Unicornuate uterus with dorsal pancreatic agenesis

Case contributed by Dr Mohamed Saber

Presentation

Menorrhagia and bilateral flank pain

Patient Data

Age: 25 years
Gender: Female

MRI study shows an abnormal configuration and location of the uterus seen deviated to the left pelvic side, appears curved and elongated giving "banana-shaped' configuration with normally appearing zonal anatomy suggesting unicornuate uterus. The body of this unicornuate uterus is seen communicating with a single cervical canal and vagina.

To the left of this unicornuate uterus, there is a rudimentary uterine horn that shows its own endometrial cavity and preserved zonal anatomy. A small band of myometrial tissue is seen connecting the rudimentary horn with the body of the unicornuate uterus. No cervical canal for this horn and no communication between both endometrial cavities 

The right ovary with multiple cysts is seen at the right pelvic side. The left ovary is surgically removed for a serous cystadenoma

CT study shows absent pancreatic neck, body, and tail with visualized head and uncinate process.

Bilateral mild dilatation of the renal pelvicalyceal systems presumed to be secondary to a mild degree of pelviureteric junction stenosis.

Case Discussion

Here is a case of a unicornuate uterus (type a1b) associated with dorsal pancreatic agenesis. Association between Mullerian duct anomalies and dorsal agenesis of the pancreas is uncommon as genitourinary organs and pancreas have a separate embryologic origin 1

A unicornuate uterus is a type of class II Mullerian duct anomaly. It is subclassified according to the American Fertility Society as follows:

  • type a: with rudimentary horn 
    • a1: horn contains endometrium
      • a1a: communicating contralateral rudimentary horn contains endometrium (10%)
      • a1b: non-communicating contralateral rudimentary horn contains endometrium (22%)
    • a2: contralateral horn has no endometrial cavity (33%)
  • type b: no horn (35%)

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