Hypospadias

Hypospadias management

1. Principles of management of hypospadias PRESENTER: DR ROBERT KYALO MBALUKA PGY3 UNIVERSITY OF NAIROBI 25th MAY 2021 PLASTIC, RECONSTRUCTIVE & SURGERY

2. OUTLINE Introduction History Incidence Embryology Etiology Anatomy of the defect Pathophysiology Classification Management Complications References

3. introduction A developmental anomaly characterized by a urethral meatus that opens onto the ventral surface of the penis, proximal to the end of the glans , which, in this condition, is splayed open 1 The meatus may be located anywhere along the shaft of the penis from the glans to the perineum 1. van der Horst, H. J., & de Wall, L. L. (2017). Hypospadias, all there is to know. European journal of pediatrics, 176(4), 435–441. https://doi.org/10.1007/s00431-017-2864-5

4. history 1 st & 2 nd Centuries – Alexandrian surgeons Heliodorus and Antyllus - First description of hypospadias and its operative correction 2,3 - Amputation 129-ca - 199 AD – Galen – One of the first descriptions 1511 - 1568 - Portuguese Amatus Lusitanus - First to carve a tunnel between the glans and the ectopical meatus King Henry of France - Chordee; corrective procedure by royal surgeon Jean Fernel 18 th Century - Morgagni questioned an association between hypospadias and infertility 2. Rogers DO. History of external genital surgery. In: Horton CE editor . Plastic and Reconstructive Surgery of the Genital Area . Boston : Little, Brown; 1973. p. 3–47. 3. Horton CE, Devine CJ, Baran N. Pictorial history of hypospadias repair techniques. In Horton CE editor. Plastic and Reconstructive Surgery of the Genital Area. Boston: Little, Brown; 1973 . p . 237–43 .

5. History 1,2 Early 19th century - Mettauer and Bush, described using a trocar to establish a channel from the meatus to the glans 1830’s - Dieffenbach also described a similar technique 1861 - Bouisson proposed a scrotal skin flap to create the inferior wall of the missing urethral segment 1869 – Thiersch -Repair using tubularized lateral skin flaps that formed the neourethra 1 . Rogers DO. History of external genital surgery. In: Horton CE editor . Plastic and Reconstructive Surgery of the Genital Area . Boston : Little, Brown; 1973. p. 3–47. 2. Horton CE, Devine CJ, Baran N. Pictorial history of hypospadias repair techniques. In Horton CE editor. Plastic and Reconstructive Surgery of the Genital Area. Boston: Little, Brown; 1973 . p . 237–43 .

6. introduction The classic triad of hypospadias; Dorsally hooded foreskin Proximal ventral urethral meatus Ventral penile curvature Other abnormal findings include; Downward glans tilt Deviation of the median penile raphe Scrotal encroachment onto the penile shaft Midline scrotal cleft Penoscrotal transposition 1.

7. history Thiersch -Repair using tubularized lateral skin flaps that formed the neourethra

8. history 1874 - Theophile Anger – Successfully used technique described in 1869 by Thiersch. This report initiated the modern era of hypospadias surgery characterized by the use of local skin flaps 1 1880 - Duplay soon described his two-stage technique 2 In the first stage, the chordee was released I n the second stage , a ventral midline strip of skin was covered by closure of the lateral penile skin flaps in the midline 1897 - Nove‐Josserand – First to describe free skin grafts to create a neourethra in scrotal hypospadias 1. Bachus LH, de Felice CA. Hypospadias, then and now. Plast Reconstr Surg 1960;25:146–60 . 2. Horton CE, Devine CJ, Baran N. Pictorial history of hypospadias repair techniques. In Horton CE editor. Plastic and Reconstructive Surgery of the Genital Area. Boston: Little, Brown; 1973 . p . 237–43.

9. history 1913 , Edmonds - Transfer of preputial skin to the ventrum of the penis at the time of release of chordee. At a second stage, the Duplay tube was created to complete the urethral closure 1932 – Matthieu – Used a flap from proximal skin with parallel sutured lines 1941 – Nesbitt – Treated congenital curvature using fundoplication of the tunica albuginea 1941 – Humby - A one-stage approach using the full thickness of the foreskin

10. history 1941 – Humby – Used buccal mucosa from the lip for urethral reconstruction 1947 – Memmelaar - Used bladder mucosa as a free graft technique in a one-stage repair Early 1950’s - Byars popularized this two-stage technique used by Edmonds in 1913 Early 1950’s - Browne – ‘Buried strip’ technique 1955 - Marshall and Spellman used bladder mucosa in a two-stage technique In China - Urologists had good success with a primary repair using bladder mucosa

11. history Sir Denis Browne’s ‘ buried strip’ technique

12. history Late 1950’s – Cecil and Culp - Buried the penis in the scrotum to obtain skin coverage Smith further improved the outcomes by denuding the epithelium of one of the lateral skin flaps to give a ‘ pantsover- vest ’ closure to reduce the risk of fistula formation 1960’s – Fuqua popularized a two stage preputial transfer procedure developed by Belt.

13. history Cecil and Culp technique

14. history 1965 - Mustardé – Used a large flap of perimeatal skin combined with a ‘V’ incision of the glans 1981 - Duckett - Meatal advancement and glanuloplasty (MAGPI) procedure 1984 – Koyanagi – Described a technique for the more complex scrotal cases 1994 – Snodgrass – Described an incision of the tubularized urethral plate

15. history Over the next 20 years - Free grafts, including saphenous vein, ureter, and appendix McCormack used a free, full-thickness skin graft in a two-stage repair Devine & Horton later popularized this free preputial graft technique with very good results Improvement in preputial and meatal-based vascularized flaps over the last 30 to 40 years have greatly advanced hypospadias repair The single stage repair - Mathieu , Barcat, Mustardé, Broadbent, Hodgson, Horton and Devine, Standoli, and Duckett

16. Incidence - Global Common congenital malformation 0.8 and 8.2 per 1,000 live male births 1 Wide variation probably represents some geographic and racial differences Of most significance is the exclusion of the more minor degrees of hypospadias If all degrees of hypospadias, even the most minor, are included, then the incidence is probably 1 in 125 live male births Sweet RA, Schrott HG, Kurland R, et al. Study of the incidence of hypospadias in Rochester, Minnesota, 1940–1970, and a case control comparison of possible etiologic factors. Mayo Clin Proc 1974;49:52–8

17. incidence USA – 1 in every 250 male births 1 > 6,000 boys are born with hypospadias each year in the USA Denmark, France, and Italy - Prevalence of 0.3% to 0.45% of male births Mai CT, Isenberg J, Langlois PH, Alverson CJ, Gilboa SM, Rickard R, Canfield M, Anjohrin SB, Lupo PJ, Jackson DR, Stallings EB, Scheuerle AE, Kirby RS for the National Birth Defects Prevention Network. Brief report, Population-based birth defects data in the United States, 2008 to 2012: Presentation of state-specific data and descriptive brief on variability of prevalence. Birth Def Res (Part A). 2015; 103:972-994.

18. incidence Race R elated Demographics Whites > Blacks More common in Jewish and Italian descendants Genetic component in certain families Familial rate standing at 7% Recurrence risk is approximately 13 times greater in first-degree relatives (brothers , fathers , offspring) 1 Risk in same-sex twins - 50% 1 Schnack TH, Zdravkovic S, Myrup C, et al. Familial aggregation of hypospadias : a cohort study. Am J Epidemiol 2008;167:251–6 .

19. Incidence - Local 1 . Agot GN, Mweu MM, Wang'ombe JK. Prevalence of major external structural birth defects in Kiambu County, Kenya, 2014-2018. Pan Afr Med J. 2020 Oct 28;37:187. doi: 10.11604/pamj.2020.37.187.26289. PMID: 33447342; PMCID: PMC7778172.

20. Incidence - local 1 . Victor K. Wu, Dan Poenaru , Marten J. Poley , Burden of Surgical Congenital Anomalies in Kenya: A Population-Based Study, Journal of Tropical Pediatrics, Volume 59, Issue 3, June 2013, Pages 195–202,

21. Embryology of penile development 4 th week - Proliferating mesenchyme produces a genital tubercle Located at the cranial end of the cloacal membrane Labioscrotal swellings and urogenital folds develop The external genital anlage - Initially indifferent - Develops the female phenotype unless exposed to androgens (8-12 weeks )

22. Embryology of penile development Masculinization of the indifferent external genitalia - Testosterone Mediates key steps in penis formation: Elongation of the genital tubercle and Fusion of urethral folds Urethral plate - Develops as an extension of endoderm from the cloaca along the ventral midline of the genital tubercle Urethral folds – Fuse ventrally to form the spongy urethra Surface ectoderm - Fuses in the median plane of the penis, forming the penile raphe and enclosing the spongy urethra within the penis

23. Embryology of penile development 12th week - Ingrowth of ectoderm occurs at the periphery of the glans penis Breaks down - Forms the prepuce (foreskin ) Corpora cavernosa & corpus spongiosum of the penis develop from mesenchyme in the phallus (forms penis ) Labioscrotal swellings - Fuse to form the scrotum Line of fusion of the labioscrotal folds - Scrotal raphe .

24. Embryology of penile development Proliferating mesenchyme - Urethral folds & Urethral groove Fusion of the urethral folds - Proximally to distally at least to the glans 2 theories are proposed for glanular urethra development: - Ectodermal ingrowth cannulating the glans to the urethral plate - Urethral plate tubularization to the tip of the glans Penis initially exhibits ventral curvature during formation Can persist in hypospadias when normal development arrests

25. Embryology of penile development

26. Embryology of penile development 1 . https://d1j63owfs0b5j3.cloudfront.net/tutorial/finalImage/1422-1497959673128.gif

27. Etiology 1,2 E xact etiology of hypospadias is unknown B elieved to include; Genetic Endocrine and Environmental factors van der Zanden L. F. M., van Rooij I. A. L. M., Feitz W. F. J., Franke B., Knoers N. V. A. M., Roeleveld N. Aetiology of hypospadias: a systematic review of genes and environment. Human Reproduction Update. 2012;18(3):260–283. doi: 10.1093/ humupd /dms002 Kalfa N., Philibert P., Sultan C. Is hypospadias a genetic, endocrine or environmental disease, or still an unexplained malformation? International Journal of Andrology . 2009;32(3):187–197. doi: 10.1111/j.1365-2605.2008.00899.x.

28. Etiology - Endocrine A defect in the androgen stimulation of the developing penis , which precludes complete formation of the urethra and its surrounding structures, is the ultimate cause of hypospadias Causes - Deficient androgen production by the testes and placenta Failure of testosterone to convert to dihydrotestosterone by 5α- reductase Deficient androgen receptors in the penis Various disorders of sexual differentiation ( DSD) can cause deficiencies at any point along the androgen-stimulation axis

29. Etiology - Endocrine Endocrine theory A decrease in available androgens Inability to use available androgen appropriately 66 % of boys with mild hypospadias and 40% with severe hypospadias were found to have a defect in testicular testosterone biosynthesis 1 Mutations in the 5-alpha reductase enzyme Nearly 10% of boys with isolated hypospadias had at least one affected allele with a 5-alpha reductase mutation 2 Qualitative and quantitative androgen receptor deficits Aaronson IA, Cakmak MA, Key LL. Defects of the testosterone biosynthetic pathway in boys with hypospadias. J Urol. 1997 May;157(5):1884-8. PMID: 9112555 . Silver RI, Russell DW. 5alpha-reductase type 2 mutations are present in some boys with isolated hypospadias. J Urol. 1999 Sep;162(3 Pt 2):1142-5. doi: 10.1097/00005392-199909000-00064. PMID: 10458450.

30. Etiology - Endocrine Endocrine theory A higher incidence of hypospadias in winter conceptions has also been proposed Males born through in-vitro fertilization (IVF ) – 5 fold increase in incidence This may reflect maternal exposure to progesterone, which is commonly administered in IVF protocols Progesterone is a substrate for 5-alpha reductase and acts as a competitive inhibitor of the T-to-DHT conversion Underlying endocrinopathies or fetal endocrine abnormalities Other contributors of infertility

31. etiology Genetic factors Higher incidence in first-degree relatives of hypospadiac patients. Risk of hypospadias in a second male sibling was 12 % If index child and father were affected, the risk for a second sibling increased to 26 % If index child and a second-degree relative (rather than the father) were affected, the risk of the sibling being affected was only 19% 1 M ultifactorial mode of inheritance These families having a higher than average number of influential genes for creation of hypospadias 1 A combination of the endocrine, environmental, and genetic factors likely determines the potential for developing the hypospadias complex in any one individual. 1. Wang MH, Baskin LS. Endocrine disruptors, genital development , and hypospadias. J Androl . 2008;29:499–505.

32. etiology Genetic factors Eightfold increase in incidence of hypospadias among monozygotic twins as compared with singletons ?Demand of two fetuses for human chorionic gonadotropin (HCG) produced by a single placenta, with an inadequate supply during critical periods of urethral development

33. etiology Environmental factors Endocrine disruption by environmental agents Estrogens Environmental substances with significant estrogenic activity are ingested as pesticides on fruits and vegetables, endogenous plant estrogens, in milk from lactating pregnant dairy cows, and in pharmaceuticals such as phthalates The association of hypospadias with increasing parity, increasing maternal age, and low birth weight noted in some studies may reflect a lifelong exposure to environmental disruptors and a possible cumulative effect 1 1. Raghavan R, Romano ME, Karagas MR, Penna FJ. Pharmacologic and Environmental Endocrine Disruptors in the Pathogenesis of Hypospadias: a Review. Curr Environ Health Rep. 2018 Dec;5(4):499-511. doi: 10.1007/s40572-018-0214-z. PMID: 30578470.

34. etiology Environmental factors Drugs - Anti‐epileptic Valproate 1 Anti‐histamine L oratadine 1 1. Carmichael SL, Shaw GM, Lammer EJ. Environmental and genetic contributors to hypospadias: A review of the epidemiologic evidence. Birth Defects Research and Clinical Molecular Teratology. 2012;94(7):499-510

35. etiology Combination theory A growing body of evidence suggests that the development of hypospadias has a two-hit etiology involving a genetic predisposition coupled with fetal exposure to an environmental disruptor

36. Etiology - Syndromic Suspected with development delay, dysmorphic facies , and/or anorectal malformation . Examples include: Smith- Lemli - Opitz syndrome —results from an autosomal recessive mutation of the DHCR7 gene on chromosome 11q13 coding for 7-dehydrocholesterol reductase . Affected individuals have mental retardation, facial dysmorphism, microcephaly, and syndactyly. WAGR syndrome (Wilms tumor, aniridia, genital anomalies, mental retardation ) —results from a deletion in chromosome 11p13 . G syndrome (Opitz G/BBB syndrome ) —occurs from X-linked mutations in the midline-1 gene or autosomal dominant deletions in chromosome 22q11. The resultant phenotype includes hypertelorism , tracheoesophageal defects, cleft lip/palate, and mild mental retardation .

37. Etiology - syndromic Wolf- Hirschhorn syndrome —derives from deletions in chromosome 4p resulting in mental retardation , seizures, abnormal facies , and midline defects . 13q deletion syndrome —characterized by mental retardation , facial dysmorphia, imperforate anus , and hypospadias with penoscrotal transposition . Hand-foot-uterus syndrome —an autosomal dominant condition caused by mutations in the HOXA13 gene on chromosome 7p14-15 , resulting in bilateral thumb and great toe hypoplasia.

38. anatomy

39. pathophysiology C ongenital defect Occurs between 8-20 weeks - Embryologic development of the urethra External genital structures are identical in males and females until 8 weeks Masculine phenotype in males - Testosterone & dihydrotestosterone The open urethral groove extends from its base to the level of the corona as the phallus grows

40. pathophysiology Classic theory Urethral folds coalesce in the midline from base to tip - Tubularized penile urethra and median scrotal raphe This accounts for the posterior and middle urethra The anterior/glanular urethra - Develops in a proximal direction Ectodermal core forms at the tip of the glans penis Canalizes Joins with the more proximal urethra at the level of the corona The higher incidence of subcoronal hypospadias supports the vulnerable final step in this theory of development

41. pathophysiology Baskin theory The urethral folds fuse to form a seam of epithelium This is then transformed into mesenchyme and subsequently canalizes by apoptosis or programmed cell resorption. [ 3] Similarly , this seam theoretically also develops at the glanular level, and the endoderm differentiates to ectoderm with subsequent canalization by apoptosis

42. pathophysiology

43. pathophysiology Prepuce Forms as a ridge of skin from the corona that grows circumferentially, fusing with the glans Failure of fusion of the urethral folds in hypospadias impedes this process, and a dorsal hooded prepuce results Megameatus intact prepuce (MIP) variant

44. pathophysiology Megameatus intact prepuce (MIP) variant

45. pathophysiology Chordee - In severe forms Result from a growth disparity between the normal dorsal tissue of the corporal bodies and the attenuated ventral urethra and associated tissues The abortive spongiosal tissue and fascia distal to the urethral meatus may form a tethering fibrous band that contributes to the ventral curvature This can range from mild to very severe (90º angle ) The location of the abnormal urethral meatus classifies the hypospadias

46. classification Several classifications have been described Most used - One proposed by Barcat and modified by Duckett Describes the location of the meatus after correction of any associated chordee Descriptive locations in this classification include the following : Anterior (glanular and subcoronal ) Middle (distal penile, midshaft) Posterior (proximal penile, penoscrotal , scrotal, and perineal ) The location is anterior in 50% of cases, middle in 20%, and posterior in 30%; the subcoronal position is the most common overall .

47. 1. v

48. Indications of operation Functional indications: Proximally located meatus Ventrally deflected urinary stream Meatal stenosis Curved penis

49. Indications of operation Cosmetic indications Abnormally located meatus Cleft glans Rotated penis with abnormal cutaneous raphe Preputial hood Penoscrotal transposition Split scrotum

50. Preoperative consideration HCG 250-500 U SC twice a week for 3 weeks Penile enlargement Advances meatus distally to decrease hypospadias severity • IM testosterone enanthate – 2mg/kg/dose given for a total of 2 or 3 doses before hypospadias repair Increases penile length and girth • Testosterone propionate cream – 2% three times daily for 3 weeks Induces neovascularization

51. Management – imaging studies Upper urinary tract anomalies - Rarely associated Other associated findings are more common - Enlarged prostatic utricle Low-grade vesicoureteral reflux Retrograde urethrography in the setting of proximal hypospadias 1 1. Hester AG, Kogan SJ. The prostatic utricle: An under-recognized condition resulting in significant morbidity in boys with both hypospadias and normal external genitalia. J Pediatr Urol. 2017 Oct;13(5):492.e1-492.e5. doi: 10.1016/j.jpurol.2017.01.019. Epub 2017 Mar 1. PMID: 28319024.

52. Management – age at repair Considerations 1 - Milestones of development Size of penis Childs response to surgery Quick healing & less scarring Anesthesia risk Toilet training Good tolerance to surgery and anesthesia - Age of 6 months The penile length at 1 year is on an average 0.8 cm less than at preschool age The child is well aware about his genitalia and toilet training by the age of 18 months 1. Manzoni G, Bracka A, Palminteri E, Marrocco G. Hypospadias surgery: when, what and by whom? BJU Int. 2004 Nov;94(8):1188-95. doi: 10.1046/j.1464-410x.2004.05128.x. PMID: 15613162.

53. Management – age at repair Another opportunity - 3-4 years 3 Genital awareness - 18 months of age 18 months - Also the start of a difficult/uncooperative behavioural phase; ill-suited to hospitalization 3 years - Child becomes sufficiently mature to collaborate with his treatment Similar outcomes to year 1 of life repairs Most surgeons - 6 to 12 months old 1 The ‘optimal window’ recommended for repair was age 6 to 15 months 2 Psychological concerns Belman AB, Kass EJ. Hypospadias repair in children less than 1 year old . J Urol. 1982;128:1273–1274 . Schultz JR, Klykylo WM, Wacksman J. Timing of elective hypospadias repair in children. Pediatrics 1983;71:342–51. Manzoni G, Bracka A, Palminteri E, Marrocco G. Hypospadias surgery: when, what and by whom? BJU Int. 2004 Nov;94(8):1188-95. doi: 10.1046/j.1464-410x.2004.05128.x. PMID: 15613162.

54. Management – objectives of repair Orthoplasty - Complete straightening of the penis Meatoplasty - Locating the meatus at the tip of the glans Urethroplasty - Neourethra uniform in caliber, with a natural slit like configuration Glanuloplasty - Forming a symmetric, conically shaped glans Cosmesis - Completing a satisfactory cosmetic skin coverage Scrotoplasty - Create normal appearing scrotum

55. Management - orthoplasty Straightening Curvature - Difficult to judge Artificial erection - Injecting physiologic saline in the corpora at the time of operation allows determination of the exact degree of curvature 1 Pharmacologic erection - injection of the arterial vasodilator prostaglandin E1 Performed after degloving penile shaft skin 1. Gittes RF, McClaughlin AP. Injection technique to induce penile erection . Urology 1974;4:473–5 .

56. Management - Orthoplasty Straightening Causes - Ventral skin or subcutaneous tissue tethering; corrected with the release of the skin and dartos layer. 1,2 Causes - True fibrous chordee ; division of the urethral plate and excision of the fibrous tissue down to the tunica albuginea Corporal body disproportion - Caused by a true deficiency of ventral corporal development Correction - Releasing incision in the ventral tunica albuginea and inserting either a dermal or a tunica vaginalis patch to expand the deficient ventral surface 3 1. Gittes RF, McClaughlin AP. Injection technique to induce penile erection . Urology 1974;4:473–5 . 2. Devine CJ, Horton CE. Chordee without hypospadias. J Urol 1973;110:264–71. 3. 69. Braga LH, Pippi Salle JL, Dave S, et al. Outcome analysis of severe chordee correction using tunica vaginalis as a flap in boys with proximal hypospadias. J Urol 07;178:1693–7 .

57. Management - orthoplasty Small intestinal submucosa as an off-the-shelf substitute for the autologous grafts 1 Excise wedges of tunica albuginea dorsally with transverse closure to shorten this dorsal surface and straighten the penis – Nesbit technique Dorsal plication without excision of tunica albuginea - midline dorsally Corporal rotation dorsally with or without penile disassembly to correct severe chordee Axial rotation/Penile torsion - Releasing the dartos layer as far proximal as possible on the penile shaft . This allows the ventral shaft to rotate back to the midline and corrects the torsion. 1. Weiser AC, Franco I, Herz DB, et al. Single layered small intestinal submucosa is the repair of severe chordee and complicated hypospadias . J Urol 2003;170:1593–5 .

58. Management - orthoplasty Ventral corporeal lengthening : Ventral corporotomy with grafting Multiple corporotomy without grafting

59. Midline dorsal plication Dermal patch Nesbit technique

60. Management - Meatoplasty Locating the Meatus Glans-channeling and glans-splitting maneuvers Glanular and subcoronal variants (Distal hypospadias) Meatoplasty with or without dorsal advancement, Distal urethral mobilization and tubularization, Meatal-based flaps Proximal variants -Creating the neourethra with local vascularized skin flaps or free grafts allows positioning the urethra at the end of the penis. -Glans channeling or glans splitting

61. Management - Meatoplasty

62. Management - glanuloplasty Glans Shape Symmetric , conical Approximating the lateral glanular tissue in the midline ventrally over a meatoplasty or meatal advancement Approximation of well-developed glans wings to the midline over a neourethra in a split glans

63. Management - Urethroplasty Urethral Construction Neourethra - Local skin flaps, free grafts, or vascularized pedicle flaps Mobilized vascularized flaps of preputium - Reliable blood supply Used as patches or may be tubularized and used as bridges A watertight closure, Uniform in caliber, Appropriate size for the age of the child This closure helps avoid stricture and the formation of saccules, diverticula, and fistulas

64. Management - Cosmesis Draping the distal preputium over the ventral surface of the penis Transfers well-vascularized skin over the repair Not cosmetically appealing Splitting the dorsal skin in the midline longitudinally and advancing the flaps around on either side to meet in the ventral midline. Allows a midline ventral closure, which simulates the median raphe. Allows a subcoronal closure to the preputial skin circumferentially, which simulates the suture lines of a standard circumcision Advance lateral flaps of inner preputial skin from each side to the ventral midline of the penis at the time of glansplasty or closure of glans wings European countries - Prefer the appearance of a non-circumcised penis.

65. Operative approaches Versatility and experience with all options of surgical treatment are the keys to successful management . Due to the wide variation in the anatomic presentation of hypospadias, no single urethroplasty is applicable for every patient.

66. Operative approaches ANTERIOR VARIANTS Glanular variants -Meatal Advancement and Glansplasty (MAGPI) repair A stenotic meatus with good mobility of the urethra Shallow ventral glanular groove Wide-mouthed meatus - Meatal-based flap repair No chordee is present Mobile , well-vascularized skin exists proximal to the meatus Wide-mouthed meatus with a flat, shallow ventral groove - Tubularized incised plate urethroplasty (TIP) is a modification of the Thiersch–Duplay tubularization, which involves a deep longitudinal incision of the urethral plate in the midline. This allows the lateral skin flaps to be mobilized and closed in the midline without tension

67. Operative approaches Glanular variants -Meatal Advancement and Glansplasty (MAGPI) repair

68. Operative approaches - TIP Urethroplasty 1.

69. Operative approaches MIDDLE VARIANTS No significant chordee - TIP repair Onlay island flap technique -Penile shaft hypospadias In milder degrees of chordee - Corrected without dividing the urethral plate by incising tethering bands lateral to the urethral plate or by dorsal plication techniques. This allows either the Onlay island flap technique or TIP to be used This moves the meatus more proximal and requires treatment as described for proximal variants

70. Operative approaches Onlay island flap technique

71. Operative approaches PROXIMAL VARIANTS Scrotal and perineal forms of hypospadias - Significant chordee Requires division of the urethral plate - Gap Repair using staged procedures Coverage of the ventral penile shaft - Rotation of dorsal flaps to the ventrum Later, tubularization to form the neourethra Tubularized free graft Anastomosed to the native urethra proximally and extended to the end of the glans by a tunneling or splitting technique. Free grafts are full-thickness skin , bladder mucosa, or buccal mucosa

72. Operative approaches PROXIMAL VARIANTS Preputial skin preferred to extragenital skin Buccal mucosa next best Vascularized flaps are a more sound alternative to free grafts - Transverse inner preputial island flap Good preputial skin Reliable blood supply Does not rely on neovascularization for healing of the neourethra Alternatives - Shiny nonhair-bearing skin around the meatus The preputial vascularized tube graft can then be used to bridge the remaining distance to the end of the penis

73. Operative approaches PROXIMAL VARIANTS When the urethral plate is transected a one-stage urethroplasty can be accomplished by tubularized preputial flaps or the Koyanagi flap or a two-stage repair done with Byars flaps or preputial grafts

74. Operative approaches PROXIMAL VARIANTS Koyanagi flap • Proposed lines of incisions to create flap • The flap can be divided into two wings as shown or maintained in one piece with a central buttonhole to transpose it ventrally

75. Operative approaches PROXIMAL VARIANTS Byars flap

76. Technical Perspectives Magnification Optical magnification Standard operating loupes , ranging from 2.5× power to 4.5 ×

77. Technical Perspectives Sutures and Instruments Fine absorbable suture – Neourethra closure Polyglycolic or polyglactin material is preferred Permanent sutures Nylon or polypropylene C ontinuous stitch Pulled out 10 to 14 days after surgery Size 6-0 or 7-0 Microscope , 8-0 or 9-0 Skin closure - Fine absorbable sutures (6-0 or 7-0 )

78. Technical Perspectives Urinary Diversion Goal - Protect the neourethra ?Decrease the complication rate - Fistulas Perineal urethrostomy Suprapubic cystostomy Indwelling 6-8 French polymeric silicone (Silastic) tubes left through the repair and into the bladder allow drainage Facilitates the outpatient care Well tolerated by the babies Stents becoming plugged or dislodged are uncommon Left for 5 to 14 days , depending on the complexity of the repair

79. Technical Perspectives Urinary Diversion Stent that traverses the repair but is not indwelling in the bladder The patient is allowed to void, but the stent protects the repair In children who will not tolerate wearing a diaper 6 to 8 French Foley catheter - Simpler distal repairs Suprapubic cystostomy - Complex repairs Suprapubic drainage - Complex reoperations or Free graft repairs Diversion is not required for simpler distal procedures - MAGPI , meatal-based flap, or distal Duplay tubes Small fistula repairs - Without diversion

80. Technical Perspectives Dressings Enough gentle pressure on the penis to help with hemostasis and to decrease edema Not compromising the vascularity of the repair Transparent adhesive dressings A DuoDerm (ConvaTec, Skillman, NJ )

81. Technical Perspectives Analgesia Postoperative pain - Oral analgesics Bladder spasms by indwelling catheters - Propantheline bromide and opium suppositories or by oral oxybutynin Dorsal penile nerve block with Bupivacaine - Postoperative pain A caudal block

82. complications Bleeding Careful technique Point tip cautery Tourniquets or cutaneous infiltrations with dilute concentrations of epinephrine Postoperative bleeding - Mildly compressive dressings Subcutaneous hematomas - Generally do not need to be drained Infection Perioperative antibiotic prophylaxis Urinary suppression with oral antibiotics - Indwelling catheters that are open to drainage in the diaper

83. complications Devitalized Skin Flaps When the devascularized skin is over a well-vascularized bed of tissue, such as with a pedicle flap, primary healing generally occurs without sequelae. If the slough is over poorly vascularized tissue , such as a free graft, breakdown of the repair can occur

84. complications Fistulas Urethrocutaneous fistula - Most common Failure of healing at some point along the neourethral suture line Range in size Small fistulas - may close spontaneously Operative closure - in at least 6 months; complete tissue healing Small fistula – Fistulectomy followed by closure of the urethral epithelium with fine absorbable suture Prevent recurrence - Approximating layers of well-vascularized subcutaneous tissue over this closure

85. complications Fistulas Urinary diversion - Not necessary in small fistula repairs - Necessary with more complicated closures Larger fistulas require more complicated closures, with mobilization of tissue flaps or advancement of skin flaps to ensure an adequate amount of well-vascularized tissue for a multilayered closure

86. complications Strictures Anywhere along neourethra course Most common sites - Meatus and p roximal anastomosis Meatal narrowing - Gentle dilation Meatotomy Meatoplasty Especially when associated with a proximal fistula or neourethral diverticulum Proximal strictures - Dilation or Internal urethrotomy performed under vision Recurrences or long strictures - Open urethroplasty with excision of the stricture and a primary urethral anastomosis or patch graft urethroplasty

87. complications Diverticulum Classic bulging of the urethra ventrally with voiding Repair - Excision of the redundant neourethra with primary closure

88. complications Retrusive Meatus Retraction of the meatus from its original position at the tip of the glans to a proximal glanular or subcoronal position can occur with any repair Caused by - Failure of the glansplasty closure - Breakdown of devascularized distal neourethra Common when the MAGPI procedure is used in patients whose meatus is too proximal or when too much tension is placed on the glansplasty closure Correction - Repeat glansplasty - TIP - Meatal-based flap procedure

89. complications Persistent Chordee Inadequate release of chordee at the original procedure Treatment - Similar to the treatment of chordee without hypospadias Degloving of the penis Takedown of any ventral tethering tissue Artificial erection technique to guide dissection Dorsal plication Ventral excision with patching Division of the urethra

90. complications Sexual Function Fertility assessed by semen analysis in patients after hypospadias repair Higher rates of oligospermia reported Most in patients with associated anomalies such as cryptorchidism , chromosome abnormalities, varicoceles, or torsion Anatomically successful hypospadias repair, no associated anomalies - a high potential for fertility and an adequate sexual function are expected

91. complications Recurrence

92. Female hypospadias Female hypospadias 1 External genitalia - Solitary wide orifice in the vestibule Urethral orifice opened widely at the anterior vaginal wall Continuous leakage of urine from both the urethral and vaginal orifices These orifices could be individually catheterized A modification of Hendren's technique for the external urethral lengthening was employed Using “advancement flap” of anteromedial part of the thigh just lateral to the left labium majus for the reconstruction of anterolateral part of the distal vagina 1. Sarin , Y. K., & Kumar, P. (2019). Female Hypospadias-Need for Clarity in Definition and Management. Journal of Indian Association of Pediatric Surgeons, 24(2), 141–143. https://doi.org/10.4103/jiaps.JIAPS_69_18

93. references van der Horst, H. J., & de Wall, L. L. (2017). Hypospadias, all there is to know. European journal of pediatrics, 176(4), 435–441. https:// doi.org/10.1007/s00431-017-2864-5 Rogers DO. History of external genital surgery. In: Horton CE editor . Plastic and Reconstructive Surgery of the Genital Area . Boston : Little, Brown; 1973. p. 3–47. Horton CE, Devine CJ, Baran N. Pictorial history of hypospadias repair techniques. In Horton CE editor. Plastic and Reconstructive Surgery of the Genital Area. Boston : Little , Brown; 1973 . p . 237–43 4. Bachus LH, de Felice CA. Hypospadias, then and now. Plast Reconstr Surg 1960;25:146–60.

94. thank you