319- What are the DSDs associated with inguinal hernia?
The descent of testes from abdominal cavity to scrotum is accompanied with a
peritoneal lining called processus vaginalis, which is gradually obliterated after
the completion of testicular descent. Persistence of processus vaginalis predis-
poses for the development of inguinal hernia and hydrocele. Inguinal hernia is
more common in boys as compared to girls (10:1) and majority
(80–85 %) of inguinal hernia is unilateral. Bilateral inguinal hernias are uncom-
mon and its presence in a girl child should raise a suspicion of complete
androgen insensitivity syndrome (CAIS). The other DSDs that are associated
with inguinal hernia are 17-β hydroxysteroid dehydrogenase type 3 defi ciency
(17-βHSD3), ovotesticular DSD (OT-DSD), and persistent Mullerian duct syn-
drome (PMDS). The contents of hernial sac differ in all these disorders; testis
in CAIS and 17β-HSD3 defi ciency, ovotestis in OT-DSD and Mullerian deriva-
tives in PMDS (Fig. 9.15 ).abFig. 9.14 ( a ) A 20-year-old patient presented with genital ambiguity and peripubertal gyneco-
mastia. Note the surgical scar of reduction mammoplasty. ( b ) External genitalia showing Tanner
pubic hair stage P 4 , small phallus, penoscrotal hypospadias, and bilateral testicular volume
10–12 ml. Hormonal profi le was suggestive of PAIS
9 Disorders of Sex Development