Penile Hypospadias Reconstruction 

Updated: May 22, 2018
Author: Fabio Santanelli di Pompeo, MD, PhD; Chief Editor: Jorge I de la Torre, MD, FACS 

Overview

Background

Congenital malformations of male genitalia include a wide range of clinical situations such as hypospadias, epispadias (in the bifid clitoris) with bladder exstrophy, and hidden penis. This article addresses the surgical treatment of penile hypospadias.

History of the Procedure

The earliest report concerning urethral hypoplasia and its treatment dates back to Celsius (25 AD) and Galen (second century AD), with the description of hypospadias malformation.[1]

Duplay began the modern era in this field in 1874 by publishing a detailed procedure for urethra reconstruction.[1] Currently, more than 200 techniques have been described. Most of the procedures are multistage reconstruction; all consist of a first emergency stage that addresses correction of the stenotic meatus, if required, and a second stage that eliminates the chordee and the recurvatum.

Techniques differ regarding the third stage, the urethroplasty (creation of a neo-urethra and its coverage).

Numerous problems are associated with the multistaged techniques: they required multiple operations, the meatus often did not reach the tip of the glans or retract with time because of multiple tissue manipulations and repeated scarring, urethral stricture and/or fistula formation were frequent, and the final aesthetic result was poor.

To overcome the high frequency of complications, Hinderer introduced one-stage hypospadias repair in 1960.

Numerous modifications and innovative techniques were promulgated by other surgeons in the subsequent decade, all affirming the advantage of using unscarred tissue to reconstruct the urethra while minimizing the number of operations.

One-stage hypospadias repair claims ideal anatomic and functional urethral reconstruction, with good aesthetic restoration of external genitalia, a low complication rate, minor psychological involvement, and reduced social costs (see Figure 1 below).

Figure 1: History of hypospadias repair

Multistaged repairs

  • The first stage addresses correction of the stenotic meatus if required.

  • The second stage eliminates the chordee and the recurvatum.

  • The third stage reconstructs a neo-urethra to the tip of the glans.

Milestones in urethroplasty[1]

  • 1874: Duplay performed tubulization around a catheter of the ventral skin of the penis.

  • 1897: Nov√® Josserand performed urethroplasty with a skin graft tubed around a catheter and anastomosed to the urethral meatus.

  • 1899: Rochet performed tubulization of a meatus-based flap from the scrotum.

  • 1917: Beck raised and tubed two paramedian ventral skin flaps.

  • 1911: Ombredanne created a turnover flap harvested proximally to the meatus and sutured to two paramedian incisions performed distally to the meatus on the ventral penile skin.

  • 1946: Denis Browne performed the buried skin stripe technique showing a spontaneous re-epithelialization of a neo-urethra around a catheter.[2]

One-stage repairs

  • This involved correction of the recurvatum and reconstruction of the urethra in the same sitting.

  • 1917: Beck performed a one-stage procedure for distal hypospadias with skeletonization and an advancement of the urethra to reach the tip of the glans.

  • 1961: Horton and Devine performed a one-stage procedure, modified in 1967, which uses two flaps, one harvested from the glans and the other from the ventral penile skin, to reconstruct the urethra in distal hypospadias while a skin graft was suggested in the more proximal malformations.

  • Hinderer in 1968,[3] Hodgson and Toksu in 1970,[4] Standoli in 1979, Duckett in 1980,[5] Scuderi in 1981, and Koyanagi in 1993[6] performed a one-stage procedure based on using a preputial flap for urethroplasty.

Problem

Hypospadias consists of external urethral meatus dystopia, which may sort on the ventral surface of the penile shaft at any distance between the tip of the glans and the perineum, ventral absence of the preputium, and wide dorsal apron.

Curvature of the penile shaft and stenosis of the external meatus are often associated.

This malformation is seldom linked with genitourinary anomalies (eg, cryptorchidism, varicocele, hydrocele, ureteral duplication) and, rarely, with cardiovascular and craniofacial malformations.

Epidemiology

Frequency

Hypospadias presents in 1 in 350 live male births in the United States and is the most frequent malformation of the genitourinary tract.

Etiology

The enlargement of the genital tubercle and subsequent development of the phallus and urethra depend on the level of testosterone during embryogenesis.

If the testes fail to produce adequate amounts of testosterone or if the cells of the genital structures lack adequate androgen receptors or the androgen-converting enzyme 5 alpha-reductase, virilization is not complete and hypospadias results.

Genetic and nongenetic factors are involved in the etiology of hypospadias. A familial occurrence of hypospadias is found in approximately 28% of patients.

The exact genetic mechanism may be complicated and variable. The possibility of an autosomal dominance inheritance with low penetrance has been discussed. Another hypothesis is an autosomal recessive inheritance with incomplete manifestation.

Chromosomal aberration is found sporadically in patients with hypospadias.

Hypospadias is associated with several uncommon syndromes.

The main nongenetic factor associated with hypospadias is the administration of sex hormones; an increased incidence of hypospadias was found among infants born to women exposed to estrogen therapy during pregnancy.

Prematurity is associated more often in patients with hypospadias than in the general population.

Pathophysiology

The penis begins to form at approximately the fifth fetal week under the influence of testosterone. The urethral folds start to fuse over the urethral groove, and by the 14th week the process is complete (see image below). A short ingrowth from the tip of the glans progresses inward to meet the urethral tube at the fossa navicularis. The prepuce is then formed at the end of the development process.

Left: External genitalia during the undifferentiat Left: External genitalia during the undifferentiated stage. Middle: Male external genitalia at ninth week of intrauterine life. Right, from above to below: Transverse section of male genital area during the development of the urethral channel.

Hypospadias occurs when the fusion of the urethral folds stops proximal to the tip of the glans penis and can occur anywhere along the urethral groove.

Severe forms of hypospadias are accompanied by shortening of the urethral groove, which causes ventral tethering of the penis, a condition termed "chordee."

Presentation

This deformity presents with different severity according to the time of pathogen noxa during embryologic development. The meatus can sort in a glanular (60%), penile (35%), or scrotoperineal position (5%) and is clinically inadequate in 75% of patients and is often stenotic (see images below).

Distal hypospadias: spatulated glans, ventrally cl Distal hypospadias: spatulated glans, ventrally cleft preputium. (Published in Scand J Plast Reconstr Surg Hand Surg 1990; 24 (1): 67-73. Reprinted with permission of the editor.)
Distal hypospadias. A traction suture is placed th Distal hypospadias. A traction suture is placed through the glans. Incision lines according to Scuderi repair: a coronal incision extended up to and around the meatus. (Published in Scand J Plast Reconstr Surg Hand Surg 1990; 24 (1): 67-73. Reprinted with permission of the editor.)

The glans is often spatulated and the prepuce is cleft ventrally with a dorsally hooded foreskin (see images above). Penile curvature of different degrees is observed in many patients (45%).

Several urogenital defects are associated with hypospadias.

  • Cryptorchidism (9%)

  • Inguinal hernia (9%)

  • Megalourethra, urethral fistulae, and hypoplastic testicles (reported less often)

  • Upper urinary tract defects (observed in association with proximally located hypospadias)

Indications

See the list below:

  • Meatotomy is indicated at any age when the meatus caliber is reduced, causing dysuria.

  • Correction of glanular hypospadias with distal urethra repositioning, creation of a symmetric glans (glanuloplasty), and preputium plasty is indicated in childhood or adolescence at the patient's request for cosmetic or psychological reasons.

  • Correction of penile and penoscrotal hypospadias is indicated in childhood in patients younger than school age for the following reasons:

    • To permit normalization of voiding

    • To allow normal erection and intercourse

    • To avoid urinary tract infections

    • To correct impotentia generandi and coeundi

    • To achieve cosmetic sexual identity

  • Hypospadias is corrected by penile "chordee" resection and by creation of a urethra of adequate caliber and length (urethroplasty).

Relevant Anatomy

The two corpora cavernosa and the corpus spongiosum of the urethra with the glans form the penis.

These structures are made from erectile tissue surrounded by the tunica albuginea (Buck fascia) and by the dartos fascia more superficially, which contains terminal branches of external pudendal arteries and veins, pudendal nerves, and the superficial lymphatics (see images below).

The dartos fascia is outlined with the terminal br The dartos fascia is outlined with the terminal branches of the external pudendal arteries and veins.
Transverse section of the penis at midshaft level. Transverse section of the penis at midshaft level. From outside, the dartos fascia is visible with the blood vessels, the tunica albuginea surrounding the corpora cavernosa, and, in a ventral position, the urethra surrounded by its erectile tissue.

The normal male urethra runs from the bladder through the penile shaft, ventrally to the corpora cavernosa, and sorts with the external urethral meatus at the tip of the glans.

Contraindications

Reconstruction of the urethra and restoration of the normal appearance of the genitalia is contraindicated in infancy because of the difficulty related to the small dimension of the structures.

 

Workup

Laboratory Studies

See the list below:

  • Obtain a complete blood cell (CBC) count for infants and elderly persons.

  • In severe forms of virilization failure, determine sex assignment with karyotype analysis and blood and urinary hormone levels.

Imaging Studies

See the list below:

  • Perform ultrasonography to assess the normality of the upper urinary system in the presence of other organ system anomalies, syndromic patients, or ambiguous genitalia.

  • Obtain a chest radiograph if indicated by examination findings or the patient's history.

Other Tests

See the list below:

  • Obtain an ECG per anesthesia or operating room guidelines.

 

Treatment

Medical Therapy

No medical treatment exists to correct hypospadias.

Surgical Therapy

The aims of the surgical procedures are as follows:

  • Widening of the meatus

  • Correction of the curvature

  • Reconstruction of the missing portion of the urethra

  • Restoration of the normal aspect of the external genitalia

Surgery differs according to the severity of the malformation.

A meatotomy is required if the size of the external urethral meatus is inferior to that considered normal according to the age of the patient.

The distal urethra missing in glanular hypospadias, usually without recurvatum, is well reconstructed with local flaps based on the meatus (eg, Santanelli procedure, Flip Flap, MAGPI [meatal advancement and glanuloplasty]), including preputium plasty at the same sitting (see images below).

Distal hypospadias. Incision lines are shown. Distal hypospadias. Incision lines are shown.
The spatulated flap is turned over and sutured to The spatulated flap is turned over and sutured to the glans.
The urethra is reconstructed and sutured between t The urethra is reconstructed and sutured between the glanular flaps.
Preputium plasty. Reconstruction of the inner laye Preputium plasty. Reconstruction of the inner layer.
Preputium plasty. Reconstruction of the outer surf Preputium plasty. Reconstruction of the outer surface.
Normal mobility of the preputium after its reconst Normal mobility of the preputium after its reconstruction.

In penile and penoscrotal types of malformation, resection of the chordee and reconstruction of the missing part of the urethra are performed with a single-stage procedure (eg, Duckett, Standoli, Scuderi, modified Koyanagi).

In some clinical situations (eg, perineal hypospadias, genital ambiguity, significant hypospadias with previous circumcision), more extensive operations are necessary, and the former multistage operations may be of occasional use.

A study by Arnaud et al indicated that in proximal penile hypospadias repair, the presence of a bifid scrotum predicts the need to transect the urethral plate. Of 18 children with a bifid scrotum, plate transection was considered necessary in 15, compared with two out of 11 children without a bifid scrotum.[7]

Single-stage procedure

See the list below:

  • Place a traction suture through the glans and extend a coronal incision around the meatus (see images below).

    Distal hypospadias: spatulated glans, ventrally cl Distal hypospadias: spatulated glans, ventrally cleft preputium. (Published in Scand J Plast Reconstr Surg Hand Surg 1990; 24 (1): 67-73. Reprinted with permission of the editor.)
    Distal hypospadias. A traction suture is placed th Distal hypospadias. A traction suture is placed through the glans. Incision lines according to Scuderi repair: a coronal incision extended up to and around the meatus. (Published in Scand J Plast Reconstr Surg Hand Surg 1990; 24 (1): 67-73. Reprinted with permission of the editor.)
  • Lift the penile skin, including the prepuce, and raise it in the plane between the Buck and dartos fascia.

  • Deglove the meatus and penile urethra and separate them from the corpora cavernosa to the point that normal spongy tissue is detected. Excise the hypoplastic stenotic portion of the urethra.

  • Perform an artificial erection with intracavernous injection of saline solution to assess the presence and degree of curvature (see images below). Perform chordectomy and straightening of the penile shaft when needed.

    Artificial erection. An intracavernous injection o Artificial erection. An intracavernous injection of saline is performed while controlling the back flow at the basis of the penis with an elastic band. (Published in Scand J Plast Reconstr Surg Hand Surg 1990; 24 (1): 67-73. Reprinted with permission of the editor.)
    Artificial erection achieved with normal saline in Artificial erection achieved with normal saline injected in the corpora cavernosa while controlling the backward flow. (Published in Scand J Plast Reconstr Surg Hand Surg 1990; 24 (1): 67-73. Reprinted with permission of the editor.)
  • At this point, evaluate the actual urethral defect and begin the reconstruction. Harvest a peno-preputial skin flap, which may include both sides of the apron to increase its length, longitudinally along the penile vascular axis according to Scuderi and Koyanagi (Scuderi technique, see images below). The preputial flap can also be raised transversally from the ventral surface (according to Duckett)[5] or from the dorsal aspect of the apron (according to Standoli).[8]

    Two 5/0 traction sutures are placed on either side Two 5/0 traction sutures are placed on either side of the preputial apron. Outlining of the vertical preputial flap after resection of the chordee and evaluation of the urethral defect. (Published in Scand J Plast Reconstr Surg Hand Surg 1990; 24 (1): 67-73. Reprinted with permission of the editor.)
    Vertical preputium flap after Scuderi repair. Note Vertical preputium flap after Scuderi repair. Note the "V" shape pattern on the inferior part of the cutaneous flap. (Published in Scand J Plast Reconstr Surg Hand Surg 1990; 24 (1): 67-73. Reprinted with permission of the editor.)
  • Mobilize the flap with a large subcutaneous pedicle from the dartos fascia to ensure an appropriate vascularization (see images below).

    The vertical preputial flap is elevated on the dor The vertical preputial flap is elevated on the dorsal cutaneous side of the penis, vertically disposed along the vascular axis (Scuderi technique), to comply with the defect to be reconstructed. The flap is mobilized with a large subcutaneous pedicle.
    Elevation of the vertical preputial flap with its Elevation of the vertical preputial flap with its subcutaneous pedicle.
  • In the Scuderi procedure, a buttonhole incision is performed bluntly into the pedicle (see first 2 images below), and the flap is transposed ventrally by passing the penile body through the pedicle (see second 2 images below).

    Buttonhole incision along the midline of the subcu Buttonhole incision along the midline of the subcutaneous pedicle of the vertical preputial flap, along the axis of the blood vessel, taking care not to jeopardize the vascularization. (Published in Scand J Plast Reconstr Surg Hand Surg 1990; 24 (1): 67-73. Reprinted with permission of the editor.)
    Buttonhole incision along the median line of the p Buttonhole incision along the median line of the pedicle. (Published in Scand J Plast Reconstr Surg Hand Surg 1990; 24 (1): 67-73. Reprinted with permission of the editor.)
    The flap is transposed ventrally through the butto The flap is transposed ventrally through the buttonhole incision without tractioning or twisting the pedicle, which may impair the blood supply. Key suture between the flap and the urethra. (Published in Scand J Plast Reconstr Surg Hand Surg 1990; 24 (1): 67-73. Reprinted with permission of the editor.)
    Key suture between the flap and the urethra. (Publ Key suture between the flap and the urethra. (Published in Scand J Plast Reconstr Surg Hand Surg 1990; 24 (1): 67-73. Reprinted with permission of the editor.)
  • If raised according to Koyanagi, the skin flap is divided into two portions at the 12 o'clock position to form a Y-shape, whereas in the modified Koyanagi repair, a button-hole is made trough the pedicle.[6]

  • If raised according to Duckett or Standoli, ventrally transpose the flap by its rotation around the corpora cavernosa.[5, 8] To reduce the incidence of stenosis of the proximal urethral anastomosis, the preputial flap must be V-shaped proximally and joined to a distal incision of 5 mm performed on the ventral wall of the urethra along its medial line.

  • Continue suturing between the proximal side of the flap and the urethra (see first 2 images below) and extend it by rolling the flap into a tube around a 12F or 14F silicone catheter (see third image below).

    The lower edge of the flap is sewn all around the The lower edge of the flap is sewn all around the urethral orifice.
    The flap is transferred ventrally, and the lower e The flap is transferred ventrally, and the lower edge of the flap is sewn all around the urethral orifice.
    The flap is tubed around a urinary catheter. In th The flap is tubed around a urinary catheter. In this manner, the anastomosis is covered by the pedicle and the longitudinal suture lies deep between the corporeal bodies, reducing the possibility of fistula occurrence. (Published in Scand J Plast Reconstr Surg Hand Surg 1990; 24 (1): 67-73. Reprinted with permission of the editor.)
  • Remove a vertical strip of tissue from the ventral surface of the glans (see first 2 images below) and raise two triangular flaps to cover the terminal part of the neo-urethra (see third image below).

    The flap is rolled into a tube. (Published in Scan The flap is rolled into a tube. (Published in Scand J Plast Reconstr Surg Hand Surg 1990; 24 (1): 67-73. Reprinted with permission of the editor.)
    The glans is split to permit the placement of the The glans is split to permit the placement of the exit of the neo-meatus at the tip of the glans. A vertical strip of tissue from the ventral surface of glans is removed, and two thick triangular flaps are raised to cover the distal part of the neo-urethra. A well-closed urethra during intercourse is achieved.
    Splitting of the glans. (Published in Scand J Plas Splitting of the glans. (Published in Scand J Plast Reconstr Surg Hand Surg 1990; 24 (1): 67-73. Reprinted with permission of the editor.)
  • Carry out the distal anastomosis. At the end of the procedure, discharge redundant poorly vascularized foreskin (see first 2 images below) and pull up the penile skin and suture it to the corona, creating an appearance similar to a circumcised penis (see second 2 images below).

    Trimming of the preputium in excess. Trimming of the preputium in excess.
    The excess preputium is removed to provide the pen The excess preputium is removed to provide the penis a circumcised appearance.
    Final appearance at the end of the operation. Final appearance at the end of the operation.
    Final appearance. (Published in Scand J Plast Reco Final appearance. (Published in Scand J Plast Reconstr Surg Hand Surg 1990; 24 (1): 67-73. Reprinted with permission of the editor.)
  • Stent the urethra and apply a mild compressive dressing.

Preoperative Details

An accurate physical examination of the external genitalia of the patient is required to assess the severity of the malformation. Check the position of the meatus, dimension of the penis, and presence of the testicles.

If a hypospadias condition is associated with impalpable testes, obtain appropriate tests (eg, complete endocrine screen, chromosome analysis, ultrasonography) to exclude an intersex condition.

Intraoperative Details

Intraoperative considerations include the following:

  • After penile degloving, an artificial erection with intracavernous injection of saline solution is performed to assess the presence and degree of curvature.

  • The external urethral meatus is then resected together with the most distal hypoplastic portion of the urethra.

  • Cordectomy and straightening of the penile shaft is performed if required.

  • At this stage, the actual urethral defect shows and the urethral reconstruction is planned.

  • A penile-preputial flap is harvested according to the preferred technique, taking into consideration the age-related urethral size. The flap is inset to the proximal urethral stump and tubed around a silicone catheter.

  • The glans is split and two flaps that reach the tip of the glans are elevated to cover the distal part of the neo-urethra.

  • The redundant foreskin is discharged and the penile skin is pulled up and sutured to the corona to achieve a final aspect similar to a circumcised penis.

Postoperative Details

Postoperative considerations include the following:

  • Restraints for arm and legs may be necessary.

  • Remove the urethral stent after 48 hours.

  • The dressings remain in place for 4 days if no problems occur.

  • Remove the diverting urinary catheter after 8-10 days.

  • Discharge the patient after removal of the urinary diversion and when spontaneous voiding occurs without difficulties.

A randomized pilot study by Canon et al suggested that postoperative prophylactic oral antibiotic use in patients who have undergone distal hypospadias surgery with urethral stent drainage does not lower the risk of symptomatic urinary tract infection.[9]

However, a prospective, randomized trial by Roth et al involving patients who underwent mid-to-distal hypospadias surgery reported that, while again, prophylactic antibiotic use did not appear to affect the incidence of postoperative symptomatic urinary tract infection, it did seem to discourage the development of bacteriuria and pyuria. At stent removal, the incidence of bacteriuria and pyuria in patients who received antibiotic prophylaxis was 11% and 18%, respectively, compared with 63% and 55%, respectively, in patients who received no antibiotics.[10]

Follow-up

See the list below:

  • Patients are observed with fluximetry tests including registration of the micturition volume, maximum flow, medium flow, and micturition time.

  • A pressure-flow study with urethrogram and endoscopy (see image below) before removal of the urinary diversion may be indicated to evaluate the detrusorial pressure and the morphologic and urodynamic aspects of the newly reconstructed urethra.

    Poor flow rate in a junctional stenosis (above). N Poor flow rate in a junctional stenosis (above). Normal flow rate (down) 1 year after dilatation. (Published in Scand J Plast Reconstr Surg Hand Surg 1990; 24 (1): 67-73. Reprinted with permission of the editor.)
  • Perform a flow rate study at 3 weeks postoperatively (see image below). Examine patients showing a normal flow rate again at 3 and 12 months postoperatively. In patients exhibiting mild stenosis, urethra dilatation is indicated; repeat the flow rate study after 3 weeks.

    Endoscopic view of the anastomosis. (Published in Endoscopic view of the anastomosis. (Published in Scand J Plast Reconstr Surg Hand Surg 1990; 24 (1): 67-73. Reprinted with permission of the editor.)
  • At the 3-month follow-up visit, question patients about the persistence of the curvature or other problems.

  • It is reported that the majority (range, 50-81%) of hypospadias repair complications (fistula, glans dehiscence, meatal stenosis, urethral stricture, and diverticulum) are diagnosed by the first year after surgery. Nevertheless, late complications are detected in patients with symptoms.[11, 12, 13]

  • Long-term follow-up care is necessary at least through puberty to exclude late failure caused by hypertrophic urethral scarring or undetected chronic inflammation.

Complications

Early complications include bleeding, infection, wound separation, flap necrosis, and edema. Temporary stenosis from edema or hypertrophic scarring at the anastomotic site may occur in 7% of repairs. Normalization is achieved after dilatation with urethral probes of progressive caliber and stabilization of the healing process.

Early urinary leakage from delayed healing of the urethral suture has been reported with an incidence of 3-9%. Spontaneous resolution occurs by keeping the suprapubic diversion for a longer time.

Urethrocutaneous fistulas with urinary leakage from the new urethra range from 0.6-23% in the one-stage operation and from 2-37.3% in the two-stage operation.[14, 15]

Urethral stricture complicates approximately 8.5% of hypospadias repairs. Persistent chordee caused by incomplete excision requires secondary surgical excision of all fibrous tissue.

Outcome and Prognosis

Functional results obtained with one-stage correction of hypospadias in terms of overall success rate, incidence of fistulas or stenosis, and mean hospitalization time have proved to be superior than those obtained with multistage procedures, and the prognosis is good.

The use of a well-nourished and innervated flap with a long and pliable pedicle is the reason for the high success rate reported in the literature when using one-stage reconstruction.

Glanuloplasty and residual preputial trimming are always associated with urethral repair and allow the reconstruction of a cosmetically acceptable glans with a neomeatus at the tip, closed during intercourse, and with a final appearance close to a normal circumcised penis.

The different techniques of preputial flap (Duckett, Standoli, Scuderi, Koyanagi) allow good functional results in primary hypospadias or in physically disabled patients where prepuce is no longer available and the flap can be harvested from the dorsal preputial skin.[5, 8, 16, 6]

 

Future and Controversies

Several problems are related to the older multistage techniques. Repeated surgery, high percentage of fistulas and strictures of the urethra, extensive scarring, and the presence of hairs in the neo-urethra were the most frequent complaints.

Tissue engineering with urothelial cells harvested by bladder wash has been proven to be a feasible method for hypospadias repair for selected patients.[17] This technique needs more long-term follow-up studies. At present, hypospadias repair with bioengineered tissue has not proven to yield better results when compared with 1-stage hypospadias repair.

One-stage hypospadias repairs offer the advantages of a single procedure using unscarred tissue performed when the patient is younger than school age.