Purpose We review the current literature regarding urological management of spina bifida from prenatal diagnosis to adulthood. Materials and Methods We searched MEDLINE® , EMBASE® and PubMed® for ...English articles published through December 2014 using search terms “spina bifida,” “spinal dysraphism” and “bladder.” Based on review of titles and abstracts, 437 of 1,869 articles were identified as addressing topics related to open spina bifida in pediatric patients, or long-term or quality of life outcomes in adults with spina bifida. We summarize this literature to inform clinical guidelines and create a framework for disease management. Results The birth prevalence of spina bifida in the United States has recently plateaued at approximately 30 per 100,000. With improved management more individuals are surviving to adulthood, with an economic impact of $319,000 during the lifetime of an individual with spina bifida. Recent advances in prenatal surgery have demonstrated that prenatal closure of spina bifida is possible. To assess safety and efficacy, the National Institutes of Health sponsored Management of Myelomeningocele Study was undertaken, in which subjects were randomized to prenatal or postnatal closure. Until the urological results of this trial are published, the impact of prenatal intervention on future bladder function remains unclear. Controversy continues regarding the optimal use and timing of urodynamic studies, and the indications for initiation of clean intermittent catheterization and anticholinergics in infants and children. Many favor expectant management, while others argue for a more proactive approach. Based on the current literature, both approaches appear to protect the child from renal injury, although delayed intervention may increase rates of bladder augmentation. The current literature regarding this topic is difficult to interpret and compare due to heterogeneity of patient populations, variable outcome measures and lack of reporting of quality of life outcomes. Surgical intervention is indicated for those at risk for renal deterioration and/or is considered for children who fail to achieve satisfactory continence with medical management. Traditionally surgery concentrates on the bladder and bladder neck, and creation of catheterizable channels. For those with a hostile bladder, enterocystoplasty remains the gold standard for bladder augmentation, although use of bowel for augmentation remains suboptimal due to secondary complications, including increased risk of infections, metabolic abnormalities, neoplastic transformation and risk of life threatening perforation. Recent advances in tissue engineering technology may provide an alternative to traditional augmentation. However, recent results from phase II trials using current techniques to augment the bladder with engineered bladder tissue are disappointing. Catheterizable channels to the bladder and ascending colon further facilitate continence measures and promote independent care. While surgical reconstruction is clearly successful in improving continence, recent outcome studies have questioned the true impact of this type of surgery on quality of life. With improved survival transitional care issues, including health related independence, sexual health needs and development of a support system, are increasingly important. Transitional care remains a significant issue for which few public health measures are being quantitatively evaluated. Conclusions Despite consensus regarding early urological involvement in the care of patients with spina bifida, controversy remains regarding optimal management. Major reconstructive urological surgeries still have a major role in the management of these cases to protect the upper urinary tract and to achieve continence. However, future studies are needed to better clarify the true impact on quality of life that these interventions have on patients and their families. Transition of urological care to adulthood remains a major avenue for improvement in disease management.
Purpose Cryptorchidism is one of the most common pediatric disorders of the male endocrine glands and the most common genital disorder identified at birth. This guideline is intended to provide ...physicians and non-physician providers (primary care and specialists) with a consensus of principles and treatment plans for the management of cryptorchidism (typically isolated non-syndromic). Materials and Methods A systematic review and meta-analysis of the published literature was conducted using controlled vocabulary supplemented with key words relating to the relevant concepts of cryptorchidism. The search strategy was developed and executed by reference librarians and methodologists to create an evidence report limited to English-language, published peer-reviewed literature. This review yielded 704 articles published from 1980 through 2013 that were used to form a majority of the guideline statements. Clinical Principles and Expert Opinions were used for guideline statements lacking sufficient evidence-based data. Results Guideline statements were created to inform clinicians on the proper methods of history-taking, physical exam, and evaluation of the boy with cryptorchidism, as well as the various hormonal and surgical treatment options. Conclusions Imaging for cryptorchidism is not recommended prior to referral, which should occur by 6 months of age. Orchidopexy (orchiopexy is the preferred term) is the most successful therapy to relocate the testis into the scrotum, while hormonal therapy is not recommended. Successful scrotal repositioning of the testis may reduce but does not prevent the potential long-term issues of infertility and testis cancer. Appropriate counseling and follow-up of the patient is essential.
Chronic kidney disease affects 25% to 50% of patients with spina bifida. Guidelines recommend kidney function surveillance in these patients but practice patterns are unknown. Variations in kidney ...function surveillance were assessed in patients with spina bifida based on the hypothesis that the treating clinic and spina bifida type would be associated with kidney function surveillance.
A retrospective cohort study was conducted of U.S. patients in the National Spina Bifida Patient Registry from 2013 to 2018. Followup was anchored at the 2013 visit. Participants with either an outcome event within 2 years of followup or more than 2 years of followup without an outcome event were included. Primary outcome was kidney function surveillance, defined as at least 1 renal ultrasound and serum creatinine within 2 years of followup. Primary exposures were clinic and spina bifida type, which were analyzed with covariates including sociodemographic and clinical characteristics in logistic regression models for their association with the outcome. Sensitivity analyses were performed using different kidney function surveillance definitions.
Of 8,351 patients 5,445 were included with a median followup of 3.0 years. Across 23 treating clinics kidney function surveillance rates averaged 62% (range 6% to 100%). In multivariable models kidney function surveillance was associated with treating clinic, younger patient age, functional lesion level, nonambulatory status and prior bladder augmentation. Treating clinic remained a significant predictor of kidney function surveillance in all sensitivity analyses.
Within the National Spina Bifida Patient Registry wide variation exists in practice of kidney function surveillance across treating clinics despite adjustment for key patient characteristics.
A substudy of the Management of Myelomeningocele Study evaluating urological outcomes was conducted.
Pregnant women diagnosed with fetal myelomeningocele were randomly assigned to either prenatal or ...standard postnatal surgical repair. The substudy included patients randomly assigned after April 18, 2005. The primary outcome was defined in their children as death or the need for clean intermittent catheterization (CIC) by 30 months of age characterized by prespecified criteria. Secondary outcomes included bladder and kidney abnormalities observed by urodynamics and renal/bladder ultrasound at 12 and 30 months, which were analyzed as repeated measures.
Of the 115 women enrolled in the substudy, the primary outcome occurred in 52% of children in the prenatal surgery group and 66% in the postnatal surgery group (relative risk RR: 0.78; 95% confidence interval CI: 0.57-1.07). Actual rates of CIC use were 38% and 51% in the prenatal and postnatal surgery groups, respectively (RR: 0.74; 95% CI: 0.48-1.12). Prenatal surgery resulted in less trabeculation (RR: 0.39; 95% CI: 0.19-0.79) and fewer cases of open bladder neck on urodynamics (RR: 0.61; 95% CI: 0.40-0.92) after adjustment by child's gender and lesion level. The difference in trabeculation was confirmed by ultrasound.
Prenatal surgery did not significantly reduce the need for CIC by 30 months of age but was associated with less bladder trabeculation and open bladder neck. The implications of these findings are unclear now, but support the need for long-term urologic follow-up of patients with myelomeningocele regardless of type of surgical repair.
Purpose Failed pyeloplasty represents a management dilemma, with treatment options including balloon dilation, endopyelotomy and reoperative pyeloplasty. We review our experience with robot-assisted ...laparoscopic reoperative repair of recurrent/persistent ureteropelvic junction obstruction in children and compare this method to other approaches. Materials and Methods We reviewed in detail all cases of failed prior ureteropelvic junction procedures, either open or laparoscopic, managed by robot-assisted laparoscopic reoperative repair between 2006 and July 2011. Results Robot-assisted laparoscopic repair was performed in 16 cases for persistent or recurrent ureteropelvic junction obstruction following a prior procedure involving the ureteropelvic junction (12 open pyeloplasties, 4 robot-assisted laparoscopic repairs). Additional interventions had been performed in 12 patients. Reoperative robot-assisted laparoscopic pyeloplasty was performed in 13 patients and reoperative robot-assisted laparoscopic ureterocalycostomy in 3. Patient age ranged from 12 months to 15.3 years (mean 6.1 years). Mean operative time and length of stay were 303 minutes and 1.6 days, respectively. Mean followup was 14.9 months. All symptomatic patients had resolution of symptoms postoperatively. A total of 14 patients (88%) had improved radiological findings. One patient underwent transfusion and conversion to an open procedure due to bleeding. Conclusions Robot-assisted laparoscopic reoperative repair of persistent/recurrent ureteropelvic junction obstruction is a safe, highly effective procedure even in the setting of multiple prior procedures. In our series all patients improved symptomatically, 88% improved radiographically and none have required further surgical intervention. Success is greater than with endopyelotomy and comparable to open reoperative repair for this challenging condition during short-term and intermediate followup.
Abstract Bladder regeneration studies have yielded inconclusive results possibly due to the use of unfavorable cells and primitive scaffold design. We hypothesized that human mesenchymal stem cells ...seeded onto poly(1,8-octanediol-co-citrate) elastomeric thin films would provide a suitable milieu for partial bladder regeneration. POCfs were created by reacting citric acid with 1,8-octanediol and seeded on opposing faces with human MSCs and urothelial cells; normal bladder smooth muscle cells and UCs, or unseeded POCfs. Partial cystectomized nude rats were augmented with the aforementioned POCfs, enveloped with omentum and sacrificed at 4 and 10 weeks. Isolated bladders were subjected to Trichrome and anti-human γ-tubulin, calponin, caldesmon, smooth muscle γ-actin, and elastin stainings. Mechanical testing of POCfs revealed a Young’s modulus of 138 kPa with elongation 137% its initial length without permanent deformation demonstrating its high uniaxial elastic potential. Trichrome and immunofluorescent staining of MSC/UC POCf augmented bladders exhibited typical bladder architecture with muscle bundle formation and the expression and retention of bladder smooth muscle contractile proteins of human derivation. Quantitative morphometry of MSC/UC samples revealed muscle/collagen ratios approximately 1.75× greater than SMC/UC controls at 10 weeks. Data demonstrate MSC seeded POCfs support partial regeneration of bladder tissue in vivo.
We evaluate the applicability of the International Index of Erectile Function in young men with spina bifida and identify spina bifida-specific sexual experiences not captured by this measure.
...Semistructured interviews were conducted between February 2021 and May 2021 with men ≥18 years of age with spina bifida. The International Index of Erectile Function was completed by participants, and perspectives on its applicability were discussed. Participant experiences and perspectives around sexual health were discussed to identify aspects of the sexual experience not well captured by the International Index of Erectile Function. Demographic and clinical characteristics were obtained from a patient survey and chart review. Conventional content analysis framework was used for transcript coding.
Of 30 eligible patients approached, 20 participated. Median age was 22.5 years (range 18-29), and 80% had myelomeningocele. Most identified as heterosexual (17/20, 85%), were not in a relationship (14/20, 70%), and were not currently sexually active (13/20, 65%). Some perceived the International Index of Erectile Function as applicable, while others reported it was not, as they do not define themselves as sexually active. Aspects of the sexual experience not captured by the International Index of Erectile Function included (1) lack of control over sexual function, (2) poor lower body sensation, (3) urinary incontinence, (4) spina bifida-specific physical limitations, and (5) psychosocial barriers. Participant suggestions for improving the International Index of Erectile Function to increase its applicability were identified.
While many perceived the International Index of Erectile Function as applicable, the measure inadequately captures the diverse sexual experiences of young men with spina bifida. Disease-specific instruments to evaluate sexual health are needed in this population.
Summary While major advances have been made in hypospadias surgery, most of these have come in the management of distal hypospadias. There is significant variance in approaches to proximal ...hypospadias repair. This is to some degree because many of us have realized that the ideal procedure to repair proximal hypospadias has not yet been determined. This article reviews the history of proximal hypospadias surgery, comparing single-stage and two-stage surgical approaches, and the authors’ current technique for proximal hypospadias repair is presented. Furthermore, available tools for hypospadias research are evaluated and the areas identified that require research efforts to improve outcomes.
Various techniques for neovaginal construction have been employed in the pediatric and adult populations, including the use of intestinal segments, buccal mucosal grafts, and skin grafts. Small ...intestinal submucosa (SIS) extracellular matrix grafts have been described as a viable alternative, though prior experience is limited. Our purpose was to assess operative characteristics and patient outcomes with neovaginal construction using SIS grafts.
Thirteen patients underwent vaginoplasty with acellular porcine SIS grafts at our institution between 2018 and 2022. Operative and clinical data, postoperative mold management, vaginal dilating length, and complications were reviewed.
Age at time of repair ranged from 13 to 30 years (median 19 years). Patient diagnosis included cloacal anomalies (n = 4), Mayer-Rokitansky-Küster-Hauser syndrome (n = 4), isolated vaginal atresia with or without a transverse vaginal septum (n = 4), and vaginal rhabdomyosarcoma requiring partial vaginectomy (n = 1). Following dissection of the neovaginal space, a silicon mold wrapped with SIS graft was placed with retention sutures and removed on postoperative day 7. Median (IQR) operative time was 171 (118-192) minutes, estimated blood loss was 10 (5-20) mL, and length of stay was 2 (1-3) days. The follow-up period ranged from 3 to 47 months (median 9 months). Two patients developed postoperative vaginal stenosis that resolved with dilation under anesthesia. Mean vaginal length on latest follow-up was 8.97 cm. All thirteen patients had successful engraftment and progressed to performing self-dilations or initiating intercourse to maintain patency. There were no cases of graft reaction or graft extrusion.
We conclude that acellular small intestinal submucosa grafts are effective and safe alternatives for mold coverage in neovaginal construction. Our experience demonstrates minimal perioperative morbidity, early mold removal, and progression to successful dilation with maintenance of a functional vaginal length. Future study on sexual outcomes, patient satisfaction, and comparison against alternative techniques has been initiated.
IV.
Retrospective Study.
Regenerative medicine strategies combine various attributes from multiple disciplines including stem cell biology, chemistry, materials science and medicine. The junction at which these disciplines ...intersect provides a means to address unmet medical needs in an assortment of pathologies with the goal of creating sustainable, functional replacement tissues. Tissue damage caused by trauma for example, requires rapid responses in order to mitigate further tissue deterioration. Cell/scaffold composites have been utilized to initiate and stabilize regenerative responses in vivo with the hope that functional tissue can be attained. Along with the gross reconfiguration of regenerating tissues, small molecules and growth factors also play a pivotal role in tissue regeneration. Several regenerative studies targeting a variety of urological tissues demonstrate the utility of these small molecules or growth factors in an in vivo setting.
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