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Hypospadias Definition
It
Abnormally located ventral meatus. Abnormal Ventral penile curvature (Chordee). Abnormal foreskin* (Deficient ventrally).
Hypospadias History
First
described by Galen in the second century. Antyl described the first surgical repair, by amputating the penis distal to the hypospaedic opening!! In the second half of 19th century, most of surgical procedures were first described. (Thiersch, Duplay, Bouisson,..)
Hypospadias Incidence
1
in 300 live male birth. This showed recent increase in incidence, which is believed to be related to increased referral of minor forms, for which parents would not previously seek medical advice. However, recent studies showed that the recent increase in incidence in US may ba attributed to (Oestrogenic pollution).
Hypospadias Pathophysiology
Developmentally,
Hypospadias Pathophysiology
Fusion
of the urethral folds in the midline, in a proximal to distal fashion, results in the formation of proximal & penile urethra (endodermal lining). 2 theories are proposed to explain the development of the glanular urethra (ectodermal lining)
Hypospadias Pathophysiology
States that the glanular urethra develops as ingrowth of the ectoderm (ectodermal pit) that grows proximally to join the penile urethra.
Hypospadias Pathophysiology
Endodermal
differentiation theory:
Recent researches done by Baskin et al., showed that the entire urethra develops in a proximal to distal fashion, with the endodermal cells capable of differentiating into stratified squamous epithelium as a result of mesenchymal/endodermal cell signalling.
Hypospadias Aetiology
Classification
Classification
Megameatus intact prepuce variant MIP: In this uncommon variant, the patient presents with completely intact prepuce, and only hypospadias is detected on complete foreskin retraction.
Chordee without hypospadias: Complete degloving will mostly result in a hypospaedic meatus.
Associated anomalies
Undescended
testis (5-10%, rising to 50% in proximal hypospadias. Persistent Utricle (20%). Urinary tract anomalies (only 2%). Severe cases can be presented as ambiguous genitalia, with bilateral undescended testicles.
Associated anomalies
Up
to 50% of patients with severe hypospadias with cryptorchidism have underlying genetic, gonadal, or phenotypic sexual abnormalities. Therefore, we need to exclude:
CAH
Complications of Hypospadias
Psychological
Management of Hypospadias
Clinical
diagnosis and assessment of the type of hypospadias. When to operate. How to operate (selection of technique). Follow up & dealing with complications.
When to operate?
Elective
procedure, general guidelines, between 6 to 18 months. Size of the phallus and adequacy of tissue needed for repair. Associated anomalies.
General principles
Hypospadias
repair in general includes 3 main entities: 1. Orthoplasty (correction of chordee). 2. Urethroplasty. 3. +/- preputioplasty.
General principles
Selection
Adrenaline
Diathermy
material.
6-7/0 PDS.
Urinary
diversion. Dressing.
General principles
Surgical
technique:
Urethroplasty is better done over a size 8F catheter. Multilayer repair is the rule. Interrupted rather than continuous sutures. Suture lines should not overly each other. Suture under tension = failure. Meatus should never be tight. Cosmetic appearance of the meatus.
There are many underlying cause for chordee. It may be, Skin, urethral plate, fibrosed tissue distal to meatus, or disproportionate corporal growth.
Anterior Hypospadias:
Meatal advancement. MAGPI. GAP procedure. Mathieu (meatal-based skin flip-flap). Urethral mobilization. TIPU.
Posterior Hypospadias:
TIPU. Onlay island flap. Ducketts urethroplasty. free flap techniques. 2-stage, 3-stage techniques.
Complicated Hypospadias:
Another classification
Common operative techniques
One-stage
Multi-stage
Without flaps
With flaps
With flaps
Without flaps
Free flaps
MAGPI
Meatal Advancement with Glanuloplasty Incorporated.
In 1981, Duckett published a simple technique in the management of AH, namely the MAGPI operation.
Patient selection
Application of tourniquet.
The midline glanular cleft incision is made, involving the posterior urethral wall.
Three sutures are applied, anchoring the urethra to the incised glans.
Complete freeing of the flap, and the glanular wings are being prepared.
Mathieu repair
In
1932, Mathieu described his Meatalbased flap technique in the management of anterior hypospadias.
Patient selection.
Starting the flap dissection, the glanular incision is not deepened before the tourniquet is applied.
Now, the glanular incision is deepened after application of the tourniquet. Note the depth is marked by the corporal body.
In the early ninties, W Snodgrass accidently invented the technique of urethral plate incision to accomplish the Duplays principle
TIPU
The idea is to relax the urethral plate using a mid-line incision, so that tubularization of the plate could be achieved without tension.
TIPU
TIPU
Urethral plate incision, balanced so that the depth of incision does not exceed the urethral plate thickness. Again, it should not extend distally till the tip of the glans.
Using the dorsla flap technique, however, with less suture lines, and avoids the circular anastomosis at the base.
Ducketts repair
Involves tubularization of dorsal preputial flap around a catheter, then transferring it ventrally to be anastomosed to the hypospaedic meatus. High rate of complications, many suture lines.
Ducketts repair
Multi-stage techniques
Usually done for proximal or complicated hypospadias. Initially, degloving and correction of Chordee will send the meatus more proximal. Free grafts can be then used ventrally, e.g. buccal mucosal, post-auricular, bladder mucosa Six months later, tubularization of the graft is to be accomplished.
Protection of urethroplasty.
Almost
invariably all Paediatric urologist will use a second, or even third vascular protective layer to protect the initial urethroplasty. The easiest, and richly vascular flap can be obtained from local tissue (dartos fascia flaps).
Pedicled flap
Dorsal Dartos flap
TIPU
FLAP
Urethral plate
Parameatal flap
to the hypospaedic meatus, the corpus spongiosus (CS) tissue fans out till the base of the glans. Careful dissection of tissue can easily demonstrate CS, which then can be utilized as a covering layer above urethroplasty. Spongioplasty
Despite the great advances in hypospadias reconstructive surgeries, many frustrating complications are still met with: Urethrocutaneous fistula Meatal stenosis Disruption Hairy Urethra Urethrocele flap complications (BXO) Recurrent chordee!
Urethro-cutaneous fistula
Disrupted repair
Hairy urethra
Can complicate repairs that include the use of skin lining of the urethra (e.g, Mathieu with long flap, or the classical Cecil Culp operation)
is The problem of complicated hypospadias? 1. Scar tissue rather than healthy one. 2. Loss of tissue (Circumcision). 3. Affected urethral plate.
rather than one stage in severe cases. Use of multilayer repair. Warn the parents about the increased incidence of recurrence.
Hypospadias-like situations
Traumatic urethral injury can happen to children, and can result in severe urethral complications (Hair strangulation)