Uterine malformation

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A uterine malformation is the result of an abnormal development of the Mullerian duct(s) during embryogenesis. The range of impact reaches from amenorrhea, infertility, pain, to normal functioning pending on the character of the defect. Uterine malformations affect about 1% of the female population.

Contents

1 Types
2 \"Double vagina\"
3 Diagnosis
4 Treatment
5 References
6 See also

Types

The American Fertility Society (now American Society of Reproductive Medicine) Classification distinguishes:

Class I: Mullerian agenesis (absent uterus).
*Uterus is not present, vagina only rudimentary or absent. The condition is also called Meyer-Rokitansky-Kustner-Hauser syndrome. The patient with MRKH syndrome will have primary amenorrhea.
Class II: Unicornuate uterus (a one-sided uterus).
*Only one side of the Mullerian duct forms. The uterus has a typical "banana shape" on imaging systems.
Class III: Uterus didelphys (double uterus).
*Both Mullerian 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 Mulllerian system fused into ist unpaired condition.
Class IV: Bicornuate uterus (uterus with two horns).
*Only the upper part of that part of the Mullerian 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 Mullerian ducts have fused, but the partition between them is still present, splitting the system into two part. 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. It is diagnosed by medical image techniques, ie a hysterosalpingogram, ultrasound, or an MRI, and can be corrected by hysteroscopic surgery.
Class VI: DES uterus.
*The uterine cavity has a "T-shape" as a result from fetal exposure to DES.

An additional variation is the arcuate uterus where there is a concave dimple in the uterine fundus within the cavity.

A rudimentary uterus is an uterine remnant not connected to cervix and vagina and may be found on the other side of an unicornuate uterus.

Patients with uterine abnormalities may have associated renal abnormalities including unilateral renal agenesis.Li S, Qayyum A, Coakley FV, Hricak H. Association of renal agenesis and mullerian duct anomalies. J Comput Assist Tomogr. 2000 Nov-Dec;24(6):829-34. PMID 11105695

\"Double vagina\"

As the vagina is largely derived from the Mullerian ducts, lack of fusion of the two ducts can lead to the formation of a vaginal septum, or "double vagina", a condition sometimes associated with a uterus didelphys or a uterine septum.Heinonen PK.Complete septate uterus with longitudinal vaginal septum.Fertil Steril. 2006 Mar;85(3):700-5. PMID 16500341

Diagnosis

Besides a physical examination, the physician will need imaging techniques to determine the character of the lesion: gynecologic ultrasonography, pelvic MRI, or hysterosalpingography. In addition, laparoscopy and/or hysteroscopy may be indicated. In some patients the vaginal development may be affected. Studies may have to address if other organs systems are affected (kidneys, spinal bone column) and when the child didn´t have her first mentruation.

Treatment

Surgical intervention depends on the extent of the individual problem.

Uterine malformation

Types

\"Double vagina\"

Diagnosis

Treatment

References

See also