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Unilateral Report
Hematocolpos of Two Cases ISABEL
C.
and lpsilateral Renal Agenesis: and Review of the Literature
YODER1
AND
RICHARD
Two cases are presented of a partially duplicated vagina characterized by a one-sided hematocolpos associated with renal agenesis on the same side. A review of the English literature reveals 39 previously reported cases. Invariably this complex of anomalies is seen in adolescents and young women with progressively severe abdominal pain, menstrual irregularities, and a pelvic mass. It is important to make an early accurate diagnosis since they are usually managed as having an ovarian mass and submitted to multipIe laparotomies and occasionally to mutilating surgery. An analysis is presented of the salient embryologic, clinical, and radiologic features of the 41 cases gathered. The need for definitive preoperative evaluation of both the urinary tract [urography and cystoscopy] and the genital tract [hysterosalpingography] cannot be overemphasized.
widely
C.
PFISTER1
excised.
Pathology
confirmed
that
this
was
vaginal
epithe-
hum. The brownish deposits were described as organizing thrombus and had disappeared at laparoscopy 4 months later. Seven months after surgery a salpingogram (fig. 5) demonstrated a normal left endocervix through which the left uterine horn and fallopian tube were filled. There was a flattened right cervix with a patulous slitlike os. The right uterine horn and tube were enlarged and distorted due to the previous hematometra. A sinuslike tract provided communication between the two uterine cavities at the level of the endocervix (fig. 6).
We recently had the occasion to study two young women with partial duplicated vagina, one-sided hematocolpos, and ipsilateral renal agenesis. This paper deals with their case histories, a review of the reported cases in the English literature, followed by a discussion of the embryologic, clinical, and radiologic features relevant to this interesting disease complex. Case Case
Reports
1
M. J., a 20-year-old right-sided
pelvic
mass.
white
female,
At
age
was admitted
1 2 she
had
an
in 1 973 with
imperforate
a
hymen
opened. At that time, laparotomy revealed a septate uterus ; a metroplasty was done, and a unognam (fig. 1 ) showed a slightly enlanged single left kidney, with cystoscopy confirming absence of the night hemitnigone. In view of the multiple congenital anomalies, the diagnosis of ovarian cyst was questioned. She underwent salpingography (fig. 2) which demonstrated an irregular filling defect of the fundus reflecting the previous metnoplasty. A sinuslike tract filled at the level of the endocervix and led into a large right paravaginal cavity (fig. 3). At laparotomy a heart-shaped uterus was seen, and a paravaginal cystic mass containing many black oval solid stones was excised. The upper part of this pouch was lined with mucinous columnar epithelium consistent with endocervix; the lining of the lower part was squamous epithelium consistent with vagina. Case 2 B. B., a 15-year-old white female, was admitted with abdominal pain and a large pelvic mass. Unognaphy showed a slightly enlarged single left kidney (fig. 4). No night uretenal orifice was found at
cystoscopy.
Prior to lapanotomy,
an intraoperative
salpingogram
showed a left horn and fallopian tube of a biconnuate uterus. At laparotomy, brownish material was adherent to the right ovary, tube.
and mesentery, resembling endometniosis. A right hematometna and hematocolpos was evacuated and the common vaginal septum I
Pfister,
Department Radiology
Am J Roentgenol
of Radiology. Research
Harvard Office,
127 : 303-308.
Medical
Massachusetts 1976
School
and
General
Massachusetts Hospital,
Boston.
Fig. with
General
1 -Intravenous associated mild
Hospital,
Massachusetts
303
Boston, 021
14.
urogram of case primary megaloureter.
1 demonstrating
Massachusetts
1 4. Address
021
solitary
reprint
left
requests
kidney
to R C
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304
YODER
AND
PFISTER
Fig. 2.-Hysterosalpingogram 1.
Irregular
A,
uterine
of case
cavity
(U)
reflecting
previous metroplasty. B, Tract (arrow) leading from endocervical canal to partially filled paravaginal cystic structure. C and D, drainage views in frontal and lateral
projections.
complete emptied
Literature
Table English ranged
1 summarizes
data
literature and the from 1 1 to 38 years
Review
on the 39 cases two with
reported
in the
present cases. Patient age an average age of 1 7 years.
Clinical presentation was that of a young woman, usually a teenager, who, after going through a normal menarche, began having menstrual irregularities and lower abdominal pain of increasing severity usually associated with menses but occasionally not. On physical examination a pelvic mass of variable size was felt and usually diagnosed as ovarian
in origin,
since
occult
hematocolpos
or
hemato-
left
in 1 2 ; side
No data lateral
were
renal
was
available
agenesis
not
specified
on the kidneys occurred
on
the
in one
case. side
Uniin
20
cases and on the left in nine cases. In these 29 cases, the kidney was invariably absent on the side where the hematocolpos was found. Demonstration of an apparent solitary
unilateral
kidney
on
agenesis
urography
is not
; absence
of the
conclusive ureter
and
evidence renal
agenesis
confirmed
the
remaining
was
of
vessels
36
cornuate in one The classification
the
uterus
uterus
with
double
vagina
Both
cases
as
renal
double,
biconnuate,
two
fundal
cases,
fundus didelphys
two
of
the
[21].
If contrast
presented
demonstrated
here a
resulting
Septate
with
a total horns, an
as well
of the and
into
diagnosis
as eight or
in
cervix,
cervices,
erroneous
communication
septum
duplication is placed
in
or sub-
a single
two
media
uni-
to describe uterus refers
of the median
describes
independent
used
elements
uterus
or
and
for two patients. is confusing. For
cervix.
refers to persistence
In
agenesis urogram
a bicornuate
a single
rerenal
laparotomy.
is loosely
of the
with
in-
vault.
intravenous
in
speaking,
one of these double cavities, unicornuate uterus will result. viewed
the
septate
fusion horns
and
while
of
bicornuate
Strictly
uterus
body
basis
described
term
uterine
of
U=semi-
=vaginal
unilateral
case. No data were given of uterine duplications
entities.
septate
arrow
and/or
probable
cases,
to an incomplete paired
the
was
in
example,
by cystoscopy
on
uterus
didelphys
uterus;
cavity
duplication;
by cystoscopy and angiography, 19 of the 41 cases was unilateral
1 0 cases,
assumed
alone. The
the
in 1 2 cases. right
confirmed In only
several
metrocolpos is seldom considered in a menstruating young woman. The hematocolpos was on the right side in 28 cases and on the
must be spectively.
C=filled
vaginal
of those short
a
only of resinus
HEMATOCOLPOS
tract
between
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cervical
the
duplex
uteri
at the
level
AND
of
the
endo-
were performed on 1 6 of the and eight postoperatively.
41
cases,
RENAL
AGENESIS
305
canal.
Salpingograms eight preoperatively In eight
patients,
surgery
was
confined
to excision
of the
common vaginal septum. The other 33 had at least one laparotomy ; of these, nine had two laparotomies, 1 1 had partial hysterectomies and/or salpingoophorectomies, and two had total hysterectomy. Laparoscopy was done in three patients. In 20 of the patients who underwent laparotomy, some
degree
of
hematometra
and
hematosalpinx
caused
by the backup of retained menses in the site of the hematocolpos was described. Another interesting finding reported in five cases was the presence of brownish deposits studding the pelvic organs and penitoneum, resembling endometniosis. These deposits were adherent to the tissues; however, when our case 2 was examined pathologically, the deposits were descnibed
as old
blood
and
on follow-up
laparoscopy
were
Fig.
no
3.-Drawing
of anatomic
relationships
(case
1).
longer seen. Thus rather than true endometniosis, this finding probably represents a backflow of menstrual blood through the fallopian tube into the penitoneal cavity. Discussion
The incidence of unilateral renal agenesis ascertained at autopsy averages between one in 600 [1 4] and one in 1,200 [22]. Concomitant genital anomalies are very common. In males, anomalies of the genital tract are less frequent and functionally less striking, the most common being cryptorchidism [23]. In females, the incidence of associated genital anomalies has been conservatively estimated
as 50%
[14].
In most
women,
consists and/or
of some degree of duplication vagina, and less commonly
atresia
[9].
the
genital
anomaly
of the uterus, cervix, some form of partial
Embryology Duplication
of the
female
reproductive
tract
results
from
a lack of fusion of the paired mOllenian ducts which appear at the sixth week of embryonic development. The mOllenian ducts develop just lateral to the mesonephnic (urinary) ducts, which have been postulated to act as a guide to the growth of the mOllenian ducts [24]. As they course caudally, the mQllenian ducts cross over the mesonephnic ducts ventrally The cranial
and fusion of the lowermost unfused portions form the
portion paired
occurs. fallopian
tubes; the caudal fused ducts form the uterus, cervix, and approximately the upper three-quarters of the vagina. The distal vagina is formed by invagination of the urogenital sinus
whose
entoderm
then
replaces
the
entire
original
vaginal epithelium [24]. All mammals below the primates exhibit some form of nonfusion of the mallenian ducts as evidenced by usual double uterus and sometimes vagina. However, it is hard to explain the lack of fusion of the human reproductive system as an atavistic step to a more primitive form, since in none of these lower mammals is a kidney absent [9]. Because the urinary and genital systems are derived from a common embryonic mesoderm, it is easy to imagine an early
0’’
..t ..‘ _i
.
Fig
4.-Intravenous
urogram
illustrating
absence
of right
kidney
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306
YODER
AND
PFISTER
ii
-
Fig. 5.-Left vagina
(open
and right arrow)
(postoperative
hysterosalpingogram
tract. B. Dilated septu m removal).
vaginal
of case 2. A. Left uterine horn and tube and early filling of right deformed right
from
Uterus
tube
(arrow),
uterus
(open
didelphys
arrow).
incomplete
with
vaginal
primary
duplication
amenorrhea
and
congenital anomalies vaginal septum causing from
the
lower
vagina
further
postulated side
on the other Toaff [20]
that
of the
in all of his cases paper
of the
that
embryologically
canal
the
the and
as evidence
wall
to
of early
mOllenian
the
the
sinus
two
pouch
begins
seen
reviewed
in this
of this
this
the be
invaginated
communication
resorption
cranially
other
a blind-ending
He offers
resorption
both
with
separating
of 1 0 cases
partial
(L)
vault
of reabsorption of ducts, and it must
isthmic
ducts.
and proceeds
and
urogenital
small
vaginal
associated
leaving
in a total
and duplicated
left
series of patients, extends longitudinally
a lack mOllenian
vagina,
side. views
displaced
are not
up
It must represent wall of the fused
one
and
[25]. In this obstruction
cervices. median only
uterine horn (arrow)
(A),
median
finding
at the
as proof
endocervical
and caudally.
Radiology Hystenosalpinograms Fig. 6.-Drawing tion (preoperative,
of normal case 2).
left
and
dilated
right
genital
duplica-
teratogenic influence leading to simultaneous defects in both systems. Woolf and Allen [9] suggest that the mesonephnic duct is the crux of the matter, and without its normal development a normal mOilenian tract seems unlikely. The above speculations do not entirely explain the
used
unilateral
where
The
lack
hematocolpos of caudal
seen (penineal)
duplicated vagina was hymen in any of these perforate
membrane
(caudal end sinus. Cases
clearly cases.
formed
in
this
opening
series on
of
one
side
patients. of the
not due to an imperforate The hymen is a transverse where
the
mOllenian
tubercle
of the mOllenian ducts) meets the urogenital of simple imperforate hymen usually present
were
done
on
both
of
our
pa-
tients. Case 1 (figs 1-3) had the study done prior to operation, and case 2 (figs. 4-6) had a complete salpingogram done postoperatively. (A modified one had been performed intnaopenatively.) Usually a Swedish vacuum cannula is we
for our had
to
routine
salpingograms.
modify
techniques
tenaculum to pull the The main differential on mesonephnic and
duct
between uterine
incomplete
the
walls normal
However, and
cervix toward diagnosis remnant. hymen
or the
and
epithelium,
while
stratified
squamous
epithelial
the
cysts
ovary
of the
the female and may communicate at any level. Gartner’s cysts are
the
cases,
use
of
the midline. is that of a Gartner’s
ligament.
resorption
in both to
Gartner’s
broad
cuboidal
revert
along They
mesonephnic
with the lined with
vaginal lining.
cyst
occur the
a
anyvagina
represent duct
an in
vagina or uterus a single layer of
duplications Usually
have Gartner’s
a
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HEMATOCOLPOS
AND
RENAL
TABLE Summary
AGENESIS
307
1
of Reported
Cases
Hematocolpos Renal Agenesis
-------
Reference
Right
Hadden[1] Punslow [2]
1 1
Wilson
[3]
Brown
and Brews
Masson
[41
and Mueller
Carrington
Left
and
.
[5]
Burlington
.
ND. ND.
ND. Double
1
ND.
Double
1
ND.
Double
ND.
Didelphys
ND.
... .
.
1
.
[6]
.
.
.
.
1
Martindale [7] Embrey [8J
1 1
Uterus
.
.
.
.
.
.
Surgery
Right hemihysterectomy, vaginal septum incision Total hysterectomy, salpingoophorectomy Laparotomy, vaginal septum incision Laparotomy, excision vaginal septum
Patient died operatively
Didelphys
Partial
Picture
ND. Yes
Double Didelphys
salpingoophorectomy Excision vaginal septum Right hemihysterectomy,
and Allen
Semmens
2
[1 0]
Gibberd Hill
[9]
1
[1 1 1
.
[1 2J
Allen
2
.
.
and Cowan
Thompson
[1 3]
and Lynn
.
3
{1 4]
.
1
.
1
.
.
.
1
Yes
Didelphys
Yes
Didelphys
Yes
Double
Yes
Didelphys
2
Yes, 3 ND., Yes
1
;
1
Johansen
[1 5]
1
.
.
.
1
[1 7]
7
.
.
.
Yes Yes
Yes, 4 ND.,
Didelphys, 3 double, 1 Double, 2 ;
Vinstein Konig Toaff
and Franken
[18]
1
1
[1 9]
Yes
1
[20]
3
Yes
1
Yes
1
3
Didelphys, double,
5 2
Septate, 1 didelphys, Didelphys
;
hemihysterectomy
Lapanotomy,
report
Note -Numbers
2
indicate
number
of patients
N D =no
.
.
.
data
Yes
.
.
.
specimen
sodium
.
Excision
vaginal
Pathology : stratified squamous epithelium
: vaginal
patients
1 ; picture
specimen,
;
1
;
excision 3;
hysterectomy, 1 laparoscopy, 1 Laparotomy, 2 ; excision vaginal septum, 1; laparoscopy, 1
mucosa
reported of gross 3
Pathology, 1 : stratified squamous epithelium
septum,
septum,
iodide
2
2
laparoscopy Laparotomy, 2
Didelphys
Septate, 1 didelphys.
with
1
vaginal
Present
8 days postof peritonitis
4;
Four
and
hemicolpectomy, 3 Laparotomy Laparotomies, hemihysterectomy and hemicolpectomy Laparotomy, 6 ; excision vaginal septum, 6; hemihysterectomy, 1
;
specimen
of gross
painted
Pathology
Laparotomy, 4 ; excision vaginal septum, 4 Laparotomy, 3 ;
;
of gross
septum
Laparotomy, excision vaginal septum Laparotomies, right hemihysterectomy
Unicornuate Didelphys
;
vaginal
Excision vaginal septum, salpingoophorectomy, Excision vaginal septum
bicornuate,
Lewis and Brent Burton [1 6]
Picture
hysterectomy,
excision
Woolf
Comments
Picture
of surgical
specimen,
Pathology,
epithelium
1
1 : vaginal
: ND.
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308
YODER
cysts do not attain the size of a hematocolpos contain blood. Ipsilateral renal agenesis may with Gartner’s duct cysts [26, 27].
AND
and do not also occur
REFERENCES 1 . Hadden 3:526,
2. Purslow
D : Double 1922
and vagina.
CE : A case of unilateral
and hematosalpinx.
3. Wilson
uterus
J Obstet
J Obstet
haematokolpos,
Gynaecol
J St G : A case of double
Am
Gynecol
hematometra
Br Emp 28 : 643,
1922
uterus and vagina with uni-
lateral haematocolpos and hematometra. J Obstet Gynaecol BrEmp 32:127-128, 1925 4. Brown AC, Brews A : Congenital retention of the menses. J Obstet Gynaecol Br Emp 37 : 233-255, 1930 5. Masson JC, Mueller SC : Uterus duplex bicornis with septate vagina open only on one side : report of case. Surg Clin North Am 13:857-861, 1933 6. Carnington GL, Burlington NC : Incomplete bipartite uterus with unilateral hematocolpos and salpingitis. Am J Obstet Gynecol 25 : 924-925, 1933 7. Martindale L : A case of uterus
haematocolpos Lancet 8. Embrey tresia. 9. Woolf quent of the 10.
11. 12.
didelphys
associated
of the right vagina and simulating
with
appendicitis.
1 :1389-1390, 1935 MP : A case of uterus didelphys with unilateral gynaBr Med J 1 : 820-821 , 19S0 RB, Allen WM : Concomitant malformations. The fresimultaneous occurrence of congenital malformations reproductive and urinary tracts. Obstet Gynecol 2 : 236-
265, 1953 Semmens JP : Uterus didelphys
and septate
vagina.
Review;
report of a case with gynatresic right vagina and associated hematoceles. J Obstet Gynecol 8 : 620-626, 19S6 Gibberd AA : Unilateral hematocolpos haematometra. Br MedJ 1 :213, 1957 Hill AM : Uterus didelphys with hematocolpos and hematome-
PFISTER
tra on one side and normal menses from the other. Report of an unusual case. J lnt Coil Surgeons 29 : 422-428, 19S8 1 3. Allan N, Cowan L : Uterus didelphys with unilateral imperforate vagina. Obstet Gynecol 22 : 422-426, 1963 14. Thompson OP. Lynn HB : Genital anomalies associated with solitary kidney, Mayo Clin Proc 41 : 538-548, 1966 1 5. Lewis BV, Brent HA : Obstetric and gynecologic complications associated with mullenian duct abnormalities. Obstet Gynecol 28:315-322, 1966 1 6. Burton E : Bicornuate uterus treated by hemihysterectomy and hemicolpectomy. Proc R Soc Med 61 : 40, 1968 1 7. Johansen K : Uterus didelphys with unilateral haematocolpos. Review of 7 cases. J Obstet Gynecol Br Commonw 79 : 85-89, 1972 1 8. Vinstein AL, Franken EA : Unilateral hematocolpos associated with agenesis of the kidney. Radiology 1 02 : 625-627, 1 972 1 9. Konig HLE : Uterus didelphys and septate vagina presenting as haematocolpos. S Afr Med J 47 : 98-1 02, 1 973 20. Toaff A : A major genital malformation-communicating uteri. Obstet Gynecol 43 : 221 -231 , 1974 21 . Siegler AM : Hysterosalpingography, 2d ed. New York, Medcom Press, 1974 22. Felding C : Obstetric studies in women with congenital solitary kidneys. Acta Obstet Gynecol Scand 44 : 555-562, 196 23. Doroshow LW, Abeshouse BS : Congenital unilateral solitary kidney : report of 37 cases and a review of the literature. Urol Survey 11 :219-229, 1961 24. Arey LB : Developmental Anatomy, 6th ed. Philadelphia, Saunders, 1965, pp 295-341 25. Reed MH, Gniscom NT : Hydrometrocolpos in infancy. Am J Roentgenol 1 18 : 1-1 3, 1973 26. Gadbois WF, Duckett JW : Gartner’s duct cyst and ipsilateral renal agenesis. Urology 4 : 720-721 , 1974 27. Goldstein Al, Ackerman ES, Woodruff A : Vaginal and cervical communication with mesonephric duct remnants : relationship to unilateral renal agenesis. Am J Obstet Gynecol 1 1 6 : 1 01 105, 1973