Predictors of becoming overweight among pediatric patients at risk for urinary tract infections.

BACKGROUND: The association between obesity and urinary dysfunction in childhood has been described, albeit through retrospective analysis, making temporal relationships difficult to establish. OBJECTIVE: The objective of this study was to determine risk factors for significant weight gain in children at risk for recurrent urinary tract infections. STUDY DESIGN: A secondary analysis of the Randomized Intervention for Children with Vesicoureteral Reflux and Careful Urinary Tract Infection Evaluation trials was conducted. The outcome of interest in these children was significant increase in body mass index (BMI) percentile (>85th BMI percentile for sex and age) in previously normal-weight children. Multivariable logistic regression was used to determine the independent effects of predetermined risk factors. RESULTS: In total, 446 patients were included in the study. Most patients aged less than 1 year at study entry (229, 51%), and 399 (89%) of patients were female. Eighty-four patients (17%) became clinically overweight. Patients assigned to prophylactic antibiotics were not more likely to gain significant BMI percentiles (adjusted odds ratio [aOR] = 1.1, 95% confidence interval [CI]=0.6-1.8). Significant BMI percentiles were gained in Hispanic/Latino patients compared with whites (aOR = 3.3, 95% CI=1.7-6.4), in children who were infants at study enrollment compared with non-infants (aOR = 2.1, 95% CI=1.2-3.8), and in those with persistent reflux during the study period (aOR = 2.1, 95% CI=1.0-4.3). Neither patients assigned to prophylactic antibiotics (aOR = 1.1, 95% CI=0.6-1.8) nor patients with bladder and bowel dysfunction (BBD) (aOR = 1.2, 95% CI=0.6-2.3) were more likely to gain significant BMI percentiles. DISCUSSION: Significant BMI percentile gain is common in patients at risk for UTIs. Hispanic/Latino ethnicity, persistent reflux, and younger age, specifically infants than non-infants, were identified as independent risk factors for becoming overweight in this population. Exposure to prophylactic antibiotics and BBD were not associated with becoming overweight. CONCLUSION: Risk for becoming overweight should be discussed when managing patients at risk for UTIs, especially in the subpopulations identified.

An evaluation of the readability, quality, and accuracy of online health information regarding the treatment of hypospadias.

BACKGROUND: Hypospadias is one of the most common genital anomalies. Treatment of hypospadias requires surgical repair, usually in childhood. Patients are increasingly using the internet to learn more about their health or that of their children, which can often empower patients to make well-informed healthcare decisions. OBJECTIVE: The objective of this study was to evaluate not only the readability but also the quality and accuracy of available online health information for the treatment of hypospadias. STUDY DESIGN: Search terms for hypospadias treatment were queried on major search engines. Each website was classified into one of four categories: institutional, commercial, charitable organization, or personal website. Content on each website discussing treatment options was analyzed for readability using three readability formulas. A validated tool, the DISCERN instrument, was used to measure the quality of online health information regarding hypospadias treatment. Accuracy was independently assessed by two pediatric urologists on a 1-5 scale, in which 1 and 5 correspond to 0% and 100% of the information in the text being accurate, respectively. RESULTS: A total of 150 search engine results were acquired, of which 46 were analyzed for readability, quality, and accuracy. The mean readability scores across all websites were 14.89 (Gunning-Fog), 11.01 Simple Measure of Goddledygook (SMOG), and 8.44 (Dale-Chall), which correspond to an 11th- to 12th-grade reading level. Most websites (65.2%) were considered of 'good' quality. Readability and quality scores were not statistically different between website categories. Institutional and charitable websites had the highest mean accuracy scores (3.91 and 3.50, respectively), with institutional websites proving to have significantly more accurate information regarding hypospadias treatment than commercial websites (3.91 and 3.42, respectively; P = 0.001). DISCUSSION: Pediatric urologists should know what information about hypospadias and its treatment exists on the Internet and understand if it is accurate and of good quality and, more importantly, if the material is written at a reading level comprehensible by the majority of parents. Limitations included analysis of only English-written websites regarding hypospadias treatment specifically, using search engines alone rather than other online resources, not evaluating online videos or illustrations, and not using more than two pediatric urologists for determining content accuracy. CONCLUSION: This study demonstrates that online health materials regarding hypospadias and its treatment are written at a level far greater than the reading level of most adults. Most websites were considered of adequate quality, and websites from institutions or references had significantly more accurate information than those from commercial websites.

What is the true clinical relevance of Simkania negevensis and other emerging Chlamydiales members?

Waddlia chondrophila and Simkania negevensis are emerging Chlamydia-related bacteria. Similar to the pathogenic organisms Chlamydia pneumoniae and Chlamydia trachomatis, these emerging bacteria are implicated in human genital infections and respiratory diseases. We used a screening strategy based on a newly developed S. negevensis-specific quantitative real-time PCR (qPCR) and a pan-Chlamydiales qPCR. We could not detect S. negevensis in 458 respiratory, genitourinary, cardiac and hepatic samples tested. One urethral swab was positive for W. chondrophila. We observed a low prevalence of Chlamydiales in respiratory samples (1/200, 0.5%), which suggests that C. pneumoniae is an uncommon respiratory pathogen. Furthermore, we screened 414 human serum samples from Switzerland, England and Israel and observed a low prevalence (<1%) of exposure to S. negevensis. Conversely, humans were commonly exposed to W. chondrophila, with seroprevalences ranging from 8.6% to 32.5%. S. negevensis is not a clinically relevant pathogen, but further research investigating the role of W. chondrophila is needed.

Preliminary report: Surgical outcomes following genitoplasty in children with moderate to severe genital atypia.

INTRODUCTION: Prior studies of outcomes following genitoplasty have reported high rates of surgical complications among children with atypical genitalia. Few studies have prospectively assessed outcomes after contemporary surgical approaches. OBJECTIVE: The current study reported the occurrence of early postoperative complications and of cosmetic outcomes (as rated by surgeons and parents) at 12 months following contemporary genitoplasty procedures in children born with atypical genitalia. STUDY DESIGN: This 11-site, prospective study included children aged ≤2 years, with Prader 3-5 or Quigley 3-6 external genitalia, with no prior genitoplasty and non-urogenital malformations at the time of enrollment. Genital appearance was rated on a 4-point Likert scale. Paired t-tests evaluated differences in cosmesis ratings. RESULTS: Out of 27 children, 10 were 46,XY patients with the following diagnoses: gonadal dysgenesis, PAIS or testosterone biosynthetic defect, severe hypospadias and microphallus, who were reared male. Sixteen 46,XX congenital adrenal hyperplasia patients were reared female and one child with sex chromosome mosaicism was reared male. Eleven children had masculinizing genitoplasty for penoscrotal or perineal hypospadias (one-stage, three; two-stage, eight). Among one-stage surgeries, one child had meatal stenosis (minor) and one developed both urinary retention (minor) and urethrocutaneous fistula (major) (Summary Figure). Among two-stage surgeries, three children developed a major complication: penoscrotal fistula, glans dehiscence or urethral dehiscence. Among 16 children who had feminizing genitoplasty, vaginoplasty was performed in all, clitoroplasty in nine, external genitoplasty in 13, urethroplasty in four, perineoplasty in five, and total urogenital sinus mobilization in two. Two children had minor complications: one had a UTI, and one had both a mucosal skin tag and vaginal mucosal polyp. Two additional children developed a major complication: vaginal stenosis. Cosmesis scores revealed sustained improvements from 6 months post-genitoplasty, as previously reported, with all scores reported as good or satisfied. DISCUSSION: In these preliminary data from a multi-site, observational study, parents and surgeons were equally satisfied with the cosmetic outcomes 12 months after genitoplasty. A small number of patients had major complications in both feminizing and masculinizing surgeries; two-stage hypospadias repair had the most major complications. Long-term follow-up of patients at post-puberty will provide a better assessment of outcomes in this population. CONCLUSION: In this cohort of children with moderate to severe atypical genitalia, preliminary data on both surgical and cosmetic outcomes were presented. Findings from this study, and from following these children in long-term studies, will help guide practitioners in their discussions with families about surgical management.

Prospective assessment of cosmesis before and after genital surgery.

INTRODUCTION: Little data exist about the surgical interventions taking place for children with disorders of sex development (DSD). Most studies that have evaluated cosmetic outcomes after genitoplasty have included retrospective ratings by a physician at a single center. OBJECTIVE: The present study aimed to: 1) describe frequency of sex assignment, and types of surgery performed in a cohort of patients with moderate-to-severe genital ambiguity; and 2) prospectively determine cosmesis ratings by parents and surgeons before and after genital surgery. STUDY DESIGN: This prospective, observational study included children aged <2 years of age, with no prior genitoplasty at the time of enrollment, moderate-to-severe genital atypia, and being treated at one of 11 children's hospitals in the United States of America (USA). Clinical information was collected, including type of surgery performed. Parents and the local pediatric urologist rated the cosmetic appearance of the child's genitalia prior to and 6 months after genitoplasty. RESULTS: Of the 37 children meeting eligibility criteria, 20 (54%) had a 46,XX karyotype, 15 (40%) had a 46,XY karyotype, and two (5%) had sex chromosome mosaicism. The most common diagnosis overall was congenital adrenal hyperplasia (54%). Thirty-five children had surgery; 21 received feminizing genitoplasty, and 14 had masculinizing genitoplasty. Two families decided against surgery. At baseline, 22 mothers (63%), 14 fathers (48%), and 35 surgeons (100%) stated that they were dissatisfied or very dissatisfied with the appearance of the child's genitalia. Surgeons rated the appearance of the genitalia significantly worse than mothers (P < 0.001) and fathers (P ≤ 0.001) at baseline. At the 6-month postoperative visit, cosmesis ratings improved significantly for all groups (P < 0.001 for all groups). Thirty-two mothers (94%), 26 fathers (92%), and 31 surgeons (88%) reported either a good outcome, or they were satisfied (see Summary Figure); there were no significant between-group differences in ratings. DISCUSSION: This multicenter, observational study showed surgical interventions being performed at DSD centers in the USA. While parent and surgeon ratings were discordant pre-operatively, they were generally concordant postoperatively. Satisfaction with postoperative cosmesis does not necessarily equate with satisfaction with the functional outcome later in life. CONCLUSION: In this cohort of children with genital atypia, the majority had surgery. Parents and surgeons all rated the appearance of the genitalia unfavorably before surgery, with surgeons giving worse ratings than parents. Cosmesis ratings improved significantly after surgery, with no between-group differences.

Rapid declines of large mammal populations after the collapse of the Soviet Union.

Anecdotal evidence suggests that socioeconomic shocks strongly affect wildlife populations, but quantitative evidence is sparse. The collapse of socialism in Russia in 1991 caused a major socioeconomic shock, including a sharp increase in poverty. We analyzed population trends of 8 large mammals in Russia from 1981 to 2010 (i.e., before and after the collapse). We hypothesized that the collapse would first cause population declines, primarily due to overexploitation, and then population increases due to adaptation of wildlife to new environments following the collapse. The long-term Database of the Russian Federal Agency of Game Mammal Monitoring, consisting of up to 50,000 transects that are monitored annually, provided an exceptional data set for investigating these population trends. Three species showed strong declines in population growth rates in the decade following the collapse, while grey wolf (Canis lupus) increased by more than 150%. After 2000 some trends reversed. For example, roe deer (Capreolus spp.) abundance in 2010 was the highest of any period in our study. Likely reasons for the population declines in the 1990s include poaching and the erosion of wildlife protection enforcement. The rapid increase of the grey wolf populations is likely due to the cessation of governmental population control. In general, the widespread declines in wildlife populations after the collapse of the Soviet Union highlight the magnitude of the effects that socioeconomic shocks can have on wildlife populations and the possible need for special conservation efforts during such times.

The power and perils of animal models with urogenital anomalies: handle with care.

Congenital abnormalities of the urogenital tracts form a major part of clinical practice for paediatric urologists, but their knowledge of normal and abnormal development is often limited. Advances in understanding frequently come from studying experimental findings from animal models, however, most clinicians underestimate both the power and perils of extrapolating scientific knowledge from animals. In this review, the key issues that urologists need to understand in order to link animal studies to clinical practice are discussed. Urologists must avoid the traps of anthropomorphism (assuming humans are always the same as animal models) or anthropocentrism (assuming humans are too different from animal models). This review used two common disorders: hypospadias and undescended testes.

Polymorphisms of MAMLD1 gene in hypospadias.

PURPOSE: Mastermind-like domain containing 1 (MAMLD1) is a causative gene for the fetal development of male external genitalia. Almost 10% of patients with both severe and non-severe hypospadias exhibit mutations of MAMLD1. The aim of this work was to determine whether polymorphisms of MAMLD1 are a genetic risk factor for hypospadias. MATERIAL AND METHODS: This study included 150 hypospadias with a range of severities and 150 controls. Direct sequencing of the MAMLD1 coding exons and their flanking splice sites was performed. In silico secondary and tertiary structure prediction and accessibility of changed amino acids were evaluated using JPred, Netsurf and PHYRE software. Functional studies of the transactivation of haplotypes on Hes3 promoter were performed in vitro using cDNAs of missense variants of MAMLD1. RESULTS: The p.P286S polymorphism was identified in 17/150 patients and 12/150 controls (11.3% vs. 8.0%, p = 0.32). The p.N589S polymorphism was identified in 22/150 patients and 12/150 controls (14.6% vs. 8.0%, p = 0.068). The double polymorphism (S-S haplotype) was present in 16/150 patients and 6/150 controls (10.6% vs. 4.0%, p = 0.044, OR = 2.87, CI from 1.09 to 7.55). The association of polymorphisms consistently revealed a modification in the structure prediction or amino acid accessibility in all three in silico models. The P286S, N589S and P286S + N589S proteins did not exhibit reduced transactivating activity on Hes3 promoter. CONCLUSION: Polymorphisms of MAMLD1 gene are frequent in patients with hypospadias. Although no change in transactivation was noted on Hes3 promoter, the in silico studies and the significantly increased incidence of the S-S haplotype in hypospadiac patients raise the hypothesis of a particular susceptibility conferred by these variants.

Hypospadias: interactions between environment and genetics.

Hypospadias is one of the most common congenital malformations. It is considered to be a mild form of the 46,XY disorders of sex development (DSD), but its precise etiology remains to be elucidated. Compromised androgen synthesis or effects can cause this frequent malformation, although the mutational analyses of the genes involved in androgen actions have identified abnormalities in only a very small portion of patients. The overwhelming majority of cases remain unexplained and hypospadias may be a highly heterogeneous condition subject to multiple genetic and environmental factors. We here review the recent advances in this field and discuss the potential interactions between the environment and genetics.

46, XY female with cloacal exstrophy and masculinization at puberty.

Cloacal exstrophy of the bladder is a rare complex disorder occurring 1 in 400,000 live births and associated with cryptorchidism, vesicoureteral reflux, severe phallic inadequacy, omphalocele with short-gut syndrome, exstrophied bladder separated by exstrophied ileocecal segment, and pubic symphyseal diastasis. The association of undescended and ectopic testis with cloacal exstrophy is not uncommon, but the presence of an unexpected persistent ectopic testis at the time of puberty is quite unusual. We report the case of a 17-year-old girl with a history of 46, XY cloacal exstrophy and gender reassignment presenting with an ectopic testis of unclear location. We then review controversial literature surrounding gender assignment in these patients.

The effect of oestrogen and testosterone on the urethral seam of the developing male mouse genital tubercle.

OBJECTIVES: To describe the effects of exogenous oestrogens and androgens on urethral formation in the mouse, as the development of the mouse and human urethra have significant similarities, and understanding normal male urethral development may help to identify the causes of abnormal development, e.g. hypospadias. MATERIALS AND METHODS: Timed-pregnant C57/6 mice were exposed to synthetic oestrogens and androgens. The morphology of the genital tubercles was examined histologically and with three-dimensional computer reconstruction. Specific attention was focused on the developing urethral seam. RESULTS: Microscopic serial analysis confirmed the presence of an arrest in seam formation in about half of oestrogen-treated male fetuses. In contrast, there was acceleration of urethral fold fusion and a longer urethral tube in those treated with androgens. Oestrogen-treated fetuses had a thin periurethral spongiosa, in contrast to androgen-treated fetuses which developed a thicker periurethral spongiosa. The effect of oestrogens on seam area formation did not depend on the dose, but in contrast, in the androgen-treated fetuses it was. CONCLUSION: Oestrogens and androgens have a direct effect on the fusion of the urethral fold that leads to seam formation. Normal urethral development depends on the delicate balance of these complementary hormones.

Neuroanatomy of the male urethra and perineum.

OBJECTIVE: To describe the topography of the perineal nerves from their pudendal origin to their course into the male genitalia, with specific attention on the course of the perineal nerve along the ventral penis, including branches into bulbospongiosus muscle and corpus spongiosum. MATERIALS AND METHODS: The study comprised 18 normal human fetal penile specimens at 17.5-38 weeks of gestation (determined by fetal heel-to-toe length). Specimens were fixed in formalin, embedded in paraffin wax and serially sectioned at 6 micro m. The penile specimens contained the whole penis from the glans to the crural bodies, beneath the pubic arch and the perineum up to the anal verge. Immunocytochemistry was assessed on selected sections with antibodies against the neuronal markers S-100 and nitric oxide synthase (nNOS). Three-dimensional computer reconstruction of serial sections allowed an in-depth analysis of the neuroanatomy of the fetal penis, perineum and surrounding structures. RESULTS: After the pudendal nerve leaves the pudendal canal it gives rise to the perineal nerve branches in the ischiorectal fossa. Perineal nerves travel alongside the ischiocavernous and bulbospongiosus muscles and before reaching the latter, nerve branches course into the bulbospongiosus muscle. During its pathway within this muscle, fine nerve fibres course into the corpus spongiosum by piercing through the junction of the muscle. At the penoscrotal area, the perineal nerves give branches to the scrotum, funnelling into the interscrotal septum. Perineal nerves continue their pathway over the ventral side of penis covering the ventral surface of corpus spongiosum. Branches of the dorsal nerve of the penis at the junction of corpus cavernosum and corpus spongiosum assemble into a network with the perineal nerves. All perineal nerves from their main trunk at the ischiorectal fossa until their interaction with dorsal nerve of penis at the base of penis were nNOS negative. After the interaction with the dorsal nerve of penis, they become nNOS positive. CONCLUSION: Integrating neuroanatomical knowledge about the perineal nerves and their communication with the dorsal nerve of penis should facilitate a strategic approach to reconstructive procedures on the penis. Special care should be taken at the junction between the corpora cavernosa and spongiosa, where the dorsal nerve joins the perineal nerve, and at the proximal bulbospongiosus muscle, thereby protecting the fine nerves piercing into the cavernosa spongiosa.

The neuroanatomy of the human scrotum: surgical ramifications.

OBJECTIVE: To define the scrotal nerve origin and distribution with respect to surrounding structures in male human fetuses, by using neuronal-specific markers and three-dimensional (3D) imaging techniques, as the developmental neuroanatomy of the human scrotum has not been studied in detail and an explicit description of nerve derivation and distribution in the human scrotum is germane to genital reconstructive surgery. MATERIALS AND METHODS: Sixteen normal human fetal penile specimens at 17.5-38 weeks of gestation were studied. Specimens were fixed in formalin, embedded in paraffin wax, serially sectioned at 6 micro m and stained with the neuronal marker S-100. All of the specimens contained the whole penis and scrotum from glans to anal verge. The gestational age of the fetuses was determined by fetal heel-to-toe length. 3D-computer reconstruction of serial sections allowed a detailed analysis of the neuroanatomy of the fetal penis and scrotum. RESULTS: The nerves innervating the ventral side of the proximal penis and scrotum originated mainly from the perineal nerves arising from pudendal nerves. The nerves travelling along the ventral side of penis coalesced at the penoscrotal area to be directed into the interscrotal septum. At the penoscrotal junction, nerves on both sides of the ventral penis shifted to the interscrotal septum in a triangular fashion. The interscrotal septum was densely occupied by nerve fibres. Nerves were distributed horizontally to both hemiscrotal walls through this interscrotal septum. Both hemiscrota seem primarily to be innervated separately. CONCLUSION: The interscrotal septum has a dense innervation. Both hemiscrota were innervated mainly by horizontally distributed nerve fibres arising from the interscrotal septum. Any procedure violating the penoscrotal and interscrotal septal area may jeopardize scrotal innervation.

Fetal intervention for myelomeningocele: effect on postnatal bladder function.

PURPOSE: Myelomeningocele is the most common congenital malformation of the central nervous system noted on prenatal ultrasound. Due to its significant postnatal sequelae, treatment in utero could potentially have a profound impact on the newborn. Others have reported fetal surgical techniques for in utero repair of myelomeningocele and its potential benefits on motor and neurological function. We report our urodynamic findings in the newborn after in utero repair of spina bifida in an effort to characterize postnatal bladder function. MATERIALS AND METHODS: A retrospective review of the fetal surgery database at University of California San Francisco was performed identifying patients with a diagnosis of myelomeningocele. Prenatal surgical repair of myelomeningocele was considered if a normal karyotype was present, no other significant congenital anomalies were evident and gestational age was less than 24 weeks. The spinal defects were in the lumbar or lumbosacral region. All surgery was performed before 24 weeks of gestations. RESULTS: Fetal surgery to correct myelomeningocele was performed in 6 patients. All patients were born premature at 32 weeks of gestation or less. Videourodynamics performed at age 1 month in 4 patients indicated decreased bladder capacity for weight, increased detrusor storage pressures and significant post-void residual. Hydronephrosis was demonstrated in 4 patients on renal/bladder ultrasound, and moderate vesicoureteral reflux was seen in 3. CONCLUSIONS: Patients with spinal bifida treated in utero appear to have the same changes in urodynamic parameters and anatomical abnormalities in the urinary tract as other children with spinal defects who have undergone standard postnatal care. In utero treatment of spinal bifida may expose the newborn to the effects of prematurity. The long-term effects on bladder function in the fetus after in utero repair of myelomeningocele remain unknown. A randomized controlled trial is necessary to evaluate long-term bladder function as well as other outcome variables in this experimental approach to patients with myelomeningocele.

Hypospadias and endocrine disruption: is there a connection?

Hypospadias is one of the most common congenital anomalies in the United States, occurring in approximately 1 in 250 newborns or roughly 1 in 125 live male births. It is the result of arrested development of the urethra, foreskin, and ventral surface of the penis where the urethral opening may be anywhere along the shaft, within the scrotum, or in the perineum. The only treatment is surgery. Thus, prevention is imperative. To accomplish this, it is necessary to determine the etiology of hypospadias, the majority of which have been classified as idiopathic. In this paper we briefly describe the normal development of the male external genitalia and review the prevalence, etiology, risk factors, and epidemiology of hypospadias. The majority of hypospadias are believed to have a multifactorial etiology, although a small percentage do result from single gene mutations. Recent findings suggest that some hypospadias could be the result of disrupted gene expression. Discoveries about the antiandrogenic mechanisms of action of some contemporary-use chemicals have provided new knowledge about the organization and development of the urogenital system and may provide additional insight into the etiology of hypospadias and direction for prevention.

Malignancies arising in oncocytic schneiderian papillomas: a report of 2 cases and review of the literature.

Oncocytic schneiderian papillomas (OSPs) are uncommon benign neoplasms that arise from the sinonasal schneiderian epithelium. Malignancies arising in OSPs are rare, and, to our knowledge, only 14 such instances have been reported in the medical literature. We report 2 additional cases--a small cell carcinoma and a sinonasal undifferentiated carcinoma arising in OSPs and presenting synchronously with the benign neoplasm. The potential for malignant transformation in OSPs is small, but warrants that these papillomas be completely excised to exclude a coexisting carcinoma.

Urethral seam formation and hypospadias.

Knowledge of the formation of the normal male urethra may elucidate the etiology of hypospadias. We describe urethral formation in the mouse, show the similarities and relevance to human urethral development, and introduce the concept of the epithelial seam formation and remodeling during urethral formation. Three mechanisms may account for epithelial seam formation: (1) epithelial-mesenchymal transformation similar to that described in the fusion of the palatal shelves, (2) apoptosis, and/or (3) tissue remodeling via cellular migration. Urethral development in the embryonic mouse (14-21 days of gestation) was compared with urethral formation in embryonic human specimens (8-16 weeks of gestation) by using histology, immunohistochemistry, and three-dimensional reconstruction. The urethra forms by fusion of the epithelial edges of the urethral folds, giving a midline epithelial seam. The epithelial seam is remodeled via cellular migration into a centrally located urethra and ventrally displaced remnant of epithelial cells. The epithelial seam is remodeled by narrowing approximately at its midpoint, with subsequent epithelial migration into the urethra or penile skin. The epithelial cells are replaced by mesenchymal cells. This remodeling seam displays a narrow band (approximately 30 microns wide) of apoptotic activity corresponding to the mesenchymal cells and not to epithelial cells. No evidence was seen of the co-expression of cytokeratin and mesenchymal markers (actin or vimentin). Urethral seam formation occurs in both the mouse and the human. Our data in the mouse support the hypothesis that seam transformation occurs via cellular migration and not by epithelial mesenchymal transformation or epithelial apoptosis. We postulate that disruption of epithelial fusion remodeling, and cellular migration leads to hypospadias.

Magnetic resonance imaging in the evaluation of congenital anomalies of the external genitalia.

OBJECTIVES: To assess the value of magnetic resonance imaging (MRI) in the anatomic evaluation and management planning of complex congenital genitourinary anomalies. METHODS: Multiplanar T(1) and T(2)-weighted MR images were obtained in 6 pediatric patients with congenital genitourinary anomalies, including aphallia, diphallia, ectopic scrotum, and epispadias. The imaging studies were read by experienced radiologists and discussed with the urologic surgeons in a multidisciplinary conference. RESULTS: Each congenital anomaly was demonstrated in detail by MRI. The MR images of penile agenesis showed hypoplastic corpora cavernosa and a vestigial bulb. In patients with penile duplication, MRI was able to delineate the course of each corporal body and the varying degree of thickness of the tunica albuginea. For the patient with scrotal ectopia, detailed MR images excluded both the possibility of urethral and corporal duplications and the presence of viable testes in the ectopic scrotum. In the case of epispadias, MRI illustrated the precise spatial relationship between the erectile bodies and urethra. Additionally, MRI identified related aberrant pelvic organs and provided images of the external genital structures. CONCLUSIONS: MRI, by rendering excellent anatomic interpretation of complex genital anomalies and associated abnormal pelvic tissues, assists surgeons in conceptualizing the anomalous structures and contributes to their formulation of management approaches.