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Chapter 16: Adenomyosis
Endometrial ablation is still used today to control menorrhagia. This may make sense if the disease is limitedto the superficial
myometrium that is removed or destroyedby the ablation.In our
experience the adenomyosis extends more than 3 or 4 mm
beyond the endo-myometrial junction and often into the middle
or outer layers of myometrium. Ablation would have no effect on
the bleeding or on the associated pain that is so often the
predominant symptom. We have also seen the development of
collections of blood just beneath the scarred surface.They are not
symmetrical or central within the uterus, but rathereccentricand
irregular in shape (Figure 16.11). These are by definition not
hematometra in that they are outside of the endometrial canal.
On several occasions I have been called to the operating room to
do a transabdominal ultrasound scan during hysteroscopy. We
had reported a “hematometra” and none could be seen. In reality
the collection was concealed by scar tissue. It was readily apparent sonographically and the hysteroscopist could then be guided
into the collection for successful drainage.
There are gynecologists who will not do an ablation if they
feel a boggy, tender uterus; now, with good evidence from
imaging, ablation would seem to be a less useful approach.
Uterine artery embolization can reduce the symptoms in
patients with adenomyosis. Kitamura studied 19 patients with a
12-month follow-up; 25% had a reduced uterine volume, with
88.9% reporting improvement in their symptoms [19].
In conclusion, adenomyosis has burst onto the horizon of
female pelvic pathology. We mu st stop to ask ourselves some
questions. Why is adenomyosis now so common? Has the
incidence always been the same that we are now seeing or is it
increasing and why should that be? Is it all clinically significant?
Many cases seen have none of the classical symptoms, but do
these have other symptoms of which we are not aware? Did they
have symptoms that resolved spontaneously? Will they develop
symptoms in the future? Is adenomyosis really a disease or not?
For those women with symptoms, our ability to make this
diagnosis and institute therapy can truly be life-altering.
References
1. Bordman R, Jackson, B. Below
the belt: approach to chronic
pelvic pain. Can Fam
Physician 2006; 52:
1556–62.
2. Stones RW, Price C. Health
services for women with
chronic pelvic pain. J R Soc
Med 2002;
95: 531–5.
3. Mathias SD, Kuppermann M,
Liberman RF, Lipschutz RC,
Steege JF. Chronic pelvic pain:
prevalence, health-related
quality of life, and economic
correlates. Obstet Gynecol
1996; 87: 321–7.
4. Zondervan KT, Yudkin PL,
Vessey MP, et al. The
community prevalence of
chronic pelvic pain in women
and associated illness
behaviour. Br J Gen Pract
2001; 51: 541–7.
5. Tocci A, Greco E, Ubaldi FM.
Adenomyosis and
“endometrial-subendometrial
myometrium unit disruption
disease” are two different
entities. Reprod Biomed
Online 2008; 17: 281–91.
6. Gordts S, Brosens JJ, Fusi L,
et al. Uterine adenomyosis: a
need for uniform terminology
and consensus classification.
Reprod Biomed Online 2008;
17:244–8.
7. Verma SK, Lev-Toa ff AS,
Baltarowich OH, et al.
Adenomyosis:
sonohysterography with
MRI correlation. AJR 2009;
192: 1112–16.
8. Reinhold C, McCarthy S,
Bret PM, et al. Diffuse
adenomyosis: comparison of
endovaginal US and MR
imaging with histopathologic
correlation. Radiology 1996;
199:151–8.
9. Meredith SM, SanchezRamos L, Kaunitz AM.
Diagnostic accuracy of
transvaginal sonography for
the diagnosisof adenomyosis:
systematic review and
metaanalysis. Am J Obstet
Gynecol 2009; 200:
7.e1–6.
10
10. Yeniel O, Cirpan T, Ulukus
M, et al. Adenomyosis:
prevalence, risk factors,
symptoms and clinical
findings. Clin Exp Obstet
Gynecol 2007; 34: 163–7.
11. Simpson JL, Elias S, Malinak
LR, Buttram VCJr. Heritable
aspects of endometriosis.
Genetic studies. Am J Obstet
Gynecol 1980; 137: 327–31.
12. Ho ML, Raptis M, Hulett R,
et al. Adenomyotic cyst of
the uterus in an adolescent; a
case report. Pediatr Radiol
2008; 38: 1239–42.
13. Gerson Weiss MD, Maseelall
P, Schott LL, et al.
Adenomyosis a variant, not a
disease? Evidence from
hysterectomized menopausal
women in the Study of
Women’s Health Across the
Nation (SWAN) Fertil Steril
2009; 91: 201–6.
14. Graham KJ, Hulst FA,
Vogelnest L, Fraser IS,
Shilton CM. Uterine
adenomyosis in an
orang-utan (Pongo abelii/
pygmaeus).AustVetJ2009;
87:66–9.
15. DeVries K, Lyons EA,
Ballard G, Levi CS, Lindsay
DJ. Contractions of the inner
third of the myometrium.
Am J Obstet Gynecol 1990;
612: 679–82.
16. Egli GE, Newton M. The
transport of carbon particles
in the human female
reproductive tract. Fertil
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12: 151–5.
17. Laoag-Fernandez JB, Maruo
T, Pakarinen P, Spitz IM,
Johansson E. Effects of
levonorgestrel-releasing
intra-uterine system on the
expression of vascular
endothelial growth factor
and adrenomedullin in the
endometrium in
adenomyosis. Hum Reprod
2003; 18: 694–
18. Cho S,
Clinical effects of the
levonorgestrel-releasing
intrauterine device in
patients with adenomyosis.
Am J Obstet Gynecol 2008;
198: 373.
19. Kitamura Y, Allison SJ, Jha
RC, et al. MRI of
adenomyosis. Changes with
uterine artery embolization.
AJR 2006; 186:
855–64.
9.
Nam A, Kim H, et al.
133
Chapter
Congenital uterine malformations
17
Mona Aboulghar
Embryological development of the uterus
The uterus develops from fusion of the paramesonephric ducts,
which join in the midline at about the 10th week of gestation to
form the unified body of the uterus (Figures 17.1, 17.2).
Abnormalities in the resorption of the fused midline tissues
occur by the 20th week and can result in the formation of septa
of variable length and position. Apoptosis has been proposed as
a mechanism by which the uterine septum regresses [1]. The
protein Bcl-2 had been suggested to be absent in cases with
failure of regression [1].
The American Fertility Society [2], on the basis of the
previous work of Buttram and Gibbons [3], classified the
anomalies of the female reproductive tract into groups according to the degree of failure of normal development with similar
clinical manifestations, treatment, and possible prognoses for
their reproductive performance. The various müllerian anomalies are the consequence of four major disturbances in the
development of the female genitalia system during fetal life:
1. Failure of one or more müllerian duct to develop (agenesis,
unicornuate uterus without rudimentary horn)
2. Failure of the ducts to canalize (unicornuate uterus with
rudimentary horn without proper cavities)
3. Failure to fuse or abnormal fusion of the ducts (uterus
didelphys, bicornuate uterus)
4. Failure of resorption of the midline uterine septum (septate
uterus, arcuate uterus)
Incidence of müllerian uterine anomalies
Incidence of müllerian uterine anomalies in the general population is reported at around 5% [4]. Raga et al. reported an
incidence of 0.1–3.8% in normal fertile females, which reached
6.7% in infertile patients (5 ). A study using ultraso und examination done for various reasons in women and girls ranging in
age from 8 to 93 years gave a prevalence of 1 in 250 [6].
Müllerian uterine anomalies have been reported to be higher
in infertile patients. A recent report by Mazouni et al. [7]indicated that the main reasons for discovery of müllerian anomalies
were infertility (33.6%) and repeat miscarriage (18.2%).
With hysterosalpinograph y (HSG) the incidence was found
to be 10%, with arcuate uterus being the most common (57.6%),
followed by subseptate uteru s (18.2%), bicornuate uterus
(10.6%), uterus didelphys (3%), septate uterus (6%), and unicornuate uterus (3%) [8].
An association has been reported between polycystic ovaries
and uterine müllerian anomalies, Appelman et al. [9] gave a
44% incidence in polycystic ovary patients compared with 18%
in the normal ovary group (P < 0.001).
Investigations of patients with müllerian anomalies should
include searching for other associated anomalies, mainly renal,
as a high degree of association has been found in up to 36% of
patients [10].
Müllerian anomalies have been implicated in female subfertility, implantation failure, miscarriages, and preterm labor [7].
Thus, accuracy of diagnosis is important for planning of
management and assessment of the need for intervention.
Multiple imaging techniques are usually needed to reach final
diagnosis and confirmation and this often delays diagnosis [7].
Ideally, a technique with the highest accuracy and lowest rate of
false-positive results is needed.
Many diagnostic modalities are available: 2D ultrasound
(2D US), 3D ultrasound (3D US), with or without saline instillation; sonohysterography, HSG, magnetic resonance imaging
(MRI), hysteroscopy, laparoscopy. Hysterosalpingography,
hysteroscopy, and laparoscopy are invasive procedures and
diagnosis is based on the subjective impression of the operator.
Hysterosalpingography (HSG)
Hysterosalpingography is the first diagnostic test used for
patients with suspec ted müllerian anomalies, but it has a low
reported sensitivity of 44% [11].
HSG can detect a two-chambered uterus and allow assessment of the size and extent of a septum [12]. It reveals an image
of two hemicavities with a clear central division showing a typical
“Y” shape. With bicornuate and didelphic uteri the hemicavities
have convex medial wallsand the angle between them is generally
>90°, whereas with septate uteri the medial walls are straighter
and the resulting angle is generally <90° [Figures 17.3, 17.4).
However, the main drawback of this measurement is that it
Ultrasonography in Reproductive Medicine and Infertility, ed. Botros R. M. B. Rizk. Published by Cambridge University Press. © Cambridge
University Press 2010.
Figure 17.1. Embryology of the müllerian system.
Chapter 17: Congenital uterine malformations
Figure 17.3. Hysterosalpingography of septate uterus.
Figure 17.2. Unified müllerian ducts.
cannot reliably differentiate between septate and bicornuate uterus. Reuter et al. revealed a diagnostic accuracy of only 55% for
HSG in differentiating between septate and bicornuate uterus
[13]. It may miss small septal defects [14].
In a study of 110 patients with müllerian anomalies, including 73 septate uterus, 20 bicornuate, 10 hypoplastic, 4 unicornuate, and 3 müllerian agenesis, comparing HSG with other
imaging modalities, HSG diagnosed uterine hypoplasia in
70% and ultrasonography (US) in 30%. Diagnosis of bicornuat e
uterus was confirmed by US in 85% and by HSG in the remaining 15%. Diagnosis of unicornuate uterus was confirmed by
HSG in one case and by US in the remaining three. All cases of
müllerian agenesis were diagnosed by US. For women with
Figure 17.4. Hysterosalpingography of septate uterus.
septate uterus, diagnosis was suspected by HSG in 21.5% and
by hysteroscopy in 19.6%. For septate uterus, standard US
technique gave a false diagnosis in 80.8% of cases [7].
HSG is considered an invasive test; it necessitates injection
of a contrast medium and exposure to irradiation, in addition
to risk of pain and infection. Howe ver, HSG offers the advantage of the ability to assess tubal patency, which is not possible
with routine ultrasonography.
Two-dimensional ultrasonography
Two-dimensional (2D) ultrasonography was previously done
by the transabdominal route, but transvaginal ultrasonography
(TVS) is superior to the transabdominal route and is now the
standard imaging technique for the uterus [15]. It avoids obesity and abdominal fat, lies closer to pelvic structures, and uses
higher-frequency probes.
Pellerito et al. reported an accuracy of 92% for ultrasound
detection of müllerian anomalies, as compared with 100%
accuracy with MRI [16]; however, ultrasound has a reported
difficulty in distinction between various types of uterine
anomalies [17]. 2D TVS has a reported sensitivity of 100%
and specificity of 80% in diagnosing septate uterus [16].
Three-dimensional ultrasonography
The main advantage of three-dimensional (3D) ultrasonography over 2D is the ability to image the three orthogonal planes
of the uterus, of which the coronal view is the most important.
This view is essential for assessing the external uterine contour
and viewing the fundus, endometrium, and myometrium –
i.e. the whole length of the uterus down to the cervix – and
consequently for determining the exact type of uterine anomaly.
It also enables the measurementof the length of a uterine septum
135
Section 2: Ultrasonography in infertility
Figure 17.7. 3D US image of septate uterus.
Figure 17.5. 3D US image of arcuate uterus.
Figure 17.6. 3D US image of arcuate uterus.
and depth of fundal cleft. Another advantage of 3D ultrasound is
the possibility to store the images and re-evaluate the volumes
later, possibly pooling the data in a database for review by
another sonographer. Disadvantages of 3D ultrasound include
the time needed for learning manipulation of the volumes and
poor visualization in cases of shadowing by fibroids.
Several studies have reported high sensitivity and specificity
with 3D ultrasound in diagnosis of major müllerian anomalies.
Figure 17.8. 3D US image of septate uterus.
In a study that included 61 patients, Jurkovic et al. [18] reported
100% sensitivity and specificity, compared with 100% and 95%,
respectively, with 2D TVS. There were no false-negatives or
false-positives for the 3D technique in diagnosing uterine
müllerian anomalies. Three-dimensional ultrasound was compared with HSG and had a higher accuracy.
In this study criteria for diagnosis of an arcuate uterus were
normal appearance of the cervix and myometrium, absence of
fundal cleft, and a rounded appearance of the fundal portion
of the uterine cavity (Figures 17.5, 17.6)[18]. In cases of sub-
septate uterus, the proximal part of the uterine cavity was
136
Figure 17.9. 3D US image of small uterine septum.
Chapter 17: Congenital uterine malformations
Figure 17.10. 3D US image of small uterine septum.
partially divided by a septum and the myometrium appeared
normal. However, if a fundal indentation was present, it had to
be less than 1 cm in depth to allow classification as a subseptate
uterus (Figures 17.7, 17.8, 17.9, 17.10, 17.11, 17.12). The
diagnosis of bicornuate uterus was made when divergent,
well-formed cornua were seen separated by a large fundal cleft
(>1 cm).
Figure 17.11. 3D US image of long uterine septum.
Figure 17.12. 3D US image of septate uterus and small fundal cleft.
Raga et al. studied 42 patients with a history of infertility,
of whom 12 had m üllerian anomalies that were all detected by
3D ultrasound, while 11 were correctly classified [5].
In a study by Wu et al. [19], 40 patients with a history
suggesting müllerian anomaly were included, out of whom 28
women were confirmed to have Müllerian anomalies (by laparoscopy and/or hysteroscopy). These comprised 3 unicornuate
uteri, 3 bicornuate uteri, 12 cases with complete or partial
septate uterus, 9 cases of arcuate uterus, and one case of didelphic uterus. Three-dimensional sonography demonstrated all
congenital uterine abnormalities with a sensitivity and specificity of 100%. Septate uterus and bicornuate uterus could be
137
Section 2: Ultrasonography in infertility
1
Small
Figure 17.15. Sonohysterographic image of two uterine cavities in transverse
section.
that in both arcuate and subseptate uteri the length of remaining
uterine cavity was significantly shorter (P < 0.01) and the distortion ratio was significantly higher (P < 0.01) in patients with
recurrent miscarriage [20](Figures 17.13, 17.14).
Three-dimensional ultrasound has been found to be a
reproducible method in diagnosing uterine congenital malformations (20).
Figure 17.13. 3D US image of subseptate uterus.
1
Septum
Figure 17.14. 3D US image of long uterine septum.
correctly diagnosed using 3D sonography in 11 of 12 cases
(92%) and 3 of 3 cases (100%), respectively.
Use of 3D ultrasound to examine patients with recurrent
miscarriage as compared with normal controls showed no difference in the relative proportions of congenital uterine anomalies
in the two groups of women; arcuate and septate uterus were the
most common anomalies prevalent (90%). The measurement of
depth of the uterine septum and residual cavity depth showed
Sonohysterography
Sonohysterography is an ultrasound-aided technique that
entails injection of normal saline into the uterine cavity; in
some situations contrast medium could be injected to allow
visualization of the external contour of the uterus and could aid
in diagnosis of the type of müllerian anomaly.
Soares et al. compared HSG with TVS and with sonohysterography using hysteroscopy as the gold standard [11]. Out of
65 patients, 9 had uterine malformations. The sensitivity, specificity, positive predictive value (PPV), and negative predictive
value (NPV) of sonohysterography were, respectively, 77.8%,
100%, 100%, 96.6% compared with values for HSG of 44%,
96.4%, 66.7%, 91.5%, and with values for TVS of 44.4%, 100%,
100%, 91.5%. Thus, in this study sonohysterography performed
better in the diagnosis of müllerian anomalies. Sonohysterography
andTVShadnofalse-positivediagnoses(Figure 17.15).
Septate uterus is the most common müllerian anomaly and
is known to result in adverse obstetric outcomes; it is therefore
of great importance to differentiate septate from bicornuate
uterus, which is much less common, so that hysteroscopic
septum excision can be performed [21]. Surgery has reportedly
improved pregnancy rate from 3–20% to 70–90% [22].
Valenzano et al. reported on 54 patients included in a study in
which sonohysterography was compared with the standard investigation of hysteroscopy (though patients also had undergone
TVS & HSG investigations) [23]. Sonohysterography was able to
detect all the anomalies. The sensitivity and specificity of sonohysterography were the same as for hysteroscopy. However, although
there was no significant difference between the diagnostic capabilities of the methods analyzed, the authors recommended that,
because it is an easy and cheap technique, it should be the first used
in infertile patients as well as in those with recurrent miscarriage.
138
Chapter 17: Congenital uterine malformations
Table 17.1. The sensitivity, specificity, and positive (PPV) and negative (NPV)
predictive values of various imaging modalities for the diagnosis of septate
uterus in 420 patients with history of infertility and recurrent miscarriage
Imaging
modality
Transvaginal
sonography
Transvaginal
color Doppler
Saline contrast
sonography
Three-
dimensional
ultrasound
Reproduced from Kupesic and Kurjak [27] with permission from the American
Institute of Ultrasound in Medicine.
Sensitivity
(%)
95.21 92.21 95.86 91.03
99.29 97.93 98.03 98.61
98.18 100.00 100.00 95.45
98.38 100.00 100.00 96.00
Specificity
(%)
PPV
(%)
NPV
(%)
Lev-Toaff et al. compared 2D sonohysterography with 3D
sonohysterography and x-ray HSG and found 3D to be advantageous over the other two technique s, with the coronal plane
being the most important in providing information [24].
Addition of sonohysterography to 3D imaging allows precise recognition and localization of the lesion; therefore if 2D
and 3D SHG are normal, invasive diagnostic procedures such as
hysteroscopy can be avoided [25].
Guimarães Filho et al. compared sonohysterography with
HSG and hysteroscopy (as the gold standard), in investigating
patients with recurrent miscarriage [26]. The accuracy of sonohysterography and HSG was 90.9 and 85.2%, respectively; the
general sensitivity of sonohysterography was superior to that of
HSG (90.5 vs. 75%), and it also had a higher degree of agreement
with hysteroscopy (Kappa = 0.81 vs. 0.68). Pain was significantly
less with sonohysterography than with the other two methods.
The main disadvantage of 2D ultrasound and sonohysterography is that the techniques are operator dependent.
Kupesic and Kurjak compared four imaging techniques –
transvaginal 2D ultrasound, color and pulsed Doppler, sonohysterography, and 3D ultrasound – preoperatively in diagnosing septate uterus [27]. Four hundred and twenty infertile
patients undergoing hysteroscopy were examined. Of these
278 patients had an intrauterine septum, all com pla in ing of
adverse obstetric complications, of which 43 had repeated
spontaneous miscarriage; 71 had one spontaneous miscarriage;
81 had primary infertility; and 20 had preterm deliveries. The
highest sensitivity and specificity were demonstrated with 3D
ultrasound (Table 17.1).
In this study the height and thickness of the septum did not
correlate with the obstetric outcome, which contrasts with a
previously mentioned study [20] that reported that in patients
with recurrent miscarriage the degree of residual uterine cavity
distortion was higher. However, septal vascularity correlated
well. Patients with vascularized septa had significantly higher
prevalence of early and late pregnancy complications than those
with avascular septa, and this may reflect an increased amount
of muscle in the septum, producing local uncoordinated myometrial contractility.
Magnetic resonance imaging
Many magnetic resonance imaging (MRI) studies have shown a
very high sensitivity of 100% [16], and more recently values of
95% have been reported in cases of müllerian anomalies [7].
The main disadvantage of MRI is high cost, in addition to lack
of information as regards tubal patency.
Conclusion
Uterine müllerian anomalies have a high frequency of adverse
obstetric implications. Diagnostic modalities for detection of
uterine anomalies are many, with varying sensitivities and
specificities. Three-dimensional ultrasound seems very encouraging; it delivers good sensitivity, it is easy and is noninvasive,
and it is becoming more available.
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140
Chapter
18
Uterine septum
Mohamed F. M. Mitwally and Mostafa Abuzeid
Introduction
There is no doubt that contemporary developments in the technology of ultrasonography (US), including enhanced resolution
with increased ability to differentiate among various tissues and
structures, have made ultrasonography a strong armamentarium
for the diagnosisand management of uterine septum and various
other congenital malformations of the uterus. This chapter discusses the role of ultrasonography in the evaluation and management of uterine septum. We recently published a comprehensive
review of the diagnosis and management of uterine septum, as
well as of the reproductive problems that could be associated
with various types of uterine septi [1]. For the convenience of the
reader and by permissionof the editor, a great proportion of that
original chapter is included here.
We believe that this topic generates significant controversy
regarding diagnosis and treatment because of the paucity of
comprehensive evidence-based data on female congenital
anomalies, in particular, uterine septum. This has resulted in
the lack of a consensus on how the presence of a uterine septum
might affect female reproduction. We will discuss the available
data, aiming to provide a balanced appraisal that can help
reproductive medicine specialists to better co unsel patients
about their reproductive potential when a uterine septum is
discovered.
Embryology of uterine septum
Around the 10th week of gestation, the uterus forms from
fusion of the paramesonephric ducts (müllerian ducts), which
join in the midline in the absence of müllerian-inhibiting substance [2]. It is interesting that the müllerian ducts can develop
into two distinct types of tissue: the smooth-muscle tissue of the
uterus and the fibrous tissue of the cervix. This could explain
the various structural subtypes of uterine septum containing
different proportions of fibrous and muscle structure. Such
structural disparity might have implications for the mechanism
of reproductive failure associated with uterine septum [2].
A uterine septum results when there is incomplete resorption
of the adjacent walls of the two müllerian ducts. The resulting
fibromuscular structure can range from a slight midline septum
in the fundus of the uterus to complete midline division of the
endometrial cavity. Even segmental septa can exist, resulting in
partial communications of a partitioned uterus [2].
Reports of cases of complete vaginal septum associated with
different degrees of uterine septum ranging from complete
uterine septum with cervical duplication [3,4] to incomplete
septum (subseptate uterus) [5] challenged the classic theory of
unidirectional (caudal to cranial) müllerian development.
Accordingly, an alternative “bidirectional” theory was proposed, which suggested that fusion and resorption begin at
the isthmus of the uterus and proceed simultaneously in both
the cranial and caudal directions [6].
Prevalence of uterine septum
Although uterine anomalies have been reported in 0.1–2.0% of
all women, in 4% with infertility, and in up to 15% of those with
recurrent miscarriage, their true incidence is not known [4].
Pedro Acien suggested that the variability in the reported incidence of uterine anomalies is due to five factors: (1) the population studied; (2) the study design and physician’s interest
and awareness in finding or rejecting a uterine anomaly; (3) the
diagnostic method used; (4) the classes included as congenital
uterine anomalies in the different studies – e.g., hypoplastic
uterus, T-shaped anomalies, and arcuate uterus frequently not
included; (5) the criteria and diagnostic tools used to classify the
different types of uterine malformation [7].
In a selected group of women undergoing hysteroscopy for
abnormal uterine bleeding, Maneschi et al. [8] assessed the
prevalence of uterine anomalies and compared the reproductive
outcome in women with müllerian anomalies with that in
women with a normal uterine cavity. The authors found
müllerian anomalies in about 10% of women. Their findings
were similar to those reported in studies dealing with the
frequency of diagnosis of uterine anomalies in women undergoing tubal sterilization investigated by hysterosalpingography
(HSG), when septate, bicornuate, and arcuate uteri were found
in 1.9%, 3.6%, and 11.5%, respectively, of women with no
history of reproductive problems [9].
Uterine septum (complete or partial) has been the most
common (34–48%) type of structural uterine anomaly
[10,11,12,13]. The significance of the uterine septum comes
Ultrasonography in Reproductive Medicine and Infertility, ed. Botros R. M. B. Rizk. Published by Cambridge University Press. © Cambridge
University Press 2010.
Section 2: Ultrasonography in infertility
from the fact that it is the form of müllerian anomaly that is
believed to be associated with the poorest reproductive outcome, including low fetal survival rates of 6–28% and high rates
of spontaneous miscarriage [12,13,14].
Types
The classification of uterine anomalies divides the uterine septum into complete (septate) or partial (subseptate) groups
according, respectively, to whether the septum approaches the
internal os or does not. The complete septum that divides both
the uterine cavity and the endocervical canal may be associated
with a longitudinal vaginal septum. However, the presence or
absence of a longitudinal vaginal septum is not considered in
the classification [15]. Different classification systems have been
proposed for müllerian anomalies, with the early classification
systems being criticized for their confusion, incompleteness, or
irrelevant details. In 1979, Buttram and Gibbons [15] introduced the classification system of müllerian anomalies shown
in Table 18.1. The American Fertility Society (currently known
as the American Society for Reproductive Medicine or ASRM)
Table 18.1. The Buttram and Gibbons classification of müllerian anomalies [15]
Uterine
morphology Fundal contour
Normal Straight or convex Uniformly convex or
Arcuate Concave fundal indentation
with central point of
indentation at obtuse
angle
Subseptate Presence of septum that does
not extend to cervix, with
central point of septum at
an acute angle
Bicornuate Two well-formed uterine
cornua, with a convex
fundal contour in each
External
contour
with indentation
<10 mm
Uniformly convex or
with indentation
<10 mm
Uniformly convex or
with indentation
<10 mm
Fundal indentation
>10 mm dividing
the two cornua
revised the Buttram and Gibbons classification [16] with the
aim to make it an easy-to-use reporting system that would allow
clinicians to classify patients better, so that data could be accumulated more readily concerning the incidence of fetal wastage
and obstetric complications for these malformations (see Box
18.1 and Figure 18.1).
As shown in Figure 18.2 , the uterine septum has three
parts: the base (where it attaches to the fundus); the body of
the septum that extends down from the fundus all the way
toward the cervix (complete septum), as shown in Figure 18.3,
or stops somewhere between the fundus and the cervix
(subseptate or incomplete or short septum), as shown in
Figures 18.4 and 18.5; and the apex of the septum (the cervical
end of the septum).
In addition to the regular classification into long (complete)
or short (incomplete)subtypes,as shown in Figures18.3and18.4,
Box 18.1 American Fertility Society classification of
congenital uterine anomalies [16] (see Figure 18.1)
I. Agenesis: vagina, cervix, uterine fundus, fallopian
tube, or any combination thereof
II. Unicornuate uterus
*
Connected
*
Not connected
*
Without a cavity
*
Without a horn
III. Uterus didelphys (double uterus and cervix)
IV. Bicornuate uterus (complete, partial, arcuate)
V. Septate uterus
*
Complete
*
Partial
VI. Arcuate
VII. DES drug related, e.g., T-shaped uterus resulting from
diethylstilbestrol exposure
142
Figure 18.1. The American Fertility Society
(subsequently the American Society for
Reproductive Medicine) classification of congenital
uterine anomalies [16].