Virginity-sparing management of hematocolpos with imperforate hymen: case report and literature review.

Imperforate hymen results from failure of the endoderm of the urogenital sinus to completely canalize and has an incidence of 0.01% to 0.05%. This sometimes presents as a pelvic mass that compresses the bladder causing acute urinary retention. A 13-year-old girl was referred to our department with a history of primary amenorrhea, cyclic lower abdominal pain, abdominal-pelvic mass, constipation and acute urinary retention. She had an ultrasonography misdiagnosis of a huge ovarian mass before referral to our unit. On examination, the vagina was bulging and compressing the rectum. Repeat abdominal ultrasonography confirmed the diagnosis of hematometrocolpos. She underwent X-shaped hymenotomy with a favorable outcome. Diagnosis of imperforate hymen requires high suspicion index. Virginity-sparing surgery constitutes a good treatment option for cultural and religious reasons.

Introduction

Imperforate hymen results from failure of the endoderm of the urogenital sinus to completely canalize[1] and has an incidence of 0.01% to 0.05%.[2,3] The presence of primary amenorrhea in an adolescent girl with cyclical abdominal pain, urinary retention, constipation and/or lower abdominal mass is suggestive of the condition.[4] Imperforate hymen could be confused with other obstructive malformations OHVIRA (obstructed hemivagina and ipsilateral renal agenesis) syndrome.[5,6] Hymenectomy after a cruciate, plus or X-shaped hymenotomy is the standard surgical treatment of imperforate hymen.[4,7] Late discovery of imperforate hymen may lead to pain, infection, hydronephrosis, endometriosis and infertility.[8,9] We are presenting a case of imperforate hymen managed at the Douala General Hospital, Cameroon.

Case report

A 13-year-old girl came to our unit with the complaint of primary amenorrhea, cyclic lower abdominal pain, pelvic mass, constipation and acute urinary retention (relieved by catheterization at another hospital 3 days earlier). She was self-medicating with anti-helminthic medication. She had an ultrasonography misdiagnosis of a huge ovarian mass but refused surgery before referral to our unit. Physical examination showed normal secondary sexual development (pubic hair and well-developed breast buds). There was a mobile, slightly tender mass arising from the pelvis and almost bordering the umbilicus, measuring 17 cm from the superior border of the symphysis pubis. On pelvic examination, the external genitalia was normal, and the hymen was closed (Figure 1). The hymen and vagina were bulging and compressing the rectum, and rectal examination revealed a mobile central pelvic mass. A repeat abdominal ultrasonography at our hospital was consistent with an ovoid mid-pelvic sonolucent mass extending from the area of the visible upper vagina to the level just below the umbilicus (Figure 2). A separate uterus could not be seen. The bladder was not distended. The history, physical examination and ultrasonography findings were suggestive of imperforate hymen with hematometrocolpos. After signing the assent form by parents, she underwent X-shaped hymenotomy under general anesthesia. The hymen was thicker than normal and hymeneal leaflets were slightly trimmed with preservation of some tissue all round for virginity preservation. The edges were oversewn with absorbable interrupted sutures to prevent bleeding, enable better scarring and prevent hymen closure. About 700-mL old blood was removed (Figure 3) and the uterus was considerably reduced in size after the procedure. Both ovaries were of normal size on palpation. Normal micturition followed 2 h after removal of the Foley catheter. The patient was discharged from hospital 72 h later and her outcome was uneventful. She became asymptomatic and had regular menstrual cycles at 2 months of follow-up.

Figure 1.

Image of vulva showing imperforate hymen.

Figure 2.

Ultrasonographic image of imperforate hymen.

Figure 3.

Vulva after hymenotomy showing dark bloody secretions.

Discussion

Imperforate hymen is the most common obstructive congenital abnormality of the female genital tract with an incidence between 0.01% and 0.05% in newborns.[2,3] The effect of maternal estrogen secretion either in the prenatal or postnatal period may cause mucus secretion by the cervical glands.[10] Imperforate hymen, transverse vaginal septum and vaginal atresia with or without persistence of a urogenital sinus of cloaca are the common causes of secretory hydrometrocolpos.[11] This condition could rarely be diagnosed in the antenatal period during third trimester ultrasonography.[12] Perforation of the hymen commonly occurs during fetal life or in the perinatal period,[13] but there are reports of spontaneous rupture of the hymen in the adolescent period.[14]

The clinical presentation of imperforate hymen is varied ranging from an incidental finding, midline lower abdominal mass with or without protruding hymen, urinary retention, urinary tract infection, acute renal failure, constipation, acute abdomen with paralytic ileus, primary amenorrhea/cyclical abdominal pain and respiratory distress.[15] Most cases are diagnosed after menarche because of accumulation of blood in the vagina (hematocolpos) and the uterus (hematometra). Urological complications have been reported in more than 50% of cases presenting with complex congenital vaginal malformation either in neonates or during puberty. Hydrometrocolpos and hematometrocolpos cause acute urinary retention from urethral compression/urethral angulation.[15] The main presentation in the index case was cyclical lower abdominal pain, constipation and acute urinary retention.

The differential diagnosis of imperforate hymen is in the context of other obstructive malformations.

During embryonic development, two Mullerian ducts fuse and form a single uterovaginal canal around 8–10 weeks, while the vagina is developed from the uterovaginal canal sinovaginal bulb.[16] Complete patent vagina is formed around the fifth month of embryonic development. The vagina has six different developmental phases, while hymen development has dual origin from sinovaginal bulb and urogenital sinus or from isolated origin of urogenital sinus.[13] It separates the vagina from urogenital cavity and vestibule. Birth defects can occur during development, differentiation, migration, fusion and canalization.[1] Vaginal atresia with hydrometrocolpos may be a part of complex syndromic anomalies which include McKusick–Kaufman syndrome (post-axial polydactyly, hydrometrocolpos from vaginal obstruction and cardiac malformations).[17] Imperforate hymen is a local fusion anomaly with defective resorption of Mullerian septum, and it is a sporadic event. However, the development of mesonephric and paramesonephric ducts and urogenital sinus structures is interdependent, and their paracrine actions also have a role in hymen development. Therefore, hymen anomalies may present with concomitant anomalies of the above structures.[13] Mullerian malformations result from defective fusion of the Mullerian ducts during development of the female reproductive system. The least common form of these malformations is the Herlyn–Werner–Wunderlich (HWW) syndrome.[18] The association of uterus didelphis with OHVIRA (obstructed hemivagina and ipsilateral renal agenesis) syndrome is a rare congenital anomaly constituting about 0.16% to 10% of Mullerian duct anomalies.[18] It is most commonly diagnosed in adolescence due to pelvic and abdominal pain, worsening dysmenorrhea, pelvic mass and an ipsilateral renal agenesis. It can rarely be found in neonates or adults with primary infertility, pyometra, urinary retention and ischiorectal swelling. This condition is rare; therefore, diagnosis may be delayed thereby resulting in complications like endometriosis, pyocolpos and infertility.[19]

Imperforate hymen and OHVIRA syndrome have similar clinical presentation: diagnosis in the adolescent period, although it could also be done in utero or early infancy in cases of hydrometrocolpos. Both conditions may cause urinary obstruction and constipation like in the index case.

The main differences between both conditions are that imperforate hymen patients do not exteriorize the menstrual flow and have normal uteri (indentation depth < 10 mm) according to CUME (Congenital Uterine Malformation by Experts) criteria,[20] while OHVIRA syndrome patients exteriorize their menses, though, some is retained in the hemi-uterus with the septum. Furthermore, OHVIRA syndrome patients have abnormal uterus (indentation depth > 10 mm) according to CUME criteria.[20]

The diagnosis of both conditions is based on good clinical examination and magnetic resonance imaging (MRI). MRI has a high sensitivity compared to ultrasonography or computed tomography (CT) scan in the diagnosis of Mullerian malformations.[21] The index case did not seek medical attention early, but there was misdiagnosis in the first health facility that was corrected later. Some hospitals in Cameroon like in other low-income countries lack qualified or certified radiologists and therefore depend on sonographers without appropriate training.[22,23]

The treatment of imperforate hymen and most Mullerian anomalies is surgical. Virginity-sparing techniques and endoscopic techniques have been described (Table 1). In recent times, a new hymen-sparing management of a blind hemivagina in OHVIRA syndrome with the use of transrectal ultrasound (TRUS)-guided vaginoscopic septoplasty supported by pre- and postoperative diagnostics with the use of three-dimensional saline-solution infusion contrast sonovaginocervicography (3D-SVC) with virtual speculoscopy has been described. This new technique provides good visualization with favorable outcomes. There is need to evaluate the validity of this technique with a larger number of cases managed with the technique.[45]

Table 1:

Literature review and management of women with haematometrocolpos (imperforate hymen and OHVIRA syndrome).

Study (reference)	Description of study	Type of Malformation	Intervention
Yogendra Sanghvi et al.[24]	Case report: 2 cases	Herlyn-Werner-Wunderlich syndrome (HWW)	Endoscopic resection of obstructing septum
Hamidi and Haidary[25]	Case report: 1 case	Late presentation MR imaging, Surgical treatment of Herlyn-Werner-Wunderlich syndrome (HWW)
Pereira et al.[26]	Case report: 1 case	Herlyn-Werner-Wunderlich syndrome (HWW)	Hydrodissection between hemivagina septum and surgical resection of septum
Kapczuk et al.[27]	Case report: 22 cases	Obstructive Müllerian Anomalies in Menstruating Adolescent Girls	Different methods
Gungor et al.[5]	Case report: 1 case	(OHVIRA syndrome) HWW presenting as acute abdomen	Unilateral hysterectomy and vaginal septum resection
Koticha[28]	Commentary	HWW (OHVIRA) and Zinner syndrome ZS (OSVIRA)
Ludwin et al.[20]	Case report	HWW (OHVIRA)	Endoscopy: TRUS guided vaginoscopic septoplasty
Agarwal et al.[29]	Case report: 1 case	OHVIRA syndrome in post-cesarean period
Kamio et al.[30]	Case report: 1 case	OHVIRA with septic shock
Cetin et al.[31]	Case report: 1 case	Imperforate hymen	Annular hymenotomy with electrocautery
Basaran et al.[32]	Case report: 2 cases	Imperforate hymen	Vertical hymenotomy
Buchan et al.[33]	Case report: 1 case	Hematocolpos	Surgery
Saleh et al.[34]	Case report: 1 case	Hematometrocolpos with imperforate hymen disguised as abdominal pain
Noviello et al.[35]	Case report: case series 6 cases	Herlyn-Werner-Wunderlich	One-stage surgical treatment; in one case second look required
El Saman et al.[36]	Case report: 1 case	Segmental vaginal aplasia	Dual-Force Vaginoplasty
Yavuz et al.[37]	Case report: 13 cases	HWW syndrome	Not described
Xu et al.[38]	Case report: 1 case	Herlyn-Werner-Wunderlich syndrome (HWWS)	Hysteroscopic resection of oblique septum
Zivkovic et al.[6]	Case report: 1 case	OHVIRA syndrome
Lakhi et al.[39]	Case report: 1 case	Hematoureter due to endometriosis with hematocolpos and septum	Laparoscopy
Bakacak et al.[40]	Case report: 1 case	Hematometrocolpos due to dysfunctional uterine bleeding following progestin use	Not described
Bhoil et al.[41]	Case report: 1 case	Herlyn-Werner-Wunderlich syndrome	Surgical drainage
Aranke et al.[42]	Case report	Haematometrocolpos and acute pelvic pain associated with cyclic uterine bleeding: OHVIRA syndrome	Not described
Sleiman et al.[43]	Case report: 1 case	OHVIRA syndrome with haematosalpinx and pyocolpos	Not described
Al Ghafri et al.[44]	Case report: 1 case	OHVIRA syndrome	Not described
Ludwin et al.[45]	Case series: 4 cases	Blind hemivagina as a component of OHVIRA syndrome and varying level and features of obstruction including: 1) hemihydrocolpos; 2) hemihematocolpos; 3) “old blood” deposits in small hemivagina; and 4) narrow hymenal opening	Transrectal ultrasound (TRUS)–guided vaginoscopic septoplasty supported by pre- and postoperative diagnostics with the use of a novel ultrasound technique: 3-dimensional saline-solution infusion contrast sonovaginocervicography (3D-SVC) with virtual speculoscopy.
Fidele et al.[46]	Retrospective study of 87 cases	Didelphys uterus with obstructed hemivaginaSeptate uterus with obstructed hemivagina Bicornuate bicollis uterus with obstructed hemivagina Didelphys uterus with monolateral cervical atresia Bicornuate uterus with septate cervix and obstructed hemivagina	Vaginal excision of obstructive septum Vaginal excision of obstructive septum and Hysteroscopic metroplasty Vaginal excision of obstructive septum Laparoscopically assisted cervico-vaginal opening (cervicoplasty) Vaginal excision of obstructive septum
Temizkan et al.[47]	Case report: 2 cases	Virginity sparing surgery for imperforate hymen	Small central circular hymenotomy, forming an intact annular hymen
Yakıştıran et al.[48]	Case report: 1 case	True management of OHVIRA syndrome	Vaginoscopy and hysteroscopy (unipolar needle electrode) without hymenotomy

HWWS: Herlyn-Werner-Wunderlich syndrome; OHVIRA: obstructed hemivagina ipsilateral renal anomaly.

In most cultures worldwide, there is still that strong desire for virginity before marriage.[47] We preserved a rim of hymenal tissue during incision and suturing in order to provide suitable ground for defloration during coitus. Apart from acting as a barrier to infections during the prepubertal period, no other exact functions of the hymen are known.

Conclusion

Early diagnosis of imperforate hymen in adolescent girls is associated with good prognosis; therefore, a thorough examination of the neonate at birth is of paramount importance. Virginity-sparing surgery constitutes a good treatment option because of cultural and religious reasons.