This document provides definitions, embryology, pathology, classification and management principles for hypospadias. Hypospadias is a congenital anomaly where the urethral opening is located abnormally on the ventral surface of the penis. It occurs in about 1 in 300 male births. The main features include an ectopic urethral opening, dorsal hooding of the prepuce, and ventral penile curvature known as chordee. The embryology of normal male urethral development is described. In hypospadias, there is a failure of midline fusion of the urethral folds leading to an incomplete or arrested development of the urethra. Single-stage

1. (German) Hypo : under
Spadizo : to tear off
HYPOSPADIAS

2. Definition
• Commonest Congenital/developmental urethral anomaly in which the external
urethral meatus is located proximally on the ventral surface (undersurface) of
the penis or in the scrotum or perineum.

3. Incidence & Genetics
• Commonly Encountered congenital malformation
• 1 in 300 male live births; mostly sporadic
• No characteristic chromosomal defect
• 9 fold increased risk in monozygotic twins
• Usually diagnosed at the time of birth
• Sometimes it is missed and becomes evident later when the boy micturates and
spoils his clothes or avoids by squatting.
• Embryogenesis is well known.

4. EMBRYOLOGY

8. Development of external genitalia

10. • 8 weeks – epithelial fold ascends and covers glans – prepuce (surrounds glans)
• Male urethra – 3 sources
• Neck to opening of ejaculatory ducts – urogenital sinus
• opening of ejaculatory ducts to corona – union of 2 urethral folds from
proximal to distal (median hyperpigmented raphe)
• Corona to tip of glans – invagination and canalization of ectoderm
• Female urethra – Urogenital sinus
• All development is governed by secretion from testicles

11. Female Structure Male
Clitoris genital tubercle Penis
Labia majora Lateral folds (genital
swellings)
Scrotum
Labia minora urethral folds over
urethral groove
Fusion from proximal to
distal forms Penile
urethra

12. • Growing phallus – Phallic part of UGS
• Endodermal cells of UGS – solid “ Urethral Plate”
• Urogenital Membrane & cells forming core of Urethral plate degenerate
• Mesenchyme around urethra – corpus spongiosum & covering fascias

13. • If at any time, there is premature involution of interstitial cells of
testicles, No testosterone Arrest in normal Development
union of 2 urethral folds will stop at that level – LEVEL OF
HYPOSPADIAL OPENING (incomplete masculisation)
• 9 months – complete phallus develops, scrotal sacs develop and
testicles descend

14. Relevant normal anatomy
• Male organ of copulation
• 2 parts: root and body
• Root
• attached portion
• composed of 3 masses : 2 crura and one bulb
• each crura is attached to the pubic arch and is covered with ischiocavernosus
• bulb is attached to the perineal membrane and Is covered with bulbospongiosus
• urethra traverses the bulb and enters corpus spongiosum
• Body
• free portion enveloped in skin
• composed of 3 elongated masses of erectile tissue corpora cavernosa (2) and corpus
spongiosum (1)

16. • Corpora cavernosa
• forward continuation of crura
• close opposition with each
other
• do not reach the end of penis
and terminate under the glance
cap as blunt conical ends
• Surrounded by tunica albugenia
• 2 corpora cav. Are not separate
structures but constitute a
single space with free
communication through midline
septum

17. • Corpus spongiosum:
• Forward Continuation of the bulb of penis
• Lies in the ventral group between 2 corpora cavernosa surrounded by thin tunica
albuginea
• At distal end - expands to form glans penis (a broad cap of erectile tissue covering
the tips of corpora cavernosa )
• Traversed by urethra throughout its extent
• Base of glans shows projecting margin – corona glandis
• Overhangs obliquely grooved constriction – coronary sulcus
• Within the glans urethra shows dilatation – navicular fossa
• external urinary meatus is slit like Lying slightly on the ventral aspect of tip of the
glans with long axis oriented vertically (5-6mm)

18. • Covering:
• Skin – thin and dark colored
• Loosely connected with fascial sheath
• Folded at neck to form prepuce
• Median fold at ventral aspect of glans – frenulum
• Superficial – Dartos fascia
• Deep – Buck’s fascia
• Arterial supply :
• Ext Pudendal A. (Skin of shaft and sup. Fascia of penis) and
• Int. Pudendal Artery (Dorsal Penile, cavernosal or deep a. and bulbourethral a.)

19. • Venous supply :
• Superficial vein of penis (in dartos)
• Intermediate – emissary veins (beneath bucks)
• Deep - Crural and Cavernosal vessels
• Nerve supply :
• Pudendal nerve – sensory – lie deep to bucks fascia
• Fascicles fan out and terminate in the glans

20. Abnormal development in Hypospadias
• Abnormal morphogenesis affects 3 main anatomical features:
1. Ectopic urethral orifice – primary anomaly
• Failure of midline perineal mesenchyme to grow ventrally to cover the urethral
plate as it canalizes
• Incomplete morphogenesis is most common defect – in hypospadias most
common arrest is at or near the coronal groove (9-10 wks embryo)
• More proximal opening means more severe defect in morphogenesis or defect in
androgenic action
2. Dorsal Hood and Raphe – Dorsal hooded prepuce is characteristic
• Failure of androgen dependent growth of ventral penile mesenchyme
• Wedge shape defect in ventral prepuce , absent frenulum
• Each corner of hood, bifurcated raphe ends in “dog ear”

21. • Median raphe is abnormal – zig zag course
• Just proximal to ectopic orifice, raphe bifurcates
• In case raphe bifurcates some distance proximal to orifice, the urethra is
superficial & lacks adequate supporting tissue & corpus spongiosum
3. Chordee – related to severity
• More proximal – hairpin bend in cor. Cavernosa (d/t apoptosis of urethral plate)
• Distal variants – arrest in mesenchyme growth / initiation of apoptosis occur
later – less severe chordee (d/t deficient peri urethral growth rather than bent in
corpora)

23. PATHOLOGIC ANATOMY
• GLANS : flat and spatulated with a shallow groove on the ventral surface and dimple
at normal site of urethral meatus
• PREPUCE – “dorsal hooded”; deficient ventrally
• SHAFT – “Chordee” (L: string); curved ventrally
• More proximal the meatus , more significant the curvature
• Erection accentuates the curvature
• Distal to ectopic meatus the subcutaneous structures are absent
• Replaced by fan shaped, fibrous , dense, inelastic tissue causing ventral bowing
of the shaft
• Extent of inelastic tissue – from around abnormally placed urethral meatus to
insert along the ventral aspect of glans from one end of hooded prepuce to the
other

25. Theories about Chordee
1. Fibrous chordee – the mesenchyme that normally form cor. Spong and fascial
layers distal to hypos meatus persists as dense fibrous inelastic tissue
2. Cutaneous - In milder degrees of chordee, this fibrous tissue not found, instead
absent Dartos with adhesion of skin to deep fascia is the cause.
3. Growth differential – between normally developed dorsal tissue and deficient
ventral tissue
4. Chordee without Hypospadias

26. Aetiology
• Incomplete masculization of external genitalia
• Defect in quantity of testosterone
• Defect in quality of testosterone
• Premature involution of interstitial cells of Leydig
• Defeciency of 5 alpha reductase
• Androgen receptor deficiencies
• Maternal environmental exposures – phthalate in some plastics & estrogens in food,
cosmetics (a hypothesis)

27. CLASSIFICATION

29. In order to achieve a universal, comparable classification, two assessments are
recommended:
(l) A preoperative assessment, based on the clinical site of the meatus, should
indicate the presence or absence of visible chordee
(2) An intraoperative assessment based on the position of the meatus after
correetion of chordee or penile curvature.
Ideally, the classification should embrace condition of prepuce, chordee, rotation and
scrotal transposition

31. To be continued…

32. Blood supply of penis &prepuce

35. Hypospadias
(Management)

36. Timing of Elective Hypospadias Repair
• Several factors influence, directly or indirectly, the timing of elective repair in
childhood
• the understanding of the psychological implications of genital surgery in children
• the improvement in the technical aspects of surgery for hypospadias
• the advances in pediatric anesthesia
• Technical considerations used to be limiting factors in determining the time in the past
when the reconstructive techniques were multi staged with first and second stage
separated by an interval of 6 to 12 months
• Today increase surgical experience, use of micro instrumentation, optical
magnification has made the operative procedure technically feasible and has changed
the operative rules - single stage hypospadias repair is performed in almost all cases
with the multistage procedure reserved for the most complicated malformations
• Surgery for hypospadias can successfully be achieved in children between 6 and 12
months of age

37. Now aim –
• Functionally and aesthetically normal penis
before the age of memory recall
• 6 months to 18 months
• The phallus size is not a all a technical
consideration as it grows only about 0.8cm
between 1 and 3 yrs

38. General Principles
• History – detailed Family history
• previous members of the family with hypospadias
• 14% of male siblings of an index child
• if 2 members a family have hypospadias then the risk is 21% in
subsequent male child
• maternal injection of hormone medications during pregnancy
• Examination – position of meatus, configuration and diameter
• Ventral chordee (best way to access the degree of chordee is intraoperative
erection test )
• Elasticity of ventral skin ,urethral plate and adequacy of foreskin
• Glance for presence of ventral groove or shallow glans
• Careful palpation of testis in the scrotum
• Any associated penoscrotal abnormality or penile torsion

39. • Imaging – Ultrasound as the screening procedure should be considered in
severe types of hypospadias since there is low incidence of upper urinary tract
anomalies in mild hypospadias
• Associated anomalies – undescended testis and hernia are most common
• Hypoplasia of the penis should be ruled out - the normal penile stretch length
in the newborn is 3.9 cm (3.1 to 4.7cm)
• Preoperative evaluation –
• early diagnosis is recommended
• usually done soon after birth
• Early referral to detect any anomaly requiring early intervention
(meatotomy for metal stenosis )and to counsel the parents about the timing
of the definitive surgery, potential treatment and outcome

40. • Preoperative preparation –
• Admission, anesthesia fitness
• Bowel preparation and avoiding Constipation
• Local application of local antiseptic over genital skin before surgery in addition to scrubs has been
shown to decrease incidence of infection and hence fistula information
• Preoperative hormonal stimulation
• hypospadias repair in child with microphallus is technically difficult
• preoperative androgen therapy is suggestive allowing early surgical correction by enhancing the
penile size and does satisfactory repair and avoiding psychological problems of late repair
• Gonadotrophin (HCG) or testosterone may be used
• Treatment with HCG has shown decrease in the degree of severity of proximal hypospadias and
improved chordee
• Testosterone has been shown to be more beneficial than HCG in hypospadiac micro penis
• can be given I/M (25mg monthly x 3 times) or applied locally as testosterone propionate cream
2% to the penis 3 times daily for 3 weeks preferably by the father OR by the mother after wearing
gloves

41. • Consent –
• Written and informed consent should be obtained
• Parents should be aware that the best procedure maybe better judged when the
child is under anesthesia and the possible alternatives must be explained in
advance
• Brief but clear outline of the outcome cosmetic results and possible complications
(particularly fistula formation, Infection or dehiscence ) must be given
• Parents should be made aware that the child may require a multi stage procedure,
redo surgery or a minor corrective procedure later on

42. Principles of hypospadias Repair
• Complete straightening of the penis (during erection ) by ortho plasty (resection of
Chordee) to allow forward directed stream and normal coitus
• Positioning of new meatus to the tip of the glans
• Construction of hairless urethra of uniform calibre
• Creation of a symmetric glans and shaft
• Normalization of voiding
• Aesthetically / cosmetically acceptable
to recreate a “normal” penis while minimising the complications
Single stage operations are better suited to meet todays surgical objectives

43. Phases of surgical repair
1. Meatoplasty and Glanuloplasty
2. Orthoplasty (straightening)
3. Urethroplasty
4. Skin cover
5. Scrotoplasty
these are applied either sequentially or in various combinations

44. Surgical approach depends upon
• degree of curvature
• location of meatus after release of curvature
• quality of available skin
• preference of the surgeon
• experience of the surgeon

45. General principles of management
1. Selection of appropriate procedure
2. correct obstructive uropathy first (Meatal stenosis)
3. never do circumcision in case of hypospadias ensure complete chordee release prior to
undertaking urethroplasty (artificial erection test )
4. Maintaining a bloodless field injecting 1:100000 adr in the lines of incision and dissection
and precise electrocautery
5. Appropriate stenting or urinary diversion
6. Optical magnification
7. micro instruments / find instruments
8. finer suture material
9. meticulous dissection and atraumatic tissue handling
10. Tensionless Multilayered closer and watertight anastomosis (Prevent tissue ischemia )
11. light occlusive and non sticky dressing
12. proper post op care and psychological support

47. Why earlier surgeons did stage surgeries ?
• Miss concept of “regrowth of chordee”
• Complete extent of chordee not well understood , therefore there used to be a
minimum gap of 6 months between the 2 stages ;
if Chordee persisted after stage one secondary release was accomplished before
proceeding with urethroplasty
Successful one stage repairs are possible today :
• Better understanding of anatomy and extent of chordee; hence removed in enterity
• Availability of artificial erection test which allows complete straightening
• Advances in instrumentation, magnification and pediatric care anesthesia
The fibrous tissue of chordee does not continue to regrow, once completely excised .

48. Advantages of single stage surgeries :
• Single hospitalization
• Cost effective
• Minimises physical and psychological trauma
• Allows completion of surgery before the age of memory recall
• Availability of non scarred and healthy tissue for reconstruction
“It is inalienable right of every boy to be a pointer instead of sitter by the time he
starts school and to write his name legibly in the snow”
- Culp and Mc Roberts

49. Various single staged surgeries
• Meatal Advancement and Glanuloplasty Procedure (MAGPI) – Duckett 1981
• Urethral advancement, Glanuloplasty and Preputioplasty in Distal hypospadias
(URAGPI) – Keramidas and Soutis 1995
• Thiersch- Duplay principle
• Local-flap Techniques:
• Hortan-Devine Flip-Flap Procedure
• Meatal-based Flap “Mathieu” Technique (Flip-Flap)
• Tubularised Incised plate Urethroplasty (TIP) – Snodgrass 1994
• Snodgraft
• Island Onlay Hypospadias Repair

50. MAGPI
• A glanular, coronal, or distal location of
the meatus with a mobile distal urethra
is ideal for a MAGPI operation.
• A more proximal hypospadias, especially
with a chordee, is unsuitable.
• Lifting the skin below the meatus and
pulling it up along with the urethra helps
one to judge whether there is enough
urethral mobility for the meatus to be
positioned at the tip of the glans.

51. URAGPI
• For Distal Hypospadias

52. Thiersch- Duplay principle
• Earlier used only for children with
meatus at or just proximal to coronal
sulcus.
• In meatal stenosis, Heineke-Mikulicz
meatoplasty at 12 o’clock
• Subsequently in proximal hypospadias
with chordee due to skin tethering or
mild chordee (<30°),due to corporal
disproportion , which was corrected by
the Nesbit technique of tunica
albuginea plication.
• C/I : moderate to sever ventral chordee
(>30°)due to corporal disproportion

53. The Hortan-Devine
Flip-Flap Procedure
• Modification of Mustarde’s operation
• Indications :
• Distal hypospadias with no or mild chordee
• Post chordee release – meatus is localized
on distal third of penile shaft
• Well vascularized and thick skin proximal to
hypospadiac meatus
• Advantages:
• Low risk of meatal stenosis
• Disadvantage:
• Snub-nose appearance of glans
• Ugly looking meatus

54. The Mustarde Procedure
• A wider meatal based flap for
tubularisation of neourethra.
• A triangular glanular flap for
construction of glanular part of
urethra
• Problems:
• Meatal stenosis
• Technical problem related to
coverage of large ventral skin
defect

55. The Meatal based “Mathieu”
Technique
• For distal hypospadias
• Pre-requisites:
• Straight penis
• Wide glanular groove
• C/I:
• Mid-shaft / proximal hypospadias, severe chordee
• Shallow glanular groove
• Unhealthy skin over distal urethra

56. Modified Matheiu – V-Y Glanuloplasty

57. Tubularized Incised Plate Urethroplasty
• Most commonly and routinely performed regardless of specific location
• Can be done in : Distal hypospadias, proximal Hypospadias, Reoperations
• Can be performed for proximal hypospadias even in some cases with severe penile
curvature – if intraoperatively, urethral plate transaction is not needed and hence
safeguarded for the TIP
• For reoperations – assess preoperatively : urethral plate is intact and not grossly
scarred
• Should be sufficiently “supple” for tubularization post incision
• The decision for Urethroplasty is no longer made before surgery. Its presumed that
TIP can be done , hence urethral Plate is initially preserved.

58. • C/I:
• previous resection of plate
or gross scarring
• Key steps:
• Degloving of Penis
• Urethroplasty – relaxing
incision over urethral plate
& Tubularization
• Glanuloplasty

60. • TIP repair creates a vertically oriented, slit neomeatus that closely resembles the
normal urethral meatus
Complications:
• Fistula in proximal >>Distal (in-turning all epithelium while tubularizing & covering
the layer with Dorsal dartos pedicle)
• Glans wings provide superior protection than dartos
• Mobilize Corpus spongiosum alongside urethral plate and close it over neourethra in
proximal hypospadias
• Meatal stenosis - neourethra should be oval not circular to avoid this
• Stricture
• Diverticulum
• Partial glans dehiscence

61. Dorsal Inlay Graft Urethroplasty – “Snodgraft”
• Modification of Snodgrass
• 1st reported in 2000
• Advantage : reduces risk of meatal stenosis
• Indications:
• narrow or shallow glans
• Insufficient urethral plate
• Small free graft harvested from preputial inner skin
• Sutured over the incised plate
• This neourethra rolled around feeding tube and sutured
• Dartos - as 2nd barrier for waterproofing laid over suture line

63. The Island
Onlay
Hypospadias
repair

65. Other Single staged repairs for proximal
/penoscrotal Hypospadias
• Tubularised (Transverse) Preputial Island Flap
• Onlay-Tube-Onlay Modification of Transverse Preputial Island Flap
• Modified Asopa (Hodgson XX)
• Koyonagi-Nonomura – Perineal
• Yoke Repair
• Lateral Based Flap for Dual Blood Supply

66. Modifies Asopa Repair

67. Grafts for one stage Repair
• Preputial Skin Graft
• Buccal Mucosa Graft
• Bladder Mucosa Graft

68. Post op Care
• Urethral tube fixed with stay/traction suture taken from glans
• Sandwich dressing is done
• Post op analgesics 6-8 hrly
• Early Discharge
• Adequate Hydration
• Maintaining Hygiene
• Stent Removal after 10-14 days

69. Complications
• Infection
• Meatal Stenosis
• Loss of skin flaps
• Odema
• Haemorrhage
• Erection
• Retrusive meatus
• Bladder spasm
• Catheter Blockage

70. thanks