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The Journal of Sexual Medicine Feb 2021Males born with bladder exstrophy-epispadias complex generally have a shorter phallus, split corpora with dorsal curvature, and a scarred and flattened glans, so...
BACKGROUND
Males born with bladder exstrophy-epispadias complex generally have a shorter phallus, split corpora with dorsal curvature, and a scarred and flattened glans, so substitution phalloplasty is often required.
AIM
The aim of this study was to review the techniques, complications, and outcomes of substitution phalloplasty in bladder exstrophy-epispadias complex patients to determine the ideal surgical procedure and gauge the risks and benefits for the patient.
METHODS
A systematic review of the literature was performed using PubMed/MEDLINE and the Cochrane Library with the following terms: ("phalloplasty"); (("epispadias") OR ("bladder exstrophy") OR ("cloacal exstrophy")). We included only full-text articles reporting data about techniques and outcomes of substitution phalloplasty in patients with bladder exstrophy-epispadias complex.
OUTCOMES
To determine whether patients with bladder exstrophy-epispadias complex might benefit from substitution phalloplasty.
RESULTS
We selected 7 studies involving 47 patients. All the studies were characterized by a low level of evidence and a heterogeneous approach during treatment and outcome assessment. The free radial forearm flap was the most commonly performed technique (89%) with an overall complication rate of 15%. Urethroplasty was performed in 22 of 47 (47%) patients, and in most cases (20/22) a "tube-within-the-tube" technique was performed simultaneously with the phalloplasty (20/47). Urethroplasty complications were recorded in 12 of 22 (54%) patients with 6 fistulae and 6 stenoses. A penile prosthesis was implanted in 32 of 47 (68%) patients and complications occurred in 8 of 32 (25%) patients with 6 erosion. Aesthetic, sexual, and psychological outcomes were satisfactory, but none of the studies used validated instruments for the final assessment.
CLINICAL IMPLICATIONS
It was not possible to formulate any recommendations based on a high level of evidence regarding substitution phalloplasty in patients with bladder exstrophy-epispadias complex.
STRENGTH & LIMITATION
To our knowledge, this is the first review to address bladder exstrophy-epispadias complex patients only. The limitations are mainly represented by the small number of cases because of the rarity of this disease and by the fact that no studies used validated instruments.
CONCLUSION
Substitution phalloplasty in patients with bladder exstrophy-epispadias complex can achieve good functional, aesthetic, psychological, and sexual outcomes. It requires multiple procedures and carries a high complication rate. Multicentric studies including the assessment of patients by means of a validated questionnaire which investigates both sexual function and psychosexual satisfaction are required. Berrettini A, Sampogna G, Gnech M, et al. Substitution Phalloplasty in Patients With Bladder Exstrophy-Epispadias Complex: A Systematic Review of Techniques, Complications, and Outcomes. J Sex Med 2021;18:400-409.
Topics: Bladder Exstrophy; Epispadias; Humans; Male; Penile Prosthesis; Surgical Flaps; Urethra
PubMed: 33223423
DOI: 10.1016/j.jsxm.2020.10.007 -
British Journal of Urology Aug 1978
Topics: Adult; Bladder Exstrophy; Female; Humans
PubMed: 753490
DOI: 10.1111/j.1464-410x.1978.tb03651.x -
The Urologic Clinics of North America Aug 2004Bladder exstrophy is a rare condition associated with lifelong issues including infection,renal insufficiency, incontinence, and fertility. Despite its seriousness, many... (Review)
Review
Bladder exstrophy is a rare condition associated with lifelong issues including infection,renal insufficiency, incontinence, and fertility. Despite its seriousness, many affected children lead normal lives. With new operative techniques, children require fewer surgeries and have more hope for continence. With close follow-up, no child should develop renal insufficiency. Families should be reassured that a normal life and parenthood with normal sexual function are possible.
Topics: Animals; Bladder Exstrophy; Humans; Infant, Newborn; Postoperative Care; Suture Techniques; Urinary Bladder
PubMed: 15313051
DOI: 10.1016/j.ucl.2004.04.023 -
Journal of Pediatric Urology Feb 2021Bladder exstrophy patients have a high prevalence of inguinal hernia that often become clinically evident following bladder closure. Understanding when the bladder...
INTRODUCTION/BACKGROUND
Bladder exstrophy patients have a high prevalence of inguinal hernia that often become clinically evident following bladder closure. Understanding when the bladder exstrophy patient is under greatest risk of developing an inguinal hernia following bladder closure is important, since incarceration resulting in strangulation of intra-abdominal contents can lead to significant morbidity if not addressed in a timely fashion. Although the incidence and risk factors of inguinal hernia have been reported, the timing of occurrence is not well understood.
OBJECTIVE
The primary objective of this study was to assess the timing of inguinal hernia following complete primary repair of bladder exstrophy (CPRE). In addition, we aimed to evaluate possible risk factors associated with inguinal hernia, including sex, age at bladder closure and iliac osteotomy status.
STUDY DESIGN
A multi-institutional retrospective review identified patients with bladder exstrophy repaired by CPRE under 6 months of age while excluding those who underwent inguinal hernia repair before or during bladder closure. Timing of inguinal hernia following bladder closure was evaluated using Kaplan-Meier methods. Cox proportional hazards model was used to investigate association of sex, age at bladder closure, and osteotomy on the risk of developing of inguinal hernia while clustering for institution.
RESULTS
91 subjects were included in our analysis with median follow-up time of 6.5 years. 34 of 53 males (64.2%) and 2 of 38 females (5.3%) underwent inguinal hernia repair. The median time to inguinal hernia was 4.7 months following closure. The greatest hazard of inguinal hernia was within the first six months following closure. In multivariate analysis, male sex was strongly associated with inguinal hernia (HR = 19.00, p = 0.0038). Osteotomy and delay in closure were not significantly associated with inguinal hernia. 7 of 36 patients (19.4%) who underwent inguinal hernia repair presented with recurrence on the ipsilateral side.
DISCUSSION
Our results suggest that the greatest risk of inguinal hernia is within the first six months following bladder closure. The decreased risk of inguinal hernia after one year of follow-up may reflect anatomic stability that is reached following major reconstruction of the pelvis. While male bladder exstrophy patients are significantly more susceptible to inguinal hernias following CPRE, osteotomy and delayed bladder closure do not appear to be protective factors for inguinal hernia development following initial bladder closure.
CONCLUSIONS
There is a heightened risk of inguinal hernia in the first six months following closure. The rate of recurrence following inguinal hernia repair is significantly elevated compared to the general pediatric population.
Topics: Bladder Exstrophy; Child; Female; Hernia, Inguinal; Humans; Male; Retrospective Studies; Treatment Outcome; Urologic Surgical Procedures
PubMed: 33317945
DOI: 10.1016/j.jpurol.2020.11.025 -
Journal of Pediatric Urology Aug 2016
Topics: Bladder Exstrophy; Humans; Infant, Newborn; Urologic Surgical Procedures
PubMed: 27593919
DOI: 10.1016/j.jpurol.2016.01.001 -
Nature Clinical Practice. Urology Feb 2006Staged reconstruction repair of bladder exstrophy results in hydronephrosis or renal scarring in 15-25% of patients. A cosmetically acceptable and functional phallus can... (Review)
Review
Staged reconstruction repair of bladder exstrophy results in hydronephrosis or renal scarring in 15-25% of patients. A cosmetically acceptable and functional phallus can be achieved in 85% of patients, 20-30% of whom will require more than one operation for penile reconstruction. Episodes of penile glans loss or corporal loss are rarely reported with this technique. Widely disparate results relating to complete urinary continence and volitional voiding have been published, with urinary continence reported to occur in 7-85% of patients. The need for bladder augmentation to obtain urinary continence also varies, with reports that somewhere between 10% and 90% of patients require an augmentation procedure to gain urinary continence. Complete primary repair of bladder exstrophy using the penile disassembly technique results in hydronephrosis or renal scarring in 0-30% of patients, and hypospadias, as a consequence of this repair, will occur in 30-70% of patients. Loss of the glans and corpora appear more frequently with penile disassembly than in staged reconstruction of bladder exstrophy, however, the exact incidence of this complication is unknown. Reported complete urinary continence and volitional voiding rates are also varied following penile disassembly, ranging from 25-65%. A modified bladder-neck reconstruction to gain urinary continence is reportedly required in 15-90% of patients, with 5-10% requiring both bladder augmentation and bladder-neck reconstruction. Experience with complete primary repair of bladder exstrophy, using the penile disassembly approach, seems promising but is not a panacea. To outline the risks and benefits regarding the various surgical techniques for bladder exstrophy, we would recommend the establishment of a national registry for patients with this disorder.
Topics: Bladder Exstrophy; Humans; Kidney; Male; Penis; Risk Assessment; Treatment Outcome; Urinary Bladder; Urologic Surgical Procedures
PubMed: 16470208
DOI: 10.1038/ncpuro0407 -
Journal of Ultrasound in Medicine :... Sep 2017Bladder exstrophy is a rare severe congenital malformation. Early prenatal diagnosis is scarcely described in the literature. Low insertion of the umbilical cord is a...
OBJECTIVES
Bladder exstrophy is a rare severe congenital malformation. Early prenatal diagnosis is scarcely described in the literature. Low insertion of the umbilical cord is a constant anatomic feature of bladder exstrophy. The aim of our study was to assess whether early measurements of the umbilical cord insertion-to-genital tubercle length may serve as quantitative measurements for a low-inserted umbilical cord in cases of bladder exstrophy.
METHODS
The umbilical cord insertion-to-genital tubercle length was prospectively measured in all cases referred for a nonvisualized urinary bladder before 18 weeks' gestation. Final diagnoses were compared with prenatal measurements.
RESULTS
Fifteen fetuses were evaluated for a nonvisualized bladder at a mean gestational age of 15.7 weeks (range, 14-17 weeks). Of them, 6 cases were diagnosed with bladder exstrophy, and 9 cases had a normal urinary bladder. All cases with bladder exstrophy had an umbilical cord insertion-to-genital tubercle length below the fifth percentile for gestational age, whereas cases with a normal bladder had a normal measurement.
CONCLUSIONS
Fetuses with bladder exstrophy have an umbilical cord insertion-to-genital tubercle length below the fifth percentile of the general population. This measurement may serve as a complementary objective sonographic parameter in the prenatal assessment and counseling of cases suspected of having bladder exstrophy during early pregnancy.
Topics: Bladder Exstrophy; Early Diagnosis; Evaluation Studies as Topic; Female; Humans; Pregnancy; Prospective Studies; Ultrasonography, Prenatal; Umbilical Cord
PubMed: 28436540
DOI: 10.1002/jum.14212 -
Indian Journal of Pediatrics Aug 2009A two-day-old newborn male child with a rare variant of exstrophy bladder was managed in our institute. The child has true duplicate bladder exstrophy which is extremely... (Review)
Review
A two-day-old newborn male child with a rare variant of exstrophy bladder was managed in our institute. The child has true duplicate bladder exstrophy which is extremely rare and only 8 cases reported in the world literature so far. We describe another one and briefly review the literature.
Topics: Bladder Exstrophy; Diagnosis, Differential; Humans; Infant, Newborn; Male; Urinary Bladder
PubMed: 19802554
DOI: 10.1007/s12098-009-0172-4 -
Urology Dec 2022To present a case series of the exstrophy-epispadias complex (EEC) with isolated ectopic bowel segment (IEBS) with the literature review, highlighting the clinical... (Review)
Review
OBJECTIVE
To present a case series of the exstrophy-epispadias complex (EEC) with isolated ectopic bowel segment (IEBS) with the literature review, highlighting the clinical findings and treatments.
MATERIALS AND METHODS
We present 3 cases of bladder exstrophy (BE) with IEBS in our institute and reviewed the literature in PubMed with the terms "("bladder exstrophy" OR "epispadias") AND ("visceral sequestration" OR "sequestered" OR "ectopic bowel")."
RESULTS
There were 2 males and 1 female. The IEBS was detected by physical examination in 2 cases and by ultrasonography in another one. All cases were BE accompanying with lower abdominal mass which adhered to the bladder wall but was separated from the digestive system. All cases underwent the IEBS excision and BE repair simultaneously. Pathological result of IEBS suggested the histological structures of colon. There were totally 13 cases of EEC with IEBS reported in the literature, including 2 (15%) epispadias, 9 (69%) covered BE, 1 (8%) duplicate BE and 1 (8%) classic bladder exstrophy. Although their clinical manifestations were various, IEBS excision were safely conducted in all cases.
CONCLUSION
EEC with IEBS is an extremely rare congenital malformation. Physical and imaging examinations are important for diagnoses. Surgical excision is safe and effective for managing IEBS.
Topics: Male; Female; Humans; Epispadias; Bladder Exstrophy; Urinary Bladder; Colon; Digestive System Abnormalities
PubMed: 35970355
DOI: 10.1016/j.urology.2022.08.003 -
Journal of Pediatric Urology Oct 2019Classic bladder exstrophy is one of the rarest congenital anomalies compatible with life. Surgical treatment of bladder exstrophy has progressed, but the goal of surgery...
INTRODUCTION
Classic bladder exstrophy is one of the rarest congenital anomalies compatible with life. Surgical treatment of bladder exstrophy has progressed, but the goal of surgery remains a successful primary bladder closure. Several factors have been identified to decrease the risk of failed closure, including appropriate use of osteotomy and adequate postoperative immobilization and analgesia. However, the role of the radical anatomic pelvic dissection, including dissection of the urogenital diaphragm fibers, in a successful closure has not yet been extensively explored.
OBJECTIVE
The objective of this study was go examine the role of radical anatomic pelvic dissection, including dissection of the urogenital diaphragm fibers, in patients with classic bladder exstrophy.
STUDY DESIGN
This was a retrospective study based on an institutional database.
METHODS
A retrospective review from an institutional approved database of more than 1,300 patients with epispadias-exstrophy complex was performed. The inclusion criteria included patients with classic bladder exstrophy with at least one failed bladder closure and a reclosure at the authors' institution with a single senior surgeon. Data collection included demographics, clinical variables, and status of urogenital diaphragm fibers. Magnetic resonance imaging (MRI) scans, if available, were reviewed with a pediatric radiologist to identify urogenital diaphragm fibers.
RESULTS
From the database, 93 patients met inclusion criteria. Of these patients, 74 had urogenital diaphragm fibers completely intact at the time of repeat closure, whereas 19 patients did not. There was no association with age or gender and status of urogenital diaphragm fibers. There was no association with osteotomy, the type of primary bladder closure, surgeon subspecialty, and the status of the urogenital fibers. Fourteen patients had at least two prior closures; surprisingly, 11 of these repeat closure patients still had intact urogenital fibers even after two prior closures.
DISCUSSION
The recent development and application of 3D MRI-guided pelvic dissection in a large group of patients led the authors to investigate whether adequate pelvic floor dissection had been accomplished at primary or secondary closure. Several patients had MRI scans performed before repeat closure in which the urogenital diaphragm fibers were identified to be intact on imaging; this was corroborated with surgical findings. Approximately 80% of patients had their urogenital diaphragm fibers completely intact and, therefore, did not have an adequate pelvic dissection during their primary or secondary bladder closure, putting the success of their previous closures at risk.
CONCLUSION
Inadequate pelvic diaphragm dissection, defined as intact urogenital diaphragm fibers, demonstrated in a large group of patients with failed exstrophy closure, may be a decisive factor in bladder closure failure. The use of 3D intra-operative image guidance may aid in a safer and more successful pelvic dissection.
Topics: Bladder Exstrophy; Female; Humans; Imaging, Three-Dimensional; Infant, Newborn; Magnetic Resonance Imaging; Male; Osteotomy; Pelvic Floor; Retrospective Studies; Treatment Outcome; Urologic Surgical Procedures
PubMed: 31383518
DOI: 10.1016/j.jpurol.2019.06.025