57: Long Term Complications of Lower Urinary Tract Reconstruction

This chapter will take approximately 20 minutes to read.

By Dan Wood, PhD, MBBS, FRCS Urol 1, 2
  1. Department of Pediatric Urology, Children's Hosptal Colorado, Aurora, CO, USA
  2. Department of Surgery, Division of Urology, University of Colorado, School of Medicine, Aurora, CO, USA

Introduction

The need for urological reconstruction is long established and the range of techniques available continues to expand. With introduction of new technology (e.g., robotic surgery) the options for achieving those techniques also looks to be expanding. The problem with all of these innovations is that whilst they are attractive (to a surgeon) and they can be made to sound attractive (to a patient) the favorable elements (e.g., reduced hospital stay, reduction in abdominal scars etc) are weighed against an influence on outcome that is not yet fully understood.

This chapter will examine some of the long-term data that have been published in relation to urological reconstruction, it will also take a brief look at some of the aspects that make our interpretation difficult including variation in technique and the incorporation of new technology.

Bladder

The long-term outcomes of cystoplasty are overall favorable. With good urodynamic data showing significant improvements in reservoir capacity, detrusor pressure, compliance and risk of renal damage. Kreb’s et al looked at outcomes in 29 patients with neuropathic lower urinary tract dysfunction, follow-up was14 years; an overall complication rate of 38% was observed, 6 (20.7%) had required further surgery for complications including bladder stones or small bowel obstruction. A further 8 (27.6%) required additional procedures to achieve continence (artificial urinary sphincter or transobturator tape). Long-term continence rates were 72% which is comparable to other cohorts.1 other authors have reported an improvement in quality of life.2 It is documented in the literature that urinary and fecal incontinence will adversely affect quality of life.3 In a series with more heterogenous diagnoses (including neurogenic, posterior urethral valves bladder exstrophy and cloacal exstrophy) 88.1% were continent after a mean follow-up of 12 years. 11.9% required an additional bladder neck procedure for continence, 4 (9.5%) required bladder stone removal, and ¾ suffered a subsequent recurrence. 50% had a Mitrofanoff channel for bladder drainage, and in three patients this was combined with bladder neck closure.4

The rate of cystoplasty has fallen in favor of less invasive techniques such as intravesical botulinum toxin. Ileum is the most common segment of bowel. Up to 75% will need to perform CISC (clean intermittent self catheterization). About 15% of patients with lower urinary tract dysfunction have end stage renal failure; the data support cystoplasty giving at least as good and in some studies better 10-year graft survival than an ileal conduit. Overall success varies with diagnosis but generally sits between 80 and 90%, the outcomes are less good following radiotherapy with a fall to 70%.5

Metabolic disturbance is often discussed and needs to be monitored as part of follow-up.6 There is probably a subclinical hyperchloremic metabolic acidosis in many patients but 16% are reported to need daily, oral bicarbonate replacement. The author’s follow-up protocol includes an annual ultrasound combined with bloods for renal function, chloride, bicarbonate, folate and vitamin B12. The reason for including vitamin B12 is that in many cases it can be difficult to determine exactly which segment of bowel had been used and thus it is safer to screen for B12 deficiency rather than assume and miss it. The consequences of the ensuing neuropathy and subacute combined degeneration of the cord are irreversible and allowing this to happen would be regarded as below an acceptable standard of care.5

Asymptomatic bacteriuria is very common—in some studies up to 100%, significant infection is less common.7,8

Bladder stones are a common complication following cystoplasty. Data offer a wide range of incidences up to 39%.9 Augmentation cystoplasty results in a 2% incidence of stones, if a patient needs to catheterize urethrally, the incidence rises 5-fold and if drainage is via a Mitrofanoff, channel this rises to 10 times. This suggests that dependent drainage is favorable and the presence of residual urine increases the risk of stones.10,11 Data suggest that reduced daily fluid intake and hypocitraturia may increase the risk of stones.12 Anecdotally, mucus production may be important and this deems to increase in some diets (e.g., dairy, spicy foods).

Perforation of a cystoplasty is a dangerous complication with a reported mortality approaching 25%, this probably arises as a result of delayed diagnosis in the hands of inexperienced clinicians, soft clinical signs and negative or equivocal radiology.13 The incidence is increased in neuropaths and those who catheterize and or who have undergone a surgical increase in bladder neck resistance e.g., artificial urinary sphincter or bladder neck reconstruction.14

Malignancy in a cystoplasty is a distressing complication the incidence is low and estimated at 1.2% with a time lag of approximately 20 years after surgery. Tumors are most commonly found in the region of the anastomosis, they are often adenocarcinoma and may present late making curative treatment difficult. Patients who have a congenital anomaly (e.g., bladder exstrophy) of the bladder may be at increased risk when compared to others. Other risks include chronic inflammation, infection, urinary stasis, bladder stones, immunosuppression and smoking.15,16,17,18

Pregnancy with a cystoplasty is (as a rule) safe. If there has been previous impaired renal function it is important to ensure renal function is sufficient and the advice of a nephrologist is advisable—otherwise there is no direct effect on renal function if this was normal before conception. Those who have had a reconstruction in the face of a congenital anomaly should be monitored through pregnancy as they are at greater risk of urological complications (63%) including upper tract obstruction (10–24%), urinary retention (37%) and obstetric/gynecology complications including prolapse (exstrophy – 42%) and hypertension/preeclampsia (10–32%).19,20

Ureter

Ureteric Reimplantation for Vesicoureteric Reflux

Published data show that open and minimally invasive techniques show excellent results for surgical reimplantation of the ureter. Open reimplantation gives excellent results in a series of 965 patients, only 6.1% had persistent reflux after surgery and 72 .2% resolved within a median time of 20. 4 months, 13% suffered febrile UTIs in a median follow up period of 47.1 months.21 Smaller studies have examined minimally invasive ureteric reconstruction with robot assisted reimplants—7.7–13% had post-operative reflux initially, 10% had febrile UTIs (none of these patients had post-operative reflux recorded.22,23 Gundeti et al recognized the learning curve and development of technique over a seven year period.22 immediate post-operative complications were recorded in 10% (including ileus, ureteric injury, ureteric obstruction and a perinephric fluid collection.23 Although these data appear to show good outcomes earlier data suggested a much higher rate of UTIs (43% vs 24% F vs M) pyelonephritis (27% vs 9.5% F vs M), hypertension in 6%, 28% of women who became pregnant reports UTIs during pregnancy and 7% suffered pre-eclampsia. These data predate other studies by more than 20 years and thus improved techniques both operatively and perioperatively may have influenced later outcomes, but it is important to remember that some earlier cohorts of patients (especially) may be vulnerable to later sequelae of these types.24

Pyeloplasty for Ureteropelvic Junction Obstruction

Overall the success rate for pyeloplasty is high. Success rates of greater than 95% are cited.25 The use of a stent appears to improve the long term success (96.3% vs 91.5%).26 There are recent data that suggest a lower rate of renal preservation in patients who have associated renal anomalies.27 Many centres would offer these using a minimally invasive techniques and the outcomes appear to support this approach.

Urethra

Epispadias/Hypospadias

Even in the best hands and high-volume centres, outcomes of hypospadias range from 5–10% for distal repairs, whereas in cases of proximal repair the incidence can range from 15% to as high as 90%.28,29,30 Recent data, examining single stage repairs for proximal hypospadias, showed an overall 56% complication rate with an increased risk of unplanned procedures and at least 2 complications (33%) when measured against a two-stage repair.31 Each subsequent redo urethroplasty carries with it a higher risk of complication.32

The most common complications are:33,34,35

A. Wound dehiscence

This is an early complication and may occur in any repair but is more common in patients with proximal hypospadias. It is also seen where there is any kind of tension on the repair—i.e., if the glans is small. If the closure fails and the urethra dehisces a revision repair is needed.

B. Fistula

Occurring in 4–50% of patients, this is the most common complication following hypospadias repair. An early fistula probably represents a technical failure and may need time to mature before closure. Fistulae may occur at any time and any age following repair—much later fistulae need to be examine to consider a distal stricture causing a blow-out fistula.

C. Diverticulae

This term describes a dilated, sacculated section of the previous repair, as with fistula it may arise with distal obstruction causing ‘back pressure’ and distension of the more proximal neourethra. Absence or deficiency corpus spongiosum tissue may be an additional factor. They may present with ventral penile swelling during voiding, poor urinary stream, postvoid dribbling, urinary tract infections and (occasionally) urethral calculi secondary to stasis. Surgical intervention requires treatment of any underlying cause e.g., distal stricture, excision of the excess tissue (wall of the diverticulum) and reclosure of the urethra.

Hypospadias surgery can be technically challenging, the added difficulty is that it is impossible to follow every infant hypospadias into adult life—many patients don’t need to be seen unless they have a problem after the first couple of years and we tried to follow them all the workload would crush any healthcare system. Rynja et al examined reported data for 1027 patients—180 had severe hypospadias they required an average of 2.7 operations per patient.36 The authors recognized the very high lost to follow-up rate. Data from Ghent showed a 24.1% reoperation rate in 543 prospective followed patients.37

D. Meatal stenosis

Meatal stenosis (defined as <8 Fr caliber) may occur in the following scenarios: inadequate mobilization of the glans wrap, tubularization of the neourethra extending too far towards the glans tip or ischemia of the glans flaps. Patients may present with voiding difficulty, urine infection (from incomplete emptying), a narrowed urinary stream or spraying.

E. Persistent Chordee

This is something more commonly seen in patients after they have passed through puberty. If the chordee is severe enough to prevent penetrative intercourse then correct should be offered.

Reported data for men who have had hypospadias surgery in childhood and thus passed through puberty is less common but very important for the overall picture. Common complaints are:

  • spraying (10–63%),
  • post void dribbling (20–40%),
  • lower urinary tract symptoms (3–85%).

Patient dissatisfaction with outcome is increasingly recognized with a reported rate of 7–81%, data quality appears to vary between studies—this may (in part) explain the variance. Broadly speaking proximal hypospadias appears to be associated with a greater chance of long-term outcome dissatisfaction. There may be in impact on sexual function, 77–100% of men expressed good sexual satisfaction, however factors such as recurrent chordee (5–23%), erectile dysfunction (0–73%) and ejaculatory difficulties (5–36%) may impact long term sexual function.38 These data were collated and examined for the recent European Consultation looking at Congenital Lifelong Urology.

Exstrophy/Epispadias

It is arguably controversial to cohort all exstrophy and epispadias together as the spectrum is wide. Epispadias may be a relatively mild epispadic urethral meatus, through to a more severe form affecting the integrity of the bladder neck. Outcomes in bladder exstrophy may be affected by a range of factors—bladder plate size, diastasis, corporal mass etc. The spectrum is further widened with cloacal exstrophy and the anatomical severity inherent in that along with additional factors that may include a neuropathic element.

In this section, the urethral outcomes are the focus— other aspects will be discussed in relation sexual and fertility outcomes.

In patients with epispadias the data relating to urethroplasty are (perhaps) most pure. In a series of 30 patients, 17% required a skin revision—20% had fistulae (although this fell to 6% in patients who had undergone a Mitchell repair), abscess 3% and diverticulum 3%. 89% expressed concern about their penile appearance, one required corporeal plication for dorsal chordee and one patient underwent a forearm flap phalloplasty.39 Long term data for patients with exstrophy suggest urinary diversion in 52–70%, in one study 30% were emptying per urethra only one was voiding spontaneously, 42% suffered moderate to severe incontinence.40,41 Ejaculatory function is reported in 82–85% of men, other factors such as orgasmic dysfunction (22–51%) and reduced sensation (26–36%) were also reported.40,42

Genitalia

In our surgical generation, one of the areas that has elicited some of the most active debate and discussion is that of genital reconstruction in patients with differences in sex development (DSD). There are a number of elements to the debate, these include:

  • who should make the decision for surgery
  • when should surgery be performed
  • who can consent for such surgery
  • how do we measure outcomes
  • how do we use outcome data to inform future parents/patients and surgeons.

It is important to understand the potential indications for surgery:

  1. The creation of a (neo) vagina as a conduit for menstruation
  2. The creation of a (neo) vagina to allow penetrative intercourse
  3. Cosmetic improvement of genital appearance
  4. Improvement of psychological wellbeing.
  5. Removal of gonads to manage malignant risk

The Chicago consensus meeting and subsequent document, published in 2006, was clear that there should be a reduction in infant clitoroplasty.43 Until recently data suggested that this change was slow to take effect, however, comparison of outcomes suggested that outcomes may be improving, although equal numbers of patients were requiring revision surgery, the procedures needed were (surgically) minor as opposed to major interventions, it appeared that bowel vaginoplasty in childhood had poor outcomes.44

There remain a paucity of data to support the assertion that surgery will lead to improved psychological outcomes and it would be impossible to run a controlled trial to examine whether the changes the reduced sensation45 experienced in older patients is an effect of surgery or an underlying disorder.

A recent large European study surveyed patients views and suggested that decisions to operate remain finely balanced. There was a tendency amongst those with congenital adrenal hyperplasia (CAH), hypospadias and XY DSD to favor early surgery—this reached significance in the CAH identifying as female group when asked to agree or disagree with the statement ‘Reduction of an enlarged clitoris is necessary in girls’—even then 39% either disagreed, were undecided or did not know. However, there is considerable variation amongst the groups. Patients who had recent contact with support groups were more likely to favor surgery at a later age. Importantly, the authors conclude that a moratorium on surgery is not justified as it does not appear to reflect the views of many patients with DSD. It is clear that multidisciplinary care is vital. A continued emphasis on gathering data for long term outcomes, ensuring informed consent/assent are important and where there is not a clear surgical indication to proceed with surgery that attempts are made to ‘motivate parents to postpone elective genital surgery.46

An in-depth discussion around DSD and outcomes is beyond the scope of this chapter. It is important that practitioners in this area are aware of the discussions, actively seek up to date information regarding outcomes and work within a multidisciplinary team to ensure the best outcome for a patient and their family.

Sexual Function and Fertility

These are important aspects of life for many patients that have had surgical treatment of congenital anomalies however the outcomes vary significantly with the underlying diagnosis and potentially the impact of surgery.

Female

Spina Bifida

Adolescents (of both sexes) with spina bifida have the normal desires and ambitions for relationships and potentially family. The subject is often noted as being poorly handled by healthcare providers. Knowledge in the area and making sure that the conversation is initiated and ongoing education are very important.47 Questions about sexual health, contraception and factors such as latex allergy (with reference to barrier contraception) are important to include.48 These young female patients tend to reach menarche earlier than their age matched counterparts. A range of factors can influence sexual debut and on-going sexual behavior including social isolation, continence, age and level of lesion. Proactive advice about supplementation with high dose folic acid in preparation for conception and in the first trimester to reduce the risk of neural tube defects in children are very important.49

Genitourinary Anomalies

Female patients with bladder exstrophy may have some initial difficulty with penetrative sexual intercourse because of vaginal stenosis and dyspareunia however 80–90% report sexual activity.50,51 In those affected by a cloacal anomaly the initial length of the common channel is significant, the reconstructive surgery in longer channels is much more challenging; bringing with it a greater risk of impact on urinary, bowel and sexual function.52 Others with an absence of the vagina or with a significant deficit need to be treated in a multidisciplinary team that can offer all options—including dilatation (which is highly successful in those with a dimple and an unoperated perineum).

The aforementioned discussion regarding differences of sex development highlights some of the concern and difficulties that accompany the management of affected patients. Previously published data have shown reduced sensation in these patients and an impact on sexual and orgasmic sensation.46,45 The big questions remain—when the surgery is done—why is it necessary, what will it achieve and is now the best time to do it? All ‘sides’ of the discussion need to remain involved and open minded to ensure that the best possible outcome is achieved allow the best possible future care for the new patients (and parents) coming into the system.

Male

Spina Bifida

Affected men have reduced penile sensation and approximately 75% will describe erectile dysfunction—this can be readily treated with phosphodiesterase inhibitors with an 80% success rate.53 Erectile function, paternity rates and mobility are all affected by the level of the lesion (with those above T10 suffering the most severe impact).54 The use of electro ejaculation is one means to assist with fertility, however despite the vast majority of men showing a normal hormonal profile many demonstrate poor semen parameters. Affected men should also be advised to take high dose folic acid as part of planned conception as there is now evidence that this will reduce their chance of having children affected by neural tube defects.55

Being in a stable relationship appears to improve overall satisfaction, approximately 50% of men affected by spina bifida report being satisfied with their sex life.56

Understanding the diagnosis and their prior surgery are part of a good care plan for any of these patients but ensuring that there is a conversation about relationships and sex life is important. In this group (and others) urinary and fecal continence may also play an important part in their confidence and well-being in this realm. Some patients will respond well to a message that the conversation is open for them to pick up and some will require much more proactive enquiry—good communication around this is very important particularly in a patient’s adolescent life, when these issues may be associated with large elements of self-doubt.57

Genitourinary Anomalies

It is perfectly normal for adolescent women and men to examine their body image and in this context to think about sexual function and whether they will satisfy a partner. Unfortunately, some of the factors that influence them are difficult to control. The most immediate of these is peer pressure/comparison; this may form part of the barriers to engagement in intimate relationships with fear of discovery, pseudo acceptance or bullying.58,59

There are some clear, anatomical issues that may make penetrative intercourse painful or very difficult (e.g., residual chordee in hypospadias or marked dorsal chordee in exstrophy).42,36 In some men with severe hypospadias or a lack of or asymmetrical corporeal tissue in exstrophy there are significant concerns about penile size. In studies that have looked specifically at micropenis 95% of patients described normal erections, with 60% having regular sexual intercourse—38% were noted to need psychological support.60 Whilst these data are not specific to either hypospadias or exstrophy they do give a strong indication of the difficulties that these patients may face and the need to incorporate psychology into their management. Data highlighting some of the areas of sexual dysfunction in men with exstrophy and hypospadias are outlined in Table 1. This table is not designed to draw comparison but highlight the similarity in issues that arise and the need for awareness in the clinic setting.

Table 1 Outline of areas of sexual dysfunction in hypospadias and exstrophy.36,38,42,59

Hypospadias issue Rate Exstrophy issue Rate
Curvature 20–30% — —
Erectile dysfunction 8.7–73% Erectile dysfunction/chordee 13–49%
Ejaculatory dysfunction 24.1–36% Ejaculatory dysfunction 15–74%
Penile appearance (dissatisfaction) 20.2–33.5% Penile appearance (dissatisfaction) 23–100
Sexual satisfaction 81.4–100% Sexual satisfaction Up to 92%

As with female sexual function, many authors have highlighted important data examining these very important concerns. These sections are, by no means, comprehensive.

Conclusions

This chapter summarizes data relating to pediatric urology and the outcomes that relate to an underlying condition and the treatment required to manage it. Each condition and each aspect of follow-up has been explored by a range of authors in much more depth than is possible here. The range of publications below will offer the reader a starting point to review further detail as they wish.

The range of concerns that may be faced by some of these patients is considerable. Over a lifetime many patients enjoy a good quality of life and very successful outcomes. However, the background of a chronic condition and altered anatomy should not be forgotten – the potential sequelae including urinary tract infection, renal impairment, bladder stones and sexual dysfunction necessitate long term follow-up with a team that understand a patient’s background and can maintain an appropriate regime to detect these concerns when they arise. A multidisciplinary approach is important for many with colleagues from a range of specialties playing parallel roles (e.g., gynecology, radiology, nephrology, gastro/colorectal, endocrinology and genetics).

The long-term care of these patients means they form close bonds with their healthcare team, continuity and consistency are very important in aiming to provide the best possible service.

Suggested Readings

  • Woodhouse CRJ. Adolescent Urology and Long Term Outcomes. Isbn. 2015. DOI: 10.1002/9781118844854.
  • Wood HM, Wood DN. Transition and Lifelong Care in Urology. Switzerland: Springer International Publishing; 2015, DOI: 10.1007/978-3-319-14042-1.
  • Wood HM, Wood DN. Congenital Lifelong Urology–Joint SIU-ICUD Consultation 2019. Societe International d’Urologie. ISBN ISBN.
  • Wood D, Baird A, Carmignani L, De Win G, Hoebeke P, Holmdahl G, et al.. Lifelong Congenital Urology: The Challenges for Patients and Surgeons. Eur Urol 2019; 75 (6): 1001–1007. DOI: 10.1016/j.eururo.2019.03.019.

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Last updated: 2024-02-16 21:59