Abstract
We report the case of a patient with hematocolpos on hymenal imperforation, revealed following cyclic pelvic pain with primary amenorrhea associated with pelvic heaviness for one year. MRI showed significant distension of the vagina forming a pseudo mass, well limited, in T1 high signal, in moderate T2 high signal, with the presence of declining deposits in hyposignal related to hemosiderin. A hymenotomy was performed under general anaesthesia, the postoperative course was favourable. This case illustrates the particularity of suspecting a hematocolpos in the presence of a pelvic mass in a young girl with amenorrhea.
Introduction
Hymen imperforation, vaginal atresia, and transverse vaginal septum have been documented as the major causes of congenital vaginal obstruction. Hematocolpos is a progressive accumulation of menstrual blood in the vaginal cavity. It is mainly caused by hymenal imperforation [1].
Hymenal imperforation is rarely diagnosed during the neonatal period and usually presents later in puberty as cyclic pelvic pain, primary amenorrhea or a pelvic mass. The diagnosis of hymenal imperforation can be made by gynecological examination. Ultrasound is decisive for the diagnosis of hématocolpos [2].
MRI is used to confirm the diagnosis and to assess the extent of the disease in search of any associated urogenital malformations [3].
The literature cites rare cases of imperforate hymen with a hematocolpos diagnosed on ultrasound and MRI.
Our case illustrates a hematocolpos secondary to hymenal imperforation diagnosed in a young girl with primary amenorrhea and a pelvic mass.
Observation
This was a 17-year-old girl referred to the gynecology consultation for cyclic pelvic pain with primary amenorrhea associated with pelvic heaviness for one year.
On clinical examination, she had no fever and her blood pressure was normal. A pelvic tuck with a median mass reaching above the umbilicus, tender, firm and mobile was found on abdominal examination. This mass was suspected to be of tumoral origin.
The biological tests (blood and urine) were normal.
A suprapubic ultrasound revealed a median, hypoechoic, retrovesical fluid formation with a fine echogenic content. This collection was surmounted by the undistended communicating uterine cavity.
The MRI showed a significant distension of the vagina forming a pseudo mass, well limited, in T1 high signal (Fig. 1A), in moderate T2 high signal (Fig. 1B), with the presence of declining deposits in hyposignal related to hemosiderin (Fig. 1C). This formation is in diffusion high signal (Fig. 2A) with intermediate ADC (Fig. 2B). It measures 9×9 cm extended by 22 cm in height and displaces the uterus upward. The uterus was of normal size, with regular contours and a small endo uterine retention, without obvious uterine distension.
MRI of the pelvis: Hematocolpos in axial sequence T1 (A), Sagittal sequence T2 (B) and Axial sequence T2 (C). Legends: Star: cervix of the uterus, Red arrow: hematocolpos pushing up the uterus on the sagittal section, Blue arrow: bladder
Citation: Imaging 14, 1; 10.1556/1647.2022.00069
MRI of the pelvis: Hematocolpos in axial sequence T1 (A), Sagittal sequence T2 (B) and Axial sequence T2 (C). Legends: Star: cervix of the uterus, Red arrow: hematocolpos pushing up the uterus on the sagittal section, Blue arrow: bladder
Citation: Imaging 14, 1; 10.1556/1647.2022.00069
MRI of the pelvis: Hematocolpos in axial sequence T1 (A), Sagittal sequence T2 (B) and Axial sequence T2 (C). Legends: Star: cervix of the uterus, Red arrow: hematocolpos pushing up the uterus on the sagittal section, Blue arrow: bladder
Citation: Imaging 14, 1; 10.1556/1647.2022.00069
MRI of the pelvis: Hematocolpos in Axial sequence Diffusion (A) and Axial sequence ADC mapping (B). Legends: Red arrow: hematocolpos pushing up the uterus on the sagittal section, Blue arrow: bladder
Citation: Imaging 14, 1; 10.1556/1647.2022.00069
MRI of the pelvis: Hematocolpos in Axial sequence Diffusion (A) and Axial sequence ADC mapping (B). Legends: Red arrow: hematocolpos pushing up the uterus on the sagittal section, Blue arrow: bladder
Citation: Imaging 14, 1; 10.1556/1647.2022.00069
MRI of the pelvis: Hematocolpos in Axial sequence Diffusion (A) and Axial sequence ADC mapping (B). Legends: Red arrow: hematocolpos pushing up the uterus on the sagittal section, Blue arrow: bladder
Citation: Imaging 14, 1; 10.1556/1647.2022.00069
The bladder was pushed forward in semi-repletion with thin walls and homogeneous contents; the rectum and sigmoid colon were pushed backward. A fluid effusion blade was at the level of the left iliac fossa.
No other utero-annexal abnormalities were found.
The diagnosis of hematocolpos was evoked. The patient was re-examined and the examination of the external genitalia showed an imperforate and bulging hymen. The diagnosis retained was hematocolpos on imperforate hymen. A hymenotomy was performed under general anesthesia, approximately 1 L 400cc was collected and the patient recovered without complications.
Discussion
Hematocolpos is the vaginal retention of menstruation. It is formed at puberty from the first menstruation, and hymenal perforation is the most common etiology. A higher situated complete vaginal diaphragm, or partial vaginal atresia are rarer causes [4]. The prevalence of hymenal imperforation is 0.1% [4] and its familial nature is exceptional [5]. Puberty is the most propitious time for discovery of hematocolpos secondary to hymen imperforation.
The hymenal imperforation could be due to a defect in apoptosis or an inappropriate hormonal environment [1]. Secondary sexual characteristics are present and coexist with primary amenorrhea.
The typical clinical picture is dominated by the presence of cyclic median pelvic pain that may sometimes take on a pseudo-appendicular character, sometimes accompanied by lumbosciatica [5]. A pelvic mass syndrome may, in case of voluminous hematoma, cause urological and/or digestive complications such as urinary retention, dysuria, hydronephrosis or constipation [6].
The consequences of hematocolpos on fertility through the risk of endometriosis or infection are not to be neglected.
The diagnosis is easy to establish clinically and is made during gynecological examinations. In gynecological examinations, an obstruction of the vaginal orifice by an intact, thin, semi-transparent, bulging membrane (hymen) is observed in a patient who has not yet menstruated despite the development of secondary sexual characteristics [4, 6].
Imaging examinations play an important role in the differential diagnosis in case of suspicion of other etiologies.
Indeed, a fine echogenic retro bladder image, surmounted by a small, dilated, communicating uterine cavity with a fluid content in case of hematometra may be observed on suprapubic ultrasound [7]. A hematosalpinx or peritoneal effusion may also be seen [5]. Uterine malformations and renal agenesis in cases of genital duplication are systematically sought [4]. Rarely, Herlyn Werner Wunderlich syndrome with a blind hemivagina, uterine duplication and unilateral renal agenesis is distinguished [2]. Ultrasound may also show ureteropyelocal dilatation in case of compression of the urinary tracts by the hematoscope [8].
Like ultrasonography, MRI has the benefit of innocuousness in a young girl. It would therefore be the best complementary exploration technique giving on T2-weighted sequences a very good anatomical morphological analysis of the malformation. Confirmation of the hematic content in the vagina and in the overlying uterine horn [3, 7] will be evidenced by the T1-weighted sequences.
The treatment of hymenal imperforation is, above all, surgical. It consists of a hymenotomy. Its aim is to drain the hematocolpos and restore vaginal flow. Several incisions have been described: vertical, T-shaped, cross, radial and circumferential incisions [9]. Circumferential incisions should be avoided because they lead to orifice stenosis, a source of dyspareunia. The hymenotomy must meet two requirements: to respect the orifices of Bartholin’s glands and to encourage urethro-hymenal disassociation. In addition, it is recommended not to exert pressure on the uterus during emptying so as not to encourage tube reflux responsible for pelvic endometriosis. A post-menstrual consultation is envisaged to ensure that there is no stenosis and to confirm hymenal healing [10].
Conclusion
Hematocolpos is a more or less complex genital anomaly, and the mode of revelation varies according to the age group.
Imaging allows to orientate the diagnosis, to look for possible associated urogenital malformations and signs of compression, especially urinary. MRI is particularly useful in the study of complex malformations.
Therapeutic management must be early to avoid complications which can be sometimes lethal.
Funding sources
No financial support was received for this case report.
Authors' contribution
Author’s contribution: All persons who meet authorship criteria are listed as authors, and all authors certify that they have participated sufficiently in the work to take public responsibility for the content, including participation in the concept, writing, or revision of the manuscript. Acquisition of images: DD, SA. Interpretation of literature: DD, SA. Drafting the manuscript: DD, SA, BT. Revising the manuscript critically for important intellectual content: BT, LD, CK, SS and EL. Approval of the version of the manuscript to be published: DD, SA, BT, LD, CK, SS and EL.
Conflict of interest
The authors declare no conflict of interest.
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