Diagnostic Considerations

In 1981, Khuri et al reviewed more than 1000 patients with hypospadias and reported that the incidence of undescended testes and inguinal hernias was 9% for each.^[32]With more severe forms of hypospadias, the incidence of undescended testes exceeded 30%, and the incidence of inguinal hernias approached 20%. It is important to evaluate the position of the testes and assess for the presence of inguinal hernia as part of the physical examination for hypospadias.

The combination of hypospadias and undescended testis can be an indicator of an underlying difference (disorder) of sex development (DSD).^[33]In a 1999 study by Kaefer et al, DSD states were identified in approximately 30% of patients with unilateral or bilateral undescended testes and hypospadias, and a more proximal meatal location carried a higher association of DSD states than a more distal meatal location.^[34]If any gonad was nonpalpable, the incidence rose to 50%; however, if both gonads were palpable, the incidence was only 15%.

A prostatic utricle is occasionally noted when catheterization of the urethra is attempted in patients with hypospadias. A prostatic utricle is a müllerian duct remnant that typically regresses in boys. There is a high association between a prominent utricle, an outpouching off the posterior aspect of the prostate, and hypospadias, particularly the more severe forms. These are often asymptomatic but can cause difficulty with catheterization, lead to stasis of urine and urinary tract infection, or collect debris or stones. They sometimes require surgical excision.^[35]

Differential Diagnoses

Workup

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Media Gallery

Proximal shaft hypospadias. Note deficient ventral foreskin, blind urethral pit at glanular level, and lighter pigmented urethral plate extending to true meatus at proximal shaft level.
Proximal shaft hypospadias. Note typical dorsal hood of foreskin and ventral penile skin deficiency.
Penoscrotal hypospadias. Note associated ventral chordee and true urethral meatus located at scrotal level.
Penoscrotal transposition. Note rugated scrotal skin lateral to penis, cephalad to its normal position.
Pedicled preputial island flap is shown. This hairless skin flap will be rotated on its vascular pedicle to ventral aspect of penis for repair of urethra.
Tubularized incised plate (TIP) technique. Urethral plate has been incised in dorsal midline; this expands width of plate and allows it to hinge forward for tubularization.
Urethrocutaneous fistula has appeared after hypospadias repair. Note one stream from true urethral meatus and second stream through more proximal fistula.
Severe penile chordee. Note extreme ventral curvature of penile shaft.
Tubularized incised plate (TIP) technique. Urethral plate has been isolated (dissected) prior to midline incision. Note starting position of urethral opening at base. Assistance with image editing provided by Joseph Borer, MD.
Tubularized incised plate (TIP) technique. Urethral plate has been incised to allow expansion for tubularization. Note deep midline incision (cut edges marked in blue) and widening of urethral plate. Assistance with image editing provided by Joseph Borer, MD.
Tubularized incised plate (TIP) technique. Incised plate has been tubularized over 8-French tube. Note final position of urethral opening at tip of penis, and note that meatus is left quite wide. Assistance with image editing provided by Joseph Borer, MD.
Megameatus intact prepuce (MIP) variant of hypospadias.
Megameatus intact prepuce (MIP) variant of hypospadias, apparent once foreskin is retracted.
Subcoronal hypospadias. Note distal blind-ending false pit at level of glans.
Glanular (balanic) hypospadias with "figure-eight" meatus. Dorsal pit is blind ending; ventral opening is actual urethral meatus.

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Author

John Michael Gatti, MD Chief of Division of Pediatric Urology, Director of Minimally Invasive Urology, Department of Pediatric Surgery and Urology, Children’s Mercy Hospital; Professor, Department of Pediatric Surgery and Urology, University of Missouri-Kansas City School of Medicine; Clinical Professor, Division of Urology, University of Kansas School of Medicine

John Michael Gatti, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Surgeons, American Urological Association, International Pediatric Urology Junior Organization, Kansas City Medical Society, Kansas City Urologic Society, Kansas Urological Society, Midwest Society for Pediatric Research, Society for Fetal Urology, Societies for Pediatric Urology

Disclosure: Nothing to disclose.

Coauthor(s)

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

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

Disclosure: Nothing to disclose.

Specialty Editor Board

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, 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, American Urological Association

Disclosure: Nothing to disclose.

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, New Hampshire Medical Society, Societies for Pediatric Urology, Society for Fetal Urology, Johns Hopkins Medical and Surgical Association, European Society for Paediatric Urology

Disclosure: Nothing to disclose.

Additional Contributors

Martin David Bomalaski, MD, FAAP Pediatric Urologist, Alaska Urology; Clinical Assistant Professor, Seattle Children's Hospital

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

Disclosure: Nothing to disclose.

Hypospadias Differential Diagnoses

Diagnostic Considerations

Differential Diagnoses

References

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