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First stage. The closed uterine cavity and the independent cervical one exposed.

the vaginal touch will show one or other of the states of the cervix which have been
named above. It will be impossible to pass a sound fully into the uterine cavity.




FIG. 122. The operation of trachelostomy.
Second stage. Reunion of the two cavities by excision of intervening tissue.

In a few cases the vault of the vagina itself may be imperforate. The rectal touch
will then enable the distended uterus to be palpated. Goullioud 1 has recently
attempted to systematize the operative treatment to be carried out under various

1 Ann. de gyncc. et cTobslel. 2nd s. ix. 700, 1912 : and These, Lyon, 1911.



ANOMALIES OF THE UTERUS



253



circumstances. The objects to be attained are relief of the haematometra, with
the establishment of a permeable cervical canal and the conservation of the patient's




FIG. 123. The operation of trachelostomy.
Third stage. Bringing together of the two mucous surfaces.

sexual organs, and, if possible, her reproductive powers. Naturally enough these
aims are not always easy of accomplishment ; but the sufferings of the patient




FIG. 124. The operation of trachelostomy.

Fourth stage. Closure of uterine incision, which is covered partly by peritoneum from the

utero-vesical pouch.

call for operative interference, and the common way chosen, namely the removal
of the ovaries, is certainly not the best conceivable. It must be remembered that
most of the patients are quite young.

For an imperforate state of the isthmus Goullioud recommends trachelostomy



254



MALFORMATIONS



in preference to blind attempts to pass a probe or other instrument up from the
vaginal and cervical canals through the block into the uterine cavity. An abdom-
inal section is performed ; the bladder is separated from the uterus, and a vertical
incision is made in the anterior wall of the latter, opening into the haematometric
cavity, and exposing the atresia ; a communication between the uterine and cervical
cavities is established by excising a wedge-shaped portion of the fibrous tissue, and
bringing the mucous membrane of the body into touch with that of the cervix, so
as to establish continuity of mucous surfaces ; the uterine incision is then closed,
and, if need be, a salpingostomy may be done to give escape to the blood in
the haematosalpinx. Goullioud senior performed this operation successfully, and
gave to it the name of abdominal trachelostomy (vide Figs. 121, 122, 123, and 124).





First stage. Second stage.

FIGS. 125, 126. Hofmeier's operation of hystero-elytrostomy. (After Goullioud.)

For the cases in which the cervical canal is replaced by a block of fibrous tissue,
the treatment formerly employed was incision of the haematometra from the vagina,
an operation which, although apparently simple, was apt to be followed by ascending
infection and even by death. Tapping of the blood-accumulation in the uterus
with a trocar is still more uncertain in its results, and dangerous in its technique.
M. Hofmeier 1 was the first to suggest a more scientific method of dealing with the
haematometra, resulting from cervical imperforation. From the vagina he made
an opening into the bulging haematometra, not directly through the fibrous piece
of occluding tissue, but to one side of it (vide Figs. 125. 126) ; in this way he estab-
lished a uterine fistula, and sutured the mucous membrane of the uterus to that of
the vagina. The operation is known as hystero-elytrostomy. Goullioud (loc. cit.}, on
the other hand, recommends a large resection of the interposed block of fibrous



1 Ztficfir.f. GebmtsJi. u. Gyniik. Hi. 1, 1904.



255



tissue, so as to open into the cavity of the haematometra and coapt the two mucous
membranes. In both these operative procedures it may be found necessary to
open the abdomen in order to determine the state of the adnexa, for if there is
haematosalpinx, the risks of ascending infection and peritonitis are much increased,
and to prevent them it will be needful to remove the tubes.

The cases in which the vagina and the uterus are completely shut off from one
another scarcely fall under the heading of uterine atresia, but rather under that
of vaginal imperforation ; a word or two may be said about them here. Halban x
met with an instance of this kind, in which the uterus, distended with blood, was







FIG. 127. Hofmeier's method of establishing communication between the uterus and the vagina.

(After Goullioud.)
u, Uterus ; c, dilated cervix ; v, vagina.

separated from the vagina by an interval too great to be pierced through ; with
great ingenuity he opened the abdomen and turned the uterus upside down
so that its fundus was brought into contact with the vaginal roof, he then
opened into the uterine cavity at the fundus and into the vagina at its roof, and
sutured the two cavities together. It may be added that there was no risk of a
pregnancy since the adnexa were diseased. Even in these difficult cases, however,
it is not hopeless to attempt to establish communication between the vagina and the
uterus, and to conserve the functions of the latter. Hofmeier (loc. cit.), for instance,
did an operation in three stages (vide Fig. 127) : First he approached the imperfora-
tion from the vaginal aspect, and began the separation of the rectum from the

1 Ztschr.f. Geburtsh. u. Gynak. xlix. 17, 1903.



256



MALFORMATIONS



I



bladder ; then he opened the abdomen, completed the separation, opened the
lower part of the haematometra, placed two silk sutures on the margins of the
opening, and passed them through into the vagina ; and finally from the vaginal
aspect again he drew down the sutures and the uterine opening, and stitched the
cervical to the vaginal mucosa. In many cases the operative plans which have
been described may be too difficult of accomplishment ; hysterectomy with the
preservation of an ovary is then the best treatment. 1

Minor Malformations of the Uterus. Sometimes stenosis of the cervix of a con-
genital kind is found, the sound passing in only with difficulty ; at other times
there is a transverse septum or diaphragm in the cervical canal. In the one case
dilatation is called for, and in the other excision of the septum or diaphragm.
Dysmenorrhoea may be complained of in either case. The os uteri externum

may be divided into two openings
by a frenum (uterus biforis), a con-
dition which is normal in the ant-
eater (Pozzi) ; and this malforma-
tion may exist without any other
sign of duplication of uterus or
vagina. It may conceivably give
rise to trouble in labour, and the
frenum may require excision. Apart
from atresia the cervix may be
small (uterus parvicollis) or absent
(uterus acollis).

Slight changes in the form of
the fundus occur. There is the
anvil-shaped uterus (uterus incudi-
formis sen biangularis), in which the
normal convexity of the fundus is
lacking, and a straight line joins
the two Fallopian tubes ; and
closely allied to it is the uterus,
with the flat fundus (uterus plani-
fundalis). Congenital prolapsus uteri is a very rare anomaly. Since 1897, when
Thomson and the present author 2 reported two (vide Fig. 128) and summarized the
six previously recorded instances, the number has been more than doubled, but

1 Berard, Lijon mcd. cxvii. 493, 1911. 2 Amer. Journ. Obstct. xxxv. 161, 1897.




Spiria
bifida



Ureth

N^ ^Jf^ ^,

Anus

FIG. 128. Case of congenital prolapsus uteri with spina
bifida. The uterus lies below the level of the pelvic
outlet, and is retroverted. (Ballantyne and Thomson.)



ANOMALIES OF THE VAGINA 257

yet it remains one of the least frequent of uterine anomalies. The association of
the displacement with spina bifida of the lumbo-sacral region, whilst not constant,
has been so common as to suggest some causal connection ; and there is some
evidence that spina bifida is accompanied by changes in innervation, and by a
thinning and weakening of the levatores ani muscles, which would serve in part to
explain the falling down of the uterus. 1 Congenital descensus is a true prolapse of
the uterus, and must be distinguished from congenital hypertrophic elongation of
the cervix ; and the distinction can usually be made quite easily by noting the length
of the vaginal canal and that of the uterus itself. There may be some degree of
rectal protrusion as well, and associated deformities are clubfoot and spina bifida.

Abnormal communications between the uterus and neighbouring organs will
be considered under the malformations of the vulva and lower part of the vagina.

ANOMALIES OF FORMATION OF THE VAGINA

It is now generally admitted that the development of the vagina is not so simple
as was once supposed ; the fusion of the lower parts of the two Miillerian ducts
does not lead to the formation of the whole canal, but only to that of its upper
portion, and probably, as Berry Hart maintains with some interesting and weighty
embryological evidence, 2 its lower third takes its origin in the coalescence of the
Wolffian bulbs and the urinogenital sinus. If this be so, then various complicated
arrangements of parts in the neighbourhood of the vaginal orifice, and all the cloacal
deformities become easier of comprehension ; it is simpler, for instance, to under-
stand how the upper two-thirds of the vagina may be lacking, whilst the lower
third is present.

Double Vagina. With the vagina, as with the uterus, what is commonly called
duplication is really lack of fusion of the two Miillerian ducts ; in the case of the
double vagina it is in their lower stretches that the ducts have failed to unite. At
the same time, true duplication of the vagina may occur, although with great rarity.
It is seen in the case of some pygopagous female twins, and there are a few cases
in which it may be hazarded that the only evidences of the fusion of two female
foetuses are to be noted in the pelvic region. Thus, in Suppiger's little patient
(a girl of 21 months) there was duplication of the lower end of the vertebral column,
of the bladder, the uterus, the rectum, of the vulva, and of the vagina ; and
it seems necessary to postulate something more, in such an arrangement of parts,

1 W. von Radwanska and R. Graf, Gyndk. Rundschau, vii. 515, 1913 ; Monatsschr. f. Geburtsh. u. Gyn.
xxxiv. 645, 1911.

2 Trans. Edin. Obst. Soc. xxvi. 259-305, 1901.

VOL. I S



258



MALFORMATIONS



than a mere failure of the Miillerian ducts to unite. 1 So, too, in the adult married
woman (vide Fig. 129) described by J. E. Gemmell and A. M. Paterson : 2 she had an
unusually wide pelvis, there were two separate vulvae, two vaginae, two uteri (each
of which had been pregnant), and two bladders, but only one anal aperture ; the
pubic bones were widely distant in front, a circumstance which made labour very
easy. The vaginal canals did not lie side by side as in the ordinary cases of uterus
didelphys and vagina septa, but rapidly diverged, and the two uteri lay far apart in
the " enormously wide " pelvis.




FIG. 129. Case of double vulva, vagina, and uterus. (After Gemmell and Paterson.)

Far commoner are the cases of double or septate vagina, in which the vulva
is single or at most there are two hymeneal openings. The two vaginal canals are
separated by a longitudinal septum, running in the great majority of cases antero-
posteriorly, as in Barozzi and Fournier's patient 3 (vide Figs. 130, 131), and only
rarely in a transverse direction ; the canals, therefore, are generally lateral in their
relation to each other, and not often one in front of the other. The vaginae are
rarely of the same size and form, one being smaller and narrower than the other,

1 Correspondenzbl.f. Schweizer. Aerzte, No. 14, p. 418, 1876 ; and No. 24, 1878.
2 Journ. Obsl. and Gyn. Brit. Emp. xxiii. 25, 1913 ; xxiii. 139, 1913. 3 La Gynecologic, xviii. 197, 1914.



DOUBLE VAGINA



259



and lying to some extent to the side of it. The septum differs in thickness and
structure, but in its most complete form it is made up of muscular tissue covered
on each side by mucous membrane. It may be defective above or, more often,
below, leading to the two varieties, vagina septa infra and vagina septa supra ; or, in
rare cases it may show perforations at places. As has been stated already (vide
p. 237), the uterus may be double, and belong to the didelphous, bicornute, or




FIG. 130. Case of double vagina. (After Barozzi and Fournier.)

septate type ; indeed it is somewhat rare for the womb to show no sign of duplication.
In Holste's patient, 1 however, the uterus was single. Sometimes the only indication
of the double nature of the vagina is found in the presence of a ridge on the anterior
and posterior wall, and these ridges are commonly all that is left after the septum has
been divided in labour. One or both the vaginae may be atresic and may contain
blood, setting up the condition known as haematocolpos (unilateral or bilateral).

1 Zenlrlbl.f. Gyndk. xxxvii. 965, 1913.



260



MALFORMATIONS



Unless there is some degree of atresia, double vagina does not usually give rise
to any symptoms drawing attention to it before marriage. If, however, one or

both cavities are imperforate below,
then blood accumulates, monthly
suffering follows, there may be a
swelling felt through the vulvar
aperture, and operation will be re-
quired to relieve the condition.
Dyspareunia has been reported, and
is due to narrowness of the canal.
Sterility may result from the dys-
pareunia, or from intercourse taking
place in that one of the two canals
which has no cervix in it (Fig. 131).
In other cases the malformation
passes unnoticed till pregnancy has
occurred (as in Barozzi and Fournier's
case, loc. cit. supra), and labour is in
progress ; then various things may
happen. The child may come down
one canal, and the septum be pressed

FIG. 131. Case of double vagina. (After Barozzi and to the side and perhaps go unob-
Fournier.)

Transverse section, showing the cervix opening into the served ; or the intervening tissue
right vagina whilst the left vagina ends in a cul-de- may nrs t stretch and finally tear,
sac ; the septum divides the two canals.

and the rupture may extend into

neighbouring organs, with dangerous or disastrous effect ; or the partition may be
recognized during delivery, ligatured, and safely divided. Some curious occurrences
have been chronicled. In Pierra's patient, 1 for instance, the uterus was bi-lobed,
there was one cervix, and the vagina was divided into two canals by a thick septum,
which ceased immediately below the cervix ; the child presented by the breech
(a not uncommon presentation in such cases), and the medical attendant drew
down one foot into the right (the larger) vagina, only to find further progress
arrested by the other foot having passed into the left canal ; two long clamps were
applied to the septum, which was divided between them ; and the child, which had
been astride the partition, was safely delivered. In Graarud's patient 2 there were
two uteri as well as two vaginal canals, and both uteri had been pregnant.
1 Bull. Soc. cVObsl. de Paris, xi. 236, 1908. 2 Norsk Mag.f. Laagevidensk. 5 R. v. 1093, 1907.




ATRESIA VAGINAE 261

Vouters, 1 in the case of a primipara, found a vaginal septum over 3 inches in length
obstructing the progress of the child's head, and calling for division between two
catgut sutures.

The diagnosis of double vagina does not, as a rule, present much difficulty :
the finger passes up first one and then the other canal, or, if one be imperforate and
dilated, it notes the swelling in the lateral wall ; a finger can usually be passed on
each side of the septum, and its extent and thickness recognized. If the two canals
communicate above, and if there be but a single cervix, it is easy to feel it through
each canal, which gives the impression that the cervix is double. C. Sauvage 2 sum-
marizes the treatment of the septum under the following rules : Do not interfere
during pregnancy, for fear of resulting cicatrization of the canal and risk of septic
infection ; interfere only exceptionally during the period of cervical dilatation ;
ligature and cut through the septum as soon as dilatation is complete, and the
presenting part is forced against the obstruction. The treatment of the lateral
haematocolpos, resulting from imperforation of one vaginal canal, will be dealt
with under atresia vaginae.

Unilateral Vagina. The rare cases of uterus unicornis are generally associ-
ated with a defective state of the vagina, for the latter is really only half a canal,
being the product of the development of but one Miillerian duct. It is narrow,
and placed rather too much to one side of the middle line. In the rare cases of
pregnancy in a uterus unicornis the narrowness of the vagina will increase the diffi-
culty and danger of the delivery.

Vagina Rudimentaria and Defectus Vaginae. Vagina rudimentaria is a vague
expression, and denotes, at best, a morbid state indistinguishable clinically from
atresia vaginae and defectus vaginae. H. Kiister, 3 however, may be consulted on the
anomaly. Complete absence of the vagina (defectus vaginae) also can hardly be
determined during life, and is best considered under atresia vaginae (q.v.).

Atresia Vaginae. In its morbid anatomy, vaginal atresia presents various
degrees of imperforation and various associated anomalies of neighbouring parts.
In its most marked form no trace of the canal is found save a fibrous or fibro-
muscular band in the tissue between the bladder and rectum ; in a less extreme
form, part of the vagina is present, whilst the remainder is solidly imperforate ;
and in a still less marked form, there is simply a membranous obstruction or per-
forated diaphragm at one part of the passage. The position, also, of the imper-
foration varies : it may exist throughout the whole length of the canal, or it may

1 Bull. Soc. d'Obst. de Paris, xiv. 448, 1911. 2 Rev. prat, (fobst. et de ptdiai. xxiii. 129, 1910.

3 Zeitschr. f. Geburtsh. u. Gyncik. Ixvii. 692, 1910.



262 MALFORMATIONS

be present only at the upper part, or the middle part. With the knowledge of the
development of the lower part of the vagina which is now possessed, it may be
regarded as probable that atresia in that position is due to defective coalescence of
the Wolffian bulbs with the urino-genital sinus. The uterus in cases of vaginal
atresia may be normal, double, rudimentary, or absent ; the ovaries and tubes are
commonly healthy, at any rate at first ; the vulva is generally normal, and
possesses a hymen, but some instances have been reported of defective develop-
ment of the pudenda ; and in married women the small vestibular canal and even
the urethra may be dilated as the result of attempts at coitus. At puberty the
commencement of menstruation may lead to pathological changes. If the uterus
be present in a more or less perfect form, and the whole vagina be absent, haemato-
metra develops, and the whole uterus is converted into a large rounded sac, con-
taining blood in various conditions of colour and consistence. In a few cases the
cervical canal alone becomes distended with blood, as in Miles H. Phillips' s case
of haematotrachelos in .a uterus bicornis unicollis, with absence of the vagina. 1
When the upper part of the vagina is present blood first accumulates in it, pro-
ducing haematocolpos, and later haematometra follows ; and when only the lower
part of the canal is atresic, nothing more than haematocolpos may be the conse-
quence, and the uterus may be found as a small body resting upon the apex of the
distended vagina. On the other hand, blood retention may extend farther up in
the genital system, and the Fallopian tubes become distended (haematosalpinx) ;
even the ovary has been found the seat of a large blood-cyst (haematovarium), as
in Abram Brothers' patient, 2 in whom the menstrual blood had been accumulating
for four years. If the blood contained in any of these organs becomes infected
from rupture, aspiration, or ineffective operation, pus may form, leading to pyo-
colpos, pyometra, pyosalpinx, and pyovarium. In a remarkable case reported
by Chaput, 3 there was haematometra of a uterus bicornis, along with torsion of
the pedicle of the haematosalpinx, which coexisted therewith. Balfour Marshall 4
has put on record a case of absence of the vagina, associated with displacement of
the left kidney into the pelvis ; ovaries were present, but the uterus was reduced
to a rudimentary horn on the right side.

Clinical Features. The clinical features of atresia vaginae will in part have been
foreshadowed by the study of the morbid anatomy ; they are in large measure the

1 Journ. Obstet. and Gynaec. Brit. Empire, xiii. 355, 1908.

2 Contrib. Sc. Med. and Surg. . . . 25th anniv. founding, of New York Postgrad. Sch. and Hosp.,
pp. 294, 339, 1908 ; abstract in Amer. Journ. Obst. Ivii. 572, 1908.

3 Rev. de gynec. et de chir. abd. x. 963, 1906.

4 Journ. Obst. and Gynaec. Brit. Empire, xxiii. 238, 1913.



ATRESIA VAGINAE 263

result of the accumulation of menstrual blood behind the imperf oration, and they there-
fore do not appear as a rule till after the period of puberty is reached. Occasionally,
however, in the young child the retention of mucus may cause a swelling which,
by pressing upon the bladder and rectum, produces dysuria and constipation ; but
as a rule puberty marks the beginning of suffering. There is then amenorrhoea,
or more correctly cryptomenorrhoea, and the gradual development of a swelling
in the lower abdominal region, which may bulge also in the region of the vulva and
perineum. In this swelling it is generally possible to make out fluctuation, but if
the uterus is poorly developed or not developed at all, the tumour due to menstrual
retention may be inconspicuous or absent. Soon severe pelvic pain, recurring
periodically, is added to the cryptomenorrhoea, whilst bleeding from other parts
of the body, such as the stomach and lungs (the so-called vicarious menstrual
haemorrhage), has been reported. If the uterus be absent, these symptoms may not
appear, and the beginning of suffering may then be postponed till marriage, when
the dyspareunia, or complete inability to accomplish cohabitation, may be the source
of much trouble and unhappiness, and may even lead to insanity. The imperfect
coitus which is possible by elongation of the short vestibular canal or by dilatation
of the urethra gives no real satisfaction, and, from the woman's point of view,
increases distress. There is, of necessity, sterility ; but it is noteworthy that
operative treatment has occasionally been followed not only by establishment
of menstruation but by the occurrence of one or more pregnancies and labours,
(although Caesarean section has now and again been required to complete the
delivery).

It is probable that most of the cases of atresia vaginae which are congenital
are developmental defects ; but the possibility that some of them have arisen during
foetal life (or may arise later in infantile existence), from inflammatory conditions
of an existing vagina leading to its obliteration, cannot be quite excluded. Of
course the acquired atresias following upon severe labours are not considered here.

Diagnosis. The diagnosis of this vaginal anomaly is not usually a matter of
difficulty, although its extent and the degree of involvement of other organs may
remain doubtful. When, in a young patient with amenorrhoea (cryptomenorrhoea)
and monthly pelvic pain of increasing severity, an abdominal tumour, which fluctuates
and gradually enlarges, is discovered, the presence of vaginal atresia may be suspected ;
and when, in addition, it is found on vaginal examination that the vagina is blocked
either near its orifice or in its upper part, the diagnosis may be safely made. Further
examination by means of rectal touch, aided by the presence of a sound in the
bladder, abdominal palpation, and vaginal touch (when the lower part of the vagina



264 MALFORMATIONS

is patent), is chiefly undertaken to find out the extent of the atresia, the state
of the uterus, and the condition of the ovaries and tubes, so that the proper
treatment may be decided upon. There is, however, a part of the diagnosis which
is extraordinarily difficult, viz. the separation of such cases as women with ovaries,
a rudimentary uterus, and an atresic vagina from those of male pseudo-herm-
aphrodites with cryptorchidism. External appearances are no certain guide, and
ovaries, especially if in an unusual position in the pelvis, can hardly be distinguished



Online LibraryThomas Watts EdenThe new system of gynaecology (Volume 1) → online text (page 24 of 73)