The diagnosis and the treatment of congenital malformations of the female genital organs with internal atypical bleeding
Received for publication: 10th of January, 2006
Revised: 14th of February, 2006
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We present a study of 6 cases with rare congenital malformations af the female genital organs with "atipical" bleeding form a period of 10 years (1995-2000). The dramatic clinical symptomatology of hematocolpos, hematometry, hematosalpinx, hematoperineum imposed the surgical intervention of these cases. In 67,2% of the cases, the symptoms were generated by the imperforated himen and in 16,7% of cases, by the vaginal transvers septes. In this paper, can be found remarcs on the diagnosis, the practiced surgical tehniques and on the functional and obstetrical prognosis.
malformations of the female genital organs, internal bleeding
The congenital malformations of the female genital organs represents a special aspect of the gynecology field and has a rare incidence. Considering all these, dealing with such a pathology, implies special problems regarding the surgical techniques and also protecting the sexual and reproductive function.
The congenital malformations can be placed in any segment: vagina, cervix, uterin corpus, tubes, ovaries. In many cases, the malformations affect more than one segment of the female genital organs as in the case of Rokitansky Küstner-Hauser Syndrome, in which, the total or partial absence of the vagina is accompagned by two hemiutherus tied together through a transversal tissue. The tubes can be normal or atrezic and the ovaries can be functionales.
The morfological congenital modifications in the segment’s structure of the female genital organs can produce amenoree, dismenoree or the impossibility of sexual activity, sterility.
The majority of cases are revealed at puberty or at the gynecological consult when the woman comes with the reclaim of sterility or other symptomatology.
Different studies made in our Clinic, on different periods revealed a incidence of congenital malformations placed between 0,02% and 16,4%. In a period of 10 years (1996-2005) we have diagnosticated and resolved 6 cases of congenital malformations of the female genital organs who’s symptom was the atipical bleedings. These cases represent 0,14% of the cases from our Clinic. The particularity of these cases is the internal pseudobleeding.
The patients were at the puberty and postpuberty (12-18 years old).
The psychosomatic growth was a feminin one, according to the age. We noticed the presence of the secondary sexual signs.
In the genital exam of these cases we noticed the presence of the uterus and the ovaies. There has not been noticed other disfunctions of the uroexcretory system.
The clinical and anamnestic symptomatology pointed out the presence of the pelvi abdominale paines in 5 cases (83,33%) and the presence of a pelvine pseudotumorale system which had changes in volume during the crisis.
The imperforated himen, under the shape of a obstructive diaphragm was under pression. It was elastic and had a blue color. We also noticed an expansion during the valsalva maneuver. Associated with this symptomatology we also noticed modifications in the general state during the crisis, urinary dissorders.
In order to put a diagnosis, it is neccesary to inspect and touch the rect. The imposibility of cateterization with the histerometrum and the transabdominal and transperineal echoraphic exam are the ones who confirm the diagnosis (Fig. 1.).
From all the cases:
n 4 (67,2%) – imperforated himen
n 1 (16,4%) – transversal vaginal sept in the hemivagina which comunicates with the “blind” hemivagina
n 1 (16,4%) – closed functional hemiuter
The complications noticed at the studied pacients were:
n hematometry with hematocolpos, 3 cases (50%)
n hematocolpos with hematometry and bilateral hematosalpinx, 1 case (16,4%)
n painfull hemiuter, 1 case (16,4%)
n hematocolpos with hematometry, bilateral hematosalpinx with hematomeprineum, 1 case (16,4%).
From the etiopathological point of view, the imperforated himen, the repellent transversal vaginal sept and the closed functional hemiuter are congenial anomalies who have an incidence of 0,16%. The utero-vaginal malformations are a part of the congenital anomalies of the genito-urinary system and they are not rare at all – 10% of the children are born with a disfunctional genito-urianry system.
The surgical treatment of the imperforated himen can be done by a straight incision with the sclapel. The result is spectacular. We obstain 1000-1500 ml of black cu aspect de smoala. The effect is on long term.
In the case of imperforated vaginal sept, the ablation with a complete excision followed by the hemostasis and the enssurement of the drainage is the best surgical solution.
The closed functional hemiuter requires the surgical ablation which can be performed after attempts of surgical opening of the hemiuterus though vaginal way. In our case, after 2 attempts followed by backsliding, we performed the ablation through abdominal way of the closed hemiuterus (Fig. 2.).
The surgical treatment is always accompagned with profilactic antibiotherapy because of the infection of the overloaded vagina due to the remaining of the secretion and of the menstruation bleeding.
In all the presented cases, the evolution was positive on short and long term. The pacients were examinated even at 3-4 years after the intervention.
The congenital malformations have a frequency of 0,1-0,4% from the gynecological pathology and they can be difficult to resolve.
It’s very important to positive diagnosis the criptomenoree in order to evoid the errors in terapeutical attitude. These errors can induce a useless laparotomy. In these cases, the treatment has to be aplied as quicly as possible in order to evoid the complications such as: hematometry, hematosalpinx, hematoperineum and the complications due to the severe pressure on the closed organs.
The cases have to be monitorized even after the surgical interventions in order to apreciate the evolution of the uro-genitale structure and functions and to prevent the backsliding in the case of imperforated vaginal sept It’s also important to monitorise the impact of the congenital dissorder on the sexual activity, on the fertility and on the pregnancy.
Because the surgical procedure implies loosing the virginity, in the case of a under 18 years old woman, the procedure has to be done with the accept of the parents as well as with documents. Sometimes these cases can become medico-legal cases.
The congenital malformations of the female genital organs are difficult in diagnosting, in surgical behaviour and in functional and obstetrical prognosis.
The surgical dealing with these cases requiers knowledgements of gynecological surgery, embryology and infantile gynecology.
The results obtained with the help of surgical therapy of these cases ensures a pshysical and psysical confort of the pacients.
The postoperatory and prognostic of these pacients suffering with congenital malformations is a good one. The obstetrical prognosis can often be reticent.
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