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19621964, 1999
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Introduction
Blind hemivagina is a rare malformation which involves
Mullerian and Wolffian ducts. Although the condition was first
recognized in 1922 (Purslow, 1922) and is represented by the
presence of a didelphic uterus and ipsilateral renal agenesis,
the pathogenesis remains unclear and its aetiology is still
unknown. Most reports concerned small series, the maximum
being a series of 36 patients (Candiani et al., 1997). Conservative surgical treatment (excision of the obstructing septum
followed by a marsupialization of the blind hemivagina)
is generally regarded as appropriate. However, long-term
functional results and reproductive performance after surgical
treatment have not been well established. We therefore studied
a series of 42 cases in order to evaluate these parameters.
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35
17
5
2
2
35
15
7
(83)
(40)
(12)
(5)
(5)
(83)
(36)
(17)
25
15
9
1
2
4
15
(61)
(37)
(22)
(10)
(37)
26
4
4
2
1
2
(70)
(11)
(11)
(5)
(3)
(5)
Complete septate
(n 5 2)
Total
n (%)
3
1
0
0
0
1
0
1
1
0
1
1
20
13 (65)
4 (20)
1
3
9 (45)
3
6
4 (20)
1
2 (10)
4 (20)
Results
Surgical treatment assessed after 3 months appeared to be
satisfactory in 39 cases (93%). In three cases (7%), a minor
and ipsilateral stricture was observed at the lateral side of the
vagina, near the fornix, twice after hemihysterectomy and
ipsilateral hemicolpectomy and once after conservative treatment. Further surgery was not required.
Thirty-eight patients (90%) answered the questionnaire.
Dysmenorrhoea and abdominal pain were resolved in 87%
(27/31) and 100% of cases, respectively. Vaginal discharge
was noted in 16% of cases. Dyspareunia was resolved in both
patients (Table I), and no new cases were noted after surgery.
With regard to reproductive performance, eight patients had
not attempted intercourse, 19 did not wish to become pregnant,
two had been trying for 6 and 12 months respectively to
achieve pregnancy. Nine women who had undergone vaginal
septum excision experienced 20 pregnancies after surgical
procedure and the results are outlined in Table IV. Four women
had 4 ipsilateral pregnancies after resection of vaginal septum.
Two of them (with a didelphic and complete septate uterus)
had vaginal deliveries after 37 weeks and two others (with
didelphic uterus) had an early spontaneous abortion and an
ectopic pregnancy. The five patients who underwent hemihysterectomy and ipsilateral hemicolpectomy did not achieve
pregnancy.
Discussion
Uterine malformation, when associated with obstructed hemivagina and ipsilateral renal agenesis, has been generally
described as a double, mainly didelphus, uterus (Rock and
Jones, 1980; Morgan et al., 1987; Stassart et al., 1992; Candiani
et al., 1997). However, the external shape of uterus was
frequently unknown since diagnosis was generally achieved
by hysterosalpingography. Rare cases of septate uterus have
been reported (Vinstein and Franken, 1972; Robert and Le
Charpentier, 1974; Yoder and Pfister, 1976; Rock and Jones,
1980; Chelli et al., 1994). In our study, laparoscopic exploration
showed a complete septate uterus in nine cases (22%).
Ipsilateral renal agenesis is prevalent since the development
of the urinary system parallels that of the genital tract (Muller
et al., 1967; Acien, 1992). However, the presence of normal
kidneys has been reported (Johnson and Hillman, 1986) and
we found a case of a normal urinary tract in our series. It has
been reported that the right one is predominantly involved,
occurring in 66% of cases (Rock and Jones, 1980; Morgan
et al., 1987). However, our results did not confirm this, right
and left sides being equally involved.
Clinical management of blind hemivagina must include
renal imaging by ultrasonography or intravenous pyelography,
both to confirm the absence of a normal kidney in the affected
side and to detect abnormalities in the contralateral urinary
tract (Stassart et al., 1992). Our series included a case of
contralateral vesico-ureteral reflux with a normal kidney which
necessitated surgical treatment. Assessment of uterine malformation by hysterosalpingography may not be beneficial
(Acien, 1997), though it may permit a communicating uterus
to be diagnosed especially in patients with haematic vaginal
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References
Acien, P. (1992) Embryological observations on the female genital tract. Hum.
Reprod., 7, 437445.
Acien, P. (1993) Reproductive performance of women with uterine malformations. Hum. Reprod., 8, 122126.
Acien, P. (1997) Incidence of Mullerian defects in fertile and infertile women.
Hum. Reprod., 12, 13721376.
American Fertility Society (1988) The American Fertility Society
classifications of adnexal adhesions, distal tubal occlusion secondary to
tubal ligation, tubal pregnancies, Mullerian anomalies and intrauterine
adhesions. Fertil. Steril., 49, 944955.
Candiani, G.B., Fedele, L. and Candiani, M. (1997) Double uterus, blind
hemivagina, and ipsilateral renal agenesis: 36 cases and long-term followup. Obstet. Gynecol., 90, 2632.
Chelli, H., Lebbi, I., Ayed, M. et al. (1994) Luterus cloisonne total avec
hemi-vagin borgne et rein dysplasique homolateral. J. Gynecol. Obstet.
Biol. Reprod., 23, 681684.
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