This document provides information on hypospadias, including its definition, embryology, classification, diagnosis, and management. It describes hypospadias as an abnormal ventral placement of the urethral opening in males, often accompanied by penile curvature and a dorsal hooded foreskin. The etiology is multifactorial involving genetic and environmental factors disrupting androgen stimulation in development. Surgical management aims to correct penile curvature, reconstruct the urethra, and provide skin coverage using techniques such as tubularized incised plate repair and preputial flaps depending on the location and severity of hypospadias.

1. Hypospadias Pushpa Lal Bhadel
Department of Pediatric surgery
Kathmandu Model Hospital

2. Introduction
Congenital anomaly of the male urethra that results in
abnormal ventral placement of the urethral opening
Defined as a combination of any or all of the following
associated penile anomalies
oEctopic urethral meatus
oPenile curvature (chordee)
oDorsal hooded prepuce

3. Fig. Composite of cases demonstrating
increasing severity of hypospadias.
(A), distal penile shaft (B, C), mid-penile
shaft (D), scrotum (E), and perineum (F)

4. Embryology
elongation and enlargement + fusion of urethral
folds
Testosterone
Penis
Genital tubercle

5. Embryology
 Cloacal ectoderm primitive urethral plate, ventrally
 Medial endoderm male urethra, ventrally
 Lateral ectoderm skin of penile shaft and prepuce
 Layers fuse posteriorly to anteriorly, forming median raphe
 Arrest in fusion hypospadias
 Abnormal development of growth plate, disproportionate corpora
and fibrosis leads to penile curvature

6. Etiology
 Multifactorial
 Proposed mechanism is disruption of androgenic stimulation required
for development of normal male external genitalia 1
 Genetic defects:
oMutation in genes that affect androgen metabolism, and estrogen and
androgen response
oHOXA4, IRX5, IRX6, EYA1, DGKK
 Associated genetic syndromes
oDenys-Drash syndrome
oWAGR syndrome
oOpitz syndrome
1 Choudhry S, Baskin LS, Lammer EJ, et al. Genetic polymorphisms in ESR1 and ESR2 genes, and risk of hypospadias in a multiethnic study population. J Urol. 2015;193(5):1625-31.

7. Etiology
Environmental exposures
oPrenatal exposure to estrogenic compounds 1
• Maternal progestin, diethylstilbestrol
oExposure to paints/solvents/adhesives, detergents, pesticides,
cosmetics and industrial chemicals 2
oMaternal high dietary intake of phytoestrogens associated with
decreased risk 3
1 Brouwers MM, Feitz WF, Roelofs LA, Kiemeney LA, De gier RP, Roeleveld N. Risk factors for hypospadias. Eur J Pediatr. 2007;166(7):671-8.
3 Carmichael SL, Cogswell ME, Ma C, Gonzalez-Feliciano A, Olney RS, Correa A, Shaw GM. Hypospadias and maternal intake of phytoestrogens.
American journal of epidemiology 2013;178(3):434-40.
2 Poon S, Koren G, Carnevale A, et al. Association of In Utero Exposure to Polybrominated Diphenyl Ethers With the Risk of Hypospadias. JAMA Pediatr.
2018;172(9):851-856.

8. Etiology
 Other risk factors 1
oAdvanced maternal age
oPreexisting maternal diabetes mellitus
oGestational age before 37 weeks
oHistory of paternal hypospadias
oExposure to smoking and pesticides
oPlacental insufficiency (low placental weight and pathology)
oPrematurity
oFetal growth restriction
oIn-vitro fertilization
1 Brouwers MM, Feitz WF, Roelofs LA, Kiemeney LA, De gier RP, Roeleveld N. Risk factors for hypospadias. Eur J Pediatr. 2007;166(7):671-8.

9. Incidence
 Most common congenital anomalies
 Ranges from 0.3-0.7 percent live births 1
 Risk is 13-fold in 1st degreerelatives
 Risk of hypospadias in next child, when one child is affected withhypospadias:
o 12% with negative family history
o 19% if cousin or uncle has hypospadias
o 26% if father or sibling has hypospadias
 8.5 times more risk in monozygotic twins
1 Nassar N, Bower C, Barker A. Increasing prevalence of hypospadias in Western Australia, 1980-2000. Arch Dis Child. 2007;92(7):580-4.

10. CLASSIFICATION
ANTERIOR
•GRANULAR
•CORONAL
•SUBCORONAL
MIDDLE
DISTAL PENILE
MID SHAFT
PROXIMALPENILE
POSTERIOR
•PENOSCROTAL
•SCROTAL
•PERINEAL

11. Classification

12. Diagnosis
Generally made during the newborn genital examination
Physical findings consistent with the diagnosis include:
oAbnormal foreskin resulting in an incomplete closure around the
glans : dorsal hooded prepuce.
oAbnormal penile curvature (chordee).
oThe appearance of "two urethral openings";
• First in the normal position at the end of the glans, which is usually a blind
ending urethral pit
• Second, the abnormally located true urethral meatus

13. Initial evaluation
 Includes focused history, close examination of genitalia and
identification of other congenital anomalies
 History: positive family history, maternal or infant risk factors
 Physical examination:
oIdentifying other associated anomalies and genital examination determining
the presence and degree of hypospadias

14. Initial evaluation
 Genital examination:
 Assessment of stretched penile length: 2.5-3.5 cm in full term male
 Assessment regarding evidence of penile curvature if so, degree of curvature
 Severity:
• Normal – No curvature to 15 degrees
• Mild – 15 to 40 degrees
• Moderate – 40 to 80 degrees
• Severe – >80 degrees
 Assessment of foreskin to ensure complete and circumferential development
without ant ventral asymmetry or deficiency
 Determination of the presence of both testicles in the normal dependent position
in the scrotum.

15. Management
Indications of surgery
Functional indications:
 Proximally located meatus
 Ventrally deflected urinary stream
 Meatal stenosis
 Curved penis

16. Management
Cosmetic indications : strongly linked patient’s future
psychology are:
oAbnormally located meatus
oCleft glans
oRotated penis with abnormal cutaneous raphe
oPreputial hood
oPenoscrotal transposition
oSplit scrotum

17. Management
Timing of surgery:
oRecommendations from American Academy of Pediatrics (AAP)
oBetween 6 months and 1 year of age
oAllows ample time for completion of two-stage procedure in
patient with severe hypospadias
oPrior to beginning of gender identification
oTime between two stages: six months

18.  Preoperative Hormonal Stimulation:
HCG 250-500 U sc twice a week for 3 weeks. Increase in penile
size and length
Decrease in hypospadias and chordee severity
Increased vascularity and thickness of corpus spongiosum
Allowance of more simple repairs
IM testosterone enanthate – 2mg/kg/dose given for a total of 2
or 3 doses before hypospadias repair
Testosterone propionate cream – 2% three times daily for 3
weeks

19. Management
General principles of hypospadias repair
oOrthoplasty
oUrethroplasty
oMeatoplasty
oGranuloplasty
oSkin coverage

20. Management
Orthoplasty
oCorrection of ventral curvature
oVentral tissues—including shaft skin, dartos, corpus
spongiosum, urethral plate, and overlying tunics of the
corpora cavernosa may be shortened relative to the dorsal
surface.
oVC occurred in 11% of primary distal cases, 30% midshaft, and
81% proximal hypospadias.

21. Management
Orthoplasty
oPreoperative assessment cannot accurately predict either the
extent of curvature or the means required for straightening.
oIntraoperative assessment of penile curvature by either
artificial or pharmacologic methods is a critical step in
hypospadias repair.
oPerformed after degloving of penile shaft skin

24. Management
 Artificial erection induced by saline injection remains the
most used means to assess presence and severity of VC.
 Pharmacologic erection allows for a more accurate and
continued assessment of penile curvature before, during,
and after its correction.
 Intracorporal injection of the arterial vasodilator
prostaglandin E1.

25. Management
Curvature up to 30 degrees
can be corrected by Midline
Dorsal Plication into the
tunica albuginea of the
corpora cavernosa directly
opposite the area of greatest
bending.

26. Management
Nesbit technique
oExcision of diamond shaped
wedge/s at the point of
maximum curvature and
closing the tunica transversely
with absorbable sutures.

27. Management
Ventral corporal lengthening
oVentral corporotomy with
grafting.
oMultiple corporotomy
without grafting.

28. Management
Dermal graft – Devine and Horton

29. Management
Urethroplasty
Distal hypospadias :
oTIP repair
oOthers like MAGPI, Mathieu flip-flap, and Urethral advancement.
Midshaft hypospadias :
oTIP repair
oOnlay preputial flap

30. Management
 Proximal hypospadias :
o TIP repair
o Onlay preputial flap
o Single stage urethroplasty with preputial flap or the
Koyanagi flap.
o Two stage repair with Byars flaps or grafts.

31. Management
Distal hypospadias
Tubularized Incised Plate (TIP) repair
oCircumscribing incision is made approximately 2 mm below meatus
oVentral V incision
oPenis degloved
oMidline incision of the urethral plate
oUrethral plate tubularization begins distally approximately 3 mm
from the end of the plate, ensuring an oval, not rounded, meatus.

32. Management
 Dartos flap is dissected from the dorsal prepuce and shaft
skin, buttonholed, and transposed ventrally to cover the
neourethra.
 Glansplasty begins distally, and a 7-0 polyglactin suture.

33. Management
Midshaft hypospadias
TIP repair
Onlay preputial flap
oThin skin proximal to the urethral meatus is incised to the midline
convergence of corpus spongiosum wings.
oThen inner prepuce is harvested on its vascular pedicle from
either the dorsal hood or dartos flap.
oThe flap should be gently stretched to fit the urethral plate without
redundancy.

36. Management
Proximal hypospadias
 The greatest controversy in primary hypospadias surgery concerns
decision making for proximal cases.
 Options depend on whether the urethral plate is available for
urethroplasty after associated VC is straightened.
 If so, then either TIP repair or an onlay preputial flap can be used.
 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.

37. Management
Proximal tubularized incised plate repair
oCircumscribing incision preserves urethral plate in patient desiring
circumcision.
oAfter degloving, glans wings are separated from the urethral plate.
oCorpus spongiosum is dissected from the cavernosal bodies
oMidline urethral plate incision.
oSpongioplasty over the neourethra.

39. Management
Tunica vaginalis flap

40. Management
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.

41. Management
The urethral plate in the center of the flap
is dissected from the corpora to near the
meatus, and the glanular portion of the
plate is excised as glans wings are made.
Inner flap margins are reapproximated,
and excess flap skin is excised.
The outer margins are closed to complete
tubularization

42. Management
Byars flap
 After degloving and release of ventral dartos, persisting
ventral curvature greater than 30 degrees led to
excision of the urethral plate.
 The dorsal preputial hood is incised in the midline and
the two flaps transposed ventrally on either side of the
penis.
 The prepuce is advanced into the glans; alternatively,
the urethral plate can be maintained within the glans

43. Management
 Flap edges are approximated
in the midline.
 Six months later a U-shaped
incision is made
approximately 10 mm wide.

44. Management
The resultant strip is tubularized in two layers.

45. Complications
 Bleeding/hematoma,
 Meatal stenosis,
 Urethrocutaneous fistula,
 Urethral stricture,
 Urethral diverticulum,
 Wound infection,
 Impaired healing, and
 Breakdown of the repair

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