Pediatric urology:Hypospadias- management

HYPOSPADIAS
Dept of Urology
Govt Royapettah Hospital and Kilpauk Medical College
Chennai
1

MODERATORS:
Professors:
Prof. Dr. G. Sivasankar, M.S., M.Ch.,
Prof. Dr. A. Senthilvel, M.S., M.Ch.,
Asst Professors:
Dr. J. Sivabalan, M.S., M.Ch.,
Dr. R. Bhargavi, M.S., M.Ch.,
Dr. S. Raju, M.S., M.Ch.,
Dr. K. Muthurathinam, M.S., M.Ch.,
Dr. D. Tamilselvan, M.S., M.Ch.,
Dr. K. Senthilkumar, M.S., M.Ch.
DEPT OF UROLOGY,GRH ANDKMC,CHENNAI. 2

HYPOSPADIAS - ETYMOLOGY
In Greek,
Hypo- Under
Spadon- Fissure/Rent
3
DEPT OF UROLOGY,GRH ANDKMC,CHENNAI.

HYPOSPADIAS - EPIDEMIOLOGY
1-3 per 1000 births
1 in 300 male children
70-85% have mild distal meatus variant.
10-25% have severe proximal hypospadias.
4

RISK FACTORS
1. Premature birth
2. Infants small for gestational age
3. Intrauterine growth restriction
4. Assisted reproduction techniques
5. Pesticide exposure
6. Hormonal exposure in utero (DES, Birth control medications)
5

SYNDROMES WITH HYPOSPADIAS
1. WAGR syndrome- Wilms Tumour, Aniridia, Genitourinary abnormalities and
developmental delay
2. Denys Drash syndrome – Genitourinary abnormalities, renal failure and high risk
for WT
3. Smith Lemli Opitz syndrome – Malformations of heart, lungs, kidneys,
gastrointestinal tract and genitalia
6

TRIAD OF HYPOSPADIAS
1. Dorsally hooded incomplete foreskin
2. Ventral penile curvature
3. Proximally placed external urethral meatus
7

EMBRYOLOGY
8

HYPOSPADIAS-
EUM LOCATIONS
9

CLASSIFICATION
10

HYPOSPADIAS-
CLASSIFICATION
11

GMS SCORE
12

EVALUATION
Careful history and physical examination
Measurement of penile length
Associated Penile anomalies – Penile torsion, penoscrotal webbing, penoscrotal
transposition
Associated common anomalies- Inguinal hernia/hydrocele (9-16%), Cryptorchidism
(7%)
Patients with severe proximal hypospadias with unilateral or bilateral undescended
testes should undergo DSD evaluation. (3x risk)
13

DSD TO BE KEPT IN MIND
5 alpha reductase deficiency
Androgen receptor insensitivity
Testicular dysgenesis
Ovotesticular DSD
14

DSD WORK UP
Ultrasound
Karyotyping
Hormonal analysis (Testosterone, FSH, LH and AMH)
15

DIEFFENBACH PROCEDURE
In 1838,
Dieffenbach perforated the glans down
to the urethral meatus and inserted a
cannula in between, until the neourethra
was covered by normal urothelium.
Not successful.
18

BOUISSON PROCEDURE
In 1861, First surgeon to use a rotated
local pedicled scrotal flap.
The inner surface of this scrotal flap was
used to create the anterior part of the
neourethra with a technique resembling
the Mathieu operation
19

ANGER PROCEDURE
Theophile Anger, used
longitudinal flaps on either
side of the urethral groove
and permitted them to
overlap without "denuding".
This manoeuvre theoretically prevented the overlapping between the
urethral and skin closures and thus reduced the rate of fistula formation
20

DUPLAY PROCEDURE – STAGE 1
In first stage, ventral transverse incision
closed longitudinally to correct the
chordee was done.
21

DUPLAY PROCEDURE – STAGE II
In the second stage he incised the ventral
skin on either side of the urethral groove.
This flap, namely the "urethral groove",
was tubularised.
The edges of the outer skin were sutured
over the tube.
22

OMBREDANNE PROCEDURE
Advised performing a large round flap
to create an urethra.
The defect in the ventral penile shaft was
covered by a dorsal preputial flap
which was brought by the philosophy of
the buttonhole technique.
It was a single-stage repair with a
reasonable rate of complication.
23

SURGERY- GOALS
Correction of the penile curvature to ensure a long, straight erection
Advancement of the urethra to ensure normal passage of urine and semen through
the glans
Creation of a cosmetically pleasing penis
25

SURGERY – CORRECTION AIMS
1. Abnormal ventral curvature or chordee, by orthoplasty
2. Abnormal proximal meatal insertion, by urethroplasty
3. Abnormal looking glans penis, by glanuloplasty and meatoplasty
4. Abnormal looking prepuce, either by circumcision or prepuce reconstruction
26

SURGERY- TIMING
Factors to be kept in mind:
1. Psychological effects of surgery
2. Anaesthetic risk of the child
3. Degree of penile development resulting in successful repair
27

SURGERY-TIMING
Genital awareness begins by 18 months of age and progresses with advancing age.
Repair below 12 months of age associated with good psycho social outcomes.
Also associated with lesser complications.
Optimum timing – 6-12 months of age
28

PREOPERATIVE ANDROGEN STIMULATION
Small glans size (< 14 mm width) associated with increased risk of complications.
DHT, hCG or testosterone have been used to increase the size of the glans and penis.
Pros: Decreased tension during glansplasty, increased amount of tissue available for
urethroplasty
Cons:
Aggressive behavior, increased erections, skin discoloration and secondary male
characteristics (Transient – resolve with in 6 months of last dose.
Androgen insensitivity leading to abbreviated response.
Poor wound healing. Increased risk of bleeding due to angiogenesis.
29

ANDROGEN STIMULATION - CAMPBELL
Testosterone cypionate intramuscular
5 and 2 weeks before planned surgery
with glans of < 15 mm.
30

ANAESTHESIA
Regional anaesthesia is preferred
With circumferential penile block, pain control is improved
Pudental nerve block is an alternative to caudal block.
Ketorolac (0.5mg/kg iv) as a single dose 30 min before concluding the procedure is
effective.
31

PENILE CURVATURE/CHORDEE
Donnahoo :4 groups based on etiology
1. Skin tethering
2. Dysgenetic Buck and dartos fascia
3. Corporal disproportion
4. Congenitally short urethra
32

CHORDEE TYPES
Two types of chordee associated with hypospadias.
Superficial or Skin Chordee
Deep or Fibrous Chordee
33

ARTIFICIAL ERECTION TEST
Done after penile degloving.
Tourniquet applied at the penoscrotal
junction.
Saline is injected through the Butterfly
needle for creating erection.
Pharmacological erection is alternative.
Chordee is then measured using
Goniometer.
34

GONIOMETER
35

CHORDEE-CATEGORIES
< 30 degrees – Mild
30-45 degrees – Moderate
> or = 45 degrees - Severe
36

MANAGEMENT
OF PENILE
CURVATURE
37

SUPERFICIAL CHORDEE
Occasionally present in patients with
distal hypospadias
It is subcutaneous, proximal to the
meatus and can be corrected by
mobilization of the skin proximal to the
meatus
38

DEEP CHORDEE
The other type of chordee is commonly associated with proximal hypospadias.
It is usually deep, fibrous and located distal to the meatus.
There are three basic techniques to correct this type of deep, fibrous chordee.
1. Dorsal plication
2. Heineke Mikulicz Procedure
3. Split and Roll technique
39

DORSAL PLICATION
Abnormal ventral curvature corrected by
dorsal plication.
Disadvantage – Penile shortening.
40

HEINEKE-MIKULICZ TECHNIQUE
Useful in proximal hypospadias where
the chordee is distal to the EUM.
A transverse incision is made and excision
of the fibrous bands can be carried out,
Then, the incision is closed in Heineke-
Mikulicz fashion.
41

CORPORAL LENGTHENING PROCEDURES
When Chordee > 30 degrees.
Repair done as two stage procedure.
Multiple ventral tunica albugenia
corporotomies followed by tunica
vaginal or dermal grafting is done
42

CORPORAL LENGTHENING PROCEDURES
43

SPLIT AND ROLL TECHNIQUE
This involves placating the dorsal albuginea leading to corporal rotation, as first
described by Koff and Eakins (l984).
Deeter (l999) added midline ventral splitting and called it the "split and roll“
technique.
44

MANAGEMENT ALGORITHM
45

DISTAL HYPOSPADIAS REPAIR
More than 200 techniques have been described.
90% of the time TIP repair is done by pediatric urologists for distal hypospadias.
80% for mid shaft repair.
46

TUBULARIZED INCISED PLATE REPAIR

(a) Horizontal line indicates
circumscribing incision to deglove the
penis. Verticallines indicate incisions
along the lateral margins of the urethral
plate.
(b) Glans wings have been mobilized. c
(c) Relaxing incision widens the urethral
plate.
48

(d) Urethral plate is tubularized with a
two-layer running subepithelial
absorbable suture, beginning at
approximately the midglans level.
(e) A dartos pedicle flap obtained from
the dorsal prepuce and the shaft skin is
buttonholed and transposed ventrally to
cover the entire neourethra.
49

(f) Glanuloplasty begins with
approximation of the glans wings at the
corona. The mucosal collar is also closed
in the midline with subepithelial stitches.
(g) Skin closure using subepithelial stiches
50

TUBULARISED INCISED PLATE REPAIR-SNODGRASS
51

MEATAL ADVANCEMENT AND GLANULOPLASTY
(MAGPI) PROCEDURE

PATIENT SELECTION
A glanular, coronal or distal location of the meatus
A mobile distal urethra
No chordee
Minor chordee and glans tilt are relative contraindications.
Megameatus intact prepuce (MIP) variant is a contraindication.
53

MAGPI
(a) Circumferential subcoronal incision 8
mm proximal to the meatus.
(b-d) Excision of bridge of tissue between
meatus and glanular groove and
transverse Heineke-Mikulicz closure.
(e, f) Two-layer closure of the glans
edges reconfigures a conical meatus.
54

MAGPI
(g) Sleeve reapproximation for skin coverage
55

URETHRAL ADVANCEMENT, GLANULOPLASTY
AND PREPUTIOPLASTY (URAGPI)

URAGPI
(a) Triangular configuration of incisions
around the meatus and the glanular
groove.
(b) Dissection of the urethra from the
glans, corpora cavernosa and ventral
skin. Lateral incisions along the coronal
sulcus.
57

URAGPI
(c) Fixation of the meatus to the tip of
the glans and reconstruction of the glans.
(d) Vertical stitching of the transversally
incised ventral parts of the prepuce
58

THIERSCH-DUPLAY PRINCIPLE
(a) A longitudinal midline incision is made at 12 o'clock. This incision is closed transversely in
Heineke-Mikulicz fashion.
(b) A subcoronal incision is made around the glans penis.
(c) An incision is made dorsally, laterally and ventrally around the meatus.
60

THIERSCH-DUPLAY PRINCIPLE
(d) The urethral plate is tubularized over the catheter using a running suture.
(e) The glanular skin is approximated with horizontal mattress interrupted 7-0 polyglycolic
sutures.
(f) The available skin and its blood supply dictate the final closure
61

THIERSCH-
DUPLAY
PRINCIPLE
(GAP- GLANS
APPROXIMATION
PROCEDURE)
62

PYRAMID PROCEDURE – FOR MIP VARIANT

MIP VARIANT
Megalomeatus and Intact prepuce variant
- Prepuce is normal, often hides the hypospadias
- Distal hypospadias with deep glanular groove
- Duckett and Keating proposed the Pyramid procedure for MIP variant in 1989.
Why not MAGPI?
Leads to patulous meatus and splaying of urinary stream
64

PYRAMID PROCEDURE
(a) Tennis-racket incision.
(b) Mobilisation of the distal urethra.
(c) Urethroplasty and glanuloplasty
65

MIP-POST CIRCUMCISION STATUS- HILLS PROCEDURE
(a) Creation of meatal-based flap.
(b) PeniIe skin is mobilized; glans flaps
and perimeter-based flap and
development.
(c) Skin flap is excised, leaving its
vascularised mesentery
66

MIP-POST CIRCUMCISION STATUS- HILLS PROCEDURE
(d) Reconstruction of neourethra
(e) Coverage of neourethra suture line
by the vascularised mesentery.
(f) Appearance after approximation of
glanular wings
67

MIP-NOMURA’S
PROCEDURE
68

MEATAL BASED FLAP-MATHIEU TECHNIQUE

PATIENT SELECTION
The ideal candidate should have:
A straight penis - However, a glanular tilt and/or limited distal chordee can be easily
corrected by the techniques of Nesbit's or Baskin's dorsal plication methods.
A wide glanular groove is very important
Contraindications:
1) Midshaft or proximal-shaft types, severe chordee.
2) Cone-shaped glans with a shallow groove.
3) A very small amount of unhealthy skin over the distal urethra (this may be the most
important factor).
70

MATHIEU TECHNIQUE
(a) Lines defining the urethral plate are
drawn.
(b) Incision along the marks; preparation
and mobilization of glanular wings
71

MATHIEU TECHNIQUE
(c) Creation of the neourethra by means
of subcuticular running sutures.
(d) Prepared Byars flaps are sutured in
the midline
72

MODIFIED
MATHIEU Y-V
GLANULOPLASY
73

MAVIS –
MATHIEU AND
‘V’INCISION
SUTURED
74

MANAGEMENT ALGORITHM
76

PROXIMAL HYPOSPADIAS-ONE STAGE REPAIRS

ISLAND ONLAY HYPOSPADIAS REPAIR –
ASOPA PROCEDURE

ISLAND ONLAY
HYPOSPADIAS
REPAIR
(a) The apex of glanuloplasty
is identified (marked by dots).
A subcoronal incision encircles
the glans and continues
around the urethral plate.
(b) The penile skin is dropped
back and the glanular wings
are mobilised.
(c) The inner preputial onlay
flap with its pedicle is
developed
(d) Suturing the onlay flap to
the urethral plate
79

ISLAND ONLAY
HYPOSPADIAS
REPAIR
(e) The glanuloplasty is the
completion of the medial
rotation of the glanular wings
that has failed during
development
80

TUBULARIZED PREPUCIAL ISLAND FLAP
* Useful when there is severe deep chordee which needs the excision
of the urethral plate

TUBULARIZED
PREPUCIAL ISLAND
FLAP
(a) A Y incision is made on the
glans and a subcoronal
circumferential incision is
made and an artificial
erection is performed.
(b) Meticulous excision of any
chordee or fibrous bands is
carried out. Urethral plate
excised.
(c) A 1.5-cm-wide rectangular
flap is prepared.
(d) The flap is tubularised.
82

TUBULARIZED
PREPUCIAL ISLAND
FLAP
(e) The lower end is sutured to
the meatus and the upper
median flap is sutured to the
upper end of the tube.
(f) A small V is excised from
the tip of the tube.
(g) The mobilised glans wings
are rotated medially and
three transverse mattress
sutures maintain firm
approximation of the glanular
wings in the midline.
(h, i) De-epithelialisation of
skin to protect the neourethra
83

ONLAY-TUBE-ONLAY MODIFICATION OF
TRANSVERSE PREPUTIAL ISLAND FLAP
* Useful when there is severe deep chordee
**Combines ventral lengthening procedure with proximal
hypospadias repair

ONLAY-TUBE-
ONLAY PREPUTIAL
FLAP
Correction of ventral deep
chordee with excision of
urethral plate.
Ventral corporal lengthening
procedure by transverse
excision
Grafting using cadaveric
pericardium (c)
Onlay flap of tunica vaginalis
(d)
85

ONLAY-TUBE-
ONLAY PREPUTIAL
FLAP
In the same setting,
Transverse tubularised
preputial onlay flap is taken
and neourethra is formed.
86

MODIFIED ASOPA PROCEDURE
* Original Asopa is associated with Penile torsion
** Penile torsion is averted

MODIFIED ASOPA
PROCEDURE
Here the tubularised inner
prepucial skin is not separated
from the dorsal penile skin.
Vertical Incision is made for
rotation of the flap ventrally.
88

MODIFIED ASOPA
PROCEDURE
Neourethra is brought
ventrally and sutured.
Excess penile skin is excised
and skin sutured.
89

KOYANAGI-NONOMURA REPAIR
* Useful in severe perineal hypospadias

KOYANAGI-
NONOMURA
REPAIR
Step 1: Outlining the Skin Incision and Dartos Mobilization

KOYANAGI-
NONOMURA
REPAIR
Step 2: Chordectomy and Creation of Parameatal Foreskin Flap

KOYANAGI-
NONOMURA
REPAIR
Step 3: Bisecting the Glans and Creation of Glanular Wings

KOYANAGI-
NONOMURA
REPAIR
Step 4: One-Stage Urethroplasty with Parameatal
Foreskin Flaps

KOYANAGI-
NONOMURA
REPAIR
Step 6: Glanulomeatoplasty and Byarsization of the Dorsal Foreskin
and Its Subcutaneous Tissue for Skin Closure

LATERAL BASED FLAP
* Similar to Orandi Procedure for stricture urethra

GRAFTS FOR ONE STAGE REPAIR
* Prepucial skin, Buccal mucosa and bladder mucosa are commonly
used

TISSUES FOR
THE
CORRECTION OF
HYPOSPADIAS
99

GRAFT MATERIAL AND BIOLOGY
Imbibition: The free-graft material initially thrives by diffusion of nutrients between
the donor and recipient sites and this phase lasts for 48 h. During this period
Inosculation: It occurs during the period of imbibition where new blood vessels are
formed to nourish the graft.
Revascularisation: Occurs between day 2 and 4. Blood recirculation is re-established
to the graft, and by day 4-5lymphatic drainage is restored.
Ideally, the donor material should allow these processes to occur efficiently, and this is
helped by graft immobilization for 7-10 days.
100

BUCCAL MUCOSAL GRAFT
(a) As an Onlay patch
(b and c) as Compound tube with
preputial graft
(d) Tube urethroplasty with two buccal
mucosal grafts
101

FIRST STAGE
The first-stage is crucial as it lays the foundation for the performance of a straight,
aesthetic and normally functioning penis in the second stage.
This stage involves:
1. Chordee correction,
2. Harvesting of the graft,
3. Laying down and anchoring of the graft and
4. Immobilisation of the graft by an appropriate dressing
103

CHORDEE CORRECTION
(a) Excision of urethral plate and all
fibrous tissue.
(b) Ventrally, clefting of the glans should
be deep enough to clearly visualise the
distal ends of the corpora cavernosa
104

LAYING DOWN THE GRAFT AND IMMOBILISATION
Graft is tailored upto the size of the
defect and anchored to the graft bed.
Graft should snuggly fit and should not
be overgenerous.
Graft immobilised with a 'tie-over'
pressure dressing prior to compressive
foam dressing
105

SECOND STAGE
U shaped grafted skin is
incised and tubularised.
The marked urethral strip
should be 3 times the
diameter of the catheter.
This is approximately1.5 cm in
a child and 2.5 cm in an
adolescent.
Intermediate layer is raised to
cover the neourethra.
Skin is sutured.
106

PROTECTIVE INTERMEDIATE LAYER
1. Dorsal subcutaneous tissue from prepuce
2. Tunica vaginalis
3. Dartos flap
4. Skin de-epithelialization and double breasting
5. External spermatic fascia
107

COMPLICATIONS
* Divided into Early complications and Late complications

EARLY COMPLICATIONS
1. Infection
2. Oedema
3. Erection
4. Ischaemia and loss of skin flaps
5. Catheter blockage
6. Bladder spasm
109

LATE COMPLICATIONS - URETHRA
1. Meatal stenosis
2. Retrusive meatus
3. Urethrocutaneous fistula
4. Glans dehiscence
5. Urethral stricture
6. Acquired megalourethra
7. Urethral diverticula
8. Recurrent curvature >30 degrees
110

URETHROCUTANEOUS FISTULA - CAUSES
1. Rough tissue handling,
2. Use of poorly vascularised tissues in repair,
3. Use of very thin or fibrotic epithelium or skin,
4. Type and size of suture material (e.g.PDS inside the urethra or non-absorbable
material),
5. Infection and
6. Distal stenosis.
111

TREATMENT OF FISTULA
- Insert a Foley catheter into the bladder for 10-14 days and give appropriate
antibiotics until tissue induration and inflammation disappears.
- No surgical repair be considered for 6-12 months. This is the minimum time required
for complete wound healing and to allow for full resolution of scar tissue.
- Recognise the presence of a urethral diverticulum or distal urethral stricture
112

FISTULA REPAIR – EXCISION OF FISTULA TRACT
Full length of the fistula tract should
be excised.
Fibrous tissue should not be left out.
Healthy margins should be
obtained.
113

FISTULA CLOSURE – SKIN FLAPS
114

FISTULA
CLOSURE- SKIN
PLUG
115

FISTULA REPAIR
116

MODIFIED CECIL CULP PROCEDURE FOR FISTULA
1. Utilizes the penile mobility to place the repair in a scrotal location.
2. This ensures a well covered suture line with vascularized tissue that is free of
tension.
117

MODIFIED CECIL
CULP PROCEDURE
(a) The edges of the urethral
fistula are approximated and
closed over with a second
layer.
(b) The scrotal dartos layer is
approximated lateral to the
urethra, providing a third
layer of coverage.
(c) The skin edges of the
penile and scrotal incisions
are approximated.
118

MODIFIED CECIL CULP PROCEDURE
119

MEATAL STENOSIS
Meatal dilatation is always worth a try.
If failed, dorsal meatotomy or meatoplasty can be done.
If the stenosis is associated with BXO changes, steroid ointment should be used in
conjunction.
120

URETHRAL STRICTURE
Dilatation or visual urethrotomy is usually only palliative in majority of the patients.
Short segment strictures, buccal mucosal graft can be applied.
In case of long segment strictures, two stage buccal mucosal graft repair popularized
by Bracka is preferred.
121

BMG GRAFT-
VENTRAL ONLAY
122

BMG GRAFT-
DORSAL INLAY
123

URETHRAL
DIVERTICULUM
Urethral diverticulum is laid
open.
Excess urethra is excised.
Urethral closure done over
catheter.
124

HYPOSPADIAS ‘REDO’ SURGERY -PRINCIPLES
(1) Ascertainment and possible correction of penile chordee;
(2) Replacement of the defective urethra using either local well-vascularised tissues or
free grafts (essentially buccal mucosa);
(3) Reconstruction of the ventral aspect of the penis (ventral radius), which includes
meatoplasty, glanuloplasty, spongioplasty and shaft skin cover.
125

SALVAGE PROCEDURES
For Distal hypospadias:
1 Mathieu Procedure
2. Thiersch- Duplay Procedure
3. Snodgrass urethroplasty
For Proximal hypospadias
1. Buccal mucosal graft urethroplasty
2. Bladder mucosal graft urethroplasty
3. Onlay Island graft
4. Two stage urethroplasty
126

HYPOSPADIAC CRIPPLE
Patients with the most serious problems are often described as hypospadiac cripples.
Symptoms are, in varying degree, of chordee, fistula, stricture and sexual dysfunction.
For the older repairs, there may be the added complications of a hair-bearing
neourethra.
From the surgical point of view, the urethra is usually too short, strictured and made
of poor material.
There is inadequate residual skin to make a comprehensive reconstruction.
127

HYPOSPADIAC CRIPPLE
Treatment is usually based on Two stage
repair using buccal mucosa or bladder
mucosa.
Complication rates are as high as 33%
128

THANK YOU
129

Pediatric urology:Hypospadias- management

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