Preface Douglas A. Canning and Thomas F. Kolon. Prenatal Management of Urogenital Disorders 389 Michael C. Carr
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1 PEDIATRIC UROLOGY FOR THE GENERAL UROLOGIST Preface Douglas A. Canning and Thomas F. Kolon xv Dedication Douglas A. Canning and Thomas F. Kolon xvii Prenatal Management of Urogenital Disorders 389 Michael C. Carr Antenatal sonography has increased the detection of urogenital anomalies markedly, including conditions that lead to significant morbidity and mortality. Prenatal intervention is feasible to arrest and sometimes reverse the sequelae of bladder-outlet obstruction, but not necessarily renal damage. Myelomeningoceles, the most severe form of spina bifida, can be corrected in utero, with improvements in hydrocephalus and a decreased incidence of ventricular shunting postnatally. Medical therapy to prevent virilization associated with congenital adrenal hyperplasia has been successful, with improved ability to detect its presence prenatally. As techniques evolve to correct underlying disease processes, it becomes important to assess the therapies critically, particularly with long-term outcome data. Initial Management of Complex Pediatric Disorders: Prunebelly Syndrome, Posterior Urethral Valves 399 William R. Strand Prunebelly syndrome and posterior urethral valves are conditions of detrusor dysfunction associated with antenatal urethral obstruction. The resultant severe hydroureteronephrosis and renal dysplasia initiate a sequence potentially leading to renal failure. This article reviews clinical features and explores neonatal evaluation and treatment for both conditions. A comprehensive approach to initial management aimed at optimal renal preservation and bladder rehabilitation is proposed. Initial Management of Complex Urological Disorders: Bladder Exstrophy 417 Darcie A. Kiddoo, Michael C. Carr, Susan Dulczak, and Douglas A. Canning Bladder exstrophy is a rare condition associated with lifelong issues including infection, renal insufficiency, incontinence, and fertility. Despite its seriousness, many affected children lead normal lives. With new operative techniques, children require fewer surgeries and have more hope for continence. With close follow-up, no child should develop VOLUME 31 Æ NUMBER 3 Æ AUGUST 2004 vii
2 renal insufficiency. Families should be reassured that a normal life and parenthood with normal sexual function are possible. Initial Urologic Management of Myelomeningocele 427 Warren T. Snodgrass and Richard Adams Disordered innervation of the detrusor musculature and external sphincter in patients with myelomeningocele adversely affects bladder function and potentially threatens the upper urinary tracts. Children with this condition can be categorized into highand low- risk groups for secondary damage from a neurogenic bladder based on intravesical pressure. Those with elevated pressure are at risk for hydronephrosis or reflux, and evidence suggests that early management of high pressure protects the bladder from additional damage, reducing the need for augmentation. Management decisions made during infancy potentially impact long-term outcomes for preserving renal function and achieving urinary continence. This discussion focuses on the initial urologic care of infants with myelomeningocele, emphasizing the controversy regarding urodynamic versus radiologic-based management of the neurogenic bladder. A Practical Approach to Intersex in the Newborn Period 435 Grace Hyun and Thomas F. Kolon Sexual determination is a complex process that occurs in an organized sequential manner. When chromosomal, gonadal, or phenotypic sex determination goes awry, intersexuality develops. Advances in molecular biology have made it easier to understand the various phenotypes that are encountered. It is easy to be overwhelmed when reviewing the testosterone synthesis pathway and the intersex differential diagnosis. This article presents a useful approach to the evaluation of the newborn with ambiguous genitalia. Hypospadias 445 Aseem R. Shukla, Rakesh P. Patel, and Douglas A. Canning Hypospadias results from abnormal development of the penis that leaves the urethral meatus proximal to its normal glanular position anywhere along the penile shaft, scrotum, or perineum. Hypospadiology is an evolving and expanding discipline that remains at the forefront of pediatric surgical innovation. Although modern experiments have begun to yield a deeper understanding of the genetic, hormonal, and environmental basis of hypospadias, the quest for a surgical procedure that consistently results in a straight penis with a normally placed glanular meatus has challenged surgeons for over two centuries. This article focuses on the advances in the understanding of the etiology of hypospadias and the current approaches to the correction of hypospadias. Male Neonatal Circumcision: Indications, Controversies and Complications 461 Joel C. Hutcheson The debate about whether to circumcise infants in the neonatal period likely will continue. As the medical and ethical issues are discussed and studied, however, economic factors are beginning to limit the practice in the United States. As the shift in reimbursement occurs, parents who believe that circumcision is a medically necessary practice will need to be reassured that their child may lead a healthy life with an intact foreskin. Cryptorchidism: Diagnosis, Treatment, and Long-term Prognosis 469 Thomas F. Kolon, Rakesh P. Patel, and Dale S. Huff Cryptorchidism is a common anomaly treated by every pediatric urologist. The etiology is multifactorial and includes hormonal and molecular factors. Diagnostic abilities may viii
3 be enhanced by radiologic advances. Hormonal and surgical treatment modalities are discussed herein, as well as the identification and prevention of long-term sequelae. Voiding Dysfunction in Children 481 Seth L. Schulman The practicing urologist commonly sees children with lowerÿurinary tract dysfunction who wet or have recurrent urinary tract infections. This article identifies the proposed etiologies of such behavior in children in whom there are no anatomic or neuropathic causes, outlines the approach to evaluating affected children, and describes a stepwise, interdisciplinary approach to treatment. Nocturnal Enuresis: Medical Management 491 Anju A. Mammen and Fernando A. Ferrer Nocturnal enuresis is a problem that affects many children and their families. The etiology seems to be multifactorial and may include a combination of genetic factors, abnormal urodynamics, alterations in vasopressin secretion, sleep factors, psychologic factors, organic disease, and maturational delay. Generally, a complete history and physical examination, with a specific focus on the genitourinary, gastrointestinal, and neurologic systems, is all is that is needed in the evaluation of a patient with enuresis. Currently, the mainstays of medical therapy are DDAVP, imipramine, and oxybutynin. Medications can help to control the symptoms of enuresis, but they generally do not provide a cure; therefore, behavioral therapy is often recommended in conjunction with pharmacotherapy. Nocturnal Enuresis: Behavioral Treatments 499 Nathan J. Blum Nocturnal enuresis is a common problem. Physiologic and environmental factors are thought to have a role in the etiology and treatment of this condition. This article discusses the association between enuresis and behavioral or emotional problems. Common behavioral treatments are described, and evidence for their efficacy is reviewed. A brief discussion of hypnosis and acupuncture is included. The Adolescent Varicocele: To Treat or Not to Treat 509 Gary W. Bong and Harry P. Koo Whether to treat adolescents with varicocele is controversial because over 80% of adult varicoceles are not associated with infertility. Most physicians agree that treating all adolescents with varicocele and subjecting boys to unnecessary surgery is inappropriate, costly, and not without ethical considerations. Waiting until patients present as adults with potentially irreversible infertility, however, is equally unacceptable. Pediatricians and urologists must determine which adolescents are at greatest risk for future fertility problems and warrant early intervention. This article reviews the current literature surrounding adolescent varicocele and offers recommendations for identifying individuals who would most benefit from treatment. Urinary Tract Infection in Children: Etiology and Epidemiology 517 Jian F. Ma and Linda D. Shortliffe The urinary tract is a relatively common site of infection in infants and young children. Urinary tract infection (UTI) may result in significant acute morbidity, as well as longterm medical complications. Recent advances elucidating the pathogenÿhost interaction have broadened the understanding of the pathogenesis and clinical progression of ix
4 pediatric UTI. This article focuses on the epidemiology and pathogenesis of pediatric UTI, and briefly discusses UTI-related complications. Urinary Tract Infections in Children: Treatment 527 Sameer M. Malhorta and William A. Kennedy II Urinary tract infection is a frequent diagnosis in children who are referred to the urologist. Infections vary in scope and severity, warranting thoughtful consideration of appropriate therapy. Infections of the genitourinary system may include bacterial, viral, fungal, or parasitic microorganisms. Adequate therapy requires rapid detection and control of these conditions to prevent pyelonephritic renal scarring and its sequelae. Vesicoureteral Reflux: Who Benefits from Surgery? 535 Christopher S. Cooper and J. Christopher Austin Increased recognition of the association between urinary tract infections and reflux, sibling screening, and the evaluation of antenatal hydronephrosis have resulted in an increase in the diagnosis of reflux early in life. Although many children would sustain no untoward affects of their condition if reflux were left untreated, the disease can be severe and even life threatening. Although antireflux operations have been refined and improved, it remains difficult to determine which individuals truly benefit from these operations. A large prospective randomized placebo-antibiotic-operation study with long-term follow-up is needed, as is the development of nomograms, to assess the individual child s risk of adverse outcomes. Vesicoureteral Reflux: Surgical Approaches 543 J. Christopher Austin and Christopher S. Cooper The treatment of vesicoureteral reflux has evolved over the past half-century from primarily surgical to nonoperative treatments. Although the benefits of surgical correction versus medical management are debated, the surgical techniques that have evolved are highly effective in correcting vesicoureteral reflux. Recently, the US Food and Drug Administration has approved an injectable implant of dextranomer/hyaluronic acid copolymer for use in children, making the endoscopic treatment of reflux a potential alternative to open surgical correction. This article reviews the different surgical techniques, postoperative management, and complications, as well as the various implants used in the endoscopic correction of vesicoureteral reflux and their outcomes. Evaluation of Hematuria in Children 559 Kevin E.C. Meyers The detection of blood in a child s urine is alarming and often prompts many laboratory studies. Hematuria is one of the most important signs of renal or bladder disease, but proteinuria often is a more important diagnostic and prognostic finding. The physician should ensure that serious conditions are not overlooked, avoid unnecessary laboratory studies, reassure the family, and provide guidelines for additional studies if there is a change in the child s course. This article provides an approach to the evaluation and management of hematuria in a child. Many tests have been recommended for the child with hematuria, but no consensus exists on a systematic evaluation. Medical Management of Pediatric Stone Disease 575 Sharon M. Bartosh Childhood urolithiasis remains endemic in certain parts of the world, namely, Turkey and the Far East. The prevalence of nephrolithiasis in North American children varies widely among geographic regions and accounts for 1 per 1000 to 1 per 7600 pediatric x
5 hospital admissions. Stones occur in children of all ages. The clinical manifestations of stone disease are often more subtle in children when compared with the dramatic adult presentation. This article discusses the evaluation and medical management of pediatric stone disease. Surgical Management of Children with Urolithiasis 589 Hsi-Yang Wu and Steven G. Docimo The management of urolithiasis in children poses specific technical challenges that require planning before endoscopy and that affect the risks and outcomes of these procedures. The indications for operative intervention in children and adults are similar: infection, persistent symptoms of flank pain, nausea, and vomiting, as well as the failure to pass a ureteral stone after an appropriate trial of observation (3ÿ6 weeks). Specific adjustments for performing extracorporeal shock wave lithotripsy, ureteroscopy, percutaneous nephrolithotomy, and cystolithotomy in children are discussed. Laparoscopic and Robotic Approach to Genitourinary Anomalies in Children 595 Craig A. Peters The evolution of laparoscopic surgery in pediatric urology has been long and slow, but is emerging steadily and seems to be here to stay. This article reviews the basic applications of laparoscopic methods in pediatric urology, including diagnostic and operative procedures. The new horizons of robotic assistance for laparoscopic surgery make highly accurate and efficient reconstructive procedures possible. Renal and Adrenal Tumors in Children 607 Stephen A. Zderic Survival rates for children with kidney tumors approach 90% for even the most advanced stages of disease, but the surgical management of large lesions remains challenging. With the development of additional chemotherapeutic regimens and the use of radiation therapy, survival rates have improved dramatically. The National Wilms Tumor Study has conducted four long-term studies addressing how adjunctive therapy may be tailored optimally to maximize survival and minimize the exposure to chemotherapy and radiation therapy. Pediatric Urologic Oncology: Bladder, Prostate, Testis 619 Hsi-Yang Wu and Howard M. Snyder III Although treatment for bladder, prostate, and testis cancer comprises a large part of adult urologic practice, the tumors that affect these organs in children are rare. Rhabdomyosarcoma, which affects the bladder, prostate, vaginal, and paratesticular areas, is treated with a combination of surgery, chemotherapy, and radiation. Most transitional cell carcinomas of the bladder and prepubertal testis tumors are managed surgically owing to the low stage at presentation. Application of the technical advances learned in adults with tumors of the bladder, prostate, and testis, combined with an understanding of the difference in tumor biology, helps urologists improve the treatment of these tumors in children. The Craft of Urologic Surgery: Modern Radical Perineal Prostatectomy 629 Jeffrey M. Holzbeierlein, H.J. Porter II, MD, and J. Brantley Thrasher Radical perineal prostatectomy (RPP) is the original approach used by urologists for removing the entire prostate for cancer treatment. From its original description xi
6 approximately 100 years ago, it has gone through periods of increased and decreased popularity. RPP has not been performed as commonly in the United States since the late 1970s, with the introduction of the radical retropubic prostatectomy. With increased emphasis on reducing morbidity associated with radical prostatectomy by less-invasive techniques, however, more surgeons are revisiting RPP. As discussed in this article, RPP offers several advantages over the retropubic or laparoscopic approach for certain patients. Index 643 xii
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