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PENILE AGENESIS
PENILE DUPLICATION
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Congenital absence of the penis, or
aphallia, is a rare anomaly caused by developmental failure of the genital tubercle.
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Approximate
incidence is 1 in 30 million.
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The phallus is
completely absent, including the corpora cavernosa and corpus spongiosum.
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The scrotum is
normal and the testes are undescended.
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The urethra
opens at any point of the perineal midline from over the pubis to, most
frequently, the anus or anterior wall of the rectum.
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Associated
anomalies:
o
Genitourinary
anomalies (50%), the most common of which is cryptorchidism; renal agenesis and
dysplasia.
o
GI defects
such as caudal axis anomalies also have been described.
o
Aphallia may
be associated with pregnancy complicated by poorly controlled maternal
diabetes.
Duplication of the
penis, or diphallia, is another rare anomaly resulting from incomplete fusion
of the genital tubercle.
- 2 distinct forms:
- The most common is associated with
bladder-exstrophy complex. The patient exhibits a bifid penis consisting
of 2 separated corpora cavernosa associated with 2 independent
hemiglands.
- The second form, or true diphallia, is
an extremely rare congenital condition. It presents in many varied ways,
ranging from duplication of the glans alone to duplication of the entire
lower genitourinary tract. The urethral opening can be in normal position
or in a hypospadiac or epispadiac position.
- Associated anomalies of the GI,
genitourinary, and musculoskeletal systems occur (these anomalies are the
principal cause of mortality).
- Gripp KW, Barr M Jr, Anadiotis G:
Aphallia as part of urorectal septum malformation sequence in an infant
of a diabetic mother. Am J Med Genet 1999; 82(5): 363-7.
- Husmann DA, Cain MP: Microphallus:
eventual phallic size is dependent on the timing of androgen
administration. J Urol 1994; 152(2 Pt 2): 734-9
- Maruyama K, Takahashi A, Kobayashi T:
Diphallia and the VATER association. J Urol 1999; 162(6): 2144.
- Rock SD, Gearhart JP: Complete lower
urinary tract duplication with true diphallia presenting as a rare
covered exstrophy variant. J Urol 1997; 157(5): 1907-8