Article Text
Summary
Imperforate hymen is the most frequent cause of haematocolpos, although it is a rare malformation (1:2000). We present two cases of young girls with cyclic abdominal pain and urinary symptoms. At gynaecological examination, they all presented imperforate hymen and ultrasound revealed significant vaginal distension. X-shaped hymenectomy was performed in all patients. The later the diagnosis of imperforate hymen, the higher the risk of complications like haematometra, haematosalpinx, haemoperitoneum and infections such as tubo-ovarian abscesses, peritonitis and endometriosis (retrograde menstruation theory).
- obstetrics, gynaecology and fertility
- congenital disorders
Statistics from Altmetric.com
Background
Woman reproductive tract malformations originate from the disruption of the regular embryological process regarding cellular differentiation, migration, fusions and canalisation. The American Society for Reproductive Medicine differentiates the congenital tract anomalies in six types: mullerian agenesis/hypoplasia; unicornuate uterus; uterus didelphys; uterus bicornuate; septate uterus and diethylstilbestrol-related anomalies.1 The hymen originates from the union of the caudal portion of the paramesonephric ducts and the urogenital sinus. The function of the hymen is not clear; however, it is thought to offer innate immunity as a mechanical barrier to infections during infancy, when vaginal immunity is not totally developed.2 The central portion of this membrane often ruptures before birth. When this does not happen, the hymen becomes imperforate, microperforate, septate or cribiform.
Imperforate hymen is usually an isolated female genital tract malformation. It takes place sporadically but hereditary cases have been known.3 A prenatal diagnosis is very difficult, but through maternal oestrogen stimulation, the fetal blind vagina can fill up with mucous and present as hydrocolpos that can be visualised in the obstetric ultrasound. These findings should be confirmed postnatally. The child usually is asymptomatic because the vaginal mucous is frequently reabsorbed but with menarche blood tends to accumulate. The vaginal distension causes cyclic abdominal or pelvic pain, but urinary symptoms due to uretral distorsion/ureteral compression, low back pain due nervous compression and pain with defecation have also been described. Delayed diagnosis of this entity increases the risk of complications like haematometra, haematosalpinx, haemoperitoneum, infections such as tubo-ovarian abscesses, peritonitis, endometriosis (retrograde menstruation theory), constipation and recurrent urinary tract infection.4 Rare causes of haematocolpos include hymenal, periurethral or vaginal cyst, others vaginal defects, ureterocele, among others. Haematocolpos from an obstructed hemivagina is almost always associated with a homolateral renal agenesia. Therefore, imagiological study of the urinary tract is strongly recommended.5
The purpose of this case report is to remind doctors of the diagnosis of imperforate hymen in adolescents with recurrent pelvic pain.
Case presentation
We present two cases of young girls with cyclic abdominal pain that were treated at our department of gynaecology and obstetrics. They were both admitted to hospital showing similar symptoms:
Girl A, 13 years old, menarche within 6 months before, came to the emergency room with abdominal pain. She referred cyclic abdominal pain prior 6 months. Her sexual development was compatible with Tanner stage III. Perineal examination revealed a brown imperforate hymen. Suprapubic ultrasound showed a normal uterine cavity but a vaginal distension of about 330 cm3 of homogenous thick fluid (figure 1).
Girl B, 14 years old, without menarche, came to the emergency room with painful abdominal distension and dysuria. Abdominal examination showed distension (figure 2) and a tumefaction extending 2 cm above the belly button. Without signs of acute abdomen, the emergency physician sent her for a gynaecological observation. At gynaecological examination, she presented sexual development compatible with Tanner stage III and a pinkish bulging imperforate hymen (figure 3). The endorectal ultrasound revealed vaginal distension filled with low-level echoes fluid about 400 cm3 and normal uterus.
Outcome and follow-up
Both patients underwent hymenectomy (figure 4), performed with a simple star incision in the central portion of the membrane. The edges of the hymen were fixed with Vicryl 2/0 sutures to hold an open incision. Girl A drained about 400 mL and girl B 500 mL of dark blood content. The procedures had no complications and they were discharged from hospital the following day. In the eighth week postoperative visit, they had no issues.
Discussion
Haematocolpos is the vaginal retention of menstruation. Imperforate hymen is the most frequent cause, although it is a rare affection (1:2000).6 Symptoms can be unspecific such as cyclic abdominal pain and dysuria, like girl A, and usually patients are asymptomatic till menarche. Mwenda described a 14-year-old Kenyan girl who presented lower abdominal pain associated with tenesmus and anorexia.7 Other forms of presentation can be an abdominal mass, like girl B, or an haemoperitoneum, as described by Acosta et al (a series of cases of haemoperitoneum due to imperforate hymen which underwent exploratory laparotomy).8 The diagnosis can be made in fetal or neonatal examinations which prevents the symptomatic forms of presentations and its complications in adolescence.9 Hydrometrocolpos is a rare condition in neonates (0.006%). Shaked et al described a 37-week pregnant woman with the incidental ultrasound diagnosis of pelvic cystic mass and bilateral hydronephrosis. At birth, it was confirmed the diagnosis of imperforate hymen. At day 21 of life, it was performed a drainage under ultrasound guidance and the second drainage at day 56. Two weeks later, the ultrasound examination has shown only a minimal amount of fluid in the vagina, no fluid in the uterus and mild left hydronephrosis with a normal right kidney. At 14 months of age, the ultrasound examination was completely normal.10
To resume, the clinical presentation may be amenorrhoea, recurrent abdominal pain, urinary outflow obstruction and its complications (urine retention, hydronephrosis, acute renal failure) and/or intestinal obstructions (constipation or tenesmo).11
The diagnosis of imperforate hymen is clinical and can be complemented with a pelvic ultrasound (abdominal/suprapubic or endorectal). As demonstrated in the pictures, the two girls had bulging imperforate hymens, and imaging examination showed significant vaginal distension, but without any other abnormalities. In these cases, it was only performed abdominal ultrasound but MR imaging can be also important; it can differentiate blood from fluid (with blood from subacute bleeding episodes having high signal intensity on T1-weighted and T2-weighted images) and to the characterisation of possible female genital tract malformations.12 13
Treatment can be performed at any age, but it is preferable at newborn, postpubertal or premenarchal stages because oestrogen stimulation of the tissues is absent, therefore limiting the development of scarring and the possibility of relapse.10
The aim of the surgical treatment is to re-establish vaginal outflow by performing a hymenotomy under local or general anaesthesia. In these cases, this procedure was done under general anaesthesia.
The patients should be stented to minimise the risk of injury of the urethra as for use of the X-shaped incision technique (which was chosen in all cases reported).14 Strain on the uterus with the purpose to discharge more blood is discouraged as it can result in retrograde float through the tubes causing endometriosis and adhesions.
Hymenotomy with a 2-week vaginal catheterisation has also been stated by Acar et al but it is not well tolerated by very young girls.15 Basaran et al described two case reports where vertical sagittal section was used, which made possible to keep the integrity of the hymen, being an option when virginity is cherished by religion, culture and familiar reasons.2 The major complication of this procedure is the formation of a hymenal ring, causing dyspareunia and urinary retention.16
Learning points
In young girls with amenorrhoea and recurrent abdominal pain, congenital genital tract anomalies must be excluded.
Frequently, haematocolpos is the primary sign of these malformations.
An overdue diagnosis can lead to haemoperitoneum, which can lead to more invasive surgical operation, such as exploratory laparotomy.
A potential effect in fertility, due to a greater risk of infections and pelvic endometriosis, must be considered in these patients.
Footnotes
Contributors VFV and BR were responsible for the conception, acquisition and interpretation of data for the case report. HA and IR were responsible for revising it critically and for the final approval of the version to be published.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Patient consent Obtained.
Provenance and peer review Not commissioned; externally peer reviewed.