Hypospadias 

  • Author: John M Gatti, MD; Chief Editor: Marc Cendron, MD   more...
 
Updated: Aug 11, 2011
 

Background

Hypospadias is an abnormality of anterior urethral and penile development in which the urethral opening is ectopically located on the ventrum of the penis proximal to the tip of the glans penis, which, in this condition, is splayed open. The urethral opening may be located as far down as in the scrotum or perineum. The penis is more likely to have associated ventral shortening and curvature, called chordee, with more proximal urethral defects.

The earliest medical text describing hypospadias dates back to the second century AD and was the work of Galen, the first to use the term. During the first millennium, the primary treatment for hypospadias was amputation of the penis distal to the meatus. Since that time, many have contributed to development of modern hypospadias repair. Over 300 different types of repairs have been described in the medical literature. Although most reports have been in the last 60 years, most basic techniques were described over a century ago.

Modern anesthetic techniques, fine instrumentation, sutures, dressing materials, and antibiotics have improved clinical outcomes and have, in most cases, allowed surgical treatment with a single-stage repair within the first year of life on an outpatient basis.

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Pathophysiology

Hypospadias is a congenital defect that is thought to occur embryologically during urethral development, from 8-20 weeks' gestation. The external genital structures are identical in males and females until 8 weeks' gestation; the genitals develop a masculine phenotype in males primarily under the influence of testosterone. As the phallus grows, the open urethral groove extends from its base to the level of the corona. The classic theory is that the urethral folds coalesce in the midline from base to tip, forming a tubularized penile urethra and median scrotal raphe. This accounts for the posterior and middle urethra. The anterior or glanular urethra is thought to develop in a proximal direction, with an ectodermal core forming at the tip of the glans penis, which canalizes to join with the more proximal urethra at the level of the corona. The higher incidence of subcoronal hypospadias supports the vulnerable final step in this theory of development.

In 2000, Baskin proposed a modification of this theory in which the urethral folds fuse to form a seam of epithelium, which is then transformed into mesenchyme and subsequently canalizes by apoptosis or programmed cell resorption.[1] Similarly, this seam theoretically also develops at the glanular level, and the endoderm differentiates to ectoderm with subsequent canalization by apoptosis.

The prepuce normally forms as a ridge of skin from the corona that grows circumferentially, fusing with the glans. Failure of fusion of the urethral folds in hypospadias impedes this process, and a dorsal hooded prepuce results. On rare occasions, a glanular cleft with intact prepuce may occur, which is termed the megameatus intact prepuce (MIP) variant.

Chordee, or ventral curvature of the penis, is often associated with hypospadias, especially more severe forms. This is thought to result from a growth disparity between the normal dorsal tissue of the corporal bodies and the attenuated ventral urethra and associated tissues. Rarely, the abortive spongiosal tissue and fascia distal to the urethral meatus forms a tethering fibrous band that contributes to the chordee. See the image below.

Severe penile chordee is shown. Note the extreme vSevere penile chordee is shown. Note the extreme ventral curvature of the penile shaft.

The location of the abnormal urethral meatus classifies the hypospadias. Although several different classifications have been described, most physicians use the classification that was proposed by Barcat and modified by Duckett, which describes the location of the meatus after correction of any associated chordee.[2, 3] Descriptive locations include anterior (glanular and subcoronal), middle (distal penile, midshaft, and proximal penile), and posterior (penoscrotal, scrotal, and perineal). The location is anterior in 50% of cases, middle in 20%, and posterior in 30%; the subcoronal position is the most common overall. See the images below.

Proximal shaft hypospadias is shown. Note the defiProximal shaft hypospadias is shown. Note the deficient ventral foreskin, blind urethral pit at the glanular level, and lighter pigmented urethral plate extending to the true meatus at the proximal shaft level. Proximal shaft hypospadias is shown. Note the typiProximal shaft hypospadias is shown. Note the typical dorsal hood of foreskin and ventral penile skin deficiency. Penoscrotal hypospadias is shown. Note the associaPenoscrotal hypospadias is shown. Note the associated ventral chordee and true urethral meatus located at the scrotal level.
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Epidemiology

Frequency

United States

Hypospadias occurs in approximately 1 in every 250 male births in the United States. In several countries, the incidence of hypospadias may be rising. In the United States, the rate of hypospadias doubled from 1970-1993. Although some have suggested that the increased incidence is, in reality, an increase in reporting of minor grades of hypospadias, increases in severe hypospadias have also been noted. Increasing sensitivity of surveillance systems alone cannot explain this two-fold increase. However, recent reports have linked the increased rate of hypospadias in boys born prematurely and small for gestational age and boys with low birth weight.

International

In several countries, the incidence of hypospadias may be rising but seems rather constant, at 0.26 per 1000 live births in Mexico and Scandinavia and 2.11 per 1000 live births in Hungary.[4]

Mortality/Morbidity

The treatment for hypospadias is surgical repair. Hypospadias is generally repaired for functional and cosmetic reasons. The more proximally ectopic the position of the urethral meatus, the more likely the urinary stream is to be deflected downward, which may necessitate urination in a seated position. Any element of chordee can exacerbate this abnormality. Fertility may be affected. The abnormal deflection of ejaculate may preclude effective insemination, and significant chordee can preclude vaginal insertion of the penis or can be associated with inherently painful erections. Although the most minor forms of hypospadias are physiologically insignificant, they too may merit repair based on the potential psychological stress of having a genital anomaly.

Race

The incidence of hypospadias is greater in whites than in blacks, and it is more common in those of Jewish and Italian descent. A genetic component may be present in certain families; the familial rate of hypospadias is about 7%.

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Contributor Information and Disclosures
Author

John M Gatti, MD  Associate Professor and Director of Minimally Invasive Urology, Department of Pediatric Surgery and Urology, Children's Mercy Hospital; Assistant Professor, Department of Pediatric Surgery and Urology, University of Missouri School of Medicine at Kansas City, Missouri; Assistant Clinical Professor, Division of Pediatric Urology, University of Kansas School of Medicine at Kansas City, Kansas

John M Gatti, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American Urological Association, Society for Fetal Urology, and Society for Pediatric Urology

Disclosure: Nothing to disclose.

Coauthor(s)

Andrew J Kirsch, MD, FAAP, FACS  Clinical Professor of Urology, Emory University School of Medicine, Children's Healthcare of Atlanta; President, Georgia Urology, PA

Andrew J Kirsch, MD, FAAP, FACS is a member of the following medical societies: American Academy of Pediatrics, American Urological Association, and Society for Fetal Urology

Disclosure: QMED Grant/research funds Investigation, Consulting; COOK Urological Royalty Consulting

Howard M Snyder III, MD  Professor, Department of Surgery, Division of Pediatric Urology, University of Pennsylvania School of Medicine

Howard M Snyder III, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Surgeons, American Medical Association, American Urological Association, and National Kidney Foundation

Disclosure: Nothing to disclose.

Specialty Editor Board

Martin David Bomalaski, MD, FAAP  Pediatric Urologist, Alpine Urology

Martin David Bomalaski, MD, FAAP is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, and American Urological Association

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Harry P Koo, MD  Chairman of Urology Division and Director of Pediatric Urology, Professor of Surgery, Virginia Commonwealth University School of Medicine, Medical College of Virginia; Director of Urology, Children's Hospital of Richmond

Harry P Koo, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Surgeons, and American Urological Association

Disclosure: Nothing to disclose.

Daniel Rauch, MD, FAAP  Director, Pediatric Hospitalist Program, Associate Professor, Department of Pediatrics, New York University School of Medicine

Daniel Rauch, MD, FAAP is a member of the following medical societies: Ambulatory Pediatric Association, American Academy of Pediatrics, and Society of Hospital Medicine

Disclosure: Baxter Honoraria Consulting

Chief Editor

Marc Cendron, MD  Associate Professor of Surgery, Harvard School of Medicine; Consulting Staff, Department of Urological Surgery, Children's Hospital Boston

Marc Cendron, MD is a member of the following medical societies: American Academy of Pediatrics, American Urological Association, European Society for Paediatric Urology, Johns Hopkins Medical and Surgical Association, New Hampshire Medical Society, Society for Fetal Urology, and Society for Pediatric Urology

Disclosure: Nothing to disclose.

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Proximal shaft hypospadias is shown. Note the deficient ventral foreskin, blind urethral pit at the glanular level, and lighter pigmented urethral plate extending to the true meatus at the proximal shaft level.
Proximal shaft hypospadias is shown. Note the typical dorsal hood of foreskin and ventral penile skin deficiency.
Penoscrotal hypospadias is shown. Note the associated ventral chordee and true urethral meatus located at the scrotal level.
Severe penile chordee is shown. Note the extreme ventral curvature of the penile shaft.
Penoscrotal transposition is shown. Note the rugated scrotal skin lateral to the penis, cephalad to its normal position.
A pedicled preputial island flap is shown. This hairless skin flap will be rotated on its vasculare pedicle to the ventrum of the penis for repair of the urethra.
Tubularized incised plate (TIP) technique. The urethral plate has been incised in the dorsal midline, expanding the width of the plate and allowing it to hinge forward for tubularization.
A urethrocutaneous fistula has appeared after hypospadias repair. Note one stream from true urethral meatus, and second stream through more proximal fistula.
 
 
 
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