Disclosure Imperforate hymen is the most common and most distal form of vaginal outflow obstruction. As a consequence of normal development, the central portion of the hymenal membrane is usually absent. This absence creates the typical configuration of a ringlike structure at the level of the vaginal vestibule. Persistence of the intact hymenal membrane results in the condition of imperforate hymen. Diagnosis and management of imperforate hymen are straightforward, but an understanding of the differential diagnosis of vaginal outflow obstruction is imperative. For excellent patient education resources, visit eMedicine's Women's Health Center. Also, see eMedicine's patient education articles Amenorrhea and Female Sexual Problems. Problem: The imperforate hymen is a solid membrane interposed between the proximal uterovaginal tract and the introitus. Ambroise Pare first described this condition in 1633 (Wall, 2003). It is classified as a vertical fusion defect. However, it differs from other vertical fusion defects in that it is not derived from the müllerian system. The configuration and normal size of the hymenal orifice of prepubertal girls has received attention in the context of evaluating potential child abuse (Goodyear-Smith, 1998). Anatomic variations of the patent hymen exist, with the most common being an annular or circumferential hymen in which the hymen completely surrounds the vaginal orifice and has a central opening. Other appearances of the hymen include crescentic, fimbriated, septate, cribriform, and microperforate forms. In some patients, perforations do not become confluent, and a cribriform pattern with multiple small perforations may be observed (Berenson, 1994). Vaginal abnormalities in the premenarchal patient include hymenal and vaginal cysts, urethral prolapse, labial agglutination, vaginitis, foreign bodies, and lichen sclerosis Frequency: Imperforate hymen is the most frequent cause of vaginal outflow obstruction, occurring in 0.1% of infant girls. Etiology: The hymen originates from the embryonic vagina buds from the urogenital sinus. As a consequence, the hymen is a composite of vaginal epithelium and epithelium of the urogenital sinus interposed by mesoderm. Once the hymen becomes perforated or forms a central canal, it establishes a communication between the upper vaginal tract and the vestibule of the vagina (Mishell, 1997) (see Image 1). Specific etiologies for the failure to establish patency are not evident. The cause may be related to failure of apoptosis due to a genetically transmitted signal, or it may be related to an inappropriate hormonal milieu. Familial inheritance in successive generations has been described (Stelling, 2000). Pathophysiology: Any obstruction of the vaginal tract during the prenatal, perinatal, or adolescent periods results in the entrapment of vaginal and uterine secretions. In patients with imperforate hymen, this obstruction is at the level of the introitus and becomes evident when the distensible membrane bulges between the labia. Various terms, such as mucocolpos, hematocolpos, and pyocolpos, are used to describe this condition depending on the nature of the retained contents. In fetal development and in the immediate perinatal period, mucoid secretions from the uterovaginal tract result in mucocolpos under the influence of maternal estrogens. When the diagnosis is made in adolescence, the retained secretions consist of menstrual products, and the resulting mass effect in the vagina and uterus are referred to as hematocolpos and hematometrocolpos, respectively. Reflux of the endometrial tissue through the fallopian tubes (ie, hematosalpinx) may result in secondary endometriosis. An accumulation of infected material within the vaginal cavity (ie, pyocolpos) may occur because of an infection that is ascending through microperforations in the membrane. Clinical: Clinical presentations range from an incidental finding on physical examination of an asymptomatic patient to findings discovered on an evaluation for primary amenorrhea or abdominal or back pain. The neonate with imperforate hymen typically presents with a bulging membrane between the labia (see Image 2). The membrane may be white because it is distended from trapped mucoid material secreted as a result of stimulation by maternal estrogen. In severe cases, the distention is in the distal vaginal tract and extends proximally into the uterus. A lower abdominal midline mass may be evident on physical examination because the shallow pelvis of a neonate allows the uterus to be palpated above the pubis symphysis. This mucocolpos can lead to urinary tract infections or bladder obstruction. The fact that most patients with imperforate hymen present during early adolescence suggests that the diagnosis is often overlooked during neonatal examination. In the prepubertal child, an imperforate hymen can be mistakenly diagnosed as labial agglutination or a congenitally absent vagina. The differentiation on gross physical examination is often difficult because of the lack of estrogenization of the perineum. Placing the patient in the knee-chest position aids physical examination in this age group. Have the patient kneel on the examination table with her elbows on the table and her face resting in her hands. Gently spread the buttocks and labia and have the patient exhale or blow (Hewitt, 2003). If the examination is still difficult, sedation or anesthesia may be necessary. If an abnormality is suspected, rectal examination or ultrasonography may help in making the proper diagnosis. When an adolescent presents with primary amenorrhea, careful physical examination is essential. The presence or absence of secondary sexual characteristics should be noted. The most common clinical presentation includes primary amenorrhea. The adolescent with imperforate hymen typically presents with symptoms of lower abdominal or pelvic pain that may initially be cyclical. A thorough history should be obtained, and the patient and family should be questioned about the patient's abdominal or pelvic pain. They should be asked about cyclical pain, a history of vaginal bleeding (which suggests secondary amenorrhea), a family history of genitourinary abnormalities including imperforate hymen, and other factors to determine if any underlying endocrinologic problem is present. During questioning, the patient and family usually recognize a cyclic pattern to the patient's abdominal symptoms. Additional presenting symptoms are back pain, urinary retention (37-60% of patients), and constipation (Nazir, 2006). Although cyclic lower abdominal pain is most frequently reported as the presenting symptom in young women, back pain and urinary retention have also prompted evaluation and resolved after hymenotomy. In patients with a history of amenorrhea, abdominopelvic pain, and secondary sexual characteristics, the most likely diagnosis is obstruction of the uterovaginal tract with consequent hydrometrocolpos (see Image 3). However, the etiology may be the consequence of a variety of developmental anomalies. A lower abdominal mass may be palpated on physical examination, or a pelvic mass may be found on bimanual rectal examination. The diagnosis of imperforate hymen is often established during examination when a distended, bluish membrane is observed at the introitus. In the absence of this finding, only imaging findings can establish the level of obstruction. The differential diagnosis of uterovaginal obstruction includes disorders of vaginal development, such as a transverse vaginal septum or complete vaginal agenesis, which may be associated with other developmental anomalies (eg, Rokitansky-Küster-Maier-Hauser syndrome). Duplication anomalies of the uterovaginal tract often involve 1 tract that is decompressed and 1 that is obstructed. In these patients, abdominal and back pain occurs despite their having a cyclic menstrual period. One must always consider other noncongenital conditions, such as malignancies of the upper or lower genital tract, in the differential diagnosis. Several reports describe the prenatal diagnosis of imperforate hymen. Fetal diagnosis has occurred as early as 25 weeks' gestation. A thin, bulging membrane separating the labia in association with a distended vagina is apparent on sonography (Winderl, 1995). These findings are usually noted during an evaluation for fetal ascites and are thought to be the result of distal urinary tract obstruction. However, they can also be related to reflux of uterine contents through the fallopian tubes. Ascites and bladder outlet obstruction are the most common associated findings in the fetal period (Ogunyemi, 2001). Intestinal, cardiac, and anorectal defects have not been reported in conjunction with imperforate hymen.
Surgical intervention is most often required in the adolescent who presents with symptomatic vaginal outflow obstruction. Establishing a patent hymen is necessary to eliminate pain and discomfort and to establish a functional genital tract. Avoidance of persistent obstruction preserves the patient's fertility by reducing the risk of secondary endometriosis. In the infant with a bulging hymenal membrane due to the effects of maternal estrogen, the diagnosis is evident, and surgical therapy can be undertaken promptly. On occasion, the diagnosis is made serendipitously in asymptomatic premenarchal girls. Intervention can justifiably be delayed until they approach menarche. This delay ensures that a previously nonvisualized orifice, such as an anterior crescentic opening, is absent. The presence of such an opening may obviate surgical intervention.
Relevant Anatomy: Careful evaluation of the perineum of the newborn is essential. Under the influence of maternal estrogens, the female neonate typically has full labia majora. Inspection of the introitus reveals that the hymenal membrane is pink and slightly edematous. The edges of the hymenal membrane may even appear fimbriated. In the newborn with an imperforate hymen, the membrane is often bulging because of retained mucoid secretions. One must distinguish imperforate hymen from a vaginal cyst, which fills the introitus but which is attached to only 1 vaginal aspect. In the symptomatic female adolescent, genital examination typically reveals a bulging, bluish membrane across the vaginal vestibule, which represents the hematocolpos (ie, menstrual products retained in the vagina). If bulging is not noted in the resting state, it may be elicited by having the patient perform a Valsalva maneuver. The diagnosis should not be confirmed by aspirating secretions beyond the obstruction because this procedure may result in iatrogenic pyocolpos. Instead, the diagnosis should be confirmed by performing noninvasive imaging studies (eg, ultrasonography, MRI) to determine the extent of the vaginal outflow obstruction and to diagnose other associated anomalies. Although rare, combined anomalies (ie, imperforate hymen and a transverse vaginal septum) can occur (Ahmed, 1999). Contraindications: Surgical intervention is contraindicated only when the evaluating physician is unfamiliar with the condition and the differential diagnosis and when imaging modalities that help in excluding complicated anomalies of vaginal development are not available. Because evacuation of hematocolpos rarely requires emergency intervention, referral to a tertiary care center should be considered. |
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Medical therapy: Medical therapy has no role in the management of imperforate hymen because the retained secretions are typically sterile. Surgical therapy: Surgical intervention for imperforate hymen should require only 1 definitive procedure to evacuate the retained secretions and to ensure the maintenance of patency. Simple drainage of the material confined beyond the hymen is contraindicated because it does not allow for adequate drainage of the thick fluid, it is not definitive, and it increases the risk of infection (pyometras). Two techniques are most commonly advocated: simple incision and small excision of the membrane. Simple incision of the hymen may be associated with postoperative stenosis with strictures, and it is not the method generally preferred at many centers. Use of an X-shaped incision ought to be the method of choice. An elliptical excision of the membrane is performed close to the hymenal ring, followed by evacuation of the obstructed material. This technique is considered to be most effective in definitive treatment. Avoid compressing the uterus and fallopian tubes to speed evacuation of the trapped contents after the hymen is incised. Preoperative details: After the appropriate diagnostic studies are performed, an outpatient procedure to be done under general anesthesia is scheduled. Distinguishing an imperforate hymen from a transverse vaginal septum is important because the latter requires a relatively extensive procedure to reconstruct a functional vaginal tract and because it has implications in terms of reduced fertility. The clinical and radiologic distinction between the conditions is based on the presence of a thin distal membrane in an imperforate hymen versus a thick, proximal septum in a transverse vaginal septum. Transverse vaginal septum cannot be treated with a cruciate incision, and imperforate hymen does not require a procedure more extensive than hymenotomy. In contrast to imperforate hymen, transverse vaginal septum poses some concern about future pregnancy outcomes. The retained secretions are typically sterile unless previous manipulation (eg, needle aspiration) has resulted in infection. Therefore, prophylactic antibiotics are not usually required. Intraoperative details: The urethra should be identified first, and a catheter can be placed if the patient has had urinary obstructive symptoms. Various ways to make the incision have been discussed. The incision on the hymenal membrane can be made with a scalpel or with an electrocautery device. After the hymenal edges are inspected, the incision is completed, and excess tissue can be resected further if needed. To prevent recurrence, absorbable suture is used to perform formal marsupialization by anchoring the incised membrane to the vaginal wall in several locations. Local anesthetic can be injected into the edges of the hymen to achieve postoperative analgesia. As an alternative, lidocaine jelly can be applied topically. Postoperative details: For postoperative analgesia, acetaminophen or nonsteroidal anti-inflammatory drugs (NSAIDs), such as ibuprofen, are usually sufficient. The patient should anticipate the continued evacuation of retained material for a week. Uterine and/or vaginal cramping should also be anticipated and treated with NSAIDs. No further radiologic or surgical evaluation is necessary after a normal menstrual cycle is established. Follow-up care: Postoperative follow-up is deferred for 6-8 weeks to allow the patient to reestablish a menstrual cycle. Findings on evaluation of the patient's menstrual cycle determine the need for further evaluation.
Incomplete drainage and failure of marsupialization may result in recurrent obstruction and, potentially, an ascending pelvic infection. Although prophylactic antibiotics are not recommended, postoperative fever or abdominal pain must be evaluated and treated promptly. Potential complications include endometritis, salpingitis, or tuboovarian abscess—any of which can affect subsequent fertility. Concern for secondary endometriosis resulting from retrograde menstruation is sufficient for some authors to advocate irrigation of the peritoneal cavity by using a laparoscopic technique. No definitive information regarding the frequency of this condition is available, and most surgeons and gynecologists avoid a concomitant intra-abdominal procedure. Compared with primary endometriosis, secondary endometriosis generally does not become a chronic condition that impairs fertility. Endometriosis is not a uniformly chronic consequence of hematometrocolpos secondary to imperforate hymen. Retrograde menstruation can occur with secondary endometriosis as a result of vaginal outflow obstruction. However, this condition is believed to be self-limited after the primary condition is corrected.
Outcome after repair of imperforate hymen is excellent. If findings on an appropriate preoperative evaluation are normal, a patient can be reassured that her genital tract is otherwise normal. The incidence of dyspareunia is low as well.
Thorough genitourinary examination is essential in girls of all ages from birth through the onset of menarche. If primary amenorrhea is identified or if other structural abnormalities are noted, proper management is essential. By performing these examinations and by promptly diagnosing and treating imperforate hymen, primary care practitioners can help prevent the obstructive symptoms of imperforate hymen and its potential high-risk complications due to delayed care (Posner, 2005). After appropriate evaluation is completed to exclude complicated obstruction of the vaginal tract outflow, management of imperforate hymen is straightforward, and the long-term complications are minimal.
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