patient satisfaction
with bladder function was very high. This is important information
to be passed on to patients and parents.
The finding from this study on HD adolescent males’ ability to
get an erection and to ejaculate contribute to the existing, but very
limited knowledge on postoperative sexual function in HD males.
The ability to get spontaneous erections after both open and min-
imally invasive ERPT, is reported in nearly 100% of HD males [5–
9,18,27,28]. Thus, our results are in line with earlier studies. In con-
trast to the number of studies examining the ability to get sponta-
neous erections, very few studies have explored HD males’ ability
to ejaculate, and none has reported adolescents’ ability to ejacu-
late. We found that nearly all adolescents ≥15 years could ejacu-
late and none reported to have a retrograde ejaculation. A recent
study in 16 adult HD males who had undergone ERPT in childhood,
found that all could ejaculate and the first ejaculation occurred at
median 13 years [9]. Studies from the 1970s including patients op-
erated with the open Swenson procedure report absent ejaculation
in a few patients [20,27]. A recent study found that 2% of 94 adult
HD males had retrograde ejaculation after mostly open Duhamel
pull-through [10]. Since so few studies have explored ejaculation
in HD males, one cannot draw any firm conclusions, but it seems
that the ability to ejaculate is preserved in the great majority of
HD males.
In a previous qualitative study we reported that adult HD males
expressed good sexual self-esteem and few problems with inti-
mate relationships [29]. This is supported by a recent quantitative
study reporting that sexual quality of life in adult HD males was
comparable to controls [10]. The HD men in our previous study
recommended addressing sex from early teens during follow-up
and explain to the teenagers how the operation could interfere
with later sexual activity. Some had wondered if they should take
any precautions concerning sexual activities because of previous
surgery. Based on this and the experience from talking about sex
with the HD adolescents, we suggest that sex is discussed with HD
teens.
LUTS were reported by nearly half of the male HD adolescents,
but the majority had only sporadic LUTS. This observation is in ac-
cordance with recent studies which report LUTS in 37-68% of HD
patients of both genders operated with ERPT [9,15,16]. As shown
in a large population study, we report urge and stress incontinence
in very few adolescent HD males [30]. Overall, we found that one
out of ten male HD adolescents had urinary incontinence. This is
in line with a systematic review where urinary incontinence was
found in up to 11% of patients after open and minimally invasive
ERPT [4]. None of the adolescents reported social problems related
to urinary incontinence which is in accordance with other stud-
ies [9,15,16]. We found that none of the adolescents had enuresis.
493
Another study reported increased frequency of enuresis in HD pa-
tients compared to the age-matched controls, but the HD patients
in that study were young; median 8 years [15]. Recent large stud-
ies have found that enuresis are more common in constipated HD
children, and that poor bowel function is associated with frequent
urinary incontinence regardless of age [15,31]. It is well known
that both hypospadia and neurologic diseases, especially in the
central nervous system, could affect patients’ LUTS-profile nega-
tively [32,33]. One male adolescent had a neurologic disease with-
out intellectual impairment, and was the only one with low sat-
isfaction with own bladder function. It is also important to em-
phasize that the frequency of LUTS in this and most studies we
have compared our results with, report findings in non-syndromic
HD patients [9,15,34]. In syndromic HD patients, both frequency
and severity of LUTS are different from those without a syndrome
[35,36].
Very few papers report results from postoperative urodynamic
studies in HD patients, and only one recent study presents find-
ings from urodynamic studies after minimally invasive ERPT [9].
Interestingly, only half of the uroflowmetry curves were normal
(bell-shaped) in both studies. In contrast, two large studies of
1268 and 950 healthy children aged 4-14 years report 80-90%
normal curves [37,38]. The HD adolescents’ frequency of stac-
cato and interrupted curves were not different from healthy con-
trols, but plateau curves were more common. Plateau curves are
found in 5% of healthy controls, and often when voided volumes
are large [39]. The majority of the adolescents in our study had
voided volumes exceeding their expected bladder capacity. The
most plausible explanation is that they waited too long before they
voided as most reported a strong urge to void. If we had done
a second urodynamic study, this source of error might have been
ruled out.
There is a growing recognition that HD is associated with
CAKUT. CAKUT occur in 4-25% of HD patients compared to 0.3-
1.7% in the general population [24,40–45]. Among those who had
investigations of the urinary tract, we found that 24% fulfilled the
criteria for CAKUT. This percentage is probably not reflecting the
true frequency of CAKUT in this population because there was no
systematic screening and several investigations had been done due
to urinary symptoms or prenatal findings. Many studies advocate
that all HD patients should be screened for CAKUT. Whether this
is beneficial is debatable, as few with CAKUT need any treatment
[15,40,42]. Only 6% of the adolescents in this study had under-
gone an operation due to CAKUT. This is in line with other studies
which find that only a minority of HD patients with CAKUT re-
quired medical or surgical treatment [15,24,46,47].
The main strengths of this study are the high rate of attendance
and that an independent investigator interviewed the adolescents
and their parents and did the chart reviews. The use of a validated
questionnaire and urodynamic studies also strengthen the general-
izability. The modified version of the DAN-PSS have been exten-
sively used to report LUTS in HD patients and controls, thereby
making it easier to compare and contextualize the results from this
present study with other studies in HD patients [9,16,30,31,48]. We
did not use a questionnaire to evaluate sexual function. It is pos-
sible that the adolescents would have answered more honest if a
questionnaire had been used. In addition, some may regard asking
12-14 year old male adolescents about sexual function a limitation.
Based on the adolescents’ reporting on sexual function, one could
talk about sexual function from the age of 12, but study male pa-
tients’ sexual function at a higher age.
To conclude, this study suggests that minimally invasive ERPT
preserves sexual function and does not induce lower urinary tract
symptoms in adolescent HD males. Furthermore, male HD adoles-
cents appreciate the opportunity to talk about sex, and this should
be a part of transitional care.
494 A.T. Hoel, R.A. Karlsen, M.V. Fosby et al. / Journal of Pediatric Surgery 58 (2023) 490–495
Declaration of Competing Interest
The authors report no conflict of interest.
Acknowledgments
We would like to thank stoma nurses Astrid Austrheim and
Hanne Ambrose for their work in data collection and Tom Øres-
land for proof reading.
Funding
The study was funded by the Norwegian Dam Foundation.
Supplementary materials
Appendix 1. Modified version of the Danish Prostatic Symptom
Score (DAN-PSS) on lower urinary tract symptoms.
Supplementary material associated with this article can be
found, in the online version, at doi:10.1016/j.jpedsurg.2022.09.017.
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