Hypospadias
LAURENCE S. BASKIN
Hypospadias is defined by the three major anatomical defects: (a) the abnormal location of the urethral meatus, (b) penile curvature, and (c) abnormalities of the foreskin. The objective in treating patients with hypospadias is to reconstruct a straight penis for normal coitus and place the new urethral meatus on the terminal aspect of the glans to allow a forwarddirected stream. There are five basic steps for a successful hypospadias outcome: (a) orthoplasty (straightening), (b) urethroplasty, (c) meatoplasty and glanuloplasty, (d) scrotoplasty, and (e) skin coverage. These various elements of surgical technique can be applied either sequentially or in various combinations to achieve a surgical success (1,2).
DIAGNOSIS
Meatal Abnormalities
Hypospadias is characterized by an abnormality in location and configuration of the urethral meatus and surrounding urethral spongiosum (Fig. 98.1). The urethral meatus may be ventrally placed just below a blind dimple at the normal meatal opening on the glans or so far back in the perineum that it appears as a “vaginal” hypospadias. Most patients present with the urethral meatus on the glans penis, coronal margin, or distal aspect of the glans. The configuration of the meatus may vary in form, diameter, elasticity, and rigidity. It can be fissured in both transverse and longitudinal directions or can be covered with delicate skin secondary to associated atresia of the supporting urethral spongiosum. In the case of the megameatus intact prepuce, the distal urethra is enlarged, tapering to a normal caliber in the penile shaft. Often, there is an orifice of a periurethral duct located along the urethral plate distal to the meatus that courses dorsal to the urethral channel for a short distance. It is blind ending and does not communicate in any way with the urinary stream. The periurethral duct corresponds with Guérin sinus or Morgagni lacunae. Unless these ducts are inadvertently closed, leading to a blind ending epithelial pouch, they are of no clinical consequence.
Skin and Scrotal Abnormalities
The skin of the penis is changed as a result of the disturbance in the formation of the urethra. Prepuce formation is aborted distal to the abnormal meatus with excess dorsal foreskin. Proximal to the meatus, there is often a paucity of ventral skin, which may contribute to penile curvature. The frenulum is
always absent in hypospadias. Vestiges of a frenulum are sometimes found inserting on either side of the open navicular fossa.
always absent in hypospadias. Vestiges of a frenulum are sometimes found inserting on either side of the open navicular fossa.
FIGURE 98.1 Location of the hypospadias meatus: glandular, distal, midshaft, proximal, penoscrotal, scrotal, and perineal. |
The skin proximal to the urethral meatus may be extremely thin, so much so that a catheter or probe passed proximally is readily apparent through a tissue paper thickness of skin.
The urethral plate defined as a rectangular segment of tissue from the abortive hypospadias urethral meatus to the glandular groove is often well developed. Even with a meatus quite proximal on the shaft, this normal urethral plate is quite elastic and typically nontethering. After degloving the penile shaft skin, artificial erection demonstrates no ventral curvature in these situations. A normal urethral plate may be incorporated into the surgical repair. However, in the rare severe case of hypospadias if the urethral plate is underdeveloped, it will act as a tethering fibrous band that bends the penis ventrally during artificial erection. When this fibrous chordee tissue is divided, the penile curvature will frequently improve.
Normally, the penis should develop in a cranial position above the two genital swellings. The penis may be caught between the two scrotal halves and become engulfed with fusion of the penoscrotal area resulting in penile scrotal transposition.
Penile Curvature
The curvature of the penis is caused by deficiency of the normal structures, most commonly on the ventral side of the penis. It has been labeled chordee; however, this term implies a strand of connective tissue stretched like a cord between the meatus and glans, which is rarely found in practice. Penile curvature can be from skin deficiency, a true fibrous chordee with tethering of the ventral shaft, or deficiency of the corpora cavernosa on the concave side of the penis (3).
There are occasional reports of other penile anomalies that represent variations of the embryologic defect causing hypospadias. They can be characterized as a defect in the course of the urethra such as congenital urethral fistula and a group characterized by curvature of the penis without hypospadias or so-called chordee without hypospadias.
SURGICAL TECHNIQUE
Hypospadias Surgeons
Success is directly related to the experience of the surgeon (4). For a successful result in hypospadias repair, the penile tissues must be handled with great care. Experience in mobilizing and rotating skin flaps is needed, as are the minutiae involved in plastic surgical techniques. It is not enough to review pictures and follow descriptions; training in the techniques is essential. Knowledge of a few methods is not enough because the one used must be the best for the individual situation of the child. A pediatric urology fellowship is the appropriate place to become competent in hypospadias surgery.
Preoperative Evaluation
Because hypospadias is typically an isolated anomaly, the entire genitourinary tract does not require evaluation. This assumes that the remainder of the physical exam is normal including a normally placed anus and no spine abnormalities. The absence of one gonad, perineal hypospadias, severe chordee, or a bifid scrotum suggests a disorder of sex development (DSD) and requires genotypic evaluation (5). If both gonads are not palpable, consider the possibility of congenital adrenal hyperplasia in a phenotypic female.
Age for Operation
Select a time between 6 and 9 months for surgery. At this age, the infants are also easiest to manage, are not walking, and remain in diapers. Babies appear to have less bladder spasms and require smaller doses of pain medication. They do not seem to remember the surgery as teenagers and adults. The routine use of preoperative androgen stimulation is not indicated and remains controversial for patients with a small glans and penis that require an appropriate evaluation for a DSD (6). When considered indicated, testosterone may be administered to increase the size of the penis and especially the size and vascularity of the prepuce should it be needed for proximal and perineal hypospadias repair. A standard dose is 25 to 50 mg of testosterone enanthate in oil administered intramuscularly, repeated once or twice at 3-week intervals before operation.
Outpatient Repair
An uncomplicated hypospadias operation can be done without hospital admission. Preoperative education explaining the
procedure, obtaining informed consent, answering the family’s (and when age-appropriate, the patient’s) questions, and reviewing the postoperative catheter care and pain management is the norm. Patient educational material has proved to be extremely helpful.
procedure, obtaining informed consent, answering the family’s (and when age-appropriate, the patient’s) questions, and reviewing the postoperative catheter care and pain management is the norm. Patient educational material has proved to be extremely helpful.
Nerve Block
A caudal nerve block placed by the anesthesia team is an excellent form of postoperative pain control. A good alternative is a penile nerve block. To place a penile nerve block in a healthy infant use 3 to 4 mL of 0.5% long-lasting bupivacaine mixed with 1% of quick-acting lidocaine. Inject it at the base of each crus just below the notch of the symphysis, or vertically in the midline deep to the notch of the symphysis and around the ventral aspect of the base of the penis, with a 1 1/2-inch 25-gauge needle. When placed at the beginning of an operation, it will reduce the amount of general anesthesia required and will provide anesthesia that will last well into the postoperative period.
Surgical Hints
Hemostasis
For hemostasis, use 1% lidocaine with 1:100,000 epinephrine and inject it through a 27- to 29-gauge needle within the glans and the area of abortive spongiosum. Wait 7 minutes for it to act. This vasoconstrictor will reduce the bleeding during the dissection, but if the operation is prolonged beyond 90 minutes, rebound vasodilation can be expected. Avoid electrocoagulation as much as possible; if it is necessary, use a bipolar cautery or touch a monopolar cautery to only the forceps unit set at a low current. Once the glans flaps are applied, bleeding improves, and a pressure dressing will usually assure hemostasis. On rare occasion, use of tourniquet that is typically used for artificial erections can facilitate hemostasis.
Artificial Erection
To induce a saline-induced erection, place a sterile rubber band or vascular loop around the base of the penis and snug it with a hemostat. Introduce a 25-gauge butterfly needle into the corpus cavernosum. Gently distend the penis with injectable normal saline solution; avoid overdistention. Maintain the erection during evaluation of the penile curvature. After the curvature has been corrected, create a second erection to check penile alignment.
Local Urinary Diversion in Children
Diversion of urine away from the suture lines has always been a problem in children because any indwelling tube, particularly one terminating in a balloon, induces bladder spasms that force urine around it into the repair. This disrupts the suture line and leads to formation of fistulas. Besides, the lumen of a balloon catheter is small compared to that of a straight catheter, especially a plastic one.
Many techniques have been tried to minimize these problems with diversion. The simplest method for infants, one that combines stenting with drainage, is to insert a fine silicone tube, such as 6Fr peritoneal shunt tubing or neurosurgical tubing with its wand-like end, into the bladder through the urethra and fasten the end to the glans in one or two places with nonabsorbable sutures.
Alternately, place a 6Fr Kendall catheter of soft Silastic, with a Luer-lock at the end to prevent internal migration and to allow irrigation. Whatever intubation system is used in infants, collect the urine in a double diaper. For older boys, use a feeding tube, or in teenagers, a urethral balloon catheter; tape it to the abdomen so that it cannot disturb the ventral glans repair. Drainage should be continued for 4 to 7 days for distal and penile shaft repairs and 7 to 10 days more severe hypospadias repairs.
Dressings
Apply a dressing to immobilize the area, to reduce edema, and to prevent the formation of a hematoma. Use transparent and permeable absorbent plastic film (Tegaderm or OpSite) applied over Telfa or tincture of benzoin. Let the catheter drain into an outer diaper. The dressing may be removed in 2 to 3 days after a few warm baths at home. Once the dressing has been removed, use K-Y jelly on the diaper to keep the repaired penis from sticking, typically for 4 to 5 days.
Setup for Operation
Instruments
Select instruments designed for delicate handling of tissues. A reasonable list would include loupe magnification, genitourinary fine and microsurgery sets, microsurgical knife (Weck), toothed and nontoothed forceps (Adson), fine Allis clamps, fine clamps, two pairs of Bishop-Harmon forceps or 0.5 platform forceps, sharp small tenotomy scissors, iris scissors, microtip Castroviejo scissors, microtip Castroviejo needle holders, plastic needle holders and ring retractor (Scott/LoneStar), and hooks. Also, have available bougie à boule, 5Fr and 8Fr infant feeding tubes, rubber bands, a marking pen, a 25-gauge butterfly needle and syringe, and a handheld Bovie, or an ophthalmic electrocautery. Have fine sutures of appropriate sizes and types at hand but unopened—for example, 5-0 Prolene on a C-1 tapered needle for glans traction, 7-0 polydioxanone suture (PDS) and Vicryl for urethroplasty, and 6-0 PDS for the skin.
Selection of the Operative Technique
Figure 98.2 presents an algorithm for the reconstruction of hypospadias. A tried and true approach is to start each repair by preserving the urethral plate, dissecting the skin to the penile scrotal junction, and assessing for the presence of penile curvature. If curvature is not present or mild to moderate and amendable to dorsal plications, then a one-stage approach is typically successful. The specific repair is now dependent of the meatal configuration and the surgeon’s preference (1).
Treatment of Anterior Hypospadias
The Meatal Advancement and Glanuloplasty Technique
The hypospadiac penis that is amenable to the meatal advancement and glanuloplasty (MAGPI) is characterized by a dorsal
web of tissue within the glans that deflects the urine from either a coronal or a slightly subcoronal meatus (7). Once the patient is asleep, the urethra itself must have a normal ventral wall, without any thin or atretic urethral spongiosum. The urethra also must be mobile so it can be advanced into the glans (Fig. 98.3).
web of tissue within the glans that deflects the urine from either a coronal or a slightly subcoronal meatus (7). Once the patient is asleep, the urethra itself must have a normal ventral wall, without any thin or atretic urethral spongiosum. The urethra also must be mobile so it can be advanced into the glans (Fig. 98.3).