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 open1
⢠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), 435â441.
https://doi.org/10.1007/s00431-017-2864-5
4.
5. HISTORY
⢠1st & 2nd Centuries â Alexandrian surgeons Heliodorus and Antyllus - First description
of hypospadias and its operative correction2,3 - Amputation
⢠129-ca - 199 AD â Galen â 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
⢠18th 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. 3â47.
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.
6. HISTORY1,2
⢠Early 19th century - Mettauer and Bush, described using a trocar to establish a channel
from the meatus to the glans
⢠1830âs - Dieffenbach also described a similar technique
⢠1861 - Bouisson proposed a scrotal skin flap to create the inferior wall of the missing
urethral segment
⢠1869 â 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. 3â47.
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.
7. 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.
9. HISTORY
⢠1874 - Theophile Anger â Successfully used technique described in 1869 by
Thiersch. This report initiated the modern era of hypospadias surgery
characterized by the use of local skin flaps1
⢠1880 - Duplay soon described his two-stage technique2
In the first stage, the chordee was released
In the second stage, a ventral midline strip of skin was covered by closure of the lateral penile
skin flaps in the midline
⢠1897 - NoveâJosserand â 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;25:146â60.
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.
10. 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 â Matthieu â Used a flap from proximal skin with parallel sutured lines
⢠1941 â Nesbitt â Treated congenital curvature using fundoplication of the tunica
albuginea
⢠1941 â Humby - A one-stage approach using the full thickness of the foreskin
11. HISTORY
⢠1941 â Humby â Used buccal mucosa from the lip for urethral reconstruction
⢠1947 â Memmelaar - Used bladder mucosa as a free graft technique in a one-stage
repair
⢠Early 1950âs - Byars popularized this two-stage technique used by Edmonds in 1913
⢠Early 1950âs - Browne â âBuried stripâ 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
13. HISTORY
⢠Late 1950âs â 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 âpantsover- vestâ closure to reduce the risk of fistula
formation
⢠1960âs â Fuqua popularized a two stage preputial transfer procedure developed
by Belt.
15. HISTORY
⢠1965 - MustardĂŠ â Used a large flap of perimeatal skin combined with a âVâ
incision of the glans
⢠1981 - Duckett - Meatal advancement and glanuloplasty (MAGPI) procedure
⢠1984 â Koyanagi â Described a technique for the more complex scrotal cases
⢠1994 â Snodgrass â Described an incision of the tubularized urethral plate
16. 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, MustardÊ, Broadbent, Hodgson,
Horton and Devine, Standoli, and Duckett
17. INCIDENCE - GLOBAL
⢠Common congenital malformation
⢠0.8 and 8.2 per 1,000 live male births1
⢠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
1. Sweet RA, Schrott HG, Kurland R, et al. Study of the incidence of hypospadias in Rochester, Minnesota, 1940â1970, and a case control comparison of possible
etiologic factors. Mayo Clin Proc 1974;49:52â8
18. INCIDENCE
⢠USA â 1 in every 250 male births1
⢠> 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
1. 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.
19. INCIDENCE
Race Related 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
1. Schnack TH, Zdravkovic S, Myrup C, et al. Familial aggregation of hypospadias: a cohort study. Am J Epidemiol 2008;167:251â6.
20. 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.
21. 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 195â202,
22. EMBRYOLOGY OF PENILE
DEVELOPMENT
⢠4th 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)
23. 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 â 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
24. 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.
25. 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
28. ETIOLOGY1,2
⢠Exact etiology of hypospadias is unknown
⢠Believed to include;
Genetic
Endocrine and
Environmental factors
1. 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):260â283. doi: 10.1093/humupd/dms002
2. 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):187â197. doi: 10.1111/j.1365-2605.2008.00899.x.
29. 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 5Îą-reductase
Deficient androgen receptors in the penis
⢠Various disorders of sexual differentiation (DSD) can cause deficiencies at any point
along the androgen-stimulation axis
30. 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 biosynthesis1
⢠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 mutation2
⢠Qualitative and quantitative androgen receptor deficits
1. 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.
2. 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.
31. ETIOLOGY - ENDOCRINE
Endocrine theory
⢠A higher incidence of hypospadias in winter conceptions has also been proposed
⢠Males born through in-vitro fertilization (IVF) â 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
32. 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
⢠Multifactorial mode of inheritance
⢠These families having a higher than average number of influential genes for creation
of hypospadias1
⢠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;29:499â505.
33. 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
34. 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 effect1
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.
35. ETIOLOGY
Environmental factors
⢠Drugs - Antiâepileptic Valproate1
Antiâhistamine Loratadine1
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
36. 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
37. ETIOLOGY - SYNDROMIC
Suspected with development delay, dysmorphic facies, and/or anorectal malformation.
Examples include:
⢠Smith-Lemli-Opitz syndromeâresults 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)âresults
from a deletion in chromosome 11p13.
⢠G syndrome (Opitz G/BBB syndrome)âoccurs 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.
38. ETIOLOGY - SYNDROMIC
⢠Wolf-Hirschhorn syndromeâderives from deletions in chromosome 4p resulting in mental retardation, seizures,
abnormal facies, and midline defects.
⢠13q deletion syndromeâcharacterized by mental retardation, facial dysmorphia, imperforate anus, and hypospadias
with penoscrotal transposition.
⢠Hand-foot-uterus syndromeâan autosomal dominant condition caused by mutations in the HOXA13 gene on
chromosome 7p14-15, resulting in bilateral thumb and great toe hypoplasia.
40. PATHOPHYSIOLOGY
⢠Congenital 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
41. 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
42. 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
44. 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
46. 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
47. 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.
50. INDICATIONS OF OPERATION
Cosmetic indications
⢠Abnormally located meatus
⢠Cleft glans
⢠Rotated penis with abnormal cutaneous raphe
⢠Preputial hood
⢠Penoscrotal transposition
⢠Split scrotum
51. 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 â 2mg/kg/dose given for a total of 2 or 3 doses
before hypospadias repair
⢠Increases penile length and girth
⢠Testosterone propionate cream â 2% three times daily for 3 weeks
⢠Induces neovascularization
52. MANAGEMENT â 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 hypospadias1
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.
53. MANAGEMENT â AGE AT REPAIR
⢠Considerations1 - 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.
54. MANAGEMENT â AGE AT REPAIR
⢠Another opportunity - 3-4 years3
⢠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 old1
⢠The âoptimal windowâ recommended for repair was age 6 to 15 months2
⢠Psychological concerns
1. Belman AB, Kass EJ. Hypospadias repair in children less than 1 year old. J Urol. 1982;128:1273â1274.
2. Schultz JR, Klykylo WM, Wacksman J. Timing of elective hypospadias repair in children. Pediatrics 1983;71:342â51.
3. 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.
55. MANAGEMENT â 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
56. MANAGEMENT - ORTHOPLASTY
1. 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 curvature1
⢠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;4:473â5.
57. MANAGEMENT - ORTHOPLASTY
1. 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 surface3
1. Gittes RF, McClaughlin AP. Injection technique to induce penile erection. Urology 1974;4:473â5.
2. Devine CJ, Horton CE. Chordee without hypospadias. J Urol 1973;110:264â71.
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;178:1693â7.
58. MANAGEMENT - ORTHOPLASTY
⢠Small intestinal submucosa as an off-the-shelf substitute for the autologous
grafts1
⢠Excise wedges of tunica albuginea dorsally with transverse closure to shorten
this dorsal surface and straighten the penis â 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;170:1593â5.
59. MANAGEMENT - ORTHOPLASTY
Ventral corporeal lengthening :
⢠Ventral corporotomy with grafting
⢠Multiple corporotomy without grafting
61. 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
63. 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
64. 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
65. 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.
66. 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.
67. 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 ThierschâDuplay 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
70. 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
72. 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
73. 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
74. 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
75. 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
80. TECHNICAL PERSPECTIVES
Sutures and Instruments
⢠Fine absorbable suture â Neourethra closure
⢠Polyglycolic or polyglactin material is preferred
⢠Permanent sutures
⢠Nylon or polypropylene
⢠Continuous 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)
81. 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
82. 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
83. 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)
84. 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
85. 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
86. 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
87. 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 â Fistulectomy followed by closure of the urethral epithelium with
fine absorbable suture
⢠Prevent recurrence - Approximating layers of well-vascularized subcutaneous
tissue over this closure
88.
89. 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
90. COMPLICATIONS
Strictures
⢠Anywhere along neourethra course
⢠Most common sites - Meatus and proximal 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
92. 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
93. 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
94. 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
96. FEMALE HYPOSPADIAS
Female hypospadias1
⢠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 âadvancement flapâ 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),
141â143. https://doi.org/10.4103/jiaps.JIAPS_69_18
97. REFERENCES
1. van der Horst, H. J., & de Wall, L. L. (2017). Hypospadias, all there is to know.
European journal of pediatrics, 176(4), 435â441. https://doi.org/10.1007/s00431-
017-2864-5
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. 3â47.
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. 237â43
4. 4. Bachus LH, de Felice CA. Hypospadias, then and now. Plast Reconstr Surg
1960;25:146â60.