Complications in adulthood for patients with paediatric genitourinary reconstruction.

BACKGROUND: Caring for adults with prior paediatric genitourinary reconstruction remains a challenge for adult providers. Reconstructions typically have occurred decades before; surgical records are not always available and patients and families may be unable to convey procedures performed. Spina bifida (SB) patients are vulnerable to cognitive decline which may compound these challenges. Changes in patient body habitus and loss of function may contribute to problems with previous reconstructions. METHODS: This is a non-systematic review of the literature and represents expert opinion where data are non-existent. This review focuses on the evaluation and management of complications arising from genitourinary reconstruction in congenital neurogenic bladder patients. RESULTS: Common complications experienced by congenital neurogenic bladder patients include recurrent urinary tract infection, incontinence of catheterizable channel and urinary reservoir as well as malignancy as this population ages. Preservation of renal function and prevention of urinary tract infection while optimizing continence are essential guiding principles in the care of these patients. Many of the recommendations, however, are gleaned from available data in the adult spinal cord patient (a more commonly studied population) or the paediatric urologic literature due to limited studies in adult management of such patients. CONCLUSION: Close follow-up and vigilance is warranted to monitor for infectious, mechanical and malignant complications while optimizing preservation of the upper urinary tracts and patient quality of life.

Pelvic Organ Prolapse and Pregnancy in the Female Bladder Exstrophy Patient.

PURPOSE OF REVIEW: Classic bladder exstrophy (BE) remains one of the most demanding reconstructive challenges encountered in urology. In female BE patients, the long-term sequela of both primary and revision genitoplasty, as well as intrinsic pelvic floor deficits, predispose adult women to significant issues with sexual function, pelvic organ prolapse (POP), and complexities with reproductive health. RECENT FINDINGS: Contemporary data suggest 30-50% of women with BE develop prolapse at a mean age of 16 years. Most women will require revision genitoplasty for successful sexual function, although in some series over 40% report dyspareunia. Current management for pregnancy includes elective cesarean section with involvement of high-risk obstetrics and urologic surgery. This review encapsulates contemporary concepts of etiology, prevalence, and management of POP and pregnancy in the adult female BE patient.

The Recommendations of the 2015 American Urological Association Working Group on Genitourinary Congenitalism.

OBJECTIVE: To develop consensus recommendations for index congenital urological cases seen in adulthood and to generate discussion among providers who treat these patients across the lifespan. This manuscript reviews the proceedings and recommendations of the 2015 American Urological Association Working Group on Urological Congenitalism. METHODS: Index cases were selected to highlight controversies in the management of different congenital patients in adulthood. Cases were presented and participants were randomized to groups to avoid clustering of individuals of similar background and experience. Expert panelists (representing backgrounds in pediatric and adult care of congenital patients) also discussed the cases. After that, a facilitated discussion was held with the entire group in an attempt to develop consensus recommendations for the questions raised in each case. RESULTS: Recommendations were generated related to management of pregnancy in young woman with bladder exstrophy and one with reflux nephropathy, as well as a young man with spina bifida with chronic kidney disease seeking undiversion. CONCLUSION: Given the lack of long-term data for patients with congenital genitourinary diseases, management of complex urological disease in these patients can be difficult. Consensus discussion with urological providers across the spectrum of the life course of these patients may help provide clinical guidance.

[MRI of the fetal abdomen]. / MRT des fetalen Abdomens.

Magnetic resonance imaging (MRI) is an important diagnostic component for central nervous system and thoracic diseases during fetal development. Although ultrasound remains the method of choice for observing the fetus during pregnancy, fetal MRI is being increasingly used as an additional technique for the accurate diagnosis of abdominal diseases. Recent publications confirm the value of MRI in the diagnosis of fetal gastrointestinal tract and urogenital system diseases. The following report provides an overview of MRI-examination techniques for the most frequent diseases of the abdomen.

[Extracardiac manifestations of undifferentiated connective tissue dysplasia]. / Vnekardial'nye proiavleniia nedifferentsirovannoi displazii soedinitel'noi tkani.

Undifferentiated connective tissue dysplasia (UDCTD) is a common type of congenital mesenchymal defect. Among the visceral manifestations of UDCTD, cardiac anomalies have been studied in detail while the nature, extent, and clinical significance of changes in other viscera are much less known. The review summaries data on currently accepted extracardiac manifestations of UDCTD, such as venous insufficiency, tracheobronchial dyskinesia, nephroptosis, genital prolapse, gastrointestinal disorders, hemostatic pathology.

Anomalías congénitas de la uretra: diagnóstico radiológico / Congenital anomalies of the uretra: radiological diagnosis

Este artículo revisa una serie de anomalías congénitas frecuentes e infrecuentes que afectan a la uretra, e ilustra la utilidad de la urografía, cistografía miccional y ecografía en el diagnóstico y manejo de estas alteraciones. Las anomalías de la uretra incluyen válvulas de uretra posterior, duplicación uretral, divertículos uretrales, megalouretra y fístula uretral. Se incluye, así mismo, la malformación de la cloaca y el seno urogenital. Las bases embriológicas y patológicas de las diferentes patologías se discuten en cada apartado (AU)

H-type anorectal malformations: incidence and clinical characteristics.

To establish the incidence of H-type anorectal malformations (ARM), the hospital records of 629 patients with ARM were studied. Patients who had a congenital rectourogenital connection and an external anal opening in a normal or ectopic position were considered to have an H-type malformation. Twenty (3.2%; 14 females, 6 males) of the 629 patients studied had an H-type ARM. Major associated anomalies were found in 60% of these patients. Thirteen patients (12 females, 1 male) had a normally placed anus; the H fistula was low rectovestibular in 10, rectovaginal in 2, and rectourethral in 1. Seven patients had an ectopic anal opening. Three males had a rectourethral H fistula; in two of these there was a double fistula. Two males had a rectovesical H fistula. Of the two females with ectopic anal openings, one had a high rectovaginal H fistula and the other had a low fistula. In only one case was the diagnosis of H fistula made immediately after birth. Three patients died of severe cardiac abnormalities during the neonatal period or early infancy. Primary perineal repair or a limited posterior surgical anorectoplasty (PSARP) was used in 15 cases; formal PSARP was used for the other two. Four patients had between one and 4 recurrences; all but one of these fistulas were repaired subsequently. Long term (median follow-up period, 12 years; range, 4 to 38 years), 12 patients had good bowel function and no faecal soiling; four of these had constipation that was manageable with laxatives. Two patients (aged 10 and 12 years) had daily soiling. Two others were too young to evaluate, and one (with severe mental retardation) has a permanent colostomy.

Laparoscopy in pediatric urology: challenge and opportunity.

The development of operative laparoscopic techniques in Pediatric Urology has been generally cautious. It is premature to attempt an assessment of the ultimate impact of laparoscopy in pediatric urology, yet several areas of application have significant potential. Diagnostic laparoscopy for cryptorchidism has evolved into operative laparoscopy for intra-abdominal testes, and a significant number of cases have been performed successfully, with the possibility of improved results over open techniques. Laparoscopic nephrectomy and nephroureterectomy have been spring-boards for complex reconstructive renal surgery, although it is clear that new tissue joining technologies are needed. Complex reconstructive surgery is just beginning, yet may have a role in practice. These developments and the current status of laparoscopy in pediatric urology will be reviewed.

Laparoscopy in pediatric urology: too much of a good thing?

Laparoscopy is still an evolving technique; its rightful place in pediatric urology has yet to be defined. Although both the first reported use of diagnostic laparoscopy in pediatric surgery and its initial use in pediatric urology were published in 1973, the field lay dormant until recently. Once operative laparoscopy was initiated with the report of laparoscopic cholecystectomy in 1987, a torrent of articles followed; many operative procedures in adult urology and subsequently in pediatric urology appeared. This article presents the advantages and the disadvantages of several of the more important and common possible indications for laparoscopy in pediatric urology.