Hypospadias DEFINITION a congenital anomaly characterized by a ventrally placed urethral meatus in a more proximal position on the midline than its normal position in the glanular part of the penis INCIDENCE 1 in every 200 to 300 male births (G + S) Evidence of increasing incidence Older maternal age Low birth weight improved fertility in men treated for cryptorchidism and hypospadias in the postwar decades The majority of cases occur sporadically (90%) About 5-10% have a family history: Fathers have Hypospadias in 7-10% of patients. Brothers have Hypospadias in 10-20% of patients. In some families multiple members are affected. Whites > Blacks. More common in Italians and Jews ANATOMY Local abnormalities include: 1.Chordee a. formed by residual fibrous tissue of the corpus spongiosum, Bucks and Dartos fascia b. located on the ventral urethra, distal to the urethral meatus, in close contact with the tunica albuginea. c. causes ventral curving of the penis starting as early as the first 6 to 12 weeks of the intrauterine life. d. The more proximal the meatus, the more significant is the curvature. e. The curvature deformity is always present in midshaft or proximal hypospadias cases. Mild chordee may also be seen in almost all cases of distal hypospadias and some cases of coronal hypospadias, excepting the glanular cases. f. When the prepuce is gently pulled, the chordee and ventral skin shortage become visible on the lateral view g. Erection accentuates the curvature h. Other significant causes of the ventral curvature: i. asymmetric distribution of the skin around the penis can originate some degree of curvature both in distal and in proximal hypospadias. In severe cases, there may be a penoscrotal web ii. asymmetry of the tunica albuginea, which accounts for the permanence of the curvature despite the excision of the entire fibrous chordee iii. growth differential between the corpora cavernosa and the corpus spongiosum. 2.Prepuce a. In proximal hypospadias, the prepuce is asymmetric, accumulating on the dorsum of the penis and being deficient on the ventral segment. b. Gives glans a dorsal hooded appearance. c. Width of the ventral deficiency of prepuce = width of dysgenetic band causing the chordee d. The prepuce may be normal in distal hypospadias in up to 7% of cases. Axial kicking of the shaft of the penis occurs in 14% of patients, and is not dependent on the degree of hypospadias. 3.Urethral meatus stenosis a. less frequent in proximal hypospadias(15%) 4.Scrotal abnormalities a. Proximal hypospadias are usually associated with scrotal malformations, such as penoscrotal synaechia, hypoplasia, bifid scrotum and high scrotum implantation. 5.Other associations a. Undescended testes (10%) – female karyotype until proven otherwise if shows combination of undescended testes and hypospadias b. Inguinal hernia (10%) c. Upper urinary tract anomalies (1-3%) – higher risk with proximal hypospadias AETIOLOGY a deficiency of androgens during embryogenesis. CLASSIFICATION 1. Browne, 1936 Classification according to original site of meatus. 2. Barcat, 1973Classified hypospadias according to the new position of the urethral orifice acquired intraoperatively after the surgical removal of chordee This is the preferred classification (Duckett). i. Distal (80%) a) Glandular b) Coronal c) Distal penile d) Mid penile ii. Proximal (20%) a) Proximal Penis b) Penoscrotal c) Scrotal d) Perineal ASSESSMENT Whenever possible, the child should be seen as a newborn. On examination, note: i. meatal position, ii. adequacy of the meatus (if the meatus is too tight a neonatal meatotomy may be required), iii. cryptorchidism iv. ambiguous genitalia v.other congenital anomalies. Whether or not to do an IVP is controversial. Serafin + Georgiade recommend doing IVP if: 1. Hypospadias more proximal, 2. Hypospadias (of any degree) associated with cryptorchidism, 3. Hypospadias (of any degree) associated with any other organ system anomalies. If the patient has glandular or coronal Hypospadias and is otherwise normal, the yield from IVP is too low to warrant the procedure. In cases of ambiguous genitalia, tests include sexual chromatin investigation, karyotype, stimulation test using chorionic gonadotrophin, pelvic sonographic screening and retrograde and urinary urethrocystography, and eventually biopsy of the gonad. HISTORY OF HYPOSPADIAS REPAIRS Original operations were staged, first stage being correction of chordee and second, construction of a neourethra i. Stented SSG urethroplasty o Devised by Novè-Joserand. This technique consisted of a split-thickness graft to fill a channel in the penis. The split-thickness graft required stenting for many months because of the inherent contracture. Stenoses and strictures occurred with this technique, and it was abandoned. o Later popularized by McIndoe, who recommended that the stent be left in place for 6 to 12 months to overcome the tendency for contracture. This technique has many complications and is no longer used. ii. Sutured ventral tube urethroplasty o Thiersche and Duplay performed a two-stage repair in which they first resected the tissue causing chordee and straightened the penis. The penile skin was closed, and months later the urethra was constructed by making longitudinal incisions down the ventral surface of the penis to form a urethra tube undermining the lateral skin flaps and covering the buried tube of skin. The deficiency of this operation was that it never adequately extended the urethra to the tip of the glans. In many cases, adequate tissue for construction of the urethra and coverage of a new urethral tube was not present. o The technique, however, was successfully reintroduced and popularized by Blair and Byars. This operation was probably the most common type of hypospadias repair reported in the literature until one-stage repairs became popular. Browne modified the technique by not making the ventral strip of skin into a tube, but simply leaving it as a strip of skin covered by the lateral skin flaps of the ventral surface of the penis. This ventral strip of skin then tubed itself with normal circumferential growth, and the urethra was formed. This simple technique gained popularity for hypospadias repair, but the complication rate was so high that the operation has been largely abandoned by most hypospadias surgeons. All of these operations ended with a subglandular meatus. iii. Closed ventral tube urethroplasty sutured to a scrotal bed o introduced by Cecil during the mid 1940s, addressed the fact that adequate shaft skin was difficult to obtain in all cases. o after the chordee was released and the penis straightened, the urethra was constructed at a second stage, 6 months later, of tubed ventral penile skin by making parallel longitudinal incisions. o Rather than attempt coverage of the urethra with flaps of penile skin, an incision was made into the scrotum, and the penis was sutured into the scrotal bed. The penis was left in this position for 6 to 8 weeks with the new urethra covered. o At a third stage, the scrotum was released from the penis, leaving normal vascularized scrotal skin present on the ventral surface of the penis. This technique is still useful for certain complicated hypospadias cripples; however, it has largely been abandoned as a primary form of hypospadias repair. o Cannot be recommended because of the undesirable aesthetic appearance of the scrotal skin on the penis and because it is a three-stage operation with a high complication rate. In 1961, C. E. Horton and C. J. Devine, Jr., developed single-stage modern surgical techniques, namely, local skin flaps and free skin grafts, for urethra reconstruction in hypospadias repair, which may be applied to almost any case with different localizations of the meatus. Later, two new methods, advancement of the urethra and preputial island flap techniques, were added to the surgical algorithm. AIMS OF SURGICAL REPAIR One stage procedures are now regarded as being superior to multi-staged repairs. Aims: 1. A normally sited meatus at the tip of the glans. 2. A complete release of chordee with straightening of the penis. 3. A normal urinary stream without spraying. 4. Absence of excessive scar tissue or redundant skin. 5. The ability to produce a normal erection. In all but the most distal forms of hypospadias, the dysgenetic band of fibrous tissue must be removed to correct the chordee. NEVER circumcise a child with hypospadias. The extra skin of the hood of the prepuce dorsally will generally be used for the construction of the new urethra or resurfacing of the ventral skin defect. TIMING OF SURGICAL REPAIR Definitely < 12 years and most say that surgery should be complete before the child starts school. According to Devine (in Mustarde), reconstruction should be complete before the child has any memory of it, yet it should be delayed until the child can cooperate and understand what is said to him. He therefore recommends that surgery should be done around the time of the child’s 2nd birthday. At PHM, between 6 – 12 months 1. post operative care tends to be less complicated 2. size of the penis is almost equivalent to that of a 3 year old child 3. trophic conditions of the skin allow a high degree of safety during operation. 4. Children at that age suffer much less emotionally during the postoperative period than do older children and, as a consequence, parents’ anxiety is also alleviated 5. During that period children are still in diapers and have not been exposed to other people’s observation. 6. more important, in case of complications that require reintervention further correction can be carried out sometime before the second year. At about this time the genital awareness begins and the child becomes more prone to psychological problems. Penis grows more in proportion to baby for 1st six months, if remains small at 6 months, testosterone enanthate 25-50 mg intramuscularly once a month for 3 months has been shown to increase penis size. OPERATIVE PRINCIPLES 1. Orthoplasty Complete correction of chordee mandatory i. Some advocate total resection of fibrotic corpus spongiosum ii. Others raise the chordee as a flap to create the neourethra and in doing so corrects the chordee iii. Dissection along the septum between the corpus cavernosa may be required iv. Nesbit type dorsal plication of tunica albuginea Artificial erection test to confirm complete correction i. Tourniquet around base of penis, inject saline into both corpora cavernosa ii. Local injection using prostaglandin E1 said to give a more physiologic response (ie tendency to overcorrect with saline) 2. Urethroplasty Depends on the level of the meatus following chordee release i. True Glanular hypospadia (no chordee) o Magpi technique o Dividing septum between glanular pit and meatus ii. Distal penile with no chordee o Flip Flap iii. Distal penile with chordee o Mustarde iv. Mid/Proximal/Penoscrotal o Vascularised preputial flap based on dorsal artery of prepuce (tubed or onlay) – vertical, transverse, bileaflet island or penopreputial rotation flap) o Full thickness graft (Horton-Devine) Preputial skin Bladder mucosa – prone to complications Buccal mucosa – prone to stenosis at meatus Postauricular skin - least hairless skin v. Perineal hypospadia o 2 staged procedure Midline scrotal skin for proximal urethra Ventral tube or FTG urethroplasty 3. Meatoplasty Avoid tension neomeatus should be oval and not round. Horton V flap to reduce tendency to circumferential scar stricture 4. Glansplasty Important for aesthetics Tip is usually rotated ventrally (tip tilt) Undermine the ventral half of the glans to correct tip tilt and also this mobilises the glans to allow creation of urethra within the glans Excessive undermining will lead to the soft glans syndrome (unerected glans despite penile shaft erection) 5. Ventral skin coverage Byers flaps of preputial skin Preputial rotation flap is penile torque needs to be corrected SURGICAL METHODS CHORDEE The creation of an artificial erection should be done at the beginning of all operations for hypospadias so as to document the degree of chordee. This is done by the application of a tourniquet to the base of the penis followed by the injection of saline into both corpora cavernosa via a small needle. Often it is found that in a penis thought to be free of chordee when flaccid, the artificial production of an erection demonstrates that chordee is in fact present. Chordee can occur without hypospadias, ie even when the urethral meatus is in the normal position. Classification (Devine and Horton): TYPE I The urethra lies subcutaneously not surrounded by the normal layers of corpus spongiosum, Buck’s fascia or dartos fascia. This may be associated with a mild degree of hypospadias (uncommon). TYPE II The urethra is enclosed by the corpus spongiosum and the chordee is due to the fibrous tissue that lies deep and lateral to the urethra. TYPE III The urethra is enclosed normally by the corpus spongiosum and Buck’s fascia and the chordee is due to abnormal inelastic dartos fascia. TYPE IV Intrinsic fibrous abnormality on the ventral aspect of the corpora cavernosa -very uncommon. TYPE V Congenital short urethra - very uncommon. Surgical treatment of chordee without hypospadias 1. Excision of dysgenetic fibrous tissue. 2. Longitudinal incision in the tunica albuginea ventrally. 3. Transverse ventral incision and insertion of a dermal graft. 4. Excision of ellipse(s) of tunica albuginea from dorsally and placation sutures Surgical Methods Glanular Hypospadia: MAGPI dorsal Meatal Advancement and Glansplasty (MAGPI) procedure indicated for glanular hypospadia. Skin flaps used to reinforce the inferior distal surface. It cannot produce an adequate repair in subglandular cases. Method o After demonstrating that the penis is straight with an artificial erection, a circumcising incision is made. o Skin hooks are placed into the lateral edges of the glandular urethral groove and retracted laterally. o This move raises a transverse band of mucosa that is then incised longitudinally in the midline. o This incision in the dorsal glandular wall of the urethra is then closed transversely with 6-0 chromic catgut. A skin hook is placed in the skin at the margin of the corona in the ventral midline. With distal traction, the edges of the glans are pulled forward and approximated in the midline with subcuticular interrupted 5-0 PDS sutures. o The glans epithelium is closed with interrupted 6-0 chromic catgut. Redundant dorsal prepucial skin can be tailored for skin closure and reapproximated with interrupted 5-0 catgut. o Urinary diversion is not required. This procedure is routinely done in an outpatient setting. Horton Flip-Flap: Coronal/Distal hypospadias without Chordee For distal cases ending at the coronal sulcus, extensive mobilization of the urethra with advancement to the tip of the glans is recommended. For hypospadias cases in which the urethra is subglandular, a “flip-flap” operation is utilized. If the meatus is adequate and there is no chordee, two parallel incisions outlining the urethral plate continue from the meatus to the neomeatus so that the flip flap can be sutured to this glans strip, thereby constructing the distal urethra. Coronal/Distal hypospadias with Chordee When chordee is present, transection of the urethral plate and excision of the underlying dysgenetic tissue are suggested. In such cases, a V-shaped midline glans flap is elevated. Lateral glans wings are closed over the new urethra, and prepucial skin is shifted from the dorsal surface to the ventral surface, covering the penile shaft. If the meatus is small, a meatotomy is performed and the midline V-shaped glans flap advanced into the meatus. After tissue causing chordee is resected, the flip flap is sutured to the midline glans flap to construct the urethra. The lateral glandular wings are used to cover the distal urethra. The prepuce is split and shifted ventrally for resurfacing. These techniques are sufficient for about 90% of all hypospadias cases. In more proximal cases, in which the midline glans flap does not reach the native urethra without causing curvature, a new interposed urethra must be constructed to bridge the gap and meet the glans flap. TIP (Tubularised Incised Plate) – Snodgrass BJU International 2005 Essentially all patients with midshaft and more distal hypospadias can undergo TIP of the urethral plate repair consists essentially of a Duplay- type tubularized repair with the added feature of a full-thickness midline incision extending the full length of the urethral plate. This key point in the operation widens the plate and allows it to be tubularized with no additional skin flaps Concerns are with regards as to whether this leads to meatal stenosis. Experimental studies in animal models show rapid epithelialization of the defect created by a urethral incision but it remains unclear whether experimental findings in a normal, well-vascularized and spongiosum-supported urethra can be reliably extrapolated to the thin atrophic urethral plate tissue commonly encountered in hypospadias. Step 1. A 5–0 polypropylene suture is place into the glans for traction and to later secure the urethral stent. The initial skin incision depends upon whether the family prefers circumcision or foreskin reconstruction, as either can be performed. When circumcision is the desired result care is taken to preserve sufficient inner prepuce so that a so-called 'mucosal collar' can be approximated in the ventral midline after glansplasty. Then the penis is degloved to near the penoscrotal junction. If the foreskin is to be reconstructed the skin incision extends from the corners of the dorsal preputial hood to 2 mm proximal to the meatus. Ventral shaft skin is released until normal dartos tissues are encountered. An artificial erection confirms the absence of ventral curvature, but if there is significant bending a midline dorsal plication is done using a single 6–0 polydioxanone suture placed in the tunica albuginea of the corpora cavernosa directly opposite the point of maximum curvature. Step 2. Next, longitudinal incisions are made along the visible junction of the glans wings to the urethral plate. Proposed lines for incision are first infiltrated with 1 : 100 000 noradrenaline or a tourniquet is used around the base of the penis for haemostasis. After making the skin incision with the 69 Beaver scalpel, I prefer to complete the dissection and glans wings mobilization using tenotomy scissors, taking care both to preserve vascularity to the urethral plate and sufficient thickness for the wings to be securely approximated. Step 3a: The key step in the procedure is midline incision of the urethral plate. This manoeuvre is facilitated by counter-traction maintained by the surgeon and assistant along opposite margins of the plate. Using tenotomy scissors, the relaxing incision is made from within the meatus to the tip of the urethral plate. It should not be carried further distally into the glans. The depth of incision depends upon whether the plate is grooved or relatively flat, but in all cases extends down to near the corpora cavernosa. Figure 3c: A 6 F Silastic stent is passed into the bladder and secured to the glans traction suture. Then the urethral plate is tubularized beginning at the neomeatus, using 7–0 polyglactin suture. The first suture is placed through the epithelium at a point just distal to the midglans so that the meatus has an oval, not rounded, configuration. Tubularization is completed with a running two-layer subepithelial closure, turning all epithelium into the neourethral lumen. Step 3b: I recommend initially maintaining the urethral plate in all proximal hypospadias repairs, as even apparently severe ventral curvature sometimes can be straightened without transecting the plate. The skin incision should be made immediately next to the plate to minimize the risk of incorporating hair follicles into the neourethra. Similarly, the foreskin also can be preserved at the beginning of surgery as curvature is assessed, although in many patients it will be necessary to completely deglove the penis and ultimately circumcise it. My experience with foreskin reconstruction in boys with proximal defects is limited, and a desire to avoid circumcision should not outweigh the need to correct significant ventral curvature that might later impair sexual function. After degloving, the corpus spongiosum alongside the urethral plate is dissected off the underlying corpora cavernosa. This tissue later will be approximated over the neourethra as a barrier layer against fistula, and its mobilization sometimes also lessens the extent of ventral penile bending. Then an artificial erection is created; persistent mild curvature is corrected by midline dorsal plication, as described above. More severe bending next leads to dissection under the entire urethral plate, and if it still persists, to transection of the plate and, in my hands, a staged urethroplasty. Step 4: dartos pedicle flap is dissected from the preputial hood and dorsal shaft skin in patients undergoing circumcision, then button-holed and transposed ventrally to cover the entire neourethra. Glansplasty is a key determinant of the final cosmetic outcome. Over the years my technique has developed, and currently begins with a 7–0 polyglactin suture through the epithelium at the desired point for the ventral lip of the meatus. A second 7–0 suture is placed subepithelially in this same location to further buttress the neomeatus and hopefully prevent partial dehiscence that would result in a larger than normal meatus. No attempt is made to secure the glans to the underlying neourethra. The remainder of glans approximation is then done using interrupted 6–0 polyglactin subepithelial sutures proximally to the corona. It is not necessary to place sutures through the epithelium of the glans, and I have seen a few patients develop suture tracks when a second layer was created. Dripping stent is left in place inside the nappy – usually removed after 1 week. most common complication from TIP urethroplasty is fistulae. prevented by 1. turning all epithelium into the neourethra o 2 layered subepithelial suture closure 2. using sufficient barrier layers o recommend using corpora spongiosum as an additional layer for proximal hypospadias and a dartos flap harvested from the dorsum. 3. avoiding meatal stenosis o most important factor is not to tubularise the urethra too far distally o neomeatus should be oval, not round o Other key manoeuvres include incising the plate deeply to near the corpora cavernosa, so the neourethra will have an adequate diameter, and resisting the temptation to extend the relaxing incision into the glans, as this will also lead to tubularizing the plate too far distally. Proximal hypospadias Duckett preputial island flap (tubed) In certain cases, an arterialized flap for urethroplasty works with great success The procedure described here is used for proximal hypospadias when the prepuce is large and the rotation of an island flap of prepuce does not cause torsion. Initially, chordee is corrected, and penile straightness is confirmed on artificial erection. Attention is then turned to the redundant dorsal prepucial skin in order to develop a flap for the neourethra. The prepuce is freed from the penile shaft, and the length and breadth of the tube for the neourethra are marked on the inner surface of the prepuce. Careful dissection is then performed to develop a vascular pedicle for this island flap. The vascular pedicle is dissected free from the dorsal penile skin until the flap can be easily rotated to the underside of the penis. The neourethra can then be tubed around an appropriately sized stent with a subcuticular PDS suture. A tunnel can be created in the glans for the distal neourethra, or the glans can be split. The proximal elliptical anastomosis is completed first. The chances of meatal stenosis can be minimized by excising a core of glans tissue from the tunnel or by use of a V-flap in the meatus. When the urethroplasty has been completed, residual dorsal penile skin is brought ventrally for skin coverage. A stent is left in the urethra for 5 days, and urine is drained via a percutaneous suprapubic catheter until the voiding trial 10 days later. Bracka’s 2 stage repair(BJPS 1995) Stage 1: release of chordee, clefting of glans and lining with FTSG from prepuce Stage 2 (6 months apart) : closure of neourethra and waterproofing flap using subcutaneous aspect of preputial hood. Perineal, Scrotal, and Proximal Shaft Hypospadias Tube Graft Hypospadias Repair (Horton-Devine Technique) Both techniques give approximately the same success rate. Flap dissection may cause devascularization of the dorsal penile skin or result in tortuosity of the urethra and thus cause flap disturbance. For more proximal cases, the flap may not have enough length. The proximal flap is more difficult to construct and requires more operating time; it may also be more prone to form diverticula. The full-thickness graft urethroplasty allows greater freedom in resurfacing the penile shaft. Hairless groin skin, buccal mucosa, and bladder mucosa are other potential graft donor sites available to form the neourethra. Hypospadias Cripples There are many patients who have had prior unsuccessful hypospadias repairs. Typically, they have insufficient penile skin remaining so that preputial grafts or flaps are not an option. Management: 1. free tubed graft techniques, with the graft material provided by using extragenital skin, bladder mucosa, or buccal mucosa. 2. Cover with local flap or scrotum 3. Prelaminated free radial forearm flap 1st stage – insert tubed SSG into forearm tunnel 2nd stage – raise free flap and inset Buccal mucosa is reported to have certain advantages in that it is hairless, leaves no donor site defect, and may have enhanced graft take because of the thin lamina propria dense capillary network. COMPLICATIONS OF HYPOSPADIA REPAIR Attempt to reduce with a careful preoperative evaluation, precise surgical technique, meticulous and careful handling of tissues and appropriate postoperatice care 2 groups : early and late Difficulty in assessing effectiveness of hypospadia operations is that techniques continue to evolve but complications occur late (ie when the children undergo puberty) EARLY i. Bladder spasm – reflex urinary retention (morphine, oral anticholinergics, hot baths, repositioning the catheter) ii. Infection (antibiotics before and after the operation) iii. Wound dehiscence (treat conservatively and repair later, can use taping methods to hold the wound edges in close approximation) iv. Necrosis v. Haematoma (evacuated if feopardizes the graft or skin flaps otherwise manage conservatively if small) LATE i. Fistula (10-20%) a. Higher with proximal hypospadia b. prevented by turning epithelium into the neourethra, using sufficient barrier layers, and avoiding meatal stenosis c. Snodgrass reduces this risk by mobilising corpus spongiosum and closing over the neourethra, then adding a dorsal dartos pedicle flap on top of this. d. Treatment i. Early – wait until wounds heal then repair ii. Chronic (<2mm) – treat stricture, excise fistula, wide undermining and defect closure. Avoind overlapping suture lines iii. Large (>2mm) – turnover flaps, reinforce with local flaps iv. Unable to close – treat strictures, tube graft repair, local flaps or bury in scrotum ii. Diverticulum a. Neourethra too patulous iii. Strictures a. Avoid circumferential scars b. Possible benefit from dilation c. Repair by open and patch flap/graft iv. Meatal stenosis - up to 40% with severe hypospadias can have some degree of voiding problems v. Hair growth in the urethra – stones vi. Tortuosity of the new urethra causing an irregular urine flow vii. Retrusive meatus viii. Chordee (ventral bend) – poor correction ix. Soft glans syndrome x. Sexual problems affecting erection, ejaculation and sexual sensation have now been reported in more than 20% of men