|Year : 2022 | Volume
| Issue : 4 | Page : 414-420
Hematometra in pubertal girls: A report of two cases and review of literature
Christopher C Amah1, Ijeoma C Obianyo1, Nene E Agugua-Obianyo1, Henry C Nnaji2, Obioma A Okezie2
1 Department of Paediatric Surgery, University of Nigeria, Ituku/Ozalla Campus, Nigeria
2 Department of Obstetrics and Gynaecology, University of Nigeria, Ituku/Ozalla Campus, Nigeria
|Date of Submission||21-Feb-2022|
|Date of Decision||22-Jun-2022|
|Date of Acceptance||27-Jun-2022|
|Date of Web Publication||22-Sep-2022|
Ijeoma C Obianyo
Department of Paediatric Surgery, University of Nigeria, Ituku/Ozalla Campus
Source of Support: None, Conflict of Interest: None
Haematometra in pubertal girls is a late presentation of congenital genital malformations. A diagnosis of the anomaly is preferably made before menarche. This will avoid the psychosocial trauma and other complications at puberty. We draw attention to the two cases we managed, reviewed the literature, discussed the specific pitfalls in our setting and arouse clinical awareness to the problem. two cases of haematometra in pubertal girls are discussed and the literature is reviewed. The first case is a 12-year-old girl who presented with cyclic lower abdominal pain, abdominal mass and no menarche. Following investigations, a diagnosis of cervicovaginal atresia was made. She eventually had hysterectomy done by request. She is awaiting vaginoplasty. The second patient is a 14-year-old girl who presented with primary amenorrhoea, cyclic lower abdominal cramps and abdominal mass. A diagnosis of imperforate hymen was made. The hymen was excised and the vulva (introitus) re-epithelialized. Both patients have been followed up for 4 years and have been symptom free. Our literature review shows that haematometra presenting in pubertal girls is rare and the causes are commonly congenital. It is either due to malformation of the Mullerian duct system, the urogenital system, or the external genitalia. Associated malformation of the urinary system is significant. Haematometra can pose challenges in management especially in a low resource setting. Early diagnosis and multidisciplinary approach are mandatory to proffer adequate treatment and avert psychological, sexual and reproductive health complications.
Keywords: Cervicovaginal atresia, hematometra, imperforate hymen, puberty
|How to cite this article:|
Amah CC, Obianyo IC, Agugua-Obianyo NE, Nnaji HC, Okezie OA. Hematometra in pubertal girls: A report of two cases and review of literature. Int J Med Health Dev 2022;27:414-20
|How to cite this URL:|
Amah CC, Obianyo IC, Agugua-Obianyo NE, Nnaji HC, Okezie OA. Hematometra in pubertal girls: A report of two cases and review of literature. Int J Med Health Dev [serial online] 2022 [cited 2022 Oct 7];27:414-20. Available from: https://www.ijmhdev.com/text.asp?2022/27/4/414/356623
| Introduction|| |
Retention of menstrual blood in the female genital system may present as hematometra (retained in the uterus), hematocolpos (retained in the vagina), hematocolpometra (retained in both uterus and vagina) and hematosalpinx (retained in the fallopian tube). This condition is uncommon. The causes are congenital and acquired. The congenital causes include imperforate hymen, transverse vaginal septum, vaginal atresia, and Mullerian dysgenesis. The acquired causes in pubertal girls include labial agglutination due to infection and poor perineal hygiene; post circumcision labial fusion in our subregion where female circumcision is practiced; and cervical stenosis due to infection or instrumentation. The most common cause is imperforate hymen, and a common presentation is primary amenorrhea at puberty. The congenital causes may occur as isolated developmental anomaly or as part of a syndrome such as Rokitansky–Mayer–Kuster–Hauser (RMKH) syndrome, Bardet–Biedl syndrome, Kaufman–McKusick syndrome, Fraser syndrome, and Winters syndrome. In the syndromic group, RMKH syndrome is the most common type.
We present two cases of hematometra in pubertal girls managed together by pediatric surgeons, gynecologists, and plastic surgeons between the years 2012 and 2014 and discuss the challenges and pitfalls in our subregion, as well as review the English literature.
A 12-year-old girl presented with a 4-month history of cyclic lower abdominal pain lasting 3–4 days at each episode and no menarche. She had noticed progressive swelling of her lower abdomen. On examination, she had normal secondary sexual characteristics. She had a palpable suprapubic mass [Figure 1] which was also evident on digital rectal examination as a pelvic mass bulging into the rectum. At examination under anesthesia (EUA), she had a normal clitoris, labia majora and minora, and a 2.5 cm vaginal pit which ended blindly [Figure 2]. Attempt at aspiration of the hematometra via the vaginal pit was unsatisfactory. The patient was given microgynon to suppress her menstrual cycle. Trans-perineal ultrasonography showed fluid distension of the endometrial cavity and both fallopian tubes. With magnetic resonance imaging (MRI), the vagina was not visualized as a cavity, rather in its plane was an irregular structure of mixed intensity about 45 mm in length and 19 mm in width. The impression then was that of vaginal atresia, hematometra and hematosalpinx. Two months later, her lower abdominal mass and pain became intractable. Evacuation of menstruum via a hysterotomy was done and a catheter was left in situ for a uterine fistulogram [Figure 3]. The fistulogram showed absence of cervix with the uterus ending blindly in the lower segment. The salpinges were blocked. The vagina was not visualized. The diagnosis of cervicovaginal atresia was confirmed. The situation was unbearable for both the parents and the patient who repeatedly requested for hysterectomy. Since the removal of her uterus, the patient has remained asymptomatic. The uterine specimen [Figure 4] shows thickening of the myometrium and endometriosis. She is awaiting vaginoplasty which was deferred by parental request.
|Figure 1: A 12-year-old girl; lower abdominal mass of 4 months’ duration. No menstruation: mass is due to hematometra|
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|Figure 2: The vestibule shows the vulva below the urethral meatus. The vulva is the opening of a 2.5 cm blind-ended vaginal pit|
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|Figure 3: Fistulogram done through hysterotomy. The uterus ended blindly. The salpinges are blocked. The cervix and vagina are not seen|
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A 14-year-old girl who had an emergency admission with a 9-month history of cyclic abdominal pain and progressive lower abdominal distension. For the week prior to admission, she suffered constipation and vomiting. On examination she was in painful distress. She had a firm, tender, lower abdominal mass, which extended to the umbilicus [Figure 5]. A digital rectal examination showed a pelvic mass obstructing the rectum. Her secondary female characteristics were normal. Perineal examination showed a normal clitoris, labia majora and minora, but revealed an imperforate hymen. Abdominal ultrasonography confirmed the diagnosis of hematocolpometra due to imperforate hymen. MRI [Figure 6] reported that there was an hour-glass-shaped mass arising from the pelvis with extension above the umbilical region and tilting slightly to the left lumbar region. There was partial and horizontal septation of the uterine cavity with communication within the upper and lower segments through a 14 mm gap. A diagnosis of massive hematocolpometra due to imperforate hymen and congenital septation of the corpus uteri was made. Intravenous urogram showed normal kidneys and normal upper urinary tract. Cystourethroscopy and micturating cystourethrogram showed normal bladder and urethra. Under general anesthesia, the imperforate hymen was excised; 1.5 L of blood was drained from the utero-vaginal cavity. The vulva (introitus) was re-epithelized [Figure 7]. This is considered important to discourage scar tissue and encourage dilatation. The patient was trained in self-dilatation of the introitus and has remained asymptomatic for 4 years.
|Figure 7: Hymenectomy and re-epithelialization of the vulva; urethral catheter is seen ventrally and rectal sound dorsally|
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| Literature Review and Discussion|| |
Hematometra in young girls refers to retention of menstruum in the uterine cavity. It is a rare condition which commonly manifests at puberty. It is a rare cause of acute abdomen in pubertal girls. It develops when the uterus becomes distended with blood secondary to obstruction to the outflow tract. In most of the cases, the condition becomes apparent during puberty when despite otherwise normal pubertal development, menstruation does not occur. Imperforate hymen, transverse vaginal septum, cervicovaginal atresia, isolated vaginal atresia/vaginal agenesis, cervical agenesis, and incomplete longitudinal vaginal septum coexisting with uterine didelphys are usually the possible congenital causes of hematometra in pubertal girls.,, Anomaly in the development of female lower genital tract can be due to arrest of Mullerian duct development by teratogens, mesenchymal induction defect, sporadic gene mutation, inappropriate production of Mullerian inhibitory substance, or regional absence of estrogen receptor in Mullerian duct.
The urogenital sinus forms the lower third of the vagina, whereas the Mullerian duct system forms the upper two-thirds of the vagina, the cervix, the uterus, and the fallopian tubes. The upper and lower portions of the vagina meet, fuse, and canalize. When hematometra occurs in pubertal girls, attention should be paid to the three systems: the Mullerian duct system, the urogenital system, and the external genitalia. Associated anomalies with the urinary system are common and this calls for thorough investigation. Our two patients had normal urinary systems. The first patient had anomalies of the Mullerian duct system which are cervical and upper vaginal atresias.
Imperforate hymen is the commonest cause of hematometra in pubertal girls. Hymen is the membranous vestige of the junction between the sinovaginal bulb and the urogenital sinus. Imperforate hymen occurs when the sinovaginal bulb fails to canalize with the rest of the vagina. It usually becomes perforate during fetal life to establish a connection between the vaginal lumen and the perineum. The incidence rate is 1 in 1000–2000 females and the occurrence is usually sporadic and typically presents at puberty.
Vaginal atresia, occurs when the caudal portion of the vagina, contributed by the urogenital sinus fails to form, thereby leaving a deformed and non-functional vagina consisting mainly of fibrous tissues. The outflow obstruction may occur at different levels with variation in clinical presentation. In some individuals, the vaginal atresia may extend to the cervix in which case it is called cervicovaginal atresia. It may occur as an isolated developmental defect or part of a complex of syndrome that is sometimes associated with renal and anorectal malformations. The incidence of atresia of the vagina and cervix is 1 in 4,000–10,000.
Transverse vaginal septum is due to faulty fusion or canalization of the urogenital sinus and the Mullerian duct and is uncommon with an incidence of 1 in 70,000 females. About 45% of the transverse vaginal septum occur in the upper vagina, 42% in the mid-vagina, and 15% in the lower vagina. Longitudinal vaginal septum results from defective lateral fusion and incomplete reabsorption of the caudal part of the Mullerian duct. The obstructive variety known as incomplete longitudinal septum can develop hematometra in the hemivagina if it is imperforate.
Cervical agenesis typically presents with agenesis of the upper vagina with a normal uterus. Vaginal agenesis resulting from failure of the urogenital primordium to connect to the urogenital sinus can also occur on its own. If a functional endometrium is present in these cases, hematometra will develop. Patients with incomplete longitudinal vaginal septa may present with irregular bleeding and varying amounts of vaginal discharge.
Basic investigations should be done for pubertal girls presenting with hematometra to establish their general state of health. It should be accompanied by renal ultrasound due to the high incidence of coexistent renal anomalies. MRI is a more accurate diagnostic tool as it can delineate the specific anatomy, the presence of a cervix or transverse septum, and can assist in the diagnosis of other associated malformations.
Other tests that may be necessary include intravenous urogram, laparoscopy, and karyotype to rule out renal pathology and assist in diagnosing other congenital disorders that may be associated with the cause of the hematometra. However, it has been reported that diagnosis can be better confirmed by operation instead of depending on the imaging.
Modalities of treatment usually include psychological, medical, and surgical. The psychological concerns about sexual activity and fertility problems should be dealt with thoroughly to avoid long-term sexual dysfunction.
The roles of medical treatment to temporarily relieve symptoms with analgesic or contraceptive may be necessary to prepare or await surgery. A multidisciplinary surgical team including pediatric surgeons, gynecologists, and plastic surgeons is recommended for challenges of providing good cosmetic results with optimal functional and reproductive outcomes.
The surgery of imperforate hymen is hymenectomy. We prefer to re-epithelize the edges of the vulva after excision of the hymen. This discourages stenosis and encourages dilatation. Hematosalpinx is uncommon with imperforate hymen except in very long-standing cases in which laparotomy may be desirable with the distended tubes being removed or preserved as seen best.
Vaginal septum can be treated with simple excision of the septum. Dilators should be the first choice of treatment in vaginal atresia with a dimple. The principle is that the region occupied by the vagina is a potential space filled with indentation. The patient uses graduated glass dilators 10–20 min for 8–10 weeks. The sexual satisfaction associated with this far exceeds that of operative vaginoplasty.
For pubertal girls with cervicovaginal atresia, conservative surgery with fertility consideration is becoming more and more frequently recommended. Preservation of the uterus should always be attempted as there is always a possibility of pregnancy either spontaneously or through assisted reproductive technology. Basic surgical principle is to create a neovagina. This surgery should be done in the adolescent period to avoid long-standing complications of hematometra and hematosalpinx, which can lead to severe endometriosis, which will further hamper fertility potential. The most widely used surgery is McIndole and Read vaginoplasty. Some authorities have reported the use of serial vaginal dilatation to reduce the length of the atresia prior to surgery. Vecchietti’s operation involves the creation of a neovagina using dilatation of the vaginal dimple with a traction device attached to the abdomen. Follow-up care should emphasize maintenance of an adequate vaginal opening and length to prevent stenosis. Reassessment and progressive sizing of the dilators should continue until the patient is sexually active. The final option of treatment is abdominal hysterectomy with vaginoplasty. The patient may have her own genetic children using ovum retrieval and assisted conception technique with a surrogate mother.
Monitoring and follow-up is underway for our two patients for satisfactory sexual performance, fertility, and reproduction. Clinicians to whom these young girls present with cyclical lower abdominal pain should have a high index of suspicion in order to start the appropriate investigations early to avoid the psychosocial trauma these girls are subjected to. Many young girls in our subregion and with our culture are highly embarrassed and psychologically traumatized at the pubertal ages by perineal inspection and instrumentation including vaginal dilatation. This can be avoided by early diagnosis and treatment before menarche. Early diagnosis and treatment will also avoid the complications of hematometra, which include recurrent infection, endometriosis, pelvic adhesion, and infertility.
| Conclusion|| |
Hematometra in pubertal girls, though rare, poses serious challenges when they occur. Complications of hematometra such as endometriosis and infertility can be averted by early detection and treatment. With current methods of assisted reproduction, hysterectomy should not be an early option, only if all methods of relieving symptoms and pain have failed. However, requests by patient and parents after adequate counseling should be honored as is the case with our 12-year-old.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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