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Purpose:
To investigate surgical approaches to urinary incontinence and long-term continence outcomes after successful bladder reconstruction in a heterogeneous patient population with classic bladder exstrophy (CBE). We hypothesize that most patients will achieve urinary continence following surgery, however, only a select group of patients will...
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Purpose:
To report stoma stenosis rates and efferent channel (EC) complications at long term follow-up for Turin pouch (TP).
Methods:
This is a retrospective analysis of the prospectively maintained database of patients who underwent TP between March 2006 and May 2018. The TP is a U-shaped right colon pouch. The EC was conceived by the tubulariz...
Citations
... In these two groups, incontinence complaints were managed at our center by ensuring adequate bladder capacity, bladder neck manipulation, or creating a heterotopic continent pouch, with an overall success rate of 84%. Previous reports suggest that HP, AC with BN closure, AC with BN reconstruction, and PBC with BN reconstruction are more likely to result in long-term dryness 9,10 . However, the optimal incontinence management should consider factors such as the severity of incontinence, the number and type of previous bladder procedures, and the patient's capacity and willingness for self-catheterization. Bladder conservation is feasible in most cases, but for those with a urinary conduit or ureterosigmoidostomy seeking an alternative continent system, HP with CCD appears to be the preferred option. ...
... Excluding conduits and U-Sig, only 14% (4/28) of our patients maintained spontaneous urethral micturition, while the rest used a CCD for bladder emptying. This aligns with previous findings that only 15-25% of BEEC reconstructed patients can void normally via the urethra in the long term, with most relying on self-catheterization 10,11 . Surgical procedures and bladder neck and urethral surgeries can lead to bladder fibrosis, detrusor decompensation, and obstructive voiding. ...
Introduction: Bladder exstrophy-epispadias complex is a rare condition that necessitates numerous surgical procedures during a patient's youth to achieve adequate urine storage and continence. This study aimed to identify the specific needs and functional challenges faced by adults who underwent pediatric bladder exstrophy reconstructions and assessing the management of these issues in an adult population. Methods: A retrospective chart review was conducted for all bladder exstrophy complex patients who underwent surgery at a young age and were subsequently referred to our center between 2005 and 2020. Inclusion criteria included patients with cloacal or classical bladder exstrophy older than 18 years. We recorded the reasons for referral, management of contemporary complaints, types of past and present urinary reconstructions, and their current functional status. Results: The study included 38 patients. The primary reasons for referral were incontinence (39%) and catheterization difficulties (24%). Management typically involved partial or complete surgical revision of their urinary reservoir, occasionally combined with a bladder neck procedure. Ultimately, only three patients continued to experience incontinence, while none reported catheterization issues after they underwent treatment at our center. Long-term exstrophy-related reconstruction complications included urinary tract infections (39%), stones (29%), stenosis (24%), fistulas (13%), chronic renal disease (16%), metabolic abnormalities (3%), and cancer (3%). Conclusions: Adults who previously underwent bladder exstrophy reconstruction exhibit a wide range of urologic reconstructions. Their needs often revolve around continence and catheterization concerns. Most patients with satisfactory functional outcomes perform self-catheterization through a continent cutaneous channel and have either a continent pouch or an augmented bladder.
... All these procedures require a meticulous surgical technique, postoperative short and long-term care, and close follow-up looking at the need for additional surgery. 24 A comprehensive follow-up into adult life, radiological and urodynamic assessment, and psychological support should be included. 25 Primary closure is unethical whenever all specialist facilities are not available. ...
Primary closure techniques that have been updated and long-term follow-up for CBE (classic bladder exstrophy) may be out of reach for many patients living in resource-limited settings. Late referrals to medical care and primary closures that lack the necessary skills and facilities for comprehensive treatment are still common. Alternative and long-term surgical solutions may improve the lives of these unfortunate patients. During surgical outreach missions, patients with CBE, either non-operated or with a previous unsuccessful bladder closure, who were referred from vast under-resourced rural areas to three Eastern African hospitals, were studied. The following information is provided: mode of presentation, clinical history, diagnostic workout, management, and outcome. There were 25 cases (M/F ratio 17/8) ranging in age from two days to twenty years. Five of the seventeen patients who were not treated (35%) were under 120 days old and eligible for primary closure in a qualified tertiary center when one was available in the country. There were twelve late referred cases (ranging from 120 days to 20 years). Between the ages of ten months and twelve years, eight children arrived following a failed primary closure. In all of them, the bladder plate was too altered to allow closure. Following a preoperative diagnostic workout, a Mainz II continent internal diversion was proposed to fourteen patients with acceptable bowel control and postponed in the other three. Three cases were lost before treatment because parents refused the procedure. Twelve cases ranging in age from three to twenty years (mean seven years) were operated on. Eight people were followed for a total of 53.87 months (range: 36-120). Except for three people who complained of occasional night soiling, day and night continence were good. The average voiding frequency during the day was four and 1.3 at night. There was no evidence of a metabolic imbalance, urinary infection, or significant upper urinary tract dilatation. Two fatalities could not be linked to urinary diversion. Four patients were not followed up on. Due to the limited number of specialist surgical facilities, CBE late referral or failed closure is to be expected in a resource-limited context. In lieu of the primary closure, a continent internal diversion will be proposed and encouraged even at the level of a non-specialist hospital to improve the quality of life of these unfortunate patients. It is recommended that patients be warned about the procedure’s potential
long-term risks, which will necessitate a limited but regular
follow-up.
... In children born with spinal dysraphism (SD) or exstrophy epispadias complex (EEC), there are two primary urological goals: preserve renal function and treat urinary incontinence (UI) [1,2]. UI in these cases is severe with a deleterious social impact, especially at school [3][4][5][6]. UI occurs as a combination of intrinsic sphincteric deficiency (ISD) and/or bladder dysfunction such as detrusor overactivity or/and poor compliance. Many surgical techniques, sometimes combined, have been proposed to improve urethral function: bulking agent injections at bladder neck (BANI), bladder neck surgical reconstruction (BNSR), slings to support bladder neck or mid-urethra and eventually artificial urinary sphincters (AUSs). ...
Purpose:
To present our initial experience with periurethral adjustable continence therapy (ACT™) for urinary incontinence due to intrinsic sphincter deficiency (ISD) in children.
Methods:
This is an approved prospective non-randomized pilot study (NCT03351634) aiming to treat children born with spinal dysraphism (SD) or exstrophy epispadias complex (EEC) with ACT™. Endpoints were patient-reported changes in daily pad count, 24-h Pad test and complications.
Results:
Since April 2018, 13 children (six girls, seven boys) were implanted at the median age of 12 years (5-16). The etiology of incontinence was neurogenic ISD (7/13, 54%) and EEC (6/13, 46%). After ACT™ implantation, continence (no pad or 1 security pad/day) was achieved in 9(69%) patients (5/7 SD, 4/6 EEC). Additionally, two (15%) patients had a significant improvement (decreasing Pad test from 1049 to 310 g at 3 months). One patient (7%) had no improvement. Results were stable at 21 months (6-43) of follow-up. Mean final balloon volume was 2.89 ml (± 0.85) with a median of 3 fillings to obtain continence. We had four revisions due to cutaneous port erosion (n = 3) and balloon migration (n = 1) and two definitive explantations. PinQ score was significantly improved (47 vs 40.5 with balloon, p = ns). Neither degradation of the upper urinary tract nor cystomanometric changes have been observed at 6 and 12 months postoperatively.
Conclusion:
Urinary incontinence due to ISD owing to EEC or SD can be successfully treated with ACT™ periurethral balloons. Given the minimal invasiveness of this therapy, it might be a first-line option treatment in children with complex stress urinary incontinence.
... On the other hand, because it is an extremely rare and 1 3 232 Page 2 of 6 complicated condition, it is difficult to determine the optimal treatment strategies based on the findings of cases that have been treated at a single institution. Most patients with CE require multiple reconstructive urinary procedures to achieve continence [5], which often consists of bladder augmentation (BA) in addition to bladder neck reconstruction and continent catheterized channel creation after the initial approximation of the bladder halves to improve their quality of life (QOL). However, the outcomes after BA remain unclear. ...
Purpose Cloacal exstrophy (CE) patients may need bladder reconstruction after initially undergoing surgery to obtain continence
and improve their quality of life. This study attempts to clarify the clinical features of CE patients who underwent
bladder augmentation (BA) and their urinary functional outcomes based on a nationwide survey in Japan.
Methods A questionnaire survey was conducted, and 150 CE patients were enrolled. Their clinical characteristics and urinary
outcomes were reviewed.
Results BA was performed in 52 patients (34.7%). Most cases underwent early bladder closure at initial surgery in neonate
period. The age at the BA was performed 6.4 [6–9.0] years. Among them, the most used organ for BA was ileum (n = 30,
57.7%). Regarding the outcomes, the age when the renal function was evaluated was 14.0 [10.0–20.5] years and the serum
creatinine level was 0.44 [0.36–0.60] (mg/dl). Clean intermittent catheterization was required in 37 (71.2%) patients. On the
other hand, no dialysis or kidney transplantation was necessary in any of these patients.
Conclusion The renal function and conditions of patients who underwent BA were relatively well preserved. Individualized
management with a stepwise surgical approach for CE patients should thus be considered in the future.
... On the other hand, because it is an extremely rare and complicated condition, it is di cult to determine the optimal treatment strategies based on the ndings of cases that have been treated at a single institution. Most patients with CE require multiple reconstructive urinary procedures to achieve continence [5], which often consists of bladder augmentation (BA) in addition to bladder neck reconstruction and continent catheterized channel creation after the initial approximation of the bladder halves to improve their quality of life (QOL). However, the outcomes after BA remain unclear. ...
PURPOSE
Cloacal exstrophy (CE) patients may need bladder reconstruction after initially undergoing surgery to obtain continence and improve their quality of life. This study attempts to clarify the clinical features of CE patients who underwent bladder augmentation (BA) and their urinary functional outcomes based on a nationwide survey in Japan.
METHODS
A questionnaire survey was conducted, and 150 CE patients were enrolled. Their clinical characteristics and urinary outcomes were reviewed.
RESULTS
BA was performed in 52 patients (34.7%). Most cases underwent early bladder closure at initial surgery in neonate period. The age at the BA was performed 6.4 [6–9.0] years old. Among them, the most used organ for BA was ileum (n = 30, 57.7%). Regarding the outcomes, the age when the renal function was evaluated was 14.0 [10.0–20.5] years old and the serum creatinine level was 0.44 [0.36–0.60] (mg/dl). Clean intermittent catheterization was required in 37 (71.2%) patients. On the other hand, no dialysis or kidney transplantation was necessary in any of these patients.
CONCLUSION
The renal function and conditions of patients who underwent BA were relatively well preserved. Individualized management with a stepwise surgical approach for CE patients should thus be considered in the future.
... The primary issues with transitional care for voiding dysfunction in bladder exstrophy are the poor rates of volitional voiding per urethra, high incidence of repeat surgical procedures, and high rates of complication such as stone formation. Maruf and authors found in their review of 432 patients who underwent successful bladder closure and a urinary continence procedure, that only 25% could void normally per urethra at a mean follow-up of 7.2 years [26]. Following a repeat continence procedure, the rate of volitional per urethral voiding is even lower, with Burki reporting a 0% rate of volitional voiding post-redo bladder neck reconstruction (BNR) in 30 patients [27]. ...
Purpose of Review:
To provide an overview of the literature pertaining to voiding dysfunction in the transitional urology patient population. Specific note is made of Spina Bifida, Posterior Urethral Valves, Prune Belly Syndrome, and Bladder Exstrophy, as well as acquired conditions such as spinal cord injury and sequelae of childhood malignancy treatment.
Recent Findings:
There are some recently published trials that have attempted to rationalise the assessment and management of this population of patients. These range from longitudinal studies to some randomised control trials, and some remain opinion-based articles or local experience publications.
Summary:
This is a heterogenous group of patients who have a wide variety of pathology and requirements that will constantly change over time. Awareness of the variability and the need for close monitoring underpins all management strategies. There is a clear requirement for an interdisciplinary approach here to support excellent treatment adherence and management of complications.
... BNR results in social continence in half of the children (Shoukry et al., 2009). Seventy-five percentage of patients are lifelong dependent on catheterization or other non-physiologic urinary drainage (Maruf et al., 2020). Complications include recurrent infections, urinary stones, dilatation of the upper urinary tract, urinary retention, renal impairment, metabolic complications, increased risk of cancer, revision surgery, hospitalization, as well as wideranging limitations in daily life and severe psychological distress (Mouriquand et al., 2003;Surer et al., 2003;Ebert et al., 2020). ...
Objective
Idiopathic inflammatory myopathies (IIMs) are a heterogeneous group of complex autoimmune conditions characterized by inflammation in skeletal muscle and extramuscular compartments, and interferon (IFN) system activation. We undertook this study to examine the contribution of genetic variation to disease susceptibility and to identify novel avenues for research in IIMs.
Methods
Targeted DNA sequencing was used to mine coding and potentially regulatory single nucleotide variants from ~1,900 immune‐related genes in a Scandinavian case–control cohort of 454 IIM patients and 1,024 healthy controls. Gene‐based aggregate testing, together with rare variant– and gene‐level enrichment analyses, was implemented to explore genotype–phenotype relations.
Results
Gene‐based aggregate tests of all variants, including rare variants, identified IFI35 as a potential genetic risk locus for IIMs, suggesting a genetic signature of type I IFN pathway activation. Functional annotation of the IFI35 locus highlighted a regulatory network linked to the skeletal muscle–specific gene PTGES3L, as a potential candidate for IIM pathogenesis. Aggregate genetic associations with AGER and PSMB8 in the major histocompatibility complex locus were detected in the antisynthetase syndrome subgroup, which also showed a less marked genetic signature of the type I IFN pathway. Enrichment analyses indicated a burden of synonymous and noncoding rare variants in IIM patients, suggesting increased disease predisposition associated with these classes of rare variants.
Conclusion
Our study suggests the contribution of rare genetic variation to disease susceptibility in IIM and specific patient subgroups, and pinpoints genetic associations consistent with previous findings by gene expression profiling. These features highlight genetic profiles that are potentially relevant to disease pathogenesis.
... Follow-ups are frequently limited, and definitions of continence vary between authors who frequently describe "manageable" incontinence, too short catheterization intervals and/or nocturnal incontinence. Transurethral volitional micturition with continence seems to be uncommon, varying from 1/65 (4) to 25% after classical staged repair (25) and 41.4% with CPRE in Seattle (26), all data from referral centers. A more recent report the latter group, concerning exclusively females (n=18, 11 post-pubertal), is more sober: the patients needed a mean of 3.2±2.5 continence procedures and 22.2% of augmentations, with a third presenting "perfect continence" and half "partial continence" (including nocturnal incontinence) (21). ...
... Continence/dryness was assessed in 350 patients after a mean of 7.2 years. 64% and 93% were dry after BNR and BNC, respectively and about 25% were capable of volitional micturition per urethra (25). ...
INTRODUCTION
Bladder exstrophy (BE) affects continence and sexual function, impacting on social life and mental health. Long-term data from the patients´ point of view are needed to get a real-life perspective on the problem.
STUDY DESIGN
A self-developed questionnaire concerning sexual, psychosexual and psychosocial outcomes was sent to the adult members of the Brazilian Exstrophy Group.
RESULTS
Fifty out of 67 adults from the group (74.5%) responded to the questionnaire. Failure of initial bladder closure attained 62%. Almost ¾ of the patients had augmentation cystoplasty. Bladder lithiasis was common. Esthetic procedures were frequently done. Repetitive UTI (n=32, 64%) and kidney scars/disease (n=20, 40%) were frequent. Most (88%) patients either depend on CIC or remain incontinent. Sexual problems predominated in males. Surgery for continence often failed, requiring re-operations, but the prognosis without these procedures was comparatively worse. Continent patients underwent more surgeries (mean 18, 13 and 9 procedures in continent, imperfectly continent and incontinent patients, respectively). Augmented patients more frequently achieved dryness (p=0.0035). Two-thirds of the women underwent vaginoplasties, but dyspareunia/feeling of “tight” vagina still affected a quarter of them. Four women (15.4%) delivered healthy children. 91.7% of the males reported “normal” erections, but sexual inhibition was common due to feeling of having a small penis (n=18, 75%). Persistent dorsal curvature and abnormal ejaculation were common (58.3% and 77.1%, respectively). Patients´ comments related mainly to mental health issues/need for specialized care, limitations of medicine to cure/treat their disease, unavailability of experts, especially adult specialists, embarrassment over deformities and insufficient information about disease/treatment/prognosis.
DISCUSSION
Most BE patients are well-integrated into society, but feelings of sadness and low self-esteem are common. Most welcome procedures to become dry, despite self-catheterization. The results of bladder neck reconstruction are far from perfect, despite multiple attempts and bladder augmentation was often necessary. Volitional voiding is uncommon. Sexual problems are worse for males, and sexual avoidance is common. Sexual function and self-image are inter-related. It seems reasonable to offer selective esthetic procedures to improve social/sexual interaction. Obstetric complications are common, especially UTI, need for ureteral and/or conduit stenting, abnormal fetal positioning, uterine prolapse, technical problems during surgical deliveries and prematurity.
CONCLUSION
Continence/dryness in BE was mostly eventually achieved, usually depending on multiple interventions, bladder augmentation and self-catheterization. Despite multiple surgeries many adults remain incontinent. Sexual problems and avoidance are the rule in males, due to the feelings of penile inadequacy. Pregnant females deserve expert obstetric care.
... BNR results in social continence in half of the children (Shoukry et al., 2009). Seventy-five percentage of patients are lifelong dependent on catheterization or other non-physiologic urinary drainage (Maruf et al., 2020). Complications include recurrent infections, urinary stones, dilatation of the upper urinary tract, urinary retention, renal impairment, metabolic complications, increased risk of cancer, revision surgery, hospitalization, as well as wideranging limitations in daily life and severe psychological distress (Mouriquand et al., 2003;Surer et al., 2003;Ebert et al., 2020). ...
This article will review myogenic cell transplantation for congenital and acquired diseases of skeletal muscle. There are already a number of excellent reviews on this topic, but they are mostly focused on a specific disease, muscular dystrophies and in particular Duchenne Muscular Dystrophy. There are also recent reviews on cell transplantation for inflammatory myopathies, volumetric muscle loss (VML) (this usually with biomaterials), sarcopenia and sphincter incontinence, mainly urinary but also fecal. We believe it would be useful at this stage, to compare the same strategy as adopted in all these different diseases, in order to outline similarities and differences in cell source, pre-clinical models, administration route, and outcome measures. This in turn may help to understand which common or disease-specific problems have so far limited clinical success of cell transplantation in this area, especially when compared to other fields, such as epithelial cell transplantation. We also hope that this may be useful to people outside the field to get a comprehensive view in a single review. As for any cell transplantation procedure, the choice between autologous and heterologous cells is dictated by a number of criteria, such as cell availability, possibility of in vitro expansion to reach the number required, need for genetic correction for many but not necessarily all muscular dystrophies, and immune reaction, mainly to a heterologous, even if HLA-matched cells and, to a minor extent, to the therapeutic gene product, a possible antigen for the patient. Finally, induced pluripotent stem cell derivatives, that have entered clinical experimentation for other diseases, may in the future offer a bank of immune-privileged cells, available for all patients and after a genetic correction for muscular dystrophies and other myopathies.
... In this series, continence was achieved in 100% of patients who underwent bladder neck closure with continent catheterizeable stoma. In a recent publication, Maruf et al., reviewed data of 133 patients with bladder neck closure and continent stoma, and found that continence was achieved in 93% [15]. The higher continence-rate in our series can be explained by the fact that Maruf et al. reviewed continence status in a much larger study population (133 patients vs 38 patients). ...
Introduction
Scarce data is available in literature about the upper urinary tract outcomes of patients with Exstrophy-Epispadias Complex (EEC). After bladder closure during childhood, EEC bladders can become hostile to the upper tracts after bladder by exposing them to high pressures, leading to hydronephrosis (HN) and kidney damage. Similarly, vesicoureteral reflux (VUR) may be present and increase the likelihood for pyelonephritis.
Objective
We sought to assess long-term upper urinary tract outcomes by evaluating renal function, HN and VUR; and to assess if upper urinary tract outcomes are associated with continence status.
Study design
A retrospective review of EEC patients having ≥1 surger(y)(ies) at our institution from 1990 until 2019 was performed. Renal function was assessed by evaluating last available estimated glomerular filtration rate (eGFR) and creatinine values. HN was assessed on ultrasound and classified according to the SFU-classification. Patients with recurrent febrile urinary tract infections (UTI) or pyelonephritis underwent a voiding-cystourethrogram (VCUG) assessing VUR, graded following the ‘International system of radiographic grading of VUR’. Descriptive and comparative statistical analysis were performed to assess if upper tract outcomes are associated with continence status.
Results
Forty-eight patients (75% male) had a median (IQR) follow-up of 18 (10-21) years. The table shows upper tract outcomes for the entire group and stratified by continence status. The median creatinine was 0.6 (0.2-0.9) mg/dL and median eGFR was 108 (72-160) mL/min/1.73m ²
• Sinatti C.
• Waterschoot M.
• Roth J.
• Van Laecke E.
• Hoebeke P.
• Spinoit A.F.
Long-term sexual outcomes in patients with exstrophy-epispadias complex. Int J Impot Res. 2020; (Published online)https://doi.org/10.1038/s41443-020-0248-2
• Google Scholar
. In two patients (4.2%), HN (SFU-grade 2) was detected. Thirty-six patients (75%) underwent VCUG, revealing high-grade VUR (stage IV-V) in 8 patients (17%) and low-grade VUR (stage I-III) in 7 patients (15%). Continence was associated with a higher need for VCUG (p=0.02) and a higher presence of VUR (p=0.03).
Discussion
Renal function in EEC patients and non-EEC patients is comparable when age matched. Only 6% had low-grade HN which was asymptomatic. 17% had high-grade VUR, which is little compared to literature (40 - 70%). However, results in literature are described in patients with a ‘one-stage’ bladder closure, whereas some of our patients had a ‘two-stage’ procedure. A one-stage procedure creates higher bladder pressures resulting in higher VUR-rates. Statistical analysis has showed that continence is associated with a higher prevalence of recurrent febrile UTI’s or pyelonephritis and of VUR.
Conclusions
No statistically differences were found between continent and incontinent patients concerning creatinine and eGFR value (p=0.52 and p=0.29), nor in the prevalence of hydronephrosis (p=0.36). However, results of this study suggest that continent patients may portend a higher risk of upper tract deterioration with recurrent febrile UTI’s and pyelonephritis due to VUR. Close monitoring of the upper tract status is therefore as important as focus on continence. Large-scale prospective studies defining renal function as well as pyelonephritis rates are needed to optimize the management of the upper tracts in EEC patients.
