Congenital Penile Anomalies

Congenital Penile Anomalies: Excerpt from In A Page: Pediatric Signs and Symptoms

For male genital anomalies, the most important consideration is detection of the anomaly before circumcision. A surgical consultation must take place before circumcision, because the treatment plan may involve the use of the foreskin in reconstruction.

Differential Diagnosis

• Hypospadias
  –Most common penile anomaly
  –Incidence of 1/500
  –Urethral meatus is typically located on the ventral surface of the glans penis
  –The meatus may also be located on the ventral surface of the penile shaft, the scrotum, or the perineum
  –Frequently associated with a ventral curvature of the penis (chordee) and/or a hooded prepuce
  –Less commonly associated with undescended testes or inguinal hernia

• Epispadias
  –Less common than hypospadias
  –Urethral meatus on dorsal surface of the penis
• Chordee
  –Ventral curvature of the penis
  –Most often associated with hypospadias
  –May occur without hypospadias when the ventral tissue is hypoplastic or fibrotic
• Dorsal hood
  –Incomplete formation of the ventral foreskin
  –May occur with hypospadias

• Micropenis (microphallus)
  –Defined as stretched penis length shorter than 2 standard deviations below the mean for gestational age
  –Associated with Prader-Willi, Kallmann Laurence-Moon-Biedl syndrome, and growth hormone deficiency

Workup and Diagnosis

• History
  –Prenatal exposure to sex steroids
  –Associated anomalies or syndromic findings
  –Family history
• Physical exam
  –Careful examination of the glans penis, the penile shaft, the scrotum, and the perineum
  –Examination for syndromic features (e.g., hypotonia, small hands and feet, almond-shaped eyes in Prader-Willi)
• Labs
  –Micropenis: Gonadotropin-releasing hormone, anterior pituitary function studies
• Radiology
  –Hypospadias: VCUG is indicated if hypospadias is severe to rule out other anomalies of the urinary tract
  –Micropenis: MRI of the hypothalamus and anterior pituitary
  • Studies
    –Genetics consultation if features of Prader-Willi, Kallmann or Laurence-Moon-Biedl syndrome are present
    –Karyotype for patients in whom the genitalia are at all ambiguous

  Treatment

  • Routine circumcision should be avoided as the foreskin may ultimately be used in the repair
  • Surgical revision is usually performed for cosmetic and functional reasons
  • Hypospadias and epispadias
    –Canalization of the penis improves urinary flow, erectile function, and fertility
    –Optimally, a skin flap is created using the foreskin
  • Chordee
    –Ventral release and urethroplasty at age 6–12 months
  • Dorsal hood
    –May not require treatment, or a modified routine circumcision may restore expected appearance
  • Micropenis
    –Androgen stimulation before puberty has been used but is controversial because it may impair penile growth during puberty

Book Source Details

• Book Title: In A Page: Pediatric Signs and Symptoms
• Author(s): Jonathan E. Teitelbaum, Kathleen O. Deantonis, Scott Kahan
• Year of Publication: 2007
• Copyright Details: In A Page: Pediatric Signs and Symptoms, Copyright © 2007 Lippincott Williams & Wilkins.

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Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.

More About This Book: Title: In A Page: Pediatric Signs and Symptoms Authors: Jonathan E. Teitelbaum, Kathleen O. Deantonis, Scott Kahan Publisher: Lippincott Williams & Wilkins Copyright: 2007 ISBN: 1-4051-0427-9

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