Seminars in Pediatric Surgery 24 (2015) 73–78

Contents lists available at ScienceDirect

Seminars in Pediatric Surgery journal homepage: www.elsevier.com/locate/sempedsurg

Transitional care in pediatric urology Sarah M. Lambert, MDa,b,n a b

Department of Urology, Columbia University, New York, New York Division of Pediatric Urology, New York Presbyterian Hospital, Morgan Stanley Children’s Hospital, New York, New York

a r t i c l e in fo

Keywords: Neurogenic bladder Transition of care Pediatric genitourinary tumors Posterior urethral valves Bladder exstrophy Long-term outcomes

a b s t r a c t The transition from childhood to adolescence and into adulthood occurs as a natural component of human development. As children progress through school and gain independence, health care practitioners must facilitate a parallel transition from pediatric to adult providers. Modern medicine has succeeded in extending the life expectancy for many children with complex conditions, and adult providers are participating in their medical care through adulthood. Transitioning pediatric urology care to adult urology care is unique to every individual and his or her underlying condition, while the transition process is universal. The objectives of all pediatric urologists include preservation of the kidneys and lower urinary tracts, safe urine storage, safe urine drainage, urinary continence, fertility, sexual function, and genital cosmesis. For some children, these objectives can be attained during childhood, while other children require lifelong maintenance and management. Children with posterior urethral valves, exstrophy–epispadias complex, cloaca, vesicoureteral reflux, neurogenic bladder, disorders of sex development, cancer, hypospadias, nephrolithiasis, undescended testes, varicoceles, ureteropelvic junction obstruction, solitary kidney, and upper tract anomalies all require long-term evaluation and management. The obstacles of altering a patient and caregiver paradigm, locating adult urologists with special expertise, coordinating care with other adult specialties such as nephrology, and navigating the adult health care environment can impede the transition process. & 2015 Published by Elsevier Inc.

The transition from childhood to adolescence and into adulthood occurs as a natural component of human development. As children progress through school and gain independence, health care practitioners must facilitate a parallel transition from pediatric to adult providers. According to the American Academy of Pediatrics, “optimal health care is achieved when each person, at every age, receives medically and developmentally appropriate care.”1 Multiple organizations and agencies are currently focused on improving health care transition, including the American Academy of Pediatrics, American Academy of Family Physicians, and American College of Physicians. The U.S. Department of Health and Human Service’s Maternal and Child Health Bureau also promotes comprehensive services for the transition of care period.2 Transition of care is essential but not without difficulties, limitations, and frustrations. The key components of transition include the patient, his or her caregivers, the pediatric providers, the adult providers, adult and pediatric support staff, adult and n Correspondence address: Division of Pediatric Urology CHN-11, New York Presbyterian Hospital, Morgan Stanley Children’s Hospital, 3959 Broadway, New York, NY 10032. E-mail address: [email protected]

http://dx.doi.org/10.1053/j.sempedsurg.2015.01.004 1055-8586/& 2015 Published by Elsevier Inc.

pediatric hospitals, insurance companies, and the health care system. The objective of this article is to highlight specific pediatric urologic conditions that require long-term care and transition. Transition of care is not unique to urology, but it is common across all pediatric disciplines. While diseases such as diabetes and asthma are familiar to adult providers, congenital anomalies and pediatric disease historically associated with decreased life expectancy have no parallel in adult medicine. Modern medicine has succeeded in extending the life expectancy for many of these children with complex conditions, and adult providers become responsible for those who survive childhood. Cardiologists and pulmonologists are among leaders in training providers in transitional medicine that encompasses pediatric and adult care through specialized fellowship programs. Pediatric urology encompasses many genital and urinary conditions. These conditions typically present prenatally or during childhood, but some conditions emerge in adolescence as well. These conditions range from mild to life-threatening. The objectives of all pediatric urologists include preservation of the kidneys and upper tracts, safe urine storage, safe urine drainage, urinary continence, fertility, sexual function, and genital cosmesis. For some children these objectives can be attained during childhood while other children require lifelong maintenance and

74

S.M. Lambert / Seminars in Pediatric Surgery 24 (2015) 73–78

management. While all children deserve an organized transition of care, it is the latter group of children in which direct and seamless transition of care is mandatory from a urologic perspective. These children often require subspecialty care from other disciplines as well, such as orthopedics, general surgery, neurosurgery, endocrinology, nephrology, oncology, cardiology, and gynecology. Adolescents with complex urologic disease often also utilize non-physician provider services, such as catheterization nurses, stoma and ostomy nurses, social workers, psychologists, and child life specialists. The transition into late adolescence and adulthood also translates to insurance status changes and changes to caregiver relationships. These changes can impact confidentiality, informed consent, patient responsibility, transportation, and the physician–patient relationship. For pediatric urologists, the concept of transitioning long-term patients with complex conditions can be difficult and uncomfortable. Clinicians, patients, and caregivers can all experience hesitation, ambivalence, and fear. Adolescents with chronic bladder conditions develop confidence and trust with their pediatric care provider and may feel ill prepared to transfer to an adult provider. Conversely, these adolescents also reported that issues surrounding relationships, sexuality, and fertility were rarely discussed in the pediatric office.3 Adult providers may also be faced with management challenges when accepting care of these patients. Young adult patients with congenital urologic disease are complex, with multiple urologic and medical issues not easily addressed. This situation can be overwhelming and lead to frustration on the part of both patient and health care provider. However, successful management of this transition is critical, as failure to sustain long-term follow-up of chronic urologic problems places these patients at high risk for adverse health outcomes.4 Young adults lost to surveillance can present with devastating conditions, such as renal failure or complications from urinary incontinence including decubitus ulcers and sacral osteomyelitis.5 Development of a standardized transition-of-care program and collaboration with adult providers is essential to safe and successful transition. In discussing transition of urologic care, Rink6 very simply and eloquently asked, “What problems will I be leaving?” Devising a process to support seamless management of these problems is the crux of the transition process. Luckily, children with hypospadias, undescended testes, and varicoceles often require only adult urology subspecialty care, which streamlines the transition process. Children with more complex disorders, such as posterior urethral valves, vesicoureteral reflux, hypospadias, nephrolithiasis, undescended testes, varicoceles, ureteropelvic junction obstruction, solitary kidney, and upper tract anomalies all require long-term evaluation and management. Such patients often require adult urology, nephrology and, occasionally, transplant surgery management. Finally, the most complex patients, such as children with cancer, disorders of sexual development, neurogenic bladder, cloaca, and exstrophy–epispadias complex, often require multiple subspecialty care including general surgery, endocrinology, gynecology, neurology, neurosurgery, gastroenterology, oncology, nephrology, and urology. Transition-of-care plans for these children should include standardized goals, but they must be tailored to each child. An understanding of these congenital anomalies and the long-term issues associated with the disease processes, surgical interventions, and resultant complications is key to providing lifelong care. In the following sections, these conditions will be briefly reviewed and the long-term issues highlighted.

Neurologic conditions Neurologic conditions in children include myelomeningocele, tethered spinal cord, cerebral palsy, sacral agenesis, and other

spinal dysraphisms. Hinman’s syndrome, or non-neurogenic neurogenic bladder, can be included in this category as well. Spina bifida as a broad category is the most common permanent birth defect in the United States.7 These neurologic conditions can be associated with other comorbidities and syndromes or can be isolated. The management of neurogenic bladder has significantly improved since the initiation of clean intermittent catheterization and anticholinergic therapy. The treatment armamentarium also includes botulinum toxin, augmentation cystoplasty, catheterizable channel creation, antegrade continence enema creation, other urinary diversions, and vesicostomy. These children are unable to store urine safely or empty urine efficiently. Consequently, their care is lifelong, and requires monitoring as the clinical situation can deteriorate. Prior to clean intermittent catheterization, the life expectancy of children with myelomeningocele was severely limited. These children often died of urosepsis, stone disease, and renal failure. Currently, this population is one of the largest pediatric populations in need of complex transition of care. Children with neurogenic bladders are at risk for infection, upper tract damage, nephrolithiasis, urethral stricture, and bladder cancer. These children must be followed up for changes in bladder dynamics with serial urodynamic evaluation. Serial renal and bladder ultrasounds must be performed to assess upper tracts for dilation and nephrolithiasis. Urethral trauma and stricture need to be addressed and often require reconstructive surgical intervention. Incontinence of urine and stool can be a continued problem that persists into adulthood. Throughout adolescence, children with a history of myelomeningocele with worsening urodynamic parameters should be evaluated for cord tethering. Children with neurogenic bladder who undergo bladder augmentation or urinary diversion in childhood have additional longterm risks that are associated with intestinal or gastric reservoirs. Many children with bladder augmentation have catheterizable channels created for urinary drainage. These channels are at risk for stenosis and diverticulum. Bladder augmentation increases the risks for metabolic acidosis, bone mineral density abnormalities, vitamin B12 deficiency, augment perforation, and tumor formation within the augmentation. The re-augmentation risk at 10 years post initial augmentation has been reported as 3.7–5.9%.8 A large clinical series from Indiana University documented a 34% risk of further operative procedures after augmentation. Approximately 9% of children required a laparotomy for bowel obstruction. Additionally, they reported bladder perforation in 8.6% of children. Additionally, 15% of children with a mean follow-up of 13.3 years required treatment for bladder stones. The incidence of malignancy was 0.6% and all patients presented with metastases and died of disease.9 A review of adult dedicated spina bifida clinics at the Universities of Minnesota and Utah documented that 85% of young adults reported an active issue at the time of initial adult evaluation. Urinary incontinence, urinary traction infection, catheterization difficulties, and calculi were the most common. Of these young adults, 34% required a surgical intervention.10 These complications are long-term issues and therefore require lifelong surveillance and treatment throughout adulthood. In the adult population, fertility concerns become an issue. Male patients often require assistance with reproduction including electroejaculation.

Posterior urethral valves Boys with posterior urethral valves also require long-term follow-up to protect renal function and prevent further damage to the bladder and upper tracts. Although primary valve ablation or urinary tract diversion typically occurs in infancy, the valve bladder syndrome is a progressive condition that continues to

S.M. Lambert / Seminars in Pediatric Surgery 24 (2015) 73–78

evolve over the years. Valve bladder syndrome results from the initial obstructive injury to the kidneys and lower urinary tract during fetal development. The valve bladder describes chronic bladder overdistention due to polyuria, impaired bladder sensation, and residual urine volume. These factors contribute to progressive bladder deterioration and can be ameliorated by nocturnal bladder drainage.11 Adult providers should recognize the risk factors for persistent lower urinary tract dysfunction in adult men with posterior urethral valves. These risk factors include bladder wall thickening, prominent bladder neck, residual posterior urethral valves, urethral strictures, polyuria, and dilated upper urinary tracts.12 Posterior urethral valves represent a spectrum of disease, with some boys requiring renal transplantation prior to puberty. A long-term follow-up study documented a 28.5% lifetime risk of end-stage renal disease; 32% of these patients did not progress to end-stage disease until after 13 years of age.13 Therefore, renal function should be monitored by both adult urology and nephrology. The valve bladder syndrome should be recognized and the changing bladder dynamics anticipated. Often, boys require overnight catheter drainage and/or clean intermittent catheterization during the day. Complex reconstructive surgery is sometimes indicated as well. Urodynamic testing and voiding/catheterization logs are important components in the long-term follow-up of these boys. As they reach adulthood, men with posterior urethral valves can be affected by sexual dysfunction and infertility. A Finnish study evaluated the rates of sexual dysfunction and infertility in the general population and in adult men with posterior urethral valves. The International Index of Erectile Function (IIEF) and paternity rates were assessed with no statistical difference in erectile dysfunction and paternity rates between men with a history of posterior urethral valves and the general population.14 Conversely, a meta-analysis of adults with chronic kidney disease demonstrated a 70% summary estimate of erectile dysfunction in men with chronic kidney disease.15 A Cochrane database review revealed that phosphodiesterase-5 inhibitors and oral zinc were helpful in improving IIEF scores and increasing serum testosterone levels, respectively, in men with CKD.16 Infertility can be a concern and should be investigated expeditiously should difficulty in conceiving arise. Men with posterior urethral valves have been successfully achieving paternity.17 While normal sperm counts have been documented in men with posterior urethral valves, immotile sperm, increased liquefaction times, and abnormal sperm agglutination have been identified in some men.18 Other authors report low to normal sperm counts and retrograde ejaculation.19 These long-term follow-up studies are limited to small sample sizes and likely selection bias. Further investigation and collaboration between adult and pediatric providers is necessary to elucidate the true spectrum of long-term effects for men with posterior urethral valves.

Exstrophy–epispadias The exstrophy–epispadias complex commonly presents prenatally or at birth. These children undergo complex reconstructive surgery as infants and most require staged reconstructive surgery during childhood. The genitourinary reconstruction is complex and challenging for a new care provider to assimilate and assume care. Children with cloacal and bladder exstrophy may require bladder augmentation or diversion with closure of the bladder neck and creation of a catheterizable channel. These children are at risk for long-term complications associated with bowel and bladder interposition. As men reach adolescence and adulthood, concerns of sexual function, fertility, and cosmesis arise. The reduced corporeal volume and a widened pubic symphysis result in decreased functional penile length. Men with epispadias can experience difficulties with ejaculation as well. When assessed with the IIEF, adult men with bladder exstrophy

75

reported concerns regarding penile length, sexual inadequacy, poor cosmesis, and incontinence. Over 50% of men reported retrograde ejaculation with an additional 20% reporting anejaculation.20 Women can require further reconstructive surgery due to increased risk of uterine prolapse and sexual dysfunction due to an anterior introitus and a prominent posterior fourchette. As these women reach adulthood, sexual function becomes an important quality-of-life factor. Quality-of-life measurements and the female sexual function index have been successfully utilized to assess these factors in adult women with bladder exstrophy. A survey of adult women with bladder exstrophy (mean age ¼ 32 years) demonstrated that 32% of respondents had urinary incontinence, 54% used a catheterizable channel, and 25% performed clean intermittent catheterization regularly. Additionally, 57% of the women surveyed reported pelvic organ prolapse, with 88% of them requiring surgical intervention. The women surveyed reported statistically significant lower female sexual function index scores than standardized norms.21 Pregnancy is possible but with an increased risk of preterm birth. Creighton and Wood22 recommend a scheduled cesarean section at 37 weeks gestation to avoid potential complications associated with a spontaneous vaginal delivery.

Disorders of sexual development/anorectal malformations Children with disorders of sexual development (DSD) and anorectal malformations represent a broad spectrum of clinical conditions. Irrespective of the particular DSD condition, these children usually require urologic and endocrinologic care into adulthood. Psychological support must also continue throughout the transition to adulthood and beyond. Congenital adrenal hyperplasia (CAH) represents one of the most common genetic diseases in humans; 21-hydroxylase deficiency is the most prevalent etiology. These children require long-term steroid replacement and management. Objectives for steroid replacement prior to adolescence include pubertal development and attainment of adult height. In adulthood, these objectives have been attained and the objectives of therapy must shift. Children with CAH are at risk for infertility and tumor development in the adrenal glands. Additionally, reproductive and sexual function begins to be assessed in adolescence and young adulthood. Vaginal examination, vaginal dilation, and secondary operations to repair stenosis or obstruction are often indicated for pubertal girls with DSD.23 Girls with complete androgen insensitivity often undergo gonadectomy in late adolescence or early adulthood. Follow-up is essential for these adolescents as the risk of gonadal malignancy is approximately 14% in adulthood.24 Children with cloacal anomalies often require long-term general surgery and nephrologic, neurologic, gynecologic, and urology evaluation. Issues of sexual function and fertility arise in adulthood. Fecal and urinary incontinence can also persist. A review of adult men with DSD demonstrated the risk for additional operations as young adults, persistent hypospadias, persistent ventral penile curvature, and abnormal ejaculation. Hypogonadotropic hypogonadism was a necessary treatment in some men. These men were also less satisfied with their penoscrotal appearance than controls.25 There are a paucity of longterm outcomes data for these children and a structured transition to well-equipped adult multidisciplinary teams would be helpful to each adolescent and to children with DSD in general.

Genitourinary malignancies As survival rates improve for children with genitourinary malignancies, there are both men and women who are adult survivors of pediatric genitourinary tumors. Most of these tumors occur in infancy and childhood. Therefore surveillance and post-

76

S.M. Lambert / Seminars in Pediatric Surgery 24 (2015) 73–78

treatment effects often begin during childhood and adolescence but can persist and progress during adulthood. Currently, the 5-year overall survival rate for children with Wilms’ tumor is greater than 90%.26 Children with genitourinary rhabdomyosarcoma have a 5-year overall survival rate of 80%.27 Adult survivors of genitourinary pediatric cancer, including Wilms’ tumor, germ cell tumors, and rhabdomyosarcoma are at risk for long-term complications and require surveillance and serial follow-up. Children exposed to chemotherapy and radiotherapy are at higher risk for hypertension, cardiac dysfunction, pulmonary disease, and renal dysfunction. Complications and side effects from extirpative and reconstructive operations include ureteral strictures, urethral strictures, hemorrhagic cystitis, and lower urinary tract symptoms. Due to these risks, renal function should be monitored throughout adulthood in children with Wilms’ tumor, and evaluation for hypertension should be pursued. In children receiving chemotherapy, the risk for infertility should be addressed early if difficulties with conception arise. Men with difficulties in attempting to conceive should be assessed for oligospermia or azoospermia. If a Wilms’ tumor survivor has received doxorubicin, the risk for cardiac dysfunction should be noted and included in any preoperative evaluation during adulthood. Children requiring radiotherapy are also at elevated risk for posttreatment complications and long-term radiation effects. Finally, the risk for secondary malignancy requires active surveillance. Surveillance protocols are based upon the disease and specific treatment received. Children treated with doxorubicin should have cardiac monitoring with echocardiograms and complete blood counts. Exposure to etoposide also requires complete blood count monitoring yearly. Due to the endocrine and fertility risks associated with alkylating agents, follicle-stimulating hormone, luteinizing hormone, estradiol (women), semen analysis, urinalysis, and complete blood count are often indicated in exposed children. Children who underwent radiotherapy to the abdomen or flank should have echocardiograms, liver function tests, colonoscopy, urinalysis, and basic metabolic panel included in their long-term follow-up.28 Rhabdomyosarcoma is treated with multimodal therapy, including chemotherapy, radiotherapy, and extirpative surgery if indicated. Pelvic exenteration is reserved for children with residual viable tumor after chemotherapy and radiotherapy. When cystectomy or partial cystectomy is indicated, children can undergo vesicostomy, ileal conduit, end cutaneous ureterostomies, or continent urinary diversion in selected patients. Long-term complications of pelvic exenteration include ureteral stricture, hydronephrosis, bowel obstruction, fistula, and lymphedema.29 Primary chemotherapy for most children utilizes vincristine, dactinomycin, and cyclophosphamide. Exposure to alkylating agents such as cyclophosphamide is associated with a high risk of infertility and due to gonadal toxicity. A study of men exposed to high-dose cyclophosphamide as children documented 58.8% azoospermia and 29.4% oligospermia, with only 11.8% of men having normal semen analyses. Of these men, 71% had elevated follicle-stimulating hormone levels. While testosterone levels were normal in 94% of men, luteinizing hormone levels were elevated in 40% of men. These effects appear to be dose dependent, thus the degree of severity depends on the exposure dose rather than age at exposure.30 Therefore, adult providers must be alert to a history of gonadal toxicity even in early childhood. Alkylating chemotherapy including cyclophosphamide and ifosfamide also adversely affect bladder function and can lead to hemorrhagic cystitis and chronic fibrosis. The potential for development of transitional cell carcinoma is also associated with cyclophosphamide exposure.31 Long-term effects of radiotherapy include urologic, gastrointestinal, pulmonary, cardiac, orthopedic, and neurocognitive dysfunction. Pelvic radiotherapy exposure is associated with an increased risk of osteonecrosis of the hip, urethral fistula and colonic fistula.32

Children with urinary diversions due to extirpative surgery for rhabdomyosarcoma are at risk for complications of urinary diversion such as stomal stenosis, stomal irritation, urinary leakage, obstruction, urinary reflux, urinary tract infection, diarrhea, nephrolithiasis, B12 deficiency, and sexual dysfunction. Orthotopic continent urinary diversion has also been described in children after extirpative surgery.33 These children are at risk for late complications associated with orthotopic or heterotopic continent reservoirs as they continue to mature into adulthood. Boys who undergo multimodal therapy including radical cystectomy are at risk for erectile dysfunction. The degree of dysfunction varies, with approximately half of the boys having unsatisfactory erections while others appear to have normal erectile function and satisfaction.34 Women with a history of pelvic rhabdomyosarcoma are at risk for long-term adverse effects. Some of these effects result from the operative oncologic intervention required including hysterectomy, complete or partial vaginectomy, complete or partial cystectomy, and oophorectomy. In one study, over 50% of women, with a median age of 25 years, experienced genitourinary late effects. The documented genitourinary late effects included vaginal stenosis, vesicovaginal fistula, ureteral obstruction, retroperitoneal fibrosis, neurogenic bladder, recurrent urinary tract infection, urinary incontinence, chronic pelvic pain, ovarian failure, and nephrolithiasis. Additionally, 12% of women developed a secondary malignancy, including adenocarcinoma of the colon, cervical cancer, and pelvic osteosarcoma; 77% of women experienced endocrine late effects, which required surgical intervention in 54% of women. Surgical procedures included vaginal dilation, vaginal reconstruction, repair of ureteral obstruction, vesicovaginal fistula repair, and intestinal stricture repair.35 A second European series specifically evaluating women with vulvar and vaginal rhabdomyosarcoma documented 20% incidence of vaginal and urethral stenosis.36 Since the long-term effects of childhood genitourinary cancers affect multiple organ systems, these adolescents require coordinated care throughout adulthood.

Congenital kidney and urinary tract anomalies Many children present antenatally with congenital anomalies of the kidney and urinary tract (CAKUT) that require surgical intervention. These anomalies include vesicoureteral reflux, ureteropelvic junction obstruction, multicystic dysplastic kidney, ureterovesical junction obstruction, ectopic ureteral insertion, renal ectopia, duplicated collecting systems, ureteroceles, or a solitary kidney. Affected children often undergo surgical intervention in childhood but are monitored via laboratory data and radiographic imaging throughout childhood. Children with solitary kidneys are at risk for higher blood pressure, elevated resistive indices, and increasing urinary albumin excretion. These findings suggest glomerular hyperfiltration and require long-term surveillance.37 A review of a European database for renal replacement therapy demonstrated a mean age of 31 years for the onset of renal replacement therapy in individuals with CAKUT.38 Adolescence and young adulthood can be associated with medication noncompliance and transplant graft dysfunction.39 A planned and orderly transition program can minimize the risk for noncompliance and graft loss.40 Children with chronic kidney disease require lifetime follow-up to prevent the progression of disease and monitor for early signs of renal deterioration. The long-term effects of these varied congenital anomalies range in severity but include urinary tract infection, bladder dysfunction, hypertension, nephrolithiasis, and renal hyperfiltration.41 Transition of urology and nephrology care must be carefully directed to each adolescent as he or she progresses into adulthood.

S.M. Lambert / Seminars in Pediatric Surgery 24 (2015) 73–78

Hypospadias Hypospadias represents a common chief complaint and surgical indication in pediatric urology offices. Hypospadias occurs in approximately 1 in 200 to 1 in 300 live male births.42 Hypospadias includes a wide array of disease severity, ranging from megameatus and intact prepuce to penoscrotal or perineal hypospadias with penoscrotal transposition. Surgical repair of hypospadias comprises key components of correcting penile curvature and advancing the urethral meatus to the glans penis. The functional objectives of these principles are to create a normal urinary stream, normal erections, and normal coitus. Ideally, these objectives are attained with a satisfactory cosmesis as well. These objectives can be elusive and multiple operations including free graft harvesting have been described for hypospadias repair. As boys reach puberty, penile development can result in new functional concerns such as fistula enlargement, worsening penile curvature, and poor cosmesis.43 In one series, 38% of adult men who underwent hypospadias repair in childhood self-reported dissatisfaction with voiding, erection, or cosmesis.44 Dissatisfaction with cosmesis is widely varied, with some studies reporting 31% dissatisfaction while others report 81% dissatisfaction after puberty.45,46 When queried regarding voiding dysfunction, 80% of men with hypospadias reported voiding complaints in comparison to 47% in controls.47 Ejaculation abnormalities have been reported in 6–37% of men after hypospadias repair.48 Voiding complaints, urethral fistula, diverticulum, and stricture are best addressed by urethral reconstructive urologists, as general urologists often have limited exposure to the complex urethral reconstructive procedures often required to address these issues. Fertility or sexual function concerns can be evaluated by infertility or sexual function specialists, respectively. Boys with hypospadias repairs in infancy should be followed up through puberty and a directed plan for transition of care can be established.

Nephrolithiasis The incidence of nephrolithiasis has been increasing by 6–10% annually and the current incidence is approximately 50 per 100,000 adolescents.49 Nephrolithiasis is identified in children from the neonatal period through adolescence. The etiologies of these calculi vary from primary hyperoxaluria that requires liver and kidney transplant to hypercalciuria that can generally be managed by diet and/or medication. Many children with metabolic disorders are at high risk for chronic nephrolithiasis, including Dent disease, cystinuria, primary hyperoxaluria, and familial hypomagnesemia with hypercalciuria and nephrocalcinosis.50 These children and adolescents with recurrent nephrolithiasis are managed by both urologists and nephrologists. Many adult urologists and nephrologists specialize in stone disease, and the transition of adolescents with nephrolithiasis has the potential to be uncomplicated. This process becomes less straightforward if the kidney or bladder calculi present in children with complex conditions, such as myelomeningocele and an ileocystoplasty, or children with rare metabolic disorders unfamiliar to adult providers.

Varicocele Adolescent varicoceles are a source of much discussion and research in the pediatric urology community, and both the indications for surgical intervention and the timing of such interventions are debated. Boys undergo varicocelectomy for pain, persistent testicular asymmetry on scrotal ultrasound, or abnormal semen parameters. Boys who do not meet the criteria for operative intervention are typically followed up through adolescence with

77

ultrasounds and semen analyses when possible. All boys with varicoceles have the potential for infertility as adults. In a survey of pediatric urologists, 42% of practitioners did not have long-term follow-up information on their patients, and 89% did not know if their patients developed infertility. Of the men who experienced infertility, 39% underwent varicocelectomy.51 Boys who undergo varicocelectomy in adolescence are still at risk for abnormal semen parameters, including decreased sperm density and motility. Therefore, boys with a varicocele should be educated upon potential fertility concerns. Similarly, undescended testes are addressed with orchidopexy in infancy or at the time of diagnosis in boys presenting later. While the surgical repair is completed in most cases, the risk for infertility remains and requires adult intervention.52 Boys with varicoceles and undescended testes with infertility concerns should be evaluated by adult urologists. To attain the pediatric urologic goal of preservation of the kidneys and upper tracts, safe urine storage, safe urine drainage, urinary continence, fertility, sexual function, and genital cosmesis, the coordinated transition of care to adult providers is essential. The obstacles of altering the patient and caregiver paradigm, locating adult urologists with special expertise, coordinating care with other adult specialties such as nephrology, and navigating the adult health care environment must be overcome to provide quality care to our pediatric patients through this period of transition. The urology community has begun to champion this process. Reports of poor transition that result in heavy stone burden, urethral erosions, and recurrent urinary tract infections are not uncommon in the literature.53 The European Association of Urology and American Urological Association have included forums to discuss transition of urological care and specific strategies.54 There are many potential options for transition of urologic care. The objective of all pediatric urologists should include the successful transition of their patients to adult providers. Dr. Christopher Woodhouse has been a strong champion of long-term outcomes and follow-up for complex pediatric urology conditions. Dr. Woodhouse and his colleagues have structured a model for transition that is based upon a subspecialist with a broad understanding of both pediatric and adult reconstructive urology who will direct the care of these children as they progress through adolescence and adulthood. Dr. Dan Wood, a consultant in adolescent and reconstructive urology at University College London Hospitals, highlights the huband-spoke model, which depends on the partnership between a medical center with superior experience and expertise, and companion providers in the community. Patients benefit from the concentrated clinical and surgical expertise available in a tertiary care facility with established multidisciplinary clinics, while relying on the accessibility of local care when possible. The importance of teaching materials such as readiness checklists and health care diaries for transition of the adolescent is also emphasized.55 In comparison, the urology department at Vanderbilt University has structured a transition program that begins in early adolescence and culminates in a transition clinic with collaboration between adult and pediatric providers. These clinics involve active participation of adult and pediatric providers and staff members.56 Toronto Sick Kids established a “Good 2 Go” program and website with transition of care materials for clinicians, patients, and families. This information is easily accessible on the hospital website. This program is based upon the shared management model that involves provider, family, and young adult.57 These transition programs greatly benefit adolescents with complex urological conditions and ease the process of transfer from pediatric to adult care and childhood to adulthood. A secondary objective of structured programs must be longterm outcomes research. Due to the rarity of these conditions and the dispersal of care, quality prospective data, and evidence-based practices are not currently available. As children are surviving into

78

S.M. Lambert / Seminars in Pediatric Surgery 24 (2015) 73–78

adulthood in greater numbers, these data are essential to delivering high-quality lifetime care.

Conclusion Thoughtfully planned transition of care from pediatric to adult urology providers is essential if we are to attain the goal of preserving of the genitourinary tract in patients with congenital disorders. The obstacles we have outlined, including the need to shift patient– caregiver paradigms, locate adult urologists with special expertise, coordinate care with other adult specialties, and navigate the adult health care environment, must be overcome if we are to provide quality care to our pediatric patients as they move towards adulthood. References 1. American Academy of Pediatrics, American Academy of Family Physicians, Amercian College of Physicians, Transitions Clinical Report Authoring Group, Cooley WC, Sagerman PJ. Supporting the health care transition from adolescence to adulthood in the medical home. Pediatrics. 2011;128(1):182–199. 2. Stewart D. Transition to adult services for young people with disabilities: current evidence to guide future research. Dev Med Child Neurol. 2009; 51(suppl 4):169–173. 3. Van Der Toorn M, Cobussen-Boekhorst H, Kwak K, D'hauwers K, de Gier RP, Feitz WF, et al. Needs of children iwth chronic bladder in preparation for transfer to adult care. J Pediatr Urol. 2013;9(4):509–515. 4. Crowley R, Wolfe L, Lock K, McKee M. Improving the transition between paediatric and adult healthcare: a systematic review. Arch Dis Child.. 2011;96(6):548–553. 5. Wood HM. Editorial comment refers to urologic problems in SPina Bifida patients transitioning to adult care. Urology. 2014;84(2):444. 6. Rink RC. DSD, transitions and my concerns. Dialogues Pediatr Urol. 2013;34(4):6–7. 7. Spina Bifida Association. What is spina bifida. www.spinabifidaassociation.org; Accessed 21.10.14. 8. Vajda P, Buyukunal CS, Solet Y, Danismed N, Juhasz Z, Pinter AB. A therapeutic method for failed bladder augmentation in children: re-augmentation. BJU Int. 2006;97(4):816. 9. Metcalfe PD, Cain MP, Kaefer M, et al. What is the need for additional bladder surgery after bladder augmentation in childhood? J Urol. 2006;176(4, pt 2): 1801–1805. 10. Summers SJ, Elliot S, McAdams S, et al. Urologic problems in Spina Bifida patients transitioning to adult care. Urology. 2014;84(2):440–444. 11. Koff SA, Mutabagani KH, Jayanthi VR. The valve bladder syndrome: pathophysiology and treatment with nocturnal bladder emptying. J Urol. 2002;165 (1):291–297. 12. Taskinen S, Heikkila J, Rintala R. Effects of posterior urethal valves on long-term bladder and sexual function. Nat Rev Urol. 2012;9(12):699–706. 13. Heikkila J, Holmberg C, Kyllonen L, Rintala R, Taskinen S. Long-term risk of end stage renal disease in patients with posterior urethral valves. J Urol. 2011;186 (6):2392–2396. 14. Taskinene S, Heikkila J, Santtila P, Rintala R. Posterior urethral valves and adult sexual function. BJU Int. 2012;110(8):E392–E396. 15. Navaneethan SD, Vecchio M, Johnson DW, et al. Prevalence and correlates of self-reported sexual dysfunction in CKD: a meta-analysis of observational studies. Am J Kidney Dis. 2010;56(4):670–685. 16. Vecchio M, Navaneethan SD, Johnson DW, et al. Interventions for treating sexual dysfnction in patients with chronic kidney disease. Cochrane Database Syst Rev. 2010;12(12):CD007747. 17. Holmdahl G, Sillen U. Boys with posterior urethral valves: outcome concerning renal function, bladder function, and paternity at ages 31–44 years. J Urol. 2005; 174(3):1031–1034. 18. Puri A, Gaur KK, Kumar A, Bhatnagar V. Semen analysis in post-pubertal patients with posterior urethral valves: a pilot study. Pediatr Surg Int. 2002;18 (2-3):140–141. 19. Lopez Pereira P, Miguel M, Martinez Urrutia MJ, et al. Long-term bladder function,fertility and sexual function in patients with posterior urethral valves treated in infancy. J Pediatr Urol. 2013;9(1):38–41. 20. Salem HK, Eisa M. Long-term follow-up (18–35 years) of male patients with history of bladder exstrophy (BE) repair in childhood: erectile function and fertility potential outcome. J Sex Med. 2012;9(5):1466–1472. 21. Deans R, Liao LM, Wood D, et al. Sexual function and health-related quality of life in women with classic bladder exstrophy. BJU Int. 2014 [May 5, ePub]. doi:10:1111/bju.12811. 22. Creighton SM, Wood D. Complex gynecological and urological problems in adolescents: challenges and transition. Postgrad Med J. 2013;89(1047):34–38. 23. Crouch NS, Creighton SM. Transition of care for adolescents with disorders of sexual development. Nat Rev Endocrinol. 2014;10(7):436–442. 24. Deans R, Creighton SM, Liao LM, Conway GS. Timing of gonadectomy in adult women with complete androgen insensitivity syndrome CAIS: patient preferences and clinical evidence. Clin Endocrinol. 2012;76(6):894–898.

25. van der Zwan YV, Callens N, van Kuppenveld J, et al. Long-term outcomes in males with disorders of sexual development. J Urol. 2013;90(3):1038–1042. 26. Green DM. The treatment of stages I-IV favorable histology Wilms’ tumor. J Clin Oncol. 2004;22(8):1366–1372. 27. Punyko JA, Mertens AC, Baker KS, Ness KK, Robinson LL, Gurney JG. Long-term survival probabilties for childhood rhabdmyosarcoma. Cancer. 2005;103(7): 1475–1483. 28. Sadak KT, Ritchey ML, Dome JS. Paediatric genitourinary cancers and late effects of treatment. Nat Rev. 2013;10(1):15–25. 29. Michalkiewicz El, Rao BN, Gross E. Complications of pelvic exenteration in children who have genitourinary rhabydomyosarcoma. J Pediatr Surg. 1997;32(9):1277–1282. 30. Kenney LB, Laufer MR, Grant FD, Grier J, Diller L. High risk of infertility and long term gonadal damage in males treated with high dose cyclophosphamide for sarcoma during childhood. Cancer. 2001;19(3):613–621. 31. Agarwala S, Hemal AK, Seth A, Gupta AK, Bhatnagar V, Mitra DK. Transitional cell carcinoma of the urinary bladder following exposure to cyclophosphamide in childhood. Eur J Pediatr Surg. 2001;11(3):207–210. 32. Sharma Arindam, Kurtz MP, Eswara JR. Three distinct urethral fistulae 35 years after pelvic radiation. Nephrourol Mon. 2014;6(2):1–4. 33. Rigamonti W, Iafrate M, Milani C, Capizzi A, Bisogno G, Carli M, Glazel GP. Orthotopic continent urinary diversion after radical cystectomy in pediatric patients with genitourinary rhabdomyosarcoma. J Urol. 2006;175(6):1092. 34. Macedo A Jr, Ferreira PV, Barroso U Jr, et al. Sexual function in teenagers after multimodal treatment of pelvic rhabdomyosarcoma: a preliminary report. J Pediatr Urol. 2010;6(6):605–608. 35. Spunt SI, Sweeney TA, Hudson MM, Billups CA, Krasin MJ, Hester AL. Late effects of pelvic rhabdomyosarcoma and its treatment in female survivors. J Clin Oncol. 2005;23(28):7143–7151. 36. Magne N, Oberline O, Martelli H, Gerbaulet A, Chassagne D, Haie-Meder C. Vulval and vaginal rhabdomyosarcoma in children: an update and reappraisal of Institut Gustave Roussy brachytherapy experience. Int J Radiat Oncol Biol Phys. 2008;72(3):878–883. 37. Siomou E, Giapros V, Papadopoulou F, Pavlou M, Fotopoulos A, Siamopoulou A. Growth and function in childhood of a normal solitary kidney from birth or from early infancy. Pediatr Nephrol. 2014;29(2):249–256. 38. Wuhl E, van Stralen KJ, Verrina E, et al. Timing and outcome of renal replacement therapy in patients with congenital malformations fo the kidney and urinary tract. Clin J Am Soc Nephrol. 2013;8(1):67–74. 39. Rianthavorn P, Ettenger RB. Medication non-adherence in the adolescent renal transplant recipient: a clinician’s viewpoint. Pediatr Transplant. 2005;9(3):398–407. 40. Akchurin OM, Melamed ML, Hashim BL, Kaskel FJ, DelRio M. Medication adherence in the transition of adolescent kidney transplant recipients to the adult care. Pediatr Transplant. 2014;18(5):538–548. 41. Hegde S, Coulthard MG. Renal agenesis and unilateral nephrectomy: what are the risks of living with a single kidney? Pediatr Nephrol. 2009;24(3):439–446. 42. Baskin LS, Himes K, Colborn T. Hypospaidas and endocrine disruption: is there a connection? Environ Health Perspect. 2001;109(11):1175–1183. 43. Tourchi A, Hoebeke P. Long-term outcome of male genital reconstruction in childhood. J Pediatr Urol. 2013;9(6, pt B):980–989. 44. Aho MO, Tamela OK, Tammela TL. Aspects of adult satisfaction with the result of surgey for hypospadias performed in childhood. Eur Urol. 1997;32(2):218–222. 45. Bracka AA. Long-term view of hypospadias. Br J Plast Surg. 1989;42(3):251–255. 46. Mureau MA, Slijper FM, Slob AK, Verhulst FC, Nijman RJ. Satisfaction with penile appearance after hypospadias surgery: the patient and surgeon view. J Urol. 1996;155(2):703–706. 47. Aho MO, Tammela OK, Somppi EM. Sexual and social life of men operated in childhood for hypospadias and phimosis. A comparative study. Eur Urol. 2000; 37(1):95–100. 48. Singh JC, Jayanthi VR, Gopalakrishnan G. Effect of hypospdias on sexual function and reproduction. Indian J Urol. 2008;24(2):249–252. 49. Tasian GE, Copelovitch L. Evaluation and medical management of kidneys stones in children. J Urol. 2014;92(5):1329–1336. 50. Edvardsson VO, Goldfarb DS, Lieske JC. Hereditary causes of kidney stones and chronic kidney disease. Pediatr Nephrol. 2013;28(10):1923–1942. 51. Pastuszak QW, Kumar V, Shah A, Roth DR. Diagnostic and management approaches to pediatric and adolescent varicocele: a survery of pediatric urologists. Urology. 2014;84(2):450–455. 52. van Brackel J, Kranse R, de Muinck Keizer-Schrama SM, et al. Fertiity potential in men with a history of congenital undescended testes: a long-term follow-up study. Andrology. 2013;1(1):100–108. 53. Angermeier KW, Simon J, Wood HM. Transitional urology: caring for the urological needs of adults and adolescents born with complex congenital anomalies. Renal and Urology News. www.renalandurologynews.com/transitionalurology-caring-for-the-urological-needs-of-adults-and-adolescents-born-withcomplex-congenital-anomalies; 2011. Accessed 11.11.14. 54. Stein Jill. Dedicated clinic can ease transition to adult urology service. Medscape. www.medscape.com/viewarticle/720663; 2010. Accessed 11.11.14. 55. Wood D. Adolescent urology: developing lifelong care for congenital anomalies. Nat Rev Urol. 2014;11(5):289–296. 56. Tanaka ST, Kaufman MR, Brock JW III. The aging pediatric urology patient: obstacles and opportunities in transition care. J Urol. 2012;187(4):1159–1160. 57. Kieckhefer GM, Trahms CM. Supporting developemnt of children with chronic conditions: from compliance toward shared management. Pediatr Nurs. 2000; 26(4):354–363.