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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

Unilateral hematocolpos and ipsilateral renal agenesis: report of two cases and review of the literature.

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