This document provides information about hypospadias, including:
- The embryology of normal penile development and how hypospadias occurs due to abnormal anterior urethral and penile development.
- The clinical features of hypospadias such as an abnormal urethral opening along the ventral penis and abnormal ventral curvature. It can be associated with other genital anomalies.
- The various surgical techniques used to repair hypospadias depending on its location, from distal techniques like MAGPI to techniques for more proximal hypospadias like tubularized island flaps or multistage repairs.
- Post-operative considerations like catheters, dressings and potential
5. Embryology:
Genital tubercle at caudal end of cloaca
Develops a groove on its ventral surface
Corpus spongiosum
8 weeks
9 weeks
Urogenital membrane
Coronal meatus
6 weeks
Urethral mucosa
Definitive urethral groove
Genital folds fuse progressively over urethral groove posterior to anterior, thus advancing meatus to
coronal position
Elongates
Urethral plate
Followed by mesodermal cuff
Penoscrotal meatus
Swellings on either side, Genital folds
12 weeks
Penile fascia
Glans forms around distal urethra, ectodermal ingrowth forms glanular urethra
6. Hypospadias
abnormality of anterior urethral and penile development in which the
urethral opening is ectopically located along the ventral aspect of the
penis proximal to the tip of the glans.
7. Clinical features
Abnormal meatal opening along ventral aspect of penoscrotal tissue or
even on peineum
Abnormal ventral curvature and developmental anomaly of ventral penile
tissue (skin upto urethral plate)
Hooded prepuce (“feminization of male genitalia”)
Praurethral sinuses
9. Association with syndromes
approximately 200 recognized syndromes
Hand-foot-genital syndrome
OPITZ syndrome
WAGR syndrome
(Wilm’s tumor, Aniridia,Genital anomalies,mental Retardation)
10. Incidence & etiology:
1 in 100 to 1-400 live male births
Etiology; unknown
Believed as multifactorial process
GENETIC FACTORS:
Gene mutations
ENVIRONMENTAL FACTORS:
Increasing maternal age
Fertility drugs
Antiepileptic drugs
Low birth weight
Pre-eclampsia
11.
12. Chordee
Abnormal ventral curvature of penis
Hypoplasia and longitudinal shortening of ventral
tissue(skin,urethra,fascia,corpora)
Mesenchymal tissue(C.S and fascias) ; fibrous tissue
Chordee without hypospadias
Rare,late in children as downward curved erection
Normal meatus position and hypoplastic shortened ventral tissues
13. Types of chordee
Devine and Horton classified chordee into
type I (skin tethering)
type II (fibrotic dartos and buck’s fascia)
type III (corporal disproportion)
type IV (congenital short urethra)
14. Approach towards management:
preoperative assessment :
History
general physical examination
local examination and examination of associated deformities
Systemic examination
15. Local examination:
Measure:
the size of the phallus
glans cleft (flat, incomplete, or complete)
location and size of the meatus (type of hypospadias and meatal stenosis or mega-meatus)
urethral plate width (<1 cm or ≥1 cm)
type of chordee
prepuce (complete, incomplete, circumcised)
penile torsion (clockwise, anticlockwise)
shape of the scrotum (normal, penoscrotal transposition)
associated anomalies (cryptorchidism, inguinal hernia, persistent Mullerian structures)
urogenital tract anomalies such as pelviureteric junction (PUJ) obstruction, vesicoureteric reflux
and renal agenesis which should be excluded by ultrasonographic scan in every hypospadias
patient.
Proximal hypospadias with cryptorchidism, enlarged utricle, or penile size <2.5 cm should be
investigated for intersex disorders by ultrasonography, hormonal profile, and karyotyping
19. ■ normal micturition while standing
■ natural appearance
■ normal sexual function
20. Principals of repair
Surgical expertise
Minimal tissue trauma
minimal/pin-point use of cautery
well-vascularised tension free repair of all layers with epithelial
inversion(water proofing)
23. Surgical techniques
3 categories
Historical procedures:
Known by author’s names,
high complication
poor aesthetic results
Textbook operations:
Meatoplasty and glanuloplasty(MAGPI) for coronal & glanular hypospadias
Flip-flap for distal shaft hypospadias
Inner preputial island flaps for more proximal hypospadias
High complication rate,couldn’t achieve slit like meatus
Modern techniques:
Tubularization of remaining plate or it’s replacement with a graft in two stage procedure
24.
25. Options or chordee
release/orthoplasty
Simple degloving of penile skin
Nesbit operation
Heineke mikulicz operation
Dorsal midline plication
Tunica albuginea plication
Corporal rotation
Split & roll technique
Total peile disassembly- perovic
27. GAP/GRAP
(Glanular Approximation Procedure/Glanular
Reconstruction And Preputioplasty)
Uses: distal hypospadias with glanular
groove
Anesthesia: both G/A and caudal
anesthesia
Glans groove tubuarized
Distal suture is critical in determining
location of meatus
2 layered closure of neourethra with
water proof closure
Results in slit like meatus
Preputital reconstruction with
independent inner & outer layer
closure
29. MAGPI (Meatal Advancement and GlanuloPlasty
Incorporated)
glanular and coronal hypospadias
Duckett
Meatal regression if used in immobile
urethral meatus.
Complications: meatal retraction,stenosis
and fishmouth meatal opening
No slit like meatus achieved
30. Tubularization techniques:
Thiersch (1869) and Duplay (1880)
initial descriptors of the simple urethral plate tubularization technique;
known as the Thiersch-Duplay urethroplasty.
31. Tubularized Incised Plate (TIP
Urethroplasty)/ Snodgrass Repair
Initially used for distal, but used
equally for midshaft and
proximal
easily performed, with few
complications and results in a slit
like meatus
Complications: meatal stenosis,
persistent fistula, functional
urethral obstruction and
persistent chordee
5-35 % in distal hypospadias and
upto 65 % in proximal
hypospadias.
39. Inner Preputial Island Flap
Inner preputial skin
islanded and used as:
A complete tub
Onlay flap
Sutured to urethral
plate to augment
another technique
41. Sutures, Dressings, Urinary Diversion,
Catheters
Absorbable sutures: 6-0 or 7-0
vicryl(polyglactin)
PDS(poly Dioxanone)
Monocryl(poliglecaprone)
Usually no dressing; various forms of dressings (tegaderm/foam)
Stent must be placed; sutured to glans( leaves marks); a balloon catheter taped with
abdomen (preferrable)
8FrG silicon catheter used in paediatric age group
Rubber catheter avoided
Fine bore tubing working on siphon action can be used
Postoperatively:
ketoconazole( prevent androgen production from testes and adrenals, prevents erection) or
CYPROTERONE ACETATE
Oxybutynin (DITROPAN)(prevents bladder spasm)
46. Complications:
Meatal problems:
Stenosis
Techniques:
cicumferential suture line at meatus
Urethral plate midline incisions extends onto glans
Minor: dilatation
Severe: meatoplasty or redo with two stage procedure
47. Complications:
Striture:
At junction of neourethra and original urethra
Entire urethral repair
Minor and early menifestation: dilatations
Severe or late occurrence : urethroplasty/redo
48. Complications:
Skin problems:
Minor skin assymetries (correction with surgery)
Lichen sclerosus et atrophicus( balanitis xerotica obliterans)
Pre-malignant for SCC
Results in phimosis, involving repair,it may cause meatal stenosis, strictures
Minor: topical steroids
Severe: excise all skin and redo in two stage with buccal graft (no skin graft as
recurrence of LSEA is high)
49.
50.
51. Treatment Algorithm
If deep sulcus on
glans?
Yes
If significant
chordee
Yes
Can curvature be
corrected by dorsal
plication
Yes
No
No
If a dense bar distal
to meatus
Yes
TIP urethroplasty
No
GAP
No
Two staged Repair
If foreskin
repairable?
Yes
preputioplasty
No
Circumcision
5 layers: skin: expandable,pliable,high vascular network, separate blood supply than the deep fascias and tissues
Superficial fascia(dartos fascia);continuation of membranous layer of groin and perineum(colles fascia),abundant vascular supply(superficial dorsal penile vein and arteries)
3rd: loose connective tissue( tela subfascialis of eberth)
4th: deep buck’s fascia enclosing deep dorsal penile arteries and vein and corpora spongiosa and corpora cavernosa
5th: tnica albuginea; double layer,covers corpora cavernosa only
SRY Gene; Sex-determining zone of y chromosome
Testicular cells(lydig cells; FSH,LH/ sertoli cells: testosterone)
Failure of Dihydrotestosterone stimulation of genital tubercle; hypospadius
Urethral plate
endodermal growth from cloaca into ventral aspect of gential tubercle
Corpora and penile fascia
mesenchymal tissue grows along urethral plate
Genital folds
fuse ventrally; advance meatal opening from proximal to distal
An ectodermal ingrowth meets tubularized urethral plate at coronal level
prepuce
skin fuses ventrally
Based on meatal opening location ( more specifically) after release of chordee
ANTERIOR,MIDDLE AND POSTERIOR ,classification is most acceptable –OXFORD plastic surgery
Tolerance to anesthesia: 6 months
Uncooperative after 18 months to 3 years so never do surgery during this period
Difference in penile size between 1 year and preschool age: 8 mm only
6–18 months; most suitable age
next window of surgery is preschool age (3-4 years)
Medical or hormonal management : HCG, testosterone topical or injectible
1. Meatal advancement techniques (MAGPI)
2. Onlay tchniques (Mathieu;s flip flap, inner preputial island onlay)
3. Inlay technique: a vascularized flap tubed as a neourethra (MUSTARDE’S REPAIR which is similar to Mathieu’s, duckett’s inner preputial island tubularised flap, harris technique using inner preputial skin for tube flap as neourethra and outer preputial flap for skin cover.)
If groove is not deep: TIP is used
Always check for chordee, can use artificial erection test
(a) A circumferential incision 0.5cm below the corona. (b) The transverse bridge of the tissue distal to the meatus is sharply incised. (c) A Heineke-Mikulics closure opens and advances the meatus. (d) The ventral meatal edge is pulled distally and the exposed glans edges are trimmed and approximated. (e) Glanuloplasty is performed with subepithelial sutures to leave a rounded, conical glans. (f) The dorsal skin is transferred ventrally, excess skin is excised.
based on the assumption that midline incision into the urethral plate may widen it sufficiently for urethroplasty without stricture.
two important criteria to achieve good results: the urethral plate should not be less than 1 cm wide and there should be no distal deep chordee
After torniquet placement,traction suture is placed in the glans just beyond the anticipated neomeatus, circumscribing skin incision proximal to the meatus,shaft skin degloved to penoscrotal junction.
"U" shaped incision, urethral plate separated from glans wings, relaxing incision using scissors in midline from within meatus to end of the plate, stent secured into the bladder, Tubularization of urethral plate completed with 2 layer closure.
adjacent dartos tissues used to cover the neourethra,then a dartos pedicle is developed from the dorsal shaft skin, button-holed, and transposed to the ventrum to additionally cover the repair.coronal skin approximated after glanuloplasty.
Byars' flaps are created from the preputial skin to mimic the median raphe
The stent is removed approximately 1 week later.
Very effective
Sagitally slit meatus
Least complication rates
Progression of snod grass repair
If urethral plate;poorly developed, single stage snod grass is converted into staged procedure
1st stage,incision is given in midline, it is grafted
2nd stage, 3-6 months later; without midline incision, urethral plate is tubularized
for coronal, subcoronal and distal penile defects
measure the length of the defect from the urethral native meatus to the glans tip, After a straight penis is either appreciated or achieved with an orthoplasty technique, the premeasured segment of penile skin proximal to the meatus is mobilized off of the urethra in a proximal to distal direction
Patulous meatus
Proximal/distal variety with flat glans or severe chordee: two staged techniques
Midline incision given from proposed meatus to downward ectopic meatal opening, lateral incisions given,midline incision through glans is deepened enough so corpora become separated,chordee released and all scarred tissue and urethral plate excised,defect covered with a graft(preputial skin or buccal mucosal graft..it is held in place by tie over dressing.
After 6 months,2nd stage; preliminary erection test to check for any chordee,then u shaped incion and tubularization performed,glansplasty
high complications as too much dissection, risk of hemorrhage and flap necrosis
Cicumferential suture line at meatus tip & junction of native and neourethra
tip of the neo-meatus is identified
midline vertical incision in the glans,left open for secondary epithelialisation
subcoronal incision around the glans, incision continues on either side of the urethral plate, then up on either side of the glanular groove to the apex of the glans
skin is degloved
1-cm wide onlay flap is prepared from the inner prepuce. The onlay flap is sutured into place. The glans should be drawn together setting up the first stitch of the glansplasty ventrally at its apex.
The mobilized glans wings are rotated medially around the neo-urethra.
1.More proximal the original meatus,greater the risk,it can be repaired if distal to leak,urethral caliber is adequate,otherwise redo it.
2.
Balanitis: inflammation of glans
Phimosis: tight foreskin around glans that can not be pulled back
Dorsal plication has shortening effect on phallus, not advised for severe chordee
Asses relation of glans sulcus to meatus, if both adjacent, do GAP, f some dense tissue inbetween two do TIP
If hypospadiac meatus opens directly into glans groove: MEGAMEATUS
After GAP,TIP or two stage repair: assess foreskin condition