DOCSLIB.ORG

What Is New in Cryptorchidism and Hypospadias—A Critical Review On

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
What Is New in Cryptorchidism and Hypospadias—A Critical Review On Journal of Pediatric Surgery (2010) 45, 2074–2086 www.elsevier.com/locate/jpedsurg Review articles What is new in cryptorchidism and hypospadias—a critical review on the testicular dysgenesis hypothesis☆ Jorgen Thorup a,⁎, Robert McLachlan b, Dina Cortes c,d, Tamara R. Nation f, Adam Balic f, Bridget R. Southwell f, John M. Hutson e,f,g aDepartment of Pediatric Surgery, Faculty of Health Science, University of Copenhagen, Rigshospitalet, DK-2100 Copenhagen, Denmark bPrince Henrys Institute of Medical Research, Monash Medical Centre, 3168 Clayton, Australia cSection of Pediatric Endocrinology, Hvidovre Hospital, 2650 Copenhagen, Denmark dFaculty of Health Science, University of Copenhagen, DK-2100 Copenhagen, Denmark eDepartment of Pediatric Urology, Royal Children's Hospital, VIC 3052 Melbourne, Australia fDouglas Stephens Surgical Research Laboratory, Murdoch Children's Research Institute, VIC 3052 Melbourne, Australia gDepartment of Paediatrics, University of Melbourne, VIC 3010 Australia Received 15 February 2010; revised 18 July 2010; accepted 19 July 2010 Key words: Abstract It has been hypothesized that poor semen quality, testis cancer, undescended testis, and Cryptorchidism; hypospadias are symptoms of one underlying entity—the testicular dysgenesis syndrome—leading to Hypospadias; increasing male fertility impairment. Though testicular cancer has increased in many Western countries Fertility; during the past 40 years, hypospadias rates have not changed with certainty over the same period. Also, Testicular cancer; recent studies demonstrate that sperm output may have declined in certain areas of Europe but is Epidemiology probably not declining across the globe as indicated by American studies. However, at the same time, there is increasing recognition of male infertility related to obesity and smoking. There is no certain evidence that the rates of undescended testes have been increasing with time during the last 50 years. In more than 95% of the cases, hypospadias is not associated with cryptorchidism, suggesting major differences in pathogenesis. Placental abnormality may occasionally cause both cryptorchidism and hypospadias, as it is also the case in many other congenital malformations. The findings of early orchidopexy lowering the risk of both infertility and testicular cancer suggest that the abnormal location exposes the cryptorchid testis to infertility and malignant transformation, rather than there being a primary abnormality. Statistically, 5% of testicular cancers only are caused by cryptorchidism. These data point to the complexity of pathogenic and epidemiologic features of each component and the difficulties in ascribing them to a single unifying process, such as testicular dysgenesis syndrome, particularly when so little is known of the actual mechanisms of disease. © 2010 Elsevier Inc. All rights reserved. ☆ The rates of hypospadias, cryptorchidism, and testis This present review is based on lectures presented at the World cancer are thought to be increasing, whereas sperm Federation of Associations of Pediatric Surgeons' session at The International Surgical Week in Adelaide, Australia 2009. concentrations are decreasing [1]. Several epidemiologic ⁎ Corresponding author. Tel.: +45 3545 4868; fax: +45 3545 3888. studies have shown that these conditions are associated with E-mail address: [email protected] (J. Thorup). each other. Skakkebaek et al [2] have hypothesized that poor 0022-3468/$ – see front matter © 2010 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2010.07.030 Cryptorchidism and hypospadias 2075 semen quality, testis cancer, undescended testis, and hypospadias is a continuum depending on when during the hypospadias are symptoms of one underlying entity—the embryonic period the fusion fails. so-called testicular dysgenesis syndrome (TDS). They have The only hitherto well-established risk factor for suggested that an imbalance between estrogens and andro- hypospadias, when corrected for gestational age, is low gens during fetal life may be crucial. Initially, the estrogen birth weight [15-19]. In 16 of 18 monozygotic twins hypothesis argued that increasing of reproductive abnormal- discordant for hypospadias, the twin with the lowest birth ities in human males may be related to increased estrogen weight had hypospadias [15,20]. Because low birth weight exposure in utero [3]. This hypothesis has been expanded to and small-for- gestational age are associated with suboptimal include endocrine disruptors, which include environmental first trimester growth, the association with hypospadias may toxins that can disrupt the hormonal balance of the fetus and be related to early placental malfunction [21]. Accordingly, thereby disturb sexual differentiation either by an estrogenic hypospadias has been correlated with low weight of the or an antiandrogenic effect [4]. Data also indicate that placenta [15,22,23]. Abnormalities of the fetal-placental- estrogens can induce cryptorchidism and hypospadias by maternal interaction may also explain the finding of an suppressing androgen production or action. In addition, association with dystocia, as women who gave birth to boys estrogen can induce cryptorchidism by suppression of with hypospadias had a higher rate of weak contractions insulin-like factor-3 [1,5]. Various environmental chemicals during birth, induced deliveries, and caesarean sections [24]. are able to alter endogenous levels of androgens (certain Hypospadias has also been shown to be associated with phthalates) and estrogens (polychlorinated biphenyls, poly- preeclampsia later during pregnancy [18]. Interestingly, there halogenated hydrocarbons) [1,5,6], but so far, few studies is a higher incidence of hypospadias with increasing have shown any clinical impact [7]. Endocrine disruptors, maternal age, what may be a direct consequence of the age such as phthalates and bisphenol-A, have been manufactured or other factors, such as a higher frequency of in vitro for more than 50 years and are widely used in plastic bottles, fertilization in older women [25]. vinyl floors, food wraps, cosmetics, medical products, and toys, so if they had an effect, it should be seen in the 1.2. Genetics epidemiologic data. However, the data do not support this. The hypothesis of possible environmental estrogenic or Genetic mechanisms are indeed involved in the patho- antiandrogenic effect of reproductive abnormalities in human genesis of hypospadias. Familial clustering is seen in 4% to males is mainly based on animal studies [8-13]. 25% of cases [15,16,23,26-30]. The more severe the The term “syndrome” derives from its Greek roots and malformation of the index patient, the higher the recurrence means literally “run together.” as the features of the TDS risk for the next male sibling, ranging between 9% and 17% should do. However, as will be discussed in the following, [15-17,23,26]. The distribution of phenotype in sporadic and substantial data related to pathophysiology as well as familial cases differs. Minor hypospadias is more common in epidemiology do not concur TDS hypothesis. Except for a familial than sporadic cases, whereas severe variants are few individuals with abnormal 45X/46,XY karyotype and more commonly sporadic [15]. other disorders of sexual development, few patients display all features of the “syndrome,” but rather 1 or 2 features for which alternative etiologies and associations can be 1.3. Epidemiology proposed. In a syndrome, a particular set of multiple anomalies occurs repeatedly in a consistent pattern. The A study by Paulozzi [31] reviewed data from 29 complexity of pathogenic and epidemiologic features of each registries on 4 million births per year around the world and component of TDS pathology and the difficulties in ascribing revealed wide intercountry variation in rates of hypospa- them to a single unifying process indicate that this is likely dias. Given differences in registry methods, genetic varia- not a syndrome. In this review, we critically evaluate the tion, and other factors, the rates themselves were not present knowledge about the etiology and epidemiology of directly comparable; however, they showed changes within hypospadias and cryptorchidism, and speculate on alterna- systems in recent decades. The data suggested an increase tive ways to look at the conditions. in reported rates of hypospadias during the 1970s and 1980s in 2 US systems, Scandinavia, and Japan. Among other nations, increases were only seen in one Italian system and in the Israeli system. The absence of an 1. Hypospadias increase was perhaps most notable in Canada, where the society is similar to that of the United States. However, the 1.1. Pathogenesis data collected from the US Birth Defects Monitoring Program (BDMP) were not random and did not accurately Hypospadias is caused by failure of fusion of the urethral represent the US population well in terms of geography. folds, endodermal differentiation, and ectodermal ingrowth Among all systems showing an increase, rates tended to in gestational weeks 8 to 20 [14,15]. The severity of level off after 1985. We reviewed more detailed studies on 2076 J. Thorup et al. Table 1 The incidence rates of hypospadias in Denmark according to 4 different studies Reference Period Method Total no. Incidence of hypospadias (%) of boys Sorensen [32] 1910-1945 Patient files + follow-up 27 613 0.33 (at birth) Weidner et al [19] 1983-1992 National register 330 000 0.40 (discharge diagnosis) Boisen et al [33] 1997-2004 Prospective clinical
Load more

Recommended publications
  • Hypospadias by Ronald S
    Kapi‘olani Pediatric Urology Hypospadias By Ronald S. Sutherland, M.D., F.A.A.P., F.A.C.S. What is hypospadias? Defined as a congenital deformity where the urethral opening is located beneath the penis rather then the tip, hypospadias ranges from a mild to severe deformity. The more common form is the distal variety, with the opening toward the front of the penis, which can usually be repaired with pleasing cosmetic results. More severe forms may be associated with the opening located at the base of the penis or further back near the anus. Usually hypospadias is also present with downward curvature of the penis (chordee), and a flattening of the foreskin with a hood-like covering. Occasionally the scrotum is also malformed and appears higher around the penis. Hypospadias can be found in a variety of congenital syndromes, including those with cardiac, renal, and testicular anomalies. (see next page) What causes hypospadias? Hypospadias develops early in gestation and occurs for unknown reasons, although there is slight familial tendency. Recent evidence suggests that the incidence is increasing and may be linked to environmental and genetic disruptions during the period when genital development is particularly sensitive to sex-steroid hormone imbalances. Occasionally, hypospadias can be detected by prenatal ultrasound. No pre-natal treatment or intervention is available. Parents should not request a circumcision for their newborn son with hypospadias because the foreskin may be necessary to assist with surgical repair. Sometimes hypospadias is not recognized until after the circumcision is completed. These cases are usually mild forms that can be repaired without the use of foreskin.
    [Show full text]
  • Hypospadias Repair
    Patient and Family Education Hypospadias Repair What causes it? What is hypospadias? Incomplete development of the urethra causes Hypospadias is a birth defect of the penis. hypospadias. It can occur in families. The The urethral opening, the hole where the urine comes out, is not in the normal position. reason for it happening is usually not known. Instead of the tip, it is on the undersurface of Why is it important to recognize the penis. Boys with hypospadias are usually hypospadias? missing the underside of their foreskin so An abnormally placed urethral opening does that the foreskin forms a hood. For this not allow the urine to pass as it s hould. A reason, most boys born with boy with hypospadias urinates with a hypospadias are not circumcised. There is stream that is often directed downward often a bend or curve (called chordee) in the rather than out and away from the body. This penis when the boy has an erection. causes wet pants and shoes. This condition, Hypospadias may be mild, moderate, or when left uncorrected, may make future severe depending on how far back the opening sexual intercourse difficult, or is and how much curvature is present. The impossible. more severe forms of hypospadias are usually associated with worsening degrees of chordee. Can it be corrected? Yes, surgery can correct the problem. These operations are best done between 6 and 18 months of age. The repair is usually performed in one surgery. If the hypospadias is severe, it may be necessary to have more than one surgery. The Normal position for urethra surgery usually lasts 1-3 hours and the patient goes home the same day.
    [Show full text]
  • Battle of Sex Hormones in Genitalia Anomalies
    COMMENTARY COMMENTARY Battle of sex hormones in genitalia anomalies Liang Maa,b,1 exposures to antiandrogen or estrogenic com- aDivision of Dermatology, Department of Medicine, Washington University School of pounds can lead to a range of penile anoma- Medicine, St. Louis, MO 63110; and bDepartment of Developmental Biology, Washington lies similar to human CPAs (2). However, University School of Medicine, St. Louis, MO 63110 how and when EDCs may influence normal external genitalia development is not very clear. In PNAS, Zheng et al. (3) use a state- To cope with their transition to a terrestrial anomalies (CPAs), including ambiguous gen- of-the-art conditional androgen receptor lifestyle, vertebrates had to extensively modify italia, hypospadias, chordee, and micropenis, (AR) knockout mouse model to show that their reproductive organs to facilitate repro- represent one of the most common birth de- disruption of androgen signaling at different duction on land (1). The mammalian penis fects, second only to congenital cardiac de- developmental stages can produce the full represents such a pinnacle in mammalian fects. Hypospadias is an arrest in urethra spectrum of CPAs. The authors go on to evolution, which enabled internal fertilization development, such that the urethra opening show that androgen and estrogen signaling and successful land invasion. There are two is located anywhere along the ventral side of antagonize each other during neonatal mouse phases of external genitalia development in the penile shaft instead of at the glans penis. i genital development and identify a signaling mammals: ( ) an ambisexual stage, in which Chordee is the abnormal bending of the penis, molecule, Indian hedgehog (Ihh), as a novel male and female embryos undergo the same resulting from tethering of urethral epithelium AR target required for penile masculinization.
    [Show full text]
  • Evaluation of Additional Anomalies in Concomitance of Hypospadias And
    Türkiye Çocuk Hastalıkları Dergisi 222 Özgün Araştırma Original Article Turkish Journal of Pediatric Disease Evaluation of Additional Anomalies in Concomitance of Hypospadias and Undescended Testes Hipospadias ve İnmemiş Testis Birlikteliğinde Ek Anomali Sıklığının Değerlendirilmesi Ufuk ATES, Gülnur GÖLLÜ, Nil YAŞAM TAŞTEKİN, Anar QURBANOV, Günay EKBERLİ, Meltem BİNGÖL KOLOĞLU, Emin AYDIN YAĞMURLU, Tanju AKTUĞ, Hüseyin DİNDAR, Ahmet Murat ÇAKMAK Ankara University Medical School, Pediatric Surgery Department, Pediatric Urology Division, Ankara, Turkey ABSTRACT Objective: Hypospadias is a common genitourinary system (GUS) anomaly in boys occurring in 1 of 200 to 300 live births. Undescended testes is frequently detected among accompanying anomalies in cases with hypospadias. Especially in proximal hypospadias and bilateral cases, this association may indicate sexual differentiation disorders. The aim of the study was to evaluate the togetherness of additional anomalies in hypospadiac children with undescended testes. Material and Methods: Between 2007 and 2016, data of 392 children who underwent surgery for hypospadias were evaluated retrospectively. Urethral meatus was present at scrotal and penoscrotal in 65 cases (16.6%) and glanular, coronal, subcoronal and midpenile in 327 cases (83.4%). The cases were divided into two groups as those with both testes in the scrotum and those with undescended testes, and the anomalies were recorded. Results: The mean age of the children with proximal hypospadias was 21 months (6-240 months). Of the children with proximal hypospadias, 26 (40%) had undescended testes and 39 (60%) had testes in the scrotum. Undescended testes were detected bilaterally in 17 patients (65.4%) and unilaterally in nine patients (34.6%) in the undescended testes group.
    [Show full text]
  • Anne Tamar-Mattis, JD* Advocates for Informed Choice
    Report to the Inter-American Commission on Human Rights: Medical Treatment of People with Intersex Conditions as a Human Rights Violation Anne Tamar-Mattis, JD* Advocates for Informed Choice March 13, 2013 I. Introduction Americans born with intersex conditions, or variations of sex anatomy, face a wide range of violations to their sexual and reproductive rights, as well as the rights to bodily integrity and individual autonomy. Beginning in infancy and continuing throughout childhood, children with intersex conditions are subject to irreversible sex assignment and involuntary genital normalizing surgery, sterilization, medical display and photography of the genitals, and medical experimentation. In adulthood, and sometimes in childhood, people with intersex conditions may also be denied necessary medical treatment. Moreover, intersex individuals suffer life-long physical and emotional injury as a result of such treatment. These human rights violations often involve tremendous physical and psychological pain and arguably rise to the level of torture or cruel, inhuman, or degrading treatment (CIDT).† The treatment experienced by American intersex people is a violation of the American Convention on Human Rights. The Convention recognizes torture and cruel, inhuman, or degrading treatment (CIDT) as human rights abuses. Under Article V, “(1) Every person has the right to have his physical, mental, and moral integrity respected. (2) No one shall be subjected to torture or to cruel, inhuman, or degrading punishment or treatment.” §1-2. In order to demonstrate why specific medical practices are human rights abuses against intersex people, we have applied Article 16 of the Convention Against Torture (CAT), and interpretations by the European Court of Human Rights and the mandate of the Special Rapporteur on Torture (SRT).
    [Show full text]
  • Male Urethral Strictures and Their Management
    UCSF UC San Francisco Previously Published Works Title Male urethral strictures and their management. Permalink https://escholarship.org/uc/item/5wf4p510 Journal Nature reviews. Urology, 11(1) ISSN 1759-4812 Authors Hampson, Lindsay A McAninch, Jack W Breyer, Benjamin N Publication Date 2014 DOI 10.1038/nrurol.2013.275 Peer reviewed eScholarship.org Powered by the California Digital Library University of California REVIEWS Male urethral strictures and their management Lindsay A. Hampson, Jack W. McAninch and Benjamin N. Breyer Abstract | Male urethral stricture disease is prevalent and has a substantial impact on quality of life and health-care costs. Management of urethral strictures is complex and depends on the characteristics of the stricture. Data show that there is no difference between urethral dilation and internal urethrotomy in terms of long-term outcomes; success rates range widely from 8–80%, with long-term success rates of 20–30%. For both of these procedures, the risk of recurrence is greater for men with longer strictures, penile urethral strictures, multiple strictures, presence of infection, or history of prior procedures. Analysis has shown that repeated use of urethrotomy is not clinically effective or cost-effective in these patients. Long-term success rates are higher for surgical reconstruction with urethroplasty, with most studies showing success rates of 85–90%. Many techniques have been utilized for urethroplasty, depending on the location, length, and character of the stricture. Successful management of urethral
    [Show full text]
  • Medical Treatment of People with Intersex Conditions As Torture and Cruel, Inhuman, Or Degrading Treatment Or Punishment
    MEDICAL TREATMENT OF PEOPLE WITH INTERSEX CONDITIONS AS TORTURE AND CRUEL, INHUMAN, OR DEGRADING TREATMENT OR PUNISHMENT Anne Tamar-Mattis, JD* Abstract People worldwide born with intersex conditions, or variations of sex anatomy, face a wide range of violations to their sexual and reproductive rights, as well as the rights to bodily integrity and individual autonomy. Beginning in infancy, and continuing throughout childhood, children with intersex conditions are subject to irreversible sex assignment and involuntary genital normalizing surgery, sterilization, medical display and photography of the genitals, and medical experimentation. In adulthood, and sometimes in childhood, people with intersex conditions may also be denied necessary medical treatment. Moreover, intersex individuals suffer life-long physical and emotional injury as a result of such treatment. These human rights violations often involve tremendous physical and psychological pain and have been found to rise to the level of torture or cruel, inhumane, or degrading treatment. We offer recommendations for states working to address torture and inhuman treatment in medical settings. Introduction People worldwide born with intersex conditions, or variations of sex anatomy, face a wide range of violations to their sexual and reproductive rights, as well as the rights to bodily integrity and individual autonomy. Beginning in infancy and continuing throughout childhood, children with intersex conditions are subject to irreversible sex assignment and involuntary genital normalizing surgery, sterilization, medical display and photography of the genitals, and medical experimentation. In adulthood, and sometimes in childhood, people with intersex conditions may also be denied necessary medical treatment. Moreover, intersex individuals suffer life-long physical and emotional injury as a result of such treatment.
    [Show full text]
  • Ensuring the Rights of Children with Variations of Sex Characteristics in Denmark and Germany
    FIRST, DO NO HARM ENSURING THE RIGHTS OF CHILDREN WITH VARIATIONS OF SEX CHARACTERISTICS IN DENMARK AND GERMANY Amnesty International is a global movement of more than 7 million people who campaign for a world where human rights are enjoyed by all. Our vision is for every person to enjoy all the rights enshrined in the Universal Declaration of Human Rights and other international human rights standards. We are independent of any government, political ideology, economic interest or religion and are funded mainly by our membership and public donations. © Amnesty International 2017 Except where otherwise noted, content in this document is licensed under a Creative Commons Cover illustration: INTER*SHADES by Alex Jürgen*. Alex is an intersex artist living and working in (attribution, non-commercial, no derivatives, international 4.0) licence. Austria. Alex spells their name with a * to signify that intersex is not a recognized sex, and is currently https://creativecommons.org/licenses/by-nc-nd/4.0/legalcode involved in a court case to change their name and passport. For more information please visit the permissions page on our website: www.amnesty.org © Alex Jürgen Where material is attributed to a copyright owner other than Amnesty International this material is not subject to the Creative Commons licence. First published in 2017 by Amnesty International Ltd Peter Benenson House, 1 Easton Street London WC1X 0DW, UK Index: EUR 01/6086/2017 Original language: English amnesty.org CONTENTS 1. EXECUTIVE SUMMARY 7 1.1 METHODOLOGY 7 1.2 MEDICAL PRACTICES 7 1.3 THE IMPACT ON INDIVIDUALS 9 1.4 HUMAN RIGHTS AND GENDER STEREOTYPING 10 1.5 FURTHER HUMAN RIGHTS VIOLATIONS 10 1.6 PRINCIPAL RECOMMENDATIONS 11 2.
    [Show full text]
  • Clinical Features of Children with Multicystic Dysplastic Kidney
    Pediatrics International (2018) 60, 750–754 doi: 10.1111/ped.13612 Original Article Clinical features of children with multicystic dysplastic kidney Aslihan Kara,1 Metin Kaya Gurgoze,1 Mustafa Aydin2 and Zehra Pinar Koc3 Departments of 1Pediatric Nephrology, 2Neonatology and 3Nuclear Medicine, Firat University School of Medicine, Elazig, Turkey Abstract Background: To evaluate the clinical features of patients with multicystic dysplastic kidney (MCDK). Methods: The medical files of children diagnosed with MCDK between January 2008 and November 2015 were retrospectively reviewed. The demographic, clinical, laboratory and radiological data were evaluated. Results: Of 128 children with MCDK enrolled in the study, 82 (64.1%) were male, and 46 (35.9%) were female (P < 0.05). MCDK were located on left and right sides in 66 (51.6%) and 62 children (48.4%), respectively (P > 0.05). Antenatal diagnosis was present in 64 patients (50%). The mean age at diagnosis was 2.8 Æ 2.7 years (range, 0–8 years), and follow-up duration was 4.5 years. Fifteen patients (20.8%) had vesicoureteral reflux. Of these, four underwent endoscopic surgical correction. Other associated urological anomalies were ureteropelvic junction obstruction (n = 6), hypospadias (n = 1), and kidney stones (n = 1). On technetium-99 m dimercaptosuc- cinic acid scintigraphy, which was performed in all patients, no significant association between grade of reflux and presence of scarring was seen. Hypertension was diagnosed only in one child (0.8%) who required antihypertensive treatment. The prevalence of unilateral undescended testicle in children aged <1 year in the 82 male patients was 4.9%. Seventy-six patients (59.4%) developed compensatory hypertrophy in the contralateral kidney during a 1 year follow-up period.
    [Show full text]
  • Molecular Epidemiology of Hypospadias: Review of Genetic and Environmental Risk Factors
    © 2003 Wiley-Liss, Inc. Birth Defects Research (Part A) 67:825–836 (2003) Review Article Molecular Epidemiology of Hypospadias: Review of Genetic and Environmental Risk Factors Jeanne M. Manson* and Michael C. Carr Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania Received 16 September 2002; Accepted 21 May 2003 Hypospadias is one of the most common congenital anomalies in the United States, occurring in approximately 1 in 125 live male births. It is characterized by altered development of the urethra, foreskin, and ventral surface of the penis. In this review, the embryology, epidemiology, risk factors, genetic predisposition, and likely candidate genes for hypospadias are described. Recent reports have identified increases in the birth prevalence of mild and severe forms of hypospadias in the United States from the 1960s to the present. Studies in consanguineous families and small case series have identified allelic variants in genes controlling androgen action and metabolism that cause hypospadias, but the relevance of these findings to the general population is unknown. Concern has also focused on whether exposure to endocrine disrupting chemicals (EDC) with antiandrogenic activity is the cause of this increase. Hypospadias is believed to have a multifactorial etiology in which allelic variants in genes controlling androgen action and metabolism predispose individuals to develop this condition. When genetic susceptibility is combined with exposure to antiandrogenic agents, a threshold is surpassed, resulting in the manifestation of this birth defect. A clear role for exposure to antiandrogenic environmental chemicals has yet to be established in the etiology of hypospadias, although results from laboratory animal models indicate that a number of environmental chemicals could be implicated.
    [Show full text]
  • Monal Therapy for Scrotal Hypospadias with Severe Chordee, Micropenis, and Bifid Scrotum
    ISSN: 2621-2064 Open Access Journal CASE REPORT Multi-stage repair surgery in combination with hor- monal therapy for scrotal hypospadias with severe chordee, micropenis, and bifid scrotum Paulina Magdalena, Gede Wirya Kusuma Duarsa Abstract Proximal hypospadias includes proximal penile, penoscrotal, scrotal, and perineal types, which Urology Division, are a less frequent occurrence and correspond to 20% of total hypospadias. Surgical reconstruc- Department of General Surgery, tion is the only possible therapeutic option for hypospadias. The primary objectives of the recon- Sanglah Hospital/ Faculty of Medicine, University of Udayana, Denpasar, Bali struction are to create a vertically slit orthotopic meatus, straighten the penis and establish good Indonesia cosmetic results that include a conically shaped glans. Other essential aspects of the reconstruc- tion are to avoid shortening the penis and optimal skin coverage. This study reports a case with abnormal spraying during urination. Severe forms of hypospadias are typically accompanied by an abnormal ventral curvature of the penis (chordee). Surgical correction of both chordee and hypospadias is recommended. When adequately corrected, it does not cause long-term problems, and a natural appearance of the penis is usually restored. Keywords: Scrotal hypospadias, severe chordee, micropenis, bifid scrotum, cordectomy ortho- plasty, urethroplasty, scrotoplasty Introduction Hypospadias is a congenital anomaly, which affects different structures of the penis and, eventually, the scrotum and perineum. A wide range of types of hypospadias can be observed according to the original site of the opening of the urethral meatus and to other associated defor- mities. They are found in varying configurations that range from an aspect of ambiguous genitalia to a well-formed penis with a superficial defect.
    [Show full text]
  • Re-Thinking Genital Surgeries on Intersex Infants
    Re-Thinking Genital Surgeries on Intersex Infants M. Joycelyn Elders, M.D., M.S. 15th Surgeon General of the United States David Satcher, M.D., Ph.D., FAAFP, FACPM, FACP 16th Surgeon General of the United States Richard Carmona, M.D., M.P.H., FACS 17th Surgeon General of the United States June, 2017 On October 26, 2016, the 20th anniversary of Intersex Awareness Day, the U.S. State Department issued a statement recognizing that “intersex persons routinely face forced medical surgeries that are conducted at a young age without free or informed consent. These interventions jeopardize their physical integrity and ability to live free.”1 The U.S. government is one of many that have recently raised questions about infant genitoplasty, cosmetic genital surgery meant to make an infant’s genitals “match” the binary sex category they are assigned by adults entrusted with their care. Genitoplasty is often performed on infants with intersex traits, a condition known as DSD, or Disorders/Differences of Sex Development. Although well-intentioned—many parents and physicians believe it is more trying for individuals to live with atypical genitalia than to have it “corrected” early on—there is growing recognition that this belief is based on untested assumptions rather than medical research, and that cosmetic genital surgery performed on infants usually causes more harm than good. Fortunately, a consensus is emerging that concludes that children born with atypical genitalia should not have genitoplasty performed on them absent a need to ensure physical functioning. Government agencies in Germany, Switzerland, Australia, Chile, Argentina, and Malta, as well as human rights groups, including the World Health Organization, have examined this issue and found that these irreversible medical procedures, which are performed before individuals can articulate whether they wish to undergo such surgery, are not necessary to ensure healthy physical functioning, and that such surgery is not justified when performed on infants.
    [Show full text]