II. Epidemiology

  1. Prevalence: 4.7 to 8 per male births

III. Predisposing factors: Family History

  1. Father with Hypospadias: 8% risk
  2. Brother with Hypospadias: 14% risk

IV. Pathophysiology

  1. Congenital defect of Urethral meatus development
  2. Urethral folds incompletely fuse
  3. Ectopic Urethral meatus between glans and perineum

V. Associated conditions

  1. Undescended Testicle (10% association)
  2. Inguinal Hernia
  3. Hydrocele
  4. Chordee (ventral penile curvature)

VI. Classification

  1. Anterior (Distal penis and glans): 50% of cases
  2. Middle (Midshaft of penis): 30% of cases
  3. Posterior (Scrotum, Perineum): 20% of cases

VII. Management

  1. Avoid Circumcision (foreskin needed for repair)
  2. Surgical repair at 6 to 12 months of age

VIII. Complications of surgical repair

  1. Complications 5-15% (highest for posterior Hypospadias)
  2. Urethrocutaneous fistula or Urethral diverticulum
  3. Meatal stenosis
  4. Wound Infection, hematoma or dehiscence

IX. Complications if not treated

  1. Urine stream deformity
  2. Sexual Dysfunction associated with curvature
  3. Infertility associated with proximal Urethral meatus

X. References

  1. Behrman (2000) Nelson Pediatrics, Saunders, p. 1645-7
  2. Borer (1999) Urol Clin North Am 26(1): 15-37 [PubMed]

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Ontology: Hypospadias (C0848558)

Definition (NCI_NCI-GLOSS) A birth defect in which the opening of the urethra (the tube through which urine leaves the body) is not in its normal place. In males with hypospadias, the urethra opens on the underside of the penis or between the anus and the scrotum. In females with hypospadias, it opens into the vagina. Hypospadias is much more common in males than in females, and can be corrected by surgery. Children with hypospadias have an increased risk of developing Wilms tumor (a type of kidney cancer).
Definition (NCI) A congenital abnormality in which the external urethral orifice is on the underside of the penis. In a minority of cases it is associated with other genitourinary abnormalities.
Definition (MSH) A birth defect due to malformation of the URETHRA in which the urethral opening is below its normal location. In the male, the malformed urethra generally opens on the ventral surface of the PENIS or on the PERINEUM. In the female, the malformed urethral opening is in the VAGINA.
Concepts Congenital Abnormality (T019)
MSH D007021
ICD10 Q54 , Q54.9
SnomedCT 416010008
LNC LA20090-9
English Hypospadia, HYPOSPADIAS, Hypospadias, unspecified, Hypospadias [Disease/Finding], hypospadia, hypospadias, Hypospadias (disorder), Hypospadias
French HYPOSPADIAS, Hypospadias, Hypospade
German HYPOSPADIE, Hypospadie, nicht naeher bezeichnet, Hypospadie
Swedish Hypospadi
Czech hypospadie
Finnish Hypospadiat
Russian GIPOSPADIIA, ГИПОСПАДИЯ
Portuguese HIPOSPADIAS, Hipospadia
Spanish HIPOSPADIAS, Hipospadia, hipospadias (trastorno), hipospadias, Hipospadias
Korean 요도하열, 상세불명의 요도하열
Croatian HIPOSPADIJA
Polish Spodziectwo
Norwegian Hypospadi
Dutch Hypospadie, niet gespecificeerd, Hypospadie
Italian Ipospadia