307- What is hypospadias?
Hypospadias is a congenital anomaly characterized by an abnormal site of ure-
thral meatus. It is a common congenital abnormality in males and occurs in
approximately 1 in 300, whereas it is rare in females with an incidence of 1 in
500,000. In males with hypospadias, the urethral opening may be located on the
undersurface of penis between the tip of glans and perineum. Hypospadias can
be classifi ed as distal (glandular and sub-coronal), mid (distal penile, mid-shaft,
and proximal penile), or proximal (penoscrotal, scrotal, and perineal). Distal
hypospadias is the most common (70 %), followed by mid-hypospadias (20 %)
and proximal hypospadias (10 %). Proximal hypospadias is usually associated
with DSD, whereas distal hypospadias is commonly idiopathic. Hypospadias is
commonly associated with chordee (ventral curvature of penis) and/or defi cient
foreskin with a dorsal hood.- What is chordee?
Chordee is defi ned as fi xed ventral curvature of penis. Penis develops from all
three germ layers; ectoderm develops into penile skin and prepuce, mesoderm
into corpora cavernosa and glans penis, and endoderm into corpora spongiosa
and penile urethra. Development of penis is androgen-dependant and possibly
higher levels of DHT are required for the formation of penile urethra, as com-
pared to the levels required for the development of corpora cavernosa, glans
penis, and prepuce. Therefore, during the development of external genitalia inabFig. 9.7 ( a ) Micropenis in a patient with hypogonadotropic hypogonadism. Note the presence of
testes in the same patient. ( b ) Phallus in a 3-year-old child with CAH
9 Disorders of Sex Development