Hypospadias

Hypospadias is the second most common congenital abnormality of the genitourinary tract in males after cryptorchidism and occurs in 1 in 250 males. It is a triad of abnormalities including a ventrally displaced urethral meatus, ventral penile curvature or chordee, and a paucity of ventral penile skin. A prenatal diagnosis may be made in severe cases of penile hypospadias; however, most diagnoses of hypospadias are made on the newborn physical exam. Severe/proximal hypospadias, especially when identified in conjunction with gonadal abnormalities such as nonpalpable/undescended testes, prompts consideration of disorders of sexual development (DSD) such as congenital adrenal hyperplasia.

Hypospadias is characterized by a urethral meatus that opens on the ventral surface of the penis proximal to the end of the glans. The meatus may be located anywhere along the length of the penis from the glans to a proximal location as low as the perineum. Ventral curvature of the penis, chordee, has an inconsistent association with hypospadias. The degree of chordee is often associated with the severity of hypospadias, with a proximal hypospadias having a more pronounced chordee. The degree of chordee can have a significant impact on the operative technique and outcomes. For example, a subcoronal hypospadias with little or no chordee is much less complicated than is one with significant chordee and insufficient ventral penile skin.

Hypospadias can be divided into several categories that may help the clinician and patient’s family to understand the implications of the condition and develop a management plan. All conditions can be considered for surgical correction; however, the indications for, and goals of, surgery should be carefully considered and discussed with the family as some indications are largely cosmetic while others, in the more severe hypospadias cases, are functional. The following categories can be considered:

Skin abnormality only, incomplete foreskin without chordee and a normally placed meatus
Chordee, with or without incomplete foreskin, and an orthotopic meatus
Megameatus variant hypospadias with intact prepuce and no chordee
Distal hypospadias with incomplete foreskin, with or without chordee
Proximal hypospadias with a distal meatus—presents at first glance as a distal hypospadias, but upon further inspection, the ventral urethra is atretic and repair must be undertaken as for a proximal repair in order to achieve an adequately straight phallus with good functional potential and cosmetic appearance
Proximal hypospadias with varying degrees of chordee and variations such as penoscrotal transposition

Embryology

Normal phallic development occurs between 6 and 14 weeks of gestation. The first stage, a hormone-independent process that occurs in both males and females, begins around 6 weeks of gestation when the genital tubercle is formed anterior to the urogenital sinus. The two genital folds form caudad to the tubercle, and a urethral plate develops between them. Human chorionic gonadotropin (hCG) from the placenta stimulates the fetal testes to produce testosterone as early as 8 weeks of gestation. Under the paracrine influence of testosterone, the inner genital folds fuse medially to create a tube that communicates with the urogenital sinus and runs distally to the base of the glans. The formation of the penile urethra is generally completed by the end of the first trimester.

Hypospadias is thought to develop due to an arrest in the normal penile and urethral development. In the male, the urethral groove fuses in a zipper-like fashion from proximal to distal to form the tubular urethral structure. This is followed by fusion of the foreskin ventrally over the urethra and a straightening of the penis, and is typically complete early in development, by about 17 weeks’ gestation. A disruption in the fusion process is thought to occur due to environmental exposure to endocrine disruptors as well as genetic causes.

Classically, the glanular urethra was thought to form as an ectodermal ingrowth on the glans. This ingrowth meets the distal urethra that has formed from the closure of the endodermal genital folds. The capacious junction of these two structures gives rise to the fossa navicularis. Recently, this theory has been challenged and the distal urethra is thought to arise from a similar process as the proximal urethra, as described above. The formation of the glanular urethra is the last step in the formation of the urethra. This accounts for the predominance of distal hypospadias (i.e., glanular, coronal, and subcoronal hypospadias).

Dorsal to the developing urethra, the paired corpora cavernosa develop from mesenchymal tissue. Mesenchyme also gives rise to Buck’s fascia, dartos fascia, and corpus spongiosum. The corpora cavernosa are the major erectile tissue components, and these are invested by the tunica albuginea. Development of the corpora cavernosa proceeds at different rates along the ventral and dorsal surface causing a temporary ventral curvature during development. The corpus spongiosum is the supportive erectile tissue that normally surrounds the urethra and communicates with the erectile tissue of the glans. Buck’s fascia is the deep layer of fascia that surrounds the corpora cavernosa and is superficial to the tunica albuginea. The dorsal neurovascular bundles are deep to this layer. Superficial to Buck’s facia is the dartos fascia, which is the loose subcutaneous layer that contains the superficial veins and lymphatics of the penis. All these structures form after completion of the urethra by medial fusion of the outer genital folds from the proximal to the distal aspect of the penis. This proximal to distal sequential development accounts for how a fully formed urethra can have sparse spongiosum, a thin overlying skin layer, and ventral tethering (i.e., chordee) despite the meatus being in an orthotopic position. The last structure to completely form is the prepuce. This epithelial layer originates at the coronal sulcus and gradually encloses the glans circumferentially from proximal to distal and from dorsal to ventral. This development is the reason a distal hypospadias often is associated with a dorsal hooded prepuce.

Arrested development of the urethra may leave the meatus located anywhere along the ventral surface of the penis. Typically, this leads to arrest in the development of the other structures that ultimately form the penis and results in foreshortening of the ventral aspect of the penis distal to the meatus, which in turn leads to chordee and failure of the prepuce to form circumferentially.

History

Hypospadias was recognized as a congenital anomaly in antiquity. References to hypospadias and techniques for repair from the first and second centuries AD have been found in Greek, Roman, and Egyptian texts by Heliodurus, Antyllus, and Galen.

Galen of Pergamon ( CE 130–199) is credited as the first to use the term hypospadias to describe the urethral meatus on the ventral surface of the penis, “the curvature of the penis prevents its normal overflow from being conveyed forwards. The theory is confirmed by the ability to beget children if the frenum is divided.” His recommended repair was a partial penectomy to the level of the urethral meatus with a conical incision to preserve glanular shape. Hemostasis was achieved by compressive dressings, vinegar water, and cauterization if necessary.

The Middle Ages followed the end of the Roman Empire and Islamic medical texts trace the history of hypospadias during this era. Albucasis of Cordoba (936–1013) and Sabuncuoglu, one of the most influential surgeons of the early Ottoman Empire, both advocated Galen’s distal amputation approach, with the latter recommending a urethral catheter as an adjunct. ^,

A. Pare (1510–90)— “ Je le pansai, Dieu le guérit. ” or “I dressed him, and God healed him .”—was a famous military surgeon and physician to the French royalty who described the benefits of releasing the hypospadias-associated chordee to improve coitus and fertility. In fact, legal documents from that era mention a husband’s hypospadias as a reason for marriage annulment. ^,

A surgical textbook from the early 1700s contained a detailed description of hypospadias repair, using a tunneled leaden pipe to bridge the distance between the hypospadiac meatus and a newly created glanular meatus. ^, Liston reported (in 1838) the successful hypospadias repair using a preputial flap, and Mettauer described a similar repair in 1842.

In 1874 Theophile Anger, a French surgeon, reported using two ventral penile skin flaps for hypospadias repair, with the first flap creating a tubularized neourethra and the second flap used for ventral skin coverage. ^, This report is considered the beginning of modern hypospadias surgery using local skin flaps. Many surgeons began refining these techniques, including NoveJosserand (1897, first free, split-thickness skin graft), ^, Ombredanne (first rudimentary flip-flap), and Duplay. Duplay used a two-stage repair with three defined steps for repair: correction of the ventral chordee with a horizontal ventral penile incision that was closed vertically, use of penile skin flaps to create a neourethra, and connection of the neourethra to the proximal meatus. In Duplay’s first stage, the chordee was released. In the second stage, a ventral midline strip of skin was covered by closure of the lateral penile skin flaps in the midline. Duplay did not believe that it was necessary to form the urethral tube completely because he thought that epithelialization would occur even if an incomplete tube was buried under the lateral skin flaps.

In the late 1940s and early 1950s, bladder mucosa was used for hypospadias repair. ^, This technique was also developed independently during the period of scientific and cultural isolation in China. Buccal mucosa from the lip was employed for urethral reconstruction in 1941 by Humby, and has since become an increasingly popular free-graft technique.

Skin coverage was advanced by Byars’ technique (1955) using two dorsal preputial flaps. Smith further improved the outcomes by denuding the epithelium of one of the lateral skin flaps to give a “pants-over-vest” closure to reduce the risk of fistula formation. Belt devised another preputial transfer, a two-stage procedure that was popularized by Fuqua in the 1960s.

One-stage hypospadias repairs became more frequent after the late 1950s and 1960s, and gradually primary repairs were extended to include more proximal hypospadias. In 1980, Duckett advanced the use of preputial tissue with his description of the transverse preputial island flap technique.

A popular technique, the single-stage tubularized, incised plate urethroplasty (TIP) was reported by Snodgrass in 1994. This technique was initially described for distal hypospadias without significant chordee and did not require preputial flaps. Improvements in a wide variety of surgical techniques over the past 40–50 years through the efforts of a number of distinguished surgeons have greatly advanced hypospadias repair.

Clinical Aspects

Incidence

A relatively recent review of the incidence and prevalence of hypospadias analyzed data from 1910 to 2013, covering approximately 90 million births. The mean prevalences were Europe 19.9 per 10,000 live births (range 1–464), North America 34.2 (range 6–129.8), South America 5.2 (2.8–110), Asia 0.6–69, Africa 5.9 (1.9–110), and Australia 17.1–34.8. As suggested from these numbers, there were major geographical, regional, and ethnic differences. Several studies showed an increasing prevalence of hypospadia, but other reports found no such increase.

Although some of this wide variation probably represents geographic and racial differences, a significant portion of the variability may be due to the exclusion of the more minor degrees of hypospadias. If even the most minor forms are included, it is likely that the incidence of hypospadias is probably 1 in 125 live male births, resulting in more than 6000 boys born with hypospadias each year in the United States. ^,

Etiology

Many theories have been proposed to explain the etiology of hypospadias. Genetic factors, inadequate hormonal stimulation, maternal/placental milieu, and environmental factors have all been implicated. It is likely that a combination of these play a role in the development of hypospadias.

Deficient hCG production by the placenta, inadequate androgen production by the testes, failure of 5α-reductase enzyme to convert testosterone to dihydrotestosterone, or faulty androgen receptors in the genitalia can each explain the development of hypospadias. Various DSDs are also associated with hormone deficiencies or derangements that lead to hypospadias or hypospadias variants.

A diminished response to hCG has been shown in some patients with hypospadias, suggesting delayed maturation of the hypothalamic–pituitary axis and poor hormonal stimulation during the crucial early weeks of development. ^, Other reports have described an increased incidence of hypospadias in monozygotic twins, suggesting an insufficient amount of hCG production by the single placenta to accommodate the two male fetuses. This has been further supported by linking maternal hypertension to an increased risk of hypospadias. In utero exposure to certain medications (e.g., valproate, loperamide, paroxetine, and estrogenic or antiandrogenic medications) have been associated with an increased risk. ^, ^, ^, A weak association between hypospadias and maternal exposure to progestin-like agents has also been reported. ^, However, no association has been found between hypospadias and oral contraceptive use before or during early pregnancy. Environmental factors such as pesticide exposure may play a role. Interestingly, there appears to be a higher incidence of hypospadias with winter conceptions.

A large number of genes have been implicated in hypospadias, and genes involved in penile development (e.g., HOX, FGF, Shh ) and testicular determination (e.g., WT1, SRY ), luteinizing hormone (LH) receptor, and androgen receptor (e.g., 5α reductase, androgen receptor) may be associated with an increased risk of developing hypospadias. ^, A genetic predisposition is highly suggested given the high incidence of this anomaly in first-degree relatives, with about 7%–10% of cases having an affected sibling or father. However, only 30% of isolated hypospadias cases have an identifiable genetic cause, and most are idiopathic. The heritability of hypospadias can range between 55% and 75%, with no difference between maternal and paternal transmission. ^, In one study of 307 families with hypospadias, the risk of hypospadias in a second male sibling was 12%. If the index child and his father were affected, the risk for a second sibling increased to 26%. If the index child and a second-degree relative (rather than the father) were affected, the risk of the sibling being affected was 19%. This pattern suggests a multifactorial mode of inheritance and penetrance. Hypospadias has been associated with multiple genetic disorders and syndromes, ^, ^, most notably WAGR syndrome (Wilms tumor, aniridia, genitourinary abnormalities, and mental retardation) and Denys–Drash syndrome (genitourinary malformations and susceptibility to Wilms tumor). Both syndromes are associated with WT1 gene mutations.

Anatomy of the Defect

The clinical significance of hypospadias is related to several factors. The abnormal location of the meatus and the tendency toward meatal stenosis result in a ventrally deflected and splayed stream. These factors make the stream difficult to control and often make it difficult for the patient to void while standing. The ventral curvature associated with chordee can lead to painful erections, especially with severe curvature. These can lead to impaired intercourse due to the chordee and inadequate insemination resulting in fertility problems. In addition, the unusual cosmetic appearance associated with the hooded foreskin, flattened glans, and ventral skin deficiency frequently has an adverse effect on the psychosexual development of the adolescent with hypospadias. ^, These factors should be discussed and considered when determining timing and indication for surgical correction of hypospadias, regardless of the severity of the defect.

The distal form of hypospadias is the most common (see Box 57.1 ). Frequently, little or no associated chordee is present ( Fig. 57.1 ). The size of the meatus and the quality of the surrounding supportive tissue as well as the configuration of the glans can be variable, and ultimately determine the appropriate operative approach for each patient. Well-formed, mobile perimeatal skin and a deep ventral glans groove may allow development of perimeatal flaps to create the urethra ( Fig. 57.2 ). In contrast, atrophic and immobile skin around the meatus may require tissue transfer, typically from the prepuce or other donor sites, to form a neourethra ( Fig. 57.3 ).

Box 57.1

Hypospadias Classification According to Meatal Location After Release of Chordee

Distal (65%–70% of cases)
Glanular
Coronal/subcoronal
Distal penile shaft
Midshaft (10%–15% of cases)
Middle penile shaft
Proximal (20% of cases)
Proximal penile shaft
Penoscrotal
Scrotal
Perineal

A clinical view shows a penis with a urethral opening present below the margin instead of the tip, marked by an arrow. A pair of surgical instruments stretches the outer skin to reveal the opening. — Fig. 57.1

A clinical view shows the opening of urethra below the head of the penis. The skin around the opening is flexible and stretched. There is a deep groove on the underside of the head of the penis. — Fig. 57.2

A clinical view shows external genitalia with arrows marking the phallic shaft and an arrow pointing to the urethral opening at the penoscrotal junction. The clinical view shows the external genitalia of a patient. The gloved hands gently hold the phallic structure upward to expose the ventral surface. The urethral opening is present at the penoscrotal junction, marked by an arrow. Multiple arrows are marked along the midline shaft of the phallus, starting from the junction. The scrotal tissue appears bifid and partially unfused. — Fig. 57.3

Most cases of hypospadias can be easily identified with physical exam based on the abnormal foreskin, chordee, and a displaced meatus, and these patients should not be offered an infant circumcision. In the case of hypospadias with no concern for an intersex condition, an outpatient referral can be made to the pediatric urologist to discuss repair.

Unexpected Hypospadias

A special circumstance is the identification of hypospadias at the time of infant circumcision. This is typically in cases of an intact prepuce and no chordee. The abnormal meatus, typically a megameatus variant, is only identified during the circumcision process after the dorsal slit is performed. Previously, when this was identified, the circumcision was aborted, and the child left with a dorsal slit, with plans to follow up for a circumcision and/or hypospadias repair at an older age. Recent studies have shown that this increases the need for procedures under anesthesia and the recommendations have now changed.

A report of 63 patients with hypospadias and a history of intact prepuce who underwent repair either after having had a circumcision or no circumcision found that prior circumcision did not increase the risk of complications in the group that had already been circumcised prior to urethral construction. Another study evaluated 93 newborns who had an aborted circumcision. Of these, 28 underwent hypospadias repair and 47 underwent circumcision completion under general anesthesia. The remaining 18 either deferred surgery or underwent in-office circumcision, thus showing that half of newborns with aborted circumcision could have avoided a procedure under anesthesia if the circumcision had simply been completed. These reports suggest that when a hypospadias is encountered while doing a circumcision for an intact prepuce variant, the hypospadias is by definition so mild that the excess foreskin is not needed for a later reconstruction. A significant portion have such a mild meatal abnormality that there is no indication for repair. If the circumcision is aborted, all of those children will ultimately require an anesthetic later to complete the circumcision and possibly repair the hypospadias. Thus, the circumcision should be completed, and a subsequent referral should be made to pediatric urology for consideration of meatal repair if necessary.

At times, the located meatus may be associated with significant chordee, sometimes of a severe degree ( Fig. 57.4 ). The release of the chordee can result in a much more proximal location of the meatus, requiring a more complicated repair to bridge the gap between the proximal meatus and the tip of the glans. When the meatus is located on the penile shaft, the character of the urethral plate (midline ventral shaft skin distal to the meatus) is important in determining what type of repair is possible. A well-developed and elastic urethral plate suggests minimal, if any, distal ventral curvature ( Fig. 57.5 ). However, a thin atrophic urethral plate heralds a significant chordee. The proximal supportive tissue of the urethra also is important. If there is a lack of spongiosum proximal to the hypospadiac meatus, this portion of the native urethra is not substantial enough to be used in the repair ( Fig. 57.6 ). Therefore, the neourethra must be constructed from the point of adequate spongiosum by cutting back.

A clinical view shows the external genitalia of an infant with a phallic structure held gently between gloved fingers. The urethral opening is visible at the base of the scrotum. — Fig. 57.4

A clinical view shows a penis with a ventral defect and a visible urethral opening marked by an arrow. The clinical view shows the ventral surface of a penis prepared for surgery. A gloved hand retracts the surrounding tissue to reveal a defect along the underside of the phallus. An arrow points to an abnormal urethral opening located on the ventral shaft rather than at the tip, and the glans is partially visible at the top. — Fig. 57.5

A surgical view shows penile shaft dissection with forceps elevating tissue during hypospadias reconstruction. The surgical view shows a pediatric hypospadias repair procedure with the penis fully exposed and surrounded by sterile drapes. The skin over the penile shaft has been incised and partially mobilized, revealing the underlying tissue and urethral plate. Two surgical forceps are used to elevate and separate the tissue planes at the distal shaft and glans region. The glans has been split to allow further exposure and reconstruction. The penile shaft appears elongated, and the native urethral groove can be identified along the ventral midline. — Fig. 57.6

The position of the meatus at the penoscrotal, scrotal, or perineal location is usually associated with severe chordee, which requires chordee release and an extensive urethroplasty ( Fig. 57.7 ). This type is usually more predictable in the preoperative period as to the choice of technique than are some of the more distal types previously discussed. Other anatomic elements of the anomaly that are important include penile torsion, glans tilt, penoscrotal transposition, and chordee without hypospadias. These are discussed in more detail later in the chapter.

A clinical view shows ambiguous genitalia with a phallic structure and a midline opening marked by an arrow. The clinical view shows the external genitalia of a pediatric patient. A prominent phallic structure is seen in the upper central region, positioned above a midline raphe. The phallus has a rounded, glans-like bud appearance. Below this structure, an arrow marks a small, visible urethral opening along the midline at the base of the fused folds of the bifid scrotum. — Fig. 57.7

Associated Anomalies

Inguinal hernia and undescended testes are the most common anomalies associated with hypospadias. They occur in 7%–13% of patients, with a greater incidence when the meatus is more proximal. ^, An enlarged prostatic utricle is also more common in proximal hypospadias, with an incidence of about 10%. Infection is the most common complication of a utricle, but excision is rarely necessary. Several reports have emphasized significantly high numbers of upper urinary tract anomalies associated with hypospadias, ^, suggesting that routine upper tract screening is necessary. However, when the association is stratified by degree of hypospadias, it is evident that the types of hypospadias at risk for significant upper tract anomalies are the penoscrotal and perineal forms, and those associated with other syndromes or organ system abnormalities. ^, When one, two, or three other organ system abnormalities are associated with hypospadias, the incidence of significant upper tract anomalies is 7%, 13%, and 37%, respectively. Associated myelomeningocele and imperforate anus carry a 33% and 46% incidence of upper urinary tract malformations, respectively. In isolated proximal hypospadias, the incidence of associated upper tract anomalies is under 5%.

In midshaft and distal hypospadias, when not associated with other organ system anomalies, the incidence of upper tract anomalies is similar to that in the general population. Therefore, it is recommended that screening for upper urinary tract abnormalities with renal ultrasonography and/or voiding cystourethrogram (VCUG) be performed only in patients with proximal hypospadias (i.e., penoscrotal and perineal), and in those with anomalies associated with at least one additional organ system. Screening should also be done in patients with other known indications, such as a history of urinary tract infection, upper or lower tract obstructive symptoms, hematuria, and in those boys having a strong family history of urinary tract abnormalities.

Males with DSD often have associated hypospadias; conversely, most isolated hypospadias is only rarely associated with DSD. When hypospadias is associated with undescended testes, micropenis, penoscrotal transposition (see Fig. 57.4 ), or bifid scrotum (see Fig. 57.7 ), DSD should be considered and warrants evaluation with karyotype along with evaluation by a multidisciplinary team with expertise and interest in DSD. As with other anomalies, the more proximal the hypospadias, the higher the association with DSD.

Treatment

The advent of safe anesthesia, fine suture material, delicate instruments, and good optical magnification has allowed virtually all types of hypospadias to be repaired in infancy. Generally, the repair is done on an outpatient basis. To deny a child the benefit of repair because the defect is “too mild” or the risk of complication is “too high” is inappropriate; however, a thorough discussion of the goals of surgery as well as the risks and benefits should be carried out with family and shared decision making should be undertaken in order to proceed with a plan that is in the best interest of the child. The chance to make the phallus as normal as possible should be offered to all male children, regardless of the severity of the defects. When examining the child with hypospadias with plans for intervention, various aspects of the abnormality should be considered for surgical planning. The availability of penile skin should be assessed and the degree of chordee be evaluated by gently compressing the corpora against the pubic bone. The placement of the urethral meatus and the quality of the urethra should also be considered. At times, the meatus appears to be in a fairly normal/distal location; however, the urethra proximal to the meatus can be very atretic. If this is suspected, a feeding tube can be placed in the urethra in clinic to get a better sense of the tissues on the ventral aspect of the phallus. This can help to properly plan surgery, alerting the surgeon to need for a formal hypospadias repair rather than isolated chordee correction, and can be very useful to counsel patients. Having an intraoperative conversation with a family to inform them of the identification of a poorly formed urethra and need for hypospadias surgery when the family was expecting a fairly simple phalloplasty procedure with no catheter left in situ after the procedure can be very difficult, and proper preoperative preparation and explanation of expectations is very helpful for families.

Age at Repair

The technical advances over the past few decades have made it possible to repair hypospadias early in life in most patients. Repair is typically undertaken between 6 months of age and 1 year of age, ^, although the timing of repair can vary, and upper preferred limit is before completion of the potty training years. Repair at older ages is associated with increased risk of complications as well as psychosocial issues. ^, Unless other health or social problems require delay, we believe the ideal time to complete penile reconstruction is between 6 and 8 months of age.

Objectives of Repair

The objectives of hypospadias correction are:

1.
Complete straightening of the penis
2.
Locating the meatus at the tip of the glans
3.
Forming a symmetric, conically shaped glans
4.
Constructing a neourethra uniform in caliber
5.
Satisfactory and cosmetic skin coverage

If these objectives can all be attained, the ultimate goal of forming a “normal” penis for the child with hypospadias can be accomplished.

Chordee

Curvature of the penis is often difficult to assess preoperatively. It is important to recognize, since uncorrected chordee can result in a poor cosmetic outcome and more severe curvature (>30 degrees) can interfere with later intercourse. Artificial erection, by injecting physiologic saline in the corpora at the time of the operation, allows determination of the exact degree of curvature, which is typically ventrally. This curvature may be caused by ventral skin or subcutaneous tissue tethering, which can be corrected with the release of skin and dartos tissue. ^, Infrequently, the curvature may be secondary to true fibrous tissue. In these cases, it is often required to divide the urethral plate and excise the fibrous tissue down to the tunica albuginea.

Occasionally, even after extensive ventral dissection of chordee tissue, a repeated artificial erection reveals persistent significant ventral curvature. This situation is typically secondary to corporal body disproportion caused by a true deficiency of ventral corporal development. This problem can be corrected by one or a combination of several methods. By dissecting and elevating the urethral plate off the corpora cavernosa, the ventral tunica albuginea can be incised and augmented by inserting either a dermal patch, a piece of small intestinal submucosa, or a tunica vaginalis patch to expand the deficient ventral surface. Elevation of the urethral plate with ventral grafting can result in devascularization of the urethral plate and can increase the risk of urethral stricture. This maneuver also prevents the use of grafts due to concern of poor revascularization of the graft and/or graft loss. Alternatively, several nearly full-thickness ventral tunica albuginea incisions (“fairy cuts”) can be made to release the chordee without the need for ventral grafting of the corpora cavernosa. An alternative technique includes excision of the tunica albuginea dorsally with transverse closure to shorten the dorsal surface and straighten the penis. ^, This should be done with caution, as it can lead to damage to the neurovascular bundle that courses dorsally. Other surgeons have had success with multiple tandem dorsal plications without excision of the tunica albuginea, but this can result in significant shortening of the phallus and the straightening may not be long lasting. Anatomic studies suggest that this plication should be done in the midline dorsally to avoid injury to the neurovascular bundle. Still others advocate for corporal rotation dorsally, with or without penile disassembly, to correct the severe chordee. ^, However, this is seldom required given the success with the less-invasive techniques mentioned above.

Axial rotation of the penis, or penile torsion, is another aspect of penis straightening that must be managed. This problem can generally be corrected by releasing the dartos layer as far proximal as possible on the penile shaft. Typically, this is done down to the penopubic and penoscrotal junction. This allows the ventral shaft to rotate back to the midline and corrects the torsion. Chordee or torsion can also rarely occur without hypospadias ( Fig. 57.8 ). The management of these boys encompasses the same spectrum of approaches as for those with hypospadias. ^,

A two-part surgical view shows penile degloving in hypospadias repair, showing an exposed shaft and mobilized vascular structures. The two-part surgical view, labeled A and B, shows key stages in hypospadias repair through penile degloving and vascular structure mobilization. In panel A, the penile shaft is fully degloved, exposing the underlying tissue after the overlying skin has been circumferentially incised and retracted. The shaft is cylindrical and elongated with visible vascular markings distributed along the surface. A catheter is inserted at the glans and extends through the urethral opening for guidance. In panel B, the dissection is further advanced, revealing mobilized vascular structures running along the ventral surface. Multiple surgical retractors are used to elevate the penis, and underlying dartos or fascial tissue planes are visible. The skin is fully separated from the shaft, and the anatomy is well exposed for further urethroplasty or curvature correction. — Fig. 57.8

Locating the Meatus

Historically, a glanular meatal location was often the goal of hypospadias repair. Currently, even with the more severe forms, the neomeatus is located at the tip of the glans. In glanular and subcoronal hypospadias, the configuration of the meatus is the factor that determines what technique is used to relocate the meatus on the glans. Meatoplasty with or without dorsal advancement, urethral mobilization and tubularization (with or without incision of the urethral plate), or meatal-based flaps are the methods selected in most children with distal hypospadias. In the more proximal forms, creating the neourethra with local vascularized skin flaps or free grafts allows positioning the urethra at the end of the penis. Alternatively, glans channeling or glans splitting allows creation of the meatus at the tip of the glans.

Glans Shape

Creation of a symmetric, conically shaped glans is the objective of the glansplasty component of the repair. Approximating the lateral glanular tissue in the ventral midline after a meatoplasty or meatal advancement corrects the flattened glans appearance and results in a conically shaped glans. Similarly, approximation of well-developed glans wings to the midline over a neourethra in a split glans restores the glans to its normal conical shape.

Urethral Construction

Formation of the neourethra can be accomplished with local skin flaps, various types of free grafts, or vascularized pedicle flaps. Local skin flaps can be formed from in situ skin or dorsal skin transferred to the ventrum during staged procedures. In either case, it is important to avoid making these flaps too narrow or thin because their blood supply can be compromised leading to complications (e.g., strictures). The hypospadiac urethral plate has been shown in histologic studies to consist of epithelium covering well-vascularized connective tissue without fibrosis. This information supports the point that urethral plate preservation is helpful for successful urethroplasty. Free grafts depend on an adequately vascularized bed for survival. Therefore, they should not be placed in a scarred channel, over fibrotic tissue, or over other nonvascularized grafts. Well-vascularized subcutaneous tissue is necessary to allow adequate neovascularization and survival of the graft.

Mobilized vascularized flaps of preputium have a more reliable blood supply than do free grafts. If they are available, these flaps are the choice of most surgeons. ^, They may be used as patches onto a strip of native urethral plate to complete the urethra, or they may be tubularized and used as bridges over the gap between a proximal native urethra and the end of the glans. ^, A watertight closure of the well-vascularized neourethra is formed, with care being taken to make it uniform in caliber and of appropriate size for the age of the child. This closure helps avoid stricture and the formation of saccules, diverticula, and fistulas.

Cosmesis

Creating cosmetically appealing, well-vascularized skin coverage of the penile shaft after urethroplasty can sometimes be challenging. Transfer of vascularized dorsal preputial skin to the ventrum can be accomplished in several ways. Buttonholes of the dorsal skin allow the penis to come through this defect, draping the distal preputium over the ventral surface of the penis. This maneuver has the advantage of transferring well-vascularized skin over the repair, but it is not cosmetically appealing.

A more satisfactory method of transferring skin to the ventrum is splitting the dorsal skin in the midline longitudinally and advancing the flaps around on either side to meet in the ventral midline. This technique allows a midline ventral closure, which simulates the median raphe. Moreover, it allows a subcoronal closure to the preputial skin circumferentially, which simulates the suture lines of a standard circumcision. ^, Another adjunct is to advance lateral flaps of inner preputial skin from each side to the ventral midline of the penis at the time of glansplasty or closure of glans wings. Approximating these flaps in the midline gives the appearance of an intact circumferential preputial collar, further enhancing the potential for an anatomically normal skin closure.

Some patients, particularly those in European countries, prefer the appearance of an uncircumcised penis. In distal repairs, reconstruction of the prepuce for an uncircumcised appearance can be accomplished if desired. Correction of the more significant degrees of penoscrotal transposition is often necessary to avoid a feminizing appearance in patients with proximal hypospadias. In some cases, this step can be done at the time of the original repair. However, when using vascularized pedicle flaps for the repair, it is usually safer to correct significant penoscrotal transposition with rotational flaps at a later time.

Operative Approaches

Due to the wide variation in the anatomic presentation of hypospadias, no single urethroplasty is applicable for every patient. At times, a final decision regarding the degree of curvature and the ultimate location of the meatus cannot be made until the operation has started, the penis is degloved, and an artificial erection is created. The surgeon who repairs hypospadias must be adaptable and experienced to deal with all variants of the defect. Versatility and experience with all options of surgical treatment are the keys to successful management. By recognizing the subtle nuances of meatal variation, glans configuration, and curvature character, the experienced surgeon can choose the optimal technique ( Fig. 57.9 ).

A flowchart outlines surgical repair options for hypospadias based on chordee presence, location, and urethral characteristics. The flowchart provides a structured decision-making algorithm for selecting appropriate surgical techniques for hypospadias repair. It categorizes cases into distal and proximal types, each further subdivided by the presence or absence of chordee. In the distal category without chordee, cases with small, mobile glanular or coronal meatus are directed to the M A G P I procedure. If megameatus with intact prepuce or immobile urethra is present, decisions depend on the deep ventral glans groove or proximal perimeatal tissue quality. Options include glans approximation procedures, meatal-based flaps, tubularized incised plate urethroplasty, or onlay island flaps. For proximal hypospadias with chordee, severity determines whether the urethral plate must be divided. Severe cases proceed to preputial island flap or free grafts using buccal, skin, or bladder tissue, typically with a two-stage repair. Mild chordee cases with intact plates are treated using an onlay island flap. In proximal cases without chordee, the flow leads directly to the onlay island flap technique. The flowchart integrates anatomical factors and tissue conditions to guide personalized surgical planning. — Fig. 57.9

Distal Variants

Some glanular variants are amenable to the meatal advancement and glansplasty (MAGPI) repair ( Fig. 57.10 ). A stenotic meatus with good mobility of the urethra and a fairly shallow ventral glanular groove are the anatomic characteristics best suited for the MAGPI. A wide-mouthed meatus is not amenable to the MAGPI repair. The meatal-based flap repair may also be used effectively in this situation, assuming no chordee is present, and mobile, well-vascularized skin is present proximal to the meatus ( Fig. 57.11 ). ^, This repair works well when there is a moderately deep ventral groove, allowing the urethra to be placed deep in the glans and a conically shaped glans to be created after closure of the glans wings over the skin flap.

A five-part surgical diagram shows sequential steps of glansplasty using a keyhole incision technique from exposure to final closure. The five-part surgical diagram, labeled A to E, shows a step-by-step representation of glansplasty using a keyhole incision technique. In panel A, the penis is degloved and the glans is exposed with lateral traction sutures, the meatus is visible near the tip. In panel B, a diamond-shaped keyhole incision is made on the glans just proximal to the meatus, indicated by a bold horizontal arrow, to create an entry site for the neourethra. Panel C shows an inset view of the excised tissue and sutures prepared for advancement. Panel D depicts the neourethral plate being advanced through the newly created keyhole and secured within the glans tissue. Panel E shows the final appearance after the glans wings have been approximated and closed over the neourethra using interrupted sutures to form a conical glans. — Fig. 57.10

A four-part surgical diagram shows steps of tubularized incised plate urethroplasty from marking and incision to tubularization and final closure. The four-part surgical diagram shows four labeled steps A through D demonstrating tubularized incised plate urethroplasty for hypospadias repair. In panel A, the penile shaft is degloved, and the urethral plate is outlined with dotted lines indicating the area to be incised and tubularized. In panel B, a vertical incision is made through the urethral plate along the midline to widen the tissue for tubularization, and the surrounding skin flaps are prepared for glans and shaft coverage. A horizontal arrow points to the incised area. In panel C, the neourethra is partially reconstructed with interrupted sutures bringing the edges of the plate together in a tubular fashion, and the glans wings are mobilized for later closure. In panel D, the neourethra is fully tubularized over a stent or catheter, the glans wings are approximated, and interrupted sutures are placed across the shaft and glans. — Fig. 57.11

The glans approximation procedure (GAP) is useful when a wide-mouthed proximal glanular meatus is found with a very deep groove ( Figs. 57.12 and 57.13 ). The pyramid procedure is well suited for the fish-mouth type of meatus seen in the megameatus intact prepuce variant. These repairs give excellent cosmetic result when performed in appropriately selected patients.

A four-part surgical diagram shows the steps of meatal advancement and glanuloplasty repair from incision marking to final glans closure. The four-part surgical diagram shows sequential stages labeled A through D representing the meatal advancement and glanuloplasty technique used in distal hypospadias repair. In panel A, the glans is exposed and traction sutures are placed laterally, while dotted lines outline the planned incisions around the urethral meatus and the glans wings. Panel B shows the glans incision being made vertically using scissors to allow for advancement of the urethral opening toward the tip of the glans. In panel C, the urethral meatus is advanced distally into the newly created glans cleft, and the glans wings are positioned on either side for closure. In panel D, the glans wings are brought together and closed over the neomeatus with a vertical line of interrupted sutures, forming a conical glans with the meatus at the tip. The surrounding penile skin remains intact and undisturbed beyond the glans level. — Fig. 57.12

A three-part surgical diagram shows glans and shaft skin closure after hypospadias repair using the transposition flap technique. The three-part surgical diagram shows three panels labeled A through C showing the transposition flap technique used during hypospadias repair for covering the neourethra and completing glans and shaft closure. In panel A, the dorsal preputial skin is rotated to the ventral surface along incisions marked by dotted lines that run obliquely from the coronal sulcus to the ventral base of the shaft. The neourethra has already been reconstructed, and the glans is closed with sutures along the midline. In panel B, the flap is fully rotated, showing a lateral view where the skin bridge overlaps the ventral aspect of the penis, covering the underlying reconstructed urethra. In panel C, the final view demonstrates a completed repair with full midline glans closure and symmetrical shaft coverage. Interrupted sutures secure the glans and the edges of the transposed skin flap. — Fig. 57.13

The tubularized incised plate urethroplasty (TIP) is a modification of the Thiersch–Duplay tubularization, which involves a deep longitudinal incision of the urethral plate in the midline ( Fig. 57.14 ). This allows the lateral borders of the urethral plate to be mobilized and closed in the midline without tension. This procedure also allows repair of the wide-mouthed meatus variant with a flat, shallow ventral groove without the need for additional flaps. Over the last decades, the TIP urethroplasty has gained wide acceptance. Its durability and long-term success have been demonstrated even in the more proximal variants. ^, In addition, it provides a cosmetically pleasing vertically oriented meatus.

A five-part surgical diagram shows steps of urethral plate tubularization with glans closure and catheter placement for hypospadias repair. The five-part surgical diagram shows sequential panels labeled A through E depicting the tubularized incised plate urethroplasty technique for distal hypospadias repair. In panel A, the glans is exposed with traction sutures, and the urethral plate is outlined with a central dotted line. Panel B shows a vertical midline incision along the outlined urethral plate, extending from the native meatus to the distal tip of the glans, designed to widen the tissue for tubularization. In panel C, the incised edges of the plate are sutured together over a catheter using a continuous suture technique, forming the neourethral tube. The glans wings are then mobilized. Panel D shows the approximation of the glans wings over the newly formed neourethra using interrupted sutures, closing the glans in the midline. Horizontal lines indicate suture placement locations. In panel E, the completed repair is shown with the glans fully closed and a catheter emerging from the neomeatus to maintain urethral patency during healing. — Fig. 57.14

Midshaft Variants

The amount of ventral curvature generally dictates the type of repair in middle- and distal-shaft hypospadias. When no significant chordee is present, the TIP repair can frequently be performed. Another approach is the onlay island flap technique ( Fig. 57.15 ). This procedure involves mobilizing an inner preputial flap on its pedicle and rotating it ventrally to lay on the well-developed ventral urethral plate to complete the tubularization of the neourethra. This technique is applicable to many forms of midshaft/penile hypospadias.

A five-part surgical diagram shows steps of onlay island flap urethroplasty from incision and flap harvest to glans and shaft closure. The five-part surgical diagram shows panels labeled A through E representing the step-by-step procedure of onlay island flap urethroplasty for hypospadias repair. In panel A, the glans is exposed with traction sutures, and the urethral plate is outlined with dotted lines, indicating the planned incision path from the meatus to the tip of the glans. In panel B, the penile skin is retracted to reveal a well-defined urethral plate and adjacent tissue. A preputial flap is outlined dorsally with horizontal dotted lines to guide flap elevation. In panel C, the preputial flap is rotated and prepared to be placed as an onlay over one side of the urethral plate. In panel D, the flap is sutured to the urethral plate to form the neourethra, and the glans wings are approximated over the flap to secure the repair distally. Interrupted sutures are shown closing the flap margins. In panel E, the glans and shaft are fully closed, with the neourethra in place and a catheter passed through the newly constructed urethral tube.

Tags: Holcomb and Ashcraft's Pediatric Surgery, Irina Stanasel

May 10, 2026 | Posted by drzezo in PEDIATRICS | Comments Off

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