Journal Archives - Volume 14
Co-Editor:
Prof. Christopher R. J. Woodhouse; MB, FRCS, FEBU.
Professor Christopher R. J. Woodhouse read medicine at Guy’s (University of London). He graduated in 1970. In 1975 he became FRCS. He did his urology training at The Royal London with Professor John Blandy and at the Institute of Urology (St Peter’s Hospitals). In 1981 he became senior lecturer at the Institute and Consultant at the Royal Marsden Hospital. He has continued to work at both of these places. The Institute of Urology was subsumed into University College, London in 1990 and he became Professor of Adolescent Urology in UCL in 2006.
Currently most pediatric urologists reconstruct the lower urinary tract with the modern staged repair of exstrophy (MSRE) or complete primary repair of exstrophy (CPRE), with radical soft-tissue mobilization (RSTM) as an alternative. MSRE is a progression of the staged repair. Initially, bladder closure was followed by bladder neck reconstruction at age two to three and later epispadias repair. MSRE instead follows closure with epispadias repair at six to twelve months of age, with the hope of increasing bladder capacity by creating a more physiologic outlet resistance. Bladder neck reconstruction is ideally offered when the child demonstrates not only interest in becoming continent, but a willingness to actively participate in the continence process. While all of the currently employed techniques offer patients the hope of continence, rates vary by patient population, technique and institution. Furthermore, continence may only be achieved after augmentation, typically necessitating clean intermittent catheterization, or diversion in some patients. In addition to clinical data such as success of initial closure and bladder capacity, research into the exstrophic smooth muscle may enhance our ability to counsel patients and prognosticate bladder growth and continence potential. This may prove particularly helpful in determining which patients are appropriate for staged bladder neck reconstruction or further continence procedures for patients who undergo CPRE. Further understanding may also reveal treatment options that increase bladder capacity or augment to urethral mechanism, ultimately resulting in improved continence. Patients must be followed into adulthood; as more exstrophy patients enter adulthood with reconstructed bladders, adult urologists may encounter complicated patients and should familiarize themselves with current management strategies.
Reconstructive surgery of the basic bladder problems has progressed so much in the last 25 years, that they can expect to have a normally working bladder or at least one that can be managed with intermittent clean self catheterisation. Although it has always been recognised that the penis required reconstruction as well, a good functional result for sexual intercourse was not always achieved. The exstrophy patient now grows up in normal society and has the same sexual and reproductive aspirations as his more normal peers. Their libido is as high as that of other adolescents. An objective measure of successful female sexual function, equivalent to erection or ejaculation, is difficult to define. Most series are confined to expressions of the patients' global satisfaction. The results of vaginal reconstruction in exstrophy appear to be satisfactory in the sense that most patients do not request further cosmetic surgery and most engage in sexual intercourse. Three series with reasonable numbers cover 43 patients, but they represent only about 20% of the women recorded in the relevant Institutions data bases. Thirty-four (79%) had regular intercourse and ten of them had dyspareunia (29% of those having regular intercourse). No other general information is available from these three series. Individually, it would appear that libido is normal; the age of sexual debut is 19.9 years; 13 of 22 (59%) who were specifically asked had regular orgasms. Although there has been no formal study, it seems likely that females with exstrophy have normal fertility unless surgery has caused tubal obstruction or some other genital complication. With modern obstetric care, pregnancy and delivery should be uncomplicated except for the risk of prolapse, at least as far as the exstrophy is concerned.
There are contradictory data on the incidence of the undervirilized penis. Several publications show an increasing incidence of these anomalies in the past decades whereas others state that is stable. The incidence of hypospadias is commonly reported to be 1 in 250 male births with significant variations between epidemiological registers. The incidence of micropenis remains undefined. Abnormal hormonal stimulation of the GT can be due to an insufficient hormonal secretion by the gonads often labeled as "gonadal dysgenesis" a failed central regulation or incompetent target tissues. There are two informative periods after birth which may provide some clues about the hormonal origin of an insufficiently developed GT: in the first day of life when the child's hormonal cascade is still under the stimulation of the placenta and in the "mini puberty" between days 15 and 90 of life when there is a flare of gonadotrophic hormones before a long period of quietness until puberty. During these two privileged periods, Leydig cell (testosterone and its precursors) Sertoli cell (AMH and B Inhibin) and gonadotrophic hormones (FSH, LH) can all be measured and Beyond the third month of life, AMH and the androgens can only be measured after HCG stimulation. However, it is important to note that there is no consensus between centres about the HCG stimulation protocols and their interpretation. In our centre, 6 intramuscular injections of 1500IU are given over a period of 12 days and plasma steroids are measured on the 13th day. Plasma testosterone above 10 nMol/L is considered as a satisfactory response to HCG stimulation. AMH above 600 pMol/L is interpreted as normal.
Virilisation in an adolescent girl may be devastating socially, and psychologically. The most outward signs are hirsutism, acne, and secondary amenorrhea. Menstrual dysfunction, fertility issues, and hirsutism have been found to have adverse effects on emotional well-being, self-perception (including poor body image, self-consciousness, & low self-esteem), social functioning, and sexual behavior. Hirsutism, generally associated with hyperandrogenemia, is most commonly caused by polycystic ovary syndrome (three out of every four cases), medication use, endocrinopathies, and neoplasms. Though these characteristics may be worrisome to the typical teenager, they may not be the only symptom of over-virilisation. Virilization of the female genitalia may vary widely in severity as measured by the Prader Scale. This scale ranges from 0 (the unvirilised female), to 5 (the completely virilised female). Issues of an appearance of gender typicality have been debated for parent and patient concerns, with risk to genital sensory function weighed against cosmesis. Timing, staging and separating clitoroplasty, and vaginoplasty, (allow patient input, decrease unwanted surgery and revisions) continue to be at issue. Simple clitoromegaly or very distal entrance of the vagina into the urogenital sinus may require no treatment. In more virilised forms (as the vagina enters the urogenital sinus more proximally and nearer the bladder neck), typical intercourse may be impossible. Reconstructive surgery may be necessary to establish sexual capability (if desired by the patient).
Posterior urethral valves (PUV) are the most common cause of obstructive uropathy involving the lower urinary tract in children. Although endoscopic valve ablation is effective in resolving the anatomical obstruction, the sequelae on the bladder and kidneys after valve ablation remain (and sometimes deteriorate) during childhood and through adolescence. It has been nearly 30 years since Mitchell coined the term valve bladder syndrome to emphasize the relationship of altered bladder function, progressive hydronephrosis and renal function deterioration. This term describes clinical findings that can continue and progress long after an obstruction caused by posterior urethral valves has been relieved. The features of this syndrome include the persistent dilation of the upper urinary tracts, a thick-walled, noncompliant urinary bladder, urinary incontinence, and polyuria secondary to nephrogenic diabetes insipidus. In general, bladders affected by valve-bladder syndrome are found to be thickened, poorly compliant and hypertonic. Histologic studies of these bladders have shown an altered ratio of collagen to smooth muscle deposition, which determines the compliance of the bladder. when considering bladder augmentation for a patient with a PUV, before surgery we must determine whether he will be able to undergo urethral CIC. Otherwise, bladder augmentation must necessarily be associated with a Mitrofanoff procedure to make it possible for him to endure CIC and void his neobladder to a satisfactory extent. The importance of checking renal and bladder function throughout life in adolescents born with PUV cannot be over emphasized. Increasing attention to bladder dysfunction and its early treatment could probably improve the long-term prognosis.
The primary aim of treatment in neurogenic bladders is attaining a significant dry period and preventing renal damage. There is no permanent cure to the condition per se and the treatment is life-long. It is important to closely monitor adolescents with significant neurological abnormalities as physical growth can unmask decompensated states. Overall passage to adolescence is kind to the bladder that has no overt disease. However children with diseased bladders, the general tendency is to worsen as aging produces muscle and nerve degeneration in the bladder wall. Almost all children with a neuropathic bladder will have received treatment of the bladder aimed at preserving the kidneys and maintaining continence. Treatment has been aimed at reducing the storage pressure to less than 40 cm water and maintaining continence by clean intermittent catheterization (CIC), anticholinergic drugs and, when necessary, bladder augmentation. Patients who have been managed optimally in childhood reach adolescence with a normal upper urinary tract, fair bladder compliance and capacity, self-emptying at acceptable intervals, and continence between catheterizations. With proper management in childhood adolescent patients are able to independently take care of themselves with CICs and bowel movements. With properly opted modalities, the patients get rid of urinary tract infections and dilated upper tracts.
The use of bowel in the urinary tract has become an important means of reconstruction in the ureter, bladder and rarely the urethra. This chapter concentrates on bladder reconstruction. Enterocystoplasty remains an important component for a reconstructive urologist to use the in the management of incontinence, preservation of upper tracts, following extirpative surgery or as treatment for congenital anomalies. It has lifelong consequences that require careful and specialist follow-up coupled with patient support. Patients need to understand these risks before they commit to such surgery and to take an active part in their own long-term management.
The current practice of urinary diversion and undiversion in children has a long history of trial and error. In this article authors trace the evolution of current views on the subject. The majority of pediatric urinary diversions are carried out for management of congenital anomalies. Other reasons for diversion include trauma, malignancy, neurologic disorders, severe inflammatory bladder disease and failed previous surgery. Over the past 30 years the incidence of urinary diversions has been decreasing, not only because better methods of managing these children have developed but also because it has become apparent that urinary diversions have complications of their own and often diversion does not serve its intended purpose. Undiversion of the previously diverted urinary tract is uncommon now since many diverted patients were reconstructed in the late 1970s and 1980s. A tremendous amount of information was gained at the time. Increasing knowledge of bowel physiology and the development of antireflux and continence procedures has made primary reconstruction the standard of care for children with severe urinary tract dysfunction. Much of what has been learned over the years still holds true today and will continue to be applicable in future cases of urinary tract reconstruction. There are numerous options available to the surgeon and careful and thoughtful evaluation and planning is essential to identify the best option for a particular child. A successful outcome is as dependent on patient selection as it is on surgical technique. In addition, the surgeon must be capable and willing to deviate from his preoperative plans if operative findings dictate.
The science of hypospadias continues to evolve. In the current scenario, the surgeon has a vast armamentarium of hypospadias repairs including single and multiple staged procedures, vascularised onlay flaps, buccal mucosal grafts, etc. The advances in the operative skills, availability of technical devices and focus on 'lateral' areas such as suture materials, dressing of the wound and post-operative care have set high standards for functional and cosmetic outcomes. Each case of hypospadias is different and the surgeon is entrusted with the responsibility of ensuring individualized evaluation and care. Despite this, There is a distinct sub-group of patients with hypospadias who are not cured even after multiple repair attempts even in the best hands and present a complex problem to the surgeon. Horton and Devine used the term "hypospadias cripple" to describe this subgroup to signify the presence of residual significant penile deformity even after multiple surgeries. It is this group who is in need for 'salvage hypospadias repair'. The situation is made complex by the general lack of healthy tissues for urethroplasty and skin coverage; the variable and poorly defined blood supply of the scarred penile skin, scarred or sacrificed urethral plate, the frustration of the patient or the parents and the inability of the surgeon to make a promise. With this review, the authors have made an attempt to review the fundamental principles of evaluation and management of a patient with failed hypospadias repair. An attempt has been made to develop an algorithm for the operative management of these patients.
Renal tract malformations (RTMs) represent deviations from normal development. They are common, for example being diagnosed by antenatal ultrasonography in 0.16% births, representing one tenth of all anomalies diagnosed in this manner. Renal tract development is characterised by changing patterns of expression of many hundreds of genes, from the inception of the kidney, ureter and bladder, through the growth of these rudiments into functional, mature organs. Many of the genes essential for renal tract development also operate in other organ systems, proving one explanation why one third of RTMs are 'syndromic', occurring in association with specific patterns of extra-renal malformations. In this chapter, the authors introduce readers to this relatively new field of the genetics of human RTMs, some general genetic concepts as well as mention specific diseases.
Penile deficiency is observed in aphallia & several disorders of sex development. Perinatal exposure to androgens often causes gender dysphoria later in life requiring male sex re-assignment. Besides, penile loss and the demand for masculinizing surgery in transsexuals, has led to an increasing need for phallic reconstruction. Due to the complexity of available procedures, morbidity & scarring at donor site and complications of single stage reconstructions, staged procedures are increasingly preferred. Author describes a simple phalloplasty as a definitive procedure which is devoid of donor site scarring and suitable for urethral inlay and penile prosthesis in subsequent stages.