The VCUAM (Vagina Cervix Uterus Adnexassociated

Malformation) Classification: a new classification

for genital malformations
Peter Oppelt, M.D.,a Stefan P. Renner, M.D.,a Sara Brucker, M.D.,b Pamela L. Strissel, Ph.D.,a
Reiner Strick, Ph.D.,a Patricia G. Oppelt, M.D.,a Hellmuth G. Doerr, M.D.,c
Guenther E. Schott, M.D.,d Juergen Hucke, M.D.,e Diethelm Wallwiener, M.D.,b
and Matthias W. Beckmann, M.D.a
a

Department of Gynecology and Obstetrics, c Department of Pediatrics, and d Department of Childrens Urology, University
Hospital, Erlangen; b Department of Gynecology and Obstetrics, University Hospital, Tbingen; and e Department of
Gynecology and Obstetrics, Bethesda Hospital, Wuppertal, Germany

Objective: With an incidence of up to 5% in the general population, genital malformations are a frequent clinical
occurrence. However, using the existing published classifications of malformations, difficulties arise in classifying genital malformations appropriately. The aim of the present study was to produce a simple, systematic, and
reproducible classification system.
Design: A systematic arrangement of genital and associated malformaltions, using a structure similar to that in
the TNM classification of oncological tumors, was developed and validated.
Setting: Patients with genital malformations in a university hospital.
Patient(s): Ninty-nine premenopausal patients with genital malformations.
Intervention(s): Patients were diagnosed for genital malformation using laparoscopy or magnetic resonance
imaging.
Main Outcome Measure(s): A new classification (VCUAM) is presented to evaluate patients with different genital
malformations.
Result(s): The external and internal female genital organs were divided into the following subgroups in
accordance with the anatomy: vagina (V), cervix (C), uterus (U), and adnexa (A). Associated malformations were
assigned to a subgroup (M) relative to each specific organ. The classification was validated in a group of 99
patients with genital malformations.
Conclusion(s): The VCUAM classification for the first time makes it possible to reflect even complex malformations in a precise and individual fashion, taking associated malformations into account. The classification
makes it easier to provide appropriate clinical care for the affected patients. (Fertil Steril 2005;84:14937.
2005 by American Society for Reproductive Medicine.)
Key Words: Genital malformation, genital classification, MRKH-syndrome, uterus subseptus, uterus didelphius,
cervix duplex, associated malformation, uterine malformation

Genital malformations have an incidence of 0.1% to 5% in

the general female population (1 4). A higher incidence of
3.5% to 6.5% has been observed in the diagnosis of groups
of patients with infertility (1, 2). The highest rate, with up to
38% of women having abnormalities in the mllerian ducts
(5), was observed in a group of patients with recurrent
abortions. In addition to uterine malformations, vaginal and
cervical abnormalities and malformations of the adnexa are
observed, as well as malformations of other organs and systems
such as the kidneys and skeleton. Adequate classification of
complex malformations of this typefor example, in MayerRokitansky-Kster-Hauser (MRKH) syndrome has not so far
been possible (6).

Received January 11, 2005; revised and accepted May 10, 2005.
Reprint requests: Peter Oppelt, M.D., Department of Gynecology and
Obstetrics, University Hospital, Universittsstrasse 2123, D-91054
Erlangen, Germany (FAX: 49-9131-8536185; E-mail: peter.Oppelt@
gyn.imed.uni-erlangen.de).

0015-0282/05/$30.00
doi:10.1016/j.fertnstert.2005.05.036

In 1907, to include various forms of genital malformation,

Strassmann (7) for the first time introduced systematic subdivisions into describing septate uterus (uterus bilocularis,
bipartite uterus, subseptate uterus) and bicornate uterus
(uterus bicornis, bifid uterus, didelphic uterus). Using this
broad classification limited to the uterus alone, it was not
possible to classify many malformations. In 1979, Buttram
and Gibbons (8) proposed a classification arranged into six
subgroups. This was revised in 1988 by the American Fertility Society (AFS) (6). In the latter scheme, the main
difference from the Buttram and Gibbons (8) classification is
that arcuate uterus is separated into its own subgroup, distinct from subseptate uterus. The limitations of the current
AFS classification lie in the impossibility of assigning variations of a malformation to precise organ subgroups. All of
the current classifications are primarily limited to the uterus
and vagina, disregarding malformations of the adnexa. Associated malformations, which are present in up to 30% of

Fertility and Sterility Vol. 84, No. 5, November 2005

Copyright 2005 American Society for Reproductive Medicine, Published by Elsevier Inc.

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TABLE 1
Description of the individual malformations relative to the organ.
Vagina (V)

0
1a
1b
2a
2b
3
4
5a
5b
S1
S2
S3
C

Normal
Partial hymenal atresia
Complete hymenal atresia
Incomplete septate vagina 50%
Complete septate vagina
Stenosis of the introitus
Hypoplasia
Unilateral atresia
Complete atresia
Sinus urogenitalis (deep confluence)
Sinus urogenitalis (middle confluence)
Sinus urogenitalis (high confluence)
Cloacae
Other
Unknown

Cervix (C)

0
1
2a
2b

Normal
Duplex cervix
Unilateral atresia/aplasia
Bilateral atresia/aplasia
Other
Unknown

Uterus (U)

0
1a
1b
1c
2
3
4a
4b

Normal
Arcuate
Septate 50% of the uterine cavity
Septate 50% of the uterine cavity
Bicornate
Hypoplastic uterus
Unilaterally rudimentary or aplastic
Bilaterally rudimentary or aplastic
Other
Unknown

Adnexa (A)

0
1a
1b
2a

3a
3b

Normal
Unilateral tubal malformation, ovaries normal
Bilateral tubal malformation, ovaries normal
Unilateral hypoplasia/gonadal streak (including tubal malformation
if appropriate)
Bilateral hypoplasia/gonadal streak (including tubal malformation
if appropriate)
Unilateral aplasia
Bilateral aplasia
Other
Unknown

0
R
S
C
N

None
Renal system
Skeleton
Cardiac
Neurologic
Other
Unknown

2b

associated Malformation (M)

Oppelt. VCUAM classification. Fertil Steril 2005.

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VCUAM classification

Vol. 84, No. 5, November 2005

FIGURE 1
VCUAM classification of uterus septus: V0, C0, U1b, A0, M0. (A) Hysteroscopic view. (B) Intraoperative
view.

Oppelt. VCUAM classification. Fertil Steril 2005.

cases depending on the genital malformation, are also not

taken into account.
MATERIALS AND METHODS
The structure of the system used to reflect oncologic tumors
in the TNM classification (9) served as the basis for establishing a new classification of genital malformations.
Because no underlying molecular-genetic alterations have
so far been identified for any of the existing malformations
(1, 10, 11), the intention was that the classification should be

oriented toward anatomic variations in the external and internal female genital organs. The aim of the classification is
to provide a description of malformations that is both as
individual and as precise as possible. The description should
be reproducible and clinically practicable.
The classification of malformations presented here was
tested in a group of 99 patients:
28 with typical MRKH syndrome
11 with atypical MRKH syndrome

FIGURE 2
VCUAM classification of uterus didelphys: V2b, C1, U2, A0, M0. (A) Vagina. (B) Intraoperative view.

Oppelt. VCUAM classification. Fertil Steril 2005.

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FIGURE 3
VCUAM classification of atypical Mayer-Rokitansky-Kster-Hauser syndrome: V5b, C2b, U4b, A0, MR.
(A) Vagina. (B) Intraoperative view.

Oppelt. VCUAM classification. Fertil Steril 2005.

17 with mllerian aplasia, renal aplasia, and cervicotho

racic somite dysostosis (MURCS) association

19 with subseptate uterus
18 with bicornate uterus, including 7 with single bicornate
uterus (uterus bicornis unicollis) and 11 with double bicornate uterus (uterus bicornis bicollis)
2 with testicular feminization
2 with didelphic uterus
1 with duplex cervix with a vaginal septum
1 with adrenogenital syndrome

All handling and examination of patients were done according to an approved ethics vote of the Department of
Medicine, University of Erlangen, Germany (Approval
Number 3074). This classification was used according to the
anatomy of internal and external genitalia for each of the
presented malformations listed in Table 1. The validation of
the malformation in its complexity was then performed.
RESULTS
The external and internal female genital organs are divided
into the following groups, in accordance with the anatomy:
vagina (V), cervix (C), and uterus (U). Because the ovular
transport mechanism plays a fairly minor role in the context
of in vitro fertilization (IVF) and intracytoplasmic sperm
injection (ICSI), the tube and ovary are combined under the
heading of adnexa (A). Systematic subclassification of
the possible organ changes was carried out in each group.
The number 0 was selected to indicate the lack of any
pathologic features. The more strongly marked the malformation was, the higher the numerical classification assigned
to it. The highest number in each group was assigned to
atresia or aplasia (see Table 1).
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VCUAM classification

Other associated malformations often occur along with

malformations of the mllerian ducts. These were included
in the classification in a separate subgroup, associated malformations (M). Because of the large number of possible
variations, precise classification of associated malformations
was avoided. Instead, only the affected organ group, such as
the renal system (R) or skeleton (S), is noted. Multiple
descriptions are possible in group M (see Table 1).
When an alteration cannot be accounted for with the
available classification, it should be documented with a plus
symbol (). When a malformation is not fully clarified, the
subgroup concerned is assigned a hash mark (#).
Three examples are given in Figures 1 through 3. All of
the patients in the group (n 99) could be described in detail
and in full using this new classification.
DISCUSSION
In the current AFS classification of genital malformations
(6), the specific term for the malformation (e.g., subseptate
uterus) is primarily used rather than a group classification (as
in group V septate). Problems arise due to the fact that
complex malformations in particular, such as those in group I
(hypoplasia/agenesis), cannot be classified individually and
precisely. The classification is also based mainly on the
description of uterine changes. Accompanying malformations such as duplex vagina, which often occurs in association with bicornate uterus (AFS group IV), cannot be recorded in the AFS classification. The same applies to the
adnexa; although only described rarely, hypoplasia or aplasia
of the adnexal region can occur in combination with mllerian aplasia, renal aplasia, and cervicothoracic somite dysostosis (MURCS) association (AFS group I) (12) or unicorn
Vol. 84, No. 5, November 2005

uterus, but it is not possible to describe and record such

malformations.
Associated malformations such as those of the renal system or skeletal system are not taken into account at all in any
of the current classifications. Depending on the individual
malformation, changes in the renal system are diagnosed in
11% to 30% of cases and changes in the skeletal system in
up to 12% of cases (13). This information, which is important for the patient, has not been reflected by any of the
classification schemes.
Other classification systems, such as the revised version
presented by Acien et al. (14), are mainly based on embryologic development. Although this includes changes in the
vagina, adnexa, and renal system in addition to those in the
uterus, it is extremely complex for use in clinical practice,
and assignment to specific classes is difficult.
In addition, the question arises of whether genital malformations need to be assigned to as many subgroups as those
presented in the AFS classification.
The primary criterion for the assessment of a complete
genital malformation should be to provide a precise and
comprehensible description, and this is not possible when
groups are assigned in relation to the uterus. The main
concern in the development of the vagina cervix uterus
adnex associated malformation (VCUAM) classification system proposed here is to provide a simple, systematic, clinical
classification in addition to providing a precise reflection of
the entire malformation.
In the same way as patients with breast cancer are described using the TNM classification, the VCUAM classification can be used to supplement the descriptive term for the
malformation (as in the previous examples). Using this precise structural assignment of the anomaly concerned, it is
possible to obtain a descriptive picture at any time of the
extent of the condition and its associated malformations.

Fertility and Sterility

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