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Urogenital Reconstruction, Penile Hypospadias Last Updated: July 22, 2005 |
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| Synonyms and related keywords: urethroplasty, hypospadias, epispadias, bifid clitoris, bladder exstrophy, hidden penis, penile curvature
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AUTHOR INFORMATION
| Section 1 of 11   |
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| Author: Fabio Santanelli, MD, PhD, Associate Professor of Plastic Surgery, University of Rome; Chairman and Head of Unit, Department of Plastic and Reconstructive Surgery, Sant'Andrea Hospital, Rome, Italy Coauthor(s): Francesca Romana Grippaudo, MD, Assistant Professor, Department of Plastic Surgery, University of Rome Policlinico Umberto I, Italy |
| Fabio Santanelli, MD, PhD, is a member of the following medical societies:
American Society of Plastic Surgeons |
| Editor(s): Dennis P Orgill, MD, PhD, Associate Professor of Surgery, Harvard Medical School; Director, Burn Center, Brigham and Women's Hospital; Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine;
Jorge de la Torre, MD, FACS, Associate Professor of Surgery and Physical Medicine & Rehabilitation, Residency Program Director, Division of Plastic Surgery, University of Alabama at Birmingham; Director, Center for Advanced Surgical Aesthetics;
Nick Slenkovich, MD, Practice Director, Colorado Plastic Surgery Center;
and Susan E Downey, MD, Clinical Associate Professor, Department of Plastic Surgery, St John's Medical Center and University of Southern California |
Disclosure
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INTRODUCTION
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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. History of the Procedure: The earliest report concerning urethral hypoplasia and its treatment date back to Celsius (25 AD) and Galen (second century AD), with the description of hypospadias malformation.
Duplay began the modern era in this field in 1874 by publishing a detailed procedure for urethra reconstruction. 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, often the meatus 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
- 1874: Duplay performed tubulization around a catheter of the ventral skin of the penis.
- 1897: Novè 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: Dennis-Browne performed the buried skin stripe technique showing a spontaneous re-epithelialization of a neo-urethra around a catheter.
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, Hodgson and Toksu in 1970, Standoli in 1979, Duckett in 1980, Scuderi in 1981, and Koyanagi in 1993 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. 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 during pregnancy to estrogen therapy.
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 1). 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.
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." Clinical: 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 Image 2, Image 3).
The glans is often spatulated and the prepuce is cleft ventrally with a dorsally hooded foreskin (see Image 2, Image 3). 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)
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INDICATIONS
| Section 3 of 11 |
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- 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).
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RELEVANT ANATOMY AND CONTRAINDICATIONS
| Section 4 of 11 |
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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 Image 4, Image 5).
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. |
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Lab Studies:
- 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:
- 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:
- Obtain an ECG per anesthesia or operating room guidelines.
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TREATMENT
| Section 6 of 11 |
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Medical therapy: No medical treatment exists to correct hypospadias. Surgical therapy: The aims of the surgical procedures are as follow:
- 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 6-11).
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.
Single stage procedure
- Place a traction suture through the glans and extend a coronal incision around the meatus (see Image 2, Image 3).
- 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 Image 12, Image 13). Perform chordectomy and straightening of the penile shaft when needed.
- 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 Image 14, Image 15). The preputial flap can also be raised transversally from the ventral surface (according to Duckett) or from the dorsal aspect of the apron (according to Standoli). Mobilize the flap with a large subcutaneous pedicle from the dartos fascia to ensure an appropriate vascularization (see Image 16, Image 17).
- In the Scuderi procedure, a buttonhole incision is performed bluntly into the pedicle (see Image 18, Image 19), and the flap is transposed ventrally by passing the penile body through the pedicle (see Image 20, Image 21).
- 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.
- If raised according to Duckett or Standoli, ventrally transpose the flap by its rotation around the corpora cavernosa. 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 Image 22, Image 23) and extend it by rolling the flap into a tube around a 12 or 14 F silicone catheter (see Image 24).
- Remove a vertical strip of tissue from the ventral surface of the glans (see Image 25, Image 26) and raise two triangular flaps to cover the terminal part of the neo-urethra (see Image 27).
- Carry out the distal anastomosis. At the end of the procedure, discharge redundant poorly vascularized foreskin (see Image 28, Image 29) and pull up the penile skin and suture it to the corona, creating an appearance similar to a circumcised penis (see Image 30, Image 31).
- 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: 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 are elevated to cover the distal part of the neo-urethra that reach the tip of the glans.
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:
- 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.
Follow-up care:
- 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 32) 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.
- Perform a flow rate study at 3 weeks postoperatively (see Image 33). 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.
- At the 3-month follow-up visit, question patients about the persistence of the curvature or other problems.
- Long-term follow-up care is necessary at least through puberty to exclude late failure caused by hypertrophic urethral scarring or undetected chronic inflammation.
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COMPLICATIONS
| Section 7 of 11 |
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- 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.
- Urethral stricture complicates approximately 8.5% of hypospadias repairs.
- Persistent chordee caused by incomplete excision requires secondary surgical excision of all fibrous tissue.
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OUTCOME AND PROGNOSIS
| Section 8 of 11 |
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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.
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FUTURE AND CONTROVERSIES
| Section 9 of 11 |
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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.
One-stage hypospadias repairs offer the advantages of a single procedure using unscarred tissue performed when the patient is younger than school age.
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PICTURES
| Section 10 of 11 |
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BIBLIOGRAPHY
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Avellan L: Morphology of hypospadias. Scand J Plast Reconstr Surg 1980; 14(3): 239-47[Medline].
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Blair VP, Byers LT: Hypospadias and epispadias. J Urol 1938; 40: 814-816.
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Browne D: An operation for hypospadias. Lancet 1946; 1: 141-151.
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Castanon Garcia-Alix M, Martin Hortiguela ME, Rodo Salas J: Complications in hypospadias repair:20 years of experience. Cir Pediatr 1995; 8: 118-122.
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Dayanc M, Tan MO, Gokalp A, et al: Tubularized incised plate urethroplasty for distal and mid-penile hypospadias. Eur Urol 2000 Jan; 37(1): 102-5[Medline].
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Devine CJ Jr, Horton CE: Hypospadias repair. J Urol 1977 Jul; 118(1 Pt 2): 188-93[Medline].
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Duckett JW: Transverse preputial island flap technique for repair of severe hypospadias. Urol Clin North Am 1980 Jun; 7(2): 423-30[Medline].
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Duckett JW: MAGPI (meatoplasty and glanuloplasty): a procedure for subcoronal hypospadias. Urol Clin North Am 1981 Oct; 8(3): 513-9[Medline].
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Ghali AM, el-Malik EM, al-Malki T, Ibrahim AH: One-stage hypospadias repair. Experience with 544 cases. Eur Urol 1999 Nov; 36(5): 436-42[Medline].
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Hayashi Y, Kojima Y, Mizuno K: The modified Koyanagi repair for severe proximal hypospadias. BJU Int 2001 Feb; 87(3): 235-8[Medline].
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Hinderer U.: Hypospadias. Rev Esp Chir Plast 1968; 1: 53-58.
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Johanson B, Avellan L: Hypospadias. A review of 299 cases operated 1957-69. Scand J Plast Reconstr Surg 1980; 14(3): 259-67[Medline].
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Koyanagi T, Nonomura K, Kakizaki H: Experience with one-stage repair of severe proximal hypospadias: operative technique and results. Eur Urol 1993; 24(1): 106-10[Medline].
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Minevich E, Pecha BR, Wacksman J, Sheldon CA: Mathieu hypospadias repair: experience in 202 patients. J Urol 1999 Dec; 162(6): 2141-2; discussion 2142-3[Medline].
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Santanelli F: Vertical preputial flap with double skin island for correction of hypospadias with severe recurvatum. Ann Plast Surg 1994 Sep; 33(3): 305-12[Medline].
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Santanelli F, D'Andrea F, Savanelli A, et al: Reconstruction of hypospadias with a vertical preputial island flap. A follow-up study of 127 patients. Scand J Plast Reconstr Surg Hand Surg 1990; 24(1): 67-73[Medline].
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Santanelli F: Distally based turnover flap and preputium plasty for distal hypospadias repair: a preliminary report. Ann Plast Surg 1992 Nov; 29(5): 413-6[Medline].
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Scuderi N, Campus GV: A new technique for hypospadias one-stage repair
. Chir Plast 1983; 7: 103-109.
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Scuderi N, Tirone L, D'Andrea F: Terapia dell'ipospadia peniena e revisione di oltre 100 casi trattati con tecnica personale. Rivista Italiana di Chirurgia Plastica 1988; 20: 339-358.
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Smith ED: The hystory of hypospadias. Pediatr Surg Int 1977; 21: 81-85.
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Soygur T, Filiz E, Zumrutbas AE, Arikan N: Results of dorsal midline plication in children with penile curvature and hypospadias. Urology 2004 Oct; 64(4): 795-8; discussion 798[Medline].
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Standoli L: Vascularized urethroplasty flaps. The use of vascularized flaps of preputial and penopreputial skin for urethral reconstruction in hypospadias. Clin Plast Surg 1988 Jul; 15(3): 355-70[Medline].
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Toksu E: Hypospadias: one-stage repair. Plast Reconstr Surg 1970 Apr; 45(4): 365-9[Medline].
Urogenital Reconstruction, Penile Hypospadias excerpt |
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