Primary non-refluxing megaureter: Natural history, follow-up and treatment.

Primary non-refluxing megaureter (PMU) is a congenital dilation of the ureter which is not related to vesicoureteral reflux, duplicated collecting systems, ureterocele, ectopic ureter, or posterior urethral valves and accounts for 5 to 10% of all prenatal hydronephrosis (HN) cases. The etiology is a dysfunction or stenosis of the distal ureter. Most often PMU remains asymptomatic with spontaneous resolution allowing for non-operative management. Nevertheless, in selective cases such as the development of febrile urinary tract infections, worsening of the ureteral dilatation, or reduction in relative renal function, surgery should be considered. CONCLUSION: Ureteral replantation with excision of the dysfunctional ureteral segment and often ureteral tapering is the gold-standard procedure for PMU, although endoscopic treatment has been shown to have a fair success rate in many studies. In this review, we discuss the natural history, follow-up, and treatment of PMU. WHAT IS KNOWN: • PMU is the result of an atonic or stenotic segment of the distal ureter, resulting in congenital dilation of the ureter, and is frequently diagnosed on routine antenatal ultrasound. WHAT IS NEW: • Most often, PMU remains asymptomatic and clinically stable, allowing for non-operative management. • Nevertheless, since symptoms can appear even after years of observation, long-term ultrasound follow-up is recommended, even up to young adulthood, if hydroureteronephrosis persists. • Ureteral replantation is the gold standard in case surgery is needed. In selected cases, however, HPBD could be a reasonable alternative.

Recent trends in the surgical management of primary vesicoureteral reflux in the era of dextranomer/hyaluronic acid.

PURPOSE: Since its inception as a technology in the United States, endoscopic correction of vesicoureteral reflux has become a popular treatment option in children with vesicoureteral reflux with reported wide use. We determined whether the increasing trend in use in the United States after the introduction of dextranomer/hyaluronic acid has been sustained. MATERIALS AND METHODS: We abstracted data on pediatric patients treated with ureteral reimplantation or dextranomer/hyaluronic acid intervention for vesicoureteral reflux from 2004 to 2011 from the PHIS (Pediatric Health Information System) database. Patients with coding data indicating diagnoses other than primary vesicoureteral reflux and hospitals reporting less than 80% of ambulatory surgery cases by CPT code were excluded from study. RESULTS: We identified 14,430 patients (17,826 procedures), of whom 49% underwent reimplantation and 51% underwent dextranomer/hyaluronic acid injection. Of the patients 83% were female with a median age at surgery of 4.7 years (IQR 2.5-7.2). Linear regression showed a significant downward trend in the average total number of antireflux operations per institution during the study period. This was attributable to a decrease in the average rate of dextranomer/hyaluronic acid interventions because the average reimplantation rate remained stable during this time. CONCLUSIONS: At freestanding pediatric hospitals enrolled in the PHIS database there is a trend toward decreasing intervention for primary vesicoureteral reflux, which appears to be due to decreased use of injection therapy. This may reflect a philosophical change in reflux management by injection therapy.

A case report and review of transitional cell carcinoma in children.

Transitional cell carcinoma (TCC) is a rare cause of hematuria in children. This type of urothelial bladder tumor is typically low grade and carries a good prognosis. In this article, a case report is presented along with a review of the literature on TCC in children.

The birth of pediatric urology in the United States: From pioneers to subcertification.

INTRODUCTION: Pediatric urology is a subspecialty of urology that emerged from a culture in which children with urological disorders were cared for by general urologists and general pediatric surgeons. The development of pediatric urology as a subspecialty was years in the making, highlighted by individuals who recognized that children with urological conditions were not just "small adults," but required special experience and training. Subspecialization was initiated by persistent visionaries who recognized the need for a trained cadre of experts to provide better care for children. It took the coordinated effort of all subgroups and leaderships in pediatric urology to push these efforts over the goal line. The focus of this review is to highlight certain individuals who played major roles in this vision and to document the efforts of many to coordinate the pathways to sub-specialization. METHODS: The careers of Meredith F. Campbell and Frank B. Bicknell were researched to identify their rationale and roles in developing pediatric urology as a distinct medical specialty in the United States. In addition, the minutes of the meetings of the Pediatric Urology Advisory Council (PUAC) with the American Board of Urology (ABU) were reviewed. The origins of the Society for Pediatric Urology (SPU) and the American Academy of Pediatrics Section of Urology (AAP-SOU) were researched. The contributions of each to the certification of pediatric urology as a distinct subspecialty was delineated. RESULTS: Campbell was Chair of Urology at the New York University (NYU) School of Medicine and wrote prolifically about pediatric urology. He published one of the first practical textbooks in pediatric urology, almost completely self-written, in 1937. Bicknell, a general urologist in Michigan on the faculty at Wayne State University School of Medicine, led the initiative to create the Society for Pediatric Urology (SPU) that first met at the 1951 annual American Urological Association (AUA) meeting in Chicago and included nine attendees. Subsequently, John Lattimer (College of Physicians and Surgeons at Columbia University) organized a well-attended meeting of urologists interested in pediatrics at the 1964 annual AAP meeting in New York City. This led to the formation of the AAP Section on Urology. Integral to the justification for the development of a subspecialty was evidence of a published corpus of content. In addition to published textbooks devoted exclusively to pediatric urology, this was further fulfilled by the AAP Section on Urology Pediatric Supplement to the Journal of Urology, first published in 1986, and later with the Journal of Pediatric Urology in 2005. The SPU and the AAP Section on Urology came together to form the Pediatric Urology Advisory Council (PUAC) in 2000, which worked with the ABU to create subspecialty certification in pediatric urology with an independent exam, first administered in 2008 to 176 applicants. CONCLUSION: The metaphor "We have stood on the shoulders of giants" is apt for pediatric urology: Meredith Campbell, Frank Bicknell, David Innes Williams (Hospital for Sick Children, London), and J. Herbert Johnston (Alder Hay Hospital, Liverpool) come to mind among the first generation of pediatric urology pioneers, and others among their colleagues also had significant impact. Clearly this is a story of persistence and attention to detail on the part of those giants and those who followed. Pediatric urology became a distinct discipline after the SPU and AAP-SOU came together to create a robust cohort of pediatric urologists who through education and negotiation were able to help the ABU and the American Board of Medical Specialties (ABMS) recognize that subspecialization would lead to better care for children with urologic disorders. This benchmark set a high bar for future subspecialization in urology and other fields.

Long-term followup of primary nonrefluxing megaureter.

PURPOSE: We evaluated outcomes of nonoperative management of primary nonrefluxing megaureter at long-term followup to identify clinical predictors of spontaneous resolution. MATERIALS AND METHODS: A total of 75 patients (88 primary megaureters) were diagnosed between 1990 and 2005 and followed for more than 6 months. Of the patients 63 (74 primary megaureters) were included in the main study population. Indications for surgery were obstructive hydroureteronephrosis, functional impairment and persistent symptoms. RESULTS: Of the 74 primary megaureters 20 (27%) required surgery up to 7 years after diagnosis. Surgery was not indicated in 82% of primary megaureters with grade I or II hydronephrosis vs 62.9% of those with grade III or higher hydronephrosis (difference not significant), nor in 76.5% of types I and II primary megaureters vs 33.3% of type III primary megaureters (p = 0.040), 78.7% of renal units with differential function 40% or greater vs 0% with differential function less than 40% (p = 0.027), 80% of primary megaureters with a nonobstructive washout pattern vs 44.4% with an intermediate/obstructive pattern (p = 0.032), 67.9% of patients with perinatal presentation vs 25% with postneonatal presentation (p = 0.008) or 63.2% of patients presenting with symptoms vs 76.4% of those who were asymptomatic (difference not significant). On multivariate analysis age at presentation and washout pattern were significant predictors of spontaneous resolution. CONCLUSIONS: Most cases of primary megaureter resolve spontaneously or improve without loss of function or development of symptoms. Careful observation allows surgery to be delayed beyond the neonatal period in most patients. Long-term followup is recommended because symptoms can develop years later. Washout pattern and age at presentation are statistically significant predictors of spontaneous resolution.

Frank B. Bicknell: Pioneer in Pediatric Urology and Founder of the American Urological Association History Forum.

OBJECTIVE: To explore and document the life and urologic contributions of Dr. Frank B. Bicknell. METHODS: We researched the life of Dr. Bicknell via his publications, archived documents from the Didusch Museum and through the description of his life and accomplishments by his colleagues including John K. Lattimer and Frank Hinman Jr. RESULTS: Frank B. Bicknell (1907-1999) attended the University of Michigan (1925-1928) and Universityof Michigan Medical School (1928-1932) prior to his internship and urology residency at the Receiving Hospital, Detroit, Michigan (1932-1936). He served in the Merchant Marine in the 1930s, sailing around the world. He was a major in World War II and served as Professor of Urology at Wayne State University. In 1951 Dr. Bicknell got together a small group of eight urologists interested in pediatric urology during the AUA Annual Meeting. Drs. Campbell, Barber, Johnson, Mertz, Hinman Jr., Spence and Lattimer all met in Dr. Bicknell's Chicago hotel room and would form The Society for Pediatric Urology. At the time, Dr. Bicknell's brother-in-law had just become president of the American Academy of Pediatrics (AAP). Dr. John Lattimer with the help of Dr. Bicknell's brother-in-law was able to get a room at the AAP meeting which he filled with 2500 people, thought to be the largest collection of urologists in one room at the time. The success of the session led the AAP to develop a Section of Urology and impressed upon the AUA the magnitude of interest in pediatric urology. This allowed pediatric urologists to secure an exclusive session on the day before the main AUA meeting which has persisted since that time.Dr. Bicknell founded the History Forum in 1966 and chaired this very popular event during its first decade. The forum now occupies an entire afternoon during the AUA annual meeting, with papers presented on historic urologic topics. The highlight of this assembly is the annual lecture on the history of medicine. In 2000, this oration was renamed the Frank Bicknell History of Urology Oration to honor the founder of the History Forum. CONCLUSION: Dr. Frank Bicknell was an early leader in pediatric urology and urologic history who helped found The Society of Pediatric Urology and the AUA History Forum.

Neo-phalloplasty in children and adolescents for acquired penile loss.

INTRODUCTION: From 2000 to 2019, the De Castro's neo-phalloplasty was used in 47 patients with congenital and acquired penile loss. PURPOSE: Herein, the technical aspects of penile reconstruction and the outcomes in 17 children and adolescents treated for total or sub-total acquired penile loss are reported. MATERIAL AND METHODS: The median age at the time of injury was 3 months (range: 2 days-15 years). Twelve patients were born with normal penis but suffered injuries (11) or underwent surgical resection of the penis (1). The remaining 5, affected by bladder exstrophy (2) or cloacal exstrophy (3), had penile loss due to surgical complications. RESULTS: The median age at the time of surgery was 5 years (range: 2-20 years). The median length of the procedure was 5 h (range 4-8 h). Skin expander were inserted in preparation of phalloplasty in 9 patients. Corpora-cavernosa remnants were found and incorporated in the neophallus in 12 patients. In 7 patients, urethral remnants were also incorporated into the repair, placing the meatus at the tip of the neophallus. In the remaining patients the urethra was left in the acquired position after penile loss creating a perineal (2), scrotal (3), peno-scrotal (3), or posterior penile (1) urethrostomy. The first patient of this series was the only patient to receive simultaneously phalloplasty and total urethroplasty, with failure of urethral reconstruction. The median follow-up was 2 years (range 1-11 years). The overall complication rate was about 47%. All of the complications were late and required surgical revision. The results were assessed regarding overall functionality (voiding, erection/erogenous areas, masturbation/sexual intercourse) and aesthetic outcome using subjective and objective parameters. Psychological evaluation for both patient and parents was performed in 4 cases. DISCUSSION: There are no specific guidelines for treating significant penile injuries in the pediatric population. We do not think secondary severe penile impairments should wait until adult age for repair. In this specific subgroup of patients technical aspects differ from phalloplasty done for congenital aphallia, due to the possible presence of corpora-cavernosa/urethral remnants and the feasibility to perform a "functional phalloplasty" with very encouraging results. CONCLUSION: The De Castro's neo-phalloplasty remains one of the few techniques available for total penile reconstruction in pediatric age group. Data demonstrate that this technique for acquired aphallia is feasible and reproducible, however, it is a challenging procedure with a high complication rate and likelihood of revisions.

The history of the pediatric inguinal hernia repair.

The history of inguinal hernia repair is a rich one. For centuries, hernia healers, doctors, anatomists, surgeons and quacks have been devoted to this pathology that has afflicted mankind throughout its evolution. The development of surgical correction mainly focused on adult pathology, with treatments that often involved the loss of the testis. Hernia management in children, however, also dates from antiquity. Described as a swelling on the surface of the belly in ancient papyri, it was treated with tight bandages by the early physicians of Alexandria. For centuries, conservative treatment had been used for the child using primordial trussess, many prayers, and often pagan rituals as the arboreal passage of children described by Marcello of Bordeaux, doctor of the Emperor Theodosius I (347-395 AD), reserving medical intervention only for cases of strangulation in which only reduction was attempted. The middle ages were characterized by an increase in cultural and scientific exchange, during which the first comprehensive surgical textbooks and atlases were written. Different approaches to the inguinal hernia were not taught and passed down through generations of surgeons. The modern era brought a better understanding of the inguinal anatomy, which led to surgical techniques associated with less post-operative complications. Today, the pediatric inguinal hernia repair is one of the most common pediatric operations performed. It is considered a safe procedure with very low complication rate.

Transurethral puncture for ureterocele-which factors dictate outcomes?

PURPOSE: We evaluated which clinical factors influence the outcome of primary transurethral puncture for ureterocele. MATERIALS AND METHODS: A total of 45 patients (47 ureteroceles) underwent primary transurethral incision between 1994 and 2008 at 2 institutions. Age at and mode of presentation, upper tract status, ureterocele site, preoperative vesicoureteral reflux and the corresponding upper pole or kidney function were analyzed to identify which factors influenced the need for secondary surgery. RESULTS: Transurethral puncture was the only treatment in 24 of 45 patients (53%) while 21 (47%) required further surgery. After transurethral puncture secondary surgery was required in 56% of patients who presented prenatally vs 27% of those who presented postnatally (p = 0.165), in 18% with a single system vs 58% with a duplex system (p = 0.036), in 30% with intravesical vs 63% with ectopic ureterocele (p = 0.039) and in 61% vs 37% with ureterocele units with vs without preoperative vesicoureteral reflux (p = 0.148). Fisher's 2-tailed exact test revealed an inconsistent distribution of negative prognostic factors, including duplex systems, ectopic ureterocele and vesicoureteral reflux at presentation, in prenatally vs postnatally and in asymptomatically vs symptomatically presenting subgroups. CONCLUSIONS: Upper tract status and ureterocele site influence the outcome of primary transurethral puncture as a definitive procedure. After puncture secondary surgery is least likely in patients with a single system and intravesical ureterocele.

An unusual clinical presentation of rhabdomyomatous Wilms' tumor.

Wilms' tumor (WT) is the most common primary malignant renal tumor in children and usually presents as an abdominal mass. Rhabdomyomatous nephroblastoma is a rare histologic variant of WT. Herein we report an unusual case of WT with rhabdomyomatous differentiation presenting with hematuria and the passage of tissue via the urethra in a 2-year-old male.

Brown Urology: A Historical Perspective.

The first Urology Residency Program in the United States was founded at the Johns Hopkins Hospital in the wake of the first structured surgery residency as established by Dr. William Halsted in the early 20th Century.1 Dr. Hugh Hampton Young was selected to lead the Genitourinary Division and the foundation for the first urology residency training program was established.2 The Brown University Medical School, initially opened in 1811, effectively closed circa 1827, and re-established in the 1970s, has a long tradition in training surgeons.3,4 The Rhode Island Hospital Urology Residency Training Program was organized in the early 1950s and will be explored in this article. Brown University affiliated with the residency program in the mid-1980s to establish the first and only academic urology residency program in Rhode Island. Today, this program provides state-of-the-art urologic care for thousands of patients in the state.

Management of boys with nonpalpable undescended testis.

Cryptorchidism is one of the most common genitourinary disorders in young boys. Although the management of boys with palpable testis is standardized, there are no formal guidelines for the management of boys with nonpalpable testis. In this Review we look at the current trends in the diagnosis and treatment of this disorder, as well as the indications for therapy and surgical procedures. On the basis of current evidence, we find that there is no optimum orchidopexy technique for the treatment of intra-abdominal testis, although it is preferable to adopt techniques that preserve the spermatic vessels. We also briefly examine the follow-up of patients with this disorder and its common complications. As yet, there are no data that assess the potential of laparoscopic orchidopexy being a risk factor for impaired fertility later in life.

Surgery of Anomalies of Gonadal and Genital Development in the "Post-Truth Era".

This article aims to examine the current issues of debate concerning the management of atypical gonadal and genital development (AGD). Understanding this complex subject begins with defining the distinct AGD conditions, the aims and nature of surgical treatments, and the perceptions of affected individuals and their families. The evolving societal and medical contexts in this field are confronting facts and opinions, leading to a significant change in attitudes and approaches.