Abstract :
Mullerian ageness is commonly known as Mayer-Rokitansky-Kuster-Hauser syndrome. It occurs in 1
among 4000-5,000 females. Mullerian ducts are the anlage of primordial tissue of female reproductive
tract, which is rare cause of agenesis or hypogenesis female genital tract. This case presented here is one
among the rarest in this tertiary care hospital.
Case History: 11-year-old female presented with complaints of fever and vague abdominal pain since
15 days. On examination, the presence of imperforate hymen was noted; USG revealed hematometra and
hematocolpus. Right ovary showed a simple cyst. Left ovary was poorly indiscernible; right kidney was
unremarkable while left kidney was not visualized.
Vaginal atresia was noted while doing hymenoplasty; hysterectomy with salpingoophorectomy was done
for hematometra with hematocolpus and hematosalpinx and the specimen was sent for HPE examination..
Conclusion: The case presented here is unique example of mullerian agenesis which presented with
unusual clinical features of fever and vague abdominal pain. It is also unique in that it is very rare case
which occurred in a female with early menarche and cryptomenorrhea that was incidentally diagnosed on
pelvic USG. Lastly, its incidence is noted to be rare in this tertiary care teaching hospital that it is the first
case of its kind reported in the last five years.
Keyword :
Mullerian agenesis, Haematometra, Haematocolpos imperforate hymen, MayerRokitanskyKuster Hauser
syndrome, Hematosalpinx unicornuate uterus vaginal atresia.