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Case of the Month

February 2012

Patient Data: 14 year old female patient

Clinical Info: Intermittent periodical pain in the lower abdomen. She has a normal menstrual cycle!

Transverse image of a cystic structure dorsal of the nearly empty bladder. There is also a small amount of fluid in Douglas pouch

Longitudinal image of the lower abdomen and the cystic structure and a uterus with a fluid filled endometrial cavity. This is a situation called hematocolpos (blood in the vagina) and hematometra (blood in the uterine cavity) Usually these patients have absent or diminished mestrual periods but this girl has a normal menstrual cycle

The solution of the normal menstrual cycle lies in this transverse image, where not only one but also a second uterine body is found (right arrow)

Another transverse image showing two uterine bodies and a normal right ovary (dorsal arrow)

Another transverse image showing the cystic fluid filled vagina and two uterine bodies one normal and one filled with blood. The right ovary (dorsal arrow) is normal

Longitudinal image of the normal uterine body and normal uterine cavity on the left side of the patient with a normal left ovary (dorsal arrow)

Transverse image of the normal left uterine body and left ovary A double uterus is called a uterus didelphys.This patient also had a double or septated vagina of which the right side was occluded causing the hematometra and hematocolpos.

In cases of genital abnormalities always check the kidneys. No right kidney was found in this girl

The left kidney is normal but slightly larger than normal

The American Fertility Society (now American Society of Reproductive Medicine) Classification distinguishes:
Class I: Müllerian agenesis (absent uterus).
Uterus is not present, vagina only rudimentary or absent. The condition is also called Mayer-Rokitansky-Kuster-Hauser syndrome. The patient with MRKH syndrome will have primary amenorrhea.
Class II: Unicornuate uterus (a one-sided uterus).
Only one side of the Müllerian duct forms. The uterus has a typical "penis shape" on imaging systems.
Class III: Uterus didelphys, also uterus didelphis (double uterus).
Both Müllerian ducts develop but fail to fuse, thus the patient has a "double uterus". This may be a condition with a double cervix and a vaginal partition (v.i.), or the lower Müllerian system fused into its unpaired condition. See Triplet-birth with Uterus didelphys for a case of a woman having spontaneous birth in both wombs with twins.
Class IV: Bicornuate uterus (uterus with two horns).
Only the upper part of that part of the Müllerian system that forms the uterus fails to fuse, thus the caudal part of the uterus is normal, the cranial part is bifurcated. The uterus is "heart-shaped".
Class V: Septated uterus (uterine septum or partition).
The two Müllerian ducts have fused, but the partition between them is still present, splitting the system into two parts. With a complete septum the vagina, cervix and the uterus can be partitioned. Usually the septum affects only the cranial part of the uterus. A uterine septum is the most common uterine malformation and a cause for miscarriages.

The patient was referred to the gynaecologist who confirmed the diagnosis

For other uterine abnormalies and examples of hematocolpos see

Copyright © Dr. T.S.A. Geertsma, Ziekenhuis Gelderse Vallei, Ede, The Netherlands. All rights reserved.

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